CTICasesPulmonary Capillary Hemangiomatosis (PCH) and Pulmonary Veno-Occlusive Disease (PVOD)
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[...]. Pulmonary Capillary Hemangiomatosis (PCH) and Pulmonary Veno-Occlusive Disease (PVOD). CTICases. Published December 12, 2014. https://str-case-explorer.pages.dev/c/eh866
Imaging findings
CT of a 64-year-old female with severe pulmonary hypertension shows diffuse centrilobular ground-glass nodules with ground-glass halos, interlobular septal thickening, and pleural fissures thickening, mimicking interstitial edema in the absence of left-sided heart disease. Biopsy confirmed a combination of PVOD and PCH.
Key takeaways
Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis are rare, overlapping causes of pulmonary arterial hypertension characterized on CT by centrilobular ground-glass nodules, septal lines, and lymphadenopathy. Vasodilator therapy is contraindicated in these patients as it can precipitate life-threatening pulmonary edema.
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