Pulmonary veno-occlusive disease: A systematic review of risk factors, clinical presentation, diagnostic investigations, treatment outcomes and prognostic factors.

Abstract

Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension. We aimed to systematically evaluate published cases of PVOD to provide an overview of their clinical presentation, management and prognosis to assist early identification and treatment. We conducted a literature search of PubMed and Embase databases for adult cases of 'pulmonary veno-occlusive disease' and 'pulmonary capillary haemangiomatosis'. Data collected included baseline demographics, medical history, clinical presentation, investigations performed, treatment and outcome. Kaplan-Meier survival analysis was used for overall survival with Cox-hazards-regression model used to evaluate treatment outcomes and prognostic factors. A total of 257 cases of PVOD from 113 articles were included in our analysis (mean age 45 ± 17 years, 54% females). Most frequent associations were smoking (28%), systemic sclerosis (10%) and mitomycin exposure (9%). Cardinal CT chest findings included ground glass opacities (75%), interlobular septal thickening (74%) and lymphadenopathy (51%); however, all three features were only seen in 23% (35/151). Median overall survival was 12 months (interquartile range, 3-48 months). Lung transplantation was the only treatment associated with improved survival (P = 0.006). Right ventricular dilatation (P = 0.005), increased mean pulmonary artery pressure (P = 0.01) and reduced 6-minute walk distance (P = 0.04) were associated with poorer overall survival. This systematic review provides a clinically relevant overview of a rare and often fatal condition. There is need for early diagnosis and referral for consideration of lung transplantation, while recognising right ventricular dilatation and elevated pulmonary pressures portend poorer prognosis. PROSPERO international register CRD42024553829.