Total Cavopulmonary Connection for Functionally Single Ventricle without Cardiopulmonary Bypass Support

Area of Science:

• Cardiology
• Pediatric Cardiac Surgery
• Congenital Heart Disease

Background:

• Single ventricle physiology presents unique challenges in congenital heart disease.
• Historically, surgical interventions for single ventricle physiology have evolved significantly, with Fontan circulation offering a definitive palliative approach.
• Controversy remains regarding the optimal surgical strategy, specifically multistage versus single-stage total cavopulmonary connections.

Observation:

• A complex case involving tricuspid atresia, transposition of great arteries, pulmonary stenosis, persistent left superior vena cava, and a functionally single ventricle was presented.
• The patient successfully underwent a single-stage extra-cardiac total cavopulmonary connection.
• This procedure involved a bilateral Glenn shunt with an extra-cardiac inferior vena cava to main pulmonary artery shunt performed off-pump.

Findings:

• Successful palliation was achieved through a single-stage extra-cardiac total cavopulmonary connection in a patient with complex congenital heart defects.
• The off-pump technique for the bilateral Glenn and extra-cardiac inferior vena cava to main pulmonary artery shunt demonstrated feasibility.
• This case supports the efficacy of single-stage total cavopulmonary connection for specific complex single ventricle anomalies.

Implications:

• Single-stage extra-cardiac total cavopulmonary connection can be a viable and effective palliative strategy for select complex congenital heart defects.
• Off-pump surgical techniques may offer advantages in managing these complex cases.
• Further research into optimizing surgical timing and techniques for single ventricle palliation is warranted.