Anatomy and Physiology of the Heart

Anatomy of the Heart

The essential function of the heart is to pump blood to various parts of the body. The
mammalian heart has four chambers: right and left atria and right and left ventricles. The two
atria act as collecting reservoirs for blood returning to the heart while the two ventricles act as
pumps to eject the blood to the body. As in any pumping system, the heart comes complete with
valves to prevent the back flow of blood. Deoxygenated blood returns to the heart via the major
veins (superior and inferior vena cava), enters the right atrium, passes into the right ventricle,
and from there is ejected to the pulmonary artery on the way to the lungs. Oxygenated blood
returning from the lungs enters the left atrium via the pulmonary veins, passes into the left
ventricle, and is then ejected to the aorta.

Pumping Action of the Heart

The pumping action starts with the simultaneous contraction of the two atria. This contraction
serves to give an added push to get the blood into the ventricles at the end of the slow-filling
portion of the pumping cycle called "diastole." Shortly after that, the ventricles contract, marking
the beginning of "systole." The aortic and pulmonary valves open and blood is forcibly ejected
from the ventricles, while the mitral and tricuspid valves close to prevent backflow. At the same
time, the atria start to fill with blood again. After a while, the ventricles relax, the aortic and
pulmonary valves close, and the mitral and tricuspid valves open and the ventricles start to fill
with blood again, marking the end of systole and the beginning of diastole. It should be noted
that even though equal volumes are ejected from the right and the left heart, the left ventricle
generates a much higher pressure than does the right ventricle.

Electrical Activity of the Heart

When vertebrate muscles are excited, an electrical signal (called an "action potential") is
produced and spreads to the rest of the muscle cell, causing an increase in the level of calcium
ions inside the cell. The calcium ions bind and interact with molecules associated with the cell's
contractile machinery, the end result being a mechanical contraction. Even though the heart is a
specialized muscle, this fundamental principle still applies.
One thing that distinguishes the heart from other muscles is that the heart muscle is a
"syncytium," meaning a meshwork of muscle cells interconnected by contiguous cytoplasmic
bridges. Thus, an electrical excitation occurring in one cell can spread to neighbouring cells.
Another defining characteristic is the presence of pacemaker cells. These are specialized
muscle cells that can generate action potentials rhythmically.

Under normal circumstances, a wave of electrical excitation originates in the pacemaker cells in
the sinoatrial (S-A) node, located on top of the right atrium. Specialized muscle fibers transmit
this excitation throughout the atria and initiate a coordinated contraction of the atrial walls.
Meanwhile, some of these fibers excite a group of cells located at the border of the left atrium
and ventricle known as the atrioventricular (A-V) node. The A-V node is responsible for
spreading the excitation throughout the two ventricles and causing a coordinated ventricular
contract.

Pathophysiology

Etiology
Tetralogy of Fallot occurs due to improper development of the heart in the first eight weeks of
fetal growth. Most of the time, this heart defect occurs sporadically (by chance), with no clear
reason evident for its development. Some congenital heart defects may have a genetic link,
either occurring due to a defect in a gene, a chromosome abnormality or environmental
exposure, causing heart problems to occur more often in certain families. Genetic studies
suggest a multifactorial etiology.

Predisposing Factors
Factors Present Mechanism Rationale
Age / Tetralogy of Fallot occurs during Tetralogy of Fallot
fetal growth, when the baby's heart refers to a type of
is developing. It occurs in congenital heart defect.
approximately 5 out of 10,000
infants, as a congenital disease
which means present at birth. It
constitutes 8% of all congenital
heart diseases. It is the most
common cyanotic congenital heart
disease beyond 1 week of age.
Sex / Incidence of tetralogy
of fallot is slightly
higher in males than in
females
Heredity / By which an offspring cell or A parent with
organism acquire or becomes congenital heart
predisposed to the characteristics defects has a higher
of its parent cell or organism. than normal risk of
The risk of recurrence in a family is having a child with
approximately 3%. tetralogy of Fallot.
Race There is higher risk for
tetralogy of Fallot
among white babies
than babies of other
races or ethnicities
Birth defect such as DiGeorge Syndrome is a partial Babies who have birth
Down Syndrome or gene deletion or deletion of a small defects are also more
DiGeorge Syndrome piece of chromosome 22 that likely to have tetralogy
results in heart defects, low of fallot
calcium levels, and immune
deficiency. Down syndrome is
caused by presence of an extra
chromosome 21 which results in
the increased expression of genes
located on this chromosomes that
will also result to heart defects.

