Pulmonary capillary hemangiomatosis: the role of invasive cardiopulmonary exercise testing

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

Pulmonary Circulation (JSTOR), September 2015, Vol. 5 Issue: Number 3 p580-586,

Abstract: 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
cardiopulmonary 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.