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Stages of Cardiac Involvement in pre-hypertrophic Fabry Disease: an integrated electrocardiographic and cardiovascular magnetic resonance approach
Session:
Sessão de Comunicações Orais - Doenças do Miocárdio
Speaker:
João Bicho Augusto
Congress:
CPC 2020
Topic:
F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme:
17. Myocardial Disease
Subtheme:
17.1 Myocardial Disease – Pathophysiology and Mechanisms
Session Type:
Comunicações Orais
FP Number:
---
Authors:
João Bicho Augusto; Nicolas Johner; Dipen Shah; Sabrina Nordin; Kristopher Knott; Stefania Rosmini; Clement Lau; Mashael Alfarih; Rebecca Hughes; Andreas Seraphim; Ravi Vijapurapu; Anish Bhuva; Linda Lin; Natalia Ojrzynska; Tarekegn Geberhiwot; Gabriella Captur; Uma Ramaswami; Richard P Steeds; Rebecca Kozor; Derralynn Hughes; James C Moon; Mehdi Namdar
Abstract
<p><strong>Background:</strong> Fabry disease (OMIM 301500; FD) is a rare X-linked lysosomal storage disorder caused by mutations in the α-galactosidase A gene (<em>GLA</em>). The consequence is progressive sphingolipid accumulation that affects multiple organs, but the main cause of death is cardiac via heart failure and arrhythmia. Cardiac involvement in FD occurs prior to left ventricular hypertrophy (LVH) and is characterized by low myocardial native T1 reflecting sphingolipid storage, as measured by cardiovascular magnetic resonance (CMR), and ECG changes. Here we hypothesize that a pre-storage (pre-low T1) myocardial phenotype might occur even earlier, prior to T1 lowering.</p> <p><strong>Methods:</strong> FD patients and age-, sex- and heart rate-matched healthy controls underwent same-day ECG with advanced analysis and multiparametric CMR (cines, global longitudinal strain [GLS], T1, T2 and stress perfusion mappings [myocardial blood flow, MBF], and late gadolinium enhancement [LGE]).</p> <p><strong>Results: </strong>114 Fabry patients (46±13 years, 61% female) and 76 controls (49±15 years, 50% female) were included.</p> <p>First, Fabry patients with LVH (n=42, 56%) showed previously described changes: lower MBF, GLS and T1, but higher T2 and %LGE (all P<0.05); ECG changes were also pronounced with LVH: longer P wave, QRS and QT times, more pathological repolarization and LVH voltage criteria (all P<0.05).</p> <p>Second, in pre-LVH FD (n=72, 63%), low T1 patients (n=32, 44%) had higher LV mass, longer QRS (90±11 vs 85±12ms), higher maximum Q wave amplitude (2[1-2] vs 1[1-2]mm) and R wave amplitude in V1 (3[2-4] vs 2[1-3]mm) than those with normal T1 (all P<0.05). With low T1, patients also had greater Sokolow-Lyon (22[16-28] vs 17[13-23]mm, P=0.031) and Cornell indexes (911[590-1330] vs 578[433-984]mm·ms, P=0.042), longer R wave peak times in V5 (42±6 vs 39±5ms, P=0.006) and a higher prevalence of fQRS (44 vs 18%, P=0.020).</p> <p>Finally, pre-LVH FD with normal T1 (n=40, 56%) also had abnormalities compared to controls: more impaired GLS (-18±2 vs -20±2%, P<0.001), microvascular changes (lower MBF 2.5±0.7 vs 3.0±0.8mL/g/min, P=0.028), T2 elevation (50±4 vs 48±2ms, p=0.027) and limited %LV LGE (%LGE 0.3±1.1 vs 0%, P=0.004). ECG abnormalities included shorter P wave duration (88±12 vs 94±15ms, P=0.010) and T wave peak time (T<sub>onset</sub>–T<sub>peak; </sub>104±28 vs 115±20ms, P=0.015), resulting in a more symmetric T wave with lower T wave time ratio (T<sub>onset</sub>–T<sub>peak</sub>)/(T<sub>peak</sub>–T<sub>end</sub>) (1.5±0.4 vs 1.8±0.4, P<0.001) than controls. </p> <p><strong>Conclusions: </strong>FD has a measurable myocardial phenotype pre-LVH and even pre-detectable myocyte storage with microvascular dysfunction, subtly impaired GLS and altered atrial depolarization and ventricular repolarization intervals. We therefore defined what should be the stages of cardiac involvement in this disease (see Figure below).</p>
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