Aktuelle Diagnostik und Therapie hypertrophischer Kardiomyopathien

Zusammenfassung

Die hypertrophische Kardiomyopathie (HCM) ist die häufigste vererbte Erkrankung des Herzens mit einer Prävalenz von 0.2 %. Ein Drittel der Patienten zeigen keine Obstruktion des linksventrikulären Ausflusstraktes, zwei Drittel weisen diesen in Ruhe und/oder unter Belastung auf. Angina pectoris, Dyspnoe, Synkopen, Symptome der Herzinsuffizienz, Vorhofflimmern und der plötzliche Herztod zeichnen die Erkrankung klinisch aus. Neben EKG und Echokardiographie sollten die Patienten mit Langzeit-EKG und Spiroergometrie regelmäßig nachverfolgt werden, um ungünstige klinische Verläufe zu antizipieren und Risikofaktoren zu identifizieren. Die kardiale MRT liefert komplementäre Informationen und unterstützt die Risikostratifizierung der Patienten im Hinblick auf eine ICD-Implantation. Grundpfeiler der konservativen Therapie stellen Betablocker dar, künftig sind neue pharmakologische Therapieansätze zu erwarten. Symptomatische Patienten mit Ausflußtraktobstruktion profitieren von einer Septum-ablativen Therapie wie der operativen Myektomie nach Morrow oder einer kathetergestützten Septumablation mittels Ethanolinjektion.

Abstract

Hypertrophic cardiomyopathy is the most common inherited disease of the heart with a prevalence of 0.2 %. One third of patients show no obstruction of the left ventricular outflow tract, whereas two third develop a significant gradient under resting conditions and/or on exertion. Angina pectoris, dyspnea, syncope, heart failure und atrial fibrillation are typical clinical features. Apart from ECG and echocardiography, holter ECG and exercise testing via ergospirometry should be routinely used to identify an adverse clinical course and potential risk factors. Cardiac MRI is a complementary and comprehensive imaging modality and further aids in risk stratification for sudden cardiac death and ICD-implantation for primary prophylaxis. Mainstay of conservative therapy are betablockers, and novel pharmacological approaches are to be expected in the near future. Symptomatic patients with outflow tract obstruction benefit from septum-reducting therapy either by surgical myectomy or catheter-based septum ablation (TASH/PTSMA) with ethanol injection.

Schlüsselwörter

hypertrophische Kardiomyopathie - Diagnostik - medikamentöse Therapie - interventionelle Therapie - Risikostratifizierung

Keywords

hypertrophic cardiomyopathy - diagnosis - pharmacological treatment - interventional treatment - risk stratification

• Literatur

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