27 years of experience with the Fontan procedure: characteristics and clinical outcomes of children in a tertiary referral hospital

Abstract

Background The Fontan operation has improved the survival of children born with congenital heart disease with single ventricle physiology. The most widely adopted variations of the Fontan procedure are the extracardiac conduit, the lateral tunnel ve the intra/extracardiac conduit with fenestration. Despite advances in the treatment and prevention of early and late complications that may develop after Fontan surgery, morbidity still remains an important problem. Methods 304 patients who underwent Fontan surgery in our center between 1995 and 2022 were included in our study. The complications that developed in patients who underwent primary Fontan or lateral tunnel surgery and extracardiac conduit Fontan application were compared. Results Classic Fontan surgery and lateral tunnel surgery were performed in 26 of the patients, and extracardiac Fontan surgery was performed in 278 patients. 218 of 304 cases were patients with single ventricular pathology. 86 cases were patients with two ventricular morphologies but complex cardiac pathology. Fenestration was performed in only 6 patients, other patients did not require fenestration. The mean follow-up period of our patients was 12 years (3 months–27 years). When the complications between Fontan procedures were compared in our study, it was found that the length of hospital stay and mortality were statistically significantly reduced in patients who underwent extracardiac Fontan surgery. There was no significant difference in terms of complications that can be seen after Fontan surgery and the length of stay in the intensive care unit. Conclusion Fontan complex is a palliative surgery for children with complex heart disease. Palliative surgical operations aimed at the preparation of the Fontan circulation lead to the preparation of the pulmonary vascular bed and the preservation of ventricular function. The techniques applied in Fontan surgery affect the early and long-term complications and the survival of the patients. In our study, when we examined the patients who extracardiac conduit Fontan procedure for the non-cardiac route, we found that mortality and morbidity were minimal.

Therapy of Patients with Cardiac Malposition

“…After initial palliation, as reviewed above, a methodical review of all the available imaging studies should be performed to assess the feasibility of undertaking biventricular repair [ 38 , 39 , 40 , 41 ]. If biventricular repair is not possible, consideration for single ventricle palliation via the Fontan route [ 42 , 43 , 44 , 45 ] should be given. Currently, the Fontan procedure is accomplished using the concept of total cavopulmonary connection (TCPC) proposed by de Leval et al [ 46 ] in a staged fashion [ 47 ].…”