[Total cavopulmonary connection in complex heart defects with a single functional ventricle]

Area of Science:

• Pediatric Cardiac Surgery
• Congenital Heart Disease Management
• Cardiovascular Physiology

Context:

• Complex congenital heart defects (CHDs) necessitate advanced surgical interventions.
• Total cavopulmonary connection (TCPC) is a palliative surgical procedure for single-ventricle physiology.
• Evaluating TCPC outcomes in a pediatric population with diverse CHDs beyond tricuspid atresia is crucial.

Purpose:

• To assess the early and late outcomes of total cavopulmonary connection (TCPC) in children with complex congenital heart defects.
• To identify risk factors associated with mortality and morbidity following TCPC.
• To evaluate the functional status and hemodynamic results in survivors.

Summary:

• Nineteen children (1.5-9.8 years) with complex CHDs (excluding tricuspid atresia) underwent TCPC.
• Early mortality was 21% (4/19), primarily in younger children (<4 years) with risk factors, due to low cardiac output, supraventricular tachycardia, and cerebral edema.
• Postoperative complications included effusions and transient tachycardia; no late deaths occurred. Survivors demonstrated good functional class (NYHA I-III) at 15 months follow-up, with satisfactory catheterization results in most.

Impact:

• TCPC can achieve satisfactory long-term functional outcomes in select pediatric patients with complex CHDs.
• Early identification and management of risk factors are critical for improving TCPC survival rates.
• This study contributes to understanding the risk-benefit profile of TCPC in a challenging pediatric cardiac surgery population.