Skip to main content

Tetralogy of fallot in addition to anomalous aortic origin of a coronary artery in a 1-year-old boy: a case report

Abstract

Background

Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital heart disease, characterized by the coronary artery inappropriately originates from the aorta. It is usually classified according to the sinus where the coronary artery arises from, while anomalous origin of the right coronary being the most common type.

Case presentation

In this case report, we described a rare case of Tetralogy of Fallot (TOF) in a 1-year-old boy, who also had the anomalous right coronary artery that originated from the left coronary sinus without an intramural segment. Besides TOF repair, lateral pulmonary translocation was undertaken in order to avoid risks of myocardial ischemia.

Conclusion

We successfully completed a one-stage operation consisting of TOF repair and pulmonary artery translocation in a 1-year-old boy. We advocated early operation of pulmonic translocation for AAOCA patients without an intramural segment instead of unroofing procedure.

Peer Review reports

Background

Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital heart condition, in which the origin of a coronary artery that arises from the aorta is abnormal, and the coronary artery usually has an intramural segment [1]. The prevalence of AAOCA is uncertain, probably ranges from 0.21 to 5.79% in the general population [2,3,4]. While in those TOF patients, it seems not so rare, accounts for about 10% [5,6,7]. Although rare, AAOCA is recognized as the second leading cause of sudden cardiac death (SAD) in children and adolescents. AAOCA is usually classified according to the location of the anomalous coronary ostium: right coronary artery (RCA) originates from the left coronary sinus, left coronary artery (LCA) originates from the right coronary sinus, or, one or both coronary arteries originate from the non-coronary sinus.

All types of AAOCA are associated with increased risks of myocardial ischemia and SAD, especially in young athletes [8]. Studies have reported that the anomalous origin of RCA is more common than the anomalous origin of LCA [9], while the anomalous origin of LCA carries a higher risk of SAD [10]. The most common surgery performed for patients with an AAOCA and an intramural segment is the unroofing procedure of the intramural segment, which may reduce the risk of SAD during physical activities [11]. On the other hand, lateral pulmonary translocation can be performed for those without an intramural segment [12]. To our knowledge, this is the first case report in the literature that described a rare presentation of a 1-year-old boy with both TOF and AAOCA who successfully underwent a combined surgical procedure of TOF with pulmonary artery translocation.

Case presentation

A 1-year-old boy presented to our center from a regional hospital for further assessment and management of TOF. The child was found to have structural abnormalities of the heart in his fetal stage. He presented with mild cyanosis, slight shortness of breath after activity, and poor feeding. Physical examination revealed a normal growth level at the height of 78 cm and the weight of 8.5 kg. On further investigation, the transoesophageal echocardiography (TEE) demonstrated the common features of TOF consisting of a 14-mm ventricular septal defect (VSD), pulmonary valve stenosis with a 5-mm valve ring, supravalvular pulmonary stenosis with a 4-mm local inner diameter, subvalvular pulmonary stenosis with a 5-mm infundibular pulmonary artery, 40% overriding of the aorta above VSD, and 6-mm-thickness right ventricular anterior wall. The Color Doppler assessment demonstrated a shunt flow mainly from the left ventricle to the right at the level of VSD, pulmonary valve stenosis and supravalvular pulmonary stenosis with a pressure gradient of 106 mmHg, infundibular pulmonary artery stenosis with a pressure gradient of 78 mmHg, and mild mitral and tricuspid regurgitation. Further assessment with computed tomography (CT) not only confirmed the diagnosis of TOF but also revealed both anomalous RCA and the normal LCA arose from the left coronary sinus, which coursed between the aorta and the pulmonary artery without an intramural segment (Fig. 1). Additionally, the CT also demonstrated stenosis of the pulmonary trunk and the normal left and right pulmonary artery (Fig. 2).

