Pediatric Radiology

, Volume 42, Issue 6, pp 647–652

Pulmonary veno-occlusive disease: a misnomer?

Review

DOI: 10.1007/s00247-012-2350-1

Cite this article as:
Miller, C.R. Pediatr Radiol (2012) 42: 647. doi:10.1007/s00247-012-2350-1

Abstract

Pulmonary veno-occlusive disease (PVOD) is a rare entity with non-specific signs and symptoms and is nearly always associated with a dismal prognosis. This review will first consider pulmonary hypertension in general and then will focus on PVOD specifically with particular attention to the pathophysiology of the disease. Classically PVOD is described as a disease primarily involving obstructed venules, with the arterial side of the circulation involved to a lesser degree. This article discusses the demographics of affected individuals; the ways in which an accurate diagnosis can be made, including imaging features; predisposing diseases and associated disorders; and potential treatment.

Keywords

Pulmonary hypertensionVaso-occlusive diseaseChildren

Copyright information

© Springer-Verlag 2012

Authors and Affiliations

  1. 1.Department of RadiologyYale University School of MedicineNew HavenUSA