Reoperations After Repair of Total Anomalous Pulmonary Venous Connection

Abstract

Total anomalous pulmonary venous connection (TAPVC) is a congenital abnormality which results from failure of pulmonary vein “buds” to link into the posterior aspect of the left atrium. Pulmonary venous drainage therefore detours by a variety of different routes, either to the right atrium directly or indirectly, via one of the great systemic veins, superior or inferior vena cava, and thence to the right atrium. The classification proposed by Darling et al. (1957) is possibly the most helpful. This is summarised in Fig. 11.1:

• Fig. 11.1a — Supracardiac (45%). All pulmonary drainage flows to the SVC via a left vertical vein or to the SVC or azygos vein directly.

• Fig. 11.1b — Cardiac (25%). All pulmonary veins enter the coronary sinus by separate openings (20%). Less commonly all pulmonary veins enter the right atrium directly (5%).

• Fig. 11.1c — Infracardiac (25%). All pulmonary drainage flows via a descending common vein to enter the portal vein (65%), or less commonly the hepatic vein, ductus venosus or IVC.

• Fig. 11.1d — Mixed type (5%). The left upper lobe of lung drains to a small-calibre left vertical vein and the remaining left and all right pulmonary veins drain to the coronary sinus. Infrequently various combinations of a, b and c are encountered as other types of mixed TAPVC.

• Fig. 11.1e — Double connection. In addition to this classification “double connection” pulmonary venous drainage is encountered occasionally (Arciprete et al. 1986).