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  • Title: [Diagnosis and treatment of hypertrophic cardiomyopathies].
    Author: Kühl C, Luedde M, Langer C, Frey N.
    Journal: Dtsch Med Wochenschr; 2013 Mar; 138(12):583-8. PubMed ID: 23483418.
    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.
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