Abstract
The case of a patient with left ventricular endomyocardial fibrosis (EMF) causing severe mitral regurgitation is presented. Excision of the fibrotic tissue through the left atrium and mitral annuloplasty resulted in symptomatic relief and uncomplicated pregnancy. An X-linked congenital dermatological condition, Bloch-Sulzberger syndrome (incontinentia pigmenti), associated with chronic eosinophilia, was also present. This occurrence with EMF has not previously been reported. Atrioventricular valve reconstruction is a feasible alternative to valve replacement in EMF.
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