Document Detail

Cardiomyopathy in Friedreich's ataxia: a Doppler-echocardiographic study.
MedLine Citation:
PMID:  1396814     Owner:  NLM     Status:  MEDLINE    
Heart involvement is frequent in Friedreich's ataxia (FA), the most prevalent of the spino-cerebellar degenerative diseases, which is inherited with an autosomal recessive pattern. However, the pathophysiological link between cardiac and neurological disorders is not yet clearly established. We compared a group of 10 patients with FA to a control group (C) of 16 normal subjects, using Doppler-echocardiography. To see whether cardiac involvement was specific to FA, the data of patients with FA were also compared to those of patients with autosomal dominant olivo-ponto-cerebellar atrophia (OPCA), another spino-cerebellar degenerative disease. There was an increase in left ventricular mass index in FA (154 +/- 9 g.m-2 vs 99 +/- 7 g. m-2 in C, P < 0.001), systolic function was normal, the ejection fraction (EF) slope and E/A ratio were decreased (85 +/- 9 mm.s-1 vs 130 +/- 7 mm.s-1 in C, P < 0.001 and 1.5 +/- 0.1 vs 1.7 +/- 0.1 in C, P < 0.01, respectively), while the isovolumic relaxation period was increased (96 +/- 3 ms vs 92 +/- 2 ms in C, P < 0.01). Deceleration time and time-velocity integrals of A wave to total mitral flow were not modified. In OPCA only the E/A ratio was decreased (1.5 +/- 0.1 vs 1.7 +/- 0.1 in C, P < 0.05). These data show the presence of cardiomyopathy in FA with left ventricular hypertrophy and suggest the presence of diastolic function abnormalities. The cardiomyopathy seems specifically associated with FA and not to spino-cerebellar degenerative disease in general.
D Morvan; M Komajda; L D Doan; A Brice; R Isnard; A Seck; P Lechat; Y Agid; Y Grosgogeat
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  European heart journal     Volume:  13     ISSN:  0195-668X     ISO Abbreviation:  Eur. Heart J.     Publication Date:  1992 Oct 
Date Detail:
Created Date:  1992-11-17     Completed Date:  1992-11-17     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  8006263     Medline TA:  Eur Heart J     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  1393-8     Citation Subset:  IM    
Service de Cardiologie, INSERM U.289, Hôpital Pitié-Salpêtrière, Paris, France.
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MeSH Terms
Analysis of Variance
Cardiomyopathy, Hypertrophic / etiology*
Echocardiography, Doppler*
Friedreich Ataxia / complications,  physiopathology,  ultrasonography*
Hypertrophy, Left Ventricular / etiology,  ultrasonography
Middle Aged
Olivopontocerebellar Atrophies / ultrasonography
Regression Analysis
Ventricular Function, Left

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