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      Clinical Features and Outcome of Patients with Apical Hypertrophic Cardiomyopathy in Taiwan


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          Objectives: The aim of this study was to analyze clinical characteristics and the outcome of patients with apical hypertrophic cardiomyopathy (ApHCM) followed in a Taiwan tertiary referral medical center. Background: ApHCM is regarded as a subgroup of nonobstructive HCM that occurs largely in Japanese patients. The clinical features, gender differences and prognosis of the disease in Taiwan are poorly understood. Methods: A retrospective cohort study with 40 patients was performed. Diagnosis was based on the demonstration of left ventricular hypertrophy by echocardiography. Clinical features, cardiovascular morbidity and mortality were analyzed. Multiple logistic regression was used to adjust for potential confounding factors. Results: Among 40 patients, males predominated with a percentage of 75%. The female patients obviously had later onset of presentation (mean age ± SD, 62.2 ± 5.7 vs. 54.1 ± 11.4 years; p = 0.038). During a mean follow-up of 72.2 ± 60.1 months, there was no mortality. However, 13 patients (32.5%) had one or more major cardiovascular morbidities, the most frequent being syncope or near syncope (15%) and ischemic stroke-associated atrial fibrillation (10%). In a multivariate analysis, left atrial enlargement (odds ratio 5.85, 95% CI 1.15–29.40; p = 0.034) was the only predictor of cardiovascular morbidity. Conclusions: Patients with ApHCM in Taiwan have a benign clinical course without association with sudden death and cardiovascular mortality. Left atrial enlargement was the only identified predictor of cardiovascular morbidity

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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.
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            Hypertrophic nonobstructive cardiomyopathy with giant negative T waves (apical hypertrophy): ventriculographic and echocardiographic features in 30 patients.

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              Comparison of prevalence of apical hypertrophic cardiomyopathy in Japan and the United States.

              The morphologic apical form of hypertrophic cardiomyopathy (HC), in which left ventricular (LV) wall thickening is confined to the most distal region at the apex, has been regarded as a phenotypic expression of nonobstructive HC largely unique to Japanese patients. To investigate this question further, we directly compared unselected and regional hospital-based cohorts of adult patients with HC ( > or =18 years of age) from Japan (Kochi; n=100) and from the United States (US) (Minneapolis; n=361). Japanese and American patients with HC had similar clinical features and did not differ significantly with regard to the severity of symptoms and frequency of outflow obstruction. Although Japanese and American patients also showed similar maximum LV thickness, they differed significantly with respect to the distribution of LV hypertrophy. In particular, the segmental form of HC, with hypertrophy confined to the LV apex, was more frequent in Japanese patients (i.e., apical HC, 15% in Japan vs 3% in US, p<0.0001). Giant negative T waves were also more common in Japanese patients with HC (26% vs 2%, p<0.001), including those with the apical form (64% vs. 30%, p<0.05). Each patient with apical HC had either no or only mild symptoms, and all survived. The morphologic form of nonobstructive HC with hypertrophy limited to the LV apex (apical form of HC) was 5 times more common in an unselected Japanese population. These findings document variability in the phenotypic expression of HC between countries and races, which may be due to differences in environmental factors or genetic background. Patients with the apical form of HC had a benign clinical course.

                Author and article information

                S. Karger AG
                June 2006
                03 July 2006
                : 106
                : 1
                : 29-35
                aDepartment of Internal Medicine, bInstitute of Clinical Medicine, School of Medicine, National Cheng Kung University Hospital, Tainan, Taiwan, ROC
                92590 Cardiology 2006;106:29–35
                © 2006 S. Karger AG, Basel

                Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                : 02 August 2005
                : 01 October 2006
                Page count
                Tables: 4, References: 22, Pages: 7
                Original Research

                General medicine,Neurology,Cardiovascular Medicine,Internal medicine,Nephrology
                Transthoracic echocardiography,Left ventricular hypertrophy,Hypertrophic cardiomyopathy


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