Left Ventricular Mass-To-Strain Ratio Predicts Cardiac Amyloid Subtype

Geenty, P.; Sivapathan, S.; Stefani, L.; Boyd, Anita; Richards, David; Kwok, Fiona; Taylor, Mark; Stewart, Graeme J.; Thomas, Liza

doi:10.1016/j.jcmg.2020.08.035

Cited by 13 publications

(4 citation statements)

References 5 publications

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“…The diagnostic value of LA strain was independent of the combination of biomarkers of LV overload (NT-proBNP), cardiomyocyte damage (hs-troponin T), LV systolic function (LVEF) and diastolic function (E/e 0 ), LV mass, LA volume index and RV function (TAPSE). Conversely, even LV MSR, which has been reported to help distinguish between ATTR-and AL-CA, 13 did not hold independent diagnostic value. LA-PALS and LA-PACS even added diagnostic value beyond the IWT score, which incorporates variables from LV STE analysis, 4 and has already been recommended as a possible tool for non-invasive diagnosis of ATTR-CA.…”

Section: Discussionmentioning

confidence: 78%

“…A deformation curve for each segment was generated, and a mean curve was derived from the average of the segments. GLS, ejection fraction strain ratio (EFSR), mass-to-strain ratio (MSR), and septal apical-to-base (SAB) ratio 13 were calculated. Normal GLS values are -21.7 ± 2.5% (lower reference limit -16.7) in men and -23.0 ± 2.7% (lower reference limit -17.8) in women.…”

Section: Left Ventriclementioning

confidence: 99%

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Multi-chamber speckle tracking imaging and diagnostic value of left atrial strain in cardiac amyloidosis

Aimo

Fabiani

Giannoni

et al. 2022

European Heart Journal - Cardiovascular Imaging

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Aims Cardiac amyloidosis (CA) affects the four heart chambers, which can all be evaluated through speckle-tracking echocardiography (STE). Methods and results We evaluated 423 consecutive patients screened for CA over 5 years at two referral centres. CA was diagnosed in 261 patients (62%) with either amyloid transthyretin (ATTR; n = 144, 34%) or amyloid light-chain (AL; n = 117, 28%) CA. Strain parameters of all chambers were altered in CA patients, particularly those with ATTR-CA. Nonetheless, only peak left atrial longitudinal strain (LA-PALS) displayed an independent association with the diagnosis of CA or ATTR-CA beyond standard echocardiographic variables and cardiac biomarkers (Model 1), or with the diagnosis of ATTR-CA beyond the validated IWT score in patients with unexplained left ventricular (LV) hypertrophy. Patients with the most severe impairment of LA strain were those most likely to have CA or ATTR-CA. Specifically, LA-PALS and/or LA-peak atrial contraction strain (PACS) in the first quartile (i.e. LA-PALS <6.65% and/or LA-PACS <3.62%) had a 3.60-fold higher risk of CA, and a 3.68-fold higher risk of ATTR-CA beyond Model 1. Among patients with unexplained LV hypertrophy, those with LA-PALS or LA-PACS in the first quartile had an 8.76-fold higher risk for CA beyond Model 1, and a 2.04-fold higher risk of ATTR-CA beyond the IWT score. Conclusions Among STE measures of the four chambers, PALS and PACS are the most informative ones to diagnose CA and ATTR-CA. Patients screened for CA and having LA-PALS and/or LA-PACS in the first quartile have a high likelihood of CA and ATTR-CA.

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Section: Discussionmentioning

confidence: 78%

Section: Left Ventriclementioning

confidence: 99%

Multi-chamber speckle tracking imaging and diagnostic value of left atrial strain in cardiac amyloidosis

Aimo

Fabiani

Giannoni

et al. 2022

European Heart Journal - Cardiovascular Imaging

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“…Other studies have shown the role of the left-ventricular (LV) mass-to-GLS ratio (massto-strain ratio (MSR)) in determining cardiac amyloidosis subtypes. MSR was also shown to be the best discriminator between ATTR and AL cardiomyopathies compared with RAS and LVEF/GLS [21].…”

Section: Introductionmentioning

confidence: 94%

Hereditary Transthyretin Amyloidosis: How to Differentiate Carriers and Patients Using Speckle-Tracking Echocardiography

