Transconal Unroofing of Anomalous Intraseptal Left Coronary Artery with RVOT Patch Reconstruction

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Improved understanding of risks associated with coronary anomalies, including myocardial ischemia and sudden cardiac death, have led to renewed enthusiasm for operative intervention to restore optimal perfusion and mitigate risk of future events. Advances in contemporary screening practices and diagnostic modalities have improved recognition of these anomalies. Young patients in particular face a lifetime of exposure to risks of these lesions, and activity restrictions can significantly affect their quality of life.

Until recently, patients with anomalous aortic origin of a left coronary artery with an intraseptal course had few surgical options, which were often reserved for those with prior cardiac arrest or myocardial infarction. Coronary artery bypass grafting (CABG), fraught with questionable long-term patency and competitive graft flow, was the prevailing reported technique.

In recent years, however, novel techniques that directly relieve the anatomic source of compression on intraseptal coronary arteries have been reported in the literature, predominantly in the adult population, with promising short-to-midterm outcomes. Notably, in 2019, Najm et. Al. published their technique for transconal unroofing of the intraseptal coronary with patch reconstruction of the posterior right ventricular outflow tract (5). At the authors’ pediatric institution, they have employed this technique in several patients, resulting in short-term reduction in ischemic symptoms, improvements in coronary perfusion, and return to an active lifestyle. This case presentation depicts the implementation of this technique in one such patient with ischemic symptoms and evidence of impaired coronary perfusion by cardiac catheterization.

Further investigation, including in the pediatric population, is warranted to delineate the long-term safety and benefits of such interventions. In the meantime, with appropriate counseling and shared decision making, these novel techniques for anatomic-directed repair of intraseptal coronary arteries are a good option for carefully selected pediatric patients.

References

1. Kalustian AB, Doan TT, Masand P, Gowda ST, Eilers LF, Reaves-O’Neal DL, Sachdeva S, Qureshi AM, Heinle J, Molossi SM, and Binsalamah ZM (2023). Evolution of surgical repair of intraseptal anomalous left coronary artery with myocardial ischaemia. Cardiology in the Young, page 1 of 8. doi: 10.1017/S1047951123000197
2. Molossi S, Agrawal H, Mery CM, et al. Outcomes in anomalous aortic origin of a coronary artery following a prospective standardized approach. Circ Cardiovasc Interv. 2020 Feb;13:e008445. doi: 10.1161/CIRCINTERVENTIONS.119.008445. Epub 2020 Feb 13. PMID: 32069111.
3. Doan TT, Zea-Vera R, Agrawal H et al. Myocardial ischemia in children with anomalous aortic origin of a coronary artery with intraseptal course. Circ Cardiovasc Interv. 2020 Mar;13:e008375. doi: 10.1161/CIRCINTERVENTIONS.119.008375. Epub 2020 Feb 27. PMID: 32102565.
4. Doan TT, Molossi S, Qureshi AM, McKenzie ED. Intraseptal anomalous coronary artery with myocardial infarction: novel surgical approach. Ann Thorac Surg. 2020 Oct;110:e271–e274. doi: 10.1016/j.athoracsur.2020.02.076. Epub 2020 Apr 9. PMID: 32278751.
5. Najm HK, Ahmad M. Transconal unroofing of anomalous left main coronary artery from right sinus with trans-septal course. Ann Thorac Surg. 2019 Dec;108:e383–e386. doi: 10.1016/j.athoracsur.2019.04.021. Epub 2019 May 15. PMID: 31102634.
6. Najm HK, Ahmad M, Hammoud MS, Costello JP, Karamlou T. Surgical pearls of the transconal unroofing procedure-modifications and midterm outcomes. Ann Thorac Surg. 2022 Apr;28:S0003–4975(22) 00641–5. doi: 10.1016/j.athoracsur.2022.04.027. Epub ahead of print. PMID: 35490772.
7. Gaillard M, Pontailler M, Danial P, et al. Anomalous aortic origin of coronary arteries: an alternative to the unroofing strategy. Eur J Cardiothorac Surg. 2020 Nov 1;58:975–982. doi: 10.1093/ejcts/ezaa129. PMID: 32572445.

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