Document Detail


The 50-year history, controversy, and clinical implications of left ventricular outflow tract obstruction in hypertrophic cardiomyopathy from idiopathic hypertrophic subaortic stenosis to hypertrophic cardiomyopathy: from idiopathic hypertrophic subaortic stenosis to hypertrophic cardiomyopathy.
MedLine Citation:
PMID:  19589431     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Dynamic obstruction to left ventricular (LV) outflow was recognized from the earliest (50 years ago) clinical descriptions of hypertrophic cardiomyopathy (HCM) and has proved to be a complex phenomenon unique in many respects, as well as arguably the most visible and well-known pathophysiologic component of this heterogeneous disease. Over the past 5 decades, the clinical significance attributable to dynamic LV outflow tract gradients in HCM has triggered a periodic and instructive debate. Nevertheless, only recently has evidence emerged from observational analyses in large patient cohorts that unequivocally supports subaortic pressure gradients (and obstruction) both as true impedance to LV outflow and independent determinants of disabling exertional symptoms and cardiovascular mortality. Furthermore, abolition of subaortic gradients by surgical myectomy (or percutaneous alcohol septal ablation) results in profound and consistent symptomatic benefit and restoration of quality of life, with myectomy providing a long-term survival similar to that observed in the general population. These findings resolve the long-festering controversy over the existence of obstruction in HCM and whether outflow gradients are clinically important elements of this complex disease. These data also underscore the important principle, particularly relevant to clinical practice, that heart failure due to LV outflow obstruction in HCM is mechanically reversible and amenable to invasive septal reduction therapy. Finally, the recent observation that the vast majority of patients with HCM have the propensity to develop outflow obstruction (either at rest or with exercise) underscores a return to the characterization of HCM in 1960 as a predominantly obstructive disease.
Authors:
Barry J Maron; Martin S Maron; E Douglas Wigle; Eugene Braunwald
Publication Detail:
Type:  Historical Article; Journal Article; Review    
Journal Detail:
Title:  Journal of the American College of Cardiology     Volume:  54     ISSN:  1558-3597     ISO Abbreviation:  J. Am. Coll. Cardiol.     Publication Date:  2009 Jul 
Date Detail:
Created Date:  2009-07-10     Completed Date:  2009-07-30     Revised Date:  2010-02-25    
Medline Journal Info:
Nlm Unique ID:  8301365     Medline TA:  J Am Coll Cardiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  191-200     Citation Subset:  AIM; IM    
Affiliation:
Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, Minnesota 55407, USA. hcm.maron@mhif.org
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MeSH Terms
Descriptor/Qualifier:
Cardiomyopathy, Hypertrophic / complications*,  history,  physiopathology,  ultrasonography
Heart Catheterization
Heart Failure
History, 20th Century
History, 21st Century
Humans
Prognosis
Time Factors
Ventricular Outflow Obstruction / etiology*,  history,  physiopathology,  ultrasonography
Comments/Corrections
Comment In:
J Am Coll Cardiol. 2010 Feb 2;55(5):502-3   [PMID:  20117470 ]
J Am Coll Cardiol. 2010 Feb 9;55(6):608; author reply 608-9   [PMID:  20152567 ]

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