View clinical trials related to Pulmonary Vein Stenosis.
Filter by:The purpose of this study is to better understand pediatric pulmonary vein stenosis (PVS), which is the narrowing of blood vessels that connect the lungs to the heart. PVS is a life-threatening disease without a clear cause. The investigators think patients who develop PVS have an increased Wall Shear Stress (WSS) level in the pulmonary veins, which is the force placed on the walls of the veins. This study will determine if WSS can be calculated in the pulmonary veins of infants using Ferumoxytol enhanced Cardiac Magnetic Resonance Imaging (FcMRI). If possible, the investigators aim to use FcMRI to better screen patients at risk of PVS and to help guide therapy in patients with PVS.
This is a prospective, single center study which applies a standardized, comprehensive catheterization assessment to patients with a known or suspected diagnosis of pulmonary vein stenosis (PVS) who are undergoing a cardiac catheterization at Boston Children's Hospital. As part of the assessment, each pulmonary vein will undergo angiography (pictures using moving x-rays and contrast dye), intravascular ultrasound (IVUS; pictures of the vein wall using a catheter inside the vein), pressure assessment and compliance testing. The status of each pulmonary vein will then be assessed 12 months after the catheterization (i.e. no disease, severe disease, etc.). Using statistics, the investigators will determine which patient and vein characteristics (obtained at the of catheterization) can predict whether or not a pulmonary vein will have disease. The investigators hypothesize that this comprehensive, standardized, invasive assessment of pediatric intraluminal PVS can predict vein outcome.
This is a prospective, single center, safety and feasibility trial to evaluate the transplantation of the left atrium and pulmonary veins in patients with pulmonary vein stenosis. Consented patients will be listed for transplantation. Once a suitable donor has been identified, the left atrium, pulmonary veins and complete lung block will be harvested from the donor and transported to Boston Children's Hospital as is the procedure for routine lung transplantation patients. The left atrium and pulmonary veins will be transplanted into the recipient. The recipient will receive the normal immunosuppression protocol used for heart transplantation. This pilot study will include 5 patients.
This pilot study evaluates the safety and feasibility of losartan in pediatric patients with pulmonary vein stenosis. It is an open label single arm interventional study. The patient population involves pediatric patients with stenosis in 2 or more pulmonary veins. Patients will be given losartan for 1 year. Outcomes include measures of safety/adverse events and progression of pulmonary vein stenosis.