The value of two dimensional echocardiography in identifying communications between the-ascending aorta and pulmonary trunk or individual pulmonary arteries was assessed in 24 children, all ofwhom had either angiocardiographic and surgical or angiocardiographic confirmation alone. Fourteen cases had truncus arteriosus, four aortopulmonary window, four anomalous origin of the left pulmonary arte...

Echocardiography was performed in 62 patients--20 with truncus arteriosus, 14 with tetralogy of Fallot, and 28 with pulmonary atresia with ventricular septal defect. Features common in all three groups were: large single systemic arterial trunk overriding the ventricular septum, mitral-semilunar continuity, large right ventricular dimension, and normal septal motion. Indocyanine green contrast ...

Echocardiographic findings are described in a patient with hypoplastic right heart syndrome (pulmonary atresia type with intact ventricular septum and small right ventricular cavity) who had an associated atrial septal aneurysm. An unusual appearance of echoes behind the aorta bulging into the left atrium in diastole on both the M-mode and cross-sectional echo suggested this diagnosis prior to ...

Hemodynamic data on 12 infants with aortic valve atresia, ranging from 1 to 3 mo of age, are presented. Peripheral arterial oxygen saturations ranged from 19 to 93%. The lowest saturations were found in patients with closed foramen ovales. Increasing age, increasing pulmonary vascular resistance, and a decrease in the pulmonary-to-systemic blood flow ratio all correlated with decreasing systemi...

The first stage of a repair of pulmonary atresia with intact ventricular septum (type I) was attempted in a 2-day-old infant. At surgery, decompression of the hypertensive small right ventricle was followed by a sudden loss of myocardial contractility and death. Postmortem examination revealed a fistula with a large orifice in the right ventricular infundibulum that communicated directly with t...

A maternally inherited 828 kb microdeletion of 2q37.3 manifested in a 3-week-old premature boy as left juxtaposition the atrial appendages associated with tricuspid atresia, double outlet infundibulum, subvalvar pulmonary large secundum septal defect, and right aortic arch mirror-image branching, consistent developmental arrest early heart looping. To best our knowledge, no previous 2q37 deleti...

Enlargement of right atrium is usually secondary to pulmonary hypertension due to valvular heart diseases or obstructive pulmonary disorders, atrial septal defect, tricuspid atresia or stenosis, pulmonary stenosis, primary pulmonary hypertension, Ebstein's anomaly. Congenital enlargement of right atrium is rare and it commonly presents in children. Our patient presented with congenital giant ri...

The management of severely hypoplastic pulmonary arteries in the adolescent or adult with congenital heart disease is challenging. A patient with pulmonary atresia and ventricular septal defect (PA-VSD) presented with severe cyanosis due to a kinked and thrombosed right ventricle to pulmonary artery conduit. Retrograde wedge angiography showed a patent right and a diminutive left pulmonary arte...