BACKGROUND Eight patients with severe congenital mitral stenosis underwent double transseptal, double-balloon valvuloplasty; two had isolated congenital mitral stenosis, six had additional cardiac defects, and one had previous surgical valvotomy. Ages ranged from 0.6 to 36 years (median, 9 years). RESULTS All procedures were tolerated well. After valvuloplasty, the left atrial a wave minus the left ventricular end-diastolic pressure (LVEDP) gradient was reduced from 25 +/- 6 mm Hg to 9 +/- 3 mm Hg (p less than 0.001), the mitral valve mean gradient was reduced from 18 +/- 7 mm Hg to 8 +/- 3 mm Hg (p = 0.003), and the LVEDP was unchanged. All patients had marked clinical improvement. Only one patient developed significant mitral regurgitation. Two of the first four patients underwent repeat balloon valvuloplasty 7 months later. Follow-up evaluation on six patients from 4 to 54 months revealed no recurrence of symptoms or increased mitral regurgitation. CONCLUSIONS Double transseptal, double-balloon valvuloplasty is an effective treatment for many forms of congenital mitral stenosis. Mitral regurgitation is uncommon after this procedure. The double transseptal approach results in less trauma to the atrial septum and femoral veins and allows easy assessment of any residual postvalvuloplasty gradient.