First, it is probably a primary phenomenon, due to mutated gene expression; in fact, not differently from other genetically-determined cardiomyopathies, it initially involves interstitium; moreover, differently from seconday fibrosis, it is apparently unrelated to the renin-angiotensin-aldosterone axis activation, since it has been shown that plasma renin activity and aldosterone levels are not significantly increased in LMNA mutation carriers [8]. The gene discussed is REN; the disease is cardiomyopathy.