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Transthyretin Amyloid Cardiomyopathy (ATTR-CM): A Potential Role for Cardiopulmonary Exercise Testing
Session:
Posters (Sessão 5 - Écran 3) - Doenças do Miocárdio e Pericárdio 2
Speaker:
Rita Amador
Congress:
CPC 2022
Topic:
F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme:
17. Myocardial Disease
Subtheme:
17.2 Myocardial Disease – Epidemiology, Prognosis, Outcome
Session Type:
Pósters Electrónicos
FP Number:
---
Authors:
Rita r Amador; Mariana Paiva; Gonçalo Cunha; Pedro Lopes; Sérgio Maltês; Catarina Oliveira; Sofia Augusto; Catarina Brízido; Christopher Strong; Anaí Durazzo; Carlos Aguiar; Bruno ml Rocha; Miguel Mendes
Abstract
<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>BACKGROUND:</strong></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Risk stratification tools used in heart failure (HF) patients are useful yet understudied in specific aetiologies. We aimed to evaluate the feasibility and safety of cardiopulmonary exercise testing (CPET) in a cohort of patients with Amyloid Transthyretin Cardiomyopathy (ATTR-CM). </span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>METHODS:</strong></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">This is a single-centre study of patients diagnosed with ATTR-CM followed in our dedicated rare disease program who underwent CPET since November 2020. As per site protocol, ATTR-CM was confirmed according to the algorithm of Gilmore and colleagues. Patients performed CPET on a treadmill using an exercise protocol with progressive increase in workload, as tolerated. The test was considered maximal if a respiratory exchange ratio (RER) ≥1.05 was obtained.</span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>RESULTS:</strong></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Overall, we identified 60 patients with ATTR-CM, of whom 23 underwent CPET (mean age 82 ± 6 years; all male with symptomatic HF). The reasons for non-referral included death (n=14), frailty (n=13) and recent diagnosis with ongoing investigation (n=10). Most patients (n=22) performed CPET with either one of two HF-adapted exercise protocols arranged by our exercise physiology team. Mean RER was 1.05 ± 0.09, with a maximal CPET in 15 (65%) patients. Out of the group who did not achieve an RER>1,05 (n=8), all patients stopped the test due to fatigue. Mean peak oxygen consumption (pVO2) was 14.6 ± 3.7mL/Kg/min. Ventilatory thresholds - VT1 and VT2 were identified in 21 (87%) and 10 (43%) patients, respectively. Median VE/VCO2 slope was 41 (36-47). Overall, 15 (65%) had exercise ventilatory oscillations. The graphical CPET of the cohort is shown in <strong><span style="color:#5b9bd5">figure 1</span></strong>. The strongest correlations were noted between the percentage of predicted pVO2 (r=0.493; p=0.027) with left ventricular ejection fraction and between VE/VCO2 slope and the interventricular septum thickness (r=0. 486; p=0.035). There were no life-threatening events nor malignant arrhythmias reported. </span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>CONCLUSION:</strong></span></span></p> <p style="text-align:justify"><span style="font-size:10pt"><span style="font-family:Calibri,sans-serif"><span style="font-size:11.0pt">This pilot study shows CPET to be feasible and well-tolerated in a small subset of patients with ATTR-CM and symptomatic HF. HF-adapted protocols allowed a maximal effort in more than half of the cohort, in parallel with the attainment of submaximal parameters. Whether CPET has prognostic value in ATTR-CM is worth being further assessed.</span></span></span></p>
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