CTICasesAdult Pulmonary Atresia with Intact Ventricular Septum and Right Ventricular Dependent Coronary Circulation
[...]. Adult Pulmonary Atresia with Intact Ventricular Septum and Right Ventricular Dependent Coronary Circulation. CTICases. Published March 31, 2017. https://str-case-explorer.pages.dev/c/fjzyp
Imaging findings
Gated cardiac CT in a 28-year-old patient with congenital heart disease shows pulmonary atresia with an intact ventricular septum. There is marked dilation of the coronary arteries, with extensive, direct vascular communications (sinusoids) connecting the right ventricle to the right and left coronary circulation.
Key takeaways
This case represents another example of right ventricle-dependent coronary circulation complicating pulmonary atresia with an intact ventricular septum. In adults who have survived with this physiology, preserving right ventricular pressure is critical for myocardial perfusion, and recognizing the complex vascular pathways is vital during surgical planning or interventional procedures.
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