[Challenges in the surgical management of hearts with a functional single ventricle].

Unlabelled: Nowadays, the complex congenital heart diseases with a functional single ventricular haemodynamics can be treated with good results with bi-directional Glenn (BDG) and total cavopulmonary connection (TCPC) procedure. The late results are determined by the ventricular function.

Aims: To assess the surgical results of "high risk" BDG and TCPC procedures, where the functional single ventricle were complicated by left sided SVS and IVC, total anomalous pulmonary venous connection (TAPVC) and severe atrioventricular valve insufficiency. These patients' were refused for surgery earlier.

Patients: Between 01. 01. 1994. and 30. 06. 2004. 112 BDG and 93 TCPC (42 pts with extracardiac conduit--ECC) procedures were performed. AGE: 3 months-29 yrs mean: 4,7 yrs, weight: 4-46 kg, mean: 15,5 kg. Among the "high risk" pts.: 1. Bilateral BDG in 19, left BDG in 9, left sided TCPC with ECC in 3 pts. were performed, 2. The associated TAPVC were operated on successfully in 3 pts. (bilateral BDG in 1, left BDG in 1, left sided TCPC with ECC in 1), 3. Mitral valvuloplasty in 2, artificial (mitral) valve implantation in 1, tricuspid valve closure or valvuloplasty in 3, (tricuspid) valve replacement in 1, common AV valve replacement in 1 pts were performed, 4. In 4 infants BDG was performed after Norwood I. procedure.

Results: Five pts. were lost after BDG procedure (Damous-Kaye-Stansen operation were performed as well in 3 pts.), and another 5 patients' systemic pulmonary shunts were re-done. Four patients after TCPC were lost and reoperation to BDG (n = 1) or shunt procedure (n = 2) was performed in 3 pts. Pre-discharge echocardiography confirmed mitral valvuloplasty with good results. No intervention was necessary in the follow up (1-3 yrs period) due to AV insufficiency and ventricular functions improved.

Conclusion: On the basis of our series the "high risk" BDG and TCPC operations can be performed with an encouraging early result. The authors previously contraindicated associated defects can be surgically managed. Our short and mid-term results are feasible in this aspect. The long-term control of the "uni-ventricular" heart's ventricular function needs further investigation.