Service de cardiologie, hôpital Cochin, 27, rue du Faubourg Saint Jacques, 75014 Paris
Laminopathies are hereditary disorders caused by mutations in the gene of nuclear envelope proteins, the lamins A and C. They cause dilated cardiomyopathy with severe conductive disease and ventricular tachycardia/fibrillation or the association with a skeletal muscle disease, like Emery Dreifuss dystrophy. Peripheral neuropathy, lipodystrophy or systemic disorders like progeria have also been described. Cardiac involvement is responsible for the bad prognosis, with a high risk of sudden death, in the third decade. Genetic screening can routinely detect the gene mutation. An invasive electrophysiological study has to be systematicaly performed. An implantable cardioverter defibrillator therapy has to be discussed in primary prevention.