Long-term valvular function after total repair of tetralogy of Fallot. Relation to ventricular arrhythmias.

Patients with total repair of tetralogy of Fallot may have residual valvular dysfunction, the long-term effect of which is poorly defined. We prospectively studied 59 patients for 18 +/- 5 (mean +/- SD) years postoperatively by Doppler echocardiography and by 24-hour electrocardiographic monitoring. Right ventricular outflow gradients were estimated from the peak continuous-wave Doppler pulmonary artery velocity. The severity of valvular regurgitation was determined by mapping the proximal chamber by pulsed Doppler methods. Right ventricular diastolic cavity area was measured by planimetry of the apical image. The right ventricular outflow tract gradient had a mean value of 9.4 +/- 9.0 mm Hg (range, 0-58 mm Hg; median, 6.6 mm Hg). Pulmonary regurgitation was present in 78% of patients, with 59% of patients graded as mild and 19% as moderate. Tricuspid regurgitation was found in 65% of patients, with 56% of patients graded as mild, 7% as moderate, and 2% as severe. The severity of pulmonary regurgitation correlated with right ventricular cavity area (p less than 0.05). The severity of tricuspid regurgitation was not related to pulmonary stenosis or to the severity of pulmonary regurgitation. Aortic regurgitation is unusual (n = 11) and correlates with aortic root size. The frequency and type of ventricular arrhythmia were not related to the severity of pulmonary stenosis; however, ventricular bigeminy and couplets were related to the severity of pulmonary regurgitation (p less than 0.025). The majority of patients with total repair of tetralogy of Fallot have remarkably good long-term valvular function. For the minority with moderate or severe valvular dysfunction, pulmonary regurgitation is the best marker for ventricular arrhythmias.