First Successful Lung Transplantation for Sickle Cell Disease with Severe Pulmonary Arterial Hypertension and Pulmonary Veno-Occlusive Disease:

摘要

Little is known about the use of lung transplantation in the management of sickle cell disease– associated pulmonary arterial hypertension (SCD-PAH). We present clinical and pathological data and report the first successful outcome of bilateral lung transplantation in a patient with severe SCD-PAH and pulmonary veno-occlusive disease (PVOD). We discuss the complexities of multidisciplinary planning and management of lung transplantation in patients with SCD-associated pulmonary vascular complications. This case reports the first documented successful lung transplant and first case of PVOD in a patient with SCD-PAH.