<abstract xmlns="http://www.w3.org/1999/xhtml"><p>Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary arterial hypertension (PAH). Because of the similar clinical picture of dyspnea on exertion and signs of right heart failure, PVOD is difficult to distinguish from idiopathic PAH. However, the distinction is mandatory because PVOD has a worse prognosis and, more importantly, the administration of PAH specific therapy (vasodilators) can precipitate severe acute pulmonary oedema. We present a challenging case of PAH in a patient with systemic sclerosis in whom a marked decrease in functional capacity after the initiation of bosentan therapy led to the diagnosis of PVOD. Management of PVOD patients is challenging and referral for lung transplantation should be done at the moment of diagnosis.</p></abstract>

Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary arterial hypertension (PAH). Because of the similar clinical picture of dyspnea on exertion and signs of right heart failure, PVOD is difficult to distinguish from idiopathic PAH. However, the distinction is mandatory because PVOD has a worse prognosis and, more importantly, the administration of PAH specific therapy (vasodilators) can precipitate severe acute pulmonary oedema. We present a challenging case of PAH in a patient with systemic sclerosis in whom a marked decrease in functional capacity after the initiation of bosentan therapy led to the diagnosis of PVOD. Management of PVOD patients is challenging and referral for lung transplantation should be done at the moment of diagnosis.

Pulmonary veno-occlusive disease: a rare cause of pulmonary hypertension in systemic sclerosis. Case presentation and review of the literature

Clinical Emergency Hospital Bucharest, Romania

“Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania

“C.C. Iliescu” Emergency Institute for Cardiovascular Diseases, Bucharest, Romania

“Prof. Dr. Marius Nasta” Pneumology Institute, Bucharest, Romania

“Cantacuzino” Clinical Hospital, Bucharest, Romania

Romanian Journal of Internal Medicine

This chapter is distributed under the terms of the Creative Commons Attribution 4.0 Public License.

Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary arterial hypertension (PAH). Because of the similar clinical picture of dyspnea on...

Pulmonary veno-occlusive disease: a rare cause of pulmonary hypertension in systemic sclerosis. Case presentation and review of the literature

Published Online: Oct 14, 2015

Page range: 175 - 183

DOI: https://doi.org/10.1515/rjim-2015-0024

Keywords
Pulmonary arterial hypertension, pulmonary venoocclusive disease, Systemic sclerosis

© 2015 Ana Maria Daraban et al., published by De Gruyter Open

This chapter is distributed under the terms of the Creative Commons Attribution 4.0 Public License.

Pulmonary veno-occlusive disease: a rare cause of pulmonary hypertension in systemic sclerosis. Case presentation and review of the literature

Published Online: Oct 14, 2015

Page range: 175 - 183

DOI: https://doi.org/10.1515/rjim-2015-0024

KeywordsPulmonary arterial hypertension, pulmonary venoocclusive disease, Systemic sclerosis

© 2015 Ana Maria Daraban et al., published by De Gruyter Open

This chapter is distributed under the terms of the Creative Commons Attribution 4.0 Public License.

Keywords
Pulmonary arterial hypertension, pulmonary venoocclusive disease, Systemic sclerosis