CTICasesUncorrected Intracardiac Total Anomalous Pulmonary Venous Return with Eisenmenger Physiology
Imaging findings
A woman in her 30s, cyanotic at birth and long labeled asthmatic, has markedly enlarged central pulmonary arteries with pulmonary artery atherosclerotic calcification on radiograph, converging on the hila without discrete lymphadenopathy. CT shows a nearly common atrium, and the right- and left-sided pulmonary veins do not connect to the left atrium but drain into a dilated coronary sinus (type 2, cardiac TAPVR). Coronary CTA during an NSTEMI shows systolic buckling of the left main coronary artery from extrinsic compression by the enlarged pulmonary arteries, and contrast shunting demonstrates right-to-left flow.
Key takeaways
Uncorrected total anomalous pulmonary venous return can rarely present in adulthood; the intracardiac (type 2) form drains into the coronary sinus and may be mistaken for a secundum ASD with a persistent left SVC, with a dilated coronary sinus as a clue. Massively enlarged pulmonary arteries from long-standing pulmonary hypertension predispose to dynamic left main coronary compression and can cause troponin elevation and NSTEMI, and pulmonary artery calcification signals chronic pulmonary hypertension warranting a search for a shunt. Eisenmenger physiology with right-to-left shunting may make the patient a candidate only for heart-lung transplant.
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