CTICasesEisenmenger Syndrome (Secundum Atrial Septal Defect) with Pulmonary Arterial Atherosclerosis and In Situ Thrombosis
Imaging findings
CT pulmonary angiography demonstrates massive dilation of the main, right, and left pulmonary arteries with extensive mural calcification. There is a large secundum atrial septal defect with contrast swirling from right to left, right atrial enlargement, and right ventricular hypertrophy. The lungs show peripheral ground-glass opacities, mosaic attenuation, and focal areas of consolidation consistent with organizing pulmonary infarcts and in situ thrombosis in the dilated, low-flow vessels.
Key takeaways
Long-standing left-to-right shunts, such as a large secundum atrial septal defect, can lead to severe pulmonary hypertension and the development of Eisenmenger syndrome. The massive volume and pressure overload cause progressive pulmonary arterial atherosclerosis, characterized by arterial dilation and extensive wall calcification. As flow dynamics decrease and vascular remodeling worsens, these patients are highly predisposed to in situ thrombosis and subsequent pulmonary infarction.
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