CTICasesIdiopathic Pulmonary Arterial Hypertension with Progressive Pulmonary Artery Intimal Calcification
Imaging findings
Serial CTs over roughly ten years in a young woman with pulmonary arterial hypertension show a progressively enlarging right heart and main pulmonary artery, with progressive calcification developing along the walls of the pulmonary arteries, analogous to severe atherosclerotic calcification of the aorta, together with ill-defined centrilobular ground-glass nodules, mosaic attenuation, and heterogeneous parenchymal destruction.
Key takeaways
The pulmonary artery wall calcifications represent intimal calcification from chronically elevated systemic-level pressures within the pulmonary arteries rather than chronic thromboembolic disease. This case demonstrates the dramatic long-term vascular and parenchymal progression of severe idiopathic pulmonary arterial hypertension without an identifiable secondary cause, in a patient awaiting heart-lung transplantation.
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