Dr.

IKA PRASETYA W, SpPD

Divisi Kardiologi
Departemen Ilmu Penyakit Dalam FKUI/RSCM
Jakarta
 Populasi usia dewasa yang menderita Penyakit Jantung
Kongenital semakin meningkat
 Sebagian besar telah menjadi prosedur tindakan operasi
untuk memperbaiki kerusakan yang terjadi
 Kemajuan di bidang surgikal dan pengobatan yang
sangat pesat beberapa tahun belakangan ini -- hampir
± 20.000 pasien penyakit jantung kongenital setiap
tahunnya akan menjadi dewasa
 ATRIAL SEPTAL DEFECT
 VENTRICULAR SEPTAL DEFECT
 PATENT DUCTUS ARTERIOSUS
 TETRALOGY OF FALLOT
 Kelainan jantung bawaan yang paling sering
didiagnosis pada saat usia dewasa (± 30%)
 Tipe-tipe :
 ASD pada ostium sekundum (75%)
 ASD pada ostium primum (15%)
 Defek pada Sinus Venosus (± 10%)
 Most infants with ASDs are asymptomatic
 Dyspnea on exertion
 Fatigue
 Recurrent lower respiratory tract infections
 In adults : decreased stamina and palpitaions due
to atrial tachyarrhythmias (right atrial
enlargement)
 Prominent systolic impulse along the lower left
sternal border : contraction of the dilated RV (RV
heaving)
 The 2nd heart sound : widened, fixed splitting pattern
 Systolic murmur at the upper-left sternal border : the
increased volume of blood flowing across the
pulmonary valve
 Mid-diastolic murmur at the lower-left sternal border
: the increased flow across the tricuspid valve
 ELECTROCARDIOGRAM :
 Right Ventricular Hypertrophy
 Right Atrial Enlargement
 Incomplete or Complete Right Bundle Branch Block
 Ostium Primum ASD : Left Axis Deviation

 CHEST X-RAY :
 Heart enlargement : right atrial and right ventricular
dilatation
 Pulmonary artery is prominent with increased pulmonary
vascular markings
 ECHOCARDIOGRAPHY :
 Right Atrial and Right Ventricle Enlargement
 Paradoxical Septal motion consistent with volume
overload of the RV
 ASD may be visualized directly : transatrial shunt by
Doppler flow interrogation
 CARDIAC CATHETERIZATION :
 Younger adults : fixed pulmonary vascular obstruction
is suspected
 Elective Surgical Repair
 The defect is repaired by direct suture closure or
with pericardial or synthetic patch
 Percutaneous ASD repair, using a closure device
deployed via an intravenous catheter : less
invasive
 ASD should be suspected in all adults who
present with effort intolerance, dyspnea,
palpitations, especially those with right-sided
heart enlargement
 Other causes of right-sided heart enlargement :
 Primary Tricuspid or Pulmonic Valve Disease
 Arrhythmogenic Right Ventricular Dysplasia
 Primary Pulmonary Hypertension
 Other Intracardiac and Extracardiac shunts
 The mortality rate has been estimated at 6% per year
after 40 years of age
 In general, young women with ASD who become
pregnant successfully deliver healthy infants without
maternal of fetal complications, provided that
pulmonary hypertension is not present
 Arrhythmias :
 Supraventricular arrhythmias
 Atrial Fibrillation
 Moderate Pulmonary Hypertension
 Severe Pulmonary Hypertension
 Development of pulmonary hypertension may occur
more frequently in patients with sinus venosus ASD
 In older patients, with significant unrepaired ASD
(left to right shunt, blood flow ratio at least 1,5:1),
right sided heart failure may develop with peripheral
edema and ascites
 Endocarditis : more common in patients with primum
ASD and cleft mitral valve
 VSD is an abnormal opening in the
interventricular septum
 Relatively common, incidence of 1,5 to 3,5 per
1000 live births
 Most often located :
 In the membranous (70%)
 In the muscular (20%)
portions of the septum. Rare VSDs occurs just below
the aortic valve or adjacent to the AV valves
 Small VSDs typically remain symptoms free
 10% of infants with VSDs have large defects and
will develop early symptoms of congestive heart
failure
 VSD complicated by pulmonary vascular disease
and reversed shunts may present with dyspnea
and cyanosis
 Bacterial Endocarditis : regardless of the size of
the VSD
 Harsh holosystolic murmur : best heard at the left sternal
border
 Smaller defects tend to have the loudest murmur because of
the great turbulence of flow
 Systolic thrill commonly be palpated in the region of the
murmur
 Mid-diastolic rumble : at the apex owing to the increased flow
across the mitral valve
 Holosystolic murmur diminishes as the pressure gradient
across the defect decreases if pulmonary vascular disease
develops
 Such patients : RV heave, a loud pulmonic closure sound and
cyanosis
 CHEST X-RAY :
 Small defects : cardiac silhouette may be normal
 Large shunts : cardiomegaly, prominent vascular markings
 Pulmonary vascular disease : enlarged pulmonary arteries

