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 patient with endocardial fibroelastosis is reported. A 14-year-old boy presented with cardiac failure. He had been diagnosed as having endocardial fibroelastosis at the age of 7 months and had undergone resection of endocardial fibrous tissue in the left ventricle at that time. Five years later his mitral valve was resected owing to mitral stenosis, with Bjork-Shiley valve replacement. Cross-sectional echocardiography on this admission showed restrictive left ventricular inflow due to a thickened immobile prosthetic valve with severely dyskinetic left ventricle (ejection fraction 8%). The electrocardiogram showed atrioventricular reentry tachycardia. Despite direct current cardioversion and continual amiodarone infusion he suffered a cardiac arrest and died 12 days after admission. Postmortem examination showed left ventricular endocardial fibroelastosis with severe inflow obstruction due to the formation of a complete fibrous ring of pannus/fibrosis around the prosthetic margin on the ventricular aspect of the left ventricle. This complication has not previously been described in children after mitral valve replacement.
Dinarevic
S
S
Department of Pediatric Cardiology, Royal Brompton National Heart and Lung Hospital, London, UK.
Redington
A
A
Rigby
M
M
Sheppard
M N
MN
D000293:Adolescent; D004452:Echocardiography; D004695:Endocardial Fibroelastosis / Q000601:surgery*; D017809:Fatal Outcome; D006350:Heart Valve Prosthesis; D006801:Humans; D008297:Male; D008943:Mitral Valve; D008946:Mitral Valve Stenosis / Q000209:etiology*; D011183:Postoperative Complications; D013997:Time Factors; D014694:Ventricular Outflow Obstruction / Q000209:etiology*