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Case Reports in Cardiology
Volume 2016 (2016), Article ID 9384126, 5 pages
Case Report

Reactive Pulmonary Capillary Hemangiomatosis and Pulmonary Veno-Occlusive Disease in a Patient with Repaired Scimitar Syndrome

1Division of Pulmonology, University Hospital Zurich and University of Zurich, 8091 Zurich, Switzerland
2Institute of Surgical Pathology, University Hospital Zurich, 8091 Zurich, Switzerland
3Department of Cardiology, University Hospital Zurich, 8091 Zurich, Switzerland

Received 4 December 2015; Revised 27 February 2016; Accepted 6 March 2016

Academic Editor: Tayfun Sahin

Copyright © 2016 Eva Güttinger et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Pulmonary capillary hemangiomatosis (PCH) is a rare histological substrate within the spectrum of pulmonary arterial hypertension that possibly represents an unusual manifestation of pulmonary veno-occlusive disease (PVOD). One of the histological hallmarks of PCH is the proliferation of pulmonary capillaries in the alveolar septa that infiltrate adjacent structures such as bronchioles, vessels, and visceral pleura. The hyperplastic process involving the smallest vessels of the pulmonary vascular bed might reflect uncontrolled angiogenesis, but whether this vascular proliferation is idiopathic or, conversely, a reactive process remains to be elucidated. Here we discuss the pathogenesis of PCH exemplified by the first reported case of a young patient with repaired scimitar syndrome that developed unilateral PCH.