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Anomalous Aortic Origin of a Coronary Artery with an Interarterial Course: Understanding Current Management Strategies in Children and Young Adults

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Abstract

Anomalous aortic origin of a coronary artery from the opposite sinus of Valsalva with an interarterial course (AAOCA) is a rare congenital anomaly that is the second leading cardiovascular cause of sudden death in children and adolescents. Management practices with respect to this lesion vary among clinicians. We surveyed practitioners’ opinions on treatment and management strategies for patients with AAOCA. A 17-question “Patient Management Questionnaire” was sent to all Congenital Heart Surgeons’ Society (CHSS) members (n = 90), who were asked to respond and to distribute the questionnaire to pediatric cardiologists and nurse practitioners at their institutions. Questions addressed patient presentation, diagnostic modalities, therapeutic intervention, complications to therapy, follow-up visits, and type and frequency of follow-up testing. Descriptive statistics were used for analyses. We received 113 responses. Almost all respondents agree that ischemia at presentation or with provocative testing warrants surgical repair, whereas the majority believes that any patient with symptoms but no evidence of ischemia should have surgery. Approximately one-half and one-third of the respondents manage patients who are awaiting surgery or not having surgery by completely restricting exercise or not allowing competitive athletics, respectively. In asymptomatic patients, patient age determines timing of surgical repair but almost one-quarter did not believe that an asymptomatic child should undergo surgery. Among patients managed medically, there were six sudden deaths; there were two deaths in those who had surgical repair. There is marked heterogeneity in physician opinions regarding AAOCA treatment and management. A multicenter registry would facilitate the development of evidence-based treatment and management guidelines for children and young adults with AAOCA.

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References

  1. Basso C, Maron BJ, Corrado D, Thiene G (2000) 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 35:1493–1501

    Article  PubMed  CAS  Google Scholar 

  2. Cheitlin MD, De Castro CM, McAllister HA (1974) Sudden death as a complication of anomalous left coronary origin from the anterior sinus of Valsalva: a not-so-minor congenital anomaly. Circulation 50:780–787

    PubMed  CAS  Google Scholar 

  3. Corrado D, Thiene G, Nava A, Rossi L, Pennelli N (1990) Sudden death in young competitive athletes: clinicopathologic correlations in 22 cases. Am J Med 89:588–596

    Article  PubMed  CAS  Google Scholar 

  4. Frescura C, Basso C, Thiene G, Corrado D, Pennelli T, Angelini A, Daliento L (1998) Anomalous origin of coronary arteries and risk of sudden death: a study based on an autopsy population of congenital heart disease. Hum Pathol 29:689–695

    Article  PubMed  CAS  Google Scholar 

  5. Kragel AH, Roberts WC (1988) Anomalous origin of either the right or left main coronary artery from the aorta with subsequent coursing between aorta and pulmonary trunk: analysis of 32 necropsy cases. Am J Cardiol 62:771–777

    Article  PubMed  CAS  Google Scholar 

  6. Liberthson RR, Dinsmore RE, Fallon JT (1979) Aberrant coronary artery origin from the aorta: report of 18 patients, review of literature and delineation of natural history and management. Circulation 59:748–754

    PubMed  CAS  Google Scholar 

  7. Maron BJ, Shirani J, Poliac LC, Poliac LC, Mathenge R, Roberts WC, Mueller FO (1996) Sudden death in young competitive athletes: clinical, demographic and pathological profiles. JAMA 276:199–204

    Article  PubMed  CAS  Google Scholar 

  8. Taylor AJ, Byers JP, Cheitlin MD, Virmani R (1997) Anomalous right or left coronary artery from the contralateral coronary sinus: “high-risk” abnormalities in the initial coronary artery course and heterogeneous clinical outcomes. Am Heart J 133:428–435

    Article  PubMed  CAS  Google Scholar 

  9. Taylor AJ, Rogan KM, Virmani R (1992) Sudden cardiac death associated with isolated congenital coronary artery anomalies. J Am Coll Cardiol 20:640–647

    Article  PubMed  CAS  Google Scholar 

  10. Maron BJ (2003) Sudden death in young athletes. N Engl J Med 349:1064–1075

    Article  PubMed  CAS  Google Scholar 

  11. Davis JA, Cecchin F, Jones TK, Portman MA (2001) Major coronary artery anomalies in a pediatric population: incidence and clinical importance. J Am Coll Cardiol 37:593–597

    Article  PubMed  CAS  Google Scholar 

  12. Pelliccia A, Spataro A, Maron BJ (1993) Prospective echocardiographic screening for coronary artery anomalies in 1, 360 elite competitive athletes. Am J Cardiol 72:978–979

    Article  PubMed  CAS  Google Scholar 

  13. Topaz O, DeMarchena EJ, Perin E, Sommer LS, Mallon SM, Chahine RA (1992) Anomalous coronary arteries: angiographic findings in 80 patients. Int J Cardiol 34:129–138