Di Lisi,

Brighina,

Manno

et al. 2023

Diagnostics

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Background: Hereditary transthyretin amyloidosis is a rare disease caused by transthyretin (TTR) gene mutations. The aim of our study was to identify early signs of cardiac involvement in patients with a TTR gene mutation in order to differentiate carriers from patients with neurological or cardiac disease. Methods: A case–control study was carried out on 31 subjects with the TTR mutation. Patients were divided into three groups: 23% with cardiac amyloidosis and polyneuropathy (group A), 42% with only polyneuropathy (group B) and 35% carriers (group C). Speckle-tracking echocardiography (left-ventricular global longitudinal strain—GLS, atrial stiffness) was performed in all patients. The apical/basal longitudinal strain ratio (SAB) and relative apical sparing (RAS) were assessed in all subjects. Results: Analyzing groups C and B, we only found a significant difference in the SAB (p-value 0.001) and RAS (p-value 0.039). These parameters were significantly more impaired in group A compared to group B (SAB p-value 0.008; RAS p-value 0.002). Also, atrial stiffness was significantly impaired in groups A and B compared to group C. Conclusions: Our study suggests the diagnostic role of the SAB and RAS in cardiac amyloidosis. The SAB and RAS showed a gradual increase from carriers to patients with neurological and cardiac diseases. Thus, these parameters, in addition to atrial stiffness, could be used to monitor carriers. More extensive data are needed.

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“…An amyloidosis index (AMYLI) score [relative wall thickness (RWT) × E / E ′] <2.2 was sensitive, excluding CA ( 8 ). Finally, our group ( 9 ) demonstrated that the LV mass-to-strain ratio (MSR) accurately distinguished ATTR and light-chain (AL) amyloidosis.…”

Section: Introductionmentioning

confidence: 97%

Cardiac “hypertrophy” phenotyping: differentiating aetiologies with increased left ventricular wall thickness on echocardiography

Ferkh

Tjahjadi

Stefani

et al. 2023

Front. Cardiovasc. Med.

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AimsDifferentiating phenotypes of cardiac “hypertrophy” characterised by increased wall thickness on echocardiography is essential for management and prognostication. Transthoracic echocardiography is the most commonly used screening test for this purpose. We sought to identify echocardiographic markers that distinguish infiltrative and storage disorders that present with increased left ventricular (LV) wall thickness, namely, cardiac amyloidosis (CA) and Anderson–Fabry disease (AFD), from hypertensive heart disease (HHT).MethodsPatients were retrospectively recruited from Westmead Hospital, Sydney, and Princess Alexandra Hospital, Brisbane. LV structural, systolic, and diastolic function parameters, as well as global (LVGLS) and segmental longitudinal strains, were assessed. Previously reported echocardiographic parameters including relative apical sparing ratio (RAS), LV ejection fraction-to-strain ratio (EFSR), mass-to-strain ratio (MSR) and amyloidosis index (AMYLI) score (relative wall thickness × E/e′) were evaluated.ResultsA total of 209 patients {120 CA [58 transthyretin amyloidosis (ATTR) and 62 light-chain (AL) amyloidosis], 31 AFD and 58 HHT patients; mean age 64.1 ± 13.7 years, 75% male} comprised the study cohort. Echocardiographic measurements differed across the three groups, The LV mass index was higher in both CA {median 126.6 [interquartile range (IQR) 106.4–157.9 g/m2]} and AFD [median 134 (IQR 108.8–152.2 g/m2)] vs. HHT [median 92.7 (IQR 79.6–102.3 g/m2), p < 0.05]. LVGLS was lowest in CA [median 12.29 (IQR 10.33–15.56%)] followed by AFD [median 16.92 (IQR 14.14–18.78%)] then HHT [median 18.56 (IQR 17.51–19.97%), p < 0.05]. Diastolic function measurements including average e′ and E/e′ were most impaired in CA and least impaired in AFD. Indexed left atrial volume was highest in CA. EFSR and MSR differentiated secondary (CA + AFD) from HHT [receiver operating curve–area under the curve (ROC-AUC) of 0.80 and 0.91, respectively]. RAS and AMYLI score differentiated CA from AFD (ROC-AUC of 0.79 and 0.80, respectively). A linear discriminant analysis with stepwise variable selection using linear combinations of LV mass index, average e′, LVGLS and basal strain correctly classified 79% of all cases.ConclusionSimple echocardiographic parameters differentiate between different “hypertrophic” cardiac phenotypes. These have potential utility as a screening tool to guide further confirmatory testing.

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Left Ventricular Mass-To-Strain Ratio Predicts Cardiac Amyloid Subtype

Cited by 13 publications

References 5 publications

Multi-chamber speckle tracking imaging and diagnostic value of left atrial strain in cardiac amyloidosis

Multi-chamber speckle tracking imaging and diagnostic value of left atrial strain in cardiac amyloidosis

Hereditary Transthyretin Amyloidosis: How to Differentiate Carriers and Patients Using Speckle-Tracking Echocardiography

Cardiac “hypertrophy” phenotyping: differentiating aetiologies with increased left ventricular wall thickness on echocardiography

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