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How to distinguish between hypertrophic cardiomyopathy and left ventricular hypertrophy secondary to Fabry disease?
Session:
CO 07 - Miocardiopatias Infiltrativas
Speaker:
Raquel Menezes Fernandes
Congress:
CPC 2021
Topic:
F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme:
17. Myocardial Disease
Subtheme:
17.3 Myocardial Disease – Diagnostic Methods
Session Type:
Comunicações Orais
FP Number:
---
Authors:
Raquel Menezes Fernandes; Olga Azevedo; Filipa Cordeiro; Mário Rui Lourenço; 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) commonly leads to left ventricular hypertrophy (LVH) that could mimic<strong> </strong>sarcomeric<strong> </strong>hypertrophic cardiomyopathy (HCM).</span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>Purpose:</strong> To determine the differences in echocardiographic parameters between FD patients with LVH and HCM patients.</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 strain analysis by two-dimensional speckle tracking imaging. Demographic, clinical characteristics and echocardiographic parameters were analysed. FD patients with LVH were compared with HCM patients, using Chi-square test for categorical variables and Student’s T-test for continuous variables. 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 a median age of 51 years-old and 62,6% of female predominance. 16,5% of patients were under enzymatic replacement therapy with agalsidase alpha and 7,7% were treated with chaperone therapy (migalastat). 33 FD patients (36%) had LVH and were older than HCM patients (63,6 vs 59,3 years-old; p=0,106). FD patients with LVH had lower interventricular septum (IVS) thickness (16,4 vs 19,6 mm, p<0,001), IVS/posterior wall ratio (1,3 vs 1,8, p<0,001) and left atrial volume index (34,45 vs 42,2 ml/m2; p=0,014). Left ventricle mass index was similar between the two groups (157,7 vs 155,5 g/m<sup>2</sup>; p=0,819), with lower left ventricular ejection fraction in FD patients (64,5% vs 70,5%; p<0,001). There were no significant differences in global longitudinal strain (-15,6% vs 15,9%; p=0,687), global circumferential strain (-19,9% vs -21,1%; p=0,218) and global radial strain (35,3% vs 33,7%; p=0,623). Interestingly, FD patients had lower base-to-apex circumferential strain gradient (5,7% vs 9,1%; p=0,002) and lower twist (17,5º vs 26,1º; p=0,001) than HCM patients. No significant differences were reported regarding mechanical dispersion (72,4 vs 71,2 ms; p=0,841). </span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>Conclusion: </strong>The pattern of LVH is different between FD and HCM patients. In our study, we revealed that base-to-apex circumferential strain gradient and twist are echocardiographic parameters that could help distinguish both entities.</span></span></p>
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