Tetralogi Fallot - Bahan

Penyakit Jantung Sianotik Sianosis adalah . Sianosis akan tampak bila Hb tereduksi > 5 g/dL.
Pada kasus anemia, Hb menurun dan Hb tereduksi < 5 g/dL sehingga tidak akan tampak sianosis. Sianosis dapat disebabkan karena penyakit jantung atau karena syok, atau bronchopneumonia berat. Sianosis dapat dibagi menjadi Sianosis peri!er "erupakan sianosis yang terjadi karena per!usi yang buruk, seperti misalnya saat mandi pagi#pagi akan menyebabkan $asokonstriksi pada peri!er dan terjadi sianosis peri!er. Sianosis peri!er dapat dilihat pada akral. Sianosis sentral "erupakan sianosis yang terjadi %alaupun per!usi masih baik. Sianosis sentral dapat dilihat pada mukosa mulut. Sianosis di!erensial "erupakan sianosis dimana terjadi perbedaan %arna tangan dan kaki. Penyakit jantung sianosis terdiri dari &etralogi 'allot (ouble )utlet *ight +entricle &ransposisi arteri besar Pada re!erat ini hanya akan dibahas tetralogi 'allot. Tetralogi Fallot a. Definisi Secara klasik, tetralogi 'allot terdiri dari +entricular Septal (e!ect ,+S( Pulmonal Stenosis )$erriding aorta Hipertro!i $entrikel kanan b. Patofisiologi c. Manifestasi klinik d. Diagnosis (ari anamnesis didapatkan Sianosis S.uatting pada anak besar (ari pemeriksaan !isik didapatkan Sianosis /unyi 0antung 1 terdengar tunggal /ising Sistolik ejection murmur (ari pemeriksaan roentgen thora2 didapatkan &idak ada kardiomegali Penurunan corakan $askuler paru Segmen pulmonal cekung /oot shaped e. f. Terapi Komplikasi 3erebro$ascular accident, terjadi setelah serangan sianotik 4bses otak 5ndokarditis en!ekti!
g.
4nemia relati! &rombosis paru Perdarahan Prognosis
Tetralogy of Fallot
Contents of this page:

Illustrations Alternative names Definition Causes, incidence, and risk factors Symptoms Signs and tests Treatment Expectations (prognosis) Complications Calling your ealt care provider !revention "eferences
Illustrations
#eart, section Tetralogy of t roug t e $allot middle Alternative names TET& T'$ Definition
Return to top
Cyanotic %Tet spell%
Return to top
Tetralogy of $allot refers to four types of eart defects present at (irt (congenital)) Causes, incidence, and risk factors
Return to top
Tetralogy of $allot is classified as a cyanotic eart defect (ecause t e condition causes too little oxygen levels in t e (lood, * ic leads to cyanosis (a (luis +purple coloration to t e skin)) T e classic form of Tetralogy includes , defects *it in t e eart structures .entricular septal defect ( ole (et*een t e rig t and left ventricles) /arro*ing of t e pulmonary outflo* tract (tu(e t at connects t e eart *it t e lungs) An aorta (tu(e t at carries oxygenated (lood to t e (ody) t at gro*s from (ot ventricles, rat er t an exclusively from t e left ventricle A t ickened muscular *all of t e rig t ventricle (rig t ventricular ypertrop y)
At (irt , infants may not s o* t e signs of t e cyanosis, (ut later may develop sudden frig tening episodes (called 0Tet spells0) of (luis skin from crying or feeding) Tetralogy of $allot occurs in approximately 1 out of 23,333 infants) T e cause of most congenital eart defects is unkno*n) 4ultiple factors seem to (e involved) !renatal factors associated *it ig er t an normal risk for t is condition include maternal ru(ella or ot er viral illnesses during pregnancy, poor prenatal nutrition, maternal alco olism, mot er over ,3 years old, and dia(etes)
T ere is a ig incidence of c romosomal disorders in c ildren *it tetralogy of $allot, suc as Do*n syndrome and Di 5eorge%s syndrome (a partial gene deletion t at results in eart defects, lo* calcium levels, and immune deficiency)) Symptoms
Return to top
Difficult feeding (poor feeding a(its) $ailure to gain *eig t !oor development Cyanosis * ic (ecomes more pronounced during periods of agitation !assing out Sudden deat Clu((ing of fingers (skin or (one enlargement around t e finger nails) S6uatting during episodes of cyanosis
Return to top
Signs and tests
A p ysical examination *it a stet oscope almost al*ays reveals a eart murmur) Tests may include E75 (electrocardiogram) may s o* t e t ickening of t e rig t ventricle muscle C8C may s o* an increase in red (lood cells C est x+ray may s o* a 0(oot s aped0 eart and dark lungs Cardiac cat eteri9ation elps s o* (lood vessels in t e lungs and eart Ec ocardiogram provides a definite diagnosis
Return to top
Treatment
Surgery to repair eart defects is al*ays done * en t e infant is very young) Sometimes more t an one surgery is needed) T e first surgery may (e done to elp increase (lood flo* to t e lungs, and a surgery to correct t e pro(lem is done at a later time) Corrective surgery is done to *iden part of t e narro*ed pulmonary tract and close t e ventricular septal defect) Tips for parents of c ildren *it tetralogy of $allot If a c ild does (ecome (lue, immediately place t e c ild on is or er side and put t e knees up to t e c est) Calm t e (a(y and seek medical attention $eed t e c ild slo*ly 5ive smaller, more fre6uent meals Decrease t e c ild%s anxiety (y remaining calm 4inimi9e crying (y trying to anticipate t e c ild%s needs "ecruit ot ers to care for t e c ild to prevent yourself from (ecoming ex austed
Return to top
E pectations !prognosis"
4ost cases can (e corrected *it surgery) 8a(ies t at ave surgery usually do *ell) :it out surgery, deat usually occurs * en t e person reac es age ;3) !atients * o ave continued, severe leakiness of t e pulmonary valve may need t e valve replaced) "egular follo* up *it a cardiologist to monitor for life+t reatening arr yt mias (irregular eart r yt ms) is recommended) Complications
Return to top
Delayed gro*t and development
Sei9ures during periods of insufficient oxygen

Return to top
Calling your health care provider
Call your ealt care provider if ne* unexplained symptoms develop or if t e patient is aving an episode of cyanosis ((lue skin)) #revention
Return to top
T ere is no kno*n prevention) $eferences

Return to top
To*nsend C4, 8eauc amp "D, Evers 84, 4attox 7<) Sabiston Textbook of Surgery: The Biological Basis of Modern Surgical Practice) 2=t ed) St) <ouis, 4'- :8 Saunders& ;33,-2>;?+2>;1) @ipes D!, <i((y !, 8ono* "', 8raun*ald E, eds) Braunwald's Heart =t ed) St) <ouis, 4o& :8 Saunders& ;331-2121) isease: ! Textbook of "ardio#ascular Medicine ,
%pdate Date: &'()'(**&

Apdated (y- Anne B) <) C un, 4)D), Assistant !rofessor of !ediatrics, Division of !ediatric Cardiology, /e* Cork Aniversity Sc ool of 4edicine, /e* Cork, /C) "evie* provided (y .eri4ed #ealt care /et*ork)
http //%%%.nlm.nih.go$/medlineplus/ency/article/667589.htm
Cardiac catheteri+ation
Contents of this page:

Illustrations Alternative names Definition #o* t e test is performed #o* to prepare for t e test #o* t e test *ill feel : y t e test is performed : at a(normal results mean : at t e risks are Special considerations
Illustrations
Cardiac Cardiac cat eteri9ation cat eteri9ation Alternative names

Return to top
Cat eteri9ation + cardiac& #eart cat eteri9ation Definition

Return to top
Cardiac cat eteri9ation involves passing a cat eter (a t in flexi(le tu(e) into t e rig t or left side of t e eart) In general, t is procedure is performed to o(tain diagnostic information a(out t e eart or its (lood vessels or to provide treatment in certain types of eart conditions) Cardiac cat eteri9ation can (e used to determine pressure and (lood flo* in t e eart%s c am(ers, collect (lood samples from t e eart, and examine t e arteries of t e eart *it an x+ray tec ni6ue called fluoroscopy) $luoroscopy provides immediate (0real+time0) visuali9ation of t e x+ray images on a screen and provides a permanent record of t e procedure) ,o- the test is performed
Return to top
Cou *ill (e given a mild sedative prior to t e test to elp you relax) An intravenous (I.) line is inserted into one of t e (lood vessels in your arm, neck, or groin after t e site as (een cleansed and num(ed *it a local anest etic) A cat eter is t en inserted t roug t e I. and into your (lood vessel) T e cat eter is carefully t readed into t e eart using an x+ray mac ine t at produces real+time pictures (fluoroscopy)) 'nce t e cat eter is in place, contrast material is inDected and pictures are taken) ,o- to prepare for the test
Return to top
$ood and fluid are restricted E to > ours (efore t e test) T e procedure takes place in t e ospital and you *ill (e asked to *ear a ospital go*n) Sometimes, admission t e nig t (efore t e test is re6uired) 't er*ise, you *ill (e admitted as an outpatient or an inpatient t e morning of t e procedure) Cour ealt care provider s ould explain t e procedure and its risks) A *itnessed, signed consent for t e procedure is re6uired)
Tell your doctor if you are allergic to seafood, if you ave ad a (ad reaction to contrast material in t e past, if you are taking .iagra, or if you mig t (e pregnant) ,o- the test -ill feel
Return to top
T e study is carried out in a la(oratory (y a trained cardiologist or radiologist and tec nicians or nurses) Cou *ill (e a*ake and a(le to follo* instructions during t e cat eteri9ation) A mild sedative is usually given ?3 minutes (efore t e procedure to elp you relax) T e procedure may last from 2 to several ours) Cou may feel some discomfort at t e site * ere t e I. is placed) <ocal anest esia *ill (e used to num( t e site, so t e only sensation s ould (e one of pressure at t e site) Cou may experience some discomfort from aving to remain still for a long time) After t e test, t e cat eter is removed) Cou mig t feel a firm pressure at t e insertion site, used to prevent (leeding) If t e I. is placed in your groin, you *ill usually (e asked to lie flat on your (ack for a fe* ours after t e test to avoid (leeding) T is may cause some mild (ack discomfort) .hy the test is performed
Return to top
Cardiac cat eteri9ation is usually performed to evaluate eart valves, eart function and (lood supply, or eart a(normalities in ne*(orns) It may also (e used to determine t e need for eart surgery) T erapeutic cat eteri9ation may (e used to repair certain types of eart defects, open a stenotic eart valve, and open (locked arteries or grafts in t e eart) .hat a/normal results mean
Return to top
T e procedure can identify eart defects or disease, suc as coronary artery disease, valve pro(lems, ventricular aneurysms, or eart enlargement) T e procedure also may (e performed for t e follo*ing !rimary pulmonary ypertension !ulmonary valve stenosis !ulmonary em(olism Tetralogy of $allot Transposition of t e great vessels Tricuspid regurgitation .entricular septal defect
Return to top
.hat the risks are
Cardiac cat eteri9ation carries a slig tly increased risk * en compared *it ot er eart tests) #o*ever, t e test is very safe * en performed (y an experienced team) 5enerally, t e risk of serious complications ranges from 2 in 2,333 to 2 in 133) T e risks include t e follo*ing Cardiac arr yt mias Cardiac tamponade Trauma to t e artery caused (y ematoma <o* (lood pressure "eaction to contrast medium #emorr age Stroke #eart attack
Considerations associated *it any type of cat eteri9ation include t e follo*ing In general, t ere is a risk of (leeding, infection, and pain at t e I. site) T ere is al*ays a very small risk t at t e soft plastic cat eters could actually damage t e (lood vessels) 8lood clots could form on t e cat eters and later (lock (lood vessels else* ere in t e (ody) T e contrast material could damage t e kidneys (particularly in patients *it dia(etes))
Return to top
Special considerations
Cardiac cat eteri9ation may include coronary angiograp y)
%pdate Date: )'0)'(**&

Apdated (y- 5lenn 5andelman, 4D, 4!#, Assistant Clinical !rofessor of 4edicine, /e* Cork 4edical College, .al alla, /C) "evie* provided (y .eri4ed #ealt care /et*ork)
http //%%%.nlm.nih.go$/medlineplus/ency/article/66:;7<.htm
"ate t is Article
Tetralogy of Fallot With Pulmonary Atresia

