Heart failure & cardiomyopathies

Inflammation on Endomyocardial Biopsy Predicts Risk of Major Adverse Cardiovascular Events (MACE) in Undefined Left Ventricular Arrhythmogenic Cardiomyopathy

JACC Clin ElectrophysiolPublished Retrospective cohort study

Authors' conclusion

Presentation with malignant VA or AM associates with MACE in ULVACM patients.

Study context

Population
Patients with undefined left ventricle (LV) arrhythmogenic cardiomyopathy (new-onset ventricular arrhythmia (VA), nonischemic LGE limited to LV, LVEF ≥40%)
Participants
313 patients
Exposure
Predictors of MACE in undefined left ventricular arrhythmogenic cardiomyopathy (ULVACM)
Outcome
MACE:
  • Cardiac death
  • Heart transplantation
  • Malignant VA
Follow-up
60 months after clinical presentation
Methodological detailsmulticentre registry
  • Multicentre registry

Main finding

As reported in the abstract

MACE · 58-month median follow-up
Hazard ratio (95% CI)P value
Presentation with malignant VAmultivariable
2.8(1.4–5.5)
P = 0.003
EMB-proven active myocardial inflammationmultivariable
3.9(1.9–7.5)
P < 0.001
Immunosuppressive therapymultivariable; MACE at 60 months
0.10(0.05–0.40)
P < 0.001
MACE in whole cohort
93 of 313 (30%)

Observational data: associations, not effects.

Further findings

Diagnostic yield of next generation sequencing (NGS) and endomyocardial biopsy (EMB)

Underwent NGS
160 (51%)
Underwent EMB
198 (63%)
P/LP cardiomyopathy variants on NGS
25 of 160 (16%)
Active myocardial inflammation on EMB
102 of 198 (52%)
AM patients receiving immunosuppressive therapy
47 (46%)

Original publication

Inflammation on Endomyocardial Biopsy Predicts Risk of MACE in Undefined Left Ventricular Arrhythmogenic Cardiomyopathy. JACC Clin Electrophysiol. Published 18 January 2023. doi:10.1016/j.jacep.2022.10.032

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

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