Normal left-atrial structure and function despite concentric left-ventricular remodelling in a cohort of patients with Anderson–Fabry disease

Putko, Brendan N.; Yogasundaram, Haran; Chow, Kelvin; Pagano, Joseph J; Khan, Aneal; Paterson, Ian; Thompson, Richard B.; Oudit, Gavin Y.

doi:10.1093/ehjci/jev057

Cited by 11 publications

(4 citation statements)

References 45 publications

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“…Using CMR with T1 mapping to classify patients with Fabry disease, Bernardini et al reported a progressive impairment of reservoir function, assessed with FT-CMR, according with the reduction of T1 mapping (index of subclinical disease) or the presence of LV hypertrophy (overt heart involvement) (185). Nevertheless, in another study, significant differences in the three LA function components were found only in Fabry disease with significant LV hypertrophy (186).…”

Section: Restrictive Cardiomyopathymentioning

confidence: 99%

Cardiac Imaging for the Assessment of Left Atrial Mechanics Across Heart Failure Stages

Bandera

Mollo

Frigelli

et al. 2022

Front. Cardiovasc. Med.

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The left atrium (LA) is emerging as a key element in the pathophysiology of several cardiac diseases due to having an active role in contrasting heart failure (HF) progression. Its morphological and functional remodeling occurs progressively according to pressure or volume overload generated by the underlying disease, and its ability of adaptation contributes to avoid pulmonary circulation congestion and to postpone HF symptoms. Moreover, early signs of LA dysfunction can anticipate and predict the clinical course of HF diseases before the symptom onset which, particularly, also applies to patients with increased risk of HF with still normal cardiac structure (stage A HF). The study of LA mechanics (chamber morphology and function) is moving from a research interest to a clinical application thanks to a great clinical, prognostic, and pathophysiological significance. This process is promoted by the technological progress of cardiac imaging which increases the availability of easy-to-use tools for clinicians and HF specialists. Two-dimensional (2D) speckle tracking echocardiography and feature tracking cardiac magnetic resonance are becoming essential for daily practice. In this context, a deep understanding of LA mechanics, its prognostic significance, and the available approaches are essential to improve clinical practice. The present review will focus on LA mechanics, discussing atrial physiology and pathophysiology of main cardiac diseases across the HF stages with specific attention to the prognostic significance. Imaging techniques for LA mechanics assessment will be discussed with an overlook on the dynamic (under stress) evaluation of the chamber.

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Section: Restrictive Cardiomyopathymentioning

confidence: 99%

Cardiac Imaging for the Assessment of Left Atrial Mechanics Across Heart Failure Stages

Bandera

Mollo

Frigelli

et al. 2022

Front. Cardiovasc. Med.

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“…Due to inherent risks and limitations of endomyocardial biopsy[ 3 ], non-invasive measures are sought as surrogates for sphingolipid deposition[ 4 , 5 ]. While the focus has been primarily on global changes in cardiac structure and function, such as increasing ventricular mass[ 6 ], atrioventricular uncoupling[ 7 ], and reduced myocardial function[ 8 – 10 ], recent studies using cardiac magnetic resonance T 1 -mapping techniques show promise in providing improved diagnostic differentiation between other causes of ventricular hypertrophy, as well as the prospect of an earlier marker of disease involvement[ 11 – 13 ].…”

Section: Introductionmentioning

confidence: 99%

Reduced Right Ventricular Native Myocardial T1 in Anderson-Fabry Disease: Comparison to Pulmonary Hypertension and Healthy Controls

