Heart failure & cardiomyopathiesCohort study

Diagnostic delay in arrhythmogenic cardiomyopathy

Eur J Prev CardiolPublished

  • Retrospective cohort study
  • multicentre

Authors' conclusion

Diagnostic delay is common in patients with ACM, particularly in the presence of LV involvement, and is associated with greater mortality at follow-up.

Study context

Population
Patients with arrhythmogenic cardiomyopathy (ACM) at three Italian cardiomyopathy referral centres
Participants
174 patients · 3 centres
Exposure
Diagnostic delay vs no diagnostic delay
Outcome
Diagnostic delay (time from first medical contact to definitive ACM diagnosis ≥2 years)

Main finding

As reported in the abstract

Diagnostic delay
Diagnostic delay
31%
Median time to diagnosis (with delay)
8 years
Delay in right-dominant ACM
20%
Delay in left-dominant ACM
33%
Delay in biventricular ACM
39%

Observational data: associations, not effects.

Further findings

ACM phenotype with LV involvement

P = 0.04

OutcomeP value
All-cause mortality · follow-up · greater with diagnostic delayP = 0.03

Plakophilin-2 variants

None with diagnostic delay had plakophilin-2 variants
0

Initial (mis)diagnoses

Dilated cardiomyopathy
51%
Myocarditis
21%
Idiopathic ventricular arrhythmia
9%

Original publication

Diagnostic delay in arrhythmogenic cardiomyopathy. Eur J Prev Cardiol. Published 27 February 2023. doi:10.1093/eurjpc/zwad058

Summarised from the published abstract; every number is checked against it