Late complex biventricular repair after bidirectional cavopulmonary shunt

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease
• Cardiac Surgery

Background:

• A child presented with complex congenital heart disease including transposition of great arteries, multiple ventricular septal defects (VSDs), and a small right ventricle.
• Initial management involved pulmonary artery banding and patent ductus arteriosus ligation, followed by a bidirectional cavopulmonary shunt as a staged univentricular repair approach.

Observation:

• The patient experienced a four-year loss to follow-up.
• Re-evaluation revealed a potentially adequate right ventricle for biventricular support if VSDs were closed percutaneously.
• Four VSDs were successfully closed using Amplatzer devices.

Findings:

• A complex biventricular repair was performed at age eight, including arterial switch, atrial septal defect closure, Glenn shunt takedown, and superior vena cava reanastomosis.
• The patient remains alive and well one year post-repair.

Implications:

• This case suggests that biventricular repair may be feasible before completing the Fontan procedure in select patients with suitable cardiac anatomy.
• Percutaneous VSD closure can be a crucial step in facilitating biventricular repair in complex congenital heart disease.
• Offers a potential alternative strategy for managing complex single-ventricle physiology and associated defects.