• Precipitating Factors
Factors Present Mechanism Rationale
Maternal Alcoholism It is where the substances of street Increase the risk of a
or Street drugs drugs and alcohol can cross over to mother having a child
the baby through the placenta’s with tetralogy of fallot
barrier so that the baby will also
have the same blood level of alcohol
as the mother which can affect the
development of the heart especially
during 2nd month of pregnancy.

Poor nutrition of the The mother’s pre-pregnancy Poor eating habits or

pregnant mother nutrition has a large effect on the an imbalanced diet
growth of the placenta, and has adverse effects to
therefore the health of the fetus. the unborn child during
Effects of poor nutrition during pregnancy.
pregnancy include increased: illness
in the infant, risk of birth defects,
risk of mental retardation, problems
with physical development, risk of
decreased learning ability, and risk
of low birth-weight baby (less than 5
1/2 pounds).
Diabetic mother Diabetes can affect the developing Poor control of
baby throughout the pregnancy. In diabetes during
early pregnancy, a mother's pregnancy increases
diabetes can result in birth the chances for birth
defects and an increased rate defects and other
of miscarriage. Many of the birth problems for the
defects that occur affect major baby.
organs such as the brain and heart.
Viral infections, such Infections known to produce Viral infections in
as German Measles congenital defects have been pregnancy are major
(Rubella) during described with the acronym TORCH causes of maternal
pregnancy (Toxoplasma, and fetal morbidity and
others, rubella, cytomegalovirus [C mortality & congenital
MV], herpes). Other viruses now defects.
known to cause congenital
infections include parvovirus
B19(B19V), varicella-zoster
virus (VZV), West Nile
virus, measles
virus, enteroviruses, adenovirus,
and human immunodeficiency virus
 (HIV).
Age of the mother The risks increases and become The risk of tetralogy of
more complicated with the Fallot also increases
increasing age of the mother. It as the age of the
increases the complications in labor pregnant mother
and delivery, genetic and birth increases.
abnormalities, medical illnesses that
affects the fetus and mother and
pregnancy loss in some rare cases.

Symptomatology
Sign & Manifested Mechanisms Rationale
Symptoms
Heart murmur / Heart murmur results due to A sound caused by
narrowing or leaking of valves or turbulent blood flow
the presence of abnormal through the heart,
passages through which blood which can be heard
flows in or near the heart through a stethoscope

