Incidence and Risk Factors of Pulmonary Vein Stenosis After Orthotopic Heart Transplantation in Pediatric Patients.

Abstract

Pulmonary vein stenosis (PVS) is a rare yet critical condition in children with a poor prognosis. The incidence and underlying risk factors of PVS following orthotopic heart transplantation (OHT) remain unclear. This study aimed to investigate the occurrence and predictors of PVS development post-OHT among pediatric recipients. This retrospective, single-center study was conducted at Cincinnati Children's Hospital Medical Center included patients who underwent OHT between 2014 and 2023. Demographics were compared between patients who developed PVS after OHT and those who did not. Cox regression analyses was used to identify predictors of PVS development. Among 114 patients (70 males, 61%) who underwent OHT at a median age of 11 years (IQR: 2-15), 12 were diagnosed with PVS (6 males, 50%) during a median follow-up of 29 months (IQR: 14-63). The median interval between OHT and diagnosis of PVS was 2 months (IQR: 1-8). All 12 PVS cases were associated with underlying congenital heart disease (12/69, 17.4%) including 2 patients with history of PVS at time of transplant. No patients with history of cardiomyopathy or myocarditis developed PVS. Among the 12 PVS patients, 3 (25%) had multivessel pulmonary vein disease and 4 patients (33%) died. Cox regression analysis, after excluding 2 patients with a prior history of PVS, identified age at OHT (Hazard ratio 0.86, p = 0.027) and partial or total anomalous pulmonary venous return (Hazard ratio 9.0, p = 0.002) as significant predictors of PVS development post-OHT. Prematurity, chronic lung disease, single ventricle physiology and donor-recipient size mismatch were not significantly associated with development of PVS post-OHT. The patients with PVS had significantly higher mortality compared to those without PVS (p = 0.025). This study highlights a low risk of PVS post-OHT in children with cardiomyopathy or myocarditis. PVS post-transplant is linked to congenital heart disease and early post-transplant period. Monitoring for PVS during the first year post-OHT is crucial, especially in children with congenital heart disease.