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Abstract(s)
A Displasia Arritmogénica do Ventrículo Direito (DAVD) é uma doença do miocárdio, de etiologia ainda desconhecida, que afecta maioritariamente o ventrículo direito (VD) e é caracterizada histologicamente por substituição dos miócitos por tecido fibroso e adiposo.
Clinicamente, manifesta-se sobretudo em adultos jovens sob a forma de arritmias ventriculares, síncope e/ou morte súbita, que caracteristicamente ocorrem, mais frequentemente, com o exercício físico. A prevalência varia amplamente segundo as séries descritas, provavelmente reflectindo uma distribuição geográfica muito heterogénea.
Pacientes diagnosticados com DAVD são candidatos a uma abordagem terapêutica activa, que inclui procedimentos electrofisiológicos invasivos e colocação de Cardioversor Implantável Desfibrilhador (CID). Os pacientes que sofrem de tempestades arrítmicas frequentes, com esgotamentos energéticos do CID e alguns casos que evoluem para insuficiência cardíaca congestiva grave, podem ser candidatos a transplante cardíaco.
No caso clínico descrito, o paciente apresenta não só múltiplas características, como também sintomatologia compatível com DAVD.
Arrythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) is a myocardial illness whose etiology is still unknown, which affects mostly the right ventricle and is histologically characterized by the replacement of myocytes by fibrous and adipose tissue. Clinically it appears mainly in young adults with episodes of arrhytmias, tachycardia, syncope and/or sudden death which usually occur related to physical exercise. According to the described series, the prevalence is widely varied, probably reflecting the geographic distribution of this pathology. The patients diagnosed with ARVD are candidates to an active therapeutic approach, which includes invasive electro physiological procedures and the placement of Implantable Cardioverter-Defribrillators (ICD). Patients who suffer frequent arrhythmic storms, with energetic break downs of ICD and some cases that envolve to severe congestive heart failure, may be candidates to cardiac transplant. In the described clinical case the patient not only presents multiple characteristics but also ARVD compatible symptomatology.
Arrythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) is a myocardial illness whose etiology is still unknown, which affects mostly the right ventricle and is histologically characterized by the replacement of myocytes by fibrous and adipose tissue. Clinically it appears mainly in young adults with episodes of arrhytmias, tachycardia, syncope and/or sudden death which usually occur related to physical exercise. According to the described series, the prevalence is widely varied, probably reflecting the geographic distribution of this pathology. The patients diagnosed with ARVD are candidates to an active therapeutic approach, which includes invasive electro physiological procedures and the placement of Implantable Cardioverter-Defribrillators (ICD). Patients who suffer frequent arrhythmic storms, with energetic break downs of ICD and some cases that envolve to severe congestive heart failure, may be candidates to cardiac transplant. In the described clinical case the patient not only presents multiple characteristics but also ARVD compatible symptomatology.
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Keywords
Arritmia Cardiomiopatia Taquicardia Insuficiência cardíaca Miocárdio - Displasia arritmogénica Ventrículo direito - Displasia
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Universidade da Beira Interior