Clinical characteristics of acute mitral regurgitation due to ruptured chordae tendineae in infancy-experience at a single institution

In infants, acute mitral regurgitation resulting from ruptured chordae tendineae is very rare, but often fatal. There are a few case reports, but the characteristics and etiology of chordae tendineae rupture have not been elucidated. Our aim was to determine the clinical characteristics of idiopathic acute mitral regurgitation due to chordal rupture in infancy. A retrospective analysis was performed on ten consecutive patients, with a mean onset age of 4.6 +/- 1.3 months. Despite nonspecific initial symptoms, all patients developed respiratory distress and four required resuscitation within a few days (mean, 1.8 +/- 1.8 days). Chest radiographs showed pulmonary congestion with a normal or mildly increased cardiothoracic ratio in all ten patients. Laboratory data and electrocardiograms showed nonspecific findings. Echocardiography revealed ruptured chordae in all patients; locations were anterior (50%), posterior (20%), and both (30%). Surgical intervention was performed within 24 h of admission in eight patients (mean, 3.6 +/- 5.1 h). Pathological findings included inflammatory cells in six specimens and myxomatous degeneration in two. No bacteria were isolated from preoperative blood cultures, pathological tissues, or excised tissue cultures. Autoantibody levels were insignificant. Three preoperatively resuscitated patients developed neurological sequelae and arrhythmias occurred in four after mitral valve replacement. Acute onset and rapid deterioration in patients with ruptured chordae tendineae necessitates early surgical intervention to improve outcomes. Though the etiology remains unknown, onset is in infants approximately 4 months of age, suggesting a definite disease entity.