CTICasesPulmonary Atresia with Intact Ventricular Septum and Right Ventricular Dependent Coronary Circulation
Imaging findings
CT angiogram demonstrates a common atrium, a normal-sized left ventricle, and a hypoplastic right ventricle with a stenotic tricuspid valve. There is pulmonary atresia with no connection between the right ventricular outflow tract and the pulmonary artery. Since the ventricular septum is intact, high right ventricular pressures drive blood flow back into the coronary circulation via extensive, tortuous coronary sinusoids branching from the right ventricle to supply the coronary tree.
Key takeaways
Pulmonary atresia with an intact ventricular septum is almost universally associated with right ventricle-dependent coronary circulation. In these patients, decompression of the right ventricle is contraindicated because myocardial perfusion depends entirely on the high, suprasystemic right ventricular pressure. Maintaining ductal patency or providing an aortopulmonary shunt (such as a Blalock-Taussig shunt) is crucial to sustain pulmonary flow.
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