LEFT-VENTRICULAR PANNUS CAUSING INFLOW OBSTRUCTION LATE AFTER MITRAL-VALVE REPLACEMENT FOR ENDOCARDIAL FIBROELASTOSIS

A case of mitral stenosis following mitral valve replacement in a pati ent with endocardial fibroelastosis is reported. A 14-year-old boy pre sented with cardiac failure. He had been diagnosed as having endocardi al fibroelastosis at the age of 7 months and had undergone resection o f endocardial fibrous tissue in the left ventricle at that time. Five years later his mitral valve was resected owing to mitral stenosis, wi th Bjork-Shiley valve replacement. Cross-sectional echocardiography on this admission showed restrictive left ventricular inflow due to a th ickened immobile prosthetic valve with severely dyskinetic left ventri cle (ejection fraction 8%). The electrocardiogram showed atrioventricu lar reentry tachycardia. Despite direct current cardioversion and cont inual amiodarone infusion he suffered a cardiac arrest and died 12 day s after admission. Postmortem examination showed left ventricular endo cardial fibroelastosis with severe inflow obstruction due to the forma tion of a complete fibrous ring of pannus/fibrosis around the prosthet ic margin on the ventricular aspect of the left ventricle. This compli cation has not previously been described in children after mitral valv e replacement.