CTICasesPulmonary Capillary Hemangiomatosis
Imaging findings
CT chest shows extensive, bilateral, ill-defined centrilobular ground-glass nodules and bulky mediastinal and hilar lymphadenopathy. Right heart catheterization confirmed severe pulmonary hypertension with normal left heart function and minimal septal thickening.
Key takeaways
Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary hypertension characterized by microvascular proliferation of capillaries within the alveolar walls. Imaging typically shows centrilobular ground-glass nodules and reactive lymphadenopathy. Distinguishing PCH from pulmonary veno-occlusive disease (PVOD) on CT is challenging, and both are indications for lung transplantation.
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