1ast %pdated: Buly 2=, ;33E
Email to a Colleague 5et C4EFCE for article
Synonyms and related key-ords: tetralogy of $allot, T'$, tetralogy of $allot *it pulmonary atresia, T'$+!A, pulmonary atresia *it ventricular septal defect, .SD, end+stage tetralogy of $allot, $allot tetralogy, $allot%s tetralogy, $allot tetrad, $allot%s tetrad
A%T,2$ I3F2$4ATI23
Section 0 of 00
Aut or Information Introduction Clinical Differentials :orkup Treatment 4edication $ollo*+up 4iscellaneous !ictures 8i(liograp y
Aut or- 4ichael #ettersen, 4D, Director of Ec ocardiograp y, Assistant !rofessor, Department of !ediatrics, C ildren%s #ospital of 4ic igan, :ayne State Aniversity Sc ool of 4edicine Coaut or(s)- Aparna 5ulkarni, 4D, $ello*, Department of Cardiology, C ildren%s #ospital of 4ic igan 4ic ael !ettersen, 4D, is a mem(er of t e follo*ing medical societies- American Academy of !ediatrics, American #eart Association, and American Society of Ec ocardiograp y Editor(s)- Ira , 6essner, 4D, !rofessor, Department of !ediatrics, Aniversity of $lorida College of 4edicine& 4ary 1 .indle, #harmD, AdDunct Assistant !rofessor, Aniversity of /e(raska 4edical Center College of ! armacy, ! armacy Editor, e4edicine)com, Inc& Ameeta 4artin, 4D, Associate !rofessor, Department of !ediatrics, Section of !ediatric Cardiology, Aniversity of /e(raska College of 4edicine& 6il/ert ,er+/erg, 4D, Assistant !rofessor, Department of !ediatrics, Section of !ediatric Cardiology, /e* Cork 4edical College& and Stuart 7erger, 4D, !rofessor of !ediatrics, Division of Cardiology, 4edical College of :isconsin& C ief of !ediatric Cardiology, 4edical Director of !ediatric #eart Transplant !rogram, 4edical Director of T e #eart Center, C ildren%s #ospital of :isconsin Disclosure
I3T$2D%CTI23
Section ( of 00
7ackground: Tetralogy of $allot (T'$) is comprised of a malaligned ventricular septal defect (.SD), anterior s ift of t e aorta over t e .SD (overriding aorta), o(struction of t e rig t ventricular outflo* tract, and rig t ventricular ypertrop y) !ulmonary atresia (!A) *it .SD is considered t e extreme end of t e anatomic spectrum of T'$) T'$+!A is *ort y of separate consideration) 8ecause of t e *ide varia(ility of pulmonary (lood supply, diagnosis and surgical management of T'$+!A is more difficult t an t at of classic T'$) Em/ryology T e lungs develop from t e foregut and carry t eir nutrient supply from t e paired dorsal aortae) T e paired sixt aortic arc es also give rise to (ranc es t at form an anastomosis *it t e pulmonary
vascular tree on day ;= of gestation) 'ver time, t e (ranc es from t e descending t oracic arc (ecome smaller, and t e sixt aortic arc (ecomes larger) T e aorta and pulmonary arteries form from t e distal (ul(us cordis and t e truncus arteriosus, * ic are positioned a(ove t e rig t ventricle) T e (ul(otruncal ridges separate t e great arteries, and t e aortic component rotates posteriorly) #o*ever, faulty rotation of t e (ul(us+truncus in T'$ results in incomplete transfer of t e aorta a(ove t e left ventricle) 4alalignment of t e infundi(ular septum to t e tra(ecular septum is present, resulting in a malalignment .SD) Anterior displacement of t e (ul(otruncal region as (een postulated to cause t e infundi(ular stenosis) Anot er t eory t at as (een suggested to cause T'$ is underdevelopment of t e su(pulmonic infundi(ulum t at results in maldevelopment of t e conal septum) <ittle or no evidence exists to support t is ypot esis, o*ever) Anatomy Anatomy of t e pulmonary arteries and t e source of pulmonary artery (lood supply may (e ig ly varia(le in T'$+!A) !ersistence of descending t oracic (ranc es accounts for t e a(normal pulmonary arterial supply in t is condition) 4aDor aortopulmonary collateral arteries may anastomose at any site in t e pulmonary vascular tree) 4ost fre6uently, t e rig t and left pulmonary arteries are patent and maintain free communication *it eac ot er& t ey are termed confluent pulmonary arteries) T e pulmonary arteries may also (e ypoplastic and nonconfluent) /o antegrade (lood flo* is present from t e rig t ventricle to t e pulmonary arteries) T e ductus arteriosus (DA) often is an important source of (lood supply, alt oug occasionally it is a(sent) Classification of !A+.SD depends on t e predominant source of (lood supply to t e (ronc opulmonary segments) T ese range from t e native confluent pulmonary arteries supplied solely (y t e DA to nonconfluent pulmonary arteries *it multiple maDor aortopulmonary collaterals supplying pulmonary (lood flo*Gtook out for consistencyH) "are sources of pulmonary (lood flo* include an aortopulmonary *indo*, a persistent fift aortic arc , and coronaryItoIpulmonary artery fistulae) Identification of t e pulmonary arterial supply is essential in planning t e type of surgical repair) #athophysiology: Clinical presentation in T'$+!A depends on t e source and volume of pulmonary (lood flo*) T is usually occurs via t e DA andFor aortopulmonary collaterals) T e ne*(orn infant, in * om t e DA is t e sole source of pulmonary (lood flo*, (ecomes increasingly cyanotic as t e DA closes) Early recognition of t e diagnosis along *it prompt institution of prostaglandin E2 (!5E2) infusion is life saving in t is instance) Conversely, * en t e aortopulmonary collaterals constitute t e source of pulmonary (lood flo*, t e clinical presentation may vary from cyanosis *it inade6uate pulmonary (lood flo* to no cyanosis *it increased pulmonary (lood flo*) Ancommonly, pulmonary (lood flo* is increased sufficiently to cause symptoms due to pulmonary overcirculation) 'lder infants and c ildren commonly present *it cyanosis) #ypoxia usually progresses furt er as t e c ild outgro*s t e source of pulmonary (lood flo*) Early surgical intervention as improved survival in t ese patients) Fre8uency:
In the %S: T e 8altimore :as ington Infant study reported an incidence of 3)3= per 2333 live (irt s for T'$+!A) T is condition accounts for 2)1J of all forms of congenital eart disease and ;3J of all forms of T'$)
4ortality'4or/idity: Survival (efore t e advent of modern surgical tec ni6ues occurred rarely, *it less t an 1J of patients reac ing age ;1 years) In patients *it opera(le pulmonary arteries, survival rates *it satisfactory 6uality of life no* reac K3J)
!atients *it inade6uate pulmonary (lood flo* and marked cyanosis develop complications affecting multiple organ systems, including ematologic, skeletal, renal, and neurologic, causing significant mor(idity and mortality) In patients *it large aortopulmonary collaterals and excessive pulmonary (lood flo*, C#$ may result in failure to t rive) !atients *it T'$+!A and nonconfluent !As are su(Dect to increased mor(idity and mortality related to t e fre6uent need for multiple cardiac surgeries) T e risks of cardiopulmonary (ypass and anest esia are present at eac stage of t e repair)
$ace: /o kno*n race predilection exists) Se : /o specific male or female preponderance of T'$+!A as (een noted) Age: T'$+!A (ecomes symptomatic at (irt in most cases) Diagnosis usually occurs at t is time)
C1I3ICA1
Section 9 of 00
,istory: Clinical presentation is varia(le and largely dependent on t e source and volume of pulmonary (lood flo*)
An infant *it tetralogy of $allot *it pulmonary atresia (T'$+!A) is often symptomatic *it in t e first ours to days of life)
o
Severe cyanosis (ecomes apparent immediately after (irt as t e DA (egins to close) In t e presence of significant aortopulmonary collaterals, cyanosis may (e mild to moderate) If ade6uate collaterals or additional sources of pulmonary (lood flo* are lacking, closure of t e DA may produce ypoxemia too severe for survival) 'n rare occasions, patients *it *ell+developed aortopulmonary collaterals or persistent patency of t e DA may present *it eart failure) Symptoms develop several *eeks after (irt as pulmonary vascular resistance decreases and pulmonary (lood flo* increases) T e older infant and c ild *it ade6uate pulmonary (lood flo* supplied (y aortopulmonary collaterals presents *it a istory of cyanosis) Impaired exercise tolerance and gro*t failure may occur)
!atients * o ave undergone palliative surgical procedures may present *it varia(le symptomatology)
o
4ost palliative procedures are intended to augment pulmonary (lood flo* (y placement of systemic+to+pulmonary artery s unts) T ese s unts may distort t e pulmonary vasculature or may cause stenosis and result in ypoxia)
Elevated pulmonary vascular resistance as (een noted in t e presence of large systemic+to+pulmonary connections) T is pro(lem *as prevalent *it t e :aterston (direct anastomosis of t e ascending aorta to t e pulmonary artery) and t e !otts (direct anastomosis of t e descending aorta to t e pulmonary artery) s unts, (ot of * ic ave (een largely a(andoned)
#hysical:
! ysical findings vary according to t e source and volume of pulmonary (lood flo*)
o
'(vious profound cyanosis may (e noted in t e neonatal period) T is (ecomes severe as t e ductus narro*s) !atients *it significant aortopulmonary collaterals may (e mildly cyanotic initially (ut (ecome increasingly cyanotic if t ey outgro* t eir source of pulmonary (lood flo*) !erip eral pulses and (lood pressures are usually normal during t e first fe* days of life) !atients *it increased pulmonary (lood flo* may (e noted to ave (ounding pulses) Auscultation reveals a normal first eart sound *it a single second eart sound) A systolic murmur may (e present at t e left lo*er sternal (order) T e typical rig t ventricular outflo* tract murmur of classic T'$ is not eard) A soft continuous murmur from t e DA may occur at t e left (ase) A continuous murmur from t e aortopulmonary collaterals may (e eard in t e (ack) 5ro*t and development often are delayed)
Causes:
4any patients *it T'$+!A ave associated syndromes and extracardiac malformations)
o
Conotruncal cardiac malformations associated *it a c romosome arm ;;622 deletion ave (een incorporated under an acronym of CATC#;; (cardiac defect, a(normal face, t ymic ypoplasia, cleft palate, ypocalcemia, microdeletion of (and ;;622)) !atients *it T'$+!A ave a ig er incidence of t is syndrome t an patients *it classic T'$) T e prevalence of deletion ;;622 is 2EJ in T'$+!A *it confluent pulmonary arteries and ,2J in patients *it T'$+!A and multiple aortopulmonary collateral arteries) 't er syndromic associations include .ATE" syndrome (verte(ral defects, anal atresia, trac eoesop ageal fistula *it esop ageal atresia, and renal and radial anomalies), C#A"5E syndrome (colo(oma, eart disease, atresia c oanae, retarded gro*t and retarded development andFor C/S anomalies, genital ypoplasia, and ear anomalies andFor deafness), Alagille syndrome, cat%s+eye syndrome, de <ange syndrome, 7lippel+ $eil syndromes, and trisomy ;2) 4aternal dia(etes mellitus& maternal p enylketonuria& and maternal ingestion of retinoic acid, trimet adione, or sex ormones increase t e risk of conotruncal a(normalities) Infants of mot ers *it dia(etes mellitus ave a ;3+fold ig er risk t an infants of mot ers *it out dia(etes mellitus)
T e recurrence risk of si(lings *it T'$ is ?+,J) T e recurrence risk increases furt er if syndromic variants are present) .aria(le patterns of in eritance may (e o(served)
Section : of 00
DIFFE$E3TIA1S
#eterotaxy, Asplenia #eterotaxy, !olysplenia !ulmonary Atresia :it Intact .entricular Septum !ulmonary Stenosis, .alvar Total Anomalous !ulmonary .enous Connection Transposition of t e 5reat Arteries Tricuspid Atresia 2ther #ro/lems to /e Considered: Dou(le outlet rig t ventricle *it severe pulmonary stenosis or atresia Single ventricle *it severe pulmonary stenosis or atresia 1a/ Studies:

'(tain a complete (lood cell count to determine emoglo(in and ematocrit) In infants * o are sick, an arterial (lood gas can assess p';, acid+(ase status)
Imaging Studies:
T e c est radiograp depicts a normal+si9ed (oot+s aped eart *it decreased pulmonary vascular markings) A concavity in t e region of t e main pulmonary artery is o(served) Approximately ;E+13J of t ese patients ave a rig t+sided aortic arc ) Increased pulmonary vascularity may (e o(served in t e presence of large aortopulmonary collaterals) T*o+dimensional ec ocardiograp y *it color flo* and ;+dimensional Doppler is t e most important tool in t e diagnosis)
o
T e parasternal long axis vie* reveals a large aortic valve t at overrides a large malalignment .SD) T*o+dimensional and color flo* imaging demonstrates lack of patency of t e rig t ventricular outflo* tract) T e suprasternal and ig parasternal vie*s provide information regarding t e pulmonary trunk, rig t and left pulmonary artery si9e, and t eir confluence) T e pulmonary arteries usually appear ypoplastic and may not (e visuali9ed at all) Color+flo* imaging identifies sources of pulmonary artery (lood flo* including t e DA and aortopulmonary collaterals) Significant ypoplasia of t e central pulmonary arteries or presence of a small patent ductus arteriosus (!DA) is ig ly predictive of t e presence of aortopulmonary collaterals) If collaterals are suspected, ec ocardiograp y
alone is inade6uate for complete delineation of pulmonary (lood flo*, and furt er imaging (y 4"I or angiograp y is recommended)
o
Determination of t e side of t e aortic arc is important, particularly if an initial aorta+ pulmonary artery s unt is planned)
In centers *it expertise, 4"I may (e used as a noninvasive met od of visuali9ing t e pulmonary arteries and t eir collateral supply)
2ther Tests:
EC5 findings are similar to t ose of ot er patients *it tetralogy of $allot (T'$)) "ig t ventricular ypertrop y *it rig t axis deviation is usually present) 8iventricular ypertrop y may occur in infants *it cardiac failure from excessive pulmonary (lood flo*) T'$+!A can (e differentiated from !A *it an intact septum (ecause patients *it t e latter diagnosis ave diminutive anterior L"S forces and left ventricular ypertrop y) $luorescent in situ y(ridi9ation ($IS#) analysis may (e performed to detect a c romosome arm ;;6 deletion)
#rocedures:
Indications- Cardiac cat eteri9ation *it angiograp y is recommended in most patients (efore surgical repair) Careful delineation of all sources of pulmonary (lood supply is necessary to facilitate surgical planning) T is includes determination of t e presence, si9e, and confluence of t e native pulmonary arteries and t e presence of maDor aortopulmonary collaterals t at may need to (e incorporated into t e repair) Tec ni6ue- A femoral venous approac may (e used to perform t e rig t eart cat eteri9ation) T e cat eter does not pass across t e pulmonary valve (ut can easily pass across t e .SD into t e left ventricle and aorta)
o
To visuali9e t e .SD, a ventriculogram s ould (e o(tained *it inDection in t e left ventricle) Coronary artery anatomy is delineated (y an aortic root inDection) Angiograp ic depiction of t e pulmonary arteries may necessitate a retrograde arterial approac ) T is also allo*s easier access to imaging of (ot surgical s unts and aortopulmonary collaterals) 8iplane angiograp y t at includes (ot lung fields is important in defining t e complete anatomy of (ot pulmonary arteries) Determining t e confluence and patency of pulmonary arteries is of utmost importance) $urt er selective angiograms may (e o(tained to delineate t e systemic+to+pulmonary collateral flo* and anatomy) In some patients, ventriculograp y and aortograp y do not demonstrate central true pulmonary arteries) In t ese patients, pulmonary vein *edge angiograp y may provide t is information) An end+ ole cat eter is passed across t e atrial septum and *edged into a pulmonary vein) (8ilateral inDections may (e necessary)) A forceful inDection of contrast (y and causes contrast to flo* retrograde t roug t e pulmonary veins reac ing t e central pulmonary arteries)
"esults- .enous cat eteri9ation usually reveals normal rig t atrial pressures) "ig t and left ventricular pressures are e6ual (ecause of t e presence of a large .SD) Aortic pressure is
normal if pulmonary (lood flo* is normal or decreased) A *ide pulse pressure may (e o(served in t e presence of a large DA) !ulmonary pressures are lo* *it normal pulmonary vascular resistance (ut may (e elevated in t e presence of a large systemic+to+pulmonary s unt)
o
Anless an atrial septal defect is present, oxygen saturation in t e rig t atrium is lo*) Systemic arterial saturation depends on t e amount of pulmonary (lood flo*) .entriculograp y reveals t e position of t e .SD) T e pulmonary arteries may (e depicted as confluent or nonconfluent) Areas of stenoses or ypoplasia in t e pulmonary arteries may (e o(served) Details of t e systemic to pulmonary collateral supply are delineated, and special attention may (e (roug t to dual supply of a lung segment) Intercommunications (et*een t e different collateral vessels and t e perip eral pulmonary artery segments may (e o(served)
!ostcat eteri9ation precautions- 5eneral postcat eteri9ation precautions include emorr age, pain, nausea and vomiting, and arterial or venous o(struction from t rom(osis or spasm) 5ive special attention to t e ydration status of infants * o re6uire multiple angiograms to outline t eir pulmonary arterial anatomy) Attempt to limit t e amount of contrast to 1+E m<Fkg)
o o
T ese patients are ypoxemic and may re6uire oxygen during and after t e procedure) 5ive special attention to o(taining emostasis and applying a pressure dressing at t e access sites postcat eteri9ation)
Complications- Taking appropriate precautions often avoids t e potential complications of cardiac cat eteri9ation, including (lood vessel inDury, perforation, tac yarr yt mias, (radyarr yt mias, and vascular occlusion)
Section & of 00
T$EAT4E3T
4edical Care:
/e*(orn infants *it cyanosis due to congenital eart disease almost al*ays (enefit from administration of !5E2 to maintain ductal patency * ile a definitive diagnosis is made) 'nce t e diagnosis of tetralogy of $allot *it pulmonary atresia (T'$+!A) is made, maintain !5E2 infusion t roug initial surgery) 'lder infants *it increased pulmonary (lood flo* may re6uire treatment for eart failure)
Surgical Care: /eonates *it ade6uate+si9ed confluent pulmonary arteries may (e amena(le to primary definitive surgical repair) A palliative procedure *it a systemicItoIpulmonary artery s unt may (e performed * ile a*aiting complete repair at a later date) T e ultimate surgical goals are to incorporate as many pulmonary artery segments as possi(le into a pulmonary artery confluence, to place a conduit from t e rig t ventricle to t e pulmonary artery confluence, and to close t e .SD)
: en t e pulmonary arteries are ypoplastic, nonconfluent, and supplied (y aortopulmonary collaterals, a multistaged repair is often re6uired) #ypoplastic pulmonary arteries generally re6uire palliative s unting to induce enlargement and gro*t of t ese vessels so t ey can (e successfully incorporated into t e complete repair) T e s unts used may (e modified 8lalock+
Taussig or central s unts, and t ey may (e unilateral or (ilateral) If t e pulmonary arteries ave gro*n after placement of t e palliative s unts, unifocali9ation of t e pulmonary arteries can (e performed& t is is done (y incorporating t e aortopulmonary collaterals and connecting t em to t e conduit from t e rig t ventricle)
$or complete repair to (e performed in a c ild * o as undergone palliation, t e central pulmonary arterial area must (e greater t an 13J of normal& predominantly left+to+rig t intracardiac s unting must (e present& t e e6uivalent of an entire lung must (e supplied (y t e central pulmonary artery confluence& and stenotic lesions in t e pulmonary artery outflo* must (e addressed) Some centers ave s ifted to*ard performing a single+stage repair, * erein all t e multiple aortopulmonary collaterals (4A!CAs) are ligated at t e aorta) T ese 4A!CAs are t en mo(ili9ed to*ard t e posterior mediastinum to construct a pulmonary artery confluence, follo*ed (y insertion of a pulmonary allograft to esta(lis continuity (et*een t ese neopulmonary arteries and t e rig t ventricle) T e .SD is closed) T ese centers ave reported good results) Infants *it postunifocali9ation pulmonary arteries t at, com(ined, are only mildly ypoplastic (M;33 mm;Fm;) ave a lo*er mortality rate and accepta(le rig t ventricular pressures) 4ost patients, o*ever, re6uire repeat cat eteri9ations for (alloon dilation or stent placements in stenotic pulmonary artery segments to alleviate elevated rig t ventricular pressures)
Consultations:

