Abstract
Background. Performing bidirectional cavopulmonary artery anastomosis, and Fontan procedure in the presence
of bilateral superior vena cava, can be technically challenging. Aim of our clinical observation: to study
the impact of the bilateral superior vena cava on the outcome in children needing single ventricle palliation.
Material and methods. Detailed operative, angiographic, and follow-up data (n=78) were analyzed in 66 patients
having bilateral superior vena cava and undergoing bidirectional cavopulmonary anastomosis and
Fontan procedure. Overall outcome was compared to 297 patients having a unilateral superior vena cava
(control group).
Results. Comparison of baseline clinical and hemodynamic parameters of patients who performed bidirectional
cavopulmonary anastomosis revealed no significant differences on most of them. At the same time,
patients with bilateral superior vena cava had significantly more incidence of heterotaxy syndrome and systemic
right ventricle, and the duration of CPB was significantly higher in this group than in control group.
These factors influence the results of surgical treatment. Comparison of hospital mortality and non-fatal
complications showed significantly higher scores in the study group compared with the control group.
However, analysis of mortality data showed significant declines over the last decade.
Baseline clinical and hemodynamic parameters before executing the Fontan operation did not differ significantly
with the exception of significantly higher percentage of patients with heterotaxy syndrome and systemic
right ventricle in patients with bilateral superior vena cava, also presented significantly longer duration
of CPB compared with the control group. Despite this, the parameters describing the immediate and longterm
results of surgical treatment were not significantly different, and in-hospital mortality in the patients
with bilateral superior vena cava was somewhat lower than in the control group. Survival analysis showed
absence of significant differences between groups.
Conclusion. Currently the presence of bilateral superior vena cava is not a factor poor outcome of bidirectional
cavo-pulmonary anastomosis. Extracardiac Fontan operation in the presence of bilateral superior vena
cava creates conditions for optimum distribution of the pulmonary blood flow, which leads to good outcome.
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