Management of Complex Pulmonary Vein Stenosis at Altitude Combining Comprehensive Percutaneous Interventional Treatment with Sirolimus, Pulmonary Hypertension Medications and Intraluminal Imaging with Optical Coherence Tomography

Pulmonary vein stenosis (PVS) is a growing problem for the pediatric congenital heart population. Sirolimus has previously been shown to improve survival and slow down the progression of in-stent stenosis in patients with PVS. We evaluated patients before and after initiation of sirolimus to evaluate its effects on re-intervention and vessel patency utilizing Optical Coherence Tomography (OCT). We performed a retrospective study, reviewing the charts of patients with PVS, who had been prescribed sirolimus between October 2020 and December 2021. OCT was performed in the pulmonary vein of interest as per our published protocol. Angiographic and OCT imaging was retrospectively reviewed. Statistical analysis was performed using Chi square and Wilcoxon signed-rank test to compare pre-and post-sirolimus data. Ten patients had been started and followed on sirolimus. Median age at sirolimus initiation was 25 months with median weight of 10.6 kg and average follow-up of 1 year. Median total catheterizations were 7 for patients prior to starting sirolimus and 2 after starting treatment (p = 0.014). Comparing pre- and post-sirolimus, patients were catheterized every 3 months vs every 11 months (p = 0.011), median procedure time was 203 min vs 145 min (p = 0.036) and fluoroscopy time, 80 min vs 57.2 min (p = 0.036). 23 veins had severe in-stent tissue ingrowth prior to SST (luminal diameter < 30% of stent diameter). Post-sirolimus, 23 pulmonary veins had moderate to severe in-stent tissue ingrowth that responded to non-compliant balloon inflation only with stent luminal improvement of > 75%. Our study suggests that the addition of sirolimus in patients with moderate-severe PVS helps to decrease disease progression with decrease frequency of interventions. Reaching therapeutic levels for sirolimus is critical and medication interactions and side-effects need careful consideration. OCT continues to be important for evaluation and treatment guidance in this patient population.