Congenital heart disease in children with Down Syndrome: what has changed in the last three decades?
| Autor: | Dias, Filipa Mestre, Cordeiro, Susana, Menezes, Isabel, Nogueira, Graca, Teixeira, Ana, Marques, Marta, Abecasis, Miguel, Anjos, Rui |
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| Jazyk: | portugalština |
| Rok vydání: | 2016 |
| Předmět: | |
| Zdroj: | Repositório Científico de Acesso Aberto de Portugal Repositório Científico de Acesso Aberto de Portugal (RCAAP) instacron:RCAAP |
| Popis: | INTRODUCTION: The prevalence of Down syndrome has increased in the last 30 years; 55% of these children have congenital heart disease.MATERIAL AND METHODS: A retrospective longitudinal cohort study; clinical data from 1982 to 2013 databases with the diagnosis of Down syndrome or trisomy 21 in a reference hospital in pediatric cardiology and cardiac surgery.OBJECTIVE: to assess the progress in the last three decades of cardiological care given to children with Down syndrome and congenital heart disease.RESULTS: We studied 102 patients with Down syndrome and congenital heart disease subjected to invasive therapy: corrective or palliative cardiac surgery and therapeutic catheterization. The referral age was progressively earlier in patients referred in the first year of life. The most frequent diagnosis was complete atrioventricular sptal defect (41%). There was a trend towards increasingly early corrective surgery in patients under 12 months (p < 0.001). Since 2000, the large majority of patients were operated before reaching six months of age. The main cardiac complications were rhythm dysfunction and low output. More frequent noncardiac complications were pulmonary and infectious. The 30-day mortality rate was 3/102 cases (2.9%). Of patients in follow-up, 89% are in NYHA class I.DISCUSSION AND CONCLUSION: The early surgical correction seen over the past 15 years follows the approach suggested in the literature. The observed 30-day mortality rate is overlapping international results. Patients with Down syndrome subjected to corrective surgery of congenital heart disease have an excellent long-term functional capacity.Introducao: A prevalencia da sindrome de Down tem aumentado nos ultimos 30 anos; 55% destas criancas apresentam cardiopatiacongenita. Material e Metodos: Estudo retrospetivo longitudinal de coorte; dados clinicos obtidos em bases de dados de 1982 a 2013 com odiagnostico de sindrome de Down ou trissomia 21 num hospital de referencia em cardiologia pediatrica e cirurgia cardiaca. Objetivo: Avaliar a evolucao, nas ultimas tres decadas, dos cuidados cardiologicos prestados as criancas com sindrome de Down ecardiopatia congenita.Resultados: Estudamos 102 doentes com sindrome de Down e cardiopatia congenita submetidos a terapeutica invasiva: cirurgiacardiaca corretiva, paliativa e cateterismo terapeutico. Em doentes referenciados no primeiro ano de vida, a referenciacao foi cadavez mais precoce. O diagnostico mais frequente foi o defeito completo do septo auriculo-ventricular (41%). Verificou-se uma tendenciapara cirurgia corretiva cada vez mais precoce em doentes abaixo dos 12 meses (p < 0,001). A partir de 2000, a grande maioria dosdoentes foi operada antes dos seis meses de idade. As principais complicacoes cardiacas foram alteracoes de ritmo e baixo debito eas principais nao cardiacas foram pulmonares e infeciosas. A taxa de mortalidade a 30 dias foi de 3/102 casos (2,9%). Dos doentesem follow-up, 89% estao em classe funcional I da NYHA. Discussao e Conclusao: A correcao cirurgica mais precoce verificada nos ultimos 15 anos vai ao encontro do proposto na literatura. Ataxa de mortalidade a 30 dias verificada e sobreponivel aos resultados internacionais. Os doentes com sindrome de Down submetidosa cirurgia corretiva de cardiopatia congenita apresentam uma excelente capacidade funcional a longo prazo. info:eu-repo/semantics/publishedVersion info:eu-repo/semantics/publishedVersion |
| Databáze: | OpenAIRE |
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