    Article  PubMed  CAS  Google Scholar 

  14. Yamanaka O, Hobbs RE (1990) Coronary artery anomalies in 126, 595 patients undergoing coronary arteriography. Cathet Cardiovasc Diagn 21:28–40

    Article  PubMed  CAS  Google Scholar 

  15. Zeppilli P, dello Russo A, Santini C, Palmieri V, Natale L, Giordano A, Frustaci A (1998) In vivo detection of coronary artery anomalies in asymptomatic athletes by echocardiographic screening. Chest 114:89–93

    Article  PubMed  CAS  Google Scholar 

  16. Frommelt PC, Frommelt MA, Tweddell JS, Jaquiss RDB (2003) 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 42:148–154

    Article  PubMed  Google Scholar 

  17. Erez E, Tam VKH, Doublin NA, Stakes J (2006) Anomalous coronary artery with aortic origin and course between the great arteries: improved diagnosis, anatomic findings, and surgical treatment. Ann Thorac Surg 82:973–977

    Article  PubMed  Google Scholar 

  18. Romp RL, Herlong JR, Landolfo CK, Sanders SP, Miller CE, Ungerleider RM, Jaggers J (2003) Outcome of unroofing procedure for repair of anomalous aortic origin of left or right coronary artery. Ann Thorac Surg 76:589–596

    Article  PubMed  Google Scholar 

  19. Cheitlin MD (2008) Finding asymptomatic people with a coronary artery arising from the wrong sinus of valsalva: consequences arising from knowing the anomaly to be familial. J Am Coll Cardiol 51:2065–2067

    Article  PubMed  Google Scholar 

  20. Gersoney WM (2007) Management of anomalous coronary artery from the contralateral sinus. J Am Coll Cardiol 50:2083–2084

    Article  Google Scholar 

  21. Ashburn DA, McCrindle BW, Tchervenkov CI, Jacobs ML, Lofland GK, Bove EL, Spray TL, Williams WG (2003) Outcomes after the Norwood operation in neonates with critical aortic stenosis or aortic valve atresia. J Thorac Cardiovasc Surg 125:1070–1082

    Article  PubMed  Google Scholar 

  22. Lofland G, McCrindle B, Wiliams W, Blackstone E, Tchervenkov C, Sittwangkul R, Jonas R, the Congenital Heart Surgeons’ Society (2001) Critical aortic stenosis in the neonate: a multi-institutional study of management, outcomes and risk factors. J Thorac Cardiovasc Surg 121:10–27

    Article  PubMed  CAS  Google Scholar 

  23. Brothers JA, McBride MG, Seliem MA, Marino BS, Tomlinson RS, Pampaloni MH, Gaynor JW, Spray TL, Paridon SM (2007) Evaluation of myocardial ischemia following surgical repair of anomalous aortic origin of a coronary artery in a series of pediatric patients. J Am Coll Cardiol 50:2078–2082

    Article  PubMed  Google Scholar 

  24. Alphonso N, Anagnostopoulos PV, Nolke L, Moon-Grady A, Azakie A, Raff GW, Karl TR (2007) Anomalous coronary artery from the wrong sinus of Valsalva: a physiologic repair strategy. Ann Thorac Surg 83:1472–1476

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. The Children’s Hospital of Philadelphia, Cardiology, Philadelphia, PA, USA

    Julie Brothers & Stephen Paridon

  2. The Children’s Hospital of Philadelphia, Cardiothoracic Surgery, Philadelphia, PA, USA

    J. William Gaynor

  3. Cleveland Clinic, Pediatric Cardiology, Cleveland, OH, USA

    Richard Lorber

  4. Drexel University College of Medicine, Cardiothoracic Surgery, Philadelphia, PA, USA

    Marshall Jacobs

Authors
  1. Julie Brothers
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  2. J. William Gaynor
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  3. Stephen Paridon
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  4. Richard Lorber
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  5. Marshall Jacobs
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Corresponding author

Correspondence to Julie Brothers.

Additional information

This study was conducted on behalf of Anomalous Coronary Artery Working Group of the Congenital Heart Surgeons’ Society.

Appendix: Anomalous Aortic Origin of a Coronary Artery with an Interarterial Course (AAOCA)

Appendix: Anomalous Aortic Origin of a Coronary Artery with an Interarterial Course (AAOCA)

Patient Management Questionnaire

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Brothers, J., Gaynor, J.W., Paridon, S. et al. Anomalous Aortic Origin of a Coronary Artery with an Interarterial Course: Understanding Current Management Strategies in Children and Young Adults. Pediatr Cardiol 30, 911–921 (2009). https://doi.org/10.1007/s00246-009-9461-y

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  • DOI: https://doi.org/10.1007/s00246-009-9461-y

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