Lately, news of the sudden death of a young child or young athlete often comes to the public. Fortunately, it is a rare phenomenon, due to specific underlying cardiac problems, “insidious”, because they usually remain asymptomatic until the fatal moment.

The European Society of Arrhythmology has estimated that 1–2/100,000 athletes between the ages of 12 and 35 die suddenly each year.

The two most frequent causes of these tragic events are:

1) Myocardiopathies and primary arrhythmogenic diseases.
2) Congenital anomalies of the coronary arteries.

Both causes can be diagnosed with a thorough (not just standard) pre-sport workup, including a detailed medical and family history, physical examination, electrocardiogram, and echocardiogram.

Further diagnosis and management of cardiomyopathies and primary arrhythmogenic lesions is the subject of Clinics specialized in Heart Failure and Electrophysiology, and may include preventive defibrillator implantation. This requires surgery in young children.

The role of pediatric cardiac surgery is crucial in the treatment of the second most common cause of sudden death in children and young people, which is various congenital anomalies of the coronary arteries. These unfortunately often remain undiagnosed for years since they are usually asymptomatic or their symptoms are misinterpreted.

While asymptomatic anomalous origin of the left (or right) coronary artery from the pulmonary artery (ALCAPA or ARCAPA) is sometimes found, these lesions usually create severe symptoms, are diagnosed in infancy and then surgically corrected with excellent results.

The most common coronary anomalies that can lead to sudden death in young adults concern the anomalous aortic origin of the left or right coronary artery from the opposite aortic sinus of Valsalva (Aanomalous Aortic Origin of Coronary Artery – AAOCA). The course of the coronary artery is also abnormal, since it passes between the large vessels, namely the aorta and the pulmonary artery. There, the abnormal coronary artery can be compressed during exercise, when the blood pressure in the large vessels is increased, resulting in the creation of ischemia, which in turn can cause fatal arrhythmia, i.e. cardiac arrest.

AAOCA’s cardiac surgical treatment.

Some subtypes of AAOCA, mainly the asymptomatic anomalous outflow of the right coronary artery from the left sinus of Valsalva (AAORCA) are considered low risk, and, on negative ischemia tests, do not necessarily need surgical treatment, but certainly restriction of vigorous sports activities.

However, abnormal outflow of the left coronary artery from the right sinus of Valsalva (AAOLCA), as well as all cases where there are symptoms or laboratory evidence of ischemia are considered dangerous. In these cases, all sporting activity must be stopped and cardiac surgical correction performed by specialized pediatric cardiac surgeons. There are various surgical correction techniques that differ depending on extremely important details of each patient’s anatomy. In many cases, the indications and the most appropriate surgical technique indicated are the subject of ongoing medical research.

Due to the rarity of these diseases, the most valid relevant studies are products of multicenter collaborations, from the American Congenital Heart Surgeons Society (CHSS), as well as the European Congenital Heart Surgeons Association (ECHSA). In fact, our ECHSA paper was honored as a keynote paper at the recent annual European Association for Cardiothoracic Surgery (EACTS) Conference in Vienna. The study proves that detailed anatomical diagnosis and correct surgical management are accompanied by excellent results, and patients can return to normal activities.