et al. 2016

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AimsAnderson-Fabry disease (AFD) is characterized by progressive multiorgan accumulation of intracellular sphingolipids due to α-galactosidase A enzyme deficiency, resulting in progressive ventricular hypertrophy, heart failure, arrhythmias, and death. Decreased native (non-contrast) left ventricular (LV) T1 (longitudinal relaxation time) with MRI discriminates AFD from healthy controls or other presentations of concentric hypertrophy, but the right ventricle (RV) has not been studied. The aims of the current study were to evaluate native RV T1 values in AFD, with a goal of better understanding the pathophysiology of RV involvement.Methods and ResultsNative T1 values were measured in the inferior RV wall (RVI), interventricular septum (IVS), and inferior LV (LVI) in patients with AFD, patients with pulmonary hypertension, who provided an alternative RV pathological process for comparison, and healthy controls. A minimum wall thickness of 4 mm was selected to minimize partial volume errors in tissue T1 analysis. T1 analysis was performed in 6 subjects with AFD, 6 subjects with PH, and 21 controls. Native T1 values were shorter (adjusted p<0.05 for all comparisons), independent of location, in subjects with AFD (RVI-T1 = 1096±49 ms, IVS-T1 = 1053±41 ms, LVI-T1 = 1072±44 ms) compared to both PH (RVI-T1 = 1239±41 ms, IVS-T1 = 1280±123 ms, LVI-T1 = 1274±57 ms) and HC (IVS-T1 = 1180±60 ms, LVI-T1 = 1183±45 ms). RVI measurements were not possible in controls due to insufficient wall thickness.ConclusionNative T1 values appear similarly reduced in the left and right ventricles of individuals with AFD and RV wall thickening, suggesting a common pathology. In contrast, individuals with PH and thickened RVs showed increased native T1 values in both ventricles, suggestive of fibrosis.

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“…In the present study AFD patients did not exhibit significant differences in LA size, in contrast with a previous work showing a substantial LA dilation even in AFD without LV hypertrophy [ 30 ]. However, the evidence of LA dilation in AFD remains controversial, as in a cardiac MRI study LA volumes of the AFD group were similar to those seen in the healthy control group [ 32 ]. In our AFD patients, PALS, but not PACS, was substantially lower than in healthy controls.…”

Section: Discussionmentioning

confidence: 99%

Association between Left Atrial Deformation and Brain Involvement in Patients with Anderson-Fabry Disease at Diagnosis

Esposito

Russo

Santoro

et al. 2020

JCM

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Background: Anderson-Fabry disease (AFD) can induce both central nervous system white matter lesions (WMLs) and cardiac abnormalities including left atrial (LA) dysfunction. We sought to evaluate the possible interrelations of LA structure and function impairment with the presence of WMLs in AFD patients. Methods 22 AFD patients and 22 controls, matched for age and sex, underwent an echo-Doppler exam including quantification of peak atrial longitudinal strain (PALS). AFD patients underwent also a 3-T brain magnetic resonance imaging with a visual quantification of WMLs by Fazekas’ score (FS) on 3D FLAIR images. Results AFD patients had significantly higher left ventricular (LV) mass index (LVMi) and relative wall thickness, and lower PALS compared to controls. Among AFD patients, 9 showed a FS = 0, and 13 a FS > 1. AFD patients with FS ≥ 1 showed lower PALS (29.4 ± 6.7 vs. 37.2 ± 3.9%, p = 0.003) than those with FS = 0, without difference in LA volume index and LVMi. In AFD patients, FS was inversely related to PALS (r = −0.49, p < 0.0001), even after adjusting for LVMi (r = −0.43, p < 0.05). Conclusions In the absence of significant alterations in LA size, AFD patients had lower PALS compared to controls. The inverse association between PALS and presence of WMLs indicates a possible parallel early involvement of heart and brain.

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Normal left-atrial structure and function despite concentric left-ventricular remodelling in a cohort of patients with Anderson–Fabry disease

Abstract: Left-atrial structure and function were relatively normal in our cohort of patients with AFD, who were also judiciously treated with a contemporary strategy that includes renin-angiotensin system antagonism, statins, and ERT.

Cited by 11 publications

References 45 publications

Cardiac Imaging for the Assessment of Left Atrial Mechanics Across Heart Failure Stages

Cardiac Imaging for the Assessment of Left Atrial Mechanics Across Heart Failure Stages

Reduced Right Ventricular Native Myocardial T1 in Anderson-Fabry Disease: Comparison to Pulmonary Hypertension and Healthy Controls

Association between Left Atrial Deformation and Brain Involvement in Patients with Anderson-Fabry Disease at Diagnosis

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