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Hypertrophic cardiomyopathy as a cause of sudden death

Hypertrophe Kardiomyopathie als Ursache für den plötzlichen Herztod

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Abstract

Hypertrophic cardiomyopathy (HCM) is the main cause of sudden unexpected death in the young and especially in young athletes with an incidence up to 2.3 per 100,000 athletes and year. Risk stratification models including patient history (syncope, familial risk of sudden death) and findings in noninvasive tests (nonsustained ventricular tachycardia, abnormal blood pressure response during exercise, maximum left ventricular wall thickness ≥ 30 mm) have been developed in order to estimate the risk of individual patients. Echocardiographic parameters are helpful in distinguishing HCM from athlete’s heart. Definitive diagnosis of HCM implicates disqualification from competitive sports resulting in a significant reduction of sudden cardiac death due to HCM during sports competition. This positive development should lead to a widespread preparticipation screening of athletes including historical, clinical, and electrocardiographic examination. At least in borderline findings and symptomatic athletes, an additional echocardiogram should be performed in order to minimize or better exclude the risk of sudden cardiac death.

Zusammenfassung

Die hypertrophe Kardiomyopathie (HCM) ist die häufigste Todesursache junger Menschen und insbesondere Athleten mit einer jährlichen Inzidenz bis 2,3 pro 100 000 Athleten. Durch Risikostratifizierungsmodelle unter Einschluss anamnestischer Angaben (Synkope, familiär auftretende plötzliche Todesfälle) und nichtinvasiver Parameter (nichtanhaltende ventrikuläre Tachykardien, pathologische Belastungsblutdruckreaktion, maximale linksventrikuläre Wanddicke ≥ 30 mm) kann das individuelle Patientenrisiko abgeschätzt werden. Die Differentialdiagnose zum Sportherz ist in der Regel durch echokardiographische und weitere nichtinvasive Parameter möglich. Die gesicherte Diagnose einer HCM führt zum Ausschluss von wettkampfsportlichen Aktivitäten, wodurch das Auftreten plötzlicher Todesfälle bei sportlicher Aktivität gesenkt werden kann. Hieraus ergibt sich als logische Konsequenz die Notwendigkeit flächendeckender Vorsorgeuntersuchungen inklusive Anamnese, körperlicher Untersuchung und Ruhe-EKG. Zumindest in Grenzfällen sollte eine Echokardiographie durchgeführt werden, um das Auftreten eines plötzlichen Herztodes möglichst sicher verhindern zu können.

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Seggewiss, H., Blank, C., Pfeiffer, B. et al. Hypertrophic cardiomyopathy as a cause of sudden death. Herz 34, 305–314 (2009). https://doi.org/10.1007/s00059-009-3248-z

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