Cyanosis / The pulmonary stenosis and right Blue coloring of the

ventricular outflow tract obstruction skin, lips & nail beds
seen with tetralogy of Fallot usually caused by a low blood
limits blood flow to the lungs. When oxygen saturation in
blood flow to the lungs is restricted, the body
the combination of the ventricular
septal defect and overriding aorta
allows oxygen-poor blood ("blue")
returning to the right atrium and
right ventricle to be pumped out the
aorta to the body.
This "shunting" of oxygen-poor
blood from the right ventricle to the
body results in a reduction in the
arterial oxygen saturation so that
babies appear cyanotic, or blue.
The cyanosis occurs because
oxygen-poor blood is darker and
has a blue color, so that the lips
and skin appear blue.
Clubbing of / The physiological mechanism that Clubbing is seen most
fingers actually causes clubbing is not well commonly with
understood. Current understanding diseases of the heart
is that these diseases cause and lungs that result in
vasodilation (blood vessel dilation) less oxygen in the
in the distal circulation which leads blood.
to hypertrophy (enlarging) of the It is a build-up of tissue
tissue of the nailbeds and thus to in the fingers, causing
the clubbed fingernails. Alterations the end of the fingers to
in size and configuration of the become enlarged and
clubbed digit result from changes in the nails to curve
 the nail bed, beginning with
increased in interstitial edema in
the early process. As clubbing
progresses, the volume of terminal
portion of the digit may increase
because of an increase in the
vascular connective tissue and
change in its quality.
Difficult or poor / The child’s heart has to pump
feeding harder than normal because of a
heart defect.
Failure to gain / Often the heart has to work so hard
weight that it takes a lot of energy to keep
it pumping - these babies have to
eat more to compensate for their
heart working harder. On the flip
side, eating is an activity that takes
a lot of energy. Lots of heart babies
have a hard time getting all the
calories they need because they
get tired before they are done
feeding, so they don't take in
enough calories to gain weight.
This can lead to failure to thrive
Passing out or Due to hypoxia will normally occur
Syncope if the air breathed is contained less
than 16% oxygen at atmospheric
pressure. Because of blood’s ability
to transport oxygen is blocked or
destroyed.
Poor / Because the patients suffers lack of
development oxygen caused by heart
malformations wherein the blood
contains nutrients and oxygen can’t
pass through normal circulation
either through obstruction to flow
through the pulmonary valve or
other called right or left shunt.
Squatting during It increases vascular systemic
episodes of resistance and allows for a
cyanosis temporary reversal of the shunt.
Increases the pressure transiently
in the aorta and left ventricle,
causing less blood to move into the
left ventricle, more out the
pulmonary artery to the lungs. The
increased aortic and left ventricular
pressure reduces the rush of blood
through the septal hole from the
downward. This results
in the fingers appearing
bulb-like, or like an
upside down spoon.
Because the baby tires
easily while nursing
There is difficulty in
feeding because the
baby tires easily while
nursing. The baby isn’t
taking enough calories
or nutrients. But even if
the baby seems to
drink enough formula or
breast milk, he or she
may still gain weight
very slowly due to the
increased caloric
needs.
Cause by insufficient
oxygen in the brain
either through cerebral
hypoxia or hypotension
Due to poorly
oxygenated blood
being pumped to the
body.
Because squatting
increases the blood
flow to the lungs
 right ventricle and improves blood
circulation to the lungs, so more
red blood reaches the tissues.
Rapid breathing / Results in low oxygenation of blood Due to poorly
due to the mixing of oxygenated oxygenated blood
and deoxygenated blood in the left being pumped to the
ventricle via VSD and preferential body
flow of the mixed blood from both
ventricles through the aorta
because of the obstruction to flow
through the pulmonary valve thus
causing shortness of breathing
Cool and / Due to low blood oxygen levels the Due to low blood
clammy skin distribution of the blood to the skin oxygen levels
also decreases, thus causing it to
have cool and clammy skin.
Fussiness or / Some babies do not
irritability have noticeable
cyanosis (blue color of
the skin, lips and
nailbeds), but instead
may be very irritable or
lethargic due to a
decreasing amount of
oxygen available in the
bloodstream.
Not respond to
you or touch
Tetralogy spells ⁄ Patients with Tetralogy of Fallot A spell occurs when
(“Tet” spells) have spells where they suddenly there is sudden drop in
turn a blue color due to a low the oxygen level in the
oxygen in their bloodstream. The blood resulting from a
main cause of inadequate oxygen transient increase in
in the bloodstream is obstruction of resistance to blood flow
blood flow to the pulmonary to the lungs with
arteries which are supposed to increased preferential
send blood to the lungs where it flow of desaturated
should be picking up oxygen. When blood to the body.
the patient has an less blood flow
through the lungs due to elevated
resistance in the pulmonary blood
vessels, the patient turns blue.
During a Tet spell, much of the
blood goes directly from the right
heart to the left heart through the
TOF defects such as the
Ventricular Septal Defect and the
overriding aorta, the bluish color of
the hypercyanotic spell is
observed. The blue color means
 hemoglobin molecules in the blood
are not bound to oxygen
(deoxygenated hemoglobin is blue
in color). The hypercyanotic (Tet)
spells can be quite severe and may
lead to death or organ damage.
Polycythemia / Usually patients with Tetralogy of Polycthemia, an
Fallot have lower than normal increase in blood
oxygen levels in their bloodstream. volume as a result of
The patient’s body makes extra red an increase in the
blood cells as a way to deal with number of red blood
the lower oxygen levels in the cells, happens as a
blood. The red blood cell count and physiologic response to
hemoglobin may be elevated as the an increased need for
body attempts to compensate for oxygenation due to low
the lack of oxygen to the tissues. oxygen level in the
The extra red blood cells result in a bloodstream.
thicker than normal blood–the
condition is referred to as
polycythemia (meaning “many
cells”).

