Outcomes after anomalous aortic origin of a coronary artery repair: A Congenital Heart Surgeons' Society Study

Anusha Jegatheeswaran; Paul J Devlin; William G Williams; Julie A Brothers; Marshall L Jacobs; William M DeCampli; Craig E Fleishman; James K Kirklin; Luc Mertens; Carlos M Mery; Silvana Molossi; Christopher A Caldarone; Nabi Aghaei; Richard O Lorber; Brian W McCrindle

doi:10.1016/j.jtcvs.2020.01.114

Outcomes after anomalous aortic origin of a coronary artery repair: A Congenital Heart Surgeons' Society Study

J Thorac Cardiovasc Surg. 2020 Sep;160(3):757-771.e5. doi: 10.1016/j.jtcvs.2020.01.114. Epub 2020 Apr 13.

Authors

Affiliations

¹ Division of Cardiovascular Surgery, Department of Surgery, University of Toronto, Hospital for Sick Children, Toronto, Ontario, Canada. Electronic address: anusha.jegatheeswaran@utoronto.ca.
² Division of Cardiovascular Surgery, Department of Surgery, University of Toronto, Hospital for Sick Children, Toronto, Ontario, Canada.
³ Division of Cardiology, Department of Pediatrics, The Children's Hospital of Philadelphia and Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pa.
⁴ Division of Cardiac Surgery, Department of Surgery, Johns Hopkins University, Baltimore, Md.
⁵ Division of Cardiac Surgery, Department of Surgery, University of Central Florida, College of Medicine, Arnold Palmer Hospital for Children, Orlando, Fla.
⁶ Division of Cardiology, Department of Pediatrics, University of Central Florida, College of Medicine, Arnold Palmer Hospital for Children, Orlando, Fla.
⁷ Division of Cardiothoracic Surgery, Department of Surgery, University of Alabama, School of Medicine, Birmingham, Ala.
⁸ Division of Cardiology, Department of Paediatrics, University of Toronto, Hospital for Sick Children, Toronto, Ontario, Canada.
⁹ Division of Pediatric and Congenital Heart Surgery, Department of Surgery and Perioperative Care, University of Texas at Austin, Dell Children's Medical Center, Austin, Tex.
¹⁰ Division of Cardiology, Department of Pediatrics, Texas Children's Hospital, Baylor College of Medicine, Houston, Tex.
¹¹ Division of Congenital Heart Surgery, Department of Surgery, Texas Children's Hospital, Baylor College of Medicine, Houston, Tex.
¹² Congenital Heart Surgeons' Society Data Center, Hospital for Sick Children, Toronto, Ontario, Canada.
¹³ Division of Cardiology, Department of Pediatrics, The Children's Hospital of San Antonio, Baylor College of Medicine, San Antonio, Tex.

PMID: 32800265
DOI: 10.1016/j.jtcvs.2020.01.114

Abstract

Objectives: It remains unclear when sudden cardiac event risk outweighs surgical risk for patients with anomalous aortic origin of a coronary artery. The Congenital Heart Surgeons' Society sought to characterize the surgical risks by determining the techniques, complications, and outcomes of repair.

Methods: Between January 2000 and September 2018, 682 patients with anomalous aortic origin of a coronary artery aged 30 years or less were enrolled. Demographic, morphologic, operative, imaging, and ischemia-related data were analyzed.

Results: There were 395 of 682 (57%) surgical patients (45 centers, median follow-up 2.8 years). In addition to primary repair (87% unroofing, 26% commissural manipulation), 13 patients had 15 coronary-related reoperations. Of 358 patients with pre/postoperative aortic insufficiency assessment, 27 (8%) developed new mild or greater aortic insufficiency postoperatively, and 7 (2%) developed new moderate or greater aortic insufficiency. Freedom from mild aortic insufficiency differed in those with versus without commissural manipulation (85%/91% at 6 months, 83%/90% at 1 year, and 77%/88% at 3 years, respectively) (P = .05). Of 347 patients with preoperative/postoperative ejection fraction, 6 (2%) developed new abnormal ejection fraction (<50%) within 30 days of surgery which persisted. Although 64 of 395 patients (16%) had preoperative ischemia, after surgery 51 of 64 patients (80%) no longer had ischemia (13 = new postoperative ischemia, P < .0001). Four patients died postoperatively (preoperatively 2 asymptomatic, 1 symptomatic, 1 in extremis). Composite surgical adverse event rates were 7% to 13% in the entire cohort (increasing/decreasing by presentation/anatomy/repair strategy).

Conclusions: Anomalous aortic origin of a coronary artery surgery may relieve ischemia with low mortality; however, it can result in a variety of important morbidities, varying by the group evaluated. Strategies avoiding commissural manipulation may decrease the risk of developing aortic insufficiency. Understanding these risks should inform surgical decision-making and support the need for standardized assessment and management.

Keywords: anomalous aortic origin of a coronary artery; complications; congenital heart disease; database; epidemiology; surgery.

MeSH terms

Adolescent
Adult
Aorta / surgery*
Aortic Valve Insufficiency / epidemiology
Cardiac Surgical Procedures* / adverse effects
Cardiac Surgical Procedures* / mortality
Cardiac Surgical Procedures* / statistics & numerical data
Child
Child, Preschool
Coronary Vessel Anomalies / surgery*
Female
Humans
Infant
Infant, Newborn
Male
Postoperative Complications* / epidemiology
Postoperative Complications* / mortality
Reoperation / statistics & numerical data
Retrospective Studies
Risk Assessment
Treatment Outcome
Young Adult