Patient Presentation
A 10-year-old female came to clinic for her health maintenance visit. There were no concerns and she was playing youth soccer and softball. The past medical history showed a history of Kawasaki disease as a 7 month old, and had been treated with intravenous gamma globulin and aspirin. Her initial and followup echocardiogram were normal.

The pertinent physical exam revealed growth parameters in the 10-25% and normal vital signs. Her examination was normal including her cardiac exam.

The diagnosis of a healthy female was made. The pediatrician knew that the natural history of Kawasaki disease was that if the echocardiogram was negative at followup that the patients did well and generally did not need lots of long-term followup. He pondered that it was funny how different coronary artery disease was in children versus adults; almost like it was two different diseases. “I really haven’t thought about this much and if I have some time at lunch, I think I’ll look this up,” he thought to himself.

Discussion
Coronary artery disease in children and young adults is overall rare fortunately. It can be symptomatic with chest pain, fatigue, palpitations or syncope, or can be asymptomatic. In children it is hard to image the coronary arteries through an echocardiogram. Arteries are small and superficial while the corresponding veins are larger. Children often move and therefore need sedation to even accomplish the procedure. Because of the potential risk for sudden death there has been much discussion about using screening electrocardiograms and echocardiograms for athletes with arguments for identifying potential disease, but also the cost and risk of false positives.

Learning Point
Common causes of coronary artery disease include:

• Aberrant coronary arteries – overall rare incidence of 0.6-1.3%
  • Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) – most common
  • Aberrant origin of the coronary artery from incorrect sinus of Valsalva
  • Single coronary artery
• Coronary artery fistula – generally rare and asymptomatic but can cause heart failure
• Kawasaki disease
  • Causes ectasias (arterial dilatation without vessel lumen irregularity) and aneurysms most commonly at the left anterior descending but other locations as well
• Multisystem inflammatory syndrome in children (MIS-C) due to COVID-19
• Ischemia
• Iatrogenic from congenital heart surgery

Although coronary artery bypass surgery is overall rare, it has become more common as part of its role in treatment for congenital heart disease.
One review found the five main indications for bypass surgery were:

• ALCAPA
• Left main coronary trunk atresia
• Transposition of the great arteries, acute and late coronary events
• Ross operation for congenital aortic stenosis especially when complicated by infective endocarditis
• Inadvertent acute injury during surgery

One study reviewing sudden cardiac death in individuals < 35 years of age, found that coronary artery disease of some type was relatively common.

Problem	Athlete	Non-Athlete	United States	Europe
Anomalous origin of coronary artery	9.7%	4.4%	14%	5.5%
Ischemic heart disease	9.1%	19.1%	8.7%	12%

Questions for Further Discussion
1. What are the recommendations for electrocardiograms or echocardiograms for athletes in your location?
2. Can Kawasaki disease recur? A review can be found here
3. How common is syncope? A review can be found here
4. What patient education and screening tests are recommended to prevent adult coronary artery disease?

Related Cases

Disease: Coronary Disease

Symptom/Presentation: Health Maintenance and Disease Prevention

Specialty: Cardiology / Cardiovascular-Thoracic Surgery

Age:School Ager

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for this topic: Coronary Artery Disease

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Brown LM, Duffy CE, Mitchell C, Young L. A Practical Guide to Pediatric Coronary Artery Imaging with Echocardiography. Journal of the American Society of Echocardiography. 2015;28(4):379-391. doi:10.1016/j.echo.2015.01.008

Kitamura S. Pediatric Coronary Artery Bypass Surgery for Congenital Heart Disease. The Annals of Thoracic Surgery. 2018;106(5):1570-1577. doi:10.1016/j.athoracsur.2018.04.085

D’Ascenzi F, Valentini F, Pistoresi S, et al. Causes of sudden cardiac death in young athletes and non-athletes: systematic review and meta-analysis. Trends in Cardiovascular Medicine. 2022;32(5):299-308. doi:10.1016/j.tcm.2021.06.001

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa