Echocardiographic features of total anomalous pulmonary venous connection

Seven patients, four months to ten years of age, with total anomalous pulmonary venous connection (TAPVC) were studied by echocardiography. The diagnosis was confirmed by cardiac catheterization, angiography, and surgery in each. The connection was to the left vertical vein in four patients, to the coronary sinus in one, to the inferior vena cava in one, and directly to the right atrium in one. All patients exhibited the echocardiographic criteria of right ventricular diastolic volume overload (RVDVO). In addition, an echo-free space was identified dorsal to the posterior wall of the left atrium. Indocyanine green dye studies done in one of the patients provided evidence that this echo-free space represents the common pulmonary venous chamber. The combination of echocardiographic evidence of RVDVO and demonstration of an echo-free space dorsal to the left atrium is strongly suggestive of TAPVC and should allow an early diagnosis in infants with this anomaly.