!ediatric cardiology consultation is advised) Consult a geneticist to evaluate t e presence of syndromic associations and gene deletions, especially in t e presence of associated anomalies or dysmorp ic features) 'nce t e anatomy of a c ild *it T'$+!A is determined (y ec ocardiograp y and angiograp y findings, consultation *it a cardiovascular surgeon is re6uired) T e caregivers need to (e a*are of t e possi(ility of a multistage repair and repeated surgeries and cat eteri9ations) If anomalies involving ot er systems are present, consultations and follo*+up *it t e appropriate specialists are re6uired)
Diet:
Infants * o are (orn *it multiple systemic+to+pulmonary collaterals and are in cardiac failure (ecause of pulmonary overcirculation re6uire caloric supplementation to esta(lis a normal gro*t pattern) Caloric intake as ig as 2?3+213 kcalFkgFd may (e re6uired) C ildren t at undergo palliative procedures also re6uire optimi9ation of t eir caloric intake) Ade6uate nutritional supplementation in t e form of total parental nutrition must also (e ascertained in t e perioperative period) T ese patients often ave a prolonged postoperative recovery course)
Activity: Exercise tolerance and need for restrictions on p ysical activity depend on t e type of repair and emodynamic state of t e patient)
!atients *it cyanosis *ill ave significantly limited exercise capacity) C ildren and adults * o ave ad complete repair of T'$+!A may ave limited exercise tolerance due to ventricular dysfunction, c ronotropic impairment, rig t ventricular outflo* tract o(structionFconduit stenosis, or distal pulmonary artery stenoses)
4EDICATI23
Section ) of 00
/e*(orns *it tetralogy of $allot *it pulmonary atresia may re6uire t e DA as t e source of pulmonary (lood flo*) A !5E2 (Alprostadil) infusion maintains patency of t e ductus) Infants *it multiple systemic pulmonary collaterals may develop symptomatic eart failure re6uiring medical t erapy)
Drug Category- Prostaglandins ++ !5E2 (Alprostadil) promotes dilatation of t e DA in infants *it

ductal+dependent cardiac a(normalities) It is also a vasodilator) Alprostadil (!rostin ." !ediatric InDection) ++ $irst+line palliative t erapy to temporarily maintain patency of DA (efore surgery) 8eneficial in infants * o ave congenital defects t at restrict pulmonary or systemic (lood flo* and * o depend on a patent DA for ade6uate oxygenation and lo*er (ody perfusion) !roduces vasodilation and increases cardiac output) Eac 2+m< ampule contains 133 mcgFm<)
Drug 3ame
Adult Dose #ediatric Dose Contraindications Interactions #regnancy
/ot indicated Initial dose- 3)31+3)2 mcgFkgFmin I. 4aintenance dose- 3)32+3), mcgFkgFmin I. Infuse I. into large vein or um(ilical cord Documented ypersensitivity& yaline mem(rane disease& respiratory distress syndrome <imited data exist& use caution *it concurrent use of antiplatelet drugs or anticoagulants N + Contraindicated in pregnancy Adverse effects and toxicity include apnea, sei9ures, fever, ypotension, leukocytosis, fever, and pulmonary overcirculation& neonates are usually intu(ated prop ylactically (ecause of potential risk of apnea (23+2;J)& prolonged use is occasionally necessary (in ypoplastic left eart syndrome transplant candidates) and may (e associated *it t ird spacing of fluid& monitor (lood oxygenation and arterial pressure
#recautions
Drug Category-
iuretic agents ++ T ese agents promote excretion of *ater and electrolytes (y
t e kidneys) T ey are used to treat eart failure or epatic, renal, or pulmonary disease * en sodium and *ater retention results in edema or ascites) C ildren * o ave C#$ symptoms often re6uire multiple diuretics for effective control) $urosemide (<asix) ++ Increases excretion of *ater (y interfering *it c loride+(inding cotransport system, * ic in turn in i(its sodium and c loride rea(sorption in ascending loop of #enle and distal renal tu(ule) Individuali9e dose to patient) Depending on response, administer adult doses at increments of ;3+,3 mg, no sooner t an E+> after previous dose, until desired diuresis occurs) : en treating infants, titrate *it 2+mgFkgFdose increments until satisfactory effect ac ieved) ;3+>3 mgFd !'FI.FI4& titrate up to E33 mgFd for severe edematous states 2+; mgFkgFdose !'& not to exceed E mgFkgFdose& do not administer more fre6uently t an 6E 2 mgFkgFdose I.FI4 slo*ly under close supervision& not to exceed E mgFkgFd Documented ypersensitivity& epatic coma& anuria& severe electrolyte depletion 4etformin decreases concentrations& interferes *it ypoglycemic effect of antidia(etic agents and antagoni9es muscle+relaxing effect of tu(ocurarine& auditory toxicity appears to (e increased *it coadministration *it aminoglycosides& earing loss
Drug 3ame
Adult Dose
#ediatric Dose
Contraindications Interactions
of varying degrees may occur& anticoagulant activity of *arfarin may (e en anced * en taken concurrently *it t is medication& increased plasma lit ium levels and toxicity are possi(le * en taken concurrently *it t is medication #regnancy C + Safety for use during pregnancy as not (een esta(lis ed) !erform fre6uent serum electrolyte, C';, glucose, creatinine, uric acid, calcium, and 8A/ determinations during first fe* mont s of t erapy and periodically t ereafter Spironolactone (Aldactone) ++ $or management of edema resulting from excessive aldosterone excretion) Competes *it aldosterone for receptor sites in distal renal tu(ules, increasing *ater excretion * ile retaining potassium and ydrogen ions) ;1+;33 mgFd !' 6d or divided (id 2)1+?)1 mgFkgFd !' 6d or divided 6E+2; Documented ypersensitivity& anuria& renal failure& yperkalemia 4ay decrease effect of anticoagulants& potassium and potassium+sparing diuretics may increase toxicity of spironolactone D + Ansafe in pregnancy Caution in renal and epatic impairment #ydroc lorot ia9ide (#ydroDIA"I<, Esidrix, 4icro9ide) ++ In i(its rea(sorption of sodium in distal tu(ules, causing increased excretion of sodium and *ater as *ell as potassium and ydrogen ions) ;1+233 mg !' 6d& not to exceed ;33 mgFd OE mont s- ;+? mgFkgFd !' divided (id ME mont s- ; mgFkgFd !' divided (id Documented ypersensitivity& anuria& renal decompensation 4ay decrease effects of anticoagulants, antigout agents, and sulfonylureas& t ia9ides may increase toxicity of allopurinol, anest etics, antineoplastics, calcium salts, loop diuretics, lit ium, dia9oxide, digitalis, amp otericin 8, and nondepolari9ing muscle relaxants C + Safety for use during pregnancy as not (een esta(lis ed) Caution in renal disease, epatic disease, gout, dia(etes mellitus, and eryt ematosus
#recautions
Drug 3ame
Adult Dose #ediatric Dose Contraindications Interactions #regnancy #recautions Drug 3ame Adult Dose #ediatric Dose Contraindications
Interactions
#regnancy #recautions
Drug Category- $notropic agents ++ !ositive inotropic agents increase t e force of contraction of
t e myocardium and are used to treat acute and c ronic C#$) !oor ventricular function may necessitate t e use of inotropic medications) Digoxin (<anoxin) ++ Cardiac glycoside *it direct inotropic effects and indirect effects on t e cardiovascular system) Acts directly on cardiac muscle, increasing myocardial systolic contractions) Indirect actions result in increased carotid sinus nerve activity and en anced sympat etic *it dra*al for any given increase in mean arterial pressure) 3)2;1+3)?=1 mg !' 6d 1+23 years- ;3+?1 mcgFkg !' M23 years- 23+21 mcgFkg !' 4aintenance dose- Ase ;1+?1J of !' loading dose
Drug 3ame
Adult Dose #ediatric Dose
Documented ypersensitivity& (eri(eri eart disease& Contraindications idiopat ic ypertrop ic su(aortic stenosis& constrictive pericarditis& carotid sinus syndrome I. calcium may produce arr yt mias in digitali9ed patients 4edications t at may increase levels include alpra9olam, (en9odia9epines, (epridil, captopril, cyclosporine, propafenone, propant eline, 6uinidine, diltia9em, aminoglycosides, !' amiodarone, antic olinergics, dip enoxylate, eryt romycin, felodipine, flecainide, ydroxyc loro6uine, itracona9ole, nifedipine, omepra9ole, 6uinine, i(uprofen, indomet acin, esmolol, tetracycline, tol(utamide, and verapamil 4edications t at may decrease levels include aminoglutet imide, anti istamines, c olestyramine, neomycin, penicillamine, aminoglycosides, !' colestipol, ydantoins, ypoglycemic agents, antineoplastic treatment com(inations (eg, carmustine, (leomycin, met otrexate, cytara(ine, doxoru(icin, cyclop osp amide, vincristine, procar(a9ine), aluminum or magnesium antacids, rifampin, sucralfate, sulfasala9ine, (ar(iturates, kaolinFpectin, and aminosalicylic acid C + Safety for use during pregnancy as not (een esta(lis ed) #ypokalemia may reduce positive inotropic effect of digitalis& ypercalcemia predisposes patient to digitalis toxicity, and ypocalcemia can make digoxin ineffective until serum calcium levels are *it in t e reference range& magnesium replacement t erapy must (e instituted in patients *it ypomagnesemia to prevent digitalis toxicity& incomplete A. (lock may progress to complete (lock * en treated *it
Interactions
#regnancy #recautions
digoxin& exercise caution in ypot yroidism, ypoxia, and acute myocarditis& adDust dose in renal impairment& ig ly toxic (overdoses can (e fatal) F2112.;%#
Section < of 00
Further Inpatient Care:

Admit for testing and surgical intervention) Significant pulmonic valve regurgitation often occurs regardless of t e type of conduit placed (et*een t e rig t ventricle and t e pulmonary arteries) Some patients develop su(stantial rig t ventricular dilation and rig t ventricular dysfunction) Surgical placement of a pulmonic valve may significantly (enefit t ese patients) Transcat eter placement of a pulmonic valve currently is under development)
Further 2utpatient Care:
Infants *it multiple aortopulmonary collaterals may re6uire outpatient medical management of eart failure) "esidual rig t ventricular ypertension *it rig t ventricular dysfunction from ypoplastic pulmonary arteries may (e present) After eac stage of surgical reconstruction, ec ocardiograp ic and Doppler evaluation of emodynamic ade6uacy s ould (e performed) After complete repair, t e patient needs to (e evaluated for t e development of rig t ventricleItoIpulmonary artery conduit stenosis as *ell as pulmonic regurgitation) A fe* patients may never reac t e stage of complete repair (ecause of very ypoplastic pulmonary arteries) T ese patients often are ypoxemic and polycyt emic and may re6uire oxygen supplementation)
Transfer:
Transfer to a tertiary care center is indicated for complete diagnostic evaluation and surgical intervention)
Complications:

"esidual rig t ventricular dysfunction from ypoplastic pulmonary arteries or conduit stenosis Cyanosis, ypoxemia, and polycyt emia Atrioventricular conduction a(normalities, rig t (undle (ranc (lock, ventricular arr yt mias in t e postoperative patients Significant pulmonic valve regurgitation
#rognosis:
T e prognosis depends on t e specific anatomy and type of intervention) <ong+term follo* up data are not *idely availa(le& o*ever, recent outcome does seem to (e more favora(le) 4ost patients * o undergo placement of a rig t ventricle to pulmonary conduit *ill re6uire one or more conduit replacements, secondary to progressive conduit stenosis or insufficiency)
#atient Education:
Educate patients and t eir families a(out anatomic details and long+term prognosis, t e potential need for multiple surgeries and cat eteri9ations, and postoperative complications) At all patient care visits, emp asi9e t e need for (acterial endocarditis prop ylaxis) $or excellent patient education resources, visit e4edicine%s #eart Center) Also, see e4edicine%s patient education article Tetralogy of $allot)
Section = of 00
4ISCE11A3E2%S
4edical'1egal #itfalls:
$ailure to consider t e diagnosis, especially in a ne*(orn *it cyanosis
Special Concerns:
5enetic counseling is strongly recommended in patients of c ild(earing age& t e c ance t at patients *it tetralogy of $allot (T'$) could ave an offspring *it C#D is as ig as 21J) !atients *it residual rig t ventricular dysfunction or pulmonary ypertension are advised to avoid pregnancy (ecause it carries significant mortality risk) All patients *it T'$+!A are re6uired to take appropriate anti(iotic (acterial endocarditis prop ylaxis) Exercise recommendations must (e tailored to individual patients (y considering t e presence of cyanosis, rig t ventricle ypertension, rig t ventricle dysfunction, or dysr yt mias)
Section 0* of 00
#ICT%$ES
Caption: !icture 2) !arasternal long axis t*o+dimensional ec ocardiograp ic image demonstrating a large malalignment ventricular septal defect *it overriding of t e aorta over t e ventricular septum)
#icture Type: 4ovie Caption: !icture ;) Su(costal sagittal plane t*o+dimensional ec ocardiograp ic image s o*ing pulmonary valve atresia, *it confluent and *ell+developed pulmonary artery (ranc es)
#icture Type: 4ovie Caption: !icture ?) Suprasternal long axis color flo* ec ocardiograp ic image s o*ing a large patent ductus arteriosus supply confluent pulmonary arteries)
#icture Type: 4ovie Caption: !icture ,) Aortopulmonary vie* angiogram, *it inDection in t e descending t oracic aorta demonstrating multiple aortopulmonary collaterals supplying pulmonary (lood flo*)
#icture Type: 4ovie Caption: !icture 1) !arasternal long axis t*o+dimensional ec ocardiograp ic image in a patient status post complete repair of tetralogy of $allot *it pulmonary atresia) A patc is visuali9ed closing t e ventricular septal defect)
#icture Type: 4ovie Caption: !icture E) !arasternal long axis color compare ec ocardiograp ic image s o*ing t e pulmonary artery conduit arising from t e rig t ventricle)
#icture Type: 4ovie http //%%%.emedicine.com/ped/topic15:<.htm
"ate t is Article
Tetralogy of Fallot
1ast %pdated: 4arc ;;, ;33E
Email to a Colleague 5et C4EFCE for article
Synonyms and related key-ords: T'$, $allot tetrad, $allot%s tetrad, congenital eart disease, tetralogy of $allot, maldevelopment of rig t ventricular infundi(ulum, su(aortic ventricular septal defect, rig t ventricular infundi(ular stenosis, aortic valve positioned to override t e rig t ventricle, rig t ventricular ypertrop y, rig t+to+left s unting, rig t ventricular outflo* tract o(struction, cyanosis, ypertrop y of t e infundi(ular septum, dyspnea, retarded gro*t , aortic eDection click, systolic t rill, systolic eDection murmur, clu((ing, scoliosis, s6uatting position, retinal engorgement, emoptysis, conotruncal a(normalities, Di5eorge syndrome, (ranc ial arc a(normalities, fetal ydantoin syndrome, fetal car(ama9epine syndrome, fetal alco ol syndrome, maternal p enylketonuria (irt defects
A%T,2$ I3F2$4ATI23
Section 0 of 00
Aut or- 4ark Spektor, D2, Assistant !rofessor, Consulting Staff, Department of Emergency 4edicine, SA/C + Do*nstate, 7ings County #ospital Center& Consulting Staff, Department of Emergency 4edicine, Aniversity #ospital in 8rooklyn, Director of Emergency 4edicine, /C##S + .A 4edical Center Coaut or(s)- 5urt #flieger, 4D, Active Staff, Department of !ediatrics, <ake !ointe 4edical Center 4ark Spektor, D', is a mem(er of t e follo*ing medical societies- American College of Emergency ! ysicians, American College of ! ysician Executives, and Society for Academic Emergency 4edicine Editor(s)- Theodore 6aeta, D2, 4#,, "esidency Director, Clinical Associate !rofessor of Emergency 4edicine in 4edicine, Department of Emergency 4edicine, /e* Cork 4et odist #ospital& Francisco Talavera, #harmD, #hD, Senior ! armacy Editor, e4edicine& 6ary Setnik, 4D, C air, Department of Emergency 4edicine, 4ount Au(urn #ospital& Assistant !rofessor, Division of Emergency 4edicine, #arvard 4edical Sc ool& >ohn ,alamka, 4D, C ief Information 'fficer, Care5roup #ealt care System, Assistant !rofessor of 4edicine, Department of Emergency 4edicine, 8et Israel Deaconess 4edical Center& Assistant !rofessor of 4edicine, #arvard 4edical Sc ool& and Charles ? #ollack, >r, 4D, 4A, FACE#, Associate !rofessor of Emergency 4edicine, Aniversity of !ennsylvania Sc ool of 4edicine& C airman, Department of Emergency 4edicine, !ennsylvania #ospital Disclosure
I3T$2D%CTI23
Section ( of 00
7ackground: Tetralogy of $allot (T'$) is a complex of anatomic a(normalities arising from t e maldevelopment of t e rig t ventricular infundi(ulum)
In 2>>>, $allot descri(ed t e anatomy as consisting of a su(aortic ventricular septal defect (.SD), rig t ventricular infundi(ular stenosis, aortic valve positioned to override t e rig t ventricle, and rig t ventricular ypertrop y (".#)) See Image 2 for a sc ematic illustration of t ese a(normalities) #athophysiology: :ide variation exists in t e (asic anatomic morp ology, pat op ysiology, clinical signs and symptoms, and surgical met ods of t erapy) !at op ysiology is dependent primarily upon severity of t e rig t ventricular outflo* tract (".'T) o(struction) "ig t+to+left s unting is typical) Fre8uency:
In the %S: T'$ represents approximately 23J of cases of congenital eart disease)
4ortality'4or/idity: /atural istory is varia(le)

