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Klinik und Therapie der hypertrophen Kardiomyopathie

Clinical appearance and treatment of hypertrophic obstructive cardiomyopathy

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Zusammenfassung

□ Bei der hypertrophen Kardiomyopathie, die definiert ist als eine primäre, zum Teil familiär auftretende und genetisch fixierte myokardiale Hypertonie, unterscheidet man zwischen der nichtobstruktiven und obstruktiven (HOCM) Form. Bei der HOCM findet sich eine dynamische Ausflußbahnobstruktion des linken, selten auch des rechten Ventrikels. Bei jüngeren Patienten ist sie verbunden mit dem gehäuften Auftreten eines plötzlichen Herztodes. Trotz Identifikation zahlreicher Riskofaktoren ist eine individuelle Prognosebestimmung nicht sicher möglich.

□ In der symptomatischen Behandlung sind die medikamentöse Therapie (Betablocker, Calciumantagonisten vom Verapamil-Typ) und die operative Myotomie/Myektomie ergänzt worden durch Einführung der DDD-Schrittmacherimplantation und insbesondere der alkoholinduzierten perkutanen transluminalen septalen Myokardablation (PTSMA). Durch die PTSMA kann bei über 90% der behandelten Patienten eine Reduktion der linksventrikulären Ausflußbahngradienten erreicht werden, die, bedingt durch das Remodeling nach induzierter umschriebener Myokardnekrose, im weiteren Verlauf weiter zunimmt. Die bedeutsamste Komplikation ist die Induktion eines trifaszikulären Blocks mit Notwendigkeit einer permanenten Schrittmacherimplantation, die aber durch Optimierung des abladierten Septumareals nach Einführung des echokardiographischen Monitorings reduziert werden konnte bei gleichzeitiger Verbesserung der Akut- und Verlaufsergebnisse. Langfristige Beobachtungen und Vergleiche mit etablierten Therapieverfahren müssen nachweisen, ob neben der symptomatischen Verbesserung auch die Prognose der Patienten günstig beeinflußt werden kann.

Summary

□ Hypertrophic cardiomyopathy is defined as a primary, sometimes familial and genetically fixed myocardial hypertrophy. In the obstructive form of the disease (HOCM) a dynamic outflow tract obstruction of the left, occasionally also the right ventricle can be found. HOCM is the most frequent cause of stress-induced syncope or sudden cardiac death in younger patients. An individual estimation of prognosis is difficult although several risk factors have been identified.

□ In addition to standard therapy of symptomatic patients (medical treatment with betablockers and calcium-antagonists of verapamil-type as well as surgical myotomy/myectomy) DDD-pacemaker implantation and percutaneous transluminal septal myocardial ablation (PTSMA) by alcohol-induced septal branch occlusion have been introduced. After PTSMA significant outflow tract gradient reduction can be achieved in > 90% of patients. Due to remodeling after circumscribed myocardial necrosis further gradient reduction has been observed during follow-up. Optimization of ablated septal area by echocardiographic monitoring resulted in reduction of the most important complication, (trifascicular block with need of permanent pacemaker implantation) and improvement of acute and follow-up results. Long-term follow-up and comparison with established treatment options are necessary to evaluate the definitive importance of the promising new treatment.

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Authors and Affiliations

  1. Kardiologische Klinik, Herz- und Diabeteszentrum NRW, Universitätsklinik der Ruhr-Universität Bochum, Georgstraße 11, D-32545, Bad Oeynhausen

    Ulrich Gleichmann & Hubert Seggewiß

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  1. Ulrich Gleichmann
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  2. Hubert Seggewiß
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Correspondence to Ulrich Gleichmann.

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Gleichmann, U., Seggewiß, H. Klinik und Therapie der hypertrophen Kardiomyopathie. Med Klin 93, 260–267 (1998). https://doi.org/10.1007/BF03044802

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