IDENTITAS PASIEN

• Ny. R
• Usia 40 tahun
• No RM 176 03 xx
• KU : sesak napas saat beraktivitas
• RPS : pasien mengeluh sesak napas sejak 3 tahun SMRS. Sesak dirasakan jika pasien beraktivitas berat dan membaik
jika istirahat. Pasien memeriksakan diri ke RS di Solo dikatakan kebocoran sekat jantung dan di sarankan periksa ke
RS Sardjito. Tahun 2017 pasien melakukan pemeriksaan echocardiography dan penyadapan serta disarankan
konferensi bedah dengan hasil penutupan sekat jantung dengan pembedahan.
• Riwayat berdebar-debar disangkal, nyeri dada disangkal, keringat dingin disangkal.
• RPD : riwayat hipertensi dan dyslipidemia sejak 5 tahun terakhir.
• RPK : riwayat penyakit jantung pada keluarga disangkal, hipertensi dan diabetes disangkal.
 PEMERIKSAAN FISIK

KU : sedang, CM
Vital Sign : TD : 140/100, Nadi : 72 x/menit, RR : 20 x/menit, Suhu : 36,8, SpO2 98 % RA
Kepala/leher : CA (-), SI (-), JVP 5+2 cmH2O
Thorax : cor : ictus cordis teraba di SIC V linea mid clavicularis sinistra, kesan cardiomegaly
(batas kanan terletak di SIC V line sternalis dextra), S1, split S2, pan sistolik murmur di SIC III
sternalis sinistra. Pulmo : simetris, sonor pada lapang paru, SDV (+/+), RBB (-/-), wheezing (-/-)
Abdomen : flat, BU (+) normal, timpani, supel, nyeri tekan (-), hepar dan lien tak teraba.
Ekstremitas : akral hangat, nadi kuat, edema (-)
EKG
LABORATORIUM

• Hb : 14,6 • Albumin : 4,49 • CK/CPK : 88 • SWAB Tenggorokan :

• AE : 4.73 • SGOT : 15 • CKMB : 10 • Bakteri Gram (+) : coccus berderet

• Hs – Troponin I : 28.80 • Bakteri gram (-) : basil soliter

• AL : 5.31 • SGPT 12
• HbA1C : 5,6 % • Hasil kultur identifikasi dan sensitifitas :
• Hmt 43,2 • BUN : 13,1
• Chol. Total : 180 • Organisme 1 : Klebsiella pneumoniae
• AT : 241 • Creatinin : 0,82 • Organisme 2 : Enterococcus faecalis
• HDL : 48
• MCV : 91,3 • GDS : 82 • LDL : 132
• MCH : 30,9 • Trigliserid : 85 • BTA : - /-
• HBsAg : Non reaktif

• PPT : 15,5 • Na : 138

• Kontrol : 13,2 • K : 3,57

• APTT : 38,4 • Cl : 102
• Kontrol : 31,3
• INR 1,14
EKOKARDIOGRAFI (TTE) – 19/02/2016

• ASD sekundum ukuran 2.8-3.3 cm, L to R shunt

• Global fungsi sistolik LV normal dengan EF 62% (simpson 60%)
• IVS paradox, LV D shape
• Fungsi sistolik RV normal
• MR mild
• TR moderate, PR mild, PH Severe
• Saran : TEE, RHC
RIGHT HEART CATH – 3/7/2016

• Tampak vena inominata

• Kateter dapat menyebrang dari RA ke LA
• Air rest : FR 1,33; PARI 9,29; Cop 5.75; Clp 3.86; COs 4.3; Cls 2.9
• Post O2 test : FR 6; PARI 2.18; Cop 8,62; Clp 5,79, Cos 1,43; Cls 0,97
• LM : normal
• LAD : normal
• LCx : normal
• RCA : normal
• Kesimpulan ASD low flow high resistence reactive O2 test, normokoroner
RHC – 5/8/2017