Narrative Pathophysiology
The actual cause of tetralogy of fallot is unknown but certain studies have identified
certain predisposing and precipitating factors that lead to this disease. Predisposing factors
include the sex, age, race, heredity, Down syndrome or DiGeorge syndrome and the
precipitating factors are maternal alcoholism or street drugs, poor nutrition of the pregnant
mother, diabetic mother, viral infections such German Measles(Rubella) during pregnancy, and
age of the mother. These factors are to be discuss one by one in a separate table to in order for
us to see if the patient if these factors are present in our patient or not. First Tetralogy of Fallot
has primary four malformations. Tetralogy denotes a four-part phenomenon in various fields,
including literature, and the four parts the syndrome’s name applies are its four signs. This is not
to be confused with the similarly named teratology, a field of medicine concerned with abnormal
development and congenital malformations, which thereby includes tetralogy of Fallot as part of
its subject matter.
As such, by definition, tetralogy of Fallot involves exactly four heart malformations which present
together:
A: Pulmonary stenosis
A narrowing of the right ventricular outflow tract and can occur at the pulmonary valve (valvular
stenosis) or just below the pulmonary valve (infundibular stenosis). Infundibular pulmonic
stenosis is mostly caused by overgrowth of the heart muscle wall (hypertrophy of the
septoparietal trabeculae),[8] however the events leading to the formation of the overriding aorta
are also believed to be a cause. The pulmonic stenosis is the major cause of the malformations,
with the other associated malformations acting as compensatory mechanisms to the pulmonic
stenosis.[9] The degree of stenosis varies between individuals with TOF, and is the primary
determinant of symptoms and severity. This malformation is infrequently described as sub-
pulmonary stenosis or subpulmonary obstruction.
B: Overriding aorta
An aortic valve with biventricular connection, that is, it is situated above the ventricular septal
defect and connected to both the right and the left ventricle. The degree to which the aorta is
attached to the right ventricle is referred to as its degree of "override." The aortic root can be
displaced toward the front (anteriorly) or directly above the septal defect, but it is always
abnormally located to the right of the root of the pulmonary artery. The degree of override is
quite variable, with 5-95% of the valve being connected to the right ventricle.
C: ventricular septal defect (VSD)
A hole between the two bottom chambers (ventricles) of the heart. The defect is centered
around the most superior aspect of the ventricular septum (the outlet septum), and in the
majority of cases is single and large. In some cases thickening of the septum (septal
hypertrophy) can narrow the margins of the defect.
D: Right ventricular hypertrophy
The right ventricle is more muscular than normal, causing a characteristic boot-shaped (coeur-
en-sabot) appearance as seen by chest X-ray. Due to the misarrangement of the external
ventricular septum, the right ventricular wall increases in size to deal with the increased
obstruction to the right outflow tract. This feature is now generally agreed to be a secondary
anomaly, as the level of hypertrophy generally increases with age.

