Angiographic evaluation of the coronary artery anatomy in patients with hypoplastic left heart syndrome

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Evaluation of coronary anatomy in survivors of staged palliation for hypoplastic left heart syndrome might be capable of identifying anomalies and morphologic changes of the coronary arteries that may have implications for outcome. We reviewed coronary angiograms obtained by native aortic root injection of 84 patients (mitral atresia/aortic atresia, n = 39; mitral stenosis/aortic stenosis, n = 25; mitral stenosis/aortic atresia (MS/AA), n = 13 and mitral atresia/aortic stenosis, n = 7). Epicardial course, anomalies, coronary dominance and native ascending aorta dimensions were analysed. Right dominance was present in 51%, left in 37% and balanced type in 12%. Dominance was unrelated to anatomic subtypes (P = 0.16). Ventriculocoronary connections (VCCs) were found in 15 (18%) and tortuosity was seen in 28 (33%) patients. Both occurred more often with MS/AA (tortuosity: 12 of 13 patients, P < 0.001; VCCs: 6 of 13 patients, P = 0.001). Collaterals to extra-cardiac vessels were visualized in 41 (49%) patients. Native ascending aorta dimensions were smaller with aortic atresia [40 (18-107) vs. 127 (32-328) mm(2)/m(2), P < 0.001]. In 18 patients with a relatively large native aorta [106 (36-328) vs. 44 (18-248) mm(2)/m(2), P < 0.001] retention of contrast in the aortic root identified areas of low blood flow. Left coronary dominance was more prevalent compared with the normal population. The impact of observed anomalies is unclear. Most VCCs are small and probably have no impact on coronary perfusion. Native ascending aorta dimensions were larger in patients with aortic stenosis. Larger aortic roots may predispose to thrombus formation and effective anticoagulation might be considered.