Introduction: Pulmonary vein stenosis (PVS) is a rare condition with limited treatment options and guarded outcomes. We sought to investigate characteristics and outcome of PVS in patients with congenital heart disease (CHD) and prematurity.
Hypothesis: Patient characteristics and survival will be significantly different between premature and CHD populations, with overall poor survival.
Methods: This is a single center retrospective cohort study of patients diagnosed with PVS from 01/06 to 05/16, identified by ICD codes and confirmed by chart review. Survival differences between populations were assessed with Kaplan Meier curves and Cox proportional hazard models.
Results: Ninety-eight patients with PVS were identified: 61 (62%) had significant CHD, 20 (20%) were only premature, 17 (18%) were neither premature nor had significant CHD, and 10 (10%) were premature with significant CHD. Premature infants without CHD were born at 28.3 ± 4.8 weeks weighing 0.9 kg (Interquartile range (IQR) 0.5, 1.4). Fourteen (70%) had chronic lung disease, 15 (75%) developed necrotizing enterocolitis and/or sepsis, and 12 (60%) had a patent ductus arteriosus. Among those with CHD, 32 (52%) had a congenital pulmonary vein anomaly and 32 (52%) had a single ventricle lesion. Age at diagnosis did not differ between the premature and CHD cohorts (200 (IQR 94, 1260) vs. 197 days (IQR 94, 1075), respectively, p=0.9). A median of 2 (IQR 1, 2) pulmonary veins were affected at diagnosis. Sixty-nine (70%) underwent a PVS intervention (balloon angioplasty: 34 (35%), surgical repair: 29 (29%), stent: 6 (6%)), and 45 (46%) underwent ≥2 interventions. Twenty-nine (30%) subjects died or underwent lung transplant 283 days (IQR 105, 528) after diagnosis with a trend towards higher mortality if pulmonary hypertension was present (38% vs 21%, p=0.07). Hazard of death/transplant did not differ between the premature and CHD groups (p=0.2). Overall 1, 3, and 5-year transplant-free survival was 81%, 72%, and 68%, respectively.
Conclusions: In this cohort, PVS developed in infancy and was associated with poor survival. Comorbidities identified may aid future prospective studies to ascertain their role in the causal pathway of PVS.