Pulmonary capillary hemangiomatosis: the role of invasive cardiopulmonary exercise testing


Pulmonary capillary hemangiomatosis (PCH) is a rare form of pulmonary arterial hypertension (PAH) characterized by pulmonary capillary proliferation and pseudoinvasion of collagenous septal structures. PCH is often accompanied by veno-occlusive changes and pulmonary hypertensive arterial remodeling. The clinical and pathological diagnosis of PCH can be subtle and easily missed. Most reported cases of PCH have been associated with resting PAH. We report the cases of 3 patients who initially presented with exertional dyspnea with normal to mildly elevated resting pulmonary arterial pressures and marked intrapulmonary shunting. In all 3 patients, invasive cardio-pulmonary exercise testing was suggestive of pulmonary vascular disease. Owing to abnormalities on invasive exercise testing, lung biopsies were performed; these were diagnostic of PCH, and the patients were referred for lung transplantation. We describe unique features of these 3 cases—including novel pathological findings and the presence of intrapulmonary shunting in all 3 patients—and we discuss the role of cardiopulmonary exercise testing in the evaluation of PCH.

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Functional Ability/ Impairment/ Quality of Life/ Physical Activity/ Exercise
Pulmonary Arterial Hypertension


Hilary M. DuBrock, Richard L. Kradin, Josanna M. Rodriguez-Lopez, Richard N. Channick

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Pulmonary Circulation Vol 5: No 3 cover image

September 2015

Pulmonary Circulation Vol 5: No 3

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