P.5.16 Predominant right ventricular involvement in patients with laminopathies

We present here five patients carrying LMNA gene mutations with a non previously reported cardiac phenotype. In addition to variable muscular phenotype (Emery-Dreifuss muscular dystrophy or limb girdle weakness without contractures) and conduction defects/arrhythmias usually observed in cardio-laminopathy, all patients had evidence for a prominent right ventricular cardiomyopathy that arose before any significant left ventricular involvement and 3 developed severe tricuspid valve regurgitation. The fatal outcome of the 2 patients who were offered tricuspid valve replacement and died soon after, suggest that heart transplantation should be proposed early in these patients. In conclusion, in familial or sporadic patients presenting a right ventricular failure with conduction defects and arrhythmias, with or without tricuspid insufficiency, LMNA gene should be considered.