An Usual Case of Total Anomalous Pulmonary Venous Return in an Adult

The survival of adult patients with total anomalous pulmonary venous return (TAPVR) is rare without surgery. A 38-year-old man was referred to us on emergency basis because of congestive heart failure. Cardiac catheterization and angiogram demonstrated TAPVR (Darling type Ia) with a Qp/Qs of 3.4, Pp/Ps of 0.17 and Rp/Rs of 0.03. Operative procedures consisted of a large anastomosis between the left atrium and the common pulmonary vein using a superior approach, closure of the atrial septal defect, and ligation of the vertical vein. Repeated catheterization three months after surgery showed normal hemodynamics and pulmonary vascular resistance. We consider that the strongest determinant factor affecting long-term survival is normal or only slightly elevated pulmonary vascular resistance, and that the superior approach is useful in adult patients for repair of supracardiac type TAPVR, because it offers an excellent operative field and the risk of postoperative atrial arrhythmia was thought to be minimal.