Impaired right ventricular function assessed by 3D echocardiography is associated with worse outcome in wildtype transthyretin amyloid cardiomyopathy

Stephanie Schwarting (München)1, L. Stolz (München)1, S. Massberg (München)1, S. Kääb (München)1

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.

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