We conducted a systematic review and meta-analysis of the literature to assess the outcomes of surgery for the anomalous aortic origin of a coronary artery in children and young adults (<30 years). Thirteen publications were selected, including a total of 384 patients. Unroofing of the intramural segment was adopted in 92% (95% CI: 81%-98%) of cases, with pooled early and late mortality of 0% (95% CI: 0%-0.3%) and 0.1% (95% CI: 0%-1%), respectively. Reoperation for aortic regurgitation was anecdotal. Surgical management of anomalous aortic origin of coronary arteries can be achieved with excellent results in pediatric patients, but concerns remain about the durability of surgery.
YukselSMericMSoyluK, et al.The primary anomalies of coronary artery origin and course: a coronary angiographic analysis of 16,573 patients. Exp Clin Cardiol. 2013;18(2):121–123.
2.
EmeryMSKovacsRJ. Sudden cardiac death in athletes. JACC Heart Fail. 2018;6(1):30–40. doi:10.1016/j.jchf.2017.07.014
3.
BrothersJAFrommeltMAJaquissRDBMyerburgRJFraserCDJrTweddellJS. Expert consensus guidelines: anomalous aortic origin of a coronary artery. J Thorac Cardiovasc Surg. 2017;153(6):1440–1457. doi:10.1016/j.jtcvs.2016.06.066
4.
BaumgartnerHDe BackerJBabu-NarayanSV, et al.2020 ESC guidelines for the management of adult congenital heart disease. Eur Heart J. 2021;42(6):563–645. doi:10.1093/eurheartj/ehaa554
5.
KarangelisDMylonasKSLoggosSAdreanidesETzifaAMitropoulosF. Surgical repair of anomalous aortic origin of coronary artery in adults. Asian Cardiovasc Thorac Ann. 2021;29(1):51–58. doi:10.1177/0218492320957818
6.
BaumgartnerHDe BackerJBabu-NarayanSV, et al.ESC Scientific document group. 2020 ESC guidelines for the management of adult congenital heart disease. Eur Heart J. 2021;42(6):563–645. doi:10.1093/eurheartj/ehaa554
7.
LeeSUppuSCLytriviID, et al.Utility of multimodality imaging in the morphologic characterization of anomalous aortic origin of a coronary artery. World J Pediatr Congenit Heart Surg. 2016;7(3):308–317. doi:10.1177/2150135116634326
8.
MeryCM. Decision making in anomalous aortic origin of a coronary artery. Congenit Heart Dis. 2017;12(5):630–632. doi:10.1111/chd.12493
9.
BassoCMaronBJCorradoDThieneG. Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes. J Am Coll Cardiol. 2000;35(6):1493–1501. doi:10.1016/S0735-1097(00)00566-0
10.
FrescuraCBassoCThieneG, et al.Anomalous origin of coronary arteries and risk of sudden death: a study based on an autopsy population of congenital heart disease. Hum Pathol. 1998;29(7):689–695. doi:10.1016/S0046-8177(98)90277-5
11.
BrothersJAMcBrideMGMarinoBS, et al.Exercise performance and quality of life following surgical repair of anomalous aortic origin of a coronary artery in the pediatric population. J Thorac Cardiovasc Surg. 2009;137(2):380–384. doi:10.1016/j.jtcvs.2008.08.008
12.
NeesSNFlyerJNChelliahA, et al.Patients with anomalous aortic origin of the coronary artery remain at risk after surgical repair. J Thorac Cardiovasc Surg. 2018;155(6):2554–2564. e3. doi:10.1016/j.jtcvs.2017.12.134
13.
MoherDShamseerLClarkeM, et al.Preferred reporting items for systematic review and meta-analysis protocols (PRISMA-P) 2015 statement. Syst Rev. 2015;4(1):1. doi:10.1186/2046-4053-4-1
14.
DownesMJBrennanMLWilliamsHCDeanRS. Development of a critical appraisal tool to assess the quality of cross-sectional studies (AXIS). BMJ Open. 2016 Dec 8;6(12):e011458. doi:10.1136/bmjopen-2016-011458
15.
