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The risk of cardiac hospitalization and arrythmias in patients with arrhythmogenic and dilated cardiomyopathies
Session:
Posters (Sessão 4 - Écran 7) - Miocardiopatias hereditárias
Speaker:
Miguel Marques Antunes
Congress:
CPC 2023
Topic:
F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme:
17. Myocardial Disease
Subtheme:
17.7 Myocardial Disease - Other
Session Type:
Pósters Electrónicos
FP Number:
---
Authors:
Miguel Marques Antunes; Isabel Cardoso; Diana Antunes; André Ferreira; José Viegas; Pedro Brás; Sílvia Aguiar Rosa; Rui Cruz Ferreira
Abstract
<p><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Background: Patients (P) with left ventricle (LV) or bi-ventricular (BiV) arrhythmogenic cardiomyopathy (ACM) have been previously misclassified as having dilated cardiomyopathy (DCM), prior to the recognition of LV/BiV ACM as an independent clinical entity. This differentiation has been made possible with the advent of genetics, and while these CMs share a common pathway of LV disfunction, there is a growing notion that clinical outcomes differ among them, given the pathophysiological subtract behind each condition.</span></span><br /> <span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Objectives: To evaluate the difference in clinical outcomes between patients with genetically confirmed LV/BiV ACM and DCM.</span></span><br /> <span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Methods: We conducted a retrospective analysis of all P with a clinical diagnosis of DCM and LV or Bi-V ACM followed in a cardiomyopathy clinic. These patients had to have a positive genetic test compatible with either disease phenotype and an identified pathologic or likely pathologic mutation. All patients without a genetic test or with a VUS were excluded. ACM was diagnosed according to the 2020 Padua Criteria. P characteristics, echocardiographic data, holter monitoring and clinical history were extracted. Outcomes consisted non-elective cardiovascular hospitalization, a composite of ventricular arrhythmic events (NSVT, VT and FV), transplantation and death. Chi-squared and Wilcoxon signed rank tests were used to determine inter-group differences. Logistical regression was used for binomial outcome assessment with derivation of the Odds-Ratios (OR) with 95%CI.</span></span><br /> <span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Results: 27P were included, 17 with DCM and 10 with ACM diagnosis, 7 of which had isolated LV ACM. Median P age was 45 IQR (30-52), with 58% of patients being male. Patients with ACM had more baseline atrial fibrillation (50 vs 11%, p=0,029). Average LVEF assessed by echocardiography was 50%±11, being 4.5% higher (p=NS) and average heart rate assessed by Holter monitoring was 4.5% lower (p=NS) in the ACM arm. General inter-group characteristics and pharmacotherapy are depicted in Table 1. In the ACM group there were 5 LMNA, 2 PKP2, 1 DSP, 1 FLNC and 1 MYBPC3 P. In the MCD group there were 12 TTN, 3 MYBPC3 and 2 MYH7 P. 8 patients (80%) in the MCA group and 6 (35%) in the DCM group had an ICD. There were 5 appropriate ICD therapies in the MCA group and 2 in the DCM group, with 1 additional inappropriate therapy. </span></span><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">There were no deaths in this cohort, and 1 patient with ACM was transplanted. Cardiac hospitalization was significantly increased in P with ACM (OR 6.3, 95%CI 1–38) as well as incident ventricular arrythmias (OR 7, 95% 1.8–50) when compared to DCM P.</span></span><br /> <span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Conclusion: In cohort of clinically and genetically confirmed DCM and LV/BiV ACM P, individuals with LV/BiV ACM had a significantly higher arrhythmic clinical burden and had a greater risk for hospitalization for cardiac cause in spite of similar ventricular function. This underscores the need for early genetic testing to identify these at-risk individuals.</span></span></p>
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