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Embolia paradoxal na Anomalia de Ebstein
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Resumo(s)
A anomalia de Ebstein corresponde a um conjunto de malformações cardíacas congénitas baseadas essencialmente em torno de alterações estruturais e funcionais da válvula tricúspide, de onde se destacam a adesão dos folhetos posterior e septal da válvula ao miocárdio subjacente bem como a inserção distal do anel tricúspide. Estas alterações condicionam a formação de uma região “auricularizada” no ventrículo direito, diminuindo a sua capacidade de reserva e funcional. O fenótipo de malformações varia de doente para doente, existindo casos publicados onde se descrevem outras alterações como a existência de comunicação interauricular, defeitos do septo interventricular, entre outros.
A embolia paradoxal é um fenómeno que ocorre em doentes com anomalia de Ebstein e comunicação interauricular, pela criação de um gradiente de pressões que favorece o shunt direito-esquerdo.
Nesta revisão serão abordados os mecanismos fisiopatológicos da anomalia de Ebstein bem como as suas características clínicas, diagnóstico e tratamento. Será ainda feita uma revisão sobre a embolia paradoxal no contexto de doentes com a referida anomalia, recorrendo à análise crítica de casos clínicos disponíveis na literatura.
Ebstein’s anomaly is caracterized by various congenital cardiac malformations based essentially on structural and functional alterations of the tricuspid valve, such as adherence of the septal and posterior leaflets to the underlying myocardium and distal insertion of the tricuspid annulus. These alterations lead to the formation of an “atrialized” region of the right ventricule, with fewer functional and reserve capacities. The fenotipe of the disease may vary within patients, as there are case reports where the authors describe alterations such as atrial septal defects, ventricular septum defects, among others. Paradoxical embolism is a phenomenon that occurs in patients suffering from Ebstein’s anomaly and atrial septal defect, as they favor the formation of a pressure gradient that can end up in atrial right-to-left shunting. In this review we will discuss the pathophysiological mecanisms of Ebstein’s anomaly as well as its clinical features, diagnosis and treatment. We will also review the major aspects about paradoxical embolism on patients with Ebstein’s anomaly, based on a critical analysis of the case reports published in the literature.
Ebstein’s anomaly is caracterized by various congenital cardiac malformations based essentially on structural and functional alterations of the tricuspid valve, such as adherence of the septal and posterior leaflets to the underlying myocardium and distal insertion of the tricuspid annulus. These alterations lead to the formation of an “atrialized” region of the right ventricule, with fewer functional and reserve capacities. The fenotipe of the disease may vary within patients, as there are case reports where the authors describe alterations such as atrial septal defects, ventricular septum defects, among others. Paradoxical embolism is a phenomenon that occurs in patients suffering from Ebstein’s anomaly and atrial septal defect, as they favor the formation of a pressure gradient that can end up in atrial right-to-left shunting. In this review we will discuss the pathophysiological mecanisms of Ebstein’s anomaly as well as its clinical features, diagnosis and treatment. We will also review the major aspects about paradoxical embolism on patients with Ebstein’s anomaly, based on a critical analysis of the case reports published in the literature.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2019
Palavras-chave
Ebstein’s anomaly Paradoxical embolism Emboli Congenital Heart Disease Cardiology