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Reactive pulmonary capillary hemangiomatosis and pulmonary veno-occlusive disease in a patient with repaired scimitar syndrome


Güttinger, Eva; Vrugt, Bart; Speich, Rudolf; Ulrich, Silvia; Schwitz, Fabienne; Arrigo, Mattia; Huber, Lars C (2016). Reactive pulmonary capillary hemangiomatosis and pulmonary veno-occlusive disease in a patient with repaired scimitar syndrome. Case Reports in Cardiology, 2016:9384126,.

Abstract

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.

Abstract

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.

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Additional indexing

Item Type:Journal Article, not refereed, further contribution
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Institute of Pathology and Molecular Pathology
04 Faculty of Medicine > University Hospital Zurich > Clinic for Pneumology
04 Faculty of Medicine > University Hospital Zurich > Clinic for Cardiology
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:2016
Deposited On:17 May 2016 08:41
Last Modified:13 Sep 2017 15:54
Publisher:Hindawi Publishing Corporation
ISSN:2090-6404
Free access at:PubMed ID. An embargo period may apply.
Publisher DOI:https://doi.org/10.1155/2016/9384126
PubMed ID:27069695

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Licence: Creative Commons: Attribution 4.0 International (CC BY 4.0)