/atural istory is determined mainly (y t e degree of ".'T o(struction) Approximately ;1J of untreated patients *it T'$ and ".'T o(struction die *it in t e first year of life, ,3J (y , years, =3J (y 23 years, and K1J (y ,3 years)
Se : Incidence is slig tly ig er in males t an females) Age: /e*(orns
C1I3ICA1
Section 9 of 00
,istory:

Cyanosis develops *it in t e first fe* years of life) $irst presentation may include poor feeding, fussiness, tac ypnea, and agitation) Symptoms generally progress secondary to ypertrop y of t e infundi(ular septum) Cyanosis occurs and demands surgical repair) Dyspnea on exertion is common) S6uatting is uni6uely c aracteristic of a rig t+to+left s unt t at presents in t e exercising c ild) #ypoxic 0tet0 spells are potentially let al, unpredicta(le episodes t at occur even in noncyanotic patients *it T'$) T ese spells can (e a(orted *it relatively simple procedures) T e rare patient may remain marginally and impercepti(ly cyanotic, or acyanotic and asymptomatic, into adult life) Severe cyanosis may present at (irt in a patient *it T'$ and associated pulmonary atresia)
8irt *eig t is lo*) 5ro*t is retarded) Development and pu(erty may (e delayed)
#hysical:

"ig t ventricular predominance on palpation 4ay ave a (ulging left emit orax Systolic t rill at t e lo*er left sternal (order Aortic eDection click Single S; + !ulmonic valve closure not eard Systolic eDection murmur + .aries in intensity inversely *it t e degree of ".'T o(struction
o o
4ore cyanotic patients ave greater o(struction and a softer murmur) An acyanotic patient *it T'$ (pink tet) as a long, loud, systolic murmur *it a t rill along t e ".'T)
Cyanosis and clu((ing + .aria(le S6uatting position Scoliosis + Common "etinal engorgement #emoptysis
Causes:
As one of t e conotruncal malformations, T'$ can (e associated *it a spectrum of lesions kno*n as CATC# ;; (cardiac defects, a(normal facies, t ymic ypoplasia, cleft palate, ypocalcemia)) Cytogenetic analysis may demonstrate deletions of a segment of c romosome (and ;;622 (Di5eorge critical region)) A(lation of cells of t e neural crest as (een s o*n to reproduce conotruncal malformations) T ese a(normalities are associated *it t e Di5eorge syndrome and (ranc ial arc a(normalities) T'$ fre6uently is associated *it t e follo*ingo o
$etal ydantoin syndrome $etal car(ama9epine syndrome
o o
$etal alco ol syndrome 4aternal p enylketonuria (!7A) (irt defects

Section : of 00
DIFFE$E3TIA1S
Acute "espiratory Distress Syndrome Anemia, Acute Anemia, Sickle Cell Aortic Stenosis Ast ma !atent Ductus Arteriosus !ediatrics, Apnea !ediatrics, 8acteremia and Sepsis !ediatrics, 8ronc iolitis !ediatrics, Crying C ild !ediatrics, $oreign 8ody Ingestion !ediatrics, !neumonia !ediatrics, "eactive Air*ay Disease !ediatrics, "espiratory Distress Syndrome !neumot orax, Iatrogenic, Spontaneous and !neumomediastinum !ulmonic .alvular Stenosis S ock, Cardiogenic S ock, Septic 2ther #ro/lems to /e Considered: E(stein malformation of t e tricuspid valve !ulmonary atresia .entricular septal defect !seudotruncus arteriosus 1a/ Studies:
'ximetry and arterial (lood gases

o
'xygen saturation is varia(le, (ut p# and pC'; are normal unless t e patient is in extremis, suc as during a tet spell) 'ximetry is particularly useful in t e dark+skinned patient or t e anemic patient * ose level of cyanosis is not apparent) Cyanosis is not evident until ?+1 gFd< of reduced emoglo(in is present) A decrease in systemic vascular resistance (S.") during exercise, (at ing, or fever potentiates a rig t+to+left s unt and causes ypoxemia)
o o
#ematology
!rolonged cyanosis causes reactive polycyt emia t at increases t e oxygen+carrying capacity) #yperviscosity and coagulopat y often ensue and are particularly deleterious in patients *it a rig t+to+left intracardiac s unt) Stroke and (rain a(scess are natural corollaries)
Imaging Studies:
C est roentgenograp y
o
Coeur en sa(ot ((oot+s aped eart) secondary to uplifting of t e cardiac apex from ".# and t e a(sence of a normal main pulmonary artery segment (see !icture ;) /ormal eart si9e due to t e lack of pulmonary (lood flo* and congestive eart failure Decreased pulmonary vascularity "ig t atrial enlargement "ig t+sided aortic arc (;3+;1J of patients) *it indentation of left*ard+positioned trac eo(ronc ial s ado* 4ay (e normal in acyanotic T'$ or may resem(le findings of small+ to moderate+si9ed .SD *it mild ".#, rig t atrial enlargement, and increased pulmonary vascular markings
o o o o
Ec ocardiograp y reveals a large .SD *it an overriding aorta and varia(le degrees of ".'T o(struction)
2ther Tests:
Electrocardiogram (see !icture ?)

o o o
"ig t axis deviation (P2;3Q to P213Q) "ig t or com(ined ventricular ypertrop y "ig t atrial ypertrop y
#rocedures:
Cardiac cat eteri9ation

o o o
Assesses pulmonary annulus si9e and pulmonary arteries Assesses t e severity of ".'T o(struction <ocates t e position and si9e of t e .SD
"ules out possi(le coronary artery anomalies

Section & of 00
T$EAT4E3T
#rehospital Care:

Any infant *it cyanosis andFor respiratory distress re6uires oxygen) 8lo*+(y '; (88';) is t e least o(Dectiona(le) Ase t e open+end of a cannula or tu(e) !ermit t e (a(y to remain *it t e mot er or fat er) Do not provoke t e infant (y attempting to start an intravenous (I.) line, especially if not skilled in pediatric I. starts) An intraosseous insertion could (e an immediate lifesaving tool)
Emergency Department Care: T e ED p ysician s ould (e a(le to recogni9e and treat a ypercyanotic episode as one of t e very fe* pediatric cardiology emergencies t at may present to t e ED)
#ypoxic tet spell- #ypercyanotic episodes are c aracteri9ed (y paroxysms of yperpnea, prolonged crying, intense cyanosis, and decreased intensity of t e murmur of pulmonic stenosis)
o
4ec anism + Secondary to infundi(ular spasm andFor decreased S." *it increased rig t+to+left s unting at t e .SD, resulting in diminis ed pulmonary (lood flo* If left untreated, may result in syncope, sei9ure, stroke, or deat
Treatment for t e acute setting of ypercyanosis includes t e follo*ingo
7nee+c est position- !lace t e (a(y on t e mot er%s s oulder *it t e knees tucked up underneat ) T is provides a calming effect, reduces systemic venous return, and increases S.") 'xygen is of limited value since t e primary a(normality is reduced pulmonary (lood flo*) 4orp ine sulfate, 3)2+3); mgFkg I4FSC, may reduce t e ventilatory drive and decrease systemic venous return) ! enylep rine, 3)3; mgFkg I., is used to increase S.") Treat acidosis *it sodium (icar(onate, * ic may reduce t e respiratory center stimulating effect of acidosis) 5eneral anest esia is a last resort)
o o
Consultations: !ediatric cardiologyFsurgery 4EDICATI23
Section ) of 00
T e goals of t erapy are to reduce t e ventilatory drive, decrease systemic venous return, and increase perip eral venous return)
Drug Category- !nalgesics ++ T ese agents reduce ventilatory drive) !ain control ensures patient
comfort and promotes pulmonary toilet) 4ost analgesics ave sedating properties, * ic are (eneficial for patients * o are aving ypercyanotic episodes) 4orp ine sulfate (Duramorp , Astramorp , 4S Contin) ++ D'C for narcotic analgesia (ecause of its relia(le and predicta(le effects, safety profile, and ease of reversi(ility *it naloxone) Administered I., may (e dosed in num(er of *ays and commonly titrated until desired effect o(tained) 3)31+3); mgFkg dose I. prn& not to exceed 21 mgFdose Documented ypersensitivity& ypotension& potentially compromised air*ay *it uncertain rapid air*ay control& respiratory depression& nausea& emesis& constipation& urinary retention ! enot ia9ines may antagoni9e analgesic effects& TCAs, 4A'Is, and ot er C/S depressants may potentiate adverse effects 8 + Asually safe (ut (enefits must out*eig t e risks) Avoid in ypotension, respiratory depression, nausea, emesis, constipation, and urinary retention& caution in atrial flutter and ot er supraventricular tac ycardias& as vagolytic action and may increase ventricular response rate
Drug 3ame
#ediatric Dose Contraindications Interactions #regnancy #recautions
Drug Category- !lpha%adrenergic agonist ++ T ese agents improve emodynamic status (y

improving myocardial contractility and increasing eart rate, resulting in increased cardiac output) !erip eral resistance is increased (y vasoconstriction) Increased cardiac output and increased perip eral resistance increase (lood pressure) ! enylep rine (/eo+Synep rine) ++ Strong postsynaptic alp a+ receptor stimulant *it little (eta+adrenergic activity& produces vasoconstriction of arterioles, increasing perip eral venous return) 1+;3 mcgFkgFdose I. (olus 623+21min prn 3)2 mgFdose I.FSC 62+; prn 3)2+3)1 mcgFkgFmin I. infusion Documented ypersensitivity& severe ypertension& ventricular tac ycardia 8retylium may potentiate action on adrenergic receptors, possi(ly resulting in arr yt mias& 4A'Is may significantly en ance adrenergic effects, and pressor response may (e increased ;+ to ?+ fold& guanet idine may increase pressor response of direct+acting
Drug 3ame
#ediatric Dose Contraindications Interactions
vasopressors, possi(ly resulting in severe ypertension #regnancy C + Safety for use during pregnancy as not (een esta(lis ed) Caution in elderly patients, ypert yroidism, myocardial disease, (radycardia, partial eart (lock or severe arteriosclerosis& in ypovolemia, not su(stitute for replacement of (lood, fluids and electrolytes, and plasma (t ese s ould (e restored promptly * en loss as occurred)
Section < of 00
#recautions
F2112.;%#
Further Inpatient Care:
!alliative surgery
o o o
8lalock+Taussig s unt !ott procedure :aterston s unt
Total surgical correction *it patc closure of t e .SD and relief of t e ventricular outflo* o(struction is preferred (see !icture ,))
Further 2utpatient Care:

5ood dental ygiene Endocarditis prop ylaxis Arr yt mia prop ylaxis may (e *arranted)
Complications:

Eryt rocytosis 8rain a(scess Acute gouty art ritis Infective endocarditis Cere(rovascular t rom(osis Delayed pu(erty !ostoperative complications
o
Congestive eart failure (rig t or left, residual outflo* o(struction, .SD, andFor pulmonic regurgitation
Atrial flutter, ventricular arr yt mias, rig t (undle+(ranc (lock, or left anterior emi(lock Infective (acterial endocarditis
#rognosis:
If rig t ventricular outflo* tract o(struction is severe, t e mortality rate is ig *it out palliative or corrective surgery)
#atient Education:
$or excellent patient education resources, visit e4edicine%s #eart Center) Also, see e4edicine%s patient education article Tetralogy of $allot)
Section = of 00
4ISCE11A3E2%S
Special Concerns:

#ypoxic tet spells ! ysical and cognitive gro*t impairment 8rain a(scess and stroke, secondary to t e rig t+to+left s unt Infective endocarditis !olycyt emia
Section 0* of 00
#ICT%$ES Caption: !icture 2) Anatomic findings in tetralogy of $allot) .ie* $ull Si9e Image
e4edicine @oom .ie* (InteractiveR)
#icture Type: Image Caption: !icture ;) Aplifted apex and a(sence of pulmonary artery segment typifies t e 0coeur en sa(ot0 (ie, (oot+s aped eart) of tetralogy of $allot) .ie* $ull Si9e Image e4edicine @oom .ie*
(InteractiveR)
#icture Type: N+"AC Caption: !icture ?) Typical preoperative EC5 for tetralogy of $allot) .ie* $ull Si9e Image
e4edicine @oom .ie* (InteractiveR) #icture Type: EC5 Caption: !icture ,) Typical findings on postoperative EC5 for tetralogy of $allot) .ie* $ull Si9e Image
e4edicine @oom .ie* (InteractiveR) #icture Type: EC5 http //%%%.emedicine.com/5"5*=/topic595.htm
Tetralogy of Fallot !Disease"
#eart, section t roug t e middle Definition
Tetralogy of $allot
Cyanotic STet spellS
A type of eart defect present at (irt (congenital) consisting of four different a(normalities) It usually results in insufficiently oxygenated (lood (eing pumped to t e (ody causing cyanosis ((luis discoloration of t e skin)) Alternative 3ames TET& T'$ Causes And $isk
T e cause of most congenital eart defects is unkno*n) 4ultiple factors seem to (e involved) !renatal factors associated *it ig er t an normal risk for t is condition include maternal ru(ella or ot er viral illnesses during pregnancy, poor prenatal nutrition, maternal alco olism, mot er over ,3 years old, and dia(etes) T ere is a ig er incidence of tetralogy of $allot in c ildren *it Do*n syndrome (a common genetic disorder * ic results from aving an extra ;2st c romosome)) Tetralogy of $allot is classified as a cyanotic eart defect (ecause t e condition causes insufficiently oxygenated (lood to (e pumped to t e (ody, * ic leads to cyanosis (a (luis +purple coloration to t e skin) and s ortness of (reat ) T e classic form of Tetralogy includes , defects *it in t e eart structures
.entricular septal defect ( ole (et*een t e rig t and left ventricles) /arro*ing of t e pulmonic outflo* tract (tu(e t at connects t e eart *it t e lungs) An aorta (tu(e t at carries oxygenated (lood to t e (ody) t at arises from (ot ventricles, rat er t an exclusively from t e left ventricle A t ickened muscular *all of t e rig t ventricle (rig t ventricular ypertrop y)
T ere is flo* of deoxygenated ((lue) (lood into t e general (ody circulation and decreased (lood flo* to t e lungs) At (irt , infants may not s o* t e signs of t e cyanosis, (ut later may develop sudden frig tening episodes of (luis skin from crying or feeding (called 0Tet spells0)) Tetralogy of $allot occurs in approximately 1 out of 23,333 infants)
#revention T ere is no kno*n prevention) Symptoms
Difficult feeding (poor feeding a(its) $ailure to gain *eig t !oor development Cyanosis * ic (ecomes more pronounced during periods of agitation !assing out Sudden deat Clu((ing of fingers (skin or (one enlargement around t e finger nails) S ortness of (reat aggravated (yexercise S6uatting during episodes of cyanosis
Signs And Tests
A p ysical examination *it a stet oscope almost al*ays reveals a eart murmur) Tests
E75 (elctrocardiogram) s o*ing t e t ickening of t e rig t ventricle muscle C8C s o*ing increased red (lood cells C est N+ray s o*ing a 0(oot s aped0 eart and dark lungs Cardiac cat eteri9ation Ec ocardiogram for t e definitive diagnosis
Treatment
Surgery to repair t e defects in t e eart is al*ays performed relatively early in life) Sometimes a preliminary surgery to create increased (lood flo* to t e lungs is done (efore definitive corrective surgery) Corrective surgery *idens t e narro*ed pulmonary valve, and t e ventricular septal defect is closed) !arents of c ildren *it tetralogy of $allot can (e assisted in coping *it t e symptoms of t e disease) Some interventions to consider include
If a c ild does (ecome (lue, t e parent s ould immediately place t e c ild on isF er side and put t e knees up to t e c est, calm t e (a(y and seek medical attention $eeding t e c ild slo*ly 5iving smaller, more fre6uent meals Decreasing t e c ildSs anxiety (y remaining calm 4inimi9ing crying (y trying to anticipate t e c ildSs needs "ecruiting ot ers to care for t e c ild to prevent parental ex austion and (urn+out
#rognosis 4ost cases can (e surgically corrected) !rognosis (pro(a(le outcome) *it surgery is good) :it out surgery, deat usually occurs around ;3 years old) Complications