There is anatomic variation between the hearts of individuals with tetralogy of Fallot. Primarily,
the degree of right ventricular outflow tract obstruction varies between patients and generally
determines clinical symptoms and disease progression.
The severity of obstruction to right ventricular outflow determines the direction of blood flow. If
the subpulmonary stenosis is mild, the abnormality resembles an isolated VSD, and the shunt
may be left to right, without cyanosis (so called “pink tetralogy”). As the obstruction increases in
severity, there is commensurately greater resistance to right ventricular outflow. As it
approaches the level of systemic vascular resistance, right-to-left shunting predominates and,
along with it cyanosis. With increasing severity of subpulmonic stenosis, the pulmonary arteries
are progressively smaller and thinner walled (hypoplastic), and the aorta is progressively larger
in diameter. As the child grows and the heart increases in size, the pulmonic orifice does not
expand proportionally, making the obstruction even worse. Thus most infants with tetralogy are
cyanotic from birth or soon thereafter. The subpulmonary stenosis, however protects the lung
from pressure overload, and right ventricular failure is rare because the right ventricle is
decompressed into the left ventricle and aorta. Complete surgical repair is possible for classic
tetralogy.
Tetralogy of Fallot with pulmonary atresia (pseudotruncus arteriosus) is a severe variant in
which there is complete obstruction (atresia) of the right ventricular outflow tract, causing an
absence of the pulmonary trunk during embryonic development. In these individuals, blood
shunts completely from the right ventricle to the left where it is pumped only through the aorta.
The lungs are perfused via extensive collaterals from the systemic arteries, and sometimes also
via the ductus arteriosus. Clinical presentation in tetralogy of Fallot with pulmonary atresia
depends on the source and volume of pulmonary blood flow. This usually occurs via the DA
and/or aortopulmonary collaterals. The newborn infant, in whom the DA is the sole source of
pulmonary blood flow, becomes increasingly cyanotic as the DA closes. Early recognition of the
diagnosis along with prompt institution of prostaglandin E1 (PGE1) infusion is life saving in this
instance. Conversely, when the aortopulmonary collaterals constitute the source of pulmonary
blood flow, the clinical presentation may vary from cyanosis with inadequate pulmonary blood
flow to no cyanosis with increased pulmonary blood flow. Uncommonly, pulmonary blood flow is
sufficiently increased to cause symptoms due to pulmonary overcirculation. Older infants and
children commonly present with cyanosis. Hypoxia usually progresses further as the child
outgrows the source of pulmonary blood flow. Early surgical intervention has improved survival
in these patients. Untreated, Tetralogy of Fallot rapidly results in progressive right ventricular
hypertrophy due to the increased resistance on the right ventricle. This progresses to heart
failure (dilated cardiomyopathy) which begins in the right heart and often leads to left heart
failure. Actuarial survival for untreated Tetralogy of Fallot is approximately 75% after the first
year of life, 60% by four years, 30% by ten years, and 5% by forty years.
Patients who have undergone total surgical repair of Tetralogy of Fallot have improved
hemodynamics and often have good to excellent cardiac function after the operation with some
to no exercise intolerance (New York Heart Association Class I-II). Surgical success and long-
term outcome greatly depends on the particular anatomy of the patient and the surgeon's skill
and experience with this type of repair.
Ninety percent of patients with total repair as infants develop a progressively leaky pulmonary
valve as the heart grows to its adult size but the valve does not. Patients also may have
damage to the electrical system of the heart from surgical incisions if the middle cardiac nerve is
accidentally tapped during surgery. If the nerve is touched, it will cause abnormalities as
detected by EKG and/or arrhythmias.
Long-term follow up studies show that patients with total repair of TOF are at risk for sudden
cardiac death and for heart failure. Therefore, lifetime follow-up care by an adult congenital
cardiologist is recommended to monitor these risks and to recommend treatment, such as
interventional procedures or re-operation, if it becomes necessary.
The use of antibiotics is no longer required by cardiologists and varies from case to case.
The outcome varies for every child. If the condition is left uncorrected it may be fatal, but the
prognosis has greatly improved over the years for babies with pulmonary atresia. Some factors
that affect how well the child does include how well the heart is beating, the condition of the
blood vessels that supply the heart, and how leaky the other heart valves are. Most cases of
pulmonary atresia can be helped with surgery. If the patient's right ventricle is exceptionally
small, many surgeries will be needed in order to help stimulate normal circulation of blood to the
heart.
If uncorrected, babies with this type of congenital heart disease may only survive for the first few
days of life while the fetal shunts between left and right circulations remain patent. However,
there are cases of uncorrected Pulmonary Atresia in which the baby has survived.
Many children with pulmonary atresia will go on to lead 'normal' lives. Patients are usually seen
regularly throughout their lifetime by a cardiologist to be sure that their heart is functioning
properly. They have an increased risk of endocarditis.
Further complications may arise to Residual right ventricular dysfunction from hypoplastic
pulmonary arteries or conduit stenosis, cyanosis, hypoxemia, polycythemia, Atrioventricular
conduction abnormalities, right bundle branch block, ventricular arrhythmias in the postoperative
patients and significant pulmonic valve regurgitation which may result in death or disability in
early adulthood.
B. Discharge Planning
Medication
Health Teachings
Continue taking home medications as
prescribed by doctor
Explain the mechanisms of drug, effects and
contraindications to the mother as well as the
side effects.
Place the infant in upright position during
giving medications, feeding & oral fluids.
Safe storage of medication (keep medication
in original labelled bottled; don’t mix
medications, store in a safe place away from
children, keep refrigerated if necessary, keep
away from heat and sunlight as indicated.
Exercise
Health Teachings
Keeping or lowering your baby’s anxiety or
stress
Limit certain types of exercise or activities
Treatment
Health Teachings
Educate the families about anatomic details
and long-term prognosis and the potential
need for multiple surgeries and
catheterizations, and postoperative
complications.
Instruct the parents on ways to prevent
overexerting their child, such as feeding him
slowly and providing smaller and more
frequent meals.
Encourage the parents to recruit other family
members in the care of the child
Rationale
To provide continuous constant care to the
patient even at home and for further treatment
of the disease or illnesses
Provide information to the mother so that they
will better understand what they are taking to
their infant.
Knowledge of the side effects will help them
stay alert in the event that they occur so that
appropriate action can be taken.
Prevents aspiration and promotes swallowing
of the medicine.
To keep harm from children, and prevent from
being destroyed and maintain its level of
potency.
Rationale
Can help prevent “tet spells” and save the
baby’s energy
You can try to predict what your baby needs
to stop or to lessen crying. For example,
picking up an infant slowly and speaking in a
soothing voice can avoid startling a baby.
It can increase the cardiac load of the baby
which can lead to cyanosis. Explain tetralogy
of Fallot to the parents. Inform them that their
child will set his own exercise limits and will
know when to rest. Make sure they understand
that their child can engage in physical activity,
and advise them not to be overprotective.
Rationale
To provide information and better
understanding of the disease-causing
condition enabling them to choose treatment
options with better understanding of it.
It would be easier for your baby to handle.
remaining calm may decrease the baby’s
anxiety and that anticipating his needs may
minimize crying.
to help prevent the parents exhaustion.
During a “Tet” spell:

