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Cardiomyopathy in Friedreich's ataxia‐assessment by cardiac MRI
Author(s) -
Meyer Chris,
Schmid Gebhard,
Görlitz Sabine,
Ernst Monika,
Wilkens Christian,
Wilhelms Inga,
Kraus Peter H.,
Bauer Peter,
Tomiuk Jürgen,
Przuntek Horst,
Mügge Andreas,
Schöls Ludger
Publication year - 2007
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.21590
Subject(s) - ataxia , cardiology , medicine , left ventricular hypertrophy , interventricular septum , cardiomyopathy , magnetic resonance imaging , muscle hypertrophy , heart disease , cardiac magnetic resonance imaging , hypertrophic cardiomyopathy , heart failure , radiology , blood pressure , ventricle , psychiatry
Cardiomyopathy is an important and frequently life limiting manifestation of Friedreich's ataxia (FA), the most prevalent form of autosomal recessive ataxia. Left ventricular mass is used as primary outcome measure in recent intervention studies but systematic analyses of FA cardiomyopathy are sparse. To assess cardiac hypertrophy by cardiac magnetic resonance imaging (MRI) in vivo, we assessed 41 adult patients with genetically confirmed FA and 33 age‐ and sex‐matched healthy controls by cardiac MRI and echocardiogarphy. Septal hypertrophy and left ventricular mass index were determined by two independent raters. MRI revealed hypertrophy of the interventricular septum in 40% and increased left ventricular mass index in 29% of patients. Interobserver variability was less than 5% for both measures. GAA repeat length had only minor influence on interventricular septum thickness. Left ventricular mass index decreased with age. Severity of ataxia did not correlate with cardiac disease. In echocardiography wall diameter was assessable only in 31 of 41 FA patients with 32% of patients presenting septal hypertrophy and 6% increased left ventricular mass index. We conclude that cardiac hypertrophy is present only in a minority of adult FA patients. If despite this limitation intervention studies use left ventricular mass as outcome measure, MRI is recommended as the most accurate assessment of cardiac anatomy in vivo. © 2007 Movement Disorder Society