Published online Nov 26, 2026. doi: 10.4330/wjc.126026
Revised: September 21, 2026
Accepted: October 23, 2026
Published online: November 26, 2026
Processing time: 116 Days and 16.5 Hours
Cardiomyopathy (CMP) is one of the main causes of heart failure in children. Genetic and metabolic etiology are common in regions with high consanguinity.
To describe the clinical, electrocardiographic and echocardiographic features of childhood CMP in the United Arab Emirates.
This retrospective cohort study was conducted at a tertiary care hospital in the United Arab Emirates, covering a period from 2020 to 2025. Historical data back to January 2013 were retrieved for survival analysis. All children with CMP were included. Demographic, clinical, electrocardiography (ECG), and echocardiogra
The study included 200 cases of CMP (60% were female). The cohort comprised 25 ethnic groups, with consanguinity status known for 70 families and positive in 31%. The most common age of presentation was 0-6 months. Syndromic features and neurodevelopmental delays were found in 28% and 33% of the cases, respectively. Long QT syndrome was diagnosed in 9% of the patients. Metabolic screening was positive in 15% of the cohort. The most common abnormalities were conditions associated with high lactate levels (31%), followed by conditions associated with high ammonia levels (15%). Genetic testing was positive in 21.5% of the cohort. The most common genetic abnormality was Noonan’s syndrome, followed by Alström and CHARGE syndromes; the latter, to our knowledge, has not been previously reported. Hypertrophic CMP (HCM) was the most common type (47%), followed by dilated CMP (36.5%), noncompaction CMP (14%), and restrictive CMP (2.5%). Patients with HCM presented significantly earlier and had more associated syndromic features (P < 0.041). There was statistically higher mortality in patients with noncompaction CMP associated with low EF or with HCM (combined) and a higher recovery rate in those with DCM (P < 0.05). The whole cohort mortality rate was 12%, and it was 25% for those with positive genetic testing. The mean (95%CI) of overall survival was 73.8 months. There were significantly lower survival rates in patients with edema.
In this highly consanguineous population, CMP is more prevalent in females and presents mostly in early infancy. The most common type is HCM. Genetic and metabolic disorders are common and are associated with higher mortality rates.
Core Tip: This retrospective study included 200 children with cardiomyopathy in a multiethnic, highly consanguineous population. It revealed a high prevalence in females presenting in young infancy, most commonly with the hypertrophic type. Genetic and metabolic disorders were detected in 21.5% and 15%, respectively. Mortality rates were higher for those with genetic disorders. The mean overall survival was 73.8 months. Genetic disorders included Noonan’s, Alstrom’s, and CHARGE syndromes. To our knowledge, the latter has not previously been reported.