Skip to main content
Clinical Cardiology logoLink to Clinical Cardiology
. 2009 Feb 3;21(10):773–776. doi: 10.1002/clc.4960211016

Anomalous origin of the right coronary artery from the main pulmonary artery: Diagnosis and management

Vahid Hekmat 1, Sudha M Rao 1, Manoj Chhabra 1, Mario Chiavarelli 1, John E Anderson 1, Dov B Nudel 1,
PMCID: PMC6655656  PMID: 9789702

Abstract

Anomalous origin of the right coronary artery (ARCA) from the main pulmonary artery (MPA) is a rare congenital anomaly, with only 18 reported cases in the pediatric age group. More than half of these had associated cardiac anomalies that masked the presence of ARCA. Conversely, in many patients with ARCA as an isolated anomaly, the diagnosis has been missed during lifetime. The only patient with an isolated ARCA who was diagnosed in infancy presented with congestive heart failure. Asymptomatic infants with ARCA from the MPA have not been previously reported. Three additional cases, two infants and a child with ARCA from the MPA, are reported in this paper. The diagnostic dilemmas and the prognosis are discussed and management is recommended.

Keywords: anomalous; right coronary artery; diagnosis, management

Full Text

The Full Text of this article is available as a PDF (1.2 MB).

References

  • 1. Brooks H: Two cases of an abnormal coronary artery of the heart arising from the pulmonary artery: With some remarks upon the effects of this anomaly in producing cirsoid dilatation of the vessels. J Anat 1885; 20: 26–29 [PMC free article] [PubMed] [Google Scholar]
  • 2. Roberts WC: Major anomalies of coronary arterial origin seen in adulthood. Am Heart J 1986; 111: 941–963 [DOI] [PubMed] [Google Scholar]
  • 3. Moss RL, Backer CL, Zales VR, Florentine MS, Mavroudis C: Tetralogy of Fallot with anomalous origin of the right coronary artery. Ann Thorac Surg 1995; 59 (1): 229–231 [DOI] [PubMed] [Google Scholar]
  • 4. Nakano M, Emoto H, Koyanagi K, Okuyama H, Saito F, Kurosawa H: Report of a case of the anomalous origin of the right coronary artery from the pulmonary artery with atrial fibrillation and bradycardia. J Jpn Assoc Thorac Surg 1993; 41 (3): 479–485 [PubMed] [Google Scholar]
  • 5. Shah RM, Nanda NC, Hsiung MC, Moos S, Roitman D: Identification of anomalous origin of the right coronary artery from the pulmonary trunk by color flow mapping. Am J Cardiol 1986; 57: 366–367 [DOI] [PubMed] [Google Scholar]
  • 6. Tosovsky J, Rohac J: Anomalous origin of the right coronary artery from the pulmonary artery. Rozhledy V Chirurgii 1993; 72 (5): 199–200 [PubMed] [Google Scholar]
  • 7. Tuma S, Hucin B, Reich O, Voriskova M, Radvansky J: Anomalous origin of the right coronary artery from the pulmonary artery. Preoperative diagnostic finding in the aortopulmonary window. Ceskosloenska Pediatric 1990; 45 (9): 543–545 [PubMed] [Google Scholar]
  • 8. Ueeda M, Yamada N, Shimizu A, Hina K, Terasaka R, Saito D, Tsujii T, Yoshida H, Haraoka S: A case of anomalous origin of the right coronary artery from the pulmonary trunk. Imaging of abnormal flow by Doppler color flow mapping. J Cardiol 1998; 18: 583–587 [PubMed] [Google Scholar]
  • 9. Brouwer MH, Beaufort‐Krol GC, Talsma MD: Aortopulmonary window associated with an anomalous origin of the right coronary artery. Int J Cardiol 1990; 28 (3): 384–386 [DOI] [PubMed] [Google Scholar]
  • 10. Donaldson RM, Raphael MJ, Radley‐Smith R, Yacoub MH: Angiographic diagnosis of anomalous origin of the right coronary artery from the pulmonary artery. Br J Radiol 1983; 56: 17–19 [DOI] [PubMed] [Google Scholar]
  • 11. Luisi SV, Ashraf MH, Gula G, Radley‐Smith R, Yacoub M: Anomalous origin of the right coronary artery with aortopulmonary window: Functional and surgical considerations. Thorax 1980; 35 (6): 446–448 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 12. Vairo U, Marino B, De Simone G, Marcelletti C: Early congestive heart failure due to origin of the right coronary artery from the pulmonary artery. Chest 1992; 102 (5): 1610–1612 [DOI] [PubMed] [Google Scholar]
  • 13. McManus BM, Waller BF, Jones M, Epstein SE, Roberts WC: The case for preoperative coronary angiography in patients with tetralogy of Fallot and other complex congenital heart disease. Am Heart J 1982; 103: 451–456 [DOI] [PubMed] [Google Scholar]
  • 14. Richardson JV, Doty DB, Rossi NP, Ehrenhaft JL: The spectrum of anomalies of aortopulmonary septation. J Thorax Cardiovasc Surg 1979; 1: 21–27 [PubMed] [Google Scholar]
  • 15. Bogers AJJC, Gittenberg‐de Groot AC, Poelman RE, Peault BM, Huysmans HA: Development of the origin of the coronary arteries, a matter of ingrowth outgrowth? Anat Embryol 1989; 180 (5): 437–441 [DOI] [PubMed] [Google Scholar]
  • 16. Bortolotti U, Casarotto D, Betti D, De Mozzi PL, Stritoni P, Cevese PG: Anomalous origin of the right coronary artery from the main pulmonary artery. Eur J Cardiol 1978; 7 (5‐6): 451–455 [PubMed] [Google Scholar]
  • 17. Screenivasan VV, Jacobstein MD: Origin of the right coronary artery from the pulmonary trunk. Am J Cardiol 1992;: 69 (17): 1513–1514 [DOI] [PubMed] [Google Scholar]
  • 18. Wald S, Stonecipher K, Baldwin BJ, Nutter DO: Anomalous origin of the right coronary artery from the pulmonary artery. Am J Cardiol 1971; 27: 677–681 [DOI] [PubMed] [Google Scholar]
  • 19. Worsham C, Sanders SP, Burger BM: Origin of the right coronary artery from pulmonary trunk: Diagnosis by 2‐D echocardiography. Am J Cardiol 1985; 55: 232–233 [DOI] [PubMed] [Google Scholar]
  • 20. Eugster GS, Olivia PB: Anomalous origin of the right coronary artery from the pulmonary artery. Chest 1973; 63, 2: 294–296 [DOI] [PubMed] [Google Scholar]
  • 21. Lerberg DB, Ogden JA, Zuberbuhler JR, Bahson HT: Anomalous origin of the right coronary artery from the pulmonary artery. Ann Thorac Surg 1979; 27: 87–94 [DOI] [PubMed] [Google Scholar]
  • 22. Huang TY, Hsueh Y, Tsung SH: Endocardial fibroelastosis and myocardial calcification secondary to anomalous right coronary artery arising from the pulmonary trunk. Hum Pathol 1985; 16: 959–960 [DOI] [PubMed] [Google Scholar]
  • 23. Tingelstad JB, Lower RR, Eldredge WJ: Anomalous origin of the right coronary artery from the main pulmonary artery. Am J Cardiol 1972; 30: 670–673 [DOI] [PubMed] [Google Scholar]

Articles from Clinical Cardiology are provided here courtesy of Wiley

RESOURCES