Anatomic variations and corresponding surgical techniques of total anomalous pulmonary venous connection

Abstract

To describe the anatomic variations of total anomalous pulmonary venous connection (TAPVC) and its corresponding surgical techniques. A total of 143 TAPVC subjects were hospitalized from April 1981 to July 2010. Those patients with other complex congenital heart diseases, such as transposition of great artery and single ventricle, were excluded. A pathological diagnosis was made by echocardiography, magnetic resonance imaging, computed tomography, catheterization and intra-operative findings. The specific types of TAPVC were as follows:supra-cardiac (49.7%, 71/143), cardiac (40.6%, 58/143), infra-cardiac (4.2%, 6/143) and mixed (5.6%, 8/143). The subtypes were classified by the pathway of common confluence, distribution of pulmonary vein and their orifice site. The techniques of surgical repairs included modified Warden procedure and pulmonary vein transplantation. The patients with supra-cardiac type were further divided into 4 subtypes according to the course of vertical veins and their orifice site: right and left veins forming a common confluence, then draining into vertical and innominate veins (n = 65); common confluence of pulmonary vein drainage into superior vena cava through a short vertical vein at the right pulmonary hilus (n = 3); right and left pulmonary veins separately draining into superior vena cava (n = 2); common confluence draining into innominate vein through a right path beside trachea (n = 1). Cardiac types were further divided into 3 subtypes: coronary sinus (n = 20), right atrium (n = 37) and right atrium & sinus (n = 1). Infra-cardiac type had no subtype. Mixed type was more complex and it was further divided into 3 subtypes: bilateral & symmetrical connection (right 2 + left 2, n = 5); bilateral & asymmetrical connection (3 + 1, n = 3). Surgical repairs were performed on 135 patients. The surgical mortality of TAPVC was 5.9% (8/135). And there was no late death. The major causes of death were pulmonary infection and low cardiac output syndrome. A detailed classification of TAPVC is of great importance for surgical approaches and methodological designs. And an individualized surgical plan yields excellent patient outcomes.