Heart transplantation in patients with laminopathy showed post-transplant survival comparable to matched patients with non-ischaemic dilated cardiomyopathy (HR 1.01; 95% CI 0.52-1.95; p=0.98).
Cohort (n=200)
Yes
Does heart transplantation yield comparable survival outcomes in patients with laminopathy compared to those with non-ischaemic dilated cardiomyopathy?
Heart transplantation in patients with laminopathy yields survival outcomes comparable to those with non-ischaemic dilated cardiomyopathy, supporting early consideration of transplantation especially in cases of arrhythmogenic instability.
Hazard Ratio: 1.01 (95% CI 0.52–1.95)
p-value: p=0.98
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.
Giordano et al. (Tue,) conducted a cohort in LMNA gene-related dilated cardiomyopathy (laminopathy) (n=200). Laminopathy vs. Non-ischaemic dilated cardiomyopathy was evaluated on 1 year and long-term post-HTx survival (HR 1.01, 95% CI 0.52-1.95, p=0.98). Heart transplantation in patients with laminopathy showed post-transplant survival comparable to matched patients with non-ischaemic dilated cardiomyopathy (HR 1.01; 95% CI 0.52-1.95; p=0.98).