Fig. 1
figure 1

Computed tomography (CT) revealed both anomalous RCA and the normal LCA arose from the left coronary sinus, which coursed between the aorta and the pulmonary artery without an intramural segment

Fig. 2
figure 2

Narrow pulmonary trunk with normal left and right pulmonary artery

Given the clinical features and radiological findings, a one-stage operation was carried out, which consisted of TOF repair (including VSD closure, trans-annular enlargement of the right ventricular outflow tract, and infundibular muscle resection) and lateral pulmonary translocation that aimed to improve coronary flow. A median sternotomy was performed, and cardiopulmonary bypass was initiated through aortic and bi-caval cannulation. Antegrade cardioplegia was administrated to induce cardiac arrest. TOF repair was first performed, followed by pulmonary artery translocation by introducing an autologous blood vessel patch of the right pulmonary artery and inserting this patch in the lateral aspect of the main pulmonary artery. Postoperatively, the TEE showed no residual shunting, mitral or tricuspid regurgitation, with the CT revealed normal blood flow of RCA without inter-arterial compression. The boy was stable during the immediate postoperative period with no arrhythmia or cyanosis. He recovered uneventfully and was discharged from the hospital.

Discussion and conclusion

Being a rare congenital cardiac disease, the reported prevalence of AAOCA in the general population varies widely, with the true incidence remains unknown [13]. As previously mentioned, the prevalence of AAOCA in TOF patients is higher than that in the general population. Compared to single AAOCA, combined with TOF may influence the selection of surgical procedures. However, recognized AAOCA in TOF patients is still a challenge, some studies reported that postmortem examinations indicate a higher incidence [14, 15]. Thus, reasonable screening and careful evaluation are advisable in TOF patients preoperatively. Selective coronary angiography was considered as a “golden standard” method to diagnose AAOCA, nevertheless, it is not a suitable method for screening. As a noninvasive method, echocardiographic screening was recommended to recognize AAOCA in TOF patients recently study [16].

The clinical manifestations of patients with AAOCA also vary, ranging from complete lack of symptoms to obvious myocardial ischemia such as chest pain and SAD, though the exact mechanisms and the crucial risk factors leading to SAD have not been well understood [17]. An increased cardiac output due to exercise or stress, leading to compression of the abnormal coronary artery between the aorta and pulmonary artery has been regarded previously as the main reason for myocardial ischemia and SAD [18]. Recently, studies have found that factors including intramural course, acute take-off angle, slit-like ostium and proximal narrowing with elliptic vessel shape may be accounted for the occurrence of myocardial ischemia and SAD [1, 19,20,21,22]. Nevertheless, AAOCA is associated with an increased risk of SAD and thus, corrective surgery is warranted to prevent adverse outcomes.

As the most accepted surgical procedure for AAOCA with intramural segments, unroofing could reduce the incidence of SAD. Alternatively, translocation and reimplantation can be performed for those without intramural segments [23], for this procedure could effectively relieve the compression of anomalous coronary artery. On the other hand, enlargement of the pulmonary artery is an essential component during TOF repair, which may result in the possibility of inter-arterial compression of the RCA. To achieve the optimal outcome for our patient, a one-stage operation consisted of TOF repair and pulmonary artery translocation was conducted to correct both the TOF and AAOCA, respectively. Another study has described the procedure named ostioplasty, which was less utilized but could be selected for AAOCA patients without acute take-off angle or commissural ostial location [24].

In summary, our case has demonstrated the feasibility and success of a one-stage operation consisting of TOF repair and pulmonary artery translocation for a patient presented with both the TOF and anomalous RCA that arose from the left coronary sinus without an intramural segment. Although rare, patients with such complex congenital heart disease should be considered for simultaneous repair of all the anomalies for the best possible long-term outcome.

Availability of data and materials

Data sharing is not applicable to this article as no datasets were generated or analyzed during the current study.

Abbreviations

AAOCA:

Anomalous aortic origin of a coronary artery

TOF:

Tetralogy of Fallot

SAD:

Sudden cardiac death

RCA:

Right coronary artery

LCA:

Left coronary artery

References

  1. 1.

    Jegatheeswaran A, et al. Features associated with myocardial ischemia in anomalous aortic origin of a coronary artery: a Congenital Heart Surgeons’ Society study. J Thorac Cardiovasc Surg. 2019;158:822-834.e823. https://doi.org/10.1016/j.jtcvs.2019.02.122.

