Cardiac involveent in type 1 myotonic dystrophy.
Myotonic dystrophy is a multisystem disorder in which cardiac involvement is common, particularly in conduction tissue. Its severity and symptoms vary, and so careful monitoring is required and a permanent pacemaker may need to be implanted. The aim of the study was to evaluate cardiac alterations and to correlate them with genetic (CTG expansion) and neurological data. Of the 46 patients studied, 80.4% presented asymptomatic cardiac conduction abnormalities but only one had impaired global systolic function, despite 32.6% having changes in left ventricular segmental contractility. Left ventricular noncompaction was found in 8 patients. Patients with greater CTG expansion had more severe neurological involvement and conduction abnormalities, and those with greater neurological disability had also more significant conduction tissue involvement.
Sá
Maria Isabel
MI
Departamento de Medicina, Serviço de Cardiologia, Hospital Geral de Santo António, Porto, Portugal. isabelpsa@portugalmail.pt
Cabral
Sofia
S
Costa
P Dias
PD
Coelho
Teresa
T
Freitas
Mário
M
Torres
Severo
S
Gomes
J Lopes
JL
D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D006331:Heart Diseases / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000150:complications* / Q000175:diagnosis; D012189:Retrospective Studies