120 - Surgical Considerations in Pulmonary Vein Anomalies

Abstract

Pulmonary vein development is now known to be involved in pathogenetic processes ranging from the onset of atrial arrhythmias to congenital malformations of the pulmonary venous system and pulmonary vein stenosis, both congenital and acquired. Anomalous pulmonary venous drainage can result from two processes: (1) failure of the left atrial outpouching and pulmonary venous plexus to fuse, or (2) malposition of the relationship between the atrial outpouching and the development of the atrial septum. If all the pulmonary veins have an anomalous connection to a single systemic vein, the lesion is called total anomalous pulmonary venous drainage (TAPVD). If all the pulmonary veins drain anomalously to multiple different systemic veins, the lesion is called mixed total anomalous pulmonary venous drainage. In partial anomalous pulmonary venous drainage (PAPVD), one to three pulmonary veins drain via an anomalous pathway to the systemic venous circulation and at least one pulmonary vein drains to the left atrium. The presence and degree of obstruction to anomalously draining pulmonary veins largely defines patient presentation and timing for surgical repair. Many imaging tools exist for patients with pulmonary vein anomalies, including echocardiography, computed tomography, lung perfusion scans, magnetic resonance imaging (MRI), and angiography. The primary goals of surgical repair of both PAPVD and TAPVD are to (1) redirect blood from the anomalous pulmonary veins to the left atrium, and (2) avoid obstruction to both pulmonary blood flow and systemic venous return. Surgical and medical innovation has continued since repairs first began in the 1950s. With the ongoing challenges of pulmonary vein stenosis (both congenital and acquired), continued advances in catheter-based therapies, surgical strategies, and adjuvant chemotherapy remain critical.