Self-Perceived Health Exceeds Societal Valuation Across Cardiac Neuromuscular Disease: Implications for Device Therapy Decision-Making

Background: Cardiac complications are a leading cause of mortality in neuromuscular disease (NMD).Clinical assessment typically infers quality of life (QOL) from functional impairment, which may overlook the disability paradox.We examined how patient-reported domain impairment, societal health valuation, and self-perceived health relate to one another in cardiac NMD. Methods:We surveyed 156 patients (61% response rate) at a specialized cardiac NMD clinic using the EQ-5D-5L.Three constructs were compared: domain impairment (five domain levels), societal valuation (Index Score), and self-perceived health (Visual Analogue Scale [VAS]). Spearman correlations quantified associations; VAS was compared across mobility strata byKruskal-Wallis testing. Results:The cohort included 47% with cardiomyopathy and 28% with conduction disease.Ninety-one percent reported impairment in at least one domain, most often mobility (74%) and pain/discomfort (71%).VAS and Index Score were moderately correlated ( = 0.544, p < 0.001).VAS declined across mobility strata (81.5 to 61.1 from no problems to unable to walk; p < 0.001), but this 25% relative decline was far shallower than the 63% decline in Index Score (0.888 to 0.329).Mean VAS was 68.6 (SD 20.6), lower than the Alberta population norm of 77.4 (Cohen's d = 0.43) but modest given the burden of domain impairment. Conclusions:In cardiac NMD, self-perceived health declines less steeply than societal valuation as functional impairment worsens, consistent with psychological adaptation.Direct measurement of patient-reported well-being should inform shared decision-making around device therapies.