 ELECTROCARDIOGRAM :
 Left Atrial Enlargement
 Left Ventricular Hypertrophy
 Right Ventricular Hypertrophy
 ECHOCARDIOGRAPHY :
 Doppler studies : determination the location of the
VSD
 Identify the direction and magnitude of the shunt

 CARDIAC CATHETERIZATION :
 Increased oxygen saturation in the RV compared with
the right atrium
 Age 2 : ± 50% of small and moderate sized VSDs
undergo sufficient partial or complete
spontaneous closure
 Surgical correction of the defect is recommended
in the first few months of life for children with
congestive heart failure or pulmonary vascular
disease
 Endocarditis prophylaxis for all patients with
VSDs
 The Ductus Arteriosus is the vessel that connects the
Left Pulmonary Artery to the Descending Aorta
during fetal life
 PDA results when the ductus fails to close after birth
: persistent connection between the great vessels
 Risk Factors for its presence :
 first trimester maternal rubella infection
 Prematurity
 Birth at a high altitude
 Small PDAs are generally asymptomatic
 Large left to right shunts :
 Early congestive heart failure
 Poor feeding
 Slow growth
 Recurrent lower respiratory tract infection
 Moderate size lesions :
 Fatigue
 Dyspnea
 Palpitations
 Atrial Fibrillation : left atrial dilatation
 Turbulent blood flow across the defect : endovascular
infection ~ endocarditis/endarteritis
 CHEST X-RAY :
 Left atrial and left ventricular enlargement
 Calcification of the ductus
 ELECTROCARDIOGRAM :
 LA enlargement and LV Hypertrophy when the large shunt is
present
 ECHOCARDIOGRAPHY :
 Visualize the defect, demonstrate flow through it and estimate
right sided systolic pressures
 CARDIAC CATHETERIZATION :
 Step up in oxygen saturation in the pulmonary artery
compared with the RV
 Angiography shows : abnormal flow of blood through the PDA
 PDA should be therapeutically occluded (in the
absence of other congenital cardiac abnormalities
or severe pulmonary vascular disease)
 Results from a single developmental defect : an
abnormal anterior and cephalad displacement of the
infundibular (outflow tract) portion of the
interventricular septum
 4 anomalies :

1. VSD
2. Sub-valvular pulmonic stenosis
3. Overriding aorta
4. Right Ventricular Hypertrophy
 In rare cases : an adult presents with unrepaired and
unpalliated TOF
 Typically have little right ventricular outflow tract
obstruction in childhood
 Symptoms of volume overload : dyspnea, exercise
intolerance, acyanotic (Pink Tetralogy)
 Adults with uncorrected TOF: severe exercise
intolerance and cyanosis, endocarditis, stroke,
supraventricular arryhtmias, brain abscess, LV failure
from progressive AR
 Peripheral cyanosis
 Clubbing finger and toes
 Elevated of jugular venous pressure
 RV heave is palpated
 2nd heart sound is loud and single (no pulmonic
component)
 Harsh systolic murmur of pulmonic obstruction
 ELECTROCARDIOGRAM :
 Right Axis Deviation
 Right Ventricular Hypertrophy
 RBBB (repaired TOF)
 Prominent R wave in leads V5-V6 (palliative shunts)
 CHEST X-RAY :
 Normal heart size with RV Hypertrophy in the lateral view
 Pulmonary Vessels are diminished (unrepaired TOF)
 ECHOCARDIOGRAPHY
 CARDIAC CATHETERIZATION
 ECHOCARDIOGRAPHY :
 Unrepaired TOF :
 Level and severity of RVOT obstruction
 Location and degree of shunting across the VSD
 Corrected TOF :
 Evaluate for RVOT obstruction
 Right Ventricular patch aneurysm
 Pulmonary insufficiency
 VSD patch leak, aortic dilatation and AR
 CARDIAC CATHETERIZATION : for the patients are
being considered for reoperation. Estimation of pulmonary
artery size and vascular resistance, ventriculography or
aortography
 Many congenital cardiac anomalies other than TOF can
cause cyanosis and systolic murmur in adults
 These anomalies :
1. Severe pulmonic stenosis
2. Pulmonic atresia
3. Ebstein’s anomaly
4. Uncorrected AV canal
5. Single ventricle states
6. Congenitally corrected transposition of the great arteries
7. Double chamber RV
8. Eisenmenger’s syndrome (VSD and PDA)
 Uncorrected TOF :
 RV strain
 Chronic cyanosis
 Erythrocytosis
 RV failure (4th and 5th decades of life in the rare adult
who survive)
 Endocarditis with septic embolization
 Corrected TOF :
1. Related to residua and sequelae of the surgery
2. Arrhythmias are the most common and troubling
problem after TOF repair
 Surgical Intracardiac Repair is recommended for
all adults (previously unrepaired TOF or
palliative shunting without repair of TOF) even
those older than 40 years