Abstract 2962: A Population-based Study Of Total Anomalous Pulmonary Venous Drainage And The Impact Of Pulmonary Venous Obstruction.
Introduction: Despite improvement in early mortality for total anomalous pulmonary venous connection (TAPVC), late mortality is frequently associated with pulmonary venous obstruction (PVO). We aimed to describe the morphological spectrum of TAPVC and identify risk factors for postoperative PVO and death in a population-based multicenter international study.
Hypothesis: Outcome and postoperative PVO following surgery for TAPVC is influenced by preoperative factors.
Methods: Retrospective international collaborative population-based study involving 19 pediatric cardiac centers in UK, Ireland and Sweden. Cases of TAPVC born between 1/1/1998 and 31/12/2004 were identified. Cases with functionally univentricular circulations or atrial isomerism were excluded. All available data and imaging were reviewed.
Results: Four hundred and twenty-two cases were identified. Sixty (14.2%) had an associated cardiac anomaly, 26 of which required intervention. Two hundred and five (48.6%) had supracardiac, 111 (26.3%) infracardiac, 66 (15.6%) cardiac, and 37 (8.8%) mixed connection. There were two cases (0.5%) of common pulmonary vein atresia. In one (0.2%) connection was unknown. Invariably preoperative investigation showed no evidence of discrete stenosis of the individual pulmonary veins, however some patients had extremely hypoplastic veins on imaging, surgical or postmortem findings. Sixteen died before intervention to repair TAPVC. Three year survival for all surgically treated patients was 85.8% (95% CI 82% - 88.9%). Multivariate risk factors for death comprised of: earlier age at surgery, preoperative mechanical support, hypoplastic pulmonary veins and confluence, associated cardiac lesions requiring staged surgery and postoperative PVO. Sixty (14.8%) of the 406 patients undergoing TAPVC repair had postoperative PVO requiring reintervention. Three year survival for patients with postoperative PVO was 58.1% (95% CI 45.8% - 68.5%). Multivariate risk factors for postoperative PVO comprised of hypoplastic pulmonary veins and confluence, emergency surgery and absence of a single confluence.
Conclusions: Preoperative clinical and morphologic features are important risk factors for postoperative PVO and outcome.