2. Congenital Heart Disease
0.5-0.8% of live births
incidence is higher in stillborns (3-4%),
abortuses (10-25%), and premature infants
(about 2%)
diagnosis is established by 1 wk of age in 40-
50% of patients with congenital heart disease
and by 1 mo of age in 50-60%
3. Relative frequency of Major
Congenital lesions
Ventricular septal defect25-30
Atrial septal defect (secundum)6-8
Patent ductus arteriosus6-8
Coarctation of aorta5-7
Tetralogy of Fallot5-7
Pulmonary valve stenosis5-7
Aortic valve stenosis4-7
4. Relative Frequency of Major
Congenital lesions
d-Transposition of great arteries3-5
Hypoplastic left ventricle1-3
Hypoplastic right ventricle1-3
Truncus arteriosus1-2
Total anomalous pulmonary venous return1-2
Tricuspid atresia1-2
Single ventricle1-2
Double-outlet right ventricle1-2 Others5-10
6. Congenital Disease
Most congenital defects are well
tolerated in the fetus because of
the parallel nature of the fetal
circulation
only after birth when the fetal
pathways (ductus arteriosus
and foramen ovale) are closed
that the full hemodynamic
impact of an anatomic
abnormality becomes apparent
7. Etiology
Cause is unknown
There is progress in identifying genetic basis
of many congenital heart lesions
small percentage - related to chromosomal
abnormalities, in particular, trisomy 21, 13, and
18 and Turner syndrome
2-4% -associated with known environmental or
adverse maternal conditions and teratogenic
influences, including maternal diabetes
mellitus, phenylketonuria, or systemic lupus
erythematosus
8. diabetic mothers are five times more likely to
have congenital cardiovascular malformations
most congenital heart disease is still relegated
to a multifactorial inheritance pattern
Fetal echocardiography improves the rate of
detection
9. 2 major groups
1. Acyanotic Congenital heart lesions
2. Cyanotic Congenital heart lesions
11. Cyanotic Congenital heart
lesions
Decreased Pulmonary Blood Flow -
obstruction to pulmonary blood flow and a
pathway by which systemic venous blood can
shunt from right to left and enter the systemic
circulation
tricuspid atresia
Tetralogy of Fallot
single ventricle with pulmonary stenosis
Increased Pulmonary Blood flow
Transposition of the great vessels
Total anomalous pulmonary venous return
Truncus arteriosus
13. Pathophysiology
blood shunts left to right through the ductus
from the aorta to the pulmonary artery
pulmonary artery pressure may be elevated to
systemic levels during both systole and
diastole
risk for the development of pulmonary vascular
disease if left unoperated
14. Manifestations
small patent ductus does not usually have any
symptoms
large PDA will result in heart failure
Cardiac enlargement
Classic continuous murmur (machinery-like)
15. Diagnosis
ECG
Left ventricular hypertrophy
Xray
prominent pulmonary artery with increased
intrapulmonary vascular markings
2D echocardiography
left atrial and left ventricular dimensions are
increased
Visualization of the patent ductus
16. Treatment
Irrespective of age, patients with PDA require
surgical or catheter closure
should not be unduly postponed after
adequate medical therapy for cardiac failure
has been instituted
thoracoscopic techniques to minimize scarring
and reduce postoperative discomfort