Clinical, laboratory, electrocardiogaphic and multimodality imaging outcome predictors in cardiac amyloidosis
Authors: Fernandez Galera R , Belahnech Y , Dentamaro I , Cia X , Gutierrez L , Galian L , Teixido G , Casas G , Servato L , Gonzalez-Alujas M T , Gonzalez M T , Guala A , Cuellar H , Burcet G , Rodriguez-Palomares J F 
Published: October 2021

Abstract

Background
Cardiac affectation in amyloidosis is presented among 50% patients what implies worse prognostic. Early diagnosis and prognostic stratification is mandatory after the appearance of new therapies that could modify the course of the disease.

Purpose
The aim of the present study was to evaluate the main outcome predictors with multiparametric approach including clinical, laboratory, electrocardiographic and imaging parameters in patients with cardiac amyloidosis (CA).

Methods
We recruited patients (p) prospectively followed in our cardiomyopathy unit, for a mean period of 10 years (from 2010 to 2020) who were diagnosed with CA. Baseline clinical, laboratory and echocardiographic data were obtained. We collected cardiovascular risk factors and previous events. We performed a multimodality imaging study including echocardiogram (TTE) and cardiac magnetic resonance (CMR). TTE hallmarks of CA were recorded, as well as CMR parameters of cardiac function, myocardial mass and tissue characterization. Finally we evaluated the parameters related with mortality in the follow-up.

Results
A total of 98 p were included, with a mean age of 67.5±16.9 years old. Mean follow-up was 42,2±32 months. 22 p (24,4%) had light-chain amyloidosis (AL), 34 p (37,8%) wild type transthyretin amyloidosis (ATTRwt) and 34 p (37,8%) familiar transthyretin amyloidosis (ATTRm). 43p (47.78%) died in the follow-up. Factors related with mortality in the the follow-up where the age (HR 1.08, ppresence of hypertension (HR 2.81, p=0,002) diabetes (HR 3.12, p=0,001) and previous stroke (HR 2.69, p=0,01); NYHA class at diagnosis (HR 2.49, p&lt;0.001), low voltage (HR 2.31, p=0,008) and pseudoinfarct pattern at ECG (HR 1.9, p=0,003); elevated BNP (HR 10.29, p&lt;0.001) and creatinine (HR 1.42, p=0,02); septum (HR 1.09, p=0,001) and posterior wall (HR 1,14, p&lt;0,001)thickness, left ventricular ejection fraction (HR 0.96, p=0,0031) global longitudinal strain (HR 1.18, p&lt;0.001), ratio E/A (HR 2.29, p&lt;0,001) and E/e' (HR 1.13, p=0,003) and left atrium size (HR 1,05, p=0,006) at echo; and the presence of left atrium fibrosis (HR 4.17, p&gt;0,001) and extension of late gadolinium enhancement (HR 1,06, p=0,03) at CMR. When evaluated within a multivariate analysis in a Cox regression model, the independent variables associated with mortality were NYHA class at diagnosis (p=0.002) and amyloidosis type (p=0.002) ATTRm had a better prognosis than ATTRwt and AL (Log-rank p&lt;0.001).

Conclusion
Mortality in cardiac amyloid patients is high. Clinical parameters like hypertension, diabetes, previous stroke and NYHA class at diagnosis; laboratory parameters like BNP and creatinine, main systolic and diastolic function at TTE, presence of auricular fibrosis and extension of late gadolinium enhancement at CRM are related with mortality in the follow-up. Among different types of CA, patients with ATTRm had a better prognosis, which may have therapeutic implications with the appearance of new therapies.

Funding Acknowledgement
Type of funding sources: None. Mortality