CTICasesSevere Group 1 Pulmonary Arterial Hypertension
[...]. Severe Group 1 Pulmonary Arterial Hypertension. CTICases. Published August 25, 2017. https://str-case-explorer.pages.dev/c/bfn4e
Imaging findings
Chest CT of a young patient shows a massively dilated main pulmonary artery and marked right ventricular hypertrophy with a wall thickness exceeding that of the left ventricle. The lung parenchyma demonstrates diffuse, bilateral, centrilobular ground-glass nodules and prominent, tortuous, corkscrew-like pulmonary vessels.
Key takeaways
Centrilobular ground-glass nodules in the setting of severe pulmonary hypertension can represent pulmonary capillary hemangiomatosis (PCH), plexiform lesions, or hypervascularity associated with severe group 1 pulmonary arterial hypertension. Distinguishing these entities clinically is critical, as patients with PCH can develop life-threatening pulmonary edema when treated with pulmonary vasodilators.
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