Dear Editor,
Congenital coronary artery anomalies are rare and uncommon lesions, occurring in 0.3–0.9% of the general population without structural heart defects and 3–6% of those with congenital heart defects. Total anomalous origin of the coronary arteries from the pulmonary artery (TCAPA) is extremely rare and carries a high mortality, if unrecognized.
We report a case of total anomalous origin of the coronary arteries from the right pulmonary artery with an intramural course in a 5-year-old boy who underwent successful surgical correction. To our best knowledge, this is the first such case in the literature.
Case report
A 5-year-old boy, being treated for heart failure on ventilatory support and inotropes, was referred to our centre for further management. Electrocardiogram (ECG) showed ischemic changes in the inferior and lateral precordial leads (Fig. 1). 2D echocardiogram showed severe mitral regurgitation (MR), poor left ventricular ejection fraction (LVEF—18%) and fibroelastosis, suspicious of abnormal coronary anatomy (Fig. 2). Cardiac computerized tomography angiogram (CTA) showed single coronary ostium arising from the pulmonary artery, bifurcating into the left and right coronary arteries (Fig. 3A). Cardiac catheterization angiogram showed total anomalous origin of the coronaries from the right coronary artery (Fig. 3B).
Fig. 1.
Electrocardiogram (ECG) showing ischemic changes
Fig. 2.
2D Echocardiogram. A Severe mitral regurgitation. B Fibroelastosis
Fig. 3.
A Computerized tomography angiogram (CTA). pa, pulmonary artery; rca, right coronary artery; lca, left coronary artery. B Pulmonary artery angiogram, showing the origin of coronaries from the right pulmonary artery
Surgical repair was an adaptation of the technique described by Adachi et al. [1] and Zhang et al. [2]. Separating the two coronary arteries was not possible as this could have led to inadequate button size to perform anastomosis. Hence, we harvested two coronary ostia as a single button from the right pulmonary artery. Unroofing of the intramural course was done and a modified trapdoor technique was used to reimplant the coronary button; the ostium was enlarged with a hood fashioned using a pericardial patch (Fig. 4). The mitral annulus was dilated; hence, a ring annuloplasty was performed using 26-mm Carpentier Edward Physio ring. A left atrial pressure monitoring line and a peritoneal dialysis catheter were inserted following the termination of cardiopulmonary bypass. Intravenous adrenaline (0.1 mcg/kg/min) and milrinone (0.5 mcg/kg/min) were started. The chest was electively left open.
Fig. 4.
Intraoperative illustration. RPA, right pulmonary artery; RCA, right coronary artery; LCA, left coronary artery
In the postoperative period, hemodynamics were stable and the left atrial pressure was less than 12 mmHg. Peritoneal dialysis was started electively within 6 h, in the intensive care unit and continued for 48 h. The chest was closed after 48 h and the patient was extubated on postoperative day 10. The perioperative period was uneventful.
At 6-month follow-up, the patient was doing well. 2D echocardiogram showed improved ventricular function (LVEF—50%), lesser than moderate degree MR (central jet) and normal coronary flow. Cardiac computed tomography showed normal coronaries without any obvious stenosis or narrowing (Fig. 5).
Fig. 5.
Follow-up computerized tomography angiogram (CTA) with 3D reconstruction. LAD, left anterior descending artery; LCX, left circumflex; RCA, right coronary artery
Discussion
Total anomalous origin of the coronary arteries from the pulmonary artery (TCAPA) is extremely rare and reported in a limited number of cases [3]. This can occur in isolation or may be associated with intracardiac structural abnormalities. They usually present early in life and carry a high mortality in the absence of surgical intervention. They manifest either as left heart failure or angina or the symptoms attributed to the underlying cardiac lesion. However, longer survival is seen in patients with additional associated cardiac lesions that increase the pulmonary artery pressure or oxygen saturation or both. Early detection and surgical intervention are associated with better survival.
Despite the excellent surgical outcomes in patients with anomalous origin of the coronary artery from the pulmonary artery in the current era, a proportion of them still require mechanical circulatory support (MCS) either before or after the surgery. Those with severe heart failure need preoperative MCS in the form of extracorporeal membrane oxygenation (ECMO) to allow for recovery of hibernating, stunned, or chronically ischemic myocardium. Postoperative temporary MCS may be required in those who fail to wean from cardiopulmonary bypass and this may take the form of ECMO or isolated left ventricular assist device (LVAD) with excellent left ventricular (LV) function recovery [4].
Data from Toronto group suggest that ventricular functional parameters (LVEF, LV dilation, and MR) usually recover within 1 year. The ejection fraction and degree of LV dilation usually improve by 4 months after the operation. Improvements in MR lag behind, but in most cases, the degree of MR should be mild or less in 7–8 months after the repair. If severe MR persists in the late post-operative period, then causes of recurrent myocardial ischemia should be investigated. [5]
Coronary artery anomalies are diverse group of congenital disorders with variable pathophysiological mechanisms and manifestations. High index of suspicion and appropriate management strategies are crucial for better outcome.
Acknowledgements
We would like to thank Dr Hung Liang Choo, Consultant Paediatrician from Hospital Kuala Lumpur, for the computerised tomography angiography images.
Funding
None.
Declarations
Ethics approval
Not applicable.
Human and animal rights statement
All procedures performed in this study involving human participants were in accordance with the ethical standards of the institutional research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. This article does not contain any studies with animals performed by any of the authors.
Consent
Obtained from the parents for publication.
Conflict of interest
The authors declare no competing interests.
Footnotes
Publisher's note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
References
- 1.Adachi I, Kagisaki K, Yagihara T, et al. Unroofing aortic intramural left coronary artery arising from right pulmonary artery. Ann Thorac Surg. 2008;85:675–677. doi: 10.1016/j.athoracsur.2007.06.085. [DOI] [PubMed] [Google Scholar]
- 2.Zhang H, Cheng P, Jin G, Han D, Luo Y, Li J. Surgical strategies for anomalous origin of the left coronary artery from the right pulmonary artery with an intramural aortic course: a report of 10 cases. J Thorac Cardiovasc Surg. 2017;153:648–653. doi: 10.1016/j.jtcvs.2016.10.063. [DOI] [PubMed] [Google Scholar]
- 3.Heifetz SA, Robinowitz M, Mueller KH, Virmani R. Total anomalous origin of the coronary arteries from the pulmonary artery. Pediatr Cardiol. 1986;7:11–18. doi: 10.1007/BF02315476. [DOI] [PubMed] [Google Scholar]
- 4.Imamura M, Dossey AM, Jaquiss RDB. Reoperation and mechanical circulatory support after repair of anomalous origin of the left coronary artery from the pulmonary artery: a twenty-year experience. Ann Thorac Surg. 2011;92:167–172. doi: 10.1016/j.athoracsur.2011.02.074. [DOI] [PubMed] [Google Scholar]
- 5.Azakie A, Russell JL, McCrindle BW, et al. Anatomic repair of anomalous left coronary artery from the pulmonary artery by aortic reimplantation: early survival, patterns of ventricular recovery and late outcome. Ann Thorac Surg. 2003;75:1535–1541. doi: 10.1016/S0003-4975(02)04822-1. [DOI] [PubMed] [Google Scholar]