1LMU Klinikum der Universität München Medizinische Klinik und Poliklinik I München, Deutschland
Background: Wildtype Transthyretin amyloid cardiomyopathy (wtATTR-CM) is an unresolvable cause of heart failure (HF) in elderly. While left ventricular dysfunction, particularly the global longitudinal strain, is a validated prognostic parameter in ATTR-CM, development of right ventricular (RV) dysfunction and its prognostic implications have not been fully elucidated.
Objectives: To evaluate the prognostic impact of RV dysfunction assessed by three-dimensional echocardiography (3DE) in wtATTR-CM patients.
Methods: We retrospectively investigated RV function by RV ejection fraction (RVEF) and free wall strain (RV-FWS) in 3DE of wtATTR-CM patients who presented between 02/2020 and 10/2022 in our dedicated outpatient setting. The primary outcome was HF hospitalisation events and death. ROC and Youden’s J analysis were used to identify cut-off values for prediction of the primary endpoint. Three different RV contraction patterns were defined as Type I (normal RVEF + RV-FWS), Type II (impaired RVEF, normal RV-FWS) or Type III (impaired RVEF + RV-FWS).
Results: Patients (n=66) included in this analysis represented a typical wtATTR-CM cohort. Baseline and echocardiographic characteristics are listed in Table 1. Within a mean follow-up of 470±172 days, the primary endpoint occurred in 17 patients. RVEF and RV-FWS were identified as strong discriminators for outcome prediction and their optimal cut-offs regarding HF hospitalisations or death were 35.35% and -12.95%, respectively. Worsening in RV contraction pattern types was significantly associated with a lower event-free survival (HR 2.4 [95% CI 1.27-4.34], p=0.007)(Fig.1). Differences were primarily driven by earlier hospitalisation events of patients with Type III contraction patterns.
Conclusion: In wtATTR-CM patients, impairment of 3DE RVEF and RV-FWS is associated with worse outcome. Thus, using 3DE assessment of RV function can help to identify wtATTR-CM patients at a higher risk of HF hospitalisation or death.