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

PVRI Member Authors: Patricia George, Mark Gladwin


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.

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Pulmonary Arterial Hypertension
Thoracic Surgery and Transplantation


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

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Pulmonary Circulation Vol 3: No 4 cover image

December 2013

Pulmonary Circulation Vol 3: No 4

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