Friedreich's Ataxia: a review from a cardiology perspective.
Ir J Med Sci
; 180(4): 799-805, 2011 Dec.
Article
em En
| MEDLINE
| ID: mdl-21822977
ABSTRACT
BACKGROUND:
Neuromuscular disorders are not among the common causes of cardiomyopathy in the general population; however, cardiomyopathy is known to occur in several neuromuscular disorders including Friedreich's Ataxia (FA). In patients with neuromuscular disorders, concomitant cardiac involvement contributes significantly to morbidity and mortality and often leads to premature death.METHODS:
An extensive literature search of Medline and Pubmed was conducted to include all published reports on cardiac involvement in FA. Secondary articles were identified from key paper reference listings.CONCLUSION:
Hypertrophic cardiomyopathy is a cardinal feature of FA; therefore all FA patients should be screened for cardiomyopathy. A cardiac examination, ECG and ECHO are advised at diagnosis, and also on the development of any cardiac symptoms. Treatment is determined by the presence of symptoms, the presence of left ventricular outflow gradient and the sudden death risk. Institution of aggressive medical therapy early in the course of the disease may help improve quality of life and provide survival benefit.
Texto completo:
1
Coleções:
01-internacional
Contexto em Saúde:
2_ODS3
Base de dados:
MEDLINE
Assunto principal:
Cardiomiopatia Hipertrófica
/
Ataxia de Friedreich
Tipo de estudo:
Diagnostic_studies
/
Prognostic_studies
Aspecto:
Patient_preference
Limite:
Humans
Idioma:
En
Revista:
Ir J Med Sci
Ano de publicação:
2011
Tipo de documento:
Article