Virtual Conference
Heart 2022 Conference

Júlia Dalegrave Marques

Universidade Nove de Julho, Brazil

Title: Cardiac amyloidosis attr: a case report with diagnosis without the need for invasive examination

Abstract

Introduction: Transthyretin amyloidosis (ATTR) is a rare cause of cardiomyopathy with a restrictive pattern that causes thickening and stiffness of the ventricular wall, coursing with heart failure (HF). Case report: Male patient, 83 years old, with systemic arterial hypertension, persistent atrial fibrillation with CHA2DS2VASC4 (no anticoagulation due to previous hematuria) and reduced ejection fraction HF. It presents the following examination findings: echocardiogram with left ventricular hypertrophy with EF of 30% at the expense of diffuse hypokinesia. The right ventricle showed mild hypokinesia and moderate tricuspid regurgitation with pulmonary systolic pressure of 49 mmHg. He had cardiac resonance with late circumferential enhancement, mainly affecting the subendocardial region of the left ventricle. Due to the suspicion of cardiac amyloidosis, protein electrophoresis and serum and urinary immunofixation were requested, with a search for light chains, and an associated component of gammopathy was ruled out. The investigation continued with pyrophosphate scintigraphy, which showed an increased concentration of the radiopharmaceutical in the cardiac area (grade 3), and the quantitative evaluation of the 1-hour images demonstrates a ratio of counts in the heart/contralateral area equal to 1.72 and at 3 hours the ratio of 1.86, being highly suggestive of ATTR amyloidosis. Due to the cost of ATTR amyloidosis-specific medications, the patient followed only with reduced ejection fraction HF treatment. 
Discussion: ATTR amyloidosis is an infiltrative disease caused by the abnormal unfolding of the transthyretin protein, affecting mainly elderly individuals. The diagnosis is usually made late, since it is a pathology whose clinical manifestations are not very specific, making it difficult to obtain an accurate opinion based only on the clinic, and are often ignored or confused with other diseases. Currently, it is possible to carry out the diagnosis without invasive tests, as is the case of patients who do not have a light chain or M protein found in immunofixation or serum and who have a positive bone tracer scintigraphy in a non-invasive way earlier and, thus, institute treatment. appropriate in a timely manner. 
Conclusion: The earlier diagnosis, the characterization of the type of amyloidosis, and the institution of a therapy are essential for a more favorable prognostic evolution of this pathology

Biography

Julia Dalegrave Marques is a medical student