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Risk Stratification in Electrical Cardiomyopathies

Risikostratifizierung bei elektrischen Kardiomyopathien

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Abstract

Electrical cardiomyopathies contain the long QT syndrome (LQTS), the short QT syndrome (SQTS), the Brugada syndrome, and the catecholaminergic polymorphic ventricular tachycardia (CPVT). Patients diagnosed with an electrical cardiomyopathy have an increased risk of syncope and sudden cardiac death (SCD). Usually, we are dealing with young patients or even children. The prevalence of these diseases is low. No large prospective randomized studies exist with respect to outcome based on different clinical and genetic parameters. Thus, risk stratification in these patients is based on retrospective data from single- or multicenter registries.

The implantable cardioverter defibrillator is the only reliable therapy in patients with Brugada syndrome and SQTS, as no pharmacological therapy has been proven to prevent SCD. In LQTS and CPVT, the primary therapy relies on β-blockers. In high-risk patients, the ICD is indicated.

In all electrical diseases, risk stratification is based on the clinical phenotype, including the electrocardiogram, the history of unexplained or disease-related syncope, and sudden cardiac arrest. In LQTS and CPVT, demographic data like age and gender are important factors for risk stratification. The genotype contributes to risk stratification only in LQTS and CPVT.

Patients with electrical cardiomyopathies have to be risk-stratified individually based on the data and the current guidelines available.

Zusammenfassung

Elektrische Kardiomyopathien umfassen das Long-QTSyndrom (LQTS), das Short-QT-Syndrom (SQTS), das Brugada-Syndrom und die katecholaminerge polymorphe ventrikuläre Tachykardie (CPVT). Patienten mit diesen Krankheitsbildern haben ein erhöhtes Risiko für Synkopen und den plötzlichen Herztod (SCD). Nach der Diagnose stellt die Risikostratifizierung bei den zumeist sehr jungen Patienten eine besondere Herausforderung dar. Aufgrund der Seltenheit der Erkrankungen und der zumeist nur relativ kurzen Nachbeobachtungszeiten stützt sich die Risikostratifizierung nicht auf große randomisierte Studien, sondern auf retrospektive Registerdaten.

Zur Verhinderung des SCD stellt der implantierbare Kardioverter-Defibrillator (ICD) bei Patienten mit Brugada-Syndrom und SQTS die einzig erwiesene Therapieoption dar. Beim LQTS und bei der CPVT besteht die Therapie primär aus einem β-Blocker. Bei Patienten mit hohem Risiko ist ebenfalls die ICD-Therapie indiziert.

Die Risikostratifizierung basiert bei allen Erkrankungen auf dem klinischen Phänotyp, bestehend aus dem Grad der elektrokardiographischen Manifestation, der Anamnese für Synkopen oder einen überlebten SCD und vereinzelt demographischen Daten wie Alter und Geschlecht. Bei der CPVT und dem LQTS trägt auch der Genotyp zur Risikostratifizierung bei.

Aufgrund des erhöhten Risikos eines SCD der häufig jungen Patienten ist eine individuelle Risikostratifizierung anhand der aktuellen Daten und Leitlinien wichtig.

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Authors and Affiliations

  1. Ist Department of Medicine – Cardiology, University Medicine Mannheim, Mannheim, Germany

    Christian Veltmann MD, Rainer Schimpf, Martin Borggrefe & Christian Wolpert

  2. I. Medizinische Klinik – Kardiologie, Universitätsmedizin Mannheim, Theodor-Kutzer-Ufer 1–3, 68167, Mannheim, Germany

    Christian Veltmann MD

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Veltmann, C., Schimpf, R., Borggrefe, M. et al. Risk Stratification in Electrical Cardiomyopathies. Herz 34, 518–527 (2009). https://doi.org/10.1007/s00059-009-3288-4

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