Coronary anomalies in single ventricles: Insights from selective angiographic assessment.

Background: Adults with congenital heart disease have a higher risk of coronary artery disease compared to the general population. However, there is limited information on coronary artery distribution in patients with a single ventricle (SV), which is important in understanding potential cardiovascular events. This study aimed to evaluate coronary artery morphology and anomalies in patients with SV based on selective coronary angiography (CAG).

Methods: We performed a retrospective single-center study including 80 patients with SV [median age, 29 years (range 13-50); 54 % males] who underwent selective CAG at our institution between 2019 and 2023. Patients were classified into either single right or left ventricular (SRV and SLV) morphologies and categorized into D-, L-, and X-loops based on the rules of cardiovascular looping. Coronary artery morphology, dominance, and the abnormality of origin were evaluated.

Results: Of the 80 SV patients, 56 had SRV and 24 had SLV. Patients with coronary artery looping surrounding the rudimentary chamber were more frequent in cases with D-loops. In patients with SV, right coronary artery dominance was observed in 70 %, and anomalous origins of coronary artery origins were found in 36.3 % of cases, with a higher frequency of L-loops. Overall, cardiovascular events were observed in patients with SV as follows: 46 % of patients had arrhythmias, 13 % had heart failure, and 8 % had thrombosis; there were no cardiovascular deaths. A single coronary artery was identified in 9 cases (11 %) of SV patients, exclusively in SRV, of which 2 cases (22 %) had arrhythmias and 1 case (11 %) had thrombus.

Conclusion: Selective CAG revealed a high prevalence of coronary artery anomalies in patients with SV, emphasizing the importance of comprehensive assessment and long-term follow-up to manage cardiovascular risk in this patient population.