Pulmonary Veno-Occlusive Disease: A Rare Cause of Pulmonary Hypertension – A Case Report and Comprehensive Review of the Literature

Pulmonary veno-occlusive disease (PVOD) is a rare and severe form of precapillary pulmonary hypertension, classified in group 1′ of the 2022 ESC/ERS guidelines [1]. It is characterized by progressive fibrotic occlusion of post-capillary pulmonary venules, frequently associated with abnormal capillary proliferation, forming a pathological spectrum with pulmonary capillary hemangiomatosis (PCH) [2,7]. The incidence is estimated at fewer than one case per million inhabitants per year, with a particularly poor prognosis (median survival less than two years without transplantation) [4]. We report the case of a 34-year-old man admitted for acute right heart failure with progressive dyspnea, productive cough, and a 10-kg weight loss. Investigations revealed severe hypoxemia, a marked and isolated reduction in diffusing capacity for carbon monoxide (DLCO, 60% of predicted values), echocardiographic signs of severe pulmonary hypertension, and the classical radiological triad on high-resolution chest computed tomography (centrilobular ground-glass opacities, septal thickening, and mediastinal lymphadenopathy) [11,12]. Dual vasodilator therapy (macitentan 10 mg/day and tadalafil 20 mg/day) was cautiously initiated, allowing clinical stabilization at six months [8]. This case illustrates the diagnostic and therapeutic challenges of PVOD, the importance of early suspicion to avoid iatrogenic worsening with vasodilators, and the need for rapid evaluation for bilateral lung transplantation, the only curative option [9].