Sustained Ventricular Tachycardia as the first presentation of transthyretin amyloid cardiomyopathy: a case report
Transthyretin Amyloid Cardiomyopathy (ATTR-CM) was considered an uncommon disease until a few years ago, but advances in the epidemiology and non-invasive diagnostic tests have increased its timely detection. We report a 71 years-old man with history of hypertension and an incidental carcinoma of th...
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| Formato: | Artículo revista |
| Lenguaje: | Inglés |
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Universidad Nacional Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología
2024
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| Acceso en línea: | https://revistas.unc.edu.ar/index.php/med/article/view/44893 |
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I10-R327-article-44893 |
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Universidad Nacional de Córdoba |
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I-10 |
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R-327 |
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Revista de la Facultad de Ciencias Médicas de Córdoba |
| language |
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| format |
Artículo revista |
| topic |
amyloidosis heart ventricular tachycardia amiloidosis corazón taquicardia ventricular amiloidose coração taquicardia arritmia |
| spellingShingle |
amyloidosis heart ventricular tachycardia amiloidosis corazón taquicardia ventricular amiloidose coração taquicardia arritmia Ramos, Hugo R. Sagripanti, Marcelo Sandrin, Ángel Balestrini, Lorena Balestrini, Victor Balestrini, Valeria Celorrio, Verónica Gigena, Adriana Coll, Marcelo Zelaya, Félix Quiroga Castro, Walter Conci, Eduardo C. Ramos, Hugo R. Ramos Sustained Ventricular Tachycardia as the first presentation of transthyretin amyloid cardiomyopathy: a case report |
| topic_facet |
amyloidosis heart ventricular tachycardia amiloidosis corazón taquicardia ventricular amiloidose coração taquicardia arritmia |
| author |
Ramos, Hugo R. Sagripanti, Marcelo Sandrin, Ángel Balestrini, Lorena Balestrini, Victor Balestrini, Valeria Celorrio, Verónica Gigena, Adriana Coll, Marcelo Zelaya, Félix Quiroga Castro, Walter Conci, Eduardo C. Ramos, Hugo R. Ramos |
| author_facet |
Ramos, Hugo R. Sagripanti, Marcelo Sandrin, Ángel Balestrini, Lorena Balestrini, Victor Balestrini, Valeria Celorrio, Verónica Gigena, Adriana Coll, Marcelo Zelaya, Félix Quiroga Castro, Walter Conci, Eduardo C. Ramos, Hugo R. Ramos |
| author_sort |
Ramos, Hugo R. |
| title |
Sustained Ventricular Tachycardia as the first presentation of transthyretin amyloid cardiomyopathy: a case report |
| title_short |
Sustained Ventricular Tachycardia as the first presentation of transthyretin amyloid cardiomyopathy: a case report |
| title_full |
Sustained Ventricular Tachycardia as the first presentation of transthyretin amyloid cardiomyopathy: a case report |
| title_fullStr |
Sustained Ventricular Tachycardia as the first presentation of transthyretin amyloid cardiomyopathy: a case report |
| title_full_unstemmed |
Sustained Ventricular Tachycardia as the first presentation of transthyretin amyloid cardiomyopathy: a case report |
| title_sort |
sustained ventricular tachycardia as the first presentation of transthyretin amyloid cardiomyopathy: a case report |
| description |
Transthyretin Amyloid Cardiomyopathy (ATTR-CM) was considered an uncommon disease until a few years ago, but advances in the epidemiology and non-invasive diagnostic tests have increased its timely detection. We report a 71 years-old man with history of hypertension and an incidental carcinoma of the left kidney detected 6 years ago, without heart failure who was performed cardiac magnetic resonance images (MRI) by suspicion of hypertrophic cardiomyopathy. Before his cardiologist be aware of the result, he suffered a severe sustained ventricular tachycardia (SVT) that required emergency cardioversion. Echocardiogram and cardiac MRI were suggestive for cardiac amyloidosis and the diagnosis was confirmed by scintigraphy with PYPTc99m (Perugini +3). Serum levels of light chains kappa and lambda were normal, and serum and urine immunofixation were negative; a genetic test had no variants, so supporting an ATTR-CM wild type. PET-CT did not detect metastasis of the renal tumor, but showed cardiac hypermetabolism and pericardial effusion. An implantable cardioverter defibrillator (ICD) was placed and after nine days a shock was delivered by the ICD due to a new event of SVT; in addition a Holter monitoring registered runs of asymptomatic atrial fibrillation. Etiologic treatment for ATTR-CM with Tafamidis 61 mg was started, amiodarone and rivaroxaban were added for control of arrhythmias and prevention of systemic embolism, respectively. After 14 months of follow-up, he is stable in class I NYHA. ATTR-CM is a complex disease, and the treatments should be indicated by a multidisciplinary team that consider the risks, benefits, and costs of each intervention. |
| publisher |
Universidad Nacional Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología |
