Key result
Systematic screening identifies familial DCM in ~7% of idiopathic DCM families despite high consanguinity.
Why the study?
The proportion of familial dilated cardiomyopathy in an Arab population with high consanguinity was unclear compared to previously published figures.
Observational (n=770)
Despite a high prevalence of consanguineous marriages, the proportion of familial dilated cardiomyopathy in this Arab population was unexpectedly low at 6.5%.
Supports family screening in pediatric idiopathic dilated cardiomyopathy; leaves open the contribution of consanguinity in larger cohorts.
UNLABELLED: 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. CONCLUSION: 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.
No takes yet. Share an insight, caveat, or question.
Venugopalan et al. (2001) conducted an observational in Idiopathic dilated cardiomyopathy (n=770). Parental consanguinity vs. No parental consanguinity was evaluated on Prevalence of familial dilated cardiomyopathy. Familial dilated cardiomyopathy was identified in 6.5% of families of hospitalized patients with idiopathic dilated cardiomyopathy, despite a high prevalence of parental consanguinity.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: