Introduction. Transthyretin amyloid cardiomyopathy (ATTR-CM) is associated with severely impaired health-related quality of life (HR-QoL). HR-QoL has been shown to be an independent predictor of outcome in heart failure, but data on ATTR-CM specifically is scarce.

Aims. We aim to analyse prognostic implications of HR-QoL in ATTR-CM.

Methods and results. 188 patients were included in the analysis with a mutation in the transthyretin gene present in 17 (9.0%) of patients. HR-QoL was evaluated utilising the shortened version of the Kansas City Cardiomyopathy Questionnaire (KCCQ) and the EuroQol five dimensions questionnaire (EQ-5D).

The KCCQ Summary score median was 58.33 (IQR: 35.42 ? 82.64). The median EQ-5D scores were 0.713 (IQR: 0.569 ? 0.780) for the Overall score and 60 (IQR: 40 ? 80) for the VAS. Between the wild-type ATTR-CM and the variant ATTR-CM cohort, no significant differences were observed.

In a singular cox-regression analysis for all-cause mortality, the KCCQ Overall score (HR: 0.895 per 5 points, p = 0.003), the EQ-5D VAS (HR: 0.853 per 5 points, p = < 0.001) and the 6-MWT distance (HR: 0.764 per 50 metres, p = 0.001) showed significant predictive value. For the composite endpoint of cardiac events and cardiac death, the KCCQ Overall implied a HR of 0.918 (per 5 points, p = 0.001), the EQ-5D VAS HR was 0.931 (per 5 points, p = 0.01) and the distance in the 6-MWT (HR: 0.899 per 50 metres, p = 0.04).

Conclusion. HR-QoL is valid predictor of outcome in ATTR-CM, both with regard to cardiac events as well as all-cause mortality.