title: Recurrence of pulmonary veno-occlusive disease after heart-lung transplantation.
abstract: Pulmonary veno-occlusive disease, a rare cause of pulmonary hypertension, is characterized by extensive and diffuse occlusion of pulmonary veins by fibrous tissue. Although the diagnosis can be suspected by the presence of the classic clinical triad of severe pulmonary arterial hypertension, radiographic evidence of pulmonary hypertension and edema, and normal pulmonary artery occlusion pressure, the definitive diagnosis is histopathologic. The prognosis of pulmonary veno-occlusive disease is poor with most described patients dying within 2 years of diagnosis. Although anti-coagulation, oxygen, and vasodilator therapies are effective temporarily, the definitive treatment is lung transplantation. We describe the recurrence of pulmonary veno-occlusive disease at 3 months after heart-lung transplantation in a 26-year-old man. Recurrence after transplantation for this disease has not been reported previously, and lung transplantation was thought to be definitive treatment. With this 1st report of early recurrence of pulmonary veno-occlusive disease after heart-lung transplantation, we believe that extrapulmonary factors may play a role in the pathogenesis of this rare disease.
authors.affiliation: Pulmonary Institute, Rabin Medical Center, Beilinson Campus, Petach Tikva and Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel.
authors.forename: Gabriel
authors.initials: G
authors.lastname: Izbicki
authors.forename: David
authors.initials: D
authors.lastname: Shitrit
authors.forename: Itzhak
authors.initials: I
authors.lastname: Schechtman
authors.forename: Danielle
authors.initials: D
authors.lastname: Bendayan
authors.forename: Gershon
authors.initials: G
authors.lastname: Fink
authors.forename: Gideon
authors.initials: G
authors.lastname: Sahar
authors.forename: Milton
authors.initials: M
authors.lastname: Saute
authors.forename: Tuvia
authors.initials: T
authors.lastname: Ben-Gal
authors.forename: Mordechai R
authors.initials: MR
authors.lastname: Kramer
mesh_terms: D000328:Adult; D016027:Heart Transplantation; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000601:surgery; D016040:Lung Transplantation; D008297:Male; D011668:Pulmonary Veno-Occlusive Disease / Q000150:complications / Q000601:surgery*; D012008:Recurrence