Anomalous systemic and pulmonary venous connections in conjunction with atriopulmonary anastomosis (Fontan-Kreutzer). Technical considerations.

Anomalous systemic or pulmonary venous connections can coexist with certain forms of complex cyanotic heart diseases that are reparable only by atriopulmonary anastomotic procedures, thus complicating the intraatrial separation of systemic and pulmonary venous pathways. Anomalous systemic or pulmonary venous connections were encountered isolated or in combination in 17 patients (10%) among a series of 170 modified Fontan-Kreutzer procedures. Fourteen of these 17 patients (82.3%) survived their operations, which utilized different techniques to deal with the various forms of anomalous systemic and pulmonary venous connections. There was one late death (5.8%). Extracardiac exclusion of a left superior vena cava with an end-to-side left cavopulmonary shunt proved to be a more successful alternative than the use of complicated intratrial baffles. Because of the complexity of the anatomic variables, repair of anomalous systemic or pulmonary venous connections in conjunction with a modified Fontan-Kreutzer procedure requires a detailed preoperative anatomical and physiologic diagnosis, and an individualized plan for each patient must be formulated to provide unobstructed venous pathways.