First Successful Lung Transplantation for Sickle Cell Disease with Severe Pulmonary Arterial Hypertension and Pulmonary Veno‐Occlusive Disease. (1st December 2013)

• Record Type: Journal Article
• Authors: George, M. Patricia; Novelli, Enrico M.; Shigemura, Norihisa; Simon, Marc A.; Feingold, Brian; Krishnamurti, Lakshmanan; Morrell, Matthew R.; Gries, Cynthia G.; Haider, Syed; Johnson, Bruce A.; Crespo, Maria M.; Bhama, Jay K.; Bermudez, Christian; Yousem, Samuel A.; Toyoda, Yoshiya; Champion, Hunter C.; Pilewski, Joseph M.; Gladwin, Mark T.
• Is Part Of: Pulmonary circulation. Volume 3:Number 4(2013)
• Page Start: 952
• Page End: 958
• Publication Date: 2013-12-01
• Subjects: lung transplantation -- sickle cell disease -- pulmonary arterial hypertension -- pulmonary veno‐occlusive disease; Pulmonary circulation -- Periodicals; Pulmonary circulation; Electronic journals -- Sciences; Periodicals; 616.24005
• DOI: 10.1086/674749
• Languages: English
• ISSNs: 2045-8932

Abstract

Abstract : 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.