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Introduction: Few data are available about pediatric cardiovascular disease (CVD) in the Horn of Africa. The objective of this study was to describe the spectrum of CVD in children in Djibouti. Methods: Clinical features and management of Djiboutian children between 1 month-old and 15 year-old with CVD were prospectively recorded over a two-year period in Bouffard Military Hospital in Djibouti (January 2009- December 2010). Results: Clinical examination and echocardiography were performed on 156 patients: 32 of them (20 %) had CVD. Three (10%) of them had Down's syndrome. The median age was 5 years (male 53%). Congenital heart disease was observed in 27 (84%) patients and dilated cardiomyopathy (DCM) in 5 (16%) patients including 2 patients with rheumatic valvular disease. Ventricular septal defect was frequent (28%). Other abnormalities were atrial septal defect (13%), Tetralogy of Fallot (9%), pulmonary stenosis (6%) and 3 other patients had multiple congenital anomalies condition. Surgical management was required in 22 (69%) patients and was performed on 15 (47%) cases. During follow up (mean 11.3 ± 6.8 months), 5 (16%) patients died. Absence of surgery was associated with significant mortality (p > 0.05) but age, sex and mean follow up were not. Conclusion: Pediatric CVD is at least as common in this Djiboutian community as in other African cohorts. The absence of surgery was a major mortality risk factor. DCM was frequent in this study. Much work remains to be done to discover the size and nature of genetic and environmental contributions to these various forms of heart diseases in the Horn of Africa. [ABSTRACT FROM AUTHOR]