Plötzlicher Herztod bei Jugendlichen und Sportlern

Abstract

Sudden cardiac death (SCD) (as defined as death within one hour after onset of symptoms) occurs with an incidence of nearly 100 000 cases per year in Germany. In the elderly acute or previous myocardial infarction or (less common) dilated cardiomyopathy are the main reasons. In persons younger than 35 years the following causes are most often found: hypertrophic cardiomyopathy, anomalous origin of the coronary arteries, myocarditis, arrhythmogenic right ventricular cardiomyopathy and primary electrical diseases, e. g., long (or short) QT syndrome, Brugada syndrome, WPW syndrome, catecholaminergic polymorphic ventricular tachycardia and probably early repolarization with idiopathic ventricular fibrillation. In one third even after autopsy no abnormalities are evident after SCD. Post mortem genetic testing allows the diagnosis of ion channel disease in a substantial number of cases. Unfortunately no risk stratification of SCD is currently possible by genetic screening methods in persons with known structural or primary electrical heart diseases.

Kernaussagen

• Die hypertrophe Kardiomyopathie, Koronaranomalien und eine Myokarditis sind die häufigsten Ursachen des plötzlichen Herztodes bei Jugendlichen und Sportlern.

• Unter primär elektrischen Herzerkrankungen subsumiert man die Long- (und Short-)QT-Syndrome, das Brugada-Syndrom, das WPW-Syndrom, die katecholaminerge polymorphe ventrikuläre Tachykardie und die frühzeitige Repolarisation bei idiopathischem Kammerflimmern.

• Das Ruhe-EKG vermag nach überlebtem plötzlichem Herztod wertvolle Hinweise zu geben. Weiterführende bildgebende Verfahren (Herzechokardiografie, Kardio-MRT) sind in vielen Fällen diagnostisch (hypertrophe Kardiomyopathie, Koronaranomalien, Myokarditis).

Molekularbiologische Untersuchungsverfahren erlauben auch post mortem eine Diagnose genetisch bedingter Ionenkanalerkrankungen (z. B. LQT-Syndrom). Intra vitam ist eine Risikoeinschätzung mittels molekularbiologischer Methoden derzeit nicht möglich.

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