Outcomes and Risk Factors of Interventions for Pediatric Post-operative Pulmonary Vein Stenosis.
Fujita KT, DiLorenzo MP, Krishnan US, Turner ME, Barry OM, Torres AJ, Bacha EA, Kalfa D, Crystal MA. Pediatr Cardiol. 2023 Dec;44(8):1778-1787. doi: 10.1007/s00246-023-03214-w. Epub 2023 Jul 9. PMID: 37422845
Take Home Points:
- Outcomes for pediatric patients with pulmonary vein stenosis remain guarded with a possible survival advantage if an aggressive treatment strategy is undertaken.
- In patients with recurrent post-operative PVS mortality is associated with complex congenital heart disease, single ventricle heart disease and genetic disorders.
Commentary from Dr. Konstantin Averin (Cohen Children’s Heart Center), catheterization section editor of Pediatric Cardiology Journal Watch:
Pulmonary vein stenosis (PVS) in pediatric patients with congenital heart disease is a complex disease that carries a high degree of morbidity and mortality. Surgery for PVS is often preferred as the baseline intervention for PVS as it may be associated with greater freedom from reintervention and death compared to a baseline trans-catheter approach. Outcomes for treatment of recurrent PVS after baseline surgical repair have not been assessed. The authors sought to evaluate outcomes and identify risk factors associated with patient mortality and vein atresia in post-operative PVS patients in a single center.
From January 2005 to July 2020, 305 patients underwent baseline surgical repair with 46 (15%) requiring reintervention for PVS and comprising the primary study cohort (Figure 1). The median age at baseline surgery of the study cohort was 18.9 days (0-42.8 months) and the time from baseline surgery to the first (index) procedure for recurrence was 4.4 months (0.7-79.5 mon). The total number of procedures varied, but 3 patients required 6 and transcatheter approaches were more commonly chosen for patients who require more interventions. Overall, 11 (23.9%) patients died at a median 1.6 months (1 day-9.7 years) after the index procedure (first procedure for recurrent PVS) with presence of a genetic disorder, single ventricle physiology and complex congenital heart disease being associated with mortality. Half of the cohort developed pulmonary vein atresia of at least one vein and interestingly all patients who had necrotizing enterocolitis developed atresia.
This small, descriptive study continues to add to the literature surrounding treatment and outcomes of pediatric patients with PVS. Many providers hypothesize that aggressive treatment of recurrent PVS with frequent, repeat interventions may confer a survival advantage and while this study found that repeat interventions were associated with improved survival this may represent survival bias as death occurred relatively soon after the index procedure. Some interesting associations were also reported (i.e. necrotizing enterocolitis and male gender with pulmonary vein atresia) but this will need to be explored in future studies with larger patient volumes.
Figure 1.
Figure 2.
