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      Cardiac Arrest and Ventricular Tachycardia in Japanese-Type Apical Hypertrophic Cardiomyopathy


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          Apical hypertrophic cardiomyopathy (HCM) is a specific variant of HCM. This disease has been first described in Japan where the prevalence is much higher than in the western world. The prognosis of apical HCM with regard to sudden cardiac death is believed to be better than that of common HCM. We present, however, two male caucasian patients with apical HCM and malignant arrhythmias. Both patients had marked apical hypertrophy on echocardiography, ‘giant’ negative T-waves on the ECG and spade-like configuration of the left ventricle on angiography. The first patient had been succesfully recussitated from cardiac arrest at the age of 52 years. The second patient had a syncope at the age of 42 years and had non-sustained ventricular tachycardia. In both cases, a cardioverter-defibrillator was implanted and treatment with verapamil was initiated. These observations suggest that the risk of sudden cardiac death might be increased not only in common HCM, but also in Japanese-type apical HCM.

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          Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy.

          Hypertrophic cardiomyopathy is a genetic disease associated with a risk of ventricular tachyarrhythmias and sudden death, especially in young patients. We conducted a retrospective multicenter study of the efficacy of implantable cardioverter-defibrillators in preventing sudden death in 128 patients with hypertrophic cardiomyopathy who were judged to be at high risk for sudden death. At the time of the implantation of the defibrillator, the patients were 8 to 82 years old (mean [+/-SD], 40+/-16), and 69 patients (54 percent) were less than 41 years old. The average follow-up period was 3.1 years. Defibrillators were activated appropriately in 29 patients (23 percent), by providing defibrillation shocks or antitachycardia pacing, with the restoration of sinus rhythm; the average age at the time of the intervention was 41 years. The rate of appropriate defibrillator discharge was 7 percent per year. A total of 32 patients (25 percent) had episodes of inappropriate discharges. In the group of 43 patients who received defibrillators for secondary prevention (after cardiac arrest or sustained ventricular tachycardia), the devices were activated appropriately in 19 patients (11 percent per year). Of 85 patients who had prophylactic implants because of risk factors (i.e., for primary prevention), 10 had appropriate interventions (5 percent per year). The interval between implantation and the first appropriate discharge was highly variable but was substantially prolonged (four to nine years) in six patients. In all 21 patients with stored electrographic data and appropriate interventions, the interventions were triggered by ventricular tachycardia or fibrillation. Ventricular tachycardia or fibrillation appears to be the principal mechanism of sudden death in patients with hypertrophic cardiomyopathy. In high-risk patients with hypertrophic cardiomyopathy, implantable defibrillators are highly effective in terminating such arrhythmias, indicating that these devices have a role in the primary and secondary prevention of sudden death.
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            The management of hypertrophic cardiomyopathy.

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              Long-term outcome in patients with apical hypertrophic cardiomyopathy.

              The aim of this study was to describe long-term outcome in patients with apical hypertrophic cardiomyopathy (ApHCM) followed in a tertiary referral center. Apical hypertrophic cardiomyopathy is a relatively rare form of hypertrophic cardiomyopathy (HCM), first described in Japan. Initial reports, based on a limited number of patients, emphasized the benign nature of this condition. A retrospective study of 105 patients with ApHCM diagnosed at the Toronto General Hospital from 1975 to 2000 was performed. Symptoms, clinical findings, mortality and cardiovascular morbidity were analyzed. The mean age at presentation was 41.4 +/- 14.5 years. During a mean follow-up of 13.6 +/- 8.3 years from presentation, cardiovascular mortality was 1.9% (2/105) and annual cardiovascular mortality was 0.1%. Overall survival was 95% at 15 years. Thirty-two patients (30%) had one or more major morbid events, the most frequent being atrial fibrillation (12%) and myocardial infarction (10%). Probability of survival without morbid events was 74% at 15 years. Three predictors of cardiovascular morbidity were identified: age at presentation or = II at baseline. Forty-four percent of the patients were asymptomatic at the time of last follow-up. Apical hypertrophic cardiomyopathy in North American patients is not associated with sudden cardiac death and has a benign prognosis in terms of cardiovascular mortality. Nevertheless, one third of these patients experience serious cardiovascular complications, such as myocardial infarction and arrhythmias. These data are likely to influence the counseling and management of patients with ApHCM.

                Author and article information

                S. Karger AG
                February 2007
                27 June 2006
                : 107
                : 2
                : 81-86
                Department of Cardiology and Pulmonology, Charité – Campus Benjamin Franklin, Berlin, Germany
                94147 Cardiology 2007;107:81–86
                © 2007 S. Karger AG, Basel

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                : 01 December 2005
                : 06 April 2006
                Page count
                Figures: 2, References: 36, Pages: 6
                Case Report

                General medicine,Neurology,Cardiovascular Medicine,Internal medicine,Nephrology
                Cardiac arrest,Giant negative T-waves,Hypertrophic cardiomyopathy,Apical,Syncope


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