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Updated: Mar 16, 2026

A Patient-Derived Xenograft Model for Venous Malformation
Published on: June 15, 2020
Pulmonary Capillary Hemangiomatosis and Pulmonary Veno-occlusive Disease
Neal F Chaisson1, Mark W Dodson2, Charles Gregory Elliott2
1Respiratory Institute, Cleveland Clinic, Lerner College of Medicine of Case Western Reserve University, 9500 Euclid Avenue, A-90, Cleveland, OH 44195, USA.
Abstract:
This article provides an overview of pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH), two disorders that challenge clinicians, radiologists, and pathologists because they often mimic pulmonary arterial hypertension (PAH). The article reviews the features that differentiate PVOD and PCH from PAH. The article also describes the overlap of PVOD and PCH, highlighted by recent reports of families diagnosed with PVOD or PCH caused by EIF2AK4 mutations. In addition, the article outlines current approaches to the diagnosis and treatment of PVOD and PCH.
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