Delayed gro*t and development Sei9ures during periods of insufficient oxygen
Call ,ealth Care #rovider Call your ealt care provider if ne* unexplained symptoms develop or if t e patient is aving an episode of cyanosis ((lue skin))
Disclaimer
"evie* Date- 1F=F;33; http //%%%.bro%ardhealth.org/7>899.c!m
Tetralogy 2f Fallot
Description of Tetralogy Of Fallot Causes and Risk Factors of Tetralogy Of Fallot Symptoms of Tetralogy Of Fallot Diagnosis of Tetralogy Of Fallot Treatment of Tetralogy Of Fallot Questions To Ask Your Doctor About Tetralogy Of Fallot
Definition of Tetralogy Of Fallot

A congenital cardiac anomaly that consists of four defects pulmonic stenosis! "entricular septal defect! malposition of the aorta so that it arises from the septal defect or the right "entricle! and right "entricular hypertrophy#
Description of Tetralogy Of Fallot

Tetralogy of Fallot is actually four defects in combination#
$irst, t e septum t at divides t e t*o ventricles is incomplete (so t ere is a ventricular septal defect), and oxygen+poor (lood is t us allo*ed to mix *it oxygen+ric (lood) Second, t e passage*ay from t e rig t ventricle to t e lungs is markedly narro*ed) T ird, t e origin of t e aorta is s ifted to*ard t e rig t side of t e eart from t e left) $ourt , t e muscle in t e *all of t e rig t ventricle is t ickened and stiffened) 'nly t e first t*o of t ese defects cause significant trou(le or re6uire an operation) Tetralogy of $allot constitutes a(out 23 percent of all congenital eart disease) It is t e most common cyanotic eart defect& nearly ?,333 ne* cases a year occur in t e A)S)) A $renc p ysician, Etienne $allot, descri(ed t is defect in 2>>>)
Causes and Risk Factors of Tetralogy Of Fallot

The cause of this congenital abnormality is not kno$n#
Symptoms of Tetralogy Of Fallot

These defects result in decreased blood flo$ to the lungs and circulation of blue %uno&ygenated' blood to the body tissues( both of these effects cause bluish skin %cyanosis'! clubbing %bulging of the nailbeds' of the fingers and toes! shortness of breath! and e&treme fatigue#
Diagnosis of Tetralogy Of Fallot

The condition is suspected on the basis of the child)s symptoms and a physical e&amination# A chest &*ray sho$s a characteristic shape of the heart# An +C,! echocardiography! and in some cases cardiac catheteri-ation are performed to determine the e&tent of the abnormality#
Treatment of Tetralogy Of Fallot

.nfants may re/uire surgery %for e&ample! the 0lalock*Taussig shunt procedure' to impro"e blood flo$ to the lungs and decrease cyanosis# Once the child is past infancy! correcti"e open heart surgery is performed# The results of successful complete repair of Tetralogy of Fallot are good cyanosis disappears! e&ercise tolerance impro"es! and people may lead normal li"es#
:it out an operation, only a(out ?3 percent of people *it Tetralogy of $allot *ould survive to t e age of ,3 years) Surgery results in almost K3 percent of patients surviving for at least ;1 years from t e time of surgery& generally, t e results are (est if t e defect is corrected (efore t e patient is 2; years old) If an infant as severe symptoms at an early age, placement of a temporary s unt may (e re6uired to provide more (lood flo* to t e lungs) T e long+term corrective procedure involves open+ eart surgery, including t*o steps- closing t e ole (et*een t e t*o ventricles and opening up t e artery from t e rig t ventricle to t e lungs) T e long+ term results of t e operation are good& most patients gro* normally and ave normal lives) A minority of patients ave difficulties later in life *it eart failure or eart r yt m disorders and may re6uire additional surgery or continued medication) If surgery is considered necessary, it is usually performed electively * en t e c ild is (et*een t e ages of ; and ,) T e oles most often can (e closed *it a suture, (ut if t ey are particularly large, a small patc made from t e patient%s o*n pericardial (outside *rapping of t e eart) tissue or a Dacron patc can (e used) T e operation provides a complete cure, and it is extremely rare for t e oles to open up again) Anti(iotic treatment to prevent infection need not (e re6uired for more t an a fe* mont s after t e operation, and t e patient s ould (e a(le to lead a full and active life) !atients receive anti(iotics (efore dental or surgical procedures ((acterial endocarditis prop ylaxis)) 't er medications may also (e prescri(ed) http //scc.healthcentral.com/bcp/main.asp?page@encyAid@977Aap@<:Abrand@19
3hronic hypo2ia caused by right#to#le!t shunting is associated %ith decreased neurologic !unction. 5pisodes o! acute hypo2ia !rom in!undibular spasm are li!e threatening. Polycythemia associated %ith chronic hypo2ia causes hypercoagulability and thrombosis. *ight#to#le!t shunting that bypasses the !iltering o! pulmonary capillaries is associated %ith a higher incidence o! systemic in!ection such as a brain abscess. http //%%%.emedicine.com/radio/byname/tetralogy#o!#!allot.htm
Tetralogy of Fallot
$rom :ikipedia, t e free encyclopedia
0ump to na$igation, search

Tetralogy of Fallot
diagram o! a healthy heart and one su!!ering !rom &etralogy o! !allot
ICD !" ICD # $MIM DiseasesD% MedlinePlus eMedicine
B17.: 9;5.1 7>9566 ;886 667589 emerg/595
T e tetralogy of Fallot is a congenital eart defect * ic classically as four anatomical components) It is t e most common cyanotic eart defect and t e most common cause of (lue (a(y syndrome) It *as first descri(ed (y /iels Stensen in 2E=;) In 2>>> t e $renc p ysician Etienne $allot accurately detailed its four anatomical c aracteristics)
Contents
ChideD

7 4natomic "orphology 1 5pidemiology and 5tiology : Pathophysiology ; &reatment o ;.7 5mergency "anagement o! &et Spells o ;.1 Palliati$e Surgery o ;.: Surgical *epair 5 52ternal links
CeditD
Anatomic Morphology
As classically descri(ed, tetralogy of $allot involves four eart malformations7. 4 $entricular septal de!ect ,+S(- a hole bet%een the t%o bottom chambers ,$entricles- o! the heart. 1. Pulmonic stenosis *ight $entricular out!lo% tract obstruction, a narro%ing at or just belo% the pulmonary $al$e. &he degree o! stenosis $aries bet%een indi$iduals %ith &)' and is the primary cause o! symptoms. :. )$erriding aorta &he aorta is positioned o$er the +S( instead o! in the le!t $entricle. ;. *ight $entricular hypertrophy ,*+H- &he right $entricle is more muscular than normal and may also be dilated. &his causes a characteristic coeur#en#sabot ,boot#shaped- appearance as seen by chest E#ray. T ere is anatomic variation (et*een t e earts of individuals *it tetralogy of $allot) T e degree of rig t ventricular outflo* tract o(struction varies (et*een patients and is generally t e primary determinant of clinical symptoms and disease progression) In addition, t is condition is also sometimes associated *it ot er anatomical analomies, suc as o o o o o
o o o
stenosis o! the le!t pulmonary artery ,in ;6F o! patientsa bicuspid pulmonary $al$e ,in ;6F o! patientsright#sided aortic arch ,in 15F o! patientscoronary artery anomalies ,in 76F o! patientsan atrial septal de!ect, in %hich case the syndrome is sometimes called the pentalogy o! 'allot an atrio$entricular septal de!ect anomalous pulmnary $enous return !orked ribs and scoliosis
Tetralogy of fallot *it pulmonary atresia (pseudotruncus arteriosus) is a severe variant in * ic t ere is complete o(struction of t e rig t ventricular outflo* tract and a(sence of of t e pulmonary trunk) In t ese individuals, t ere is complete rig t to left s unting of (lood) T e lungs are perfused via extensive collaterals from t e systemic arteries) T ese individuals are severely cyanotic and *ill ave a continuous murmur on p ysical exam due to t e collateral circulation to t e lungs) CeditD
pidemiology and tiology

Tetralogy of $allot occurs in approximately ? to E per 23,333 (irt s and represents 1+=J of congenital eart defects) Its cause is t oug t to (e due to environmental or genetic factors or a com(ination) It is associated *it c romosome ;; deletions and di5eorge syndrome) It occurs slig tly more often in males t an in females) 'ur current understanding of t e em(ryology of t is disease is t at it is a result of anterior malalignment of t e conal septum, resulting in t e clinical com(ination of a .SD, pulmonary stenosis, and an overriding aorta) T e development of rig t ventricular ypertrop y is a result of progressive rig t eart failure arising from t is com(ination, and can (e minimi9ed or even averted (y early surgical repair) CeditD
Pathophysiology
T e tetralogy of $allot generally results in lo* oxygenation of (lood due to mixing of oxygenated and deoxygenated (lood in t e left ventricle and preferential flo* of (lood from t e ventricles to t e aorta (ecause of o(struction to flo* t roug t e pulmonary valve) T is is kno*n as a rig t+to+left s unt) It is often evidenced (y a (luis tint to t e (a(y%s skin (cyanosis)) #o*ever t ere are 0pink tets0 in * ic t e degree of o(struction in t e rig t ventricular outflo* tract is lo*) 8lood flo*s preferentially from t e ventricles to t e lungs and only minimal desaturation occurs in t e systemic circulation (ecause of mixing of saturated and desaturated (lood in t e ventricles) T is degree of desaturation may (e undetecta(le to t e eye and re6uires a pulse oximeter to identify it) Even c ildren * o are generally not too deeply cyanosed ((lue) may develop acute severe cyanosis or ypoxic 0tet spells0) T e precise mec anism of spelling is in dou(t, (ut certainly t is is a dangerous event and presuma(ly results from an increase in resistance to (lood flo* to t e lungs *it increased preferential flo* of desaturated (lood to t e (ody) Antreated tetralogy of $allot, over t e long term, results in progressive rig t ventricular ypertrop y and dilatation due to t e increased resistance on t e rig t ventricle) T is progresses to rig t eart failure and deat ) CeditD
Treatment
Tetralogy of $allot is treated on t*o levels- *it immediate emergency care for its aforementioned 0 ypoxic spells0 or 0tet spells0 and *it surgery for its long term progressive effects) Actuarial survival for untreated tetralogy of $allot is approximately =1J after t e first year of life, E3J (y four years, ?3J (y ten years, and 1J (y forty years) CeditD
Emergency 4anagement of Tet Spells

Conse6uential acute ypoxia may (e treated *it (eta+(lockers suc as propranolol, (ut acute episodes may re6uire rapid intervention *it oxygen, morp ine to reduce ventilatory drive and
p enylep rine to increase (lood pressure) T ere are also simple procedures suc as t e knee+c est position * ic reduces systemic venous return (to reduce t e rig t+to+left s unting), increases systemic vascular resistance (and ence (lood pressure) and provides a calming effect * en t e procedure is performed (y t e parent) CeditD
#alliative Surgery
T e condition *as initially t oug t untreata(le until surgeon Alfred 8lalock, cardiologist #elen 8) Taussig, and la( assistant .ivien T omas at Bo ns #opkins Aniversity developed a s unt procedure, * ic involved Doining t e left su(clavian artery leaving t e eart to t e left pulmonary artery leading to t e lungs) T is gave a large portion of t e partially oxygenated (lood leaving t e eart a second c ance at oxygenation, and greatly relieved symptoms in patients) T e first 8lalock+Taussig s unt surgery *as performed on t ree+year old Eileen Saxon on /ovem(er ;K, 2K,,) T e !ott s unt and t e :aterson procedure are ot er s unt procedures * ic *ere developed for t e same purpose) CeditD
Surgical $epair
T e 8lalock+Taussig procedure *as t e first surgery to (e performed on t e great vessels surrounding t e eart, and (ecame istorically important (ecause it demonstrated to t e medical community t at eart surgery *as indeed possi(le) T e first total repair of tetralogy of $allot *as performed (y C) :alton <ille ei at t e Aniversity of 4innesota in 2K1, on a 23+mont (oy) Total surgical repair initially carried a ig mortality risk, (ut t is as consistently improved over t e years) Surgery is no* often carried out in infants 2 year of age or younger *it a O1J perioperative mortality) T e surgery generally involves making incisions into t e eart muscle, relieving t e rig t ventricular outflo* tract stenosis (y careful resection of muscle, and repairing t e .SD using a 5ore+ Tex or Dacron patc or a omograft) Additional reparative or reconstructive *ork is done on some patients, since t e anatomy varies) !atients * o ave undergone 0total0 repair of tetralogy of $allot often ave good or excellent cardiac function after t e operation *it some to no exercise intolerance and ave t e potential to lead normal lives) Surgical success greatly depends on t e particular anatomy of t e patient and t e surgeon%s skill and experience *it t is type of repair) : ile current surgical tec ni6ues greatly improve t e emodynamic function of t e defective eart, it does not completely correct t e defect) !atients *it repaired tetralogy of $allot often ave a leaky pulmonary valve, some degree of residual rig t outflo* tract stenosis, and damage to t e electrical system of t e eart from t e surgical incisions) <ong+term follo* up studies s o* t at t ese patients are at risk for sudden cardiac deat and eart failure) T erefore, long+term follo*+up care (y a cardiologist *it expertise in congenital eart disease is recommended to monitor t ese risks and to recommend any action, suc as interventional procedures or re+operation, if it (ecomes necessary) As *it patients t at ave undergone any eart surgery, anti(iotic prop ylaxis is indicated during dental treatment in order to prevent infective endocarditis)
http //en.%ikipedia.org/%iki/&etralogyGo!G'allot
Congenital Cardiovascular Defects

