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Twist, left ventricular longitudinal and circumferential strain are early markers of cardiac involvement in 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) is an X-linked progressive and multisystemic disease. Cardiac involvement is common and left ventricular hypertrophy (LVH) is the main cardiac manifestation.</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 without LVH and healthy controls.</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 without LVH were compared with healthy controls, 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). 58 patients (64%) did not present LVH. FD patients without LVH were younger (44,2 vs 52,9 years-old, p<0,001), had higher interventricular septum thickness (9,3 vs 8,4 mm, p=0,006) and left ventricular mass index (77,2 vs 68,4 g/m2, p=0,003) than healthy controls. Left ventricular ejection fraction was preserved in both groups (63,3% vs 65,4%; p=0,067). Global longitudinal strain (-19,6% vs -20,9%; p=0,003) and global circumferential strain (-17,9% vs -20,9%; p<0,001) were significantly lower in FD patients without LVH compared to healthy controls. Global radial strain was also lower, although without statistical significance (36,4% vs 41,4%, p=0,058). FD patients without LVH presented a lower base-to-apex circumferential strain gradient (5,7% vs 7,7%; p=0,035), but a higher base-to-apex longitudinal strain gradient (7,5% vs 4,3%; p<0,001), compared to controls. Left ventricular twist was also significantly lower in FD patients without LVH (13,8º vs 21,7º, p<0,001).</span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>Conclusion:</strong> Left ventricular strain and twist analysis are useful to identify subclinical myocardial impairment in FD patients without LVH.</span></span></p>
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