Sustained Ventricular Tachycardia as the first presentation of transthyretin amyloid cardiomyopathy: a case report

Hugo R. Ramos; Marcelo Sagripanti; Ángel Sandrin; Lorena Balestrini; Victor Balestrini; Valeria Balestrini; Verónica Celorrio; Adriana Gigena; Marcelo Coll; Félix Zelaya; Walter Quiroga Castro; Eduardo C. Conci

doi:10.31053/1853.0605.v81.n4.44893

Autores

Hugo R. Ramos Instituto Modelo de Cardiología. División Cardiología
Marcelo Sagripanti Instituto Modelo de Cardiología. División Cardiología
Ángel Sandrin Instituto Modelo de Cardiología. División Cardiología
Lorena Balestrini Instituto Modelo de Cardiología. División Cardiología
Victor Balestrini Instituto Modelo de Cardiología. División Cardiología
Valeria Balestrini Instituto Modelo de Cardiología. División Cardiología
Verónica Celorrio Instituto Modelo de Cardiología. División Cardiología
Adriana Gigena Instituto Modelo de Cardiología. División Cardiología
Marcelo Coll Instituto Modelo de Cardiología. División Cardiología
Félix Zelaya Instituto Modelo de Cardiología. División Cardiología
Walter Quiroga Castro Instituto Modelo de Cardiología. División Cardiología
Eduardo C. Conci Instituto Modelo de Cardiología. División Cardiología

DOI:

https://doi.org/10.31053/1853.0605.v81.n4.44893

Palavras-chave:

amiloidose, coração, taquicardia, arritmia

Resumo

A Cardiomiopatia Amiloide por Transtiretina (ATTR-CM) foi considerada até há pouco tempo, uma doença pouco frequente, mas os avanços no conhecimento da sua epidemiologia e dos testes não invasivos aumentaram o diagnóstico oportuno. Apresentamos um caso de um homem de 71 anos com antecedentes de hipertensão arterial e tumor renal a células claras com cirurgia de 6 anos de data, sem insuficiência cardíaca. Realizou-se ressonância magnética cardíaca por suspeita de miocardiopatia hipertrófica. Antes da revisão do estudo pelo seu cardiologista, o paciente apresentou taquicardia ventricular sustentada (TVS) severa que exigiu cardioversão eléctrica de emergência. Ecocardiograma e ressonância magnética foram sugestivas e o diagnostico foi confirmado com cintilografia com PYPTc99m (Perugini +3). Correntes claras kappa e lambda em souro e imunofixação em sangue e urina foram negativos e o teste genético não amostrou variações, confirmando ATTR-CM wild type. O PET-CT não mostraram metátese do tumor renal, mas detecto hipermetabolismo miocárdico e derrame pericárdico. Colocou-se um cardio-desfibrilador implantável (CDI) e nove dias depois apresentou uma nova TVS que foi detectada e tratada adequadamente pelo CDI. Além disso, o monitoreo-Holter detectou eventos assintomáticos de fibrilação auricular. Começou-se um tratamento etiológico de ATTR-CM com Tafamidis 61 mg e se adicionou amiodarona para prevenir novos eventos de TVS e rivaroxaban para prevenção de embolismo sistémico. Após 14 meses de seguimento, o paciente continua em tipo I de NYHA. CM-ATTR e uma doença complexa e os tratamentos deveriam ser indicados por uma equipe multidisciplinar que considere os riscos, benefícios e custos de cada intervenção.

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Biografia do Autor

Hugo R. Ramos, Instituto Modelo de Cardiología. División Cardiología

M.D., Ph.D., from the Universidad Nacional de Córdoba (UNC). Associate Professor of Internal Medicine in the UNC. Cardiologist, Coordinator of the Amyloidosis Group, Instituto Modelo de Cardiología de Córdoba. Field of research: cardiac biomarkers, ischemic heart disease, heart failure, cardiac amyloidosis.
Marcelo Sagripanti, Instituto Modelo de Cardiología. División Cardiología

M.D. from the Universidad Nacional de Córdoba. Cardiologist, Instituto Modelo de Cardiología de Córdoba.
Ángel Sandrin, Instituto Modelo de Cardiología. División Cardiología

M.D. from the Universidad Nacional de Córdoba. Cardiologist, Instituto Modelo de Cardiología de Córdoba.
Lorena Balestrini, Instituto Modelo de Cardiología. División Cardiología

M.D. from the Universidad Católica de Córdoba. Head of the Section of Echocardiography, Instituto Modelo de Cardiología de Córdoba. Field of research: echocardiography, pediatric cardiology.
Victor Balestrini, Instituto Modelo de Cardiología. División Cardiología

M.D. from the Universidad Nacional de Córdoba. Head of the Section of Nuclear Cardiology, Instituto Modelo de Cardiología de Córdoba. Field of research: nuclear cardiology, ischemic heart disease, cardiac amyloidosis.
Valeria Balestrini, Instituto Modelo de Cardiología. División Cardiología

M.D. from the Universidad Católica de Córdoba. Section of Nuclear Cardiology, Instituto Modelo de Cardiología de Córdoba. Field of research: nuclear cardiology, ischemic heart disease, cardiac amyloidosis.
Verónica Celorrio, Instituto Modelo de Cardiología. División Cardiología

M.D. from the Universidad Nacional de Córdoba. Section of Echocardiography and Cardiac Magnetic Resonance Images, Instituto Modelo de Cardiología de Córdoba. Field of research: echocardiography, cardiac magnetic resonance images.
Adriana Gigena, Instituto Modelo de Cardiología. División Cardiología

M.D. from the Universidad Nacional de Córdoba. Section of Echocardiography, Instituto Modelo de Cardiología de Córdoba. Field of research: echocardiography.
Marcelo Coll, Instituto Modelo de Cardiología. División Cardiología

M.D. from the Universidad Nacional de Córdoba. Expert in Electrophysiology by the Consejo de Médicos de la Provincia de Córdoba. Head of the Section of Arrhythmias and Electrophysiology, Instituto Modelo de Cardiología de Córdoba. Field of research: arrhythmias.
Félix Zelaya, Instituto Modelo de Cardiología. División Cardiología

M.D. from the Universidad Nacional de Tucumán. Head of the Intensive Care Unit, Coordinator Acute Heart Failure Program, Instituto Modelo de Cardiología de Córdoba. Field of research: ischemic heart disease, heart failure.
Walter Quiroga Castro, Instituto Modelo de Cardiología. División Cardiología

M.D. from the Universidad Nacional de Córdoba. Intensive Care Unit, Coordinator of the Chest Pain Unit, Instituto Modelo de Cardiología de Córdoba. Field of research: chest pain, ischemic heart disease.
Eduardo C. Conci, Instituto Modelo de Cardiología. División Cardiología

M.D. from the Universidad Católica de Córdoba. Chairman of the Division of Cardiology, Instituto Modelo de Cardiología de Córdoba. Field of research: ischemic heart disease, cardiac amyloidosis.

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