* bring the baby’s or child’s knees up tight *It increases the blood flow to the lungs. It
against his or her chest (this is called the increases vascular systemic resistance and
knee- chest position) allows for a temporary reversal of the shunt
* Have the child squat down * It increases the blood flow to the lungs

• Instruct the mother to take the child to • To provide emergency treatment.

a hospital emergency department right away
if the child develops any of the following
symptoms, if the child develops any of the
following symptoms: Bluish discoloration,
Trouble breathing, Seizures, Fainting and
Extreme fatigue or weakness

Hygiene
• Promote good hygiene & prevent • A child with severe heart defects may
infection. need to take preventive antibiotics before
• certain dental and surgical procedures.
•
Warn the parents to keep their child away from To prevent infective endocarditis and other
people with infections. Urge them to infections since they are higher risk to have
encourage good dental hygiene, and tell them these.
to watch for ear, nose, and throat infections
and dental caries, all of which necessitate
immediate treatment. When dental care,
infections, or surgery requires prophylactic
antibiotics, tell the parents to make sure the
child completes the prescribed regimen.
Instruct mother and other family members To prevent contamination and infection.
taking care of the child to maintain proper
hygiene like washing their hands before
handling the baby.

Out patient
Encourage regular follow up with a pediatric For routine heart check ups & routine exams
cardiologist or a pediatrician. and especially to monitor for life-threatening
arrhythmias (irregular heart rhythms)

Diet
Continue breastfeeding or appropriate formula
feeding
Give supplement or extra feeding that can give
the baby more calories, vitamins, or iron.
Because breast milk is the optimal food for
infant and may continue for up to two years
and beyond. And also it enhances the immune
system of the baby.
It can enhance the nutrition and immune
system
both ventricles recieve and pump out the same amount of blood in a given amount of time. the
main difference is that the left ventricle has to pump the blood against a greater resistance in
the aorta and peripheral circulation whereas the right ventricle pumps blood into the pulmonary
circulation, which has less resistance. this requires the a greater force of contraction (and more
muscle mass) for the left ventricle
Blalock–Taussig shunt
From Wikipedia, the free encyclopedia
(Redirected from Blalock-Taussig shunt)
The Blalock-Taussig shunt (also referred to as a Blalock-Thomas-Taussig shunt) is a surgical
procedure to give palliation to cyanotic heart defects which are common causes of blue baby
syndrome. In modern surgery, this procedure is temporarily used to direct blood flow to the
lungs and relieve cyanosis while the infant is waiting for corrective or palliative surgery.
One branch of the subclavian artery or carotid artery is separated and connected with the
pulmonary artery. The lung receives more blood with low oxygenation from the body. The first
area of application was tetralogy of Fallot.
[edit]Alternatives

The procedure is no longer in use in its original form. Now a length of artificial tubing, 3 to 4
millimeters in diameter, is sewn between either the subclavian or the carotid artery and the
corresponding side branch of the pulmonary artery, thus obviating the need to cut off blood
supply and making it easier to regulate the blood flow to the lungs. Some centers now use a
shunt directly from the right ventricle to the pulmonary artery, a Sano shunt. This is done to
avoid the reduced diastolic blood flow in the coronary circulation associated with the Blalock-
Taussig shunt.

Patent ductus arteriosus (PDA) is a congenital disorder in the heart wherein a neonate's ductus
arteriosus fails to close after birth. Early symptoms are uncommon, but in the first year of life
include increased work of breathing and poor weight gain. With age, the PDA may lead to
congestive heart failure if left uncorrected.

