congenital diseases dr. meg-angela christi amores
TRANSCRIPT
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%
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
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-
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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
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
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
Acyanotic Congenital heart lesions
Increased volume load: ASD (atrial septal defect) VSD (ventricular septal defect) AV septal defects PDA (patent ductus arteriosus)
Increased pressure load: valvular pulmonic stenosis valvular aortic stenosis coarctation of the aorta
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
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
Manifestations
small patent ductus does not usually have any symptoms
large PDA will result in heart failure Cardiac enlargement Classic continuous murmur (machinery-
like)
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
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