| publishDate |
2024 |
| url |
https://revistas.unc.edu.ar/index.php/med/article/view/44893 |
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I10-R327-article-448932024-12-13T14:58:43Z Sustained Ventricular Tachycardia as the first presentation of transthyretin amyloid cardiomyopathy: a case report Taquicardia ventricular sostenida como primera manifestación de amiloidosis cardiaca por transtiretina: reporte de un caso Taquicardia ventricular sustentada como primeira manifestação de cardiomiopatia amioloide por transtiretina: relatório de um caso Ramos, Hugo R. Sagripanti, Marcelo Sandrin, Ángel Balestrini, Lorena Balestrini, Victor Balestrini, Valeria Celorrio, Verónica Gigena, Adriana Coll, Marcelo Zelaya, Félix Quiroga Castro, Walter Conci, Eduardo C. Ramos, Hugo R. Ramos amyloidosis heart ventricular tachycardia amiloidosis corazón taquicardia ventricular amiloidose coração taquicardia arritmia Transthyretin Amyloid Cardiomyopathy (ATTR-CM) was considered an uncommon disease until a few years ago, but advances in the epidemiology and non-invasive diagnostic tests have increased its timely detection. We report a 71 years-old man with history of hypertension and an incidental carcinoma of the left kidney detected 6 years ago, without heart failure who was performed cardiac magnetic resonance images (MRI) by suspicion of hypertrophic cardiomyopathy. Before his cardiologist be aware of the result, he suffered a severe sustained ventricular tachycardia (SVT) that required emergency cardioversion. Echocardiogram and cardiac MRI were suggestive for cardiac amyloidosis and the diagnosis was confirmed by scintigraphy with PYPTc99m (Perugini +3). Serum levels of light chains kappa and lambda were normal, and serum and urine immunofixation were negative; a genetic test had no variants, so supporting an ATTR-CM wild type. PET-CT did not detect metastasis of the renal tumor, but showed cardiac hypermetabolism and pericardial effusion. An implantable cardioverter defibrillator (ICD) was placed and after nine days a shock was delivered by the ICD due to a new event of SVT; in addition a Holter monitoring registered runs of asymptomatic atrial fibrillation. Etiologic treatment for ATTR-CM with Tafamidis 61 mg was started, amiodarone and rivaroxaban were added for control of arrhythmias and prevention of systemic embolism, respectively. After 14 months of follow-up, he is stable in class I NYHA. ATTR-CM is a complex disease, and the treatments should be indicated by a multidisciplinary team that consider the risks, benefits, and costs of each intervention. Cardiomiopatía Amiloide por Transtiretina (ATTR-CM) fue considerada hasta hace poco tiempo una enfermedad poco frecuente, pero los avances en el conocimiento de su epidemiología y de los tests no invasivos han aumentado su diagnóstico oportuno. Presentamos un hombre de 71 años con historia de hipertensión arterial y un tumor renal a células claras operado 6 años antes, sin insuficiencia cardíaca a quien se realizó una resonancia magnética cardiaca por sospecha de miocardiopatía hipertrófica; antes de que su cardiólogo viera el resultado, presentó una taquicardia ventricular sostenida (TVS) severa que requirió cardioversión eléctrica de urgencia. Ecocardiograma y resonancia magnética cardiaca fueron sugestivos y el diagnóstico fue confirmado por centellografía con PYPTc99m (Perugini +3). Cadenas livianas kappa y lambda en suero e inmunofijación en sangre y orina fueron negativas y el test genético no mostró variantes, confirmando ATTR-CMwt. PET-CT no mostró metastasis del tumor renal pero detectó hipermetabolismo miocárdico y derrame pericárdico. Se colocó un cardio-desfibrilador implantable (CDI) y nueve días después tuvo una nueva TVS que fue detectada y tratada adecuadamente por el CDI. Además, el monitoreo Holter detectó eventos asintomáticos de fibrilación auricular. Se inició tratamiento etiológico de ATTR-CM con Tafamidis 61 mg y se agregó amiodarona para prevenir nuevos eventos de TVS y rivaroxaban para prevención de embolismo sistémico. A 14 meses de seguimiento el paciente permanece en clase I de NYHA. CM-ATTR es una enfermedad compleja y los tratamientos deberían ser indicados por un equipo multidisciplinario que considere los riesgos, beneficios y costos de cada intervención. 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. Universidad Nacional Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología 2024-12-13 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion text/html application/pdf https://revistas.unc.edu.ar/index.php/med/article/view/44893 10.31053/1853.0605.v81.n4.44893 Revista de la Facultad de Ciencias Médicas de Córdoba.; Vol. 81 No. 4 (2024); 768-782 Revista de la Facultad de Ciencias Médicas de Córdoba; Vol. 81 Núm. 4 (2024); 768-782 Revista da Faculdade de Ciências Médicas de Córdoba; v. 81 n. 4 (2024); 768-782 1853-0605 0014-6722 10.31053/1853.0605.v81.n4 eng https://revistas.unc.edu.ar/index.php/med/article/view/44893/47185 https://revistas.unc.edu.ar/index.php/med/article/view/44893/47595 Derechos de autor 2024 Universidad Nacional de Córdoba https://creativecommons.org/licenses/by-nc/4.0 |