Intravascular Ultrasound for Pulmonary Vein Stenosis Interventions in Congenital Heart Disease

Objective. Pulmonary vein stenosis (PVS) is aggressive, with high morbidity and mortality. Surgical and catheter interventions yield modest success, at best. Refinements in catheter interventions could potentially improve outcomes in this patient population. The goal of this study was to determine the utility of intravascular ultrasound (IVUS) for patients with congenital heart disease and PVS. Methods. Single-center, retrospective review of patients with congenital heart disease and PVS undergoing diagnostic or interventional catheterizations from March 2015 to February 2020. IVUS of the pulmonary veins was performed using an Eagle Eye Platinum IVUS catheter (Volcano Corporation). Results. Five patients underwent 6 procedures (2 diagnostic, 4 interventional). Median age was 1.5 years (range, 0.7-47.5 years) and weight was 8.8 kg (range, 7.3-61 kg). For the interventional procedures, mean pulmonary vein gradient was 8.7 mm Hg with reduction to 1.1 mm Hg (P<.001). Four patients had congenital PVS and 1 patient was post repair of Scimitar syndrome with an obstructed pulmonary venous baffle. Use of IVUS allowed confirmation of stent expansion and apposition, interval vessel growth after initial stenting, and detection of long-segment hypoplasia, unlikely to respond to intervention. There were no thrombotic complications related to IVUS use. Conclusions. IVUS of the pulmonary veins is safe and easy to perform, and provides detailed imaging of PVS to help guide therapy. For those requiring intervention, adequate stent apposition to the pulmonary vein walls, as well as limiting vessel overdilation, may minimize future in-stent stenosis and need for reintervention in this challenging disease.