JegatheeswaranADevlinPJWilliamsWG, et al.Outcomes after anomalous aortic origin of a coronary artery repair: a Congenital Heart surgeons' Society study. J Thorac Cardiovasc Surg. 2020;160(3):757–771. e5. doi:10.1016/j.jtcvs.2020.01.114
16.
OsakiMMcCrindleBWVan ArsdellGDipchandAI. Anomalous origin of a coronary artery from the opposite sinus of Valsalva with an interarterial course: clinical profile and approach to management in the pediatric population. Pediatr Cardiol. 2008;29(1):24–30. doi:10.1007/s00246-007-9054-6
17.
Wittlieb-WeberCAParidonSMGaynorJWSprayTLWeberDRBrothersJA. Medium-term outcome after anomalous aortic origin of a coronary artery repair in a pediatric cohort. J Thorac Cardiovasc Surg. 2014;147(5):1580–1586. doi:10.1016/j.jtcvs.2013.07.022
18.
HerrmannJLGoldbergLAKhanAM, et al.A comparison of perioperative management of anomalous aortic origin of a coronary artery between an adult and pediatric cardiac center. World J Pediatr Congenit Heart Surg. 2016;7(6):721–726. doi:10.1177/2150135116668333
19.
BalasubramanyaSMongéMCEltayebOM, et al.Anomalous aortic origin of a coronary artery: symptoms do not correlate with intramural length or ostial diameter. World J Pediatr Congenit Heart Surg. 2017;8(4):445–452. doi:10.1177/2150135117710926
20.
SachdevaSFrommeltMAMitchellMETweddellJSFrommeltPC. Surgical unroofing of intramural anomalous aortic origin of a coronary artery in pediatric patients: single-center perspective. J Thorac Cardiovasc Surg. 2018;155(4):1760–1768. doi:10.1016/j.jtcvs.2017.11.003
21.
MeryCMDe LeónLEMolossiS, et al.Outcomes of surgical intervention for anomalous aortic origin of a coronary artery: a large contemporary prospective cohort study. J Thorac Cardiovasc Surg. 2018;155(1):305–319. e4. doi:10.1016/j.jtcvs.2017.08.116
22.
Bonilla-RamirezCMolossiSSachdevaS, et al.Outcomes in anomalous aortic origin of a coronary artery after surgical reimplantation. J Thorac Cardiovasc Surg. 2021;162(4):1191–1199. doi:10.1016/j.jtcvs.2020.12.100
23.
FrommeltPCFrommeltMATweddellJSJaquissRD. Prospective echocardiographic diagnosis and surgical repair of anomalous origin of a coronary artery from the opposite sinus with an interarterial course. J Am Coll Cardiol. 2003;42(1):148–154. doi:10.1016/S0735-1097(03)00503-5
24.
ErezETamVKDoublinNAStakesJ. Anomalous coronary artery with aortic origin and course between the great arteries: improved diagnosis, anatomic findings, and surgical treatment. Ann Thorac Surg. 2006;82(3):973–977. doi:10.1016/j.athoracsur.2006.04.089
25.
elZeinCHanhanZMassadM, et al.Surgical repair of anomalous coronary arteries arising from the opposite sinus of Valsalva in infants and children. J Card Surg. 2009;24(4):466–469. doi:10.1111/j.1540-8191.2009.00884.x
26.
BrothersJAKimTSFogelMA, et al.Cardiac magnetic resonance imaging characterizes stenosis, perfusion, and fibrosis preoperatively and postoperatively in children with anomalous coronary arteries. J Thorac Cardiovasc Surg. 2016;152(1):205–210. doi:10.1016/j.jtcvs.2015.12.057
27.
YerebakanCOzturkMMotaL, et al.Complete unroofing of the intramural coronary artery for anomalous aortic origin of a coronary artery: the role of commissural resuspension?J Thorac Cardiovasc Surg. 2019;158(1):208–217. e2. doi:10.1016/j.jtcvs.2019.01.140
28.
