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A novel risk score to predict survival in advanced heart failure due to cardiac amyloidosis

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Abstract

Background

Cardiac amyloidosis, caused by deposition of immunoglobulin light chains (AL) or transthyretin (ATTR), carries a poor prognosis. Established risk scores for amyloidosis may not predict outcomes in those patients who develop advanced heart failure and who are potential candidates for heart transplantation. Here, we aimed to identify predictive parameters for patients with severe heart failure due to amyloidosis.

Methods

Out of > 1000 patients with cardiac amyloidosis (AL or ATTR) admitted to our centre between September 1998 and January 2016, a cohort of 120 patients with a complete cardiac assessment at diagnosis, including right heart catheterization, echocardiography and biomarkers, was analysed retrospectively in this study. Primary endpoint was all-cause mortality. We performed univariate and multivariate Cox regression analysis, generated risk scores to predict outcomes in AL and ATTR amyloidosis and compared those to established risk models for amyloidosis.

Results

In the Cox multivariate model, high-sensitivity troponin T (hsTnT; hazard ratio (HR) 1.003; confidence interval (CI) 1.001–1.005; p = 0.009) and mean pulmonary artery pressure (HR 1.061; CI 1.024–1.100; p = 0.001) were found to significantly and independently predict outcomes for AL amyloidosis, whereas QRS duration (HR 1.021; CI 1.004–1.039; p = 0.013), hsTnT (HR 1.021; CI 1.006–1.036; p = 0.006) and N-terminal pro-brain natriuretic peptide (HR 1.0003; CI 1.0001–1.0004; p = 0.002) were the best predictors for ATTR amyloidosis. A simple risk score (“HeiRisk”) including these parameters for AL and ATTR allowed a more precise risk stratification in our patient population compared to established risk models.

Conclusions

Risk stratification for cardiac amyloidosis with the newly developed “HeiRisk” score may be superior to other staging systems for patients with advanced heart failure due to amyloid cardiomyopathy.

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Acknowledgements

We thank Tanja Proctor, M.Sc. (Institute for Medical Biometry and Medical informatics, University of Heidelberg, Heidelberg, Germany) for statistical advice and review.

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Author notes

  1. Michael M. Kreusser and Martin J. Volz have contributed equally to this work.

Authors and Affiliations

  1. Division of Cardiology, Department of Internal Medicine III, University of Heidelberg, Im Neuenheimer Feld 410, 69120, Heidelberg, Germany

    Michael M. Kreusser, Martin J. Volz, Benjamin Knop, Philipp Ehlermann, Hugo A. Katus & Philip W. Raake

  2. DZHK (German Centre for Cardiovascular Research), Partner Site Heidelberg/Mannheim, Heidelberg, Germany

    Michael M. Kreusser, Arjang Ruhparwar, Hugo A. Katus & Philip W. Raake

  3. Department of Cardiac Surgery, University of Heidelberg, Heidelberg, Germany

    Bastian Schmack & Arjang Ruhparwar

  4. Division of Hematology and Oncology, Department of Internal Medicine V, University of Heidelberg, Heidelberg, Germany

    Ute Hegenbart & Stefan O. Schönland

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  1. Michael M. Kreusser
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Kreusser, M.M., Volz, M.J., Knop, B. et al. A novel risk score to predict survival in advanced heart failure due to cardiac amyloidosis. Clin Res Cardiol 109, 700–713 (2020). https://doi.org/10.1007/s00392-019-01559-y

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