Predictors of survival stratification in patients with wild-type cardiac amyloidosis

Siepen, Fabian aus dem; Bauer, Ralf; Voss, Andreas; Hein, Selina; Aurich, Matthias; Riffel, Johannes; Mereles, Derliz; Röcken, Christoph; Buss, Sebastian J.; Katus, Hugo A.; Kristen, Arnt V.

doi:10.1007/s00392-017-1167-1

Cited by 51 publications

(44 citation statements)

References 43 publications

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“…In contrast, AL patients were younger with a female predominance, higher NYHA functional class and lower blood pressure, and a larger percentage of proteinuria. Discrepancies between groups in our cohort of CA patients are corroborated by comparable data from the existing literature [2,7,13,14,27,28]. It is speculated that differences in extracellular amyloid deposition and a likely toxic effect on cardiomyocytes and their architecture, as demonstrated in AL amyloidosis [29], account for differences in phenotypic appearance and-together with non-cardiological factorsalso for differences in mortality [2,9].…”

Section: Discussionsupporting

confidence: 79%

When and how do patients with cardiac amyloidosis die?

et al. 2019

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Background Cardiac amyloidosis (CA) is an underappreciated cause of morbidity and mortality. Light-chain (AL) and transthyretin (ATTR) amyloidosis have different disease trajectories. No data are available on subtype-specific modes of death (MOD) in patients with CA. Methods and results We retrospectively investigated 66 with AL and 48 with wild-type ATTR amyloidosis (ATTRwt) from 2000 to 2018. ATTRwt differed from AL by age (74.6 ± 5.4 years vs. 63 ± 10.8 years), posterior wall thickness (16.8 ± 3.3 mm vs. 14.3 ± 2.2 mm), left ventricular mass index (180.7 ± 63.2 g/m 2 vs. 133.5 ± 42.2 g/m 2), and the proportions of male gender (91.7% vs. 59.1%), atrial enlargement (92% vs. 68.2%) and atrial fibrillation (50% vs. 12.1%). In AL NYHA Functional Class and proteinuria (72.7% vs. 39.6%) were greater; mean arterial pressure (84.4 ± 13.5 mmHg vs. 90.0 ± 11.3 mmHg) was lower. Unadjusted 5-year mortality rate was 65% in AL-CA vs. 44% in the ATTRwt group. Individuals with AL-CA were 2.28 times ([95%CI 1.27-4.10]; p = 0.006) more likely to die than were individuals with ATTRwt-CA. Information on MOD was available in 56 (94.9%) of 59 deceased patients. MOD was cardiovascular in 40 (66.8%) and non-cardiovascular in 16 (27.1%) patients. Cardiovascular [28 (68.3%) vs. 13 (80%)] death events were distributed equally between AL and ATTRwt (p = 0.51). Conclusion Our data indicate no differences in MOD between patients with AL and ATTRwt cardiac amyloidosis despite significant differences in clinical presentation and disease progression. Cardiovascular events account for more than twothirds of fatal casualties in both groups.

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Section: Discussionsupporting

confidence: 79%

When and how do patients with cardiac amyloidosis die?

et al. 2019

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“…Mean arterial pressure is a predictor of mortality in the THAOS database among patients in the United States [36]. Low blood pressure has also been associated with greater mortality in patients with light-chain cardiac amyloidosis [16,37] as well as heart failure with reduced ejection fraction [38,39]. Decreases in arterial blood pressure may represent worsening amyloid with lower cardiac stroke volume or even the effects of progressive autonomic dysfunction in effected individuals [40].…”

Section: Discussionmentioning

confidence: 99%

“…We compared the association of EF and MCF with time to death from enrollment in THAOS using unadjusted Cox proportional hazards regression models. For multivariate analysis, a stepwise variable selection procedure for Cox proportional hazards model included demographic features (age, gender, race) as well as parameters that are either easily obtainable in clinic and/or have been shown in previous studies to predict outcomes in subjects with cardiac amyloidosis (New York Heart Association (NYHA) class [16,17], presence of a TTR mutation [18], systolic blood pressure (SBP) [16], estimated glomerular filtration rate (eGFR) [19] and health status) along with echocardiographic measures of left ventricular function, EF and/or MCF. Because we utilized a step-wise regression model, hazard ratios for non-significant variables are not retained in the final model and therefore cannot be presented.…”

Section: Methodsmentioning

confidence: 99%

Myocardial Contraction Fraction by M-Mode Echocardiography Is Superior to Ejection Fraction in Predicting Mortality in Transthyretin Amyloidosis

Rubin

Steidley

Carlsson

et al. 2018

Journal of Cardiac Failure

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“…Recently introduced speckle-tracking-derived measures of myocardial deformation, mainly the global peak systolic longitudinal strain (GLS), have emerged as a new standard in the assessment of LV function, with the potential of complementing or even replacing ejection fraction (EF) due to the well-recognized examiner-dependency of the latter [1–4]. GLS has been reported to be superior to EF in the detection of incipient heart failure of different etiologies, for example the cardio toxic effects of cancer therapy [5, 6], aortic stenosis [7] or amyloidosis [8]. Since GLS is a semi-automatically generated parameter, it might be less affected by user input.…”

Section: Introductionmentioning

confidence: 99%

Test–retest reliability of new and conventional echocardiographic parameters of left ventricular systolic function

et al. 2018

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BackgroundReliability of left ventricular function measurements depends on actual biological conditions, repeated registrations and their analyses.ObjectiveTo investigate test–retest reliability of speckle-tracking-derived strain measurements and its determinants compared to the conventional parameters, such as ejection fraction (EF), LV volumes and mitral annular plane systolic excursion (MAPSE).MethodsIn 30 patients with a wide range of left ventricular function (mean EF 46.4 ± 16.4%, range 14–73%), standard echo views were acquired independently in a blinded fashion by two different echocardiographers in immediate sequence and analyzed off-line by two independent readers, creating 4 data sets per patient. Test–retest reliability of studied parameters was calculated using the smallest detectable change (SDC) and a total, inter-acquisition and inter-reader intra-class correlation coefficient (ICC).ResultsThe smallest detectable change normalized to the mean absolute value of the measured parameter (SDCrel) was lowest for MAPSE (10.7%). SDCrel for EF was similar to GLS (14.2 and 14.7%, respectively), while SDCrel for CS was much higher (35.6%). The intra-class correlation coefficient was excellent (> 0.9) for all measures of the left ventricular function. Intra-patient inter-acquisition reliability (ICCacq) was significantly better than inter-reader reliability (ICCread) (0.984 vs. 0.950, p = 0.03) only for EF, while no significant difference was observed for any other LV function parameter. Mean intra-subject standard deviations were significantly correlated to the mean values for CS and LV volumes, but not for the other studied parameters.ConclusionsIn a test–retest setting, both with normal and impaired left ventricular function, the smallest relative detectable change of EF, GLS and MAPSE was similar (11–15%), but was much higher for CS (35%). Surprisingly, reliability of GLS was not superior to that of EF. Acquisition and reader to a similar extent influenced the reliability of measurements of all left ventricular function measures except for ejection fraction, where the reliability was more dependent on the reader than on the acquisition.

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Predictors of survival stratification in patients with wild-type cardiac amyloidosis

Cited by 51 publications

References 43 publications

When and how do patients with cardiac amyloidosis die?

When and how do patients with cardiac amyloidosis die?

Myocardial Contraction Fraction by M-Mode Echocardiography Is Superior to Ejection Fraction in Predicting Mortality in Transthyretin Amyloidosis

Test–retest reliability of new and conventional echocardiographic parameters of left ventricular systolic function

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