Prognostic value of cardiopulmonary exercise testing in patients with transthyretin cardiac amyloidosis

• Published in: Internal and emergency medicine 2023-03, Vol.18 (2), p.585-593
• Main Authors: Silverii, Maria Vittoria, Argirò, Alessia, Baldasseroni, Samuele, Fumagalli, Carlo, Zampieri, Mattia, Guerrieri, Ludovica, Bartolini, Simone, Mazzoni, Carlotta, Burgisser, Costanza, Tomberli, Alessia, Di Mario, Carlo, Marchionni, Niccolò, Olivotto, Iacopo, Perfetto, Federico, Fattirolli, Francesco, Cappelli, Francesco
• Format: Article
• Language: English
• Subjects: Aged, 80 and over; Amyloidosis; Congestive heart failure; EM - Original; Exercise Test; Female; Heart; Heart Failure; Humans; Internal Medicine; Male; Medical prognosis; Medicine; Medicine & Public Health; Multivariate analysis; Oxygen consumption; Prealbumin; Prognosis; Transthyretin
• ISSN: 1828-0447; 1970-9366
• DOI: 10.1007/s11739-022-03125-3

The aim of this study is to evaluate the prognostic value of cardiopulmonary testing (CPET) in a cohort of patients with transthyretin cardiac amyloidosis (ATTR-CA). ATTR-CA is associated with a progressive reduction in functional capacity. The prognostic role of CPET parameters and in particular of normalized peak VO2 (%ppVO2) remains to be thoroughly evaluated. In this study, 75 patients with ATTR-CA underwent cardiological evaluation and CPET in a National Referral Center for cardiac amyloidosis (Careggi University Hospital, Florence). Fifty-seven patients (76%) had wild-type ATTR. Median age was 80 (75–83) years, 68 patients (91%) were men. Peak oxygen consumption (14.1 ± 4.1 ml/kg/min) and %pp V O 2 (68.4 ± 18.8%) were blunted. Twenty-seven (36%) patients had an abnormal pressure response to exercise. After a median follow-up of 25 (12–31) months, the composite outcome of death or heart failure hospitalization was registered in 19 (25.3%) patients. At univariate analysis %pp V O 2 was a stronger predictor for the composite outcome than peak V O 2 . %pp V O 2 and NT-proBNP remained associated with the composite outcome at multivariate analysis. The optimal predictive threshold for %pp V O 2 was 62% (sensitivity: 71%; specificity: 68%; AUC: 0.77, CI 0.65–0.88). Patients with %pp V O 2  ≤ 62%and NT-proBNP > 3000 pg had a worse prognosis with 1- and 2-year survival of 69 ± 9% and 50 ± 10%, respectively. CPET is a safe and useful prognostic tool in patients with ATTR-CA. CPET may help to identify patients with advanced disease that may benefit from targeted therapy.