.hat is a congenital cardiovascular defect@ Congenital means in(orn or existing at (irt ) Among t e terms you may ear are congenital eart defect, congenital eart disease and congenital cardiovascular disease) T e *ord 0defect0 is more accurate t an 0disease)0 A congenital cardiovascular defect occurs * en t e eart or (lood vessels near t e eart don%t develop normally (efore (irt ) .hat causes congenital cardiovascular defects@ Congenital cardiovascular defects are present in a(out 2 percent of live (irt s) T ey%re t e most common congenital malformations in ne*(orns) In most cases scientists don%t kno* * y t ey occur) Sometimes a viral infection causes serious pro(lems) 5erman measles (ru(ella) is an example) If a *oman contracts 5erman measles * ile pregnant, it can interfere *it o* er (a(y%s eart develops or produce ot er malformations) 't er viral diseases also may cause congenital defects) #eredity sometimes plays a role in congenital cardiovascular defects) 4ore t an one c ild in a family may ave a congenital cardiovascular defect, (ut t is rarely occurs) Certain conditions affecting multiple organs, suc as Do*n%s syndrome, can involve t e eart, too) Some prescription drugs and over+t e+counter medicines, as *ell as alco ol and 0street0 drugs, may increase t e risk of aving a (a(y *it a eart defect) "esearc ers are studying ot er factors) .hat are the types of congenital defects@ 4ost eart defects eit er o(struct (lood flo* in t e eart or vessels near it, or cause (lood to flo* t roug t e eart in an a(normal pattern) "arely defects occur in * ic only one ventricle (single ventricle) is present, or (ot t e pulmonary artery and aorta arise from t e same ventricle (dou(le outlet ventricle)) A t ird rare defect occurs * en t e rig t or left side of t e eart is incompletely formed I ypoplastic eart) Cyanotic defects Anot er type of eart defect is congenital cyanotic (si0a +/'T%ik) eart defects) In t ese defects, (lood pumped to t e (ody contains less oxygen t an normal) T is causes a condition called cyanosis (si0a +/'%sis), a (lue discoloration of t e skin) Infants *it cyanosis are often called 0(lue (a(ies)0 Examples of cyanotic defects are tetralogy of $allot, transposition of t e great arteries, tricuspid atresia, pulmonary atresia, truncus arteriosus and total anomalous pulmonary venous connection)
Tetralogy of Fallot (TE%T"A<%o+De of fal+'%) as four components) T e t*o maDor ones are a large ole, or ventricular septal defect, t at lets (lood pass from t e rig t to t e left ventricle *it out going t roug t e lungs& and a narro*ing (stenosis) at or Dust (eneat t e pulmonary valve) T is narro*ing partially (locks t e (lood flo* from t e eart%s rig t side to t e lungs) T e ot er t*o components are- t e rig t ventricle is more muscular t an normal& and t e aorta lies directly over t e ventricular septal defect) T is results in cyanosis ((lueness), * ic may appear soon after (irt , in infancy or later in c ild ood) T ese 0(lue (a(ies0 may ave sudden episodes of severe cyanosis *it rapid (reat ing) T ey may even (ecome unconscious) During exercise, older c ildren may (ecome s ort of (reat and faint) T ese symptoms occur (ecause not enoug (lood flo*s to t e lungs to supply t e c ild%s (ody *it oxygen) Some infants *it severe tetralogy of $allot may need an operation to give temporary relief (y increasing (lood flo* to t e lungs *it a s unt) T is is done (y making a connection (et*een t e aorta and t e pulmonary artery) T en some (lood from t e aorta flo*s into t e lungs to get more oxygen) T is reduces t e cyanosis and allo*s t e c ild to gro* and develop until t e pro(lem can (e fixed * en t ey are older) 4ost c ildren *it tetralogy of $allot ave open+ eart surgery (efore sc ool age) T e operation involves closing t e ventricular septal defect and removing t e o(structing muscle) After surgery t e long+term outlook varies, depending largely on o* severe t e defects *ere (efore surgery) <ifelong medical follo*+up is needed) !eople *it tetralogy of $allot, (efore and after treatment, are at risk for getting an infection *it in t e aorta or t e eart valves (endocarditis)) To elp prevent t is, t ey%ll need to take anti(iotics (efore certain dental and surgical procedures)
Transposition of the great arteries T T e positions of t e pulmonary artery and t e aorta are reversed) T e aorta is connected to t e rig t ventricle, so most of t e (lood returning to t e eart from t e (ody is pumped (ack out *it out first going to t e lungs) T e pulmonary artery is connected to t e left ventricle, so most of t e (lood returning from t e lungs goes (ack to t e lungs again) Infants (orn *it transposition survive only if t ey ave one or more connections t at let oxygen+ric (lood reac t e (ody) 'ne suc connection may (e a ole (et*een t e t*o atria, called atrial septal defect, or (et*een t e t*o ventricles, called ventricular (ven+T"I7%u+ler) septal defect) Anot er may (e a vessel connecting t e pulmonary artery *it t e aorta, called patent ductus arteriosus (!A%tent DA7%tus ar+te0re+'%sis)) 4ost (a(ies *it transposition of t e great arteries are extremely (lue (cyanotic) (si0a +/'T%ik) soon after (irt (ecause t ese connections are inade6uate) To improve t e (ody%s oxygen supply, a special procedure called (alloon atrial septostomy (sep+T'S%to+me) is used) T*o general types of surgery may (e used to elp fix t e transposition) 'ne is a venous s*itc or intra+atrial (affle procedure t at creates a tunnel inside t e atria) Anot er is an arterial s*itc ) After surgery, t e long+term outlook varies 6uite a (it) It depends largely on o* severe t e defects *ere (efore surgery) <ifelong follo*+up is needed) !eople *it transposition of t e great arteries, (efore and after treatment, are at risk for getting an infection on t e eart%s *alls or valves (endocarditis)) To elp prevent t is, t ey%ll need to take anti(iotics (efore certain dental and surgical procedures)
http //%%%.americanheart.org/presenter.jhtml?identi!ier@;585
Congenital Cardiovascular Defects Treatments

4any c ildren *it congenital eart and (lood vessel defects may need medical treatment suc as diuretics, digoxin or ot er drugs) Diuretics elp t e (ody excrete *ater and salts (y promoting urination) Digoxin strengt ens t e eart%s contractions, slo*s t e eart rate and elps remove extra fluid from (ody tissues) Some c ildren may need surgery) .hat surgical procedures are used@ T e follo*ing surgical procedures are descri(ed in t is sectionArterial s*itc 8alloon atrial septostomy 8alloon valvuloplasty Damus+7aye+Stansel procedure $ontan procedure or operation !ulmonary artery (anding "oss procedure S unt or s unting procedure .enous s*itc or intra+atrial (affle
Arterial (ar+TE%re+al) s-itch ++ A surgical procedure in * ic t e maDor arteries are s*itc ed in (a(ies * ose great arteries are transposed) T e aorta is connected to t e left ventricle, * ic pumps oxygen+ric (red) (lood to t e (ody) T e pulmonary artery is connected to t e rig t ventricle, * ic pumps venous ((luis ) (lood to t e lungs) T is arterial s*itc may (e done in t e first fe* *eeks after (irt or, depending on various factors, slig tly later) If t ere%s a large ventricular (ven+T"I7%u+ler) septal defect or ot er defects related to t e transposition, t e repair gets more complicated) T en ot er surgical procedures may (e needed) 7alloon atrial septostomy (A%tre+al sep+T'S%to+me) ++ A special procedure used during eart cat eteri9ation to improve t e (ody%s oxygen supply in (a(ies * ose great arteries are transposed) It enlarges t e atrial opening and elps reduce cyanosis (si0a +/'%sis) ((lueness)) 7alloon valvuloplasty (.A<%vu+lo+plas+te) ++ A procedure in * ic a special cat eter (a tu(e introduced into a (lood vessel and t readed to t e eart) containing a deflated (alloon is inserted into t e opening of a narro*ed eart valve) : en t e (alloon is inflated, t e valve is stretc ed open& t en t e (alloon is removed) T e procedure is used *it favora(le results to improve (lood flo* in pulmonary stenosis (sten+'%sis)) It%s also used in some cases of aortic stenosis, * ere t e long+term results are still (eing studied) Damus;5aye;Stansel procedure ++ A surgical tec ni6ue used to repair congenital transposition of t e great arteries of t e eart (y dividing (cutting) t e pulmonary artery in t*o, and attac ing t e closest (proximal) section to t e ascending aorta and connecting t e fart est (distal) section to t e rig t ventricle) Fontan procedure or operation ++ A surgical procedure in * ic t e rig t atrium is connected to t e pulmonary artery eit er directly or *it a conduit) T is allo*s (lood to (ypass an incomplete or underdeveloped rig t ventricle, as in tricuspid atresia (a +T"E%9 u ) and pulmonary atresia) T e atrial defect is also closed to relieve cyanosis (si0a +/'%sis) ((lueness)) #ulmonary artery /anding ++ A procedure in * ic a surgeon places a (and around t e pulmonary artery to narro* it and reduce t e (lood flo* and ig pressure in t e lungs) T is is done to relieve suc defects as ventricular (ven+T"I7%u+ler) septal defect, atrioventricular (A%tre+o+ven+T"I7%u+ler) canal defect, and tricuspid atresia (a +T"E%9 u )) : en t e c ild is older, doctors can remove t e (and and fix t e defect *it open+ eart surgery) $oss procedure ++ A procedure in * ic a person%s diseased or a(normal aortic valve is replaced *it t e patient%s o*n pulmonary valve (pulmonary autograft)) A omograft valve (valve from a uman donor) is t en placed * ere t e pulmonary valve *as) Shunt or shunting procedure ++ An operation t at forms a passage (et*een (lood vessels to divert (lood from one part of t e (ody to anot er) It%s used to reduce t e cyanosis (si0a +/'%sis) ((lueness) in infants *it severe tetralogy of $allot (TE%T"A<%o+De of fal+'%) and t ose *it tricuspid atresia (a +T"E%9 u ) or pulmonary atresia) ?enous s-itch or intra;atrial /affle ++ A procedure t at creates a tunnel inside t e atria to elp correct transposition of t e great arteries) It redirects oxygen+ric (red) (lood to t e rig t ventricle and aorta, and redirects venous ((luis ) (lood to t e left ventricle and pulmonary artery) In t e 4ustard procedure, t e intra+atrial (affle is made of tissue from t e pericardium (pair0e+ 7A"%de+um)) In t e Senning procedure, t e intra+atrial (affle is made of flaps from t e atrial *all)
http //%%%.americanheart.org/presenter.jhtml?identi!ier@;5>6
Definition
Return to top
Congenital eart diseases are a(normalities of t e eart%s structure and function caused (y a(normal or disordered eart development (efore (irt ) Causes, incidence, and risk factors
Return to top
Congenital eart disease (C#D) is a (road term t at can descri(e a num(er of different a(normalities affecting t e eart) Congenital eart disease is, (y definition, present at (irt alt oug its effects may not (e o(vious immediately) In some cases, suc as coarctation of t e aorta, it may not present itself for many years and a fe* lesions suc as a small ventricular septal defect (.SD) may never cause any pro(lems and are compati(le *it normal p ysical activity and a normal life span) According to t e American #eart Association, approximately ?1,333 (a(ies are (orn eac year *it some type of congenital eart defect) Congenital eart disease is responsi(le for more deat s in t e first year of life t an any ot er (irt defects) 4any of t ese defects need to (e follo*ed carefully& t oug some eal over time, ot ers *ill re6uire treatment Some congenital eart diseases can (e treated *it medication alone, * ile ot ers re6uire one or more surgeries) T e risk of deat from congenital eart disease surgery as dropped from approximately ?3J in t e 2K=3s to less t an 1J in most cases today) Congenital eart disease is often divided into t*o types- t ose *it cyanosis ((lue discoloration caused (y a relative lack of oxygen) and t ose *it out cyanosis) T e follo*ing lists cover t e most common of t e congenital eart diseasesCyanotic Tetralogy of $allot Transposition of t e great vessels Tricuspid atresia Total anomalous pulmonary venous return Truncus arteriosus #ypoplastic left eart #ypoplastic rig t eart E(stein%s anomaly
/on+cyanotic .entricular septal defect (.SD) Atrial septal defect (ASD) !atent ductus arteriosus (!DA) Aortic stenosis !ulmonic stenosis Coarctation of t e aorta Atrioventricular canal (endocardial cus ion defect)
T ese a(normalities may occur as single defects or in various com(inations) .SD is t e most commonly diagnosed congenital eart defect (a(out one+t ird of all cases) and it is seen almost t ree times as often as ASD and !DA, * ic are t e next most common) T e maDority of congenital eart diseases occur as an isolated defect and are not associated *it ot er diseases) #o*ever, t ey can also (e a part of various genetic and c romosomal syndromes, suc as Do*n syndrome, trisomy 2?, Turner%s syndrome, 4arfan syndrome, /oonan syndrome, Ellis+van Creveld syndrome) Drugs, c emicals, and infections during pregnancy can also cause congenital eart a(normalities) $etal ru(ella, maternal alco ol use (fetal alco ol syndrome), and use of retinoic acid (for acne) are some causes of congenital eart disease in an infant)
Symptoms
Return to top
See t e individual congenital conditions) Signs and tests

Return to top
See t e individual congenital conditions) Treatment

Return to top
See t e individual congenital conditions) 4ost types re6uire medications andFor surgery to repair t e defect) E pectations !prognosis"
Return to top
T e prognosis varies depending on t e type and extent of t e defect) Complications

Return to top
4any of t ese disorders respond *ell to treatment) See t e individual disorders) Calling your health care provider
Return to top
Call your ealt care provider if you suspect t at your c ild as a eart pro(lem) #revention
Return to top
Avoid alco ol and ot er drugs during pregnancy) ! ysicians s ould (e made a*are t at a *oman is pregnant (efore prescri(ing for any medications for er) T e immune status for ru(ella s ould evaluated early in t e pregnancy) If t e mot er is not immune, s e must avoid any possi(le exposure to ru(ella and s ould (e immuni9ed immediately follo*ing delivery) T ere may (e some ereditary factors t at play a role in congenital eart disease) It is rare (ut not impossi(le for more t an one c ild in a family to ave a congenital eart defect) Experts (elieve t at some prescription and over+t e+counter medications and street drugs used during pregnancy increase t e risk of eart defects) T ere is, o*ever, no definitive cause t at can (e identified for most congenital eart defects) Congenital eart diseases continue to (e investigated and researc ed) 'ne of t e most important factors in determining t e outcome of a (a(y (orn *it a congenital eart disease is * et er t e defect *as found and follo*ed during t e pregnancy) T erefore, it is of t e utmost importance t at expectant mot ers receive good prenatal care) 4any of t ese defects can (e discovered on routine ultrasound examinations performed (y an o(stetrician) T e delivery can t en (e anticipated and t e appropriate medical personnel (suc as a pediatric cardiologist, a cardiot oracic surgeon, ans a neonatologist) can (e present, ready to intervene as necessary) T is can make t e difference (et*een life and deat for some (a(ies)
%pdate Date: A'0)'(**:

Apdated (y- Elc anan 8ruck eimer 488S, Director of !ediatric Cardiac Cat eteri9ation, Sc neider C ildren%s 4edical Center Israel, !etac Tikva, Israel) "evie* provided (y .eri4ed #ealt care /et*ork)
http //%%%.nlm.nih.go$/medlineplus/ency/article/66777;.htm
.hat Is a Congenital ,eart Defect@ A congenital eart defect is a structural pro(lem (or defect) in t e eart t at is present at (irt ) A (a(y%s eart (egins to develop s ortly after conception) During development, structural defects can occur) T ese defects can involve t e *alls of t e eart, t e valves of t e eart, and t e arteries and veins near t e eart) Congenital eart defects can disrupt t e normal flo* of (lood t roug t e eart) T e (lood flo* can
Slo* do*n 5o in t e *rong direction or to t e *rong place 8e (locked completely
Congenital eart defect is t e most common type of maDor (irt defect) Eac year, more t an ?3,333 (a(ies in t e Anited States are (orn *it congenital eart defects) Types of Congenital ,eart Defects T ere are many types of congenital eart defects) T ey include
A(normal passages in t e eart or (et*een (lood vessels !ro(lems *it t e eart valves !ro(lems *it t e placement or development of (lood vessels near t e eart !ro(lems *it development of t e eart itself
To (etter understand t e effects of t ese pro(lems, see 0#o* t e #eart :orks)0 Some of t ese pro(lems are descri(ed (elo*) A/normal passages in the heart or /et-een /lood vessels

Atrial septal defect !ASD" is a ole in t e *all t at separates t e upper c am(ers (atria (AC+tree+u )) of t e eart) T is causes (lood to leak from one atrium to t e ot er) ?entricular septal defect !?SD" is a ole in t e *all t at separates t e lo*er c am(ers (ventricles (.E/+tri +kuls)) of t e eart) T is causes (lood to leak from one ventricle to t e ot er) Atrioventricular septal defect !A?SD" includes an ASD, .SD, and a(normal development of t e atrioventricular valves (tricuspid (tri+CASS+pid) and mitral (4I+trul))) T is causes (lood to flo* a(normally inside t e eart) An A.SD is also kno*n as an atrioventricular canal defect)
#atent ductus arteriosus !#DA" is a persistent connection (et*een t e aorta and t e pulmonary (!A<<+mun+ary) artery) T is connection is called t e ductus arteriosus and is normally present (efore (irt ) In most (a(ies, t e vessel closes *it in a fe* ours or days after (irt ) In some c ildren, t e vessel fails to close, resulting in !DA)
#ro/lems -ith the heart valves Congenital eart defects can involve any of t e valves and include t e follo*ing types of pro(lems-
StenosisB T e valve opening is narro* and does not open completely) AtresiaB T e valve does not form, so t ere is no opening for (lood to pass from one c am(er to anot er) $egurgitationB T e valve does not close completely, so (lood can leak (ack t roug t e valve)
Examples of particular eart valve pro(lems include

