CurrentCardio
Get Weekly

Heart failure & cardiomyopathiesCohort study

Cardiac and extracardiac outcomes after heart transplantation in laminopathies

HeartPublished

  • retrospective
  • consecutive patients
  • three French centres
  • propensity score matching
Read the original article ↗

Authors' conclusion

In this first systematic evaluation of HTx in patients with laminopathy, we show that post-transplant survival was comparable between LMNA-HTx and matched DCM-HTx patients and was not associated with neuromuscular involvement. In contrast, ES occurred in one-third of patients prior to HTx and was associated with mortality. These findings support considering HTx early in the disease course, particularly in the setting of arrhythmogenic instability, while mild neuromuscular involvement should not be viewed as a contraindication.

Study context

Population
Patients with laminopathy (LMNA gene-related dilated cardiomyopathy) who underwent heart transplantation between 2003 and 2024, compared with matched non-ischaemic dilated cardiomyopathy (DCM) patients who underwent HTxpatients with laminopathy who underwent HTx, compared with 160 matched DCM-HTx patients · LMNA-HTx 40 · DCM-HTx 160
Participants
40 patients
Exposure
Patients in the LMNA-HTx group were compared with patients with matched non-ischaemic DCM who underwent HTx (DCM-HTx) using propensity score matching
  • LMNA-HTxn = 40
  • DCM-HTxn = 160reference
Outcome
Post-HTx survival
Follow-up
1 year and long-term

Main finding

As reported in the abstract

Hazard ratio

1.01

95% CI 0.52–1.95

P = 0.98

By group

LMNA-HTx

90%

Survival at 3 months · Survival at 1 year 74% · Survival at 5 years 70%

LMNA-HTx vs DCM-HTx
HR 1.01 (95% CI 0.52–1.95); P = 0.98

Observational data: associations, not effects.

Secondary outcomes

Electrical storm preceding HTx

HTx followed electrical storm (electrical storm (ES))
33%

Mortality risk factor: ES in the days preceding HTx

HR for mortality with ES in the days preceding HTx
8.46
95% CI
1.66 to 43.17
p
P = 0.002
  • only factor associated with mortality
  • Kaplan-Meier survival analysis

Neuromuscular and extracardiac involvement

Not associated with mortality and did not worsen after HTx

Original publication

Cardiac and extracardiac outcomes after heart transplantation in laminopathies. Heart. Published 23 June 2026. doi:10.1136/heartjnl-2026-327844
Read the original article ↗PubMedCite RIS · BibTeX

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