The modified Fontan operation for children less than 4 years old.

Of 500 patients who had a modified Fontan operation at this institution between 1973 and 1987, 54 (33 boys and 21 girls) were less than 4 years old. This retrospective study related preoperative clinical and hemodynamic data to subsequent survival. Twenty patients less than 4 years old had tricuspid atresia, 13 had double inlet ventricle and 21 had other complex heart defects. There were 14 early deaths (less than 30 days after operation) and 6 late deaths. Multivariate analysis of survival for the entire group of 500 patients revealed the following factors to be significantly associated with poorer survival: absence of tricuspid atresia (p = 0.011), asplenia (p less than 0.001), age less than 4 years at operation (p = 0.042), atrioventricular valve dysfunction (p = 0.017), early calendar year of operation (p less than 0.001) and the presence of either one or more of the following: left ventricular ejection fraction less than 60%, mean pulmonary artery pressure greater than 15 mm Hg and pulmonary arteriolar resistance greater than 4 U.m2 (p less than 0.001). On the basis of this study of 500 patients, age less than 4 years at operation appears to be an independent risk factor for poorer survival after the modified Fontan operation.

Histopathologic abnormalities of the sinus node compared with electrocardiographic evidence of sinus node dysfunction after the modified Fontan operation: an autopsy study of 14 cases.

Autopsy specimens from 14 patients who had undergone a modified Fontan operation were evaluated to correlate the extent of histopathologic disruption of the sinus node with electrocardiographic findings of sinus node dysfunction. Patients with sinus node dysfunction (n=7) and those without (n=7) were similar in age, complexity of cardiac malformation, and number of postoperative days at time of death. The degree of fibrosis, local hemorrhage, necrosis, lymphocytic infiltration, and focal calcification of the sinus node and perinodal tissue was also similar in both groups. These findings, which showed a comparable amount of sinus node disruption in patients with normal sinus rhythm and in those with sinus node dysfunction, indicate a lack of correlation between the extent of histopathologic abnormality of the sinus node and electrocardiographic evidence of sinus node dysfunction.