Images in cardiovascular medicine. Extracardiac venous heterotaxy syndrome: complete noninvasive diagnosis by multimodality imaging.

A 20-year-old man was referred for the assessment of palpitations and associated dyspnea during exertion. He had no other associated cardiovascular symptoms. Physical examination revealed a healthy male with stable vital signs and a normal cardio respiratory examination. ECG, (Figure 1A), chest x-ray (Figure 1B), and blood counts as well as electrolyte panel were normal. Transthoracic echocardiography revealed a dilated coronary sinus (Figure 2); in addition, a persistent left superior vena cava was noted (Figure 3). A small coronary-pulmonary arterial fistula was noted (Figure 4). Two-dimensional subcostal echocardiography view demonstrated absence of the hepatic portion of the inferior vena cava with the hepatic veins draining directly into the right atrium (interrupted inferior vena cava). The right suprasternal view revealed venous flow into the right superior vena cava from a dilated azygous vein (Figure 5). Left and right ventricles were normal in size and function.