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Sudden cardiac death in an adolescent with unusual coronary artery anomaly

Published online by Cambridge University Press:  28 April 2025

M. Holtrup ,
L. Hernandez Open the ORCID record for L. Hernandez [Opens in a new window]  and
M. Diamond Open the ORCID record for M. Diamond [Opens in a new window]
M. Holtrup
Affiliation:
Joe DiMaggio Children’s Hospital, Hollywood, FL, USA
L. Hernandez
Affiliation:
Joe DiMaggio Children’s Hospital, Hollywood, FL, USA
M. Diamond*
Affiliation:
Joe DiMaggio Children’s Hospital, Hollywood, FL, USA
*
Corresponding author: Melissa Diamond; Email: mdiamond@mhs.net
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Abstract

A case of an adolescent male with hypoplastic coronary arteries and myocardial bridging who had a sudden cardiac death event playing soccer. This rare anomaly is not easily identified and may be missed during routine work-up. Treatment options are limited and there is a need for discussion of additional treatment considerations that may prevent sudden cardiac death in this population.

Keywords

Hypoplastic coronary arteriesmyocardial bridgingsudden cardiac death

Information

Type
Brief Report
Information
Cardiology in the Young , Volume 35 , Issue 5 , May 2025 , pp. 1051 - 1053
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Copyright
© The Author(s), 2025. Published by Cambridge University Press

Introduction

Paediatric and adolescent patients have an overall annual risk of 1 in 100,000 of dying from sudden cardiac arrest.Reference Al-Khatib, Stevenson and Ackerman1 While ischaemic heart disease is the most common cause of sudden cardiac death in adults, inherited cardiac diseases such as cardiomyopathies and channelopathies, myocarditis, and CHD play an important role in children and adolescents.Reference Al-Khatib, Stevenson and Ackerman1 The relevance of coronary artery anomalies in sudden cardiac death is not completely understood. The clinical presentation can vary widely, from a lack of symptoms to acute, even life-threatening symptoms.Reference Angelini2 Although a coronary artery anomaly may be suspected in a young individual with ischaemia-like symptoms, they are often incidental findings discovered during routine cardiac workup.Reference Gentile, Castiglione and De Caterina3 This case demonstrates a rare combination of coronary artery anomalies consistent with myocardial bridging and diffuse coronary artery hypoplasia leading to an adverse event.

Case report

This 12-year-old Hispanic boy presented to our hospital in March 2023 with chest pain and loss of consciousness after running to class. He had a history of syncope in 2019, where he underwent complete evaluation revealing hypoplastic coronary arteries and myocardial bridging. Treatment with beta-blocker and antiplatelet therapy were initiated. On admission, electrocardiogram demonstrated ST segment depression with elevated troponin I. Initial echocardiogram demonstrated normal coronary artery origin with no evidence of dilatation, aneurysm or ectasia and normal biventricular function with no segmental wall motion abnormalities. Coronary CT confirmed the findings from prior assessment. Regadenoson stress perfusion cardiac MRI showed a large area of perfusion defect at the interventricular septum at rest (Figure 1g) and during hyperaemia (Figure 1h) consistent with a fixed coronary artery obstruction. There was septal mid-myocardial late gadolinium enhancement consistent with myocardial replacement fibrosis (Figure 1i). Cardiac catheterisation showed a truncated right coronary artery with distal atresia (Figure 1d and 1e) and a large intramyocardial segment of the left anterior descending coronary artery (Figure 1f). The patient’s treatment plan included beta-blocker. He was advised to avoid physical exertion. A loop recorder was implanted (Figure 2a). During a one-year follow-up period, the patient did not experience any further syncopal events, palpitations, or chest pain. The loop recordings showed no significant findings. Shortly after the one year follow-up, while playing soccer, the patient experienced a sudden cardiac arrest with unsuccessful resuscitation attempts on the field and in the paediatric emergency room. Post-mortem analysis of the loop recorder revealed episodes of acute myocardial ischaemia leading to bradycardia and fatal ventricular arrhythmia (Figure 2b).

Figure 1.

CT scan showing significant hypoplasia of the right coronary artery (a; b) and a large intramyocardial segment of the left anterior descending coronary artery (c). Cardiac catheterization showing diffuse hypoplasia of the distal coronary artery tree (d; e; f). Stress perfusion CMR showing a large area of perfusion defect at the interventricular septum at rest (g) and under the effect of regadenoson (h). Septal mid-myocardial late gadolinium enhancement consistent with scar (i).

Figure 2.

Loop recorder pre-event with sinus rhythm and no evidence of ST elevation (a) and at the time of cardiac event with significant ST elevation (b).