    Article  PubMed  Google Scholar 

  2. 2.

    Pérez-Pomares JM, et al. Congenital coronary artery anomalies: a bridge from embryology to anatomy and pathophysiology—a position statement of the development, anatomy, and pathology ESC Working Group. Cardiovasc Res. 2016;109:204–16. https://doi.org/10.1093/cvr/cvv251.

    CAS  Article  PubMed  PubMed Central  Google Scholar 

  3. 3.

    Cheezum MK, et al. Anomalous aortic origin of a coronary artery from the inappropriate sinus of valsalva. J Am Coll Cardiol. 2017;69:1592–608. https://doi.org/10.1016/j.jacc.2017.01.031.

    Article  PubMed  PubMed Central  Google Scholar 

  4. 4.

    Gräni C, et al. Prevalence and characteristics of coronary artery anomalies detected by coronary computed tomography angiography in 5 634 consecutive patients in a single centre in Switzerland. Swiss Med Wkly. 2016;146: w14294. https://doi.org/10.4414/smw.2016.14294.

    Article  PubMed  Google Scholar 

  5. 5.

    Dabizzi RP, et al. Distribution and anomalies of coronary arteries in tetralogy of fallot. Circulation. 1980;61:95–102. https://doi.org/10.1161/01.cir.61.1.95.

    CAS  Article  PubMed  Google Scholar 

  6. 6.

    Shrivastava S, Mohan JC, Mukhopadhyay S, Rajani M, Tandon R. Coronary artery anomalies in tetralogy of Fallot. Cardiovasc Intervent Radiol. 1987;10:215–8. https://doi.org/10.1007/bf02593873.

    CAS  Article  PubMed  Google Scholar 

  7. 7.

    Li J, Soukias ND, Carvalho JS, Ho SY. Coronary arterial anatomy in tetralogy of Fallot: morphological and clinical correlations. Heart (British Cardiac Society). 1998;80:174–83. https://doi.org/10.1136/hrt.80.2.174.

    CAS  Article  Google Scholar 

  8. 8.

    Bigler MR, et al. Hemodynamic relevance of anomalous coronary arteries originating from the opposite sinus of valsalva-in search of the evidence. Front Cardiovasc Med. 2020;7: 591326. https://doi.org/10.3389/fcvm.2020.591326.

    Article  PubMed  Google Scholar 

  9. 9.

    Agrawal H, Mery CM, Krishnamurthy R, Molossi S. Anatomic types of anomalous aortic origin of a coronary artery: a pictorial summary. Congenit Heart Dis. 2017;12:603–6. https://doi.org/10.1111/chd.12518.

    Article  PubMed  Google Scholar 

  10. 10.

    Yerebakan C, 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;158:208-217.e202. https://doi.org/10.1016/j.jtcvs.2019.01.140.

    Article  PubMed  Google Scholar 

  11. 11.

    Vinnakota A, et al. Anomalous aortic origin of the coronary arteries: a novel unroofing technique in an adult cohort. Ann Thorac Surg. 2019;107:823–8. https://doi.org/10.1016/j.athoracsur.2018.08.036.

    Article  PubMed  Google Scholar 

  12. 12.

    Zheng J, Lan Y, Fan Q, Ling Y, Qian Y. Anomalous aortic origin of the coronary arteries in a 12-year-old male: a case report. BMC Surg. 2020;20:311. https://doi.org/10.1186/s12893-020-00984-5.

    Article  PubMed  PubMed Central  Google Scholar 

  13. 13.

    Mazine A, Fernandes IM, Haller C, Hickey EJ. Anomalous origins of the coronary arteries: current knowledge and future perspectives. Curr Opin Cardiol. 2019;34:543–51. https://doi.org/10.1097/hco.0000000000000663.

    Article  PubMed  Google Scholar 

  14. 14.

    Longenecker CG, Reemtsma K, Creech O Jr. Anomalous coronary artery distribution associated with tetralogy of Fallot: a hazard in open cardiac repair. J Thorac Cardiovasc Surg. 1961;42:258–62.

