Repair of Supracardiac Total Anomalous Pulmonary Venous Return

Abstract

Tlle classification of total anomalous pulnlonary re-l_ nous return (TAPVR) is based on the route of blood return fronl the hmgs to tile lleart. In the nlost colnmon type, the supra-cardiac form of TAPVR (TAPVR type I), blood from tllc pulmonary veins joins into a common pulmonary venous confluence posterior to tllc left atriuln.L2 Tim blood then drains superiorly from this confluence through a vertical vein into either the left innominate veiu, tllc superior vcna cava, or the azygous veiu. Tile severity of synlptoms in TAPVR depends pri-nlarily on tile degree of pulnmnary venous ollstruction, a conlplication tllat is less frequent in tile supracardiac type tlmn in tim infracardiac type. In cases witll little or no ollstruction, tile only common symptom is lnild ey-anosis, wllich may go UlmOticcd at birth. But with severe pldmonary venous obstruction, patients can I)resent in extremis with severe cyanosis, respiratory distress, and acidosis witllin tile first few hours of life. Chest radiographs typically sllow a lmrnml heart size with evidence of Imlmonary venous congestion. Tile diagnosis is usually established by two-dimensional echoeardiogral)hy. Cardiac catlleterization is seldom necessary unless required for therapeutic measures, sucll as enlargement of a restrictive atrial septal defect with an atrial septostomy.