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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Article info
Publication history
Published online: October 28, 2013
Accepted:
October 4,
2013
Received:
July 9,
2013
Footnotes
See page 447 for disclosure information.
Identification
Copyright
© 2014 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.
