[Progression of cardiopathology in Friedreich ataxia: clinico-instrumental study].
Clinical, electrocardiographic and echocardiographic 5-year follow-up was performed in our institution on 61 patients with Friedreich's ataxia. Cardiac failure was evident in 5% of the patients, and was the most common cause of death. Cardiac arrhythmias, most commonly supraventricular in origin, usually occurred together with the onset of cardiac failure and in 1 case resulted in sudden death. ST-T abnormalities were present in 91% of the cases, and were independent from other clinical parameters. On the contrary, pseudonecrotic (5%) and right ventricular hypertrophy pattern were associated with a poor prognosis. Left ventricular hypertrophy was evident at the echocardiogram in 75% of cases and remained unchanged throughout the entire follow-up period. In 1 case left ventricular hypertrophy turned to dilative cardiomyopathy. Autopsy was performed in 2 out of 4 decreased patients and revealed massive interstitial fibrosis with cellular degeneration in the absence of coronary lesions.
Casazza
F
F
Divisione di Cardiologia, Ospedale S Carlo Borromeo, Milano.
Ferrari
F
F
Piccone
U
U
Maggiolini
S
S
Capozi
A
A
Morpurgo
M
M
D000293:Adolescent; D000328:Adult; D001145:Arrhythmias, Cardiac / Q000150:complications / Q000503:physiopathology; D006332:Cardiomegaly / Q000000981:diagnostic imaging / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D003645:Death, Sudden; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D005621:Friedreich Ataxia / Q000150:complications / Q000000981:diagnostic imaging / Q000503:physiopathology*; D006321:Heart / Q000503:physiopathology*; D006333:Heart Failure / Q000150:complications / Q000503:physiopathology; D006801:Humans; D008297:Male; D009206:Myocardium / Q000473:pathology
Evoluzione della cardiopatia nell'atassia di Friedreich: studio clinico-strumentale.