Unklare Synkopen und plötzlicher Herztod bei jungen Patienten

 

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Zusammenfassung

In Deutschland sterben pro Jahr mehr als 100 000 Menschen an einem plötzlichen Herztod. Bei einer Großzahl der verstorbenen Personen liegt ursächlich eine strukturelle Herzerkrankung, wie beispielsweise eine koronare Herzerkrankung oder eine dilatative Kardiomyopathie, vor. Etwa 5 bis 10 % der plötzlichen Herztodesfälle betreffen allerdings strukturell völlig herzgesunde Personen. Der Anteil jüngerer Menschen (< 40 Lebensjahre) beträgt in dieser Gruppe sogar 10 bis 20 %. Bei älteren Personen sind koronare Herzerkrankung und dilatative Kardiomyopathie für den überwiegenden Teil der plötzlichen Herztodesfälle verantwortlich. Bei jüngeren Patienten sind neben strukturellen Erkrankungen des Herzmuskels primär elektrische Erkrankungen des Herzens als Ursache des plötzlichen Todes als wesentliche Differenzialdiagnose in Betracht zu ziehen. Dazu gehören das Long-QT-Syndrom, das Short-QT-Syndrom, das Brugada-Syndrom und die katecholaminerge polymorphe ventrikuläre Tachykardie. Tritt in einer Familie ein plötzlicher Herztod eines jungen Menschen auf, kann durch eine detaillierte Familienanamnese inklusive EKG, Ergometrie und molekulargenetischer Diagnostik in ca. 40 % eine Ursache gefunden werden. Durch diese Maßnahmen können auch weitere, potenziell bedrohte Familienmitglieder identifiziert werden und durch eine entsprechende Therapie vor einem arrhythmogenen Ereignis geschützt werden. Häufig werden die Erkrankungen nicht erkannt, da sie bis zum Zeitpunkt des tödlichen Ereignisses nicht selten ohne Symptome verlaufen oder Frühsymptome wie Synkopen als Prodromi verkannt werden. Das konventionelle EKG gewinnt durch die Beschreibung des neuen Krankheitsbildes Short-QT-Syndrom in der Differenzialdiagnose an Bedeutung.

Summary

In Germany more than 100 000 persons die each year from sudden cardiac death. In most of them it is caused by structural heart disease, such as coronary heart disease or dilated cardiomyopathy. However, about 5-10% cases of sudden death occur in persons with structurally completely normal hearts. Young persons (under 40 years of age) make up 10-20% of this group, while in older persons coronary heart disease and dilated cardiomyopathy are by far the most common cause of sudden death. In young persons primary electrical diseases should also be included in the differential diagnosis of the cause of sudden death. These abnormalities include both long and short QT syndromes, the Brugada syndrome and catecholaminergic polymorphic ventricular tachycardia. If sudden death has occurred in a young person, a detailed history and investigation of family members, including an electrocardiogram (ECG), ergometry and molecular diagnosis, will reveal the underlying cause in approximately 40% of cases. The measures may also identify other family members who are potentially at risk: appropriate treatment may protect them from arrhythmogenic events. The underlying abnormality will often not have been recognized, because quite commonly there will have been no symptoms before the sudden death or early symptoms, such as syncope as prodromal event, may have been misinterpreted. Conventional electrocardiography has gained importance in differential diagnosis since the description of a new disease entity, the short QT syndrome.

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Dr. med. Rainer Schimpf

I. Medizinische Klinik, Universitätsklinikum Mannheim

Theodor-Kutzer-Ufer 1-3

68167 Mannheim

Phone: ++49/621/3832206

Fax: ++49/621/3833061

Email: rainer.schimpf@med.ma.uni-heidelberg.de