Supravalvar mitral stenosis: risk factors for recurrence or death after resection.

OBJECTIVE: To assess the medium term outcome in infants and children after surgical resection of supravalvar mitral stenosis with special reference to risk factors for mortality or recurrence of supravalvar mitral stenosis. No detailed follow up has been previously reported in this uncommon condition.

DESIGN: Prospective cross sectional clinical and echocardiographic follow up.

SETTING: Paediatric cardiothoracic unit.

PATIENTS AND METHODS: 23 consecutive children (14 male, nine female, mean age 3 years 2 months at surgery) who underwent resection of supravalvar mitral stenosis between 1978 and 1993.

RESULTS: Follow up was for a mean of 58 months (range 0.5-167) after resection of supravalvar mitral stenosis. Four patients developed recurrent supravalvar mitral stenosis: this has not been reported previously. This was recognised 14-108 months after resection and confirmed at repeat operation. Three of these patients had successful reoperations but one died. Five other patients died. On multivariate analysis the only variable associated with survival free of recurrent supravalvar mitral stenosis was older age (18 months or more) at time of surgery (hazard ratio 0.17, 95% confidence interval (CI) 0.03 to 0.95, P < 0.05). Five year actuarial survival free of recurrent obstruction when supravalvar mitral stenosis was resected at age less than 18 months was only 39% (95% CI 9 to 69%) compared with 73% (95% CI 24 to 93%) in older patients.

CONCLUSION: Supravalvar mitral stenosis is part of a spectrum of obstructive lesions affecting the left heart. Recurrent supravalvar mitral stenosis can develop after surgical resection. The prognosis in those who require resection within the first 18 months of life is poor: mortality is high, as is the risk of recurrent supravalvar mitral stenosis in survivors, probably because of continuing turbulent flow across a small left ventricular inflow tract.