Pulmonary Veno-Occlusive Disease: A Comprehensive Review of Diagnostic Challenges, Therapeutic Limitations, and...

Pulmonary veno-occlusive disease (PVOD) is a rare and under-recognized cause of pulmonary hypertension. It is characterized by fibrotic obstruction of small pulmonary veins and venules. Its clinical presentation closely mimics pulmonary arterial hypertension (PAH), leading to frequent misdiagnosis, delayed recognition, and potentially harmful exposure to PAH-specific vasodilator therapy. This review aims to synthesize our evolving understanding of PVOD, discussing its etiologies, role of genetic underpinnings, histopathologic features, pathophysiology, clinical presentation, and characteristic imaging findings. It then discusses management strategies emphasizing early recognition, supportive care, avoidance of inappropriate PAH therapies due to poor response, and timely referral for lung transplantation. Despite advances in identification and management, PVOD remains a fatal condition with a median survival of less than two years, underscoring the importance of early recognition and multidisciplinary care.