Discussion

Coronary artery anomalies can manifest in various ways and have different outcomes.Reference Gentile, Castiglione and De Caterina3 Symptoms such as exertion-related ischaemia often trigger further clinical investigation. However, these anomalies are more commonly discovered incidentally.Reference Gentile, Castiglione and De Caterina3 Some anomalies have clear clinical significance, while others are more challenging to diagnose.Reference Gentile, Castiglione and De Caterina3 Evidence linking coronary artery anomalies to sudden cardiac death often comes from studies of young athletes, where maximum exertion plays a crucial role.Reference Finocchiaro, Westaby, Sheppard, Papadakis and Sharma4 Myocardial bridging is a congenital anatomic anomaly in which a segment of the artery tunnels beneath a section of the myocardium.Reference Tarantini, Migliore, Cademartiri, Fraccaro and Iliceto5 The depth and length of the tunnelled segment significantly contribute to the development of ischaemic symptoms.Reference Tarantini, Migliore, Cademartiri, Fraccaro and Iliceto5 Myocardial bridging has a dynamic effect that varies with the cardiac cycle, heart rate, and sympathetic tone.Reference Tarantini, Migliore, Cademartiri, Fraccaro and Iliceto5 Consequently, it remains a considerable diagnostic challenge to determine when symptoms are related to myocardial bridging, particularly when diagnosed in childhood. Experience and studies are limited to case reports and small case series.Reference Kiess, Vollroth and Bakhtiary6,Reference Sharma, Hellenbrand, Kleinman and Mosca7 Hypoplastic coronary artery disease, on the other hand, is a rare congenital malformation where one or more major coronary arteries are underdeveloped.Reference Zugibe, Zugibe, Costello and Breithaupt8 A luminal diameter of 1.5 mm has been suggested as a criterion for diagnosis.Reference Zugibe, Zugibe, Costello and Breithaupt8 Sudden cardiac death is a common presentation, and diagnosis is often made postmortem.Reference Zugibe, Zugibe, Costello and Breithaupt8 In our case, the findings were only identified on advanced cardiac imaging modalities (coronary CT and MRI). Treatment strategies vary from lifestyle changes to medical therapy to cardiac catheterisation and surgical intervention.Reference Tarantini, Migliore, Cademartiri, Fraccaro and Iliceto5 Medical therapy, such as beta blockers and calcium channel blockers, are aimed at reducing triggers that worsen myocardial ischaemia.Reference Gentile, Castiglione and De Caterina3 Among the surgical and interventional options, coronary artery bypass and percutaneous coronary intervention have been proposed for the treatment of hypoplastic coronary arteries as well as myocardial bridging.Reference Tarantini, Migliore, Cademartiri, Fraccaro and Iliceto5 Other surgical options include myotomy, where the overlying myocardium is resected (also known as “unroofing”).Reference Tarantini, Migliore, Cademartiri, Fraccaro and Iliceto5 While patient autografts remain the gold standard for vascular transplantation, recent research in molecular and cellular cardiology has directed attention toward in situ tissue engineering, which may offer a potential treatment option in the future.Reference Wei, Wang, Guo and Zhao10 Furthermore, advancements in 3 D printing techniques have opened up new possibilities for creating patient-specific vascular grafts.Reference Wei, Wang, Guo and Zhao10 Lifestyle changes, including the restriction of physical exertion, are advised in this patient population.Reference Pelliccia, Sharma and Gati9 Eligibility to participate in competitive sports must be carefully evaluated.Reference Pelliccia, Sharma and Gati9 Due to the extent of coronary artery hypoplasia, a surgical anastomosis was discussed with the surgical team but ultimately not pursued. The degree of myocardial bridging raised concerns about the feasibility of completely resecting the deep bridges and the overall success of the procedure, given the high risk of ventricular perforation. Additionally, the target vessels were too small to be considered for coronary artery stenting. Therefore, medical treatment in combination with the restriction of exertion were the only treatment options. ICD implantation has been suggested for the secondary prevention of sudden cardiac death when myocardial ischaemia is the underlying cause of ventricular arrhythmia and revascularisation is not possible.Reference Al-Khatib, Stevenson and Ackerman1 The American Heart Association Guideline for the management of patients with ventricular arrhythmia refers to anomalies of the coronary origin, but there is no clear recommendation for coronary artery disease consistent with hypoplastic coronary artery anomalies and myocardial bridging.Reference Al-Khatib, Stevenson and Ackerman1

Conclusion

The unfortunate outcome of our patient once again demonstrates the clinical significance that coronary artery anomalies may have. This rare combination of coronary artery hypoplasia and myocardial bridging is not easily identified and requires advanced imaging for diagnosis, as it can be missed during standard cardiac workup. The lack of effective intervention left treatment options limited. This leads to the discussion of additional treatment considerations that may prevent sudden cardiac death in this young population, who are difficult to restrict from physical activity.

Financial support

None.

Competing interests

None.

Ethical standard

Informed consent was obtained from the patient’s guardians.

References

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Figure 0

Figure 1. CT scan showing significant hypoplasia of the right coronary artery (a; b) and a large intramyocardial segment of the left anterior descending coronary artery (c). Cardiac catheterization showing diffuse hypoplasia of the distal coronary artery tree (d; e; f). Stress perfusion CMR showing a large area of perfusion defect at the interventricular septum at rest (g) and under the effect of regadenoson (h). Septal mid-myocardial late gadolinium enhancement consistent with scar (i).

Figure 1

Figure 2. Loop recorder pre-event with sinus rhythm and no evidence of ST elevation (a) and at the time of cardiac event with significant ST elevation (b).