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

Authors

M.D., Ph.D. Instituto Modelo de Cardiología. División Cardiología
M.D. Instituto Modelo de Cardiología. División Cardiología
M.D. Instituto Modelo de Cardiología. División Cardiología
M.D. Instituto Modelo de Cardiología. División Cardiología
M.D. Instituto Modelo de Cardiología. División Cardiología
M.D. Instituto Modelo de Cardiología. División Cardiología
M.D. Instituto Modelo de Cardiología. División Cardiología
M.D. Instituto Modelo de Cardiología. División Cardiología
M.D. Instituto Modelo de Cardiología. División Cardiología
M.D. Instituto Modelo de Cardiología. División Cardiología
M.D. Instituto Modelo de Cardiología. División Cardiología
M.D. Instituto Modelo de Cardiología. División Cardiología

DOI:

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

Keywords:

amyloidosis, heart, ventricular, tachycardia

Abstract

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.

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Author Biographies

M.D., Ph.D., 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.
M.D., Instituto Modelo de Cardiología. División Cardiología

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

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