Mostefa KaraMFournierECohenS, et al.Anomalous aortic origin of coronary arteries: is the unroofing procedure always appropriate?Eur J Cardiothorac Surg. 2020:59(3);705–710. doi: 10.1093/ejcts/ezaa379
29.
AgrawalHMeryCMSamiSA, et al.Decreased quality of life in children with anomalous aortic origin of a coronary artery. World J Pediatr Congenit Heart Surg. 2021;12(2):204–210. doi:10.1177/2150135120978766
30.
SingACTsaurSParidonSMBrothersJA. Quality of life and exercise performance in unoperated children with anomalous aortic origin of a coronary artery from the opposite sinus of Valsalva. Cardiol Young. 2017;27(5):895–904. doi:10.1017/S1047951116001542
31.
AngeliniPMuthupillaiRLopezA, et al.Young athletes: preventing sudden death by adopting a modern screening approach? A critical review and the opening of a debate. Int J Cardiol Heart Vasc. 2021;34:100790.
32.
CheezumMKLiberthsonRRShahNR, et al.Anomalous aortic origin of a coronary artery from the inappropriate sinus of Valsalva. J Am Coll Cardiol. 2017;69(12):1592–1608. doi:10.1016/j.jacc.2017.01.031
33.
ThankavelPPBalakrishnanPLLemlerMSRamaciottiC. Anomalous left main coronary artery origin from the right sinus of Valsalva: a novel echocardiographic screening method. Pediatr Cardiol. 2013;34(4):842–846. doi:10.1007/s00246-012-0550-y
34.
BrothersJAWhiteheadKKKellerMS, et al.Cardiac MRI and CT: differentiation of normal ostium and intraseptal course from slitlike ostium and interarterial course in anomalous left coronary artery in children. AJR Am J Roentgenol. 2015;204(1):W104–W109. doi:10.2214/AJR.14.12953
35.
HarrisMAWhiteheadKKShinDCKellerMSWeinbergPMFogelMA. Identifying abnormal ostial morphology in anomalous aortic origin of a coronary artery. Ann Thorac Surg. 2015;100(1):174–179. doi:10.1016/j.athoracsur.2015.02.031
36.
MolossiSAgrawalHMeryCM, et al.Outcomes in anomalous aortic origin of a coronary artery following a prospective standardized approach. Circ Cardiovasc Interv. 2020;13(2):e008445. doi:10.1161/CIRCINTERVENTIONS.119.008445
37.
PoynterJAWilliamsWGMcIntyreSBrothersJAJacobsML. Congenital heart surgeons society AAOCA working group. Anomalous aortic origin of a coronary artery: a report from the congenital heart surgeons society registry. World J Pediatr Congenit Heart Surg. 2014;5(1):22–30. doi:10.1177/2150135113516984
38.
DoanTTMolossiSSachdevaS, et al.Dobutamine stress cardiac MRI is safe and feasible in pediatric patients with anomalous aortic origin of a coronary artery (AAOCA). Int J Cardiol. 2021;334:42–48. doi:10.1016/j.ijcard.2021.04.031
39.
AgrawalHWilkinsonJCNoelCV, et al.Impaired myocardial perfusion on stress CMR correlates with invasive FFR in children with coronary anomalies. J Invasive Cardiol. 2021;33(1):E45–E51.
40.
DoanTTZea-VeraRAgrawalH, et al.Myocardial ischemia in children with anomalous aortic origin of a coronary artery with intraseptal course. Circ Cardiovasc Interv. 2020;13(3):e008375.
Supplementary Material
Please find the following supplemental material available below.
For Open Access articles published under a Creative Commons License, all supplemental material carries the same license as the article it is associated with.
For non-Open Access articles published, all supplemental material carries a non-exclusive license, and permission requests for re-use of supplemental material or any part of supplemental material shall be sent directly to the copyright owner as specified in the copyright notice associated with the article.
