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[Syncopes and channelopathies]
Johanna Müller-Leisse1, Christos Zormpas1, Thorben König1
1Rhythmologie und Elektrophysiologie, Klinik für Kardiologie und Angiologie, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland.
Insights
Syncope may signal rare cardiac channelopathies like Brugada syndrome. Recognizing typical symptoms and ECG patterns in young patients is crucial for diagnosing these conditions and preventing sudden cardiac death.
Area of Science:
- Cardiology
- Genetics
- Electrophysiology
Background:
- Syncope is a common symptom, but can be the initial presentation of life-threatening cardiac channelopathies.
- Cardiac channelopathies, including Brugada syndrome, long QT syndrome, short QT syndrome, and catecholaminergic polymorphic ventricular tachycardia (CPVT), increase the risk of sudden cardiac death (SCD).
Purpose of the Study:
- To review diagnostic findings suggestive of channelopathies in patients presenting with syncope.
- To discuss the prognostic and therapeutic implications of syncope in individuals with diagnosed channelopathies.
Main Methods:
- Literature review focusing on clinical presentation, diagnostic criteria, and management of cardiac channelopathies.
- Analysis of typical medical history elements and electrocardiogram (ECG) patterns associated with channelopathies.
Main Results:
- Syncope in young patients with structurally normal hearts, typical arrhythmogenic history, characteristic ECG, or family history warrants channelopathy investigation.
- Syncope in diagnosed channelopathy patients can indicate increased SCD risk, though predictive value varies by specific disease.
Conclusions:
- Early consideration of channelopathies in unexplained syncope, particularly in specific patient groups, is vital.
- Understanding syncope's role in diagnosed channelopathies is essential for risk stratification and tailored treatment strategies.
Abstract:
Syncope can be the first manifestation of cardiac channelopathies, namely Brugada syndrome, long QT syndrome, short QT syndrome and catecholaminergic polymorphic ventricular tachycardia (CPVT). Patients affected by these rare diseases are at increased risk for sudden cardiac death due to ventricular tachyarrhythmias and require specific therapy and follow-up. As syncope is common in the general population, only few cases are caused by an underlying channelopathy. Nevertheless, the diagnosis should be considered in young patients with structurally normal hearts, especially if the history of syncope is typical for an arrhythmogenic cause, in the presence of characteristic echocardiogram (ECG) patterns, and if there is a family history of channelopathies or sudden cardiac death. On the other hand, syncope plays an important role in the management of patients with diagnosed channelopathies, as they may indicate an increased risk for sudden cardiac death. The predictive value and consequences for treatment vary between the different channelopathies. However, data on this issue are scarce due to the low prevalence of these diseases. This review highlights typical findings in the medical history and diagnostic tests that may point towards an underlying channelopathy in patients with syncope. It also discusses the prognostic and therapeutic implications of a history of syncope in patients with known channelopathies.
