P1240Is resynchronization therapy really efficient in laminopathy?

Abstract

Abstract Introduction Laminopathy (LMNA) is a group of rare disease caused by a mutation of lamin A/C genes. Heart transplantation (HT) is often required. Cardiac resynchronization therapy (CRT) may be an option to postpone HT. Purpose To describe characteristics and outcome of LMNA patients receiving CRT. Methods All consecutive LMNA patients implanted with a CRT device for conventional indications were included in the study. Clinical and echocardiographic (TTE) data were collected during the follow-up period. Results From 2002 to 2017, 68 LMNA patients had CRT implantation. Despite CRT, 30/68 patients (44%) had HT. Population divided into two groups according to response to CRT. Patients were considered without benefit (WHOB-CRT group) if they experienced severe events (inscription on heart transplantation list or death) within two years after CRT implantation. Other patients were in the WB-CRT group. TTE and clinical parameters are described in Table 1. Table 1 Parameters WB-CRT (n=33) WHOB-CRT (n=35) P-value At implantation Age (years) 52.3±9.7 50.6±9.5 0.27 Women 9 (27%) 13 (37%) 0.45 NYHA class 2.7±0.6 2.8±0.7 0.45 LVEF (%) 33.2±8.8 31.3±7 0.64 LVEDD (mm) 60±6.9 60±6.9 0.96 TAPSE (mm) 23±3.7 14±4.8 0.002 At last follow up NYHA class 2.2±0.6 2.9±0.7 <0.001 LVEF (%) 36.4±11 27±9 <0.001 LVEDD (mm) 59±5.5 59±7.7 0.98 TAPSE (mm) 19.9±5.5 12.3±3.3 0.003 Left ventricular ejection fraction (LVEF); Left ventricular end diastolic diameter (LVEDD); Tricuspid annular plane systolic excursion (TAPSE). Conclusion Cardiac resynchronization therapy is less efficient in LMNA patients. An impaired right ventricular stroke function seems to be the only predictive factor leading to poor response to CRT.