PMID- 14519277 OWN - NLM STAT- MEDLINE DCOM- 20040213 LR - 20190916 IS - 0300-8932 (Print) IS - 0300-8932 (Linking) VI - 56 IP - 9 DP - 2003 Sep TI - [Heart malformations in children with Down syndrome]. PG - 894-9 AB - INTRODUCTION AND OBJECTIVES: A longitudinal, retrospective, observational descriptive study was done at the National Institute of Pediatrics in Mexico City to determine the incidence, type of heart disease and clinical course in patients with Down syndrome (DS), and to compare the findings with data from other countries. Down syndrome is a disease caused by trisomy of chromosome 21. The frequency of presentation in one in 650 live births. Frequency in the general population is about 1%. Cardiac malformation is the main cause of mortality in the first 2 years of life. PATIENTS AND METHOD: In a 5-year period 275 patients (aged neonate to 13 years) were diagnosed with DS. Diagnosis was based on echocardiogram, catheterization, genetics, surgical exploration or necropsy. Age, sex, clinical manifestations, mother's age, type of heart defect were recorded. RESULTS: Of the 275 children with DS, 160 had congenital heart disease. The most frequent cardiopathies were interauricular septal defect (IASD), interventricular septal defect (IVSD) and patent ductus arteriousus (PDA) (90%). In contrast to the data from other countries, only 14 patients (8%) had atrioventricular septal defect (AVSD). Twenty-five patients died (15%) from sepsis and cardiogenic shock. CONCLUSIONS: At our institute 58% of the children with DS had congenital heart malformation. The most frequent cardiopathies were different from those reported in other countries. FAU - de Rubens Figueroa, Jesus AU - de Rubens Figueroa J AD - Instituto Nacional de Pediatria. Mexico, D.F. Mexico. derubens@hotmail.com FAU - del Pozzo Magana, Blanca AU - del Pozzo Magana B FAU - Pablos Hach, Jose L AU - Pablos Hach JL FAU - Calderon Jimenez, Claudia AU - Calderon Jimenez C FAU - Castrejon Urbina, Rocio AU - Castrejon Urbina R LA - spa PT - Comparative Study PT - English Abstract PT - Journal Article PT - Review TT - Malformaciones cardiacas en los ninos con sindrome de Down. PL - Spain TA - Rev Esp Cardiol JT - Revista espanola de cardiologia JID - 0404277 SB - IM CIN - Rev Esp Cardiol. 2004 May;57(5):482; author reply 482. PMID: 15151784 MH - Adolescent MH - Child MH - Child, Preschool MH - Down Syndrome/*complications MH - Female MH - Heart Defects, Congenital/*complications/epidemiology MH - Humans MH - Incidence MH - Infant MH - Longitudinal Studies MH - Male MH - Retrospective Studies RF - 29 EDAT- 2003/10/02 05:00 MHDA- 2004/02/14 05:00 CRDT- 2003/10/02 05:00 PHST- 2003/10/02 05:00 [pubmed] PHST- 2004/02/14 05:00 [medline] PHST- 2003/10/02 05:00 [entrez] AID - 13051617 [pii] AID - 10.1016/s0300-8932(03)76978-4 [doi] PST - ppublish SO - Rev Esp Cardiol. 2003 Sep;56(9):894-9. doi: 10.1016/s0300-8932(03)76978-4.