title: 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.affiliation: Departments of Medicine, Pediatrics, Pathology, and Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
authors.forename: M Patricia
authors.initials: MP
authors.lastname: George
authors.affiliation: Departments of Medicine, Pediatrics, Pathology, and Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
authors.forename: Enrico M
authors.initials: EM
authors.lastname: Novelli
authors.affiliation: Departments of Medicine, Pediatrics, Pathology, and Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
authors.forename: Norihisa
authors.initials: N
authors.lastname: Shigemura
authors.affiliation: Departments of Medicine, Pediatrics, Pathology, and Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
authors.forename: Marc A
authors.initials: MA
authors.lastname: Simon
authors.affiliation: Departments of Medicine, Pediatrics, Pathology, and Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA ; Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania, USA.
authors.forename: Brian
authors.initials: B
authors.lastname: Feingold
authors.affiliation: Departments of Medicine, Pediatrics, Pathology, and Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA ; Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania, USA.
authors.forename: Lakshmanan
authors.initials: L
authors.lastname: Krishnamurti
authors.affiliation: Departments of Medicine, Pediatrics, Pathology, and Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
authors.forename: Matthew R
authors.initials: MR
authors.lastname: Morrell
authors.affiliation: Departments of Medicine, Pediatrics, Pathology, and Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
authors.forename: Cynthia G
authors.initials: CG
authors.lastname: Gries
authors.affiliation: Departments of Medicine, Pediatrics, Pathology, and Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
authors.forename: Syed
authors.initials: S
authors.lastname: Haider
authors.affiliation: Departments of Medicine, Pediatrics, Pathology, and Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
authors.forename: Bruce A
authors.initials: BA
authors.lastname: Johnson
authors.affiliation: Departments of Medicine, Pediatrics, Pathology, and Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
authors.forename: Maria M
authors.initials: MM
authors.lastname: Crespo
authors.affiliation: Departments of Medicine, Pediatrics, Pathology, and Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
authors.forename: Jay K
authors.initials: JK
authors.lastname: Bhama
authors.affiliation: Departments of Medicine, Pediatrics, Pathology, and Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
authors.forename: Christian
authors.initials: C
authors.lastname: Bermudez
authors.affiliation: Departments of Medicine, Pediatrics, Pathology, and Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
authors.forename: Samuel A
authors.initials: SA
authors.lastname: Yousem
authors.affiliation: Temple University School of Medicine, Philadelphia, Pennsylvania, USA.
authors.forename: Yoshiya
authors.initials: Y
authors.lastname: Toyoda
authors.affiliation: Departments of Medicine, Pediatrics, Pathology, and Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
authors.forename: Hunter C
authors.initials: HC
authors.lastname: Champion
authors.affiliation: Departments of Medicine, Pediatrics, Pathology, and Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
authors.forename: Joseph M
authors.initials: JM
authors.lastname: Pilewski
authors.affiliation: Departments of Medicine, Pediatrics, Pathology, and Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
authors.forename: Mark T
authors.initials: MT
authors.lastname: Gladwin
keywords: lung transplantation; pulmonary arterial hypertension; pulmonary veno-occlusive disease; sickle cell disease