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[Therapy and risk-stratification in hypertrophic cardiomyopathy--a current survey]
1Medizinische Klinik I, Klinikum Krefeld, Lutherplatz 40, 47805 Krefeld, Germany. sereith@gmx.de
Insights
Hypertrophic cardiomyopathy (HCM) treatments vary, with myectomy as the gold standard. New options like TASH offer alternatives, but long-term data is needed to assess their impact on HCM
Area of Science:
- Cardiology
- Genetics
- Cardiac Surgery
Context:
- Hypertrophic cardiomyopathy (HCM) is a common genetic heart disease with diverse clinical presentations.
- Severe symptomatic obstructive HCM has limited treatment options beyond standard drugs.
- Myectomy is the established surgical treatment, but newer interventions are emerging.
Purpose:
- To review current and emerging therapeutic strategies for obstructive hypertrophic cardiomyopathy (HCM).
- To compare the efficacy and long-term outcomes of myectomy, dual-chamber pacing, and transcoronary ablation of septal hypertrophy (TASH).
- To emphasize the importance of risk stratification for sudden cardiac death in HCM patients.
Summary:
- Myectomy remains the gold standard for severe obstructive HCM, though TASH and dual-chamber pacing are viable alternatives, especially for high-risk patients.
- While these interventions improve symptoms and reduce gradients, their impact on the natural history of HCM requires further long-term study.
- Risk stratification for sudden cardiac death, utilizing non-invasive assessments and potentially genotyping, is crucial for managing HCM prognosis.
Impact:
- Provides an overview of therapeutic options for hypertrophic cardiomyopathy, aiding clinical decision-making.
- Highlights the need for comparative trials to establish the long-term efficacy of newer HCM interventions.
- Underscores the critical role of risk stratification in preventing sudden cardiac death in HCM patients.
Abstract:
Hypertrophic cardiomyopathy (HCM) is a relatively common disease of the cardiac sarcomere with broad heterogeneity in terms of the disease-causing gene mutation, phenotypic expression, therapy and prognosis. Besides the standard drug treatment, there are several therapeutic options available for severe refractory symptomatic HCM with obstruction. Dual-chamber pacing and transcoronary ablation of septal hypertrophy (TASH) have recently emerged as alternatives to myectomy. However, myectomy remains the current gold standard of therapy for HCM until the promising initial follow-up data for TASH can be transferred into a long-term follow-up period, or prospective randomized comparative trials between these therapies are available. However, even now, TASH represents an important therapeutic alternative in patients with relevant co-morbidities and a high operative risk. Despite significant gradient reduction and amelioration of clinical symptoms, none of these treatment strategies has a proven influence on the natural history of HCM. Hence, regarding the long-term prognosis of the disease, risk stratification of sudden cardiac death using non-invasive risk assessment has become of paramount importance, while genotyping might become the determinant and stratifying marker in the near future. At present, according to secondary prevention, treatment with an implanted cardioverter-defibrillator +/- amiodarone therapy is mandatory, while according to primary prevention treatment should particularly depend on the individual risk profile.