[Barlow's disease and risk for life]

Idiopathic mitral valve prolapse, also called Barlow's disease, has been estimated to affect 5 p. 100 of the general population. Normally a benign disease, it becomes life-threatening in only a very small number of cases. High risk subjects could be detected by simple TM-mode echocardiography, provided this examination is of good quality and reproducible, for it shows a 5 mm or more thickening of mitral leaflets. Should this be the case, according to the Mayo Clinic authors, serious complications, such as sudden death, infective endocarditis or ischaemic cerebral vascular accidents, would be expected to occur in 10 p. 100 of the patients. Sudden death, of which only 60 cases have been published, is exceptional; it mainly concerns young subjects (mean age 40 years), predominantly women, with a family history of sudden death, who have experienced one or several syncopes and present with severe per- or intercritical dysrhythmias. Such subjects must be regularly supervised clinically as well as by basal or ambulatory electrocardiography and, if necessary, by electrophysiological or even haemodynamic exploration. Treatment with appropriate beta-blockers or antiarrhythmic agents is often required as is, in refractory cases, implantation of an automatic defibrillator. Infective endocarditis is a cause of death in 10 to 20 p. 100 of the patients. It mainly threatens subjects whose mitral valve regurgitation is suspected on the presence of a holo- or end-systolic murmur and confirmed by Doppler echocardiography.(ABSTRACT TRUNCATED AT 250 WORDS)