Treatment and prognosis of pulmonary veno-occlusive disease in adults
- Jess Mandel, MD
Jess Mandel, MD
- Section Editor — Pulmonary Vascular Disease
- Professor of Medicine
- University of California, San Diego
- Barbara LeVarge, MD
Barbara LeVarge, MD
- Instructor in Medicine
- Harvard Medical School
Pulmonary veno-occlusive disease (PVOD) is a rare condition that represents a subgroup of patients with pulmonary hypertension (table 1). In general, PVOD progresses rapidly such that early recognition and treatment of this entity is critical.
The approach to treating patients with PVOD varies from center to center. Our approach is reviewed in this topic. Issues related to the epidemiology, pathogenesis, clinical features, and diagnosis of PVOD as well as the diagnosis and management of pulmonary arterial hypertension are discussed separately. (See "Epidemiology, pathogenesis, clinical evaluation, and diagnosis of pulmonary veno-occlusive disease in adults" and "Classification and prognosis of pulmonary hypertension in adults" and "Clinical features and diagnosis of pulmonary hypertension in adults" and "Treatment of pulmonary hypertension in adults".)
All patients with a clinical or histopathologic diagnosis of pulmonary veno-occlusive disease (PVOD) should be managed in centers with expertise in pulmonary arterial hypertension (PAH). Details regarding diagnosis of PVOD are provided separately. (See "Epidemiology, pathogenesis, clinical evaluation, and diagnosis of pulmonary veno-occlusive disease in adults", section on 'Evaluation and approach to clinical diagnosis'.).
The approach used by most experts is based upon data derived from small case series of patients with PVOD-associated pulmonary hypertension as well as data extrapolated from larger randomized trials performed in patients with PAH. While considerable variation exists among centers, we suggest that the principles of managing patients with PVOD-associated pulmonary hypertension closely parallel that for patients with PAH (algorithm 1 and table 2). However, the clinician should be aware of important differences and potential harms of therapy that can occur in patients with PVOD (algorithm 2):
●General measures – Patients with PVOD should receive routine conventional therapies (eg, oxygen, diuretic, vaccination) but are thought to be at greater risk of alveolar hemorrhage such that, as a general measure, routine anticoagulation is avoided by some experts. (See 'Conventional therapies' below and 'Anticoagulants' below.)
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- GENERAL PRINCIPLES
- GENERAL MEASURES
- Conventional therapies
- ADVANCED THERAPY
- Minimizing the risk
- Assess vasoreactivity
- Vasoreactive patients
- - Calcium channel blockers (CCB)
- Nonvasoreactive or CCB failure
- - WHO functional class I
- - WHO functional class II and III
- Agent selection
- - Endothelin receptor antagonists
- - Phosphodiesterase type 5 inhibitors
- - WHO functional class IV
- Refractory or progressive disease
- - Combination
- LUNG TRANSPLANTATION
- OTHER THERAPIES
- Immunomodulatory medications
- Surgical or mechanical therapies
- EXPERIMENTAL THERAPIES
- SUMMARY AND RECOMMENDATIONS