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Aktuelle Kardiologie 2012; 1(04/05): 268-275
DOI: 10.1055/s-0032-1324819
Übersichtsarbeit
Georg Thieme Verlag KG Stuttgart · New York

Hypertrophische Kardiomyopathie: Diagnostik und Therapie

Hypertrophic Cardiomyopathy: Diagnosis and Treatment
M. Luedde
1   Innere Medizin III, Kardiologie und Angiologie, Universitätsklinikum Schleswig Holstein, Campus Kiel
,
T. Klein
1   Innere Medizin III, Kardiologie und Angiologie, Universitätsklinikum Schleswig Holstein, Campus Kiel
,
T. Demming
1   Innere Medizin III, Kardiologie und Angiologie, Universitätsklinikum Schleswig Holstein, Campus Kiel
,
N. Frey
1   Innere Medizin III, Kardiologie und Angiologie, Universitätsklinikum Schleswig Holstein, Campus Kiel
› Institutsangaben
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Publikationsverlauf

Publikationsdatum:
16. Oktober 2012 (online)

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Zusammenfassung

Die hypertrophische Kardiomyopathie (HCM) ist mit einer Prävalenz von 1 : 500 die häufigste kardiale Erbrankheit. Bei ca. 30 % der Patienten liegt die nonobstruktive Form (HNCM) vor, bei ca. 70 % ist ein Gradient im Ausflusstrakt des linken Ventrikels nachweisbar, zum Teil nur unter körperlicher Belastung. Klinisch ist die Erkrankung gekennzeichnet durch Herzinsuffizienzsymptomatik, Angina pectoris sowie ein erhöhtes Risiko für plötzlichen Herztod. Entscheidend zur bestmöglichen Therapie von HCM-Patienten ist eine optimale Diagnostik, neue Untersuchungsmethoden können die altbewährten Methoden EKG und Echo sinnvoll ergänzen. Basis der Therapie sind Betablocker. HOCM-Patienten profitieren von einer operativen Myektomie nach Morrow oder einer kathetergestützter Alkoholablation des Septums. Neue Therapieoptionen werden erprobt und könnten in der Zukunft ermöglichen, Symptomatik und Prognose der betroffenen Patienten zu verbessern.

Abstract

With a prevalence of 1 : 500, hypertrophic cardiomyopathy (HCM) is the most common hereditary cardiac disease. While 30 % of HCM patients suffer from a nonobstructive form (HNCM), a larger part develops a significant pressure gradient of the left ventricular outflow tract (LVOT) at rest or due to physical stress. The disease is characterized by heart failure, angina pectoris and potentially fatal cardiac arrhythmias. To achieve optimal medical treatment, a refined diagnostic approach is needed. Novel diagnostic tools like cardiac magnetic resonance tomography can augment established methods such as ECG and echocardiography. Beta-blockers are first-line medical treatment of all symptomatic patients. Surgical and catheter-based treatment of the LVOT gradient are recommended by international guidelines, with no apparent difference in procedure-related benefits or risk. Novel specific treatment approaches are currently under investigation which may further improve symptoms and prognosis of affected patients.

Schlüsselwörter

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

Key words

hypertrophic cardiomyopathy - diagnosis - drug therapy - interventional therapy - risk stratification
 

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