Document Detail


Clinicopathological profiles of progressive heart failure in hypertrophic cardiomyopathy.
MedLine Citation:
PMID:  20513729     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
AIMS: Hypertrophic cardiomyopathy (HCM) is an important cause of heart failure-related disability over a wide range of ages. Profiles of severe progressive heart failure symptoms and death, or heart transplantation deserve more complete definition within large patient cohorts.
METHODS AND RESULTS: Clinical and morphological features of heart failure were assessed in 293 consecutive HCM patients over a median follow-up of 6 (inter-quartile range 2-11) years. Gross and histopathological features were analysed in 12 patients for whom the heart was available for inspection. Of the 293 patients, 50 (17%) developed severe progressive heart failure, including 18 who died or were transplanted. Three profiles of heart failure were identified predominantly associated with: (i) end-stage systolic dysfunction (ejection fraction <50%) (15; 30%); (ii) left ventricular (LV) outflow obstruction at rest (11; 22%); and (iii) non-obstructive with preserved systolic function (24; 48%). Overall, atrial fibrillation (AF) contributed to heart failure in 32 patients (64%) among the three profiles. Compared with other patients, those non-obstructive with preserved systolic function had earlier onset of heart failure symptoms mainly due to diastolic dysfunction, and the most accelerated progression to advanced heart failure and adverse outcome (P = 0.04). Thrombi were identified in the left atrial appendage of five gross heart specimens all belonging to patients with AF, including three of which were unrecognized clinically and had previously embolized. Extensive myocardial scarring with LV remodelling was evident in all end-stage patients; no or only focal scars were present in other patients.
CONCLUSION: Profiles of advanced heart failure in HCM are due to diverse pathophysiological mechanisms, including LV outflow obstruction and diastolic or global systolic ventricular dysfunction. Atrial fibrillation proved to be the most common disease variable associated with progressive heart failure. Recognition of the heterogeneous pathophysiology of heart failure in HCM is relevant, given the targeted management strategies necessary in this disease.
Authors:
Paola Melacini; Cristina Basso; Annalisa Angelini; Chiara Calore; Fabiana Bobbo; Barbara Tokajuk; Nicoletta Bellini; Gessica Smaniotto; Mauro Zucchetto; Sabino Iliceto; Gaetano Thiene; Barry J Maron
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2010-05-31
Journal Detail:
Title:  European heart journal     Volume:  31     ISSN:  1522-9645     ISO Abbreviation:  Eur. Heart J.     Publication Date:  2010 Sep 
Date Detail:
Created Date:  2010-09-02     Completed Date:  2011-08-31     Revised Date:  2013-05-29    
Medline Journal Info:
Nlm Unique ID:  8006263     Medline TA:  Eur Heart J     Country:  England    
Other Details:
Languages:  eng     Pagination:  2111-23     Citation Subset:  IM    
Affiliation:
Department of Cardiac, Thoracic and Vascular Sciences, University of Padua Medical School, Via Giustiniani 2, 35100 Padova, Italy. paola.melacini@unipd.it
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MeSH Terms
Descriptor/Qualifier:
Adult
Aged
Arrhythmias, Cardiac / genetics,  pathology
Cardiomyopathy, Hypertrophic / genetics,  pathology*
Female
Heart Failure / genetics,  pathology*
Humans
Male
Middle Aged
Protein C / genetics
Ventricular Dysfunction, Left / genetics,  pathology
Young Adult
Chemical
Reg. No./Substance:
0/Protein C
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