CTICasesChronic Pulmonary Hypertension and Ventricular Septal Defect
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Imaging findings
Chest radiography and CT demonstrate massive enlargement of the main, right, and left pulmonary arteries with extensive intimal atherosclerosis and heavily calcified in situ thrombus. An undiagnosed ventricular septal defect is present. The peripheral pulmonary vasculature demonstrates a characteristic corkscrew, tortuous, and beaded neovascular pattern.
Key takeaways
Chronic, uncorrected left-to-right shunts (such as a ventricular septal defect) lead to severe, progressive pulmonary hypertension. Over time, high pressures cause aneurysmal dilation of the central pulmonary arteries, atherosclerosis, and in situ calcified thrombosis, along with peripheral neovascular remodeling.
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