• Tampak v. inominata
• Tampak ASD
• Pre oksigen test
Pressure Ao 135/81, PA 75/28 (43)
CO : 2, CI 1,39, PARI 5,7, FR 2,27
Post oksigen test
Pressure Ao : 138/86 (107), PA 78/19 (47)
CO 3,3, Cl 2,3, PARI 3,37, FR 2,24
Kesimpulan : ASD High flow low resistance dengan non reactive oxygen test
EKOKARDIOGRAFI (TTE) - 12/03/2018

• ASD sekundum ukuran 2,8-3,3 cm, bidirectional shunt dominan L to R

• RA dan RV dilatasi
• Global fungsi sistolik LV normal dengan EF 71%
• IVS paradox, LV D shape
• Fungsi sistolik RV normal
• MR mild, PR mild
• TR mild to moderate, high probability of PH
RONTGEN THORAKS – 31/07/2018

• Pneumonia bilateral dengan emfisematous lung

• Cardiomegali LAH RVH
• Hipertensi pulmonal
DIAGNOSIS

• Atrial septal defect high flow low resistence, pulmonary hypertension moderate

PLAN
• Pro ASD closure by surgery
ATRIAL SEPTAL
DEFECT
ANGGIA FITRIA AGUSTIN
INTRODUCTION

• Asianosis
• Characterized by defect in the interatrial septum causing a left to right flow between the
atria.
• Severity depends on :
• Size of shunt
• Size of defect
• Associates anomaly

• Resulting in spectrum of:

Asymptomatic to right sided overload, PAH, atrial arrhytmias
EMBROYOLGY OF HEART
Septum formation in primitive
atrium
INCIDENCE & ETIOLOGY

• ASD constitutes 8-10%of congenital heart defect in children

• 56 per 100.000 live births
• Female : Male ratio  3:1 (ASD Secundum)
• Etiology : unknown
• Risk factors :
• Genetic factor
• Environment (antenatal teratogenic drugs, infection)
 GENETICS
 The genetic basis of ASD is not completely understood.
 In the majority of cases this is a sporadic lesion, yet some
homeobox gene defects have been found to explain some
of the well known familial cases of ASDs, such as NKX2-
chromosome-5, which has an autosomal dominant
inheritence and AVconduction defect.
 Other genetic syndromes with skeletal abnormalities HOLT-
ORAM Syndrome, which is accused by mutations in the
transcription factor TBX5, essential in development of both
the heart and upper limbs.

 ASD can be part of many other syndromes like DOWN

syndrome and Noonansyndrome
 HEMODYNAMICS

Desaturated blood enters

the right atrium from the
vena cava at a volume of 3
L/min/m2 and mixes with
an additional 3 L of fully
saturated blood shunting
left to right across the ASD
Results in :
increase in oxygen saturation
in the right atrium.
Six liters of blood flows
through the tricuspid valve
and causes a mid-diastolic
flow rumble.

Oxygen saturation may be

slightly higher in the right
ventricle because of
incomplete mixing at the
atrial level.
The full 6 L flows across
the right ventricular
outflow tract and causes a
systolic ejection flow
murmur.
Six liters returns to the left
atrium, with 3 L shunting
left to right across the
defect and 3 L crossing the
mitral valve to be ejected
by the left ventricle into
the ascending aorta.
PATHOPHYSIOLOGY
 TYPES

Ostium Secundum (75-85%)

Ostium Primum (10-15%)
Sinus Venosus (5-10%)
Coronary Sinus septal
defect (1%)
Ostium Secundum

• Most common type.

• Defect in the region of
fossa ovalis.
• Single or Multiple.
• May be associated with
partial anomalous venous
return most commonly of
the right upper pulmonary
vein.
Ostium Primum

• Situated in the lower

portion of the artrial
septum and overlies the
mitral and tricuspid valve.

