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Review
. 2024 Nov 29;13(23):7285.
doi: 10.3390/jcm13237285.

Advancing Cardiac Amyloidosis Care Through Insights from Cardiopulmonary Exercise Testing

Pietro Pugliatti  1 , Giancarlo Trimarchi  1   2 , Federico Barocelli  3 , Fausto Pizzino  4 , Francesco Di Spigno  5 , Andrea Tedeschi  5 , Maurizio Cusmà Piccione  1 , Pierangela Irrera  1 , Daniela Aschieri  5 , Giampaolo Niccoli  3   6 , Umberto Paradossi  4 , Gianluca Di Bella  1
Affiliations
Review

Advancing Cardiac Amyloidosis Care Through Insights from Cardiopulmonary Exercise Testing

Pietro Pugliatti et al. J Clin Med. .

Abstract

Cardiac amyloidosis, encompassing both transthyretin (ATTR) and light-chain (AL) types, poses considerable challenges in patient management due to its intricate pathophysiology and progressive course. This narrative review elucidates the pivotal role of cardiopulmonary exercise testing (CPET) in the assessment of these patients. CPET is essential for evaluating disease progression by measuring cardio-respiratory performance and providing prognostic insights. This functional test is crucial not only for tracking the disease trajectory, but also for assessing the effectiveness of disease-modifying therapies. Moreover, CPET facilitates the customization of therapeutic strategies based on individual patient performance, enhancing personalized care. By objectively measuring parameters such as peak oxygen uptake (VO2 peak), ventilatory efficiency, and exercise capacity, clinicians can gain a deeper understanding of the degree of functional impairment and make informed decisions regarding treatment initiation, adjustment, and anticipated outcomes. This review emphasizes the importance of CPET in advancing personalized medicine approaches, ultimately striving to improve the quality of life and clinical outcomes for patients with cardiac amyloidosis.

Keywords: cardiopulmonary exercise testing; heart failure; peak oxygen uptake; transthyretin amyloid cardiomyopathy.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Graphical abstract summarizing the role of the cardiopulmonary exercise test (CPET) in cardiac amyloidosis (CA).
Figure 2
Figure 2
Wasserman Graphs. A 79-year-old male with transthyretin wild-type cardiac amyloidosis. Reduced peak VO2 of 13.1 mL/kg/min, corresponding to 67% of the predicted value (a). Heart rate plateau at peak exercise, suggestive of chronotropic incompetence (b). Increased slope of VE/VCO2 curve (c). Abbreviations: VO2—oxygen uptake, VCO2—carbon dioxide production, VE—pulmonary ventilation, HR—heart rate.
Figure 3
Figure 3
Wasserman Graphs illustrating the improvement in CPET parameters in a 64-year-old male patient with wild-type cardiac amyloidosis before and 12 months after starting tafamidis therapy. Notable improvements include an increase in VO2 peak (from 22 to 26) and a decrease in the slope of the VE/VCO2 curve. Abbreviations: VO2—oxygen uptake, VCO2—carbon dioxide production, VE—pulmonary ventilation, HR—heart rate.
See this image and copyright information in PMC

References

    1. Saito Y., Nakamura K., Ito H. Molecular Mechanisms of Cardiac Amyloidosis. Int. J. Mol. Sci. 2022;23:25. doi: 10.3390/ijms23010025. - DOI - PMC - PubMed
    1. Clerc O.F., Cuddy S.A.M., Jerosch-Herold M., Benz D.C., Katznelson E., Canseco Neri J., Taylor A., Kijewski M.F., Bianchi G., Ruberg F.L., et al. Myocardial Characteristics, Cardiac Structure, and Cardiac Function in Systemic Light-Chain Amyloidosis. JACC Cardiovasc. Imaging. 2024;17:1271–1286. doi: 10.1016/j.jcmg.2024.05.004. - DOI - PMC - PubMed
    1. Oghina S., Bougouin W., Bézard M., Kharoubi M., Komajda M., Cohen-Solal A., Mebazaa A., Damy T., Bodez D. The Impact of Patients with Cardiac Amyloidosis in HFpEF Trials. JACC Heart Fail. 2021;9:169–178. doi: 10.1016/j.jchf.2020.12.005. - DOI - PubMed
    1. Skow R.J., Sarma S., MacNamara J.P., Bartlett M.F., Wakeham D.J., Martin Z.T., Samels M., Nandadeva D., Brazile T.L., Ren J., et al. Identifying the Mechanisms of a Peripherally Limited Exercise Phenotype in Patients with Heart Failure with Preserved Ejection Fraction. Circ. Heart Fail. 2024;17:e011693. doi: 10.1161/CIRCHEARTFAILURE.123.011693. - DOI - PMC - PubMed
    1. Juarez M., Castillo-Rodriguez C., Soliman D., Del Rio-Pertuz G., Nugent K. Cardiopulmonary Exercise Testing in Heart Failure. J. Cardiovasc. Dev. Dis. 2024;11:70. doi: 10.3390/jcdd11030070. - DOI - PMC - PubMed

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