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Quantification of regional right and left ventricular function by ultrasonic strain rate and strain indexes in Friedreich’s ataxia

2003; Elsevier BV; Volume: 91; Issue: 5 Linguagem: Inglês

10.1016/s0002-9149(02)03325-8

1879-1913

Frank Weidemann, Bénédicte Eyskens, Luc Mertens, Giovanni Di Salvo, J STROTMANN, Gunnar Buyse, Piet Claus, Jan D’hooge, Bart Bijnens, Marc Gewillig, George R. Sutherland,

Neurological disorders and treatments

Friedreich's ataxia (FA) is an autosomal recessive neurodegenerative disease caused by a defect in the gene that encodes for a mitochondrial proteinnamed frataxin. 1 Campuzano V. Montermini L. Molto M.D. Pianese L. Cossee M. Cavalcanti F. Monros E. Rodius F. Duclos F. Monticelli A. et al. Friedreich's ataxia autosomal recessive disease caused by an intronic GAA triplet repeat expansion. Science. 1996; 271: 1423-1427 Crossref PubMed Scopus (2375) Google Scholar Myocardial involvement in FA is well documented, with the most frequent cardiac finding being concentric or asymmetric left ventricular (LV) hypertrophy. 2 Child J.S. Perloff J.K. Bach P.M. Wolfe A.D. Perlman S. Kark R.A. Cardiac involvement in Friedreich's ataxia a clinical study of 75 patients. J Am Coll Cardiol. 1986; 7: 1370-1378 Abstract Full Text PDF PubMed Scopus (121) Google Scholar , 3 Dutka D.P. Donnelly J.E. Palka P. Lange A. Nunez D.J. Nihoyannopoulos P. Echocardiographic characterization of cardiomyopathy in Friedreich's ataxia with tissue Doppler echocardiographically derived myocardial velocity gradients. Circulation. 2000; 102: 1276-1282 Crossref PubMed Scopus (89) Google Scholar Clinical signs of cardiac involvement typically occur late in the course of the disease and many patients die from heart failure. 4 Hewer R.L. Study of fatal cases of Friedreich's ataxia. BMJ. 1968; 3: 649-652 Crossref PubMed Scopus (80) Google Scholar Ultrasound-based strain rate imaging is a new technique that has recently been shown to quantify regional changes in myocardial deformation. 5 Heimdal A. Støylen A. Torp H. Skjaerpe T. Real-time strain rate imaging of the left ventricle by ultrasound. J Am Soc Echocardiogr. 1998; 11: 1013-1019 Abstract Full Text Full Text PDF PubMed Scopus (740) Google Scholar Myocardial strain measurements have been validated in both correlative experimental sonomicrometric 6 Urheim S. Edvardsen T. Torp T. Angelsen B. Smiseth O.A. Myocardial strain by Doppler echocardiography. Validation of a new method to quantify regional myocardial function. Circulation. 2000; 102: 1158-1164 Crossref PubMed Scopus (989) Google Scholar and in clinical 7 Edvardson T. Skulstad H. Aakhus S. Urheim S. Ihlen H. Regional myocardial systolic function during acute myocardial ischemia assessed by strain Doppler echocardiography. J Am Coll Cardiol. 2001; 37: 726-730 Abstract Full Text Full Text PDF PubMed Scopus (236) Google Scholar studies. When compared with myocardial velocities, they have been shown to be less influenced by overall cardiac motion and tethering effects. 6 Urheim S. Edvardsen T. Torp T. Angelsen B. Smiseth O.A. Myocardial strain by Doppler echocardiography. Validation of a new method to quantify regional myocardial function. Circulation. 2000; 102: 1158-1164 Crossref PubMed Scopus (989) Google Scholar Thus, with strain rate imaging it is possible to quantify the amount of deformation and the velocity of deformation in all LV and right ventricular (RV) segments. The aim of the present study was to investigate the regional deformation properties of the left and right ventricles in patients with FA and to compare these functional measurements with myocardial wall thickness.