Objetivo: Avaliar a presença de malformações cardiovasculares em 33 pacientes com síndrome de Turner acompanhadas no Hospital de Clinicas de Porto Alegre. Métodos: Estudo transversal no qual foi realizada ressonância magnética cardíaca (RMC) e torácica com ênfase na avaliação da aorta (RMA) em 33 pacientes. Resultados: As pacientes tinham idade media de 20 anos e 10 meses e altura de 138,7 cm. Aproximadamente 42,42% das pacientes apresentavam cariótipo 45,X e 33,33% pescoço alado. À RMC, 54,54% das pacientes apresentaram alteração, sendo válvula aórtica bicúspide o achado mais frequente, presente em 24,24%. À RMA, malformações cardiovasculares foram encontradas em 42,42%, sendo o alongamento do arco transverso presente em 27,27% das pacientes. Dilatação da aorta estava presente em 66,66% das pacientes, considerada severa em 12,12%. O local mais frequente de dilatação foi na raiz da aorta e na porção tubular da aorta ascendente. Conclusões: Os resultados do presente estudo corroboram achados preliminares da literatura que demonstram que anomalias cardiovasculares são comuns na síndrome de Turner, especialmente diagnosticadas pela ressonância magnética. Dilatação da aorta, principalmente na aorta ascendente, e extremamente frequente na síndrome de Turner e proporciona elevado risco de evento aórtico agudo como a dissecção, potencialmente fatal. / Objective: To evaluate the incidence of cardiovascular malformations in 33 patients with Turner's syndrome followed up in the Hospital de Clinicas of Porto Alegre. Methodology: A transversal study in which a Cardiac and Thoracic Magnetic Resonance Imaging (MRI ) with focus on the evaluation of the aorta was performed in 33 patients. Results: The patients had average age of 20 years and 10 months and height of 138,7 cm. Approximately 42,42% of the patients presented karyotype 45,X and 33,33% webbed neck. Through MRI 54,54% of patients showed anomalies; the bicuspid aortic valve was the most frequent found present in 24,24% of patients. Through MRI, cardiovascular malformations were found in 42,42%, and elongation of the transverse arch was present in 27,27% of patients. Aortic dilatation was found in 66,66% of patients, and it was considered severe in 12,12%. The most frequent place of dilatation was in the aortic root and in the tubular portion of the ascending thoracic aorta. Conclusion: The results of present study corroborate with the literature that says cardiovascular anomalies are common in Turner's syndrome, especially diagnosed through magnetic resonance imaging. Aortic dilatation, most prominent in the ascending aorta, is very frequent in Turner's syndrome and that predicts high risk for acute aortic events such as dissection, potencially fatal.
| Identifer | oai:union.ndltd.org:IBICT/oai:lume.ufrgs.br:10183/17899 |
| Date | January 2009 |
| Creators | Pereira, Betina Feijó |
| Contributors | Czepielewski, Mauro Antonio |
| Source Sets | IBICT Brazilian ETDs |
| Language | Portuguese |
| Detected Language | English |
| Type | info:eu-repo/semantics/publishedVersion, info:eu-repo/semantics/masterThesis |
| Format | application/pdf |
| Source | reponame:Biblioteca Digital de Teses e Dissertações da UFRGS, instname:Universidade Federal do Rio Grande do Sul, instacron:UFRGS |
| Rights | info:eu-repo/semantics/openAccess |
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