Open heart surgery immediately after birth following prenatal diagnosis of a large right pulmonary artery to left atrium communication.

摘要

A 24-year-old gravida II para I was referred at 37 + 4 weeks of pregnancy for prenatal ultrasound because of fetal cardiomegaly. The second trimester scanning was reported as normal. High resolution ultrasound (HDI 5000, ATL, Zipf, Austria, 5 to 7 MHz curved array) confirmed massive cardiomegaly with ventricular hypertrophy (apical wall thickness 11 mm; Figure la) and 100?left axis deviation. Mild regurgitation of the mitral and tricuspid valve was present. The left atrium showed an anomalous saccular aneurysm of about 12 mm in diameter. Color Doppler sonography demonstrated continuous turbulent flow in this area, directing toward the left atrium (Figure lb). At atrial level a secundum atrial septal defect with left to right shunting was found. The main pulmonary artery was markedly dilated (21 mm, >95th percentile) and showed regurgitation with oscillating flow (Figure lc). A right pulmonary artery (RPA) to left atrium (LA) fistula was diagnosed based on visualization of a large vessel (11 mm) originating from the pulmonary artery communicating with the aneurysmatic bulge of the left atrium (Figure Id). The pulmonary veins appeared to drain normally into the LA. No other malformations were found. Further Doppler examination revealed absent end-diastolic flow in the umbilical artery and an abnormal flow in the ductus venosus but no other hemodynamic impairment signs. Two days later the situation deteriorated, as evidenced by a reverse flow in the ductus, increasing atrioventricular regurgitation and intermittent premature atrial contractions, so that delivery was planned. In view of the expected poor circulatory condition, an emergency cardiac surgical repair was scheduled in continuity with the cesarean section.