Introduction
The surgical outcomes are unsatisfactory for patients having single-ventricle with total anomalous pulmonary venous connection (TAPVC) and heterotaxy syndrome. A major concern is that the anatomical feature of extracardiac TAPVC in right atrial isomerism (RAI) frequently causes pulmonary venous obstruction (PVO) after surgery (1-3). The reported rates of hospital mortality and postoperative PVO are 43%-53% (1,2) and 42% (1), respectively. Thus, both mixed-type TAPVC and preoperative PVO have been reported as risk factors for PVO after TAPVC repair (1-3). Alterations in anatomical factors resulting from surgery may also affect the incidence of PVO. Therefore, the identification of such factors may help modify the surgical strategy and methods.
The purpose of this study was to evaluate the surgical outcomes of TAPVC repair and determine anatomical risk factors for postoperative PVO in neonates and infants with RAI, single ventricle, and extracardiac TAPVC. We hypothesized that 1) the anatomical relationship between the arch and apex and 2) the apex rotation into the thoracic cavity after surgery are both related to postoperative PVO, as they both indicate a narrowing of the posterior space from the caudal side of the pulmonary artery (PA) to the lower end of the lower pulmonary vein (PV) and the space between the left and right phrenic nerves.