Cavopulmonary anastomosis as long-term palliation for patients with tricuspid atresia

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease Surgery
• Thoracic Surgery

Background:

• Historically, systemic to pulmonary artery shunts (SPAS) were standard palliation for tricuspid atresia (TA) with reduced pulmonary blood flow.
• Tricuspid atresia (TA) is a complex congenital heart defect requiring surgical intervention for survival and improved quality of life.

Purpose of the Study:

• To evaluate the efficacy and long-term outcomes of side-to-side cavopulmonary anastomosis (CPA) in patients with tricuspid atresia (TA).
• To compare CPA with previous palliative strategies like SPAS in managing TA.

Main Methods:

• Retrospective review of 19 patients with TA who underwent side-to-side cavopulmonary anastomosis (CPA) between 1967 and 1988.
• Inclusion criteria: TA with reduced pulmonary blood flow; age range from 16 days to 23 years.
• Data collection included operative details, perioperative outcomes, need for re-operation, and long-term clinical status including exercise tolerance and oxygen saturation.

Main Results:

• No operative mortality; one late death due to pneumonia.
• All patients demonstrated immediate clinical improvement post-CPA.
• Six patients required further surgery after a mean interval of 8.9 years.
• Long-term follow-up (mean 9.5 years) showed good exercise tolerance (9/12 in NY-HA Class 1 or 2) and significantly improved resting transcutaneous oxygen saturations (mean 85% vs. preoperative).

Conclusions:

• Side-to-side cavopulmonary anastomosis (CPA) is a safe and effective palliative procedure for tricuspid atresia (TA).
• CPA offers advantages over systemic to pulmonary artery shunts (SPAS), including avoidance of SPAS complications and potential for definitive long-term palliation.
• CPA leads to sustained clinical improvement, enhanced exercise capacity, and improved oxygenation in pediatric patients with TA.