• Often associated with

clefts in the anterior mitral
and septal tricuspid valve
leaflets and small VSDs.
Sinus Venosus

• Least common type.

• Situated in the upper
part of atrial septum in
close relation to the
entry of the Superior
venacava.
• Abnormal fusion
between embryologic
sinus venosus and
atrium.
ACCORDING TO SIZE

In younger children • In older children

• Small defect: <3 mm • Small defect: <6 mm
• Moderate defect: 3 – 8 • Moderate defect: 6 –
mm 12mm
• Large defect: >8 mm • Large defect: >12 mm
ASSOCIATIONS
Associated malformations are nearly 30% of cases. Like:
• Secundum ASD
● Pulmonic stenosis
● Mitral valve prolapse
● Partial anomalous pulmonary
venous connection
• Primum ASD
● Cleft mitral valve
● Discrete subaortic stenosis
• Sinus Venosus septal defect
● Partial anomalous pulmonary
venous return
• Coronary Sinus septal defect
● Partial and total anomalous
pulmonary venous return
● Persistent left superior vena
cava
SYMPTOMSANDSIGNS
J

Vary with the size of defect.

Small defect: Asymptomatic and is usually diagnosed during
a routine health check up.
Large defect: Symptomatic and patients usually present
with
 Failure to thrive.
 Easy fatigability.
 Increased perspiration
 Recurrent Pulmonary infections.
 Platypnea
 Orthodeoxia
ONEXAMINATION

General examination
 Appearance: Usually normal
 Heart rate: Normal
 Respiratory rate: Normal
 Weight and height: may be less than 10th centile.
 PRECORDIUM

 Inspection:
 Slight prominence of
precordium

 Palpation:
 Apex beat may be shifted to left
 P2 may be palpable
 Left parasternal heave may be
present
 AUSCULTATION:

S1 is normal
S2 is widely splitted and fixed
Ejection systolic murmur
,medium pitched, soft, grade 1-
3/6 & best heard at left 2nd & 3rd
ICS
A diastolic flow rumble across
the tricuspid valve region.
 INVESTIGATIONS
Routine tests :(CBC, septic screening, s.electrolyte, s. creatinine,
blood grouping, coagulation profile, etc) should be done before
management.
Diagnostic Investigations includes-
-X-ray
-Ecg
-Echocardiography
-Sometimes cardiac catheterization
XRAYFINDINGS

 Cardiomegaly
 RA enlargement
 RV enlargement
 Full pulmonary conus
 Increased pulmonary
vascular markings
 Plethoric lung fields
ECG

Also note that the aVF

Enlarged ‘p’ is predominantly
rsR’ seen and tall R
wave upwards as compared
wave
indicating to Lead I indicating
Indicating RBBB and
Right atrial Right Axis Deviation
RVH
hypertrophy
LADwith rSR’ in V1 is suggestive
of Ostium primum defect
 Associated lesions-
- Right atrial and RV enlargement with diastolic flattening and
paradoxical IVS motion are evidence of RV volume overload and a
significant left- to-right shunt,
- mitral valve prolapse,
- cleft mitral valve,
- anomalous pulmonary veins.
 Contrast echocardiography with intravenous agitated saline may be
used to confirm the presence of a shunt if color Doppler are not
conclusive.
 ECHOCARDIOGRAM
LA
RA
 Primary diagnostic imaging
modality for ASD.
RV
 Provides:
-exact localization of ASD
-size of ASD
-measurement of septal rims
-Confirmation of the shunt
-Abnormal motion of
ventricular septum.
-Associated lesions can be identified
CARDIAC
CATHETERIZATION
 Patients with the classic features of a
hemodynamically significant ASD on physical
examination and chest radiography, in whom
echocardiographic identification of an isolated
secundum ASD is made, need not undergo diagnostic
catheterization before repair, with the
Exception: anolder patient, in whom pulmonary
vascular resistance may be a concern.
 NATURALHISTORY

 In patients with an ASD <3 mm in size

diagnosed before 3 months of age, spontaneous
closure occurs in 100% of patients at 1½ years of age.
 Spontaneous closure occurs more than 80% in
patients with defects between 3-8 mm before 1½
years of age.
 An ASD with a diameter > 8mm rarely closes
spontaneously
Most children with an ASD remain active and asymptomatic.
Rarely, congestive heart failure (CHF) can develop in infancy.

