Right heart bypass operation of complex cardiac anomalies in 4 patients with viscero-atrial heterotaxic syndrome

Four patients with viscero-atrial heterotaxic syndrome underwent a right heart bypass operation. 2 patients had right isomerism, and two had left isomerism. 3 patients had common atrio-ventricular canal, three had double outlet right ventricle, one had mitral valve hypoplasia, and one had double inlet right ventricle. All patients had anomalous systemic or pulmonary venous drainage. Redirection of systemic venous drainage was accomplished by total cavo-pulmonary shunt (TCPS) (2 patients), atrial baffle (1 patient), and intraatrial conduit (1 patient). In one patient underwent TCPS, regurgitant common atrioventricular valve was replaced with a prosthetic valve. One who had total anomalous pulmonary venous drainage (type Ib) died after cardiac repair. The three survivors are in improved condition. The right heart bypass operation, like total cavopulmonary shunt or connection, simplifies the redirection of systemic venous drainage and offers improved results in the surgical treatment of complex cardiovascular anomalies with viscero-atrial heterotaxic syndrome.