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Alterações cardiovasculares na síndrome de Turner : revisão do seguimento em cardiologia pediátrica no Hospital de Santa Maria
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Introdução e Objetivos: As manifestações cardiovasculares na Síndrome de Turner são importantes pela sua significativa prevalência e impacto no prognóstico. Neste trabalho pretende-se rever o seguimento cardiovascular no departamento de Pediatria do CHULN - HSM e propor um protocolo de seguimento em Cardiologia Pediátrica.
Métodos: Estudo observacional transversal por consulta dos processos clínicos das doentes com Síndrome de Turner seguidas em Endocrinologia Pediátrica no CHULN – HSM entre 01/01/2017 e 31/12/2021. Foram analisados dados demográficos, dados clínicos das consultas de Cardiologia Pediátrica, exames complementares e terapêutica.
Resultados: Foram identificadas 21 doentes, das quais 19% (n=4) 45,X e 57% (n=12) com cariótipo em mosaico. Detetaram-se alterações cardiovasculares típicas em 62% (n=13), das quais se apurou VAB em 33% (n=7), DA em 29% (n=6), CoA em 19% (n=4), HTA em 14% (n=3) e alterações elétricas em 9.5% (n=2). Todas as doentes tiveram pelo menos uma avaliação cardiovascular, sendo 52% (n=11) referenciadas pelo médico assistente após diagnóstico de ST. Destas, 57% (n=12) eram seguidas no CHULN – HSM em Cardiologia Pediátrica. A avaliação cardiovascular era em média anual (0.68 consultas/ano), com reavaliação imagiológica a cada 1-2 anos em 50% (n=6). Esta avaliação foi realizada com recurso a ecoTT em 75% (n=9), a ecoTT e RM cardíaca em 25% (n=3), dos quais 8% (n=1) com angio-TC adicional. Das 21 doentes iniciais, 33% (n=7) realizava terapêutica farmacológica e 24% (n=5) tinha sido submetida a terapêutica cirúrgica.
Conclusão: O compromisso cardiovascular é frequente nestas doentes, como sugerido no estudo, exigindo um seguimento regular. As recomendações mais recentes eram cumpridas em 91.7% (n=11) das doentes da consulta de Cardiologia Pediátrica deste hospital, sendo necessários ainda estudos mais robustos para refletir a prática clínica
Introduction and Objectives: Cardiovascular disease constitutes an important presentation of Turner Syndrome for its significant prevalence and impact on prognosis. This study aims to review the cardiovascular care in the Pediatrics Department of CHULN-HSM and propose a follow-up protocol in Pediatric Cardiology. Methods: Cross-sectional observational study based on a review of patient records with Turner Syndrome followed by Pediatric Endocrinology of CHULN - HSM between 01/01/2017 and 31/12/2021. Demographic data, data from Pediatric Cardiology consults, cardiac imaging and treatment were analysed. Results: A total of 21 patients was included, of which 19% (n=4) had 45,X karyotype and 57% (n=12) had a mosaic karyotype. Cardiovascular abnormalities were detected in 62% (n=13), of which BAV in 33% (n=7), aortic dilation in 29% (n=6), CoA in 19% (n=4), hypertension in 14% (n=3) and electrocardiographic abnormalities in 9.5% (n=2). All the patients had at least one cardiovascular evaluation, being referred after TS diagnosis by primary physician in 52% (n=11). Of these, 57% (n=12) were followed by Pediatric Cardiology of CHULN – HSM. A complete cardiovascular evaluation was conducted in average annually (0.68 consults/year), with imaging follow-up every 1-2 years in 50% (n=6). Cardiovascular imaging was performed by TTE in 75% (n=9), by TTE + cMRI in 25% (n=3), of which 8% (n=1) with aditional CT angiography. Of the initial 21 patients, 33% (n=7) had medical treatment and 24% (n=5) had undergone surgery. Conclusion: As suggested by this study Turner Syndrome has an increased prevalence of cardiovascular disease, demanding a regular follow-up. Of the patients followed in this hospital, 91.7% (n=11) are receiving the recommended medical care according to recent guidelines. Further studies are required to properly reflect clinical practice.
Introduction and Objectives: Cardiovascular disease constitutes an important presentation of Turner Syndrome for its significant prevalence and impact on prognosis. This study aims to review the cardiovascular care in the Pediatrics Department of CHULN-HSM and propose a follow-up protocol in Pediatric Cardiology. Methods: Cross-sectional observational study based on a review of patient records with Turner Syndrome followed by Pediatric Endocrinology of CHULN - HSM between 01/01/2017 and 31/12/2021. Demographic data, data from Pediatric Cardiology consults, cardiac imaging and treatment were analysed. Results: A total of 21 patients was included, of which 19% (n=4) had 45,X karyotype and 57% (n=12) had a mosaic karyotype. Cardiovascular abnormalities were detected in 62% (n=13), of which BAV in 33% (n=7), aortic dilation in 29% (n=6), CoA in 19% (n=4), hypertension in 14% (n=3) and electrocardiographic abnormalities in 9.5% (n=2). All the patients had at least one cardiovascular evaluation, being referred after TS diagnosis by primary physician in 52% (n=11). Of these, 57% (n=12) were followed by Pediatric Cardiology of CHULN – HSM. A complete cardiovascular evaluation was conducted in average annually (0.68 consults/year), with imaging follow-up every 1-2 years in 50% (n=6). Cardiovascular imaging was performed by TTE in 75% (n=9), by TTE + cMRI in 25% (n=3), of which 8% (n=1) with aditional CT angiography. Of the initial 21 patients, 33% (n=7) had medical treatment and 24% (n=5) had undergone surgery. Conclusion: As suggested by this study Turner Syndrome has an increased prevalence of cardiovascular disease, demanding a regular follow-up. Of the patients followed in this hospital, 91.7% (n=11) are receiving the recommended medical care according to recent guidelines. Further studies are required to properly reflect clinical practice.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2022
Palavras-chave
Síndrome de Turner Alterações cardiovasculares Seguimento cardiovascular Cardiologia pediátrica Pediatria