Host Factor Vulnerability and Development of Progressive Intraluminal Pulmonary Vein Stenosis after Congenital Heart Surgery

Objective. The aim of this study is to explore the risk factors for progressive intraluminal pulmonary vein stenosis (PVS) in patients after surgery for congenital heart disease using a case-control design. Design. Using the Children's Hospital Cardiovascular database, all patients who developed progressive intraluminal obstruction of ≥2 pulmonary veins after cardiac surgery between 1989 and 2003 with at least 1 year of follow-up were selected, along with a random sample of control patients undergoing surgery who did not develop PVS. Because initial findings showed heterotaxy syndrome and total anomalous pulmonary venous return (TAPVR) to be strongly associated with PVS, controls were frequency-matched to cases based on these anatomic subtypes. Demographic, neonatal, anatomic, surgical, and postoperative variables were compared for cases vs. controls. Results. Controlling for heterotaxy and TAPVR, younger age at first surgery was identified as being associated with development of PVS (median age 6 days for cases vs. 38 days for controls, P= .01). In subsequent bivariate analyses, adjusting for younger age, blood type B (odds ratio [OR]= 7.6, P= .04) and cardiopulmonary bypass in the first 3 months of life (OR = 3.4, P= .04) were also associated with development of PVS. Other surgical variables indicative of a complex operative course (longer cross-clamp time and total pump time) were more frequent in controls than among cases. Conclusion. Progressive postoperative PVS is associated with anatomic defects, age, and blood type B, suggesting host factor vulnerability. Variables indicative of surgical complexity were not associated with development of progressive intraluminal PVS.