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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Autores principales: 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
Formato: Artículo revista
Lenguaje:Inglés
Publicado: 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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Sumario: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.