CTICasesPulmonary Capillary Hemangiomatosis (PCH)
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Imaging findings
Radiograph shows generous pulmonary arteries. CT demonstrates enlarged right heart chambers, a small left atrium, and prominent central pulmonary arteries. Thin sections of the lung reveal centrilobular ground-glass opacities and nodules, diffusely distributed but less profuse in the upper lobes, with minimal septal thickening.
Key takeaways
Pulmonary Capillary Hemangiomatosis (PCH) is a rare cause of post-capillary pulmonary hypertension, often considered a spectrum alongside pulmonary veno-occlusive disease (PVOD) in the Nice classification. Imaging findings, such as centrilobular ground-glass nodules and pulmonary hypertension with a small left atrium, are characteristic. PCH is often associated with a specific genetic mutation, requires lung transplantation, and patients should not be given vasodilators.
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