CTICasesEisenmenger Syndrome with Giant Pulmonary Artery Aneurysm Secondary to Secundum Atrial Septal Defect
[...]. Eisenmenger Syndrome with Giant Pulmonary Artery Aneurysm Secondary to Secundum Atrial Septal Defect. CTICases. Published July 20, 2017. https://cticases.com/c/jhyaa
Imaging findings
Chest radiograph reveals massive cardiomegaly and giant enlargement of the main and central pulmonary arteries with prominent peripheral tapering and curvilinear calcification. CT shows a massive secundum-type atrial septal defect, severe main pulmonary artery dilation measuring nearly seven centimeters, extensive mural calcification (indicative of pulmonary atherosclerosis), and a small-caliber aorta due to chronically reduced systemic forward cardiac output.
Key takeaways
This case represents a classic imaging presentation of Eisenmenger syndrome resulting from a long-standing, unrepaired secundum atrial septal defect. Chronic left-to-right shunting leads to severe, irreversible pulmonary vascular disease and subsequent right-to-left shunting (Eisenmenger physiology). Giant pulmonary artery aneurysms in this setting frequently exhibit heavy atherosclerosis and in-situ mural thrombus, and can carry risks of dissection, rupture, or mechanical compression of adjacent structures such as the left main coronary artery.
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