Pulmonary Capillary Hemangiomatosis without Pulmonary Hypertension: An Early Stage of Disease?

A 14‐year‐old male visited the First Affiliated Hospital of Guangzhou Medical University with a complaint of a 2‐year history of progressive exertional dyspnea and fingertips cyanosis. Physical examination revealed remarkable desaturation measured by pulse oximetry (80% at rest) and marked cyanosis of lips. The high‐resolution computed tomography scanning revealed no significant abnormality in the lung field [Figure 1a and 1b]. Computed tomography of pulmonary angiography showed no filling defect in trunk or branches of pulmonary arteries, no dilated central pulmonary arteries, and no cardiomegaly [Figure 1c and 1d]. Cardiac catheterization showed a mean right atrial pressure of 6 mmHg, pulmonary artery pressure with mean of 12 mmHg, pulmonary capillary wedge pressure of 9 mmHg, cardiac output of 6.2 L/min, and cardiac index of 4.10 L·min−1·m−2. Pulmonary vascular resistance was slightly elevated at 4.52 wood units. Lung biopsy revealed that lower lobe alveolar spaces were filled with red blood cells or hemosiderin cells, and alveolar septum was wider than normal. Hematoxylin and eosin staining shows that capillary proliferations within alveolar and bronchiolar walls were visible throughout most areas [Figure 2a‐2c]. Immunostaining of lung section was strongly positive for CD34 [Figure 2d], CD31 [Figure 2e], and F8 [Figure 2f]. The clinical manifestations, pathological findings, and immunohistochemistry supported definitive diagnosis of pulmonary capillary hemangiomatosis (PCH) with normal pulmonary artery blood pressure and normal radiological appearance. Because of financial difficulties, the patient abandoned heart–lung transplantation and left hospital without therapeutic intervention. Follow‐up to nearly a years, the patient was stable with no prominent hypoxia on 2 L/min supplemental nasal cannula oxygen at home. Written informed consent was obtained from the parents of patient for publication of this case report and any accompanying images. All patients with PCH usually have elevated pulmonary arterial pressures measured by right heart catheterization with normal or low pulmonary capillary wedge pressure and this have been demonstrated.[1] The infiltration of pulmonary vessel and intrapulmonary shunting caused by uncontrolled proliferation of the pulmonary capillaries might be responsible for the pulmonary hypertension and Pulmonary Capillary Hemangiomatosis without Pulmonary Hypertension: An Early Stage of Disease?