Comparison of Cardiac Magnetic Resonance Imaging and Echocardiography in Assessment of Left Ventricular Hypertrophy in Fabry Disease

Hazari, Hassan; Belenkie, Israel; Kryski, Albert; White, James A.; Oudit, Gavin Y.; Thompson, Richard B.; Fung, Tak; Dehar, Navdeep; Khan, Aneal

doi:10.1016/j.cjca.2018.03.011

Cited by 22 publications

(18 citation statements)

References 35 publications

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“…LVH secondary to FD is most commonly concentric and symmetric [25,32] (Figure 1); however, other patterns can also occur, including asymmetric septal hypertrophy, eccentric hypertrophy, and apical hypertrophy [25,40,41]. nosis of FD, to detect LVH, as well as to monitor the progression of cardiac involvement and response to therapy [36,37]. Cardiac magnetic resonance imaging (MRI) can detect cardiac involvement even when the LVH severity is mild, allowing to reclassify 21% of FD patients as having cardiac involvement that was previously unrecognized [38].…”

Section: Left Ventricular Hypertrophymentioning

confidence: 99%

“…Although electrocardiographic changes of LVH may predate imaging evidence of LVH in Fabry patients [ 35 ], multimodality cardiac imaging is useful to suggest the diagnosis of FD, to detect LVH, as well as to monitor the progression of cardiac involvement and response to therapy [ 36 , 37 ]. Cardiac magnetic resonance imaging (MRI) can detect cardiac involvement even when the LVH severity is mild, allowing to reclassify 21% of FD patients as having cardiac involvement that was previously unrecognized [ 38 ].…”

Section: Cardiac Manifestations Of Fdmentioning

confidence: 99%

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Fabry Disease and the Heart: A Comprehensive Review

Azevedo

Cordeiro

Gago

et al. 2021

IJMS

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Fabry disease (FD) is an X-linked lysosomal storage disorder caused by mutations of the GLA gene that result in a deficiency of the enzymatic activity of α-galactosidase A and consequent accumulation of glycosphingolipids in body fluids and lysosomes of the cells throughout the body. GB3 accumulation occurs in virtually all cardiac cells (cardiomyocytes, conduction system cells, fibroblasts, and endothelial and smooth muscle vascular cells), ultimately leading to ventricular hypertrophy and fibrosis, heart failure, valve disease, angina, dysrhythmias, cardiac conduction abnormalities, and sudden death. Despite available therapies and supportive treatment, cardiac involvement carries a major prognostic impact, representing the main cause of death in FD. In the last years, knowledge has substantially evolved on the pathophysiological mechanisms leading to cardiac damage, the natural history of cardiac manifestations, the late-onset phenotypes with predominant cardiac involvement, the early markers of cardiac damage, the role of multimodality cardiac imaging on the diagnosis, management and follow-up of Fabry patients, and the cardiac efficacy of available therapies. Herein, we provide a comprehensive and integrated review on the cardiac involvement of FD, at the pathophysiological, anatomopathological, laboratory, imaging, and clinical levels, as well as on the diagnosis and management of cardiac manifestations, their supportive treatment, and the cardiac efficacy of specific therapies, such as enzyme replacement therapy and migalastat.

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Section: Left Ventricular Hypertrophymentioning

confidence: 99%

Section: Cardiac Manifestations Of Fdmentioning

confidence: 99%

Fabry Disease and the Heart: A Comprehensive Review

Azevedo

Cordeiro

Gago

et al. 2021

IJMS

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“…CMR was solely responsible for the diagnosis of the HCM phenotype in a significant number of patients with non-diagnostic echocardiography [ 63 ]. In 32 AFD patients, Azari et al showed that echocardiography consistently overestimates LV mass index compared to CMR [ 64 ]. Whereas CMR showed an increasing trend over time in LV mass index, cardiac ultrasound failed to identify this trend, which indicates that differences in this parameter may not be detected by echocardiography because of the tendency to always be overestimated and the high variability between measurements [ 64 ].…”

Section: Cardiac Magnetic Resonancementioning

confidence: 99%

“…In 32 AFD patients, Azari et al showed that echocardiography consistently overestimates LV mass index compared to CMR [ 64 ]. Whereas CMR showed an increasing trend over time in LV mass index, cardiac ultrasound failed to identify this trend, which indicates that differences in this parameter may not be detected by echocardiography because of the tendency to always be overestimated and the high variability between measurements [ 64 ]. Because patients treated with ERT had a slower LV mass increase than those without therapy [ 65 ], CMR could be the preferred technique in AFD, not only for the diagnosis but also for the monitoring of the effectiveness of the treatment.…”

Section: Cardiac Magnetic Resonancementioning

confidence: 99%

Cardiac Imaging in Anderson-Fabry Disease: Past, Present and Future

Esposito

Santoro

Mandoli

et al. 2021

JCM

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Anderson-Fabrydisease is an X-linked lysosomal storage disorder caused by a deficiency in the lysosomal enzyme α-galactosidase A. This results in pathological accumulation of glycosphingolipids in several tissues and multi-organ progressive dysfunction. The typical clinical phenotype of Anderson-Fabry cardiomyopathy is progressive hypertrophic cardiomyopathy associated with rhythm and conduction disturbances. Cardiac imaging plays a key role in the evaluation and management of Anderson-Fabry disease patients. The present review highlights the value and perspectives of standard and advanced cardiovascular imaging in Anderson-Fabry disease.

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“…Hazari et al compare echocardiography and cardiac magnetic resonance (CMR) imaging in the longitudinal evaluation of myocardial mass in patients with Fabry disease. 21 They note that left ventricular mass, as measured by CMR, increases with the development of cardiac fibrosis and that enzyme-replacement therapy slows these changes, pointing to the value of this indicator. Kato et al apply automated, real-time 3-dimensional volume colour-flow Doppler echocardiography to obtain single-beat right-heart flow measurements in children with atrial shunts.…”

Section: Focus On Congenital Heart Disease and Pediatric Cardiologymentioning

confidence: 99%

The Canadian Journal of Cardiology: Open and Growing

Nattel

2019

Canadian Journal of Cardiology

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Comparison of Cardiac Magnetic Resonance Imaging and Echocardiography in Assessment of Left Ventricular Hypertrophy in Fabry Disease

Cited by 22 publications

References 35 publications

Fabry Disease and the Heart: A Comprehensive Review

Fabry Disease and the Heart: A Comprehensive Review

Cardiac Imaging in Anderson-Fabry Disease: Past, Present and Future

The Canadian Journal of Cardiology: Open and Growing

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