Heart transplantation led to 1-year survival in three patients with advanced arrhythmogenic left ventricular cardiomyopathy, severe LV dysfunction (EF <30%), and nondesmosomal genetic variants.
Heart transplantation is a viable treatment for advanced ALVC, with all three patients surviving at 1 year and demonstrating distinct nondesmosomal genetic variants and histopathological features.
Absolute Event Rate: 0% vs 0%
Arrhythmogenic left ventricular cardiomyopathy (ALVC) is a left ventricle (LV)-dominant arrhythmogenic cardiomyopathy subtype that presents with malignant ventricular tachycardia and LV dysfunction and can lead to sudden cardiac death (SCD). Three male patients—Case L (48 years old), Case N (41 years old), and Case Z (60 years old)—were admitted for heart transplantation because of advanced heart failure. Echocardiography revealed severely reduced LV systolic function (ejection fraction EF T for Case L, MYH6 c.3413G > A and LDB3 c.1223A > G for Case N, and TNNC1 c.397A > T and FLNC c.2653G > A for Case Z were confirmed, which were all nondesmosomal variants. All patients were diagnosed with ALVC based on multimodal imaging, genetic analysis, and histopathological findings. All patients survived for 1 year after heart transplantation. The findings revealed by cardiac magnetic resonance, genetic testing, and histopathological staining provide a deeper understanding of the clinical features and outcomes of patients with advanced ALVC.
Han et al. (Tue,) reported a other. Heart transplantation led to 1-year survival in three patients with advanced arrhythmogenic left ventricular cardiomyopathy, severe LV dysfunction (EF <30%), and nondesmosomal genetic variants.