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Background: Little evidence is available about heart rate (HR) response to exercise as well as its relationship with functional capacity in amyloid cardiomyopathy. Then, in a multicentre cohort of patients with amyloid cardiomyopathy, we investigated the prevalence of chronotropic incompetence (CI) and its relationships with cardiopulmonary exercise testing (CPET) variables.
Methods: Data from 172 outpatients with amyloid cardiomyopathy who performed a maximal CPET and who had no significant rhythm disorders were analysed.
Results: The prevalence of CI differed depending on the age-predicted peak HR (pHR%) cut-off value adopted, ranging from 16% to 59%. pHR% correlated non-linearly with peak oxygen uptake (pVO), either as expressed as a percentage of the maximum predicted or as mL/kg/min (p<0.001). Although to a lesser extent, pHR% correlated inversely with ventilatory efficiency (p<0.001). A pHR%≤75% resulted in the most accurate cut-off value in identifying a moderate-to-severe exercise impairment (sensitivity 72%; specificity 73%; area under the curve 77.2%).
Conclusions: CI is prevalent in patients with amyloid cardiomyopathy in sinus rhythm, its percentage varying according to the pHR% cut-off value. A blunted exercise-induced HR response correlated with a poor exercise capacity even in this setting of patients, a pHR%≤75% cut-off value being possibly useful in centres without CPET availability to identify a significant exercise impairment.
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http://dx.doi.org/10.1136/heartjnl-2024-324607 | DOI Listing |
Amyloid
September 2025
Department of Public Health and Clinical Medicine, Umeå University Hospital, Umeå, Sweden.
JACC Case Rep
September 2025
Hypertrophic Cardiomyopathy Program, NYU Langone Health, New York, New York, USA. Electronic address:
Background: We present the case of a 74-year-old woman diagnosed with obstructive hypertrophic cardiomyopathy.
Case Summary: Amyloidosis was initially considered because she was genotype positive in the transthyretin gene. However, because of 2 negative 99m technetium pyrophosphate radionuclide scans, this diagnosis was considered unlikely, and endomyocardial biopsy was deferred.
ESC Heart Fail
September 2025
French Referral Centre for Cardiac Amyloidosis, GRC Amyloid Research Institute, Amyloidosis Mondor Network, Henri-Mondor Teaching Hospital, AP-HP, Creteil, France.
Objectives: Currently, there are two prognosis staging systems validated for transthyretin amyloidosis (ATTR). We sought to develop a new staging system dedicated to hereditary transthyretin amyloidosis (ATTRv) patients on specific treatments.
Methods And Results: A total of 258 patients diagnosed with ATTRv from two cardiac amyloidosis reference centres in France and Romania were stratified into three disease stages based on NT-proBNP, estimated glomerular filtration rate (eGFR) and global longitudinal strain (GLS).
Anal Chim Acta
October 2025
Center of Laboratory Medicine, Beijing, Key Laboratory for Molecular Diagnostics of Cardiovascular Diseases, State Key Laboratory of Cardiovascular Disease, National Center for Cardiovascular Diseases & Fuwai Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, 1000
Background: The treatment and prognosis of cardiac amyloidosis (CA) depend heavily on the accurate identification of amyloid protein types. Histopathological methods are the most commonly used approach, but often produce inconclusive results. The application of mass spectrometry with laser microdissection mass spectrometry based on non-targeted proteomics in CA diagnosis is gradually being recognized, but it is expensive, time-consuming, and still in the early stages of scientific research applications.
View Article and Find Full Text PDFJ Am Soc Echocardiogr
September 2025
Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN.
Background And Aims: Light chain cardiac amyloidosis (AL-CA), wild-type-transthyretin cardiac amyloidosis (ATTRwt-CA) and hereditary type-transthyretin cardiac amyloidosis (ATTRv-CA) have distinct presentations, clinical courses, and prognosis. To identify differentiating echocardiographic features and their prognostic significance, we investigated a large cohort of patients with CA.
Methods: In this multi-site cohort study, CA diagnosis was verified according to guidelines.