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Left Atrial Stenosis Induced Pulmonary Venous Arterialization and Group 2 Pulmonary Hypertension in Rat
Published on: November 18, 2018
Pulmonary veno-occlusive disease
D Montani1, L C Price, P Dorfmuller
1Centre National de Référence de l'Hypertension Artérielle Pulmonaire, Department of Pneumology and Intensive Care, Hôpital Antoine Béclère, Assistance Publique - Hôpitaux de Paris, Université Paris-Sud, Clamart, France.
The European Respiratory Journal
|January 2, 2009
Summary
Pulmonary veno-occlusive disease (PVOD) is a rare condition often mistaken for pulmonary arterial hypertension (PAH). Early detection using noninvasive methods is crucial due to its poor prognosis and risks with certain PAH therapies.
Area of Science:
- Cardiology
- Pulmonology
- Radiology
Background:
- Pulmonary veno-occlusive disease (PVOD) is a subgroup of pulmonary arterial hypertension (PAH), accounting for 5-10% of idiopathic PAH cases.
- PVOD can be idiopathic or associated with conditions like connective tissue diseases, HIV, and bone marrow transplantation.
- PVOD shares clinical and genetic similarities with PAH but presents distinct physiological characteristics.
Purpose of the Study:
- To differentiate PVOD from PAH.
- To highlight diagnostic approaches for PVOD.
- To discuss treatment implications and prognosis.
Main Methods:
- Comparison of clinical presentation, genetics, and hemodynamics between PVOD and PAH.
- Analysis of high-resolution computed tomography (HRCT) findings suggestive of PVOD.
- Evaluation of noninvasive diagnostic tools including HRCT, arterial blood gases, pulmonary function tests, and bronchoalveolar lavage.
Main Results:
- PVOD exhibits a higher male ratio, increased tobacco exposure, and lower oxygenation compared to PAH.
- HRCT may show centrilobular ground-glass opacities, septal lines, and lymphadenopathy in PVOD.
- Occult alveolar hemorrhage is linked to PVOD, and noninvasive diagnostics can aid detection, potentially avoiding risky biopsies.
Conclusions:
- PVOD has a poor prognosis and can lead to severe pulmonary edema with specific PAH therapies.
- Lung transplantation is the primary treatment for PVOD.
- Cautious use of PAH therapies may benefit select PVOD patients.
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