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Curso de Atualização em Medicina Cardiovascular 2019
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Late onset hypertrophic cardiomyopathy – same disease or disguised impostor?
Session:
Posters 4 - Écran 4 - Doenças do Miocárdio
Speaker:
Luís Puga
Congress:
CPC 2019
Topic:
F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme:
17. Myocardial Disease
Subtheme:
17.1 Myocardial Disease – Pathophysiology and Mechanisms
Session Type:
Posters
FP Number:
---
Authors:
Luís Puga; Rogerio Teixeira; Liliana Reis; Alexandrina Siserman; José Pedro Sousa; Diana Decampos; João Gameiro; Carolina Saleiro; Joana M. Ribeiro; Lino Gonçalves
Abstract
<p><strong>Background</strong>: Hypertrophic cardiomyopathy (HCM) frequently develops in teenage or early adulthood but rarely it can be diagnosed in older ages. Cardiac amyloid deposition in elderly patients is gaining increased recognition with new imaging modalities.</p> <p><strong>Purpose</strong>: To evaluate phenotype and outcomes in patients with late HCM diagnosis. </p> <p><strong>Methods</strong>: Single-centre retrospective cohort consisting of 106 patients attending a tertiary hospital with the diagnose of HCM. The sample was divided in 2 groups, A (n=65) age of diagnosis < 65 years old and B (n=41) age of diagnosis ≥ 65 years old. The groups were compared for clinical, phenotypic characteristics (echocardiographic and cardiac magnetic resonance (CMR) data) and outcomes (implantable cardiac defibrillator (ICD), ventricular tachycardia, death, ICD shocks).</p> <p><strong>Results</strong>: The sample included of 47% (50) males, mean age of 66±17 years old. Regarding the echocardiographic variables, both groups were homogeneous. Mean interventricular septum thickness was similar (A 16.9±5.0mm vs B 17.0±5.0mm, <em>P</em>=0.7), as was the left ventricular (LV) mass index, left atrium volume, and LV obstrution. Regarding CMR phenotype the groups were similar for LV ejection fraction, maximum wall thickness, presence of late gadolinium enhancement), but there was a trend for increased LV mass index in group A patients (A 91±40g/m2 vs B 68±44g/m2, <em>P</em>=0.074). HCM Risk-SCD score was significantly higher in group A (A 3.8±3.0 vs B 2.0±1.0, <em>P</em><0.01). Family history of sudden cardiac death was also higher in group A (A 17/64 (27%) vs B 1/41 (2%), <em>P</em><0.01). Regarding clinical outcomes, the rate of ventricular tachycardia during follow up was equal (A 14/62 (23%) vs B 7/38 (18%), <em>P</em><0.62). Mortality was higher in group B (A 2/63 (3%) vs B 8/40 (20%), <em>P</em><0.01), and ICD implant was higher in group A (19/65 (29%) vs B 5/40 (13%), p<0,047) althoug ICD shocks were similar between groups (A 1/24 (4,2%) vs B 0/12 (0%), <em>P</em><0.473)</p> <p><strong>Conclusions</strong>: Late onset HCM patients had a similar phenotype, a lower estimated risk of sudden cardiac death but a worse prognosis.</p>
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