CTICasesTricuspid Atresia with Coronary Sinusoids
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Imaging findings
This young patient had tricuspid atresia with an intact ventricular septum and a large ASD, resulting in a functional monoventricle. After a BT shunt was taken down and a Glenn shunt placed, the patient developed thrombus along the Glenn shunt extending into the pulmonary artery. Gated CT demonstrated a left coronary vein, LAD, and circumflex, but no visible RCA. Instead, small outpouchings forming vessel-like structures were seen from the excluded right ventricle (RV). Catheterization confirmed that these coronaries, termed coronary sinusoids, arose directly from the RV. Importantly, these sinusoids filled the left circulation, with retrograde flow into the left main coronary artery and aorta.
Key takeaways
Coronary sinusoids are an unusual finding, typically seen in a small percentage of patients with tricuspid atresia and an intact ventricular septum. In this case, the sinusoids originated from the excluded RV and supplied both the right and left coronary systems via retrograde flow. A critical management consideration is avoiding decompression of the RV (e.g., via septostomy), as the high RV pressure is essential to perfuse the coronary circulation through these sinusoids; decompression would lead to coronary steal and death.
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