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Canadian Journal of Cardiology

Comparison of the Prevalence, Clinical Features, and Long-term Outcomes of Midventricular Hypertrophy vs Apical Phenotype in Patients With Hypertrophic Cardiomyopathy

  • Chi Cai
    Affiliations
    Key Laboratory of Clinical Trial Research in Cardiovascular Drugs, Ministry of Health, State Key Laboratory of Cardiovascular Diseases, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
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  • Fu-jian Duan
    Affiliations
    Department of Ultrasound, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
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  • Yin-jian Yang
    Affiliations
    Key Laboratory of Clinical Trial Research in Cardiovascular Drugs, Ministry of Health, State Key Laboratory of Cardiovascular Diseases, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
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  • Xi-ying Guo
    Affiliations
    Key Laboratory of Clinical Trial Research in Cardiovascular Drugs, Ministry of Health, State Key Laboratory of Cardiovascular Diseases, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
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  • Yan-ling Liu
    Affiliations
    Department of Ultrasound, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
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  • Yu-qing Liu
    Affiliations
    Key Laboratory of Clinical Trial Research in Cardiovascular Drugs, Ministry of Health, State Key Laboratory of Cardiovascular Diseases, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
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  • Li-rong Yan
    Affiliations
    Key Laboratory of Clinical Trial Research in Cardiovascular Drugs, Ministry of Health, State Key Laboratory of Cardiovascular Diseases, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
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  • Zhi-min Xu
    Affiliations
    Key Laboratory of Clinical Trial Research in Cardiovascular Drugs, Ministry of Health, State Key Laboratory of Cardiovascular Diseases, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
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  • Shi-hua Zhao
    Affiliations
    Department of Radiology, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
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  • Wei Hua
    Affiliations
    The Cardiac Arrhythmia Center, State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
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  • Yi-shi Li
    Affiliations
    Key Laboratory of Clinical Trial Research in Cardiovascular Drugs, Ministry of Health, State Key Laboratory of Cardiovascular Diseases, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
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  • Chao-mei Fan
    Correspondence
    Corresponding author: Dr Chao-mei Fan, Beilishi road No. 167, Xicheng district, Beijing 100037, China. Tel.: +86-10-8839-8628; fax: +86-10-6835-4535.
    Affiliations
    Key Laboratory of Clinical Trial Research in Cardiovascular Drugs, Ministry of Health, State Key Laboratory of Cardiovascular Diseases, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
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Published:October 28, 2013DOI:https://doi.org/10.1016/j.cjca.2013.10.005

      Abstract

      Background

      Previous studies on the association between the distribution of left ventricle hypertrophy and the clinical features of hypertrophic cardiomyopathy (HCM) have yielded unclear results. The aim of this study was to investigate the differences in the prevalence, clinical features, management strategies, and long-term outcomes between patients with midventricular hypertrophic obstructive cardiomyopathy (MVHOCM) and patients with apical HCM (ApHCM).

      Methods

      A retrospective study of 60 patients with MVHOCM and 263 patients with ApHCM identified in a consecutive single-centre cohort consisting of 2068 patients with HCM was performed. The prevalence, clinical features, and natural history of the patients in these 2 groups were compared.

      Results

      Compared with ApHCM patients, patients with MVHOCM tended to be much younger and more symptomatic during their initial evaluation. Over a mean follow-up of 7 years, the probability of cardiovascular mortality and that of morbidity was significantly greater in MVHOCM patients compared with ApHCM patients (log-rank, P < 0.001).

      Conclusions

      Our results suggest that, compared with ApHCM, MVHOCM represents an uncommon presentation of the clinical spectrum of HCM that is characterized by progressive clinical deterioration leading to increased cardiovascular mortality and morbidity. Our results also underscore the importance of the timely recognition of MVHOCM for the prediction of prognosis and the early consideration of appropriate management strategies.

      Résumé

      Introduction

      Des études antérieures portant sur le lien entre la distribution de l’hypertrophie ventriculaire gauche et les caractéristiques cliniques de la cardiomyopathie hypertrophique (CMH) ont abouti à des résultats imprécis. Le but de cette étude était d’examiner les différences dans la prévalence, les caractéristiques cliniques, les stratégies de prise en charge et les résultats à long terme entre les patients ayant une CMH avec obstruction médioventriculaire et les patients ayant une CMH apicale.

      Méthodes

      Une étude rétrospective de 60 patients ayant une CMH avec obstruction médioventriculaire et 263 patients ayant une CMH apicale sélectionnés à partir d’une cohorte unicentrique consécutive regroupant 2068 patients ayant une CMH a été réalisée. La prévalence, les caractéristiques cliniques et l’évolution naturelle de la maladie chez ces patients de ces 2 groupes ont été comparées.

      Résultat

      Comparativement aux patients ayant une CMH apicale, les patients ayant une CMH avec obstruction médioventriculaire étaient en générale plus jeunes et plus symptomatiques lors de l’évaluation initiale. Durant un suivi moyen de 7 ans, la probabilité de mortalité liée à la maladie cardiovasculaire et de celle liée à la morbidité a été significativement plus grande chez les patients ayant une CMH avec obstruction médioventriculaire que chez les patients ayant une CMH apicale (test logarithmique par rangs, P < 0,001).

      Conclusions

      Nos résultats suggèrent que comparativement aux MCH apicales les MCH avec obstruction médioventriculaire démontrent un spectre clinique rare de CMH caractérisé par une détérioration clinique progressive entraînant l’augmentation de la mortalité et de la morbidité liées à la maladie cardiovasculaire. Nos résultats soulignent également l’importance du dépistage de la CMH avec obstruction médioventriculaire au moment opportun pour prédire le pronostic et rapidement tenir compte des stratégies de prise en charge appropriées.
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