A new staging system for cardiac transthyretin amyloidosis

Abstract

This simple, universally applicable staging system stratifies patients with both ATTRwt and ATTRv amyloid cardiomyopathy into prognostic categories. It will be of value in the design of forthcoming clinical trials of novel amyloid-specific therapies.

Transthyretin cardiac amyloidosis: an update on diagnosis and treatment

“…Large cohort study indicated that most patients diagnosed with ATTR-CA were in NYHA class II (>70%). 98 Also, with the rise of nuclear imaging techniques, the early noninvasive diagnosis has become possible with high diagnostic accuracy. 48 Moreover, the results of the ATTR-ACT trial highlight the significance of high clinical suspicion and early diagnosis of ATTR-CA given that tafamidis provides huge benefits 1135 H. Yamamoto and T. Yokochi to patients with early-stage ATTR-CA.…”

“…The drug (R)-1-[6-[(R)-2-carboxypyrrolidin-1-yl]-6-oxo-hexanoyl]pyrrolidine-2-carboxylic acid (CPHPC) has been developed to work as a competitive inhibitor of SAP binding to amyloid fibrils and efficiently depletes circulating SAP yet leaves some residual SAP in amyloid deposition. 98 Also, with the rise of nuclear imaging techniques, the early noninvasive diagnosis has become possible with high diagnostic accuracy. [94][95][96][97] Conclusions Both diagnosing and treating ATTR-CA remain challenging.…”

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

“…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.…”

Phenotypic profile of Ile68Leu transthyretin amyloidosis: an underdiagnosed cause of heart failure

“…Age at diagnosis and advanced heart failure symptoms at presentation were the only factors independently associated with mortality in Ile68Leu ATTRm. However, our search for risk factors was limited by missing laboratory data and due to this limitation it was not possible to stratify patients' survival according to the scores proposed by Grogan et al 24 and Gillmore et al 25…”