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.
Michael J ShorofskyGareth J MorganErnesto MejiaSalvador Rodriguez FrancoMegan GreenePaige SheaksDunbar IvyJenny E ZablahPublished in: Pediatric cardiology (2023)
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.