First successful lung transplantation for sickle cell disease with severe pulmonary arterial hypertension and pulmonary veno-occlusive disease.

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.

• Authors: M Patricia George, Enrico M Novelli, Norihisa Shigemura, Marc A Simon, Brian Feingold, Lakshmanan Krishnamurti, Matthew R Morrell, Cynthia G Gries, Syed Haider, Bruce A Johnson, Maria M Crespo, Jay K Bhama, Christian Bermudez, Samuel A Yousem, Yoshiya Toyoda, Hunter C Champion, Joseph M Pilewski, Mark T Gladwin
• Journal: Pulmonary circulation (Pulm Circ) Vol. 3 Issue 4 Pg. 952-8 (Dec 2013) ISSN: 2045-8932 [Print] United States
• PMID: 25006411 (Publication Type: Case Reports)