DuBrock Hilary M, Kradin Richard L, Rodriguez-Lopez Josanna M, Channick Richard N
Division of Pulmonary and Critical Care, Massachusetts General Hospital, Boston, Massachusetts, USA.
Pulm Circ. 2015 Sep;5(3):580-6. doi: 10.1086/682227.
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.
肺毛细血管血管瘤病（PCH）是一种罕见的肺动脉高压（PAH）形式，其特征为肺毛细血管增生以及胶原间隔结构的假性浸润。PCH常伴有静脉闭塞性改变和肺动脉高压性动脉重塑。PCH的临床和病理诊断可能较为隐匿，容易漏诊。大多数报道的PCH病例都与静息性PAH相关。我们报告了3例患者的病例，这些患者最初表现为劳力性呼吸困难，静息肺动脉压正常至轻度升高，且存在明显的肺内分流。在所有3例患者中，有创心肺运动试验提示存在肺血管疾病。由于有创运动试验异常，进行了肺活检；这些活检诊断为PCH，患者被转诊进行肺移植。我们描述了这3例病例的独特特征，包括新的病理发现以及所有3例患者均存在肺内分流，并讨论了心肺运动试验在PCH评估中的作用。
