Modified fontan procedure in ninety-nine cases of atrioventricular valve regurgitation

Abstract

Between January 1985 and August 1995, among 242 patients who underwent a modified Fontan procedure, 99 had atrioventricular valve regurgitation ranging in degree from 1 to 4, for which concomitant repair of the atrioventricular valve regurgitation was done in the majority of cases. In all but 4 cases the atrioventricular valve was repaired mainly by circular annuloplasty and valve replacement was not done in any case. Although the hospital mortality rate was significantly higher in cases with atrioventricular valve regurgitation (12/99, 12%) than in cases without (4/143, 3%; p < 0.0037, chi 2 test), actuarial survival in atrioventricular valve regurgitation was 84% for years 5 through 10. The degree of atrioventricular valve regurgitation before operation was 1.6 +/- 0.7 on average: in 49 cases with higher than grade 2 regurgitation before operation there was a significant decrease to 0.4 +/- 0.49 (p < 0.0001) after operation in short-term survivors. Patients with atrioventricular valve regurgitation can be treated with reasonable risk, provided proper repair of the valve is done. Circular annuloplasty is a simple and uniformly effective method to control regurgitation even in cases of common atrioventricular valve.

Cardiovascular adaptation to the Fontan circulation

“…Even minimal regurgitation appears to have a clinically relevant contribution to ventricular volume loading, underscoring the importance of early detection and intervention. Remodeling occurs by means of dilation and increased ventricular sphericity, greater annular dilation, valve tethering, and worsened regurgitation, setting up a vicious cycle . Not surprisingly, the presence of AV valve regurgitation is associated with higher mortality .…”

“…Remodeling occurs by means of dilation and increased ventricular sphericity, greater annular dilation, valve tethering, and worsened regurgitation, setting up a vicious cycle. 29 Not surprisingly, the presence of AV valve regurgitation is associated with higher mortality. 28 The need for surgical management of AV valve regurgitation in single ventricle physiology predicts worse outcomes with respect to overall and transplant free survival, thrombotic risk and, incidence of protein losing enteropathy.…”

Efficacy of Pulmonary Vasodilator Therapy in Patients With Functionally Single Ventricle

“…18) In patients with moderate to severe atrioventricular valve regurgitation, vasodilators or β-blockers should be administered as first-line therapy, and surgery may be required if there is no significant improvement in the regurgitation. 19,20) Vasodilators such as nitric oxide, prostaglandin I 2 , endothelin receptor antagonists, and PDE-5 inhibitors have been used to treat pulmonary vascular obstructive disease, and were reported to be effective in a number of studies. [21][22][23][24][25] However, Ovaert, et al 26) reported that bosentan therapy was not effective in patients with a failing Fontan circulation and reduced left ventricular compliance, and suggested that investigation of the efficacy of vasodilator therapy in Fontan patients should be focused on those with high PAP or PVR due to pathology of the pulmonary vessels.…”

Rational Approach to Surgical Management of Complex Forms of Double Outlet Right Ventricle with Modified Fontan Operation

“…The natural history of common AVVR in patients with functional single ventricle is poor. In a report with 242 patients undergoing an FO including 99 patients with AVVR, Imai and associates 17 reported a significantly higher mortality in children who had AVVR compared with those without AVVR, even if valve repair was attempted. In our study, 15 patients had mild or worse AVVR including five patients with greater than mild AVVR.…”