Loss of base‐to‐apex circumferential strain gradient: A specific pattern of Fabry cardiomyopathy?

Labombarda, Fabien; Saloux, Éric; Milesi, Guillaume; Bienvenu, Boris

doi:10.1111/echo.13496

Cited by 33 publications

(28 citation statements)

References 30 publications

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“…For example, a pronounced base-to-apex LS gradient with preserved apical longitudinal strain has been shown to differentiate cardiac amyloid from other causes of LVH including FD [28, 29]. We found that there was no significant difference in base-to-apex LS gradient between FD patients and controls, similar to a prior study that evaluated strain using echocardiography [12].…”

Section: Discussionsupporting

confidence: 84%

“…Gruner et al found that GLS and GCS were reduced in FD patients with and without LVH, although there was a greater decrease in GLS among LVH positive patients [10]. Loss of base-to-apex CS gradient assessed by echocardiography has previously been reported in patients with FD [10, 12]. The current study confirms this finding using CMR feature tracking strain analysis, and provides additional information on the association of base-to-apex CS gradient with other CMR parameters including T1 mapping.…”

Section: Discussionmentioning

confidence: 99%

“…1). Base-to-apex LS and CS gradients were calculated as the peak gradient difference between basal and apical LS and CS, respectively [12]. T1 values were evaluated by drawing a single region of interest in the mid interventricular septum excluding any areas with LGE [6, 19].…”

Section: Methodsmentioning

confidence: 99%

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Loss of base-to-apex circumferential strain gradient assessed by cardiovascular magnetic resonance in Fabry disease: relationship to T1 mapping, late gadolinium enhancement and hypertrophy

Mathur

Dreisbach

Karur

et al. 2019

Journal of Cardiovascular Magnetic Resonance

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Background Cardiac involvement is common and is the leading cause of mortality in Fabry disease (FD). We explored the association between cardiovascular magnetic resonance (CMR) myocardial strain, T1 mapping, late gadolinium enhancement (LGE) and left ventricular hypertrophy (LVH) in patients with FD. Methods In this prospective study, 38 FD patients (45.0 ± 14.5 years, 37% male) and 8 healthy controls (40.1 ± 13.7 years, 63% male) underwent 3 T CMR including cine balanced steady-state free precession (bSSFP), LGE and modified Look-Locker Inversion recovery (MOLLI) T1 mapping. Global longitudinal (GLS) and circumferential (GCS) strain and base-to-apex longitudinal strain (LS) and circumferential strain (CS) gradients were derived from cine bSSFP images using feature tracking analysis. Results Among FD patients, 8 had LVH (FD LVH+, 21%) and 17 had LGE (FD LGE+, 45%). Nineteen FD patients (50%) had neither LVH nor LGE (FD LVH- LGE-). None of the healthy controls had LVH or LGE. FD patients and healthy controls did not differ significantly with respect to GLS (− 15.3 ± 3.5% vs. − 16.3 ± 1.5%, p = 0.45), GCS (− 19.4 ± 3.0% vs. -19.5 ± 2.9%, p = 0.84) or base-to-apex LS gradient (7.5 ± 3.8% vs. 9.3 ± 3.5%, p = 0.24). FD patients had significantly lower base-to-apex CS gradient (2.1 ± 3.7% vs. 6.5 ± 2.2%, p = 0.002) and native T1 (1170.2 ± 37.5 ms vs. 1239.0 ± 18.0 ms, p < 0.001). Base-to-apex CS gradient differentiated FD LVH- LGE- patients from healthy controls (OR 0.42, 95% CI: 0.20 to 0.86, p = 0.019), even after controlling for native T1 (OR 0.24, 95% CI: 0.06 to 0.99, p = 0.049). In a nested logistic regression model with native T1, model fit was significantly improved by the addition of base-to-apex CS gradient (χ 2 (df = 1) = 11.04, p < 0.001). Intra- and inter-observer agreement were moderate to good for myocardial strain parameters: GLS (ICC 0.849 and 0.774, respectively), GCS (ICC 0.831 and 0.833, respectively), and base-to-apex CS gradient (ICC 0.737 and 0.613, respectively). Conclusions CMR reproducibly identifies myocardial strain abnormalities in FD. Loss of base-to-apex CS gradient may be an early marker of cardiac involvement in FD, with independent and incremental value beyond native T1.

