Heart failure & cardiomyopathies

Lifetime Clinical Course of Hypertrophic Cardiomyopathy: Outcome of the Historical Florence Cohort Over 5 Decades

JACC AdvPublished Retrospective cohort study

Authors' conclusion

In this unique HCM cohort followed for up to 50 years, often before contemporary therapies became widely implemented for HCM, HF frequently progressed over time, while arrhythmic SCD events were less common and remained constant over time. Despite spanning different management eras over 5 decades, HCM-related mortality remained relatively low (1.3%/y).

Study context

Population
Patients with hypertrophic cardiomyopathy (HCM) diagnosed at one centre 1970–1992, followed annuallyfollow-up 27 ± 6 years
Participants
202 patients
Outcome
HCM-related mortality (HF/stroke-related, SCD) and non-HCM-related mortality
Methodological detailssingle-centre, long-term follow-up
  • Single-centre
  • Long-term follow-up

Main finding

As reported in the abstract

HCM-related mortality · follow-up 27 ± 6 [range: 3-50] years
Survived
97 (48%)
Died
105 (52%)
HCM-related deaths
69
HF-related deaths
53
Fatal embolic strokes
11
SCDs
16
Annual overall HCM-related mortality
1.3%/y
Annual HCM-related mortality, first decade vs second/third decade
0.7% vs 1.8% (P < 0.01)
HF-/stroke-related events, first vs second/third decade
0.6% vs 1.3%
SCD mortality rate, similar in the 2 periods
0.1% vs 0.44%, P = 0.10
HCM deaths before widespread effective contemporary treatment, considered potentially preventable
29 (42%)

Observational data: associations, not effects.

Original publication

Lifetime Clinical Course of Hypertrophic Cardiomyopathy: Outcome of the Historical Florence Cohort Over 5 Decades. JACC Adv. Published 24 May 2023. doi:10.1016/j.jacadv.2023.100337

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