    CAS  Article  Google Scholar 

  15. 15.

    Meng CC, Eckner FA, Lev M. Coronary artery distribution in tetralogy of fallot. Arch Surg (Chicago, Ill, 1960). 1965;90:363–6. https://doi.org/10.1001/archsurg.1965.01320090041009.

    CAS  Article  Google Scholar 

  16. 16.

    Bianco F, et al. Echocardiographic screening for the anomalous aortic origin of coronary arteries. Open Heart. 2021. https://doi.org/10.1136/openhrt-2020-001495.

    Article  PubMed  PubMed Central  Google Scholar 

  17. 17.

    Molossi S, Martínez-Bravo LE, Mery CM. Anomalous aortic origin of a coronary artery. Methodist Debakey Cardiovasc J. 2019;15:111–21. https://doi.org/10.14797/mdcj-15-2-111.

    Article  PubMed  PubMed Central  Google Scholar 

  18. 18.

    Angelini P. Coronary artery anomalies: an entity in search of an identity. Circulation. 2007;115:1296–305. https://doi.org/10.1161/circulationaha.106.618082.

    Article  PubMed  PubMed Central  Google Scholar 

  19. 19.

    Brothers JA, 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:W104-109. https://doi.org/10.2214/ajr.14.12953.

    Article  PubMed  Google Scholar 

  20. 20.

    Gräni C, et al. Hybrid CCTA/SPECT myocardial perfusion imaging findings in patients with anomalous origin of coronary arteries from the opposite sinus and suspected concomitant coronary artery disease. J Nucl Cardiol. 2017;24:226–34. https://doi.org/10.1007/s12350-015-0342-x.

    Article  PubMed  Google Scholar 

  21. 21.

    Cheezum MK, et al. Anomalous origin of the coronary artery arising from the opposite sinus: prevalence and outcomes in patients undergoing coronary CTA. Eur Heart J Cardiovasc Imaging. 2017;18:224–35. https://doi.org/10.1093/ehjci/jev323.

    Article  Google Scholar 

  22. 22.

    Diao KY, et al. Prognostic value of dual-source computed tomography (DSCT) angiography characteristics in anomalous coronary artery from the opposite sinus (ACAOS) patients: a large-scale retrospective study. BMC Cardiovasc Disord. 2020;20:25. https://doi.org/10.1186/s12872-019-01285-3.

    CAS  Article  PubMed  PubMed Central  Google Scholar 

  23. 23.

    Corrado D, Basso C, Schiavon M, Thiene G. Does sports activity enhance the risk of sudden cardiac death? J Cardiovasc Med. 2006;7:228–33. https://doi.org/10.2459/01.Jcm.0000219313.89633.45.

    Article  Google Scholar 

  24. 24.

    Gaillard M, et al. Anomalous aortic origin of coronary arteries: an alternative to the unroofing strategy. Eur J Cardio-thorac Surg. 2020;58:975–82. https://doi.org/10.1093/ejcts/ezaa129.

    Article  Google Scholar 

Download references

Acknowledgements

Not applicable.

Funding

All authors declare that there is no funding to this publication.

Author information

Affiliations

Authors

Contributions

YL and TL performed the surgery. LD provided pathology images. TL prepared the manuscript. MT and YL revised the manuscript critically. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Menglin Tang.

Ethics declarations

Ethics approval and consent to participate

Not applicable.

Consent for publication

Written informed consent was obtained from the patient’s parents for publication of this case report and any accompanying images.

Competing interests

All authors declare that they have no competing interests.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Deng, L., Li, T., Ling, Y. et al. Tetralogy of fallot in addition to anomalous aortic origin of a coronary artery in a 1-year-old boy: a case report. BMC Surg 21, 384 (2021). https://doi.org/10.1186/s12893-021-01380-3

Download citation

  • Received:

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1186/s12893-021-01380-3

Keywords

  • Anomalous aortic origin of a coronary artery (AAOCA)
  • Tetralogy of Fallot (TOF)
  • Infant
  • Case report