Is resynchronization therapy really efficient in laminopathy?

Abstract

Laminopathy (LMNA) is a group of rare, hereditary, disease mostly caused by a mutation of lamin A/C genes. Heart involvement leads to arrhythmias, conduction disorders, and dilated cardiomyopathy. At time, heart transplantation (HT) is often required. Cardiac resynchronization therapy (CRT) may be an option to postpone HT. To describe characteristics and outcome of LMNA patients receiving CRT. All consecutive LMNA patients implanted with a CRT device for conventional indications were included in the study. Clinical, echocardiographic (TTE) and device interrogation data were collected during the follow-up (FU) period. From 2002 to 2017, 68 LMNA patients had CRT implantation (46 males (67%); mean age 52 ± 10 years) in the cardiology departments of 5 French University centers. Despite CRT, 30/68 patients (44%) had HT after a 23.4 ± 26 months’ mean FU period. We divided our population into two groups according to response to CRT. Patients were considered without benefit (WoB-CRT group) if they experienced severe events (inscription on heart transplantation list or heart transplantation or death) within two years after CRT implantation. Other patients were in the WB-CRT group. Results are presented as mean ± SD. Cardiac resynchronization therapy seems to be less efficient in LMNA patients compared to usual implanted patients with other type of advanced cardiomyopathy. An impaired right ventricular stroke function seems to be the only predictive factor leading to poor response to CRT.