CTICasesPulmonary Atresia with Intact Ventricular Septum
Imaging findings
CT shows pulmonary atresia with an intact ventricular septum, a hypoplastic tricuspid valve, and no main pulmonary artery. Coronary-cameral sinusoids connect the high-pressure right ventricle to the coronary arteries, and there are large intercostal and bronchial collaterals plus venous collaterals draining to pulmonary veins from a Fontan circuit. A delayed acquisition shows extensive subendocardial enhancement of the hypoplastic right ventricle and possible atrial wall enhancement, confirmed on cardiac MRI.
Key takeaways
In pulmonary atresia with intact ventricular septum, one must look for coronary sinusoids, since the right coronary may be supplied entirely by sinusoids from the high-pressure right ventricle (RV-dependent coronary circulation), meaning the RV cannot be decompressed without causing coronary ischemia. Late subendocardial enhancement of the hypoplastic RV may reflect endocardial fibroelastosis or chronic pressure-related injury, and Fontan physiology drives systemic-to-pulmonary venous collaterals.
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