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Etiology and Phenotypes of Cardiomyopathy in Southern Africa: The IMHOTEP Multicenter Pilot Study
Sarah M Kraus1,2,3,4, Jacqui Cirota1,3, Shahiemah Pandie1,3
1Department of Medicine, The Cardiac Clinic, University of Cape Town and Groote Schuur Hospital, Cape Town, South Africa.
Insights
Dilated cardiomyopathy (DCM) is the most common cardiomyopathy in Southern Africa, affecting younger individuals and women more frequently. This study highlights significant sex and ethnic disparities in DCM within the African population.
Area of Science:
- Cardiology
- Genetics
- Epidemiology
Background:
- Cardiomyopathies are a significant cause of heart failure in Africa.
- There is a lack of comprehensive data on cardiomyopathy etiology and clinical characteristics on the continent.
Purpose of the Study:
- To systematically collect and analyze data on individuals diagnosed with cardiomyopathy in Africa.
- To characterize the phenotypes and etiologies of different cardiomyopathy types within the African population.
Main Methods:
- A multicenter pilot study involving patients aged 13 years and older with a diagnosis of cardiomyopathy or myocarditis.
- Cases were categorized into dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), arrhythmogenic cardiomyopathy (ACM), and restrictive cardiomyopathy (RCM).
- Data were collected from 4 centers across South Africa and Mozambique.
Main Results:
- 665 unrelated index cases were recruited, with DCM being the most prevalent type (72%).
- DCM and RCM showed a younger age of onset and higher prevalence in women and individuals of African ancestry compared to European/North American populations.
- Etiologies were diverse, including familial (27%), idiopathic (36%), and secondary (37%) causes.
Conclusions:
- Dilated cardiomyopathy is the dominant form in Southern Africa.
- African patients with DCM exhibit a significantly younger age of onset, with notable sex and ethnic disparities.
- Findings underscore the need for further research into the unique aspects of cardiomyopathy in African populations.
Background:
Cardiomyopathies are an important cause of heart failure in Africa yet there are limited data on etiology and clinical phenotypes.
Objectives:
The IMHOTEP (African Cardiomyopathy and Myocarditis Registry Program) was designed to systematically collect data on individuals diagnosed with cardiomyopathy living in Africa.
Methods:
In this multicenter pilot study, patients (age ≥13 years) were eligible for inclusion if they had a diagnosis of cardiomyopathy or myocarditis. Cases were grouped and analyzed according to phenotype; dilated cardiomyopathy (DCM) including myocarditis and peripartum cardiomyopathy, hypertrophic cardiomyopathy (HCM), arrhythmogenic cardiomyopathy (ACM), and restrictive cardiomyopathy (RCM).
Results:
A total of 665 unrelated index cases (median age 35 [27-44] years; 51.1% female) were recruited at 3 centers in South Africa and 1 center in Mozambique. DCM (n = 478) was the most common type of cardiomyopathy, accounting for 72% of the cohort; ACM (n = 78), HCM (n = 70), and RCM (n = 39) were less frequent. While the age of onset and sex distribution of HCM and ACM were similar to European and North American populations, DCM and RCM had a younger age of onset and occurred more frequently in women and those with African ancestry. Causes of cardiomyopathy were diverse; familial (27%), nonfamilial/idiopathic (36%), and secondary (37%) etiologies were observed.
Conclusions:
In the largest study of cardiomyopathy to-date on the African continent, we observe that DCM is the dominant form of cardiomyopathy in Southern Africa. The age of onset was significantly younger in African patients with notable sex and ethnic disparities in DCM.
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