If untreated, pulmonary hypertension and subsequent CCF may

develop during or after third decade, and reversal of shunt may
occur (rare), it may be progressive with pregnancy

With or without surgery, atrial arrhythmias (flutter or

fibrillation) may occur in adults.
Infective endocarditis does not occur in patients with
isolated ASDs.

Cerebrovascular accident, resulting from paradoxical

embolization through an ASD,is a rare complication.

Mitral stenosis may occur as a result of rheumatic fever

in a case of ASD(Lutembacher syndrome).
COMPLICATIONSOFASD
 Right sided heart failure
 Frequent pulmonary infections
 Flow-related PAH
 Pulmonary vascular obstructive disease
 Paradoxical embolism
 Tricuspid and mitral insufficiency
 Atrial arrhythmias—atrial flutter, atrial fibrillation,
and Sick Sinus Syndrome.
 MANAGEMENT

 Patients with small shunts and normal RVsize are generally

asymptomatic and require no therapy but need longtime follow up for
spontaneous closure.
 Moderate to large shunt and/or symptomatic ASD should be managed
with following strategies:
- Medical therapy
- Interventional therapy
- Surgical therapy
 MEDICAL MANAGEMENT

 Aim to reduce volume overload and to strengthen

functions of heart muscles.
 Symptomatic children :
 Diuretics:
-These agents relieve ventricular overload, peripheral
and pulmonary congestion
 Digoxin:
-Helps to strengthen the heart muscle, enabling it to pump more
efficiently
 Afterload reducers:
- Enalapril

- Captopril
 Exercise restriction is no necessary
 Prophylaxis for infective endocarditis is not indicated
Atrial arrythmias : Appropriate Antiarrhythmic drugs.
Atrial fibrillation : Antiarrhythmic drugs + anticoagulants.
Irreversible PAH :dobutamine, calcium channel blockers (high
dose), diuretics, prostacycline, sildenafil or oxygen therapy.

Treatment of Other complications, like- pulmonary infections,

thrombo- embolic events or heart failure should also be treated
accordingly.
INTERVENTIONAL
THERAPY
 Closure of ASD :
In patients with small secundum ASDs and
minimal left-to-right shunts without right
ventricular enlargement, closure is not required
Indications of ASDclosure-
 All symptomatic patients
 Asymptomatic patients with-
• Qp : Qs ratio of at least 2 : 1
• Right ventricular enlargement
Time of closure- usually after the 1st yr and
before entry into school
INTERVENTIONALTHERAPY
Indication:
i. Echocardiographic evidence of ostium secundum ASD
ii. Clinical evidence of RV volume load ( i.e. 1.5:1 degree
of left to right shunt or RV enlargement)
iii. ASD diameter less than 36 mm
iv. Presence of sufficient rim of tissue( at least 5 mm)
v. Patient with fenestrated Fontan lateral tunnel if
temporary balloon occlusion is tolerated
CONTRAINDICATION:

Sinus venosus, coronary sinus or primum ASD

Extensive congenital cardiac anomaly.
Known sepsis within one month prior to implantation or any
untreated systemic infection prior to device placement.
Bleeding disorder, untreated ulcer or any other
contraindications to aspirin therapy.
Demonstrated intracardiac thrombi on echo.
Any patient whose size or condition would cause to be a poor
candidate for cardiac catheterization.
DIFFERENT ASDCLOSURE DEVICES:

 Clamshell(TM) device
 Buttoned device
 Angel wings(TM) device
 Atrial septal defect occluder system device
Advantages of device closure-
 Disadvantages of
It is safe and cost-effective than surgery device closure-
Successful implantation rates more Higher rate of small
residual leak
than 96%,

Fewer complications: Major<1%,

 Shortened hospitalization
Avoidance of pain and residual
thoracotomy scars

 Reduced need for blood products.

