Recurrence of pulmonary veno-occlusive disease after heart-lung transplantation.
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.
Izbicki
Gabriel
G
Pulmonary Institute, Rabin Medical Center, Beilinson Campus, Petach Tikva and Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel.
Shitrit
David
D
Schechtman
Itzhak
I
Bendayan
Danielle
D
Fink
Gershon
G
Sahar
Gideon
G
Saute
Milton
M
Ben-Gal
Tuvia
T
Kramer
Mordechai R
MR
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