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Totally Anomalous Pulmonary Venous Connection and Complex Congenital Heart Disease

Prenatal Echocardiographic Diagnosis and Prognosis

Department of Pediatrics, Division of Pediatric Cardiology (C.R.P., J.R.L.), and Department of Cardiothoracic Surgery (M.L.S., P.C.S.), Children’s Hospital Medical Center of Akron, Akron, Ohio USA; and Department of Obstetrics and Gynecology, Northeastern Ohio Universities College of Medicine, Rootstown, Ohio USA (S.S.C.).

Address correspondence to Chandrakant R. Patel, MBBS, The Heart Center, Children’s Hospital Medical Center of Akron, 2 Perkins Sq, Akron, OH 44308-1062 USA.

Objective. The purpose of this study was to determine the accuracy of prenatal cardiac diagnosis, prognosis, and outcome of totally anomalous pulmonary venous connection (TAPVC) and to determine echocardiographic clues in the prenatal diagnosis of isolated TAPVC or TAPVC in association with other complex congenital heart disease (CHD). Methods. We reviewed our 13-year experience of prenatal diagnosis of TAPVC. Thirteen fetuses were identified with the diagnoses of TAPVC. We systematically analyzed the individual pulmonary veins by color and pulsed Doppler imaging, the presence of a pulmonary venous confluence, the pulsed and color Doppler evaluation of the vertical vein, and sites of connections. Prenatal diagnosis was confirmed by postnatal echocardiography, cardiac catheterization, surgery, or autopsy. Results. The mean gestational age at diagnosis of TAPVC was 26.3 weeks (range, 20–33 weeks). There were 8 fetuses with TAPVC and right isomerism, 3 fetuses with other associated CHD, and 2 with isolated TAPVC. There were 7 fetuses with supracardiac TAPVC, 4 with infracardiac TAPVC, and 2 with mixed TAPVC. Pulmonary vein color and pulsed Doppler data were available in 10 of 13 fetuses. The pulmonary venous confluence was visualized in all fetuses except 1. The vertical vein was visualized in all fetuses. Five fetuses had suspected signs of obstruction. The diagnosis was confirmed postnatally or at autopsy in 12 cases. Eight patients underwent surgery; 6 died, and 2 were alive. Two patients had compassionate care and died; 3 pregnancies were terminated. Conclusions. It is possible to diagnose accurately complex CHD, including the pulmonary venous connections. When diagnosed prenatally, TAPVC carries a poor prognosis.

Key Words: congenital heart diseases • prenatal diagnosis • right isomerism • totally anomalous pulmonary venous connections

Abbreviations: CHD, congenital heart disease • TAPVC, totally anomalous pulmonary venous connection

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