Extract
Cardiac amyloidosis originates primarily from the accumulation of insoluble misfolded protein deposits within the myocardial interstitium [1–3]. While >30 proteins are known to be capable of aggregating as amyloid in vivo, nonmutated (ATTRwt) and variant transthyretin (ATTRv) are the most frequent amyloidogenic proteins impacting the heart. Both ATTRwt and ATTRv cardiac amyloidosis can elicit restrictive cardiomyopathy, leading to poor outcomes including heart failure and death [1–3]. While myocardial dysfunction is often cited as the predominant mechanism for dyspnoea and exercise intolerance in patients with cardiac amyloidosis, growing evidence suggests that extracardiac causes, including abnormal lung function, may also be responsible for these clinical symptoms [1–3].
Abstract
Restrictive spirometry pattern and abnormal cardiopulmonary response to exercise might prove insightful in better understanding the functional profile of transthyretin cardiac amyloidosis https://bit.ly/3m6sWYd
Acknowledgements
Our thanks go to all the patients and medical staff who participated in this research.
Footnotes
The data that support the findings of this study are available from the corresponding author (R. Neviere), but restrictions apply to the availability of these data, which were used under license for the current study, and so are not publicly available. However, data are available from the authors upon reasonable request and with permission of the corresponding author.
Author contributions: G. Vergaro, A. Deney, A. Monfort, M. Emdin, O. Lairez, A.G. Giguet, J. Inamo and R. Neviere collected patient data. R. Banydeen and R. Neviere analysed and interpreted patient data. R. Banydeen and R. Neviere wrote the manuscript. All authors read and approved the final manuscript.
Conflict of interest: R. Banydeen has nothing to disclose.
Conflict of interest: G. Vergaro has nothing to disclose.
Conflict of interest: A. Deney has nothing to disclose.
Conflict of interest: A. Monfort has nothing to disclose.
Conflict of interest: M. Emdin has nothing to disclose.
Conflict of interest: O. Lairez has nothing to disclose.
Conflict of interest: A.G. Giguet has nothing to disclose.
Conflict of interest: J. Inamo has nothing to disclose.
Conflict of interest: R. Neviere has nothing to disclose.
- Received September 8, 2021.
- Accepted December 9, 2021.
- Copyright ©The authors 2022. For reproduction rights and permissions contact permissions{at}ersnet.org