The Role of Strain in the Prognosis of Transthyretin Amyloidosis

Silva, Ana Karolina Bento Bento da; Cavalcanti, Leonardo Torreão Bezerra; Barberato, Silvio Henrique; Felix, Alex dos Santos; Melo, Marcelo Dantas Tavares de

doi:10.36660/ijcs.20240082

Ana Karolina Bento Bento da Silva

Conception and design of the research

analysis and interpretation of the data and writing of the manuscript

acquisition of data

Universidade Federal da Paraíba, João Pessoa, PB – Brazil

https://orcid.org/0000-0003-1985-6397

Leonardo Torreão Bezerra Cavalcanti

Conception and design of the research

analysis and interpretation of the data and writing of the manuscript

acquisition of data

Universidade Federal da Paraíba, João Pessoa, PB – Brazil

https://orcid.org/0009-0004-6559-3895

Silvio Henrique Barberato

critical revision of the manuscript for intellectual content

Cardioeco, Centro de Diagnóstico Cardiovascular, Curitiba, PA – Brazil

https://orcid.org/0000-0002-0711-7995

Alex dos Santos Felix

critical revision of the manuscript for intellectual content

Instituto Nacional de Cardiologia, Rio de Janeiro, RJ – Brazil

https://orcid.org/0000-0001-7046-8707

Marcelo Dantas Tavares de Melo

Conception and design of the research

analysis and interpretation of the data and writing of the manuscript

critical revision of the manuscript for intellectual content

Universidade Federal da Paraíba, João Pessoa, PB – Brazil

https://orcid.org/0000-0001-8809-8783

Mailing Address: Ana Karolina Bento Bento da Silva Universidade Federal da Paraíba, Campus I. Loteamento, cidade universitária. Postal code: 58051-900. João Pessoa, PB – Brazil E-mail: Anakarolinabentos@gmail.com

Potential Conflict of Interest

No potential conflict of interest relevant to this article was reported.

Editor responsible for the review: Marcus Vinícius Simões

Studies	Biomarkers cut-off value	Staging	Prognosis (in months)
Grogan et al., 2016¹¹	wtTTRA: NT-proBNP > 3000 ng/l cTnT > 0.05 mcg/l	Stage I: NT-proBNP < 3000 ng/l and/or cTnT <0.05 mcg/l Stage II: NT-proBNP OR cTnT above the cut-off value Stage III: NT-proBNP and cTnT above the cut-off value	Stage I: 66 Stage II: 40 Stage III: 20
Hanson et al., 2018¹²	wtTTRA: sTTR < 18 mg/dL Tncl < 0.049 ng/ml	sTTR < 18 mg/dL sTTR > 18 mg/dL	33 49
Gillmore et al., 2018¹³	wtTTRA, NT-proBNP > 3000 ng/l hTTRA, eGFR < 45 ml/min	Stage I: NT-proBNP < 3000 ng/l and/or eGFR > 45ml/min Stage II: NT-proBNP OR eGFR > 45ml/min Stage III: NT-proBNP and eGFR > 45ml/min	Stage I: 69,2 Stage II: 46,7 Stage III: 24,1

Author, date	Objectives	Sample	Echocardiographic Parameters	Findings	Results
Bodez et al., 2016¹⁴	To investigate the prognostic value of RV dysfunction in patients with CA.	n = 82	TAPSE RVFWS	TAPSE < 14mm	TAPSE was found to be a prognostic marker, reflecting RV and LV involvement.
Solomon et al.,, 2019¹⁵	Effects of Patisiran on cardiac structure and function in patients with CA.	n = 126	LV thickness GLS LVEF	GLS reduction > 2%	Treatment with Patisiran resulted in reduced LV wall thickness and improved GLS compared to placebo.
Minamisawa et al, 2019¹⁶	To evaluate the association of Patisiran treatment with a regional strain of the LV myocardium in the cardiac manifestation of TTRA amyloidosis.	n = 126	LVGLS	LVGLS improvement = 1.4% compared to placebo	Patisiran prevented the deterioration of LV GLS over 18 months, mainly by attenuating disease progression in the basal region.
Fine et al., 2020¹⁷	Prognostic relevance of right and left chamber function in CA.	n = 97	TAPSE LVEF GLS RVFWS	RVFWS reduction > 1.2% GLS reduction > 1.4%	RVFWS was associated with mortality and the need for cardiovascular hospitalization. GLS changes were associated with adverse outcomes.
Huntjens et al., 2021⁷	Prognostic value of atrial and ventricular deformation in patients with CA.	n = 136	LVEF TAPSE GLS RVFWS LAS RAS LV thickness	RVFWS < 17.0% GLS < 10.4% LAS < 13.2% RAS < 12.4%	Decreased RVFWS, GLS, LAS, and RAS indexes showed a correlation with the chance of death: 2.8x, 2.3x, 7.5x and 3.5x greater the chance, respectively.
Tjahjadi et al., 2022¹⁸	To investigate the prognosis associated with right chamber dysfunction in patients with CA.	n = 93	RVFWS TAPSE	RVFWS <16%	The use of RVFWS was associated with an incremental prognostic value compared to conventional echocardiographic parameters such as TAPSE.
Giblin et al., 2022¹⁹	Natural history of myocardial function in untreated CA, and determination of the effect of Tafamidis on myocardial functional parameters in a 12-month treatment program.	n = 45	GLS Myocardial work index Myocardial efficiency	GLS: -0.3% vs -1.1%*	Tafamidis in TTRA-CM decreases the deterioration of cardiac function, as measured by GLS and myocardial work index over 1 year, when compared with an untreated cohort.
Usuku et al., 2022²⁰	To investigate whether GLS provides prognostic information in patients with CA.	n = 129	RVFWS GLS	RVFWS < 11.59%	GLS has prognostic value in patients with TTRA and provides greater prognostic power compared to conventional echocardiographic findings.
Shah et al., 2024²¹	To analyze the effect of Tafamidis on cardiac function in patients with TTRA	n = 436	LVGLS	LVGLS decline = 1.84% compared to placebo	A mild decline was seen in LVSG with Tafamidis, 80 mg, vs. placebo in the mid and basal regions, while an attenuated decline in LV circumferential and radial strain was observed in the mid and apical regions.

[1] Mailing Address: Ana Karolina Bento Bento da Silva Universidade Federal da Paraíba, Campus I. Loteamento, cidade universitária. Postal code: 58051-900. João Pessoa, PB – Brazil E-mail: Anakarolinabentos@gmail.com

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The Role of Strain in the Prognosis of Transthyretin Amyloidosis

Abstract