Etiology
A patent ductus arteriosus can be idiopathic (i.e. without an identifiable cause), or secondary to
another condition. Some common contributing factors in humans include:
Preterm birth
Congenital rubella syndrome
Chromosomal abnormalities such as Down syndrome
[edit]Normal ductus arteriosus closure
In the developing fetus, the ductus arteriosus (DA) is the vascular connection between the
pulmonary artery and the aortic arch that allows most of the blood from the right ventricle to
bypass the fetus' fluid-filled compressed lungs. During fetal development, this shunt protects the
right ventricle from pumping against the high resistance in the lungs, which can lead to right
ventricular failure if the DA closes in-utero.
When the newborn takes its first breath, the lungs open and pulmonary vascular resistance
decreases. After birth, the lungs release bradykinin to constrict the smooth muscle wall of the
DA and reduce bloodflow through the DA as it narrows and completely closes, usually within the
first few weeks of life. In most newborns with a patent ductus arteriosus the blood flow is
reversed from that of in utero flow, i.e. the blood flow is from the higher pressure aorta to the
now lower pressure pulmonary arteries.
In normal newborns, the DA is substantially closed within 12–24 hours after birth, and is
completely sealed after three weeks. The primary stimulus for the closure of the ductus is the
increase in neonatal blood oxygen content. Withdrawal from maternal circulating prostaglandins
also contributes to ductal closure. The residual scar tissue from the fibrotic remnants of DA,
called the ligamentum arteriosum, remains in the normal adult heart.
[edit]Patent ductus arteriosus
The ductus arteriosus is a normal fetal blood vessel that closes soon after birth. In a patent
ductus arteriosus (PDA) the vessel does not close and remains "patent" resulting in irregular
transmission of blood between two of the most important arteries close to the heart, the aorta
and the pulmonary artery. PDA is common in neonates with persistent respiratory problems
such as hypoxia, and has a high occurrence in premature children. In hypoxic newborns, too
little oxygen reaches the lungs to produce sufficient levels of bradykinin and subsequent closing
of the DA. Premature children are more likely to be hypoxic and thus have PDA because of their
underdeveloped heart and lungs.
A patent ductus arteriosus allows a portion of the oxygenated blood from the left heart to flow
back to the lungs by flowing from the aorta (which has higher pressure) to the pulmonary artery.
If this shunt is substantial, the neonate becomes short of breath: the additional fluid returning to
the lungs increases lung pressure to the point that the neonate has greater difficulty inflating the
lungs. This uses more calories than normal and often interferes with feeding in infancy. This
condition, as a constellation of findings, is called congestive heart failure.
In some cases, such as in transposition of the great vessels (the pulmonary artery and the
aorta), a PDA may need to remain open. In this cardiovascular condition, the PDA is the only
way that oxygenated blood can mix with deoxygenated blood. In these cases, prostaglandins
are used to keep the patent ductus arteriosus open.
[edit]Prognosis
Without treatments, the disease may progress from left-to-right (noncyanotic heart) shunt to
right-to-left shunt (cyanotic heart) called Eisenmenger syndrome.
The ductus arteriosus is a normal fetal structure, allowing blood to bypass circulation to the
lungs. Since the fetus does not use his/her lungs (oxygen is provided through the mother’s
placenta), flow from the right ventricle needs an outlet. The ductus provides this, shunting flow
from the left pulmonary artery to the aorta just beyond the origin of the artery to the left
subclavian artery. The high levels of oxygen which it is exposed to after birth causes it to close
in most cases within 24 hours. When it doesn’t close, it is termed a Patent Ductus Arteriosus.

The defect often corrects itself within several months of birth, but may require infusion of
chemicals, the placement of "plugs" via catheters, or surgical closure.
The flow pattern is similar to the septal defects noted above, except that the shunting occurs
outside of the heart. The left ventricle has to pump blood out through the aorta, only to have
some of it flow to the lower pressure pulmonary artery, and directly back to the left atrium and
ventricle. If a large PDA is not corrected, then the pressures in the pulmonary arteries may
become very high and induce changes in the arteries themselves such that even closure of the
defect will no longer improve the patient. In this case, the pressures in the right side of the heart
are high enough that blood may begin to flow from the right to the left side of the heart. This
situation is called "Eisenmenger’s syndrome", a condition which may result from several similar
abnormalites.