pediatrics
Other Congenital Cardiac Anomalies
Pulmonary atresia with intact ventricular septum
Pulmonary atresia with an intact ventricular septum, in which the pulmonary valve is not properly formed, thus impeding blood flow from the heart to the lungs, frequently occurs together with hypoplasia of the tricuspid valve and right ventricle hypoplasia. This association is easily understood based on the fact that normal ventricular growth in fetal life depends on adequate inflow and outflow of that ventricle. Coronary arterial abnormalities, particularly fistulous connections of the coronary arteries to the hypoplastic right ventricle and coronary artery stenoses, are common and have a major impact on prognosis and surgical options; surgery is inevitably required. Immediate postnatal survival is dependent on patency of the ductus arteriosus. Infants present with cyanosis, and auscultatory findings may include a murmur of tricuspid regurgitation or a patent ductus arteriosus. If not previously diagnosed on prenatal ultrasound, postnatal diagnosis is through echocardiography. 