Clinical profile, surgical approach and outcomes of children with Fontan procedure in a country with limited resources

Abstract

Background: For more than five decades, the Fontan or Fontan-Kreutzer procedure has been the mainstay treatment for congenital heart disease with a single functioning ventricle. Data concerning epidemiological profiles are poor, especially in countries with limited resources. Here, we present the cases of children with complex congenital heart disease (CHD) born in Kosovo who underwent some forms of Fontan palliation measuring the renal resistive index (RRI) to assess ventricular function and renal complications after the Fontan procedure. Objectives: This study aimed to describe the primary pathology, age, place of surgical intervention, and outcomes of children in Kosovo, a country with limited resources, who underwent the Fontan procedure in different countries. Results: from January 2018 to December 2021, 40 patients (28 male and 12 female) aged 6 to 19 years (mean 6.03 years) after a total Cavo-pulmonary connection were examined for renal insufficiency and thrombotic complications. The renal resistive index (RRI) and hematological parameters were analyzed as criteria for possible early and late complications. Two patients only developed complications in the cohort group. In both cases, the second and third stages of surgery were performed late, at the ages of 12 and 9 years. The first patient manifested a severe form of protein-lousing enteropathy, renal insufficiency, and plastic bronchitis, while the other patient presented with initial signs of protein-lousing enteropathy and recurrent ventricular tachycardia. Conclusion: Survival after Fontan operation in our group was excellent. Survival is closely dependent on the primary diagnosis, associated anomalies, age at palliation, and place of surgery