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Section: Discussionsupporting

confidence: 84%

Section: Discussionmentioning

confidence: 99%

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Loss of base-to-apex circumferential strain gradient assessed by cardiovascular magnetic resonance in Fabry disease: relationship to T1 mapping, late gadolinium enhancement and hypertrophy

Mathur

Dreisbach

Karur

et al. 2019

Journal of Cardiovascular Magnetic Resonance

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“…Deformation imaging can also depict the typical pattern of distribution of lower longitudinal strain mainly involving the inferolateral segments of the LV. Moreover, several studies 47, 48 demonstrated that while longitudinal strain was impaired in both AFD and HCM ( Figure 9 ), circumferential strain was significantly lower in AFD than in HCM, in AFD patients with LV hypertrophy and in those without. Both studies demonstrated a loss of the base-to-apex circumferential gradient AFD patients, the gradient being significantly lower as compared to HCM patients.…”

Section: Challenges Of Imaging In LV Hypertrophymentioning

confidence: 94%

Added value of cardiac deformation imaging in differential diagnosis of left ventricular hypertrophy

Lončarić

Bijnens

Sitges

2018

gcsp

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[first paragraph of article]Hypertrophic cardiomyopathy is clinically defined by the presence of increased left ventricular (LV) wall thickness that is not solely explained by abnormal loading conditions. The aetiology is diverse, but in 60% of patients the disease is an autosomal dominant trait caused by cardiac sarcomere protein gene mutation (HCM). In 10% of cases, the cause is inherited metabolic and neuromuscular disease, chromosomic abnormalities and genetic syndromes, whereas, in the remaining 30% of patients the aetiology remains unknown.

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“…Whilst some studies have demonstrated a decrease in GLS this reduction is generally due to a regional decrease in strain in the basal inferolateral regions (Figure 8) [77,80,81]. Furthermore, the circumferential strain is also reduced in patients with AFD with a decrease in both the global circumferential strain and a loss of the normal base to apex gradient [77,80].…”

Section: Advanced Echocardiographic Imagingmentioning

confidence: 99%

Echocardiography in Infiltrative Cardiomyopathy

Perry

Selvanayagam

2019

Heart, Lung and Circulation

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Left ventricular (LV) wall thickening can occur due to both physiological and pathological processes. Some LV wall thickening is caused by infiltrative cardiac deposition diseases-rare disorders from both inherited and acquired conditions, with varying systemic manifestations. They portend a poor prognosis and are generally not reversible except in rare circumstances when early diagnosis and treatment may alter the outcome (e.g., Fabry disease). Cardiac involvement is variable and depends on the degree of infiltration and type of infiltrate. These changes often lead to the development of abnormalities in both the relaxation and contractile function of the heart ultimately resulting in heart failure. Echocardiography is generally the first investigation of choice as it is easily available and gives valuable information about the thickness of the ventricular walls as well as systolic and diastolic function. It is also able to identify unique, characteristic features of the disease as well as detecting any haemodynamic sequelae. This review looks at the role of echocardiography in the diagnosis and prognosis of infiltrative cardiac deposition diseases.

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Loss of base‐to‐apex circumferential strain gradient: A specific pattern of Fabry cardiomyopathy?

Abstract: Loss of base-to-apex CS gradient may be a specific left ventricular deformation pattern of Fabry cardiomyopathy in patients with and without LVH.

Cited by 33 publications

References 30 publications

Loss of base-to-apex circumferential strain gradient assessed by cardiovascular magnetic resonance in Fabry disease: relationship to T1 mapping, late gadolinium enhancement and hypertrophy

Loss of base-to-apex circumferential strain gradient assessed by cardiovascular magnetic resonance in Fabry disease: relationship to T1 mapping, late gadolinium enhancement and hypertrophy

Added value of cardiac deformation imaging in differential diagnosis of left ventricular hypertrophy

Echocardiography in Infiltrative Cardiomyopathy

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