Early repolarization syndrome can be identified by abnormalities on the electrocardiogram, described below, along with clinical signs such as unexplained syncope, ventricular fibrillation or sudden cardiac death.
The ECG depicts heartbeats as waves. Each heartbeat is divided into several phases and each one is assigned a letter: P, Q, R, S and T.
Early repolarization syndrome is characterized by an abnormality in the ST segment and J-point. The J-point is a small positive wave observed at the junction between the end of the QRS complex and the beginning of the ST segment. This wave is often pointed among individuals with this syndrome, which leads to a characteristic elevation in the ST segment.
What is an electrocardiogram?
It is a test that records the heart’s electrical activity over a given period of time. It identifies the speed at which the heart is beating (heart rate), whether it is beating normally (heart rhythm) and the effectiveness of the heart muscle. An electrocardiogram is also called an ECG or EKG.
Causes
Since the syndrome is relatively new, there is still much to learn about this rare disorder, both in terms of its genetics and pathophysiology. No definitive etiology is currently known.
Symptoms and diagnosis
As stated at the beginning of this article, the diagnosis of early repolarization syndrome is based on:
associated symptoms such as: suspected arrhythmic syncope, ventricular fibrillation, surviving sudden cardiac death, or a family history of sudden cardiac death.
An electrocardiographic appearance indicating J-point elevation, resulting in a characteristic elevation of the ST segment;
Treatments
Treatment varies according to symptoms associated with the syndrome:
- if the patient is asymptomatic: there is no specific treatment except if certain ECG abnormalities are present or if there is a family history of sudden cardiac death.
- If the patient has a history of suspected arrhythmic syncope: they must receive medical follow-up at a specialized medical center. Several solutions may be proposed depending on the characteristics of the syncope and appearance of the ECG: insertion of a cardiac monitor or defibrillator or, in some cases, administration of medication called quinidine.
- If the patient has ventricular fibrillation or has survived sudden cardiac death: they must be monitored at a specialized medical center. Several treatments may be proposed, such as implanting a defibrillator or taking medication. A family history assessment should also be performed.
There are no specific restrictions regarding physical and sports activities.
It is strongly recommended that first-degree relatives of patients with early repolarization syndrome be screened with an ECG.
Researchers are continuing this work to better understand the underlying mechanisms of this rare disease.
Reviewed and approved by Frédéric Sacher, Professor, MD, PhD, Cardiologist at Bordeaux University Hospital (CHU)
