Low proportion of familial dilated cardiomyopathy in an arab population with a high prevalence of consanguineous marriages.
In this study, 770/890 (87%) first-degree relatives from 108 families of hospitalized patients with idiopathic dilated cardiomyopathy (IDC) were screened using clinical examination, electrocardiography and echocardiography. Thirty percent of the patients were born to consanguineous parents. Familial dilated cardiomyopathy (FDC) was found in 7 (6.5%) families, which is lower than the earlier published figures of 20-25%. Patients with IDC were younger at presentation (p = 0.002) and were more often associated with parental consanguinity (p = 0.04). but the survival rates of familial patients did not differ significantly.


CONCLUSIONS
Despite the high prevalence of consanguinity, there was a low proportion of FDC in the study population. With the prospects of treatment of asymptomatic IDC to slow the progression of the disease, all family members of newly identified IDC patients should receive screening and counselling, with appropriate therapy where indicated.
Venugopalan
P
P
Department of Child Health and Medicine, Sultan Qaboos University Hospital, Muscat, Sultanate of Oman.
Agarwal
A K
AK
de Bono
D
D
D000293:Adolescent; D000328:Adult; D002311:Cardiomyopathy, Dilated / Q000208:ethnology* / Q000235:genetics*; D002648:Child; D002675:Child, Preschool; D003241:Consanguinity; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009850:Oman / Q000453:epidemiology; D011446:Prospective Studies; D018709:Statistics, Nonparametric