Long-Term Results of Modified Fontan Operation for Single-Ventricle Patients Associated With Atrioventricular Valve Regurgitation

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The long-term outcome of modified Fontan operation concomitant with a valve operation for atrioventricular valve (AVV) regurgitation is not well described.


Between 1977 and 2003, 500 children who underwent modified Fontan operation were subdivided into 192 with AVV plasty (group P) and 308 without AVV plasty (group N). Factors associated with patient outcome were investigated retrospectively.


Surgical techniques to correct valve incompetence included circular annuloplasty, partial annuloplasty with Kaye-Reed methods, edge-to-edge repair methods, and valvoplasty, which were combined according to the etiology of the valve lesion. The estimated actuarial survival rates at 10 and 20 years were, respectively, 82.0% and 76.6% in group P (p < 0.05) and 90.8% and 86.8% in group N (p = 0.001). The estimated actuarial survival rates at 10 years among patients with AVV plasty did not show a statistically significant difference (circular annuloplasty, 79.0%; partial annuloplasty, 81.6%; edge-to-edge, 83.3%; valvoplasty, 82.6%; p = 0.90). A Cox regression model revealed that a higher preoperative ventricular ejection fraction was associated with a significant reduction in long-term mortality in group P (hazard ratio, 0.921; 95% confidence interval, 0.873 to 0.972; p < 0.05). Each repair method showed acceptable durability at medium-term follow-up, without progression of lesions.


Given worse results with poor ventricular function, early intervention against valve pathology before development of ventricular failure may improve long-term outcomes. Multiple methods are appropriate for a variety of valve lesions; however, circular annuloplasty remains a reliable repair option due to its technical simplicity.

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