Systematic review and meta-analysis of prognostic markers in transthyretin amyloid cardiomyopathy

Abstract

BACKGROUND

Transthyretin amyloid cardiomyopathy (ATTR-CM) is a rare and fatal cardiomyopathy resulting from myocardial deposition of misfolded transthyretin protein. The literature on review of prognostic tools in this patient population is limited.

AIM

To determine which parameters are commonly used in prognostication of ATTR-CM.

METHODS

We conducted a systematic review of studies assessing prognostic role in ATTR-CM amyloidosis. Studies published from inception till December 2022 were included in this review. The databases searched were PubMed, EMBASE, Scopus and ACP Journal Club, Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews, Cochrane Methodology Register Database, Database of Abstracts of Reviews of Effects, Health Technology Assessment Database, National Health Service Economic Evaluation Database.

RESULTS

Of 7272 studies initially identified, 42 studies comprising 13145 transthyretin amyloid (ATTR) patients were included. The median mortality of patients with ATTR was 33.6%. Majority of studies assessed biomarkers [Troponin, B type Natriuretic peptide (BNP), NTpro-BNP, glomerular filtration rate (eGFR)], echocardiographic findings [interventricular septal diameter (IVSd), posterior wall thickness, left ventricle ejection fraction (LVEF)], New York heart association (NYHA) class, Gillmore staging in predicting mortality, they were included in meta-analysis. In meta-analysis, higher Gillmore staging, BNP, N Terminal pro-B Type Natriuretic peptide (NT-proBNP), troponin-T, IVSd, and NYHA were associated with higher risk of mortality. On other hand, higher eGFR, was associated with lower risk of mortality.

CONCLUSION

Most commonly used parameters for prognostication of ATTR-CM studies included Gillmore staging, eGFR, Trop-T, NT pro-BNP and LVEF. Prognostication of ATTR-CM patients can help predict disease severity, survival outcomes and subsequently timely initiation of appropriate treatment strategies.

Keywords: Transthyretin amyloid cardiomyopathy; Prognosis; Gillmore staging; Biomarkers; Echocardiogram; Glomerular filtration rate

Core Tip: We conducted systematic review and meta-analysis assessing prognostic tools in transthyretin amyloid cardiomyopathy (ATTR-CM) amyloidosis. Out of 7272 studies identified, 42 studies (13145 patients) were included. Prognostic factors associated with higher odds of mortality in transthyretin cardiomyopathy included: higher Gillmore staging, B type Natriuretic peptide, NT pro-BNP, troponin-T, interventricular septal thickness, New York heart association class. Prognostication of ATTR-CM patients can significantly aid in predicting disease severity and guide timely initiation of appropriate treatment strategies.