COMPLICATIONS OF DEVICE
CLOSURE:
Device misalignment/embolization
Device erosion of atrial wall or aorta
Device impingement on adjacent structures AV valve,
Coronary sinus, SVC, Pulmonary veins,Aorta
Infection including endocarditis
Thromboembolic Complication
Allergic reaction
Valvular regurgitation
Residual shunt
Follow– Up After Device Closure:

 Clinical - assessment of symptoms of arrhythmia,

chest pain, or embolic events.
 Echocardiography surveillance - device position, residual
shunting, and complications such as thrombus formation
or pericardial effusion.
 Frequency of follow-up echocardiography - usually at
24 hours, 1 month, 6 months, and 1 year and at
regular intervals thereafter.
SURGICAL
MANAGEMENT
 SURGICAL MANAGEMENT

 Surgical closure has been the “gold standard” form

of treatment of ASD
 Surgeons need proper training and expertise in
performing operations.
 The surgical approach can be by right thoracotomy or
sternotomy, and more limited incisions are feasible
with either approach.
Procedure- Simple suture or
patch closure

Timing-
Surgery is usually delayed
until the patient is 2 to 4 years
of age because the possibility
of spontaneous closure exists.
In infancy – If CCF not
respond t0 medical
management
 Indication:
ASD with RA and RV enlargement with / without
symptoms.
ASD minimum diameter > 10 mm on echocardiography
A sinus venosus, coronary sinus or primum ASD
Chronic atrial arrythmia with ASD (concomitantMaze
 procedure)
 Contraindication:
 Patients with severe irreversible PAH& reverseshunt
 SPO2 < 90%

Advantages of Surgery-
Can be performed in any type
of ASD
Associated anatomical
abnormality can be corrected
concurrently.
Excellent late outcome.
DISADVANTAGES
OF SURGERY-
Costly
Needs expertise hands
Prolong Hospital stay
pain and residual
thoracotomy scars
COMPLICATIONS:
●Pericardial effusion / constriction
●Residual shunt
●RV systolic and diastolic dysfunction
●Pulmonary artery pressure
●Mitral regurgitation
●Pulmonary vein stenosis or caval vein stenosis (sinus venosus
defects)
●Arrhythmia
●Tricuspid regurgitation
 FOLLOW –UPAFTER SURGICAL
CLOSURE:
Early postoperative follow-up:
-Symptoms of undue fever, fatigue, vomiting, chest pain, or abdominal pain
(may represent post pericardiotomy syndrome with tamponade and needs
immediate evaluation with echocardiography.)
Annual clinical F/U: (if following conditions persist or develop)
- PAH.
- Atrial arrhythmias.
- RV or LVdysfunction.
- Coexisting valvular or other cardiac lesion
PROGNOSIS:
Patients generally survive up to adulthood without
surgical or percutaneous intervention mainly with small
to moderate size ASD and many patients live to advanced
age.
The results after surgical or device closure in children
with moderate to large shunts are excellent.
Mortality is less than 2% after surgical closure of
uncomplicated ASD
Mortality and morbidity increase with pulmonary
vascular disease
TAKE –HOME MESSAGES
Atrial septal defects are relatively common CHD
Early symptoms are usually rare except very large deffect.
Any kind of closure is safe and effective and associated with
improved life expectancy
A comprehensive treatment plan should include input from
the primary care provider, the Paediatric Cardiologist and the
Paediatric Cardiovascular surgeon.
THANK YOU