Abstract
Background: A small proportion of congenital heart disease cases are united under the diagnosis of a functionally single ventricle. The only management strategy for this group of patients is a series of palliative surgical procedures creating a unique situation of the so-called Fontan circulation. Currently, total cavopulmonary connection (TCPC) is the target intervention in which the superior vena cava is connected directly to the pulmonary artery branch and the inferior vena cava via a valveless intracardiac or extracardiac conduit. The heart is excluded from the pulmonary circulation and the functionally single ventricle is connected only to the systemic circulation.
Patients and methods: We aimed to analyse the number of cardiac surgical and catheterisation procedures in a population of patients with TCPC (N = 407) treated in a single paediatric centre covering the whole region of the Czech Republic.
Results: The 10-year and 20-year survival probability rates in our study group were 96% and 92%, respectively. A total of 1,128 cardiac surgical procedures, 299 catheterisation procedures, and 738 diagnostic catheterisations were performed.
Conclusion: Overall, long-term survival in the population of TCPC patients in the Czech Republic is favourable. However, this group of patients requires a high number of repeated cardiac interventions.