Self-reported questionnaires are useful for estimating the health-related quality of life (HR-QoL), impact of interventions, and prognosis. To our knowledge, no HR-QoL questionnaire has been developed for cardiac amyloidosis (CA).

This study aimed to validate Amylo-AFFECT-QOL questionnaire to assess HR-QoL and its prognostic value in CA.

A self-reported questionnaire, “Amylo-AFFECT” had been designed and validated for CA symptoms evaluation and screening by physicians. It was adapted here to assess HR-QoL (Amylo-AFFECT-QOL) and its prognostic value in CA. To validate the theoretical model, internal consistency and convergent validity were assessed, particularly correlations between Amylo-AFFECT-QOL and the HR-QoL Minnesota Living Heart Failure (MLHF) questionnaire.

Amylo-AFFECT-QOL was completed by 515 patients, 425 of whom (82.5%) had CA. Wild-type and hereditary transthyretin amyloidosis (ATTRwt and ATTRv) and immunoglobulin light-chain amyloidosis (AL) were diagnosed in 47.8%, 14.7%, and 18.8% of cases, respectively. The best HR-QoL evaluation was obtained with five dimensions: “Heart failure”, “Vascular dysautonomia”, “Neuropathy”, “Ear, gastrointestinal and urinary dysautonomia” and “Skin or mucosal involvement”. The global Amylo-AFFECT-QOL and MLHF scores showed significant positive correlations (rs =0.72, P<0.05). Patients with a final diagnosis of CA had a global Amylo-AFFECT-QOL score significantly higher than the control group composed by patients with other diagnoses (22.2±13.6 vs. 16.2±13.8, respectively; P-value<0.01). According to the Amylo-AFFECT-QOL global results, ATTRv patients’ QoL was more affected than AL patients’ QoL or ATTRwt patients’ QoL. Patients with a higher HR-QoL score had a greater risk of death or heart transplant after 1 year of follow-up (log-rank<0.01).

Amylo-AFFECT-QOL demonstrates good psychometric properties and is useful for quantifying HR-QoL and estimating CA prognosis. Its use may help to improve overall management of patients with CA.