Author/Authors :
Güttinger, Eva Division of Pulmonology - University Hospital Zurich and University of Zurich, Zurich, Switzerland , Vrugt, Bart Institute of Surgical Pathology - University Hospital Zurich, Zurich, Switzerland , Speich, Rudolf Division of Pulmonology - University Hospital Zurich and University of Zurich, Zurich, Switzerland , Ulrich, Silvia Division of Pulmonology - University Hospital Zurich and University of Zurich, Zurich, Switzerland , Schwitz, Fabienne Department of Cardiology - University Hospital Zurich, Zurich, Switzerland , Arrigo, Mattia Department of Cardiology - University Hospital Zurich, Zurich, Switzerland , Huber, Lars C. Division of Pulmonology - University Hospital Zurich and University of Zurich, Zurich, Switzerland
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.
