Phase II Fontan treatment of complex congenital heart disease in children

Release date: 2007-10-18

The use of staged total cavopulmonary connection (TCPC) in the second phase of Fontan procedures for children with complex congenital heart disease has shown promising results in broadening eligibility criteria and improving surgical outcomes. This approach, when applied outside traditional center pipelines, offers several advantages, making it a valuable option for patients with challenging cardiac conditions.

Congenital heart disease refers to structural abnormalities in the heart that develop during fetal growth, often due to incomplete or defective development of heart structures. A study published in the February issue of the Chinese Journal of Thoracic and Cardiovascular Surgery explored the effectiveness of Phase II Fontan procedures in 28 pediatric patients with complex intracardiac malformations. These patients ranged in age from 3.0 to 16.5 years, with an average age of 7.3 years and body weights between 13.5 kg and 61.0 kg, averaging 21.0 kg.

The most common conditions included asplenia syndrome, multiple splenic anomalies, tricuspid atresia (TA), and double outlet right ventricle (DORV) with discordant atrioventricular connections. In the first stage, procedures such as unilateral Glenn, bilateral Glenn, and semi-Fontan were performed. The time interval between the two stages varied from 0.8 to 7.3 years, averaging 3.9 years. Five patients had pulmonary artery shunts before the Glenn procedure.

Preoperative assessments included two-dimensional Doppler ultrasonography, with 23 patients undergoing cardiac catheterization and angiography. Four different techniques were used to connect blood from the inferior vena cava to the right pulmonary artery, completing the second-stage TCPC. Despite efforts to reduce pulmonary vascular resistance and enhance cardiac output, four patients died post-surgery (14.2%).

Additionally, 12 patients developed low cardiac output syndrome, and eight required peritoneal dialysis due to impaired kidney function. Two cases of right atrial and venous thrombosis were managed through re-thoracic surgery. Postoperatively, arterial oxygen saturation levels ranged from 0.89 to 0.95. Follow-up for outpatients lasted between three months and two years, with no deaths reported and no complications like chronic effusion or protein-losing enteropathy observed.

Overall, this study highlights the potential of staged TCPC in expanding treatment options for complex congenital heart diseases and improving surgical success rates. Its application beyond centralized medical facilities offers flexibility and broader access to advanced care for pediatric patients.

——Midi Medical Network

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