AHA/ACC: Consensus recommendations for young athletes with congenital heart disease

View on the News

Avoiding ‘one size fits all’

For many busy clinicians, societal guidelines, task force recommendations, expert consensus statements, and similar authoritative tomes are resources that are scarcely ever read carefully. This is likely not a reflection of the inherent value of such documents, but rather related to the observation that updated guidelines generally reflect, at most, a small change from predecessor versions. (It also should be mentioned that many such contributions are fairly heavy going for even the most determined reader.)

Occasionally, however, a new guideline may signal a dramatic shift in practice, and the recently published AHA/ACC Scientific Statement on Eligibility and Disqualification Recommendations for Competitive Athletes with Cardiovascular Abnormalities (Congenital Heart Disease) contains such a change.

Dr. Robert Jaquiss

In particular, the new recommendation suggests that athletes with anomalous aortic origin of the right coronary from the left coronary sinus, who have neither symptoms nor a positive stress test, may be allowed to participate in competitive athletics without undergoing surgical repair. As before, those with anomalous left coronary should not be allowed to participate until after surgical treatment.

Prior guidelines suggested that all patients, both anomalous left from right sinus and right from left sinus, be restricted prior to surgery. Because anomalous right coronary is five to six times more common than anomalous left coronary and because it is certainly much less ominous, the previous “one size fits all” approach almost certainly resulted in overtreatment, unnecessary restriction of participation, or both. Furthermore, because anomalous aortic of a coronary artery is so common, occurring in 0.1%-0.2% of the population (300,000 to 600,000 people in the United States), many thousands of competitive athletes will be impacted by the changed guidelines.

Most cardiologists, surgeons, and, most especially, patients will welcome the updated recommendations. Nonetheless, it must be emphasized that anomalous coronary arteries, even anomalous right coronary arteries, may indicate an increased risk of sudden death and that a complete assessment, including stress testing when feasible, and thorough discussion with expert clinicians is still absolutely necessary for such patients and their families.

Dr. Robert Jaquiss of Duke University, Durham, N.C., is the congenital heart section associate medical editor for Thoracic Surgery News.




Most children and young adult patients with congenital heart disease can and should engage in some form of physical activity and should avoid a sedentary lifestyle, according to a task force scientific statement from the American Heart Association and the American College of Cardiology (AHA/ACC).

This recommendation comes despite the fears of sudden cardiac death (SCD) in young athletes, which formed the initial impetus of the entire series of task force reports.

The recommended level of sports participation for patients with treated or untreated congenital heart defect, however, should consider the training and the competitive aspects of the sport itself and must be individualized to the patient. This means taking into account the patient’s current functional status, history of surgery, and the presence of implanted cardiac devices, according to the report by Dr. George F. Van Hare of Washington University, St. Louis, and his colleagues, which was published online in the Journal of the American College of Cardiology.

© Jody Dingle/

The report breaks down its specific recommendations based upon the various types of congenital heart defect (CHD). Full details and nuances of the recommendations and their specific levels of evidence for each individual condition and the many variants can be found in the online publication. Below is a brief and selected summary for some of the most common defects and some of those most pertinent to sudden cardiac death in young athletes.

Simple shunting lesions (atrial septal defect, ventricular septal defect, patent ductus arteriosus): Treated and untreated

In addressing the three most common subtypes of CHD – ventricular septal defect (VSD, 34%), atrial septal defect (ASD, 13%), and patent ductus arteriosus (PDA, 10%) – the committee found no data that children with these lesions are related to acknowledged episodes of sudden cardiac death (SCD). This applied whether the defects were closed or remained open. “With rare exceptions, patients with hemodynamically insignificant CHD such as VSD, ASD, and PDA may participate competitively in all sports,” it concluded. These recommendations fall under class I; level of evidence C for almost all of these patients, according to the writing committee.

Congenital coronary anomalies: Treated and untreated

Anomalies of coronary arteries are the second-most commonly identified structural causes of SCD in competitive athletes, accounting for about 17% of such deaths in the United States, according to the report. The vast majority of sudden deaths associated with coronary anomalies occur during or shortly after exercise. Despite being less commonly represented in patients, among athletes who have died suddenly, anomalous origin of the left main or left anterior descending coronary artery from the right sinus of Valsalva is far more prevalent. In addition, SCDs are most strongly associated with the pattern in which the anomalous left coronary artery passes between the aorta and main pulmonary artery. Recommended return to intense athletic activities is only to be permitted at least 3 months after surgery, and with a demonstration of the absence of ischemia on postoperative stress testing, with evidence levels depending on the type of anomaly. Of note, in contrast, the committee indicated that athletes with an anomalous origin of a right coronary artery from the left sinus of Valsalva should simply be evaluated by an exercise stress test, and for those without symptoms or a positive exercise stress test, permission to compete can be considered after adequate counseling (class IIa; level of evidence C).

Pulmonary valve stenosis: Treated and untreated

The committee determined that athletes with mild pulmonary stenosis (PS) and normal right ventricular (RV) function can participate in all competitive sports, although annual reevaluation also is recommended (class I; level of evidence B). In addition, athletes treated by operation or balloon valvuloplasty who have achieved adequate relief of PS (gradient less than 40 mm Hg by Doppler) can participate in all competitive sports (class I; level of evidence B). Other patients should be restricted to low-intensity sports, according to the committee.

Aortic valve stenosis: Treated and untreated

Children and adolescents with aortic stenosis (AS) are differentiated between those with mild, moderate, and severe AS by physical examination, ECG, and Doppler echocardiography. In all cases, regardless of the degree of stenosis, patients with a history of fatigue, light-headedness, dizziness, syncope, chest pain, or pallor on exercise deserve a full evaluation. Annual re-evaluation is required for all patients with AS because the disease can progress. Patients with severe AS are at risk of sudden death, particularly with exercise. The committee determined that athletes with mild AS can participate in all competitive sports (class I; level of evidence B), but that athletes with severe AS should be restricted from all competitive sports, with the possible exception of low-intensity sports (class III; level of evidence B).


Recommended Reading

Hypertension, blood pressure down among U.S. children, teens
MDedge Cardiology
Proglycem found to increase pulmonary hypertension risk in infants
MDedge Cardiology
E-cigarettes precede tobacco smoking in ninth-graders
MDedge Cardiology
Evidence of a respiratory virus does not exclude Kawasaki disease
MDedge Cardiology
Timely epinephrine for pediatric in-hospital cardiac arrest
MDedge Cardiology
Severity of obesity matters in pediatric population
MDedge Cardiology
AHA releases first-ever pediatric pulmonary hypertension guideline
MDedge Cardiology
Stem cell benefits endure 3 years in infants
MDedge Cardiology
Pediatric heart transplant results not improving
MDedge Cardiology
Experts call for international cooperation on human gene editing
MDedge Cardiology