| Summary: | <p><strong>Background</strong>: congenital heart diseases are the most frequent malformations and may be associated with genetic syndromes.</p><p><strong>Objective</strong>: to characterize clinicoepidemiological variables of cardiopathy patients with an associated genetic syndrome treated at the “Hermanos Cordové” Pediatric Hospital of Manzanillo, Granma, from January 2011 to December 2018.</p><p><strong>Methods</strong>: a descriptive, retrospective, cross-sectional study was carried out with a universe of 46 patients with the criteria defined in the objective. The following variables were assessed: sex, genetic syndrome, type of cardiopathy, progress and complications. Descriptive statistics was used to process the data.</p><p><strong>Results</strong>: Down syndrome was more frequent as the main congenital anomaly, with a total of 22 patients for 47,8 % and the female sex with 28 cases representing 60,8 %. Cardiac shunt predominated in 33 patients (71,7 %), 19 of them suffered from Down syndrome. Most of the patients had a favorable progress. Malnutrition was the complication that stood out in 30 patients (65,2 %).</p><p><strong>Conclusions</strong>: the predominance of cardiac shunt, in several cases associated with Down syndrome, was the most relevant characteristic in the studied population.</p>
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