Hypertrophe obstruktive Kardiomyopathie – aktueller Stand in Diagnostik und Therapie

Zusammenfassung

Die hypertrophe Kardiomyopathie ist die häufigste genetisch bedingte kardiale Erkrankung. Wesentliches pathophysiologisches Zeichen bei ca. 70 % der betroffenen Patienten ist eine dynamische (Ausflussbahn-)Obstruktion des linken Ventrikels. Der klinische Verlauf wird bestimmt durch das erhöhte Risiko eines plötzlichen Herztods insbesondere bei jüngeren Patienten sowie eine häufig variable kardiale Symptomatik (Angina pectoris, Dyspnoe, Synkope). Die diagnostischen Maßnahmen sollten unter den Gesichtspunkten einer möglichen durchzuführenden prognostischen – mit Implantation eines ICD bei erhöhtem Risiko eines plötzlichen Herztods – und symptomatischen Therapie durchgeführt werden. Für die symptomatische Therapie stehen in Abhängigkeit von der zugrunde liegenden Morphologie in erster Linie die perkutane Septumablation und die operative Myektomie zur Verfügung.

Abstract

Hypertrophic cardiomyopathy is the most common genetically induced cardiac disease. An important pathophysiological sign in about 70 % of the affected patients is the dynamic left ventricular (outflow) obstruction. The clinical course is characterised by the high risk of sudden cardiac death, especially for young patients, as well as usually variable cardiac symptoms (angina, dyspnoea, syncope). The diagnostic assessment should be made in view of a possible prognostic – with implantation of an ICD due to high risk of sudden cardiac death – and symptomatic management. For the symptomatic treatment of hypertrophic obstructive cardiomyopathy the available options according to the underlying morphology are the percutaneous transluminal alcohol septal ablation (PTSMA) and the surgical myectomy.

Schlüsselwörter

hypertrophe Kardiomyopathie - Obstruktion - perkutane Septumablation - Myektomie - plötzlicher Herztod

Key words

hypertrophic cardiomyopathy - obstruction - percutaneous septal ablation - myectomy - sudden death

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