[Total abnormal pulmonary venous return. Preoperative stabilization by extracorporeal veno-venous circulation].

Abstract

Background: Total anomalous pulmonary venous return (TAPVR) is a rare congenital cardiac disease. When this includes pulmonary artery hypertension, the infant is severely ill. Survival beyond infancy is rare without surgical correction.

Case report: A girl in whom hydramnios and a short femur were discovered by ultrasonography at the 25th week of gestation was admitted to the intensive care unit a few hours after birth because of respiratory distress. Blood PaO2 was 37 mmHg, PaCO2, 36 mmHg, and pH 7.25. She had tachycardia (190/min) and circulatory insufficiency. Echocardiography showed right ventricular overload, a right-to-left shunt through a patent ductus arteriosus and foramen ovale and tricuspid insufficiency. This refractory hypoxemia was not corrected by conventional respiratory support, high-frequency oscillation plus dobutamine and dopamine followed by noradrenaline infusion. Because of further deterioration, the baby was given extracorporeal lung support; this rapidly improved the respiratory and hemodynamic conditions. Persistent pulmonary artery hypertension led to a second investigation that showed TAPVR She underwent emergency surgery.

Conclusion: Extracorporeal lung support can stabilize a precarious state in a case of severe congenital cardiac disease, so allowing surgical treatment under satisfactory conditions.