Sustained Ventricular Tachycardia as the first presentation of transthyretin amyloid cardiomyopathy

• Published in: Revista de la Facultad de Ciencias Médicas de Córdoba 2024-12, Vol.81 (4)
• Main Authors: 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
• Format: Article
• Language: English
• Subjects: amyloidosis; heart; tachycardia; ventricular
• ISSN: 0014-6722; 1853-0605
• DOI: 10.31053/1853.0605.v81.n4.44893

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.