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Published on: August 2, 2024
Cardiac and extracardiac outcomes after heart transplantation in laminopathies
Gauthier Giordano1,2, Guillaume Coutance3, Karim Wahbi4,5,6
1APHP, Department of Cardiology, Pitié-Salpêtrière Hospital, Paris, France.
Insights
Heart transplantation (HTx) outcomes for laminopathy patients are comparable to other dilated cardiomyopathy (DCM) patients. Electrical storm prior to HTx is a mortality risk factor, but neuromuscular involvement is not.
Area of Science:
- Cardiology
- Genetics
- Transplantation Medicine
Background:
- LMNA gene mutations are a frequent genetic cause of dilated cardiomyopathy (DCM), leading to severe heart failure and neuromuscular issues.
- Laminopathy-associated DCM often necessitates heart transplantation (HTx), but outcomes in this specific population remain unevaluated.
Purpose of the Study:
- To evaluate clinical outcomes and survival rates after heart transplantation (HTx) in patients with laminopathy (LMNA-HTx).
- To compare LMNA-HTx outcomes with matched patients undergoing HTx for non-ischaemic dilated cardiomyopathy (DCM-HTx).
- To identify mortality risk factors and assess the impact of neuromuscular symptoms post-HTx.
Main Methods:
- Retrospective analysis of consecutive LMNA-HTx patients from three French centers (2003-2024).
- Propensity score matching to compare LMNA-HTx patients with DCM-HTx patients.
- Primary endpoints: 1-year and long-term post-HTx survival; Secondary endpoints: mortality risk factors and neuromuscular symptom progression.
Main Results:
- Forty LMNA-HTx patients underwent transplantation; 33% had a preceding electrical storm (ES).
- Post-HTx survival rates at 3 months, 1 year, and 5 years were 90%, 74%, and 70%, comparable to matched DCM-HTx patients (HR: 1.01; p=0.98).
- Electrical storm (ES) before HTx was the sole mortality risk factor (HR: 8.46; p=0.002); neuromuscular involvement did not impact mortality or worsen post-HTx.
Conclusions:
- Heart transplantation (HTx) offers comparable survival for laminopathy patients to other dilated cardiomyopathy (DCM) patients.
- Electrical storm (ES) preceding HTx is a critical mortality predictor, suggesting early HTx consideration in arrhythmogenic cases.
- Mild neuromuscular involvement should not contraindicate HTx in laminopathy patients.
Background:
LMNA gene-related dilated cardiomyopathy (DCM), or laminopathy, leads to severe heart failure, neuromuscular involvement and ventricular arrhythmia and has been described as the most frequent DCM genetic cause requiring heart transplantation (HTx). However, no study has specifically evaluated outcomes of HTx in patients with laminopathy. We aimed to describe and evaluate the clinical outcomes of this specific population.
Methods:
Data from all consecutive patients with laminopathy who underwent HTx (LMNA-HTx) between 2003 and 2024 in three French centres were retrospectively extracted. Patients in the LMNA-HTx group were compared with patients with matched non-ischaemic DCM who underwent HTx (DCM-HTx) using propensity score matching. The primary endpoints were 1 year and long-term post-HTx survival. Secondary objectives were to describe mortality risk factors and assess the progression and impact of neuromuscular symptoms after HTx.
Results:
40 patients with laminopathy underwent HTx (median age 45.4 years, 57.5% males). HTx followed electrical storm (ES) in 33% of cases. Post-HTx survival at 3 months, 1 year and 5 years was 90%, 74% and 70%, respectively, comparable to 160 matched patients in the DCM-HTx group (Kaplan-Meier survival analysis: HR: 1.01; 95% CI 0.52 to 1.95; p=0.98). The occurrence of ES in the days preceding HTx emerged as the only factor associated with mortality (Kaplan-Meier survival analysis: HR: 8.46; 95% CI 1.66 to 43.17; p=0.002). Neuromuscular and extracardiac involvement was not associated with mortality and did not worsen after HTx.
Conclusions:
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.
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