Aortic valve stenosis is a narro*ing of t e aortic (ay+'"+tik) valve in t e eart t at causes it to open incompletely) T is can reduce (lood flo* to t e (ody) #ulmonary valve atresia is a defect in * ic a solid s eet of tissue forms in place of t e pulmonary valve) T is prevents (lood in t e rig t side of t e eart from traveling normally to t e lungs to pick up oxygen) #ulmonary valve stenosis is a narro*ing of t e pulmonary valve) T e narro*ing slo*s t e flo* of (lood from t e rig t side of t e eart to t e lungs) T e eart must pump arder to pus (lood t roug t e smaller opening) Tricuspid valve atresia is a defect in * ic a solid s eet of tissue forms in place of t e tricuspid valve) :it out t e tricuspid valve, (lood entering t e rig t atrium cannot travel normally to t e rig t ventricle and t en to t e lungs to pick up oxygen) E/steinCs anomaly is a defect in * ic t e tricuspid valve is (ot displaced and a(normally formed) T e valve leaks and allo*s (lood to flo* (ack into t e rig t atrium instead of to t e lungs to pick up oxygen)
#ro/lems -ith placement or development of /lood vessels near the heart
Transposition of the great vessels is a defect in * ic t e location of t e 0great vessels0 (t e aorta and pulmonary artery) coming off t e eart is s*itc ed) T e aorta comes off t e rig t ventricle instead of t e left ventricle) T e pulmonary artery comes off t e left ventricle instead of t e rig t ventricle) T erefore, (lood *it out oxygen is continually pumped to t e (ody, instead of (lood *it oxygen)
Tetralogy of Fallot is a com(ination of four defects-
o o o o
!ulmonary valve stenosis is t e narro*ing of t e pulmonary valve) T e narro*ing slo*s t e flo* of (lood from t e rig t ventricle to t e lungs) .SD is a ole in t e *all t at separates t e left and rig t ventricles) 'verriding aorta is a defect in * ic t e aorta is positioned (et*een t e left and rig t ventricles, over t e .SD) "ig t ventricular ypertrop y is t e t ickening of t e rig t ventricle) T e t ickening is caused (y t e eart aving to *ork arder (ecause of t e ot er defects)
Truncus arteriosus is a defect of t e great vessels) T e aorta and pulmonary artery do not form as separate arteries) Instead, a large artery, called t e truncus, comes from t e eart) As t e truncus leaves t e eart, it may (ranc into arteries t at carry (lood to t e (ody and to t e lungs)
Coarctation of the aorta is a narro*ing of t e aorta) It slo*s or (locks t e flo* of (lood from t e eart to t e (ody) Anomalous pulmonary venous return is a defect in * ic one or more of t e four pulmonary veins, * ic normally return oxygen+ric (lood from t e lungs to t e eart, return to t e *rong c am(er in t e eart)
#ro/lems -ith development of the heart
#ypoplastic left eart syndrome is a com(ination of defects in * ic t e left side of t e eart does not develop properly) Defects usually include mitral atresia, aortic atresia, and a tiny left ventricle)
4itral atresia occurs * en a solid s eet of tissue forms instead of t e mitral valve, * ic separates t e left atrium and t e left ventricle)
Aortic atresia occurs * en a solid s eet of tissue forms instead of t e aortic valve, * ic separates t e left ventricle from t e aorta)
Single ventricle descri(es a group of eart defects in * ic only one ventricle is present instead of t*o) It can (e a single rig t or a single left ventricle) T e ot er ventricle is usually a(sent or very tiny) #ypoplastic left eart syndrome is an example of a single ventricle defect)
Today, t e outlook for an infant (orn *it a eart defect is muc (etter t an it *as ?3 years ago) "apid advances in infant and c ild ood surgery, (etter tests, and ne* medicines elp most c ildren *it congenital eart defects) 4any c ildren (orn *it more complex or severe eart defects no* reac adult ood) Today, t ere are more t an 2 million adults living *it congenital eart defects) http //%%%.nhlbi.nih.go$/health/dci/(iseases/chd/chdG%hat.html
Congenital Heart Defects

1ore than 23!444 infants %one out of e"ery 536 to 564' are born $ith heart defects each year in the 7nited States# The defect may be so slight that the baby appears healthy for many years after birth! or so se"ere that its life is in immediate danger# 8eart defects are among the most common birth defects! and are the leading cause of birth defect*related deaths# 8o$e"er! ad"ances in diagnosis and surgical treatment o"er the past 94 years ha"e led to dramatic increases in sur"i"al for children $ith serious heart defects# 0et$een 5:;< and 5::<! the death rates from congenital heart defects dropped 32 percent# What is a congenital heart defect? A condition is called congenital $hen it is present at birth# 8eart defects originate in the early part of pregnancy $hen the heart is forming# Congenital heart defects can affect any of the different parts or functions of the heart# How does the heart work? The heart is a muscle that pumps blood to the body# .t is di"ided into four hollo$ parts called chambers# T$o chambers are located on the right side of the heart! and t$o are on the left# =ithin the heart are four "al"es %one*$ay openings' that let the blood go for$ard and keep it from going back# 0lood goes from the heart to the lungs $here it picks up o&ygen# The blood carrying o&ygen! $hich appears bright red! goes back to the heart# The heart then pumps the o&ygen*rich blood through the body by $ay of arteries# As the o&ygen is used up by the body)s tissues and organs! the blood becomes dark and returns by $ay of "eins to the heart! $here the process starts o"er again# How do heart defects affect a child? Some babies and children $ith heart defects e&perience no symptoms# The heart defect may be diagnosed if the doctor hears an abnormal sound! referred to as a murmur# Children $ith normal hearts also can ha"e heart murmurs# These are called >innocent> or >functional> murmurs# A physician may suggest tests to rule out a heart defect# Certain heart defects pre"ent the heart from pumping ade/uate blood to the lungs or other parts of the body# This can cause congesti"e heart failure# An affected child may e&perience a rapid heartbeat and breathing difficulties! especially during e&ercise %or in infants! during feeding?sometimes resulting in inade/uate $eight gain'# S$elling of the legs or abdomen or around the eyes also may occur# Some heart defects result in a pale grayish or bluish coloring of the skin %called cyanosis'! usually appearing soon after birth or during infancy# On occasion! it may be delayed until later in childhood# .t is a sign of defects that pre"ent the blood from getting enough o&ygen# Children $ith cyanosis may tire easily# Symptoms such as shortness of breath and fainting often $orsen $hen the child e&erts himself# Some youngsters may s/uat fre/uently to ease their shortness of breath# What causes congenital heart defects? .n most cases! scientists do not kno$ $hat makes a baby)s heart de"elop abnormally# 0oth genetic and en"ironmental factors appear to play roles# Among the fe$ en"ironmental factors kno$n to contribute to congenital heart defects are a "irus and certain drugs# =omen $ho contract rubella %,erman measles' during the first three months of pregnancy ha"e a high risk of ha"ing a baby $ith a heart defect# Other "iral infections also may contribute# Certain medications also increase the risk# These include the acne medication Accutane! lithium %used to treat certain forms of mental illness' and! possibly! certain anti*sei-ure medications# Drinking alcohol in pregnancy also can increase the risk of heart defects?babies $ith fetal alcohol syndrome %FAS' often ha"e them# Studies also suggest that use of cocaine in pregnancy increases the risk of these birth defects# Certain chronic illnesses in the mother also can increase the risk of heart defects# For e&ample! $omen $ith diabetes are at increased risk of ha"ing a baby $ith a heart defect! although this risk can be reduced or eliminated if the diabetes is closely controlled! starting before pregnancy# =omen $ith an inborn error of body chemistry called phenylketonuria %@A7' also are at high risk of ha"ing a baby $ith a heart defect! unless they follo$ a special diet before pregnancy and during the first trimester# Se"eral studies suggest that $omen $ho do not consume enough of the 0 "itamin folic acid before and during the early $eeks of pregnancy are at increased risk of ha"ing a baby $ith a heart defect# =hile most families ha"e no more than one child $ith a heart defect! these malformations are more likely to occur in siblings or offspring of people $ho ha"e heart defects than in unaffected families# This fact has long suggested that genetics plays a role in heart defects! at least in those families# .n fact! scientists ha"e recently disco"ered more than 544 mutations %changes' in more than a do-en genes that directly impair the heart# 1any of these mutations cause cardiomyopathy %enlargement of the heart' or heart rhythm disturbances that can be fatal in childhood! adolescence or adulthood# 8o$e"er! scientists also ha"e pinpointed se"eral mutations that affect the formation of the heart! leading to congenital heart malformations# For e&ample! in 5::: a 1arch of Dimes grantee at the 7ni"ersity of Te&as South$estern 1edical Center in Dallas disco"ered a gene that appears to contribute to a common! important group of malformations affecting the heartBs outflo$ tract and the blood "essels arising from it# Researchers at 8ar"ard 1edical School identified a gene responsible for
the heart defect called atrial septal defect %a hole bet$een the upper chambers of the heart' in four families $ith multiple members affected by heart disease# The same researchers also identified another gene mutation that causes atrial septal defects accompanied by arm and hand malformations %8olt*Oram syndrome'# Researchers appear to be on the brink of disco"ering the genes that underlie numerous heart defects# They ha"e recently identified se"eral genes that direct de"elopment of the embryonic heart in mice# This should greatly impro"e our understanding of these genesB human counterparts?and possibly lead to $ays to pre"ent the "arious heart defects that mutations of those genes may cause# 8eart defects also can be part of a $ider pattern of birth defects# For e&ample! more than one*third of children $ith the chromosomal abnormality Do$n syndrome %characteri-ed by mental retardation and physical birth defects' ha"e heart defects! as do about a /uarter of girls $ith another chromosomal abnormality called Turner syndrome %short stature! lack of se&ual de"elopment and other problems'# .n fact! appro&imately 54 percent of children $ith heart defects ha"e a chromosomal abnormality# Children $ith Do$n! Turner and certain other chromosomal abnormalities should be routinely e"aluated for heart defects# 8eart defects also are common in children $ith a "ariety of inherited disorders! including Coonan %short stature! learning disabilities'! Alagille %li"er and other problems'! 1arfan %skeletal and eye defects' and =illiams %mental retardation' syndromes# How are congenital heart defects treated? The outlook has ne"er been brighter for babies and children $ith congenital heart defects# Today! most heart defects can be corrected! or at least helped! by surgery! medicine or de"ices such as artificial "al"es and pacemakers# .n the last 24 years! ad"ances in treatment of heart defects ha"e enabled nearly 5 million 7#S# children $ith significant heart defects to sur"i"e into adulthood# 8alf the children $ho re/uire surgical repair of a heart defect no$ undergo surgery before age t$o# 7ntil fairly recently! it $as often necessary to make temporary repairs and postpone correcti"e surgery until later in childhood# +arly correcti"e surgery often pre"ents de"elopment of additional complications and allo$s the child to li"e a more normal life sooner# Some of the most common defects include
Patent ductus arteriosus# 0efore birth! much of a fetusBs blood goes through a passage$ay %ductus arteriosus' from one blood "essel to another instead of through the lungs! because the lungs are not yet in use# The passage$ay should close soon after birth! so the blood can take the normal route from heart to lungs and back# .f it doesn)t close! blood doesn)t flo$ correctly# This problem occurs most fre/uently in premature babies# .n some cases! drug treatment can help close the passage$ay# .f that doesn)t $ork! surgery can close it# Septal defects# .f the defect is a hole in the $all %septum' that di"ides the t$o upper or t$o lo$er chambers! the blood can)t circulate as it should and the heart has to $ork too hard# A surgeon can close the hole by se$ing or patching it# Small holes may heal by themsel"es or not need repair at all# Coarctation of the aorta# @art of the aorta! the large artery that sends blood from the heart to the rest of the body! may be too narro$ for the blood to flo$ e"enly# A surgeon can cut a$ay the narro$ part and se$ the open ends together! replace the constricted section $ith man*made material! or patch it $ith part of a blood "essel taken from else$here in the body# Sometimes! this narro$ed area can be $idened by inflating a balloon on the tip of a catheter inserted through an artery# Heart valve abnormalities# Some babies are born $ith heart "al"es that do not close normally or are narro$ed! closed or blocked and pre"ent blood from flo$ing smoothly# Surgeons usually can repair the "al"es or replace them $ith man*made ones# 0alloons on catheters also are fre/uently used to fi& faulty "al"es# Tetralogy of allot# A combination of four heart defects keeps some blood from getting to the lungs! so that a baby has episodes of cyanosis and may gro$ poorly# Ce$ surgical techni/ues allo$ early repair of this comple& heart defect! so that most affected children li"e normal or near*normal li"es# Transposition of the great arteries# 8ere! the positions of the t$o maDor arteries lea"ing the heart are re"ersed! so that each arises from the $rong pumping chamber# Recent surgical ad"ances ha"e enabled correction of this other$ise lethal defect in the early ne$born period# Hypoplastic left heart syndrome# A combination of defects results in a left "entricle %the heartBs main pumping chamber' that is too small to support life# This defect is the most common cause of death from congenital heart disease# Ce$ surgical procedures and heart transplants ha"e begun to sa"e some of these babies! but the long*term outlook for these babies remains uncertain#
Children and adults $ith certain heart defects! e"en after surgical repair! remain at increased risk of infection in"ol"ing the heart and its "al"es# @arents of children $ith heart defects and adults $ith repaired heart defects should discuss $ith their doctor $hether they need to take antibiotics before certain dental and surgical procedures in order to pre"ent these infections# !s there a prenatal test for congenital heart defects? A special form of sonography %looking at the fetus by means of sound $a"es' called echocardiography can accurately identify many heart defects# .f certain heart problems! such as a heart that is beating too fast or too slo$ly! are diagnosed before birth! medications may restore a normal heart rhythm before the fetal heart starts to fail# .n other cases! $here the heart defect can)t be treated before birth! kno$ing that it e&ists enables doctors to be ready to gi"e the baby the treatment it needs as soon as it is born#
Can congenital heart defects be prevented? =hile most congenital heart defects cannot yet be pre"ented! there are some steps a $oman can take that may help reduce her risk of ha"ing a baby $ith a heart defect# A $oman should be tested prior to pregnancy for immunity to rubella! and "accinated if she is not immune# @regnant $omen should a"oid alcohol and unprescribed drugs# Those $ith chronic health conditions such as diabetes! sei-ure disorders and @A7 should consult their doctors before they attempt to concei"e so that their medications andEor diets can be adDusted# Any $oman $ho could become pregnant should take a multi"itamin containing 944 micrograms of folic acid daily to reduce the risk of serious birth defects of the brain and spinal cord and! possibly! other birth defects including heart defects# ,enetic counselors can tell parents of affected children roughly $hat the chances are that any future child of theirs $ill ha"e a heart defect# Siblings of an affected child are slightly more likely than other children to ha"e the same kind of heart defect as their brother or sister# .n some cases! if the affected child)s heart defect is part of a syndrome of other defects! the recurrence risk may be higher# @arents $ho themsel"es ha"e a heart defect also are at increased risk of ha"ing a child $ith a heart defect# What research is under way on congenital heart defects? Scientists funded by the 1arch of Dimes are among many $ho are trying to learn more about the causes of heart defects! so that they can de"elop better $ays of pre"enting and treating them# For e&ample! se"eral 1arch of Dimes grantees are studying genes that may underlie specific heart defects# =hile nearly all heart defects are attributed to interactions of unkno$n genes $ith usually unkno$n en"ironmental factors! fe$ causal genes ha"e yet been linked $ith specific heart defects# ,rantees also are seeking to de"elop better $ays to treat babies $ith serious heart defects#
References American Heart Association. Congenital cardiovascular disease. Heart and Stroke A-Z Guide. American Heart Association, 2000. Brickner, M.E., et al. Congenital eart disease in adults, !art ". #e$ England %ournal o& Medicine, volume '(2, num)er (, %anuar* 2+, 2000, !ages 2,--2-'. Brickner, M.E., et al. Congenital eart disease in adults, !art 2. #e$ England %ournal o& Medicine, volume '(2, num)er ,, .e)ruar* ', 2000, !ages ''(-'(2. /e$in, M.B. 0 e genetic )asis o& congenital eart disease. 1ediatric Annals, volume 22, num)er 3, August 2000, !ages (-2-(30. Morris, C.4., et al. 0 irt*-*ear incidence o& in&ective endocarditis a&ter surger* &or congenital eart de&ect. %ournal o& t e American Medical Association, volume 2+2, num)er 3, .e)ruar* 2,, "223, !ages ,22--0'.
4:*3F6*44 5E45
Cyanosis
Cyanosis is a condition in $hich the lips! fingers! and toes appear blue# .t happens in some people $ith congenital heart defects that cause the blood to circulate abnormally# .n normal circulation! o&ygen*poor blood enters the right side of the heart# The right side of the heart pumps blood to the lungs! $here the blood picks up o&ygen# From there! it enters the left side of the heart! $hich pumps the o&ygen*rich blood to the rest of the body# .n people $ith congenital heart defects! cyanosis can happen if the defect allo$s o&ygen*poor blood from the right side of the heart to enter the left side of the heart directly! instead of tra"eling to the lungs for more o&ygen# .n the left side of the heart! the o&ygen*poor blood mi&es $ith o&ygen*rich blood to be pumped through the body# Although o&ygen*poor blood is not blue! it is not as bright red as o&ygen*rich blood# The lo$ o&ygen le"el in the blood is $hat makes the lips! fingers! and toes look blue# Congenital heart defects that cause cyanosis include
Tetralogy of Fallot Transposition of the great arteries @ersistent truncus arteriosus Tricuspid atresia @ulmonary atresia Total anomalous pulmonary "enous connection
8ypoplastic left heart syndrome http //%%%.te2asheartinstitute.org/HH3/&opics/3ond/cyanosis.c!m
Tetralogy of allot
Tetralogy of Fallot is made up of 9 heart defects A hole in the $all bet$een the lo$er chambers %the "entricles'! $hich lets o&ygen*poor blood mi& $ith o&ygen*rich blood# This is called a "entricular septal defect# A narro$ed outlet to the pulmonary artery! usually combined $ith an abnormal pulmonary "al"e# This can block blood flo$ from the lo$er*right chamber %the right "entricle' into the lungs# An aorta that straddles the $all %septum' bet$een the lo$er chambers %the "entricles'# This lets o&ygen*poor blood flo$ into the aorta %the main blood supplier to the body'# Thickened and enlarged heart muscle tissue in the lo$er*right chamber %the right "entricle'#
The narro$ed pulmonary "al"e limits blood flo$ to the lungs! and the "entricular septal defect allo$s o&ygen*poor blood to be pumped into the body along $ith o&ygen*rich blood# Together! these defects make the le"el of o&ygen in the blood too lo$# =hen o&ygen*poor blood is pumped into the body! the fingers! toes! and lips may appear blue# This condition is called cyanosis# 8o$ is it treatedG .nfants $ith tetralogy of Fallot usually need surgery# One type of surgery that is used to temporarily fi& the condition is called a shunt procedure# This is a short*term solution that allo$s more blood to reach the lungs until the defects can be corrected# This reduces cyanosis and lets the child gro$ until the problem can be fi&ed $hen he or she is older# Open heart surgery to correct the defects is usually performed bet$een the ages of ; months and 6 years# The "entricular septal defect is usually co"ered $ith a patch! the pulmonary "al"e is $idened! and a patch may also be placed on the right "entricle to increase blood flo$ to the lungs# Studies ha"e sho$n that the earlier surgery is done! the better the heart $orks later in life#
http //%%%.te2asheartinstitute.org/HH3/&opics/3ond/tetralog.c!m
3H(7
Common congenital heart defects
Congenital eart defects are a(normalities t at develop (efore (irt ) T ey can occur in t e eart%s c am(ers, valves or (lood vessels) A (a(y may (e (orn *it only one defect or several t at tend to occur in com(ination) 'f t e do9ens of eart defects, some are mild and may need minimal or no medical treatment even t roug adult ood, * ile ot ers are life+t reatening, eit er immediately to t e ne*(orn or over time) #ere%s a look at some of t e more common congenital eart defects)
http //%%%.mayoclinic.com/health/congenital#heart#de!ects/3366618 3H( 5

