Poor right ventricular function is associated with impaired exercise capacity and ventilatory efficiency in transthyreti
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IM - ORIGINAL
Poor right ventricular function is associated with impaired exercise capacity and ventilatory efficiency in transthyretin cardiac amyloid patients Simone Bartolini1,2 · Samuele Baldasseroni3 · Francesco Fattirolli4 · Maria Vittoria Silverii4 · Lucrezia Piccioli4 · Federico Perfetto1 · Niccolò Marchionni5 · Carlo Di Mario5,6 · Raffaele Martone1 · Giulia Taborchi1 · Sofia Morini1 · Elisa Vignini1 · Francesco Cappelli1,6 Received: 29 May 2020 / Accepted: 8 August 2020 © Società Italiana di Medicina Interna (SIMI) 2020
Abstract CardioPulmonary Exercise Test (CPET) is the gold standard to evaluate functional capacity in patients at high risk of heart failure (HF). Few studies with a limited number of subjects and conflicting results, analyzed the role of CPET in patients with systemic amyloidosis. Aims of our study were the assessment of the response to exercise in patients with Transthyretin amyloid (ATTR) cardiomyopathy (CA), and the correlation of clinical, biohumoral and echocardiographic parameters with CPET parameters, such as VO2 peak and VE/VCO2 slope. From February 2018 to March 2019, 72 cardiac ATTR patients were prospectively enrolled and underwent a complete clinical, biohumoral, echocardiographic and CPET assessment. All patients completed the exercise stress test protocol, without any adverse event. At CPET, they achieved a mean VO2 peak of 14 mL/Kg/min and a mean VE/VCO2 slope of 31. The blood pressure response to exercise was inadequate in 26 (36%) patients (flat in 25 and hypotensive in 1), while 49/72 patients (69%) showed an inadequate heart rate recovery. In multivariate analysis, s’ tricuspidalic was the only independent predictor of V O2 peak, while in the two test models performed to avoid collinearity, both TAPSE and s’ tricuspidalic were the strongest independent predictors of VE/VCO2 slope. Our data demonstrate the role of right ventricular function as an independent predictor of exercise capacity and ventilatory efficiency in ATTR. In CPET evaluation, a significant proportion of patients presented an abnormal arterial pressure response and heart rate variation to exercise. Keywords TTR amyloidosis · Exercise capacity · CPET · Right ventricular function
Introduction Amyloidosis comprises a unique group of rare diseases characterized by the extracellular deposition of insoluble fibrillar proteins, such as immunoglobulin light-chain Simone Bartolini and Samuele Baldasseroni contributed equally to this manuscript. * Francesco Cappelli cappellif@aou‑careggi.toscana.it 1
Tuscan Regional Amyloidosis Centre, Careggi University Hospital, Florence, Italy
2
Cardiology Department, Azienda Sanitaria Firenze (ASF), Florence, Italy
3
Geriatric Medicine and UTIG, Azienda Ospedaliera Careggi, Florence, Italy
(AL) and transthyretin (ATTR) [1]. ATTR can be further classified into hereditary transthyretin-related amyloidosis (ATTRm)—due to mutated gene codifying for transthyretin—and non-inherited form caused by the deposition of the wild-type transthyretin protein (ATTRwt) [
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