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Mechanical dispersion: can it predict the occurrence of ventricular arrhythmias in Fabry disease?
Session:
Comunicações Orais (Sessão 29) - Doenças do Miocárdio e Pericárdio 2 - Vários Tópicos
Speaker:
Raquel Menezes Fernandes
Congress:
CPC 2022
Topic:
F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme:
17. Myocardial Disease
Subtheme:
17.2 Myocardial Disease – Epidemiology, Prognosis, Outcome
Session Type:
Comunicações Orais
FP Number:
---
Authors:
Raquel Menezes Fernandes; Olga Azevedo; Filipa Cordeiro; António Lourenço
Abstract
<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>Introduction: </strong>Fabry disease (FD) is an X-linked progressive and multisystemic disease, that could lead to left ventricular hypertrophy (LVH). Mechanical dispersion (MD) has been associated to a risk of ventricular arrhythmias and sudden death in several cardiomyopathies.</span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>Purpose: </strong>To evaluate MD and arrhythmic burden in FD patients with and without LVH.</span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>Methods: </strong>We conducted a prospective study encompassing FD patients followed in a Reference Center of Lysosomal Storage Disorders. All patients performed a complete echocardiographic evaluation, including left ventricular (LV) strain analysis by two-dimensional speckle tracking imaging. Clinical characteristics, echocardiographic parameters and 24h-holters results were analysed. FD patients were divided in two groups according to the presence or absence of LVH. Chi-square test and Student’s T-test were applied for categorical and continuous variables, respectively. The significance level was 0,05. </span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>Results: </strong>A total of 91 FD patients were included, with female predominance (62,6%) and a LVH prevalence of 36,3%. Patients with LVH were older (63,6 vs 44,2 years-old; p<0,001), were predominantly male (60,6% vs 24,1%; p=0,001), and had a larger prevalence of arterial hypertension (63,6% vs 10,3%; p<0,001) and stroke (9,1% vs 0%; p=0,02). In FD patients with LVH, mean interventricular septum thickness was 16,4 mm, mean LV mass index was 157,7 g/m<sup>2</sup>, mean left atrial volume index was 34,4 ml/m<sup>2</sup> and mean E/e’ was 12,6 with 63,6% of these patients having diastolic dysfunction. Global longitudinal strain (GLS) was lower in patients with LVH (-15,6% vs -19,6%; p<0,001), with a higher base-to-apex longitudinal strain gradient (9,3% vs 6,5%; p=0,013). Global radial and circumferential strains were not statistically different between both groups. Interestingly, twist was higher in patients with LVH (17,5º vs 13,8º) despite the lower GLS, but this finding did not reach statistical significance (p=0,077). MD was higher in patients with LVH (72,4 vs 38,3 ms; p<0,001), as well as the prevalence of premature ventricular contractions (PVC) (93,8% vs 61,8%; p=0,001) and non-sustained ventricular tachycardia (NSVT) (18,8% vs 9,1%; p=0,191). Patients with higher frequency of PVC had higher MD (55,2 vs 35,6 ms; p=0,001) and the same was verified for NSVT (65 vs 48,6 ms; p=0,054), despite not reaching statistical significance.</span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>Conclusion: </strong>FD patients with LVH present lower GLS, with a higher base-to-apex longitudinal strain gradient and higher MD, compared to patients without LVH. Both PVC and NSVT were significantly associated with LVH and higher MD, but the latter did not reach statistical significance.</span></span></p>
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