#ulmonary stenosis
In t is condition, t e flo* of (lood from t e rig t ventricle to t e pulmonary artery is o(structed (y narro*ing at t e pulmonary valve) : en t ere%s an o(struction (stenosis), t e rig t ventricle must pump arder to get (lood into t e pulmonary artery) T e defect may occur along *it ot er defects, suc as t ickening of t e muscle of t e rig t ventricle immediately (elo* t e valve) In many cases, pulmonary stenosis is mild and doesnUt re6uire treatment) 8ut (ecause it can cause eart failure, arr ytmias or enlargement of t e rig t eart c am(ers, surgery may (e necessary to repair t e stenosis or replace t e valve) Special (alloons to *iden t e valve ((alloon valvuloplasty) may also (e used) ttp-FF***)mayoclinic)comF ealt Fcongenital+ eart+defectsFCC333;EVslideW1
Transposition of the great arteries

:it t is defect, t e positions of t e aorta and t e pulmonary artery (t e great arteries) are reversed (transposed)) T e aorta arises from t e rig t ventricle instead of t e left and t e pulmonary artery arises from t e left ventricle instead of t e rig t) T is creates a circulatory pattern t at prevents nouris ing oxygenated (lood from reac ing t e (ody) T is condition *ould 6uickly (e fatal to a ne*(orn except it%s generally accompanied (y anot er defect T commonly a septal defect or patent ductus arteriosus T t at does allo* oxygen+ric (lood to get to t e (ody) Surgical repair is usually necessary s ortly after (irt )
8y 4ayo Clinic Staff Bun =, ;331

X 2KK>+;33E 4ayo $oundation for 4edical Education and "esearc (4$4E")) All rig ts reserved) A single copy of t ese materials may (e reprinted for noncommercial personal use only) 04ayo,0 04ayo Clinic,0 04ayoClinic)com,0 04ayo Clinic #ealt Information,0 0"elia(le information for a ealt ier life0 and t e triple+s ield 4ayo logo are trademarks of 4ayo $oundation for 4edical Education and "esearc ) CC333;E
ttp-FF***)mayoclinic)comF ealt Fcongenital+ eart+defectsFCC333;EVslideW>
Tetralogy of Fallot
T is defect is a com(ination of four (tetralogy) congenital a(normalities) T e four defects typically are ventricular septal defect (.SD), pulmonary stenosis, a misplaced aorta and a t ickened rig t ventricular *all (rig t ventricular ypertrop y)) T ey usually result in an insufficient amount of oxygenated (lood reac ing t e (ody) Complications of tetralogy of $allot (fu +<'E) include cyanosis T sometimes called 0(lue (a(y syndrome,0 since t e lips, fingers and toes may ave a (luis tinge from lack of oxygen T as *ell as poor eating, ina(ility to tolerate exercise, arr yt mias, delayed gro*t and development, and stroke) Surgical repair of t e defects is re6uired early in life)

ttp-FF***)mayoclinic)comF ealt Fcongenital+ eart+defectsFCC333;EVslideWK
,ypoplastic left heart syndrome

In t is condition, t e left side of t e eart is underdeveloped ( ypoplastic), including t e aorta, aortic valve, left ventricle and mitral valve) As a result, t e (ody doesn%t receive enoug oxygenated (lood) In t e first fe* days after a (a(y is (orn, t e ductus arteriosus remains open (patent), allo*ing normal circulation, so t e (a(y may seem fine initially) 8ut * en t e ductus arteriosus naturally closes, signs and symptoms (egin, including a (luis cast to t e skin from lack of oxygen, difficulty (reat ing and poor feeding) T is condition may (e accompanied (y an atrial septal defect) Treatment options for t is life+t reatening condition are a eart transplant or a multistage surgical procedure done during t e first fe* years of life)

ttp-FF***)mayoclinic)comF ealt Fcongenital+ eart+defectsFCC333;EVslideW2;
Truncus arteriosus
T is is a defect in * ic t e normally distinct pulmonary artery and aorta merge into one single great vessel (truncus) arising from t e rig t and left ventricles) In addition, t ere%s usually a large ventricular septal defect, essentially turning t e rig t and left ventricles into a single c am(er) T is allo*s oxygenated and unoxygenated (lood to mix) Too muc (lood may flo* to t e lungs, flooding t em and making it difficult to (reat e) It can also result in life+t reatening pulmonary ypertension) Surgery is needed to close t e septal defect *it a patc and to separate t e pulmonary arteries from t e trunk) A conduit is placed to connect t e rig t ventricle to t e pulmonary artery) 8ecause t e conduit doesn%t gro* *it t e c ild, repeat surgery may (e necessary over time)
"#$% #& TH!S T#P!C
Congenital heart defects: When your baby's born with a heart malformation Aortic valve stenosis Heart arrhythmias Heart transplant: A treatment for end-stage heart failure Heart Disease Center

ttp-FF***)mayoclinic)comF ealt Fcongenital+ eart+defectsFCC333;EVslideW2?
,o- the ,eart .orks Cour c ildUs eart is a muscle a(out t e si9e of your c ild%s fist) T e eart *orks like a pump and (eats a(out 233,333 times a day) T e eart as t*o sides, separated (y an inner *all called t e septum) T e rig t side of t e eart pumps (lood to t e lungs to pick up oxygen) T en, oxygen+ric (lood returns from t e lungs to t e left side of t e eart, and t e left side pumps it to t e (ody) T e eart as four c am(ers and four valves, and it is connected to various (lood vessels) .eins are t e (lood vessels t at carry (lood from t e (ody to t e eart, * ile arteries are t e vessels t at carry (lood a*ay from t e eart to t e (ody)
Illustration- #ealt y #eart Cross+Section ,eart Cham/ers T e eart as four c am(ers or 0rooms0Tt*o on t e left side of t e eart and t*o on t e rig t)

T e atria (AC+tree+u ) are t e t*o upper c am(ers t at collect (lood as it comes into t e eart) T e ventricles are t e t*o lo*er c am(ers t at pump (lood out of t e eart to t e lungs or ot er parts of t e (ody)
,eart ?alves $our valves control t e flo* of (lood from t e atria to t e ventricles and from t e ventricles into t e t*o large arteries connected to t e eart) T e four valves are-
T e tricuspid (tri+CASS+pid) valve is in t e rig t side of t e eart, (et*een t e rig t atrium and t e rig t ventricle) T e pulmonary valve is in t e rig t side of t e eart, (et*een t e rig t ventricle and t e entrance to t e pulmonary artery t at carries (lood to t e lungs) T e mitral (4I+trul) valve is in t e left side of t e eart, (et*een t e left atrium and t e left ventricle) T e aortic (ay+'"+tik) valve is in t e left side of t e eart, (et*een t e left ventricle and t e entrance to t e aorta, t e artery t at carries (lood to t e (ody)
.alves are like doors t at open and close) T ey open to allo* (lood to flo* t roug to t e next c am(er or to one of t e arteries, and t en t ey s ut to keep (lood from flo*ing (ack*ards) : en your eart%s valves open and close, t ey make t e familiar 0lu(+DA80 or 0lu(+DA!!0 sounds t at your doctor can ear (y using a stet oscope)

T e first sound is made (y t e tricuspid and mitral valves closing at t e (eginning of systole (SIS+toe+lee)) Systole is * en t e eart contracts, or s6uee9es, and pumps (lood out of t e eart) T e second sound is made (y t e aortic and pulmonary valves closing at t e (eginning of diastole (di+AS+toe+lee)) Diastole is * en t e eart relaxes and fills *it (lood)
Arteries T e arteries are t e maDor (lood vessels connected to your eart)

T e pulmonary artery carries (lood pumped from t e rig t side of t e eart to t e lungs to pick up a fres supply of oxygen) T e aorta is t e main artery t at carries oxygen+ric (lood pumped from t e left side of t e eart out to t e (ody) T e coronary arteries are t e ot er important arteries attac ed to t e eart) T ey carry oxygen+ric (lood to t e eart muscle, * ic must ave its o*n (lood supply to function)
?eins T e veins are maDor (lood vessels connected to your eart)

T e pulmonary veins carry oxygen+ric (lood from t e lungs to t e left side of t e eart so t e (lood can (e pumped out to t e (ody) T e vena cava is a large vein t at carries oxygen+poor (lood from t e (ody (ack to t e eart)
The ,eart .ith Tetralogy of Fallot
In tetralogy of $allot, t ere are four specific defects in t e eart)
!ulmonary valve stenosis is a narro*ing of t
e pulmonary valve and t e area (elo* t e valve) T is narro*ing slo*s
t e flo* of (lood from t e rig t side of t e eart to t e lungs) T e eart must pump arder to pus (lood t roug t e smaller opening to t e lungs * ere t e (lood picks up oxygen)
.entricular septal defect (.SD) is a
ole in t e *all t at separates t e lo*er c am(ers (ventricles) of t e eart)
'verriding aorta is a defect in t e position of t e large artery (aorta) t at takes oxygen+ric (lood to t e (ody) In a normal eart, t e aorta attac es to t e left lo*er c am(er of t e eart (ventricle)) In tetralogy of $allot, t e aorta sits (et*een t e left and rig t ventricles, over t e .SD) T is causes mixing of oxygen+ric (lood and oxygen+poor (lood)
"ig t ventricular ypertrop y is t e t ickening of t e rig t lo*er c am(er of t e eart (ventricle)) Anlike ot er muscles in your (ody, * en t e eart t ickens, it does not *ork *ell) T e eart as to pump arder to move (lood t roug t e narro*ed pulmonary valve and t e area (elo* it)
ttp-FF***)n l(i)ni )govF ealt FdciFDiseasesFtofFtofY eart*orks) tml

Tetralogi Fallot - Bahan

Diunggah oleh

Informasi Dokumen

Hak Cipta

Format Tersedia

Bagikan dokumen Ini

Bagikan atau Tanam Dokumen

Opsi Berbagi

Apakah menurut Anda dokumen ini bermanfaat?

Apakah konten ini tidak pantas?

Hak Cipta:

Format Tersedia

Tetralogi Fallot - Bahan

Diunggah oleh

Hak Cipta:

Format Tersedia

Penyakit Jantung Sianotik Sianosis adalah . Sianosis akan tampak bila Hb tereduksi > 5 g/dL.

4nemia relati! &rombosis paru Perdarahan Prognosis

Contents of this page:

Cyanotic %Tet spell%

Signs and tests

Delayed gro*t and development

Sei9ures during periods of insufficient oxygen

Calling your health care provider

T ere is no kno*n prevention) $eferences

%pdate Date: &'()'(**&

Contents of this page:

Cardiac Cardiac cat eteri9ation cat eteri9ation Alternative names

Cat eteri9ation + cardiac& #eart cat eteri9ation Definition

.hat the risks are

Cardiac cat eteri9ation may include coronary angiograp y)

%pdate Date: )'0)'(**&

Tetralogy of Fallot With Pulmonary Atresia

Email to a Colleague 5et C4EFCE for article

Drug Category- Prostaglandins ++ !5E2 (Alprostadil) promotes dilatation of t e DA in infants *it

Adult Dose #ediatric Dose Contraindications Interactions #regnancy

iuretic agents ++ T ese agents promote excretion of *ater and electrolytes (y

Adult Dose #ediatric Dose

Further Inpatient Care:

Further 2utpatient Care:

$ailure to consider t e diagnosis, especially in a ne*(orn *it cyanosis

#icture Type: 4ovie http //%%%.emedicine.com/ped/topic15:<.htm

Email to a Colleague 5et C4EFCE for article

4ortality'4or/idity: /atural istory is varia(le)

Se : Incidence is slig tly ig er in males t an females) Age: /e*(orns

$etal ydantoin syndrome $etal car(ama9epine syndrome

$etal alco ol syndrome 4aternal p enylketonuria (!7A) (irt defects

'ximetry and arterial (lood gases

Electrocardiogram (see !icture ?)

Cardiac cat eteri9ation

"ules out possi(le coronary artery anomalies

Treatment for t e acute setting of ypercyanosis includes t e follo*ingo

Consultations: !ediatric cardiologyFsurgery 4EDICATI23

#ediatric Dose Contraindications Interactions #regnancy #recautions

Drug Category- !lpha%adrenergic agonist ++ T ese agents improve emodynamic status (y

#ediatric Dose Contraindications Interactions

Further Inpatient Care:

8lalock+Taussig s unt !ott procedure :aterston s unt

Further 2utpatient Care:

e4edicine @oom .ie* (InteractiveR)

e4edicine @oom .ie* (InteractiveR) #icture Type: EC5 http //%%%.emedicine.com/5"5*=/topic595.htm

Tetralogy of Fallot !Disease"

#eart, section t roug t e middle Definition

Cyanotic STet spellS

Signs And Tests

Delayed gro*t and development Sei9ures during periods of insufficient oxygen

"evie* Date- 1F=F;33; http //%%%.bro%ardhealth.org/7>899.c!m

Definition of Tetralogy Of Fallot

Description of Tetralogy Of Fallot

Causes and Risk Factors of Tetralogy Of Fallot

Symptoms of Tetralogy Of Fallot

Diagnosis of Tetralogy Of Fallot

Treatment of Tetralogy Of Fallot

0ump to na$igation, search

diagram o! a healthy heart and one su!!ering !rom &etralogy o! !allot

ICD !" ICD # $MIM DiseasesD% MedlinePlus eMedicine

B17.: 9;5.1 7>9566 ;886 667589 emerg/595

pidemiology and tiology

Emergency 4anagement of Tet Spells

Congenital Cardiovascular Defects

$ailure to consider t e diagnosis, especially in a ne(orn it cyanosis

e pulmonary valve and t e area (elo* t e valve) T is narroing slos

ole in t e all t at separates t e loer c am(ers (ventricles) of t e eart)