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Updated: Jun 27, 2026
Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 18, 2014
Late complex biventricular repair after bidirectional cavopulmonary shunt
Howaida O Al Qethamy1, Reida M El Oakley, Mohammed M Tageldin
1Department of Cardiac Surgery, Prince Sultan Cardiac Center, Riyadh, Kingdom of Saudi Arabia. halqethamy26@hotmail.com
Insights
This study details a complex case of congenital heart defect repair in a child with transposition of great arteries and multiple ventricular septal defects. Biventricular repair was successfully achieved before Fontan completion, offering a new treatment possibility.
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.
Abstract:
A child diagnosed with transposition of great arteries, multiple (Swiss cheese) ventricular septal defects, and a small right ventricle underwent pulmonary artery banding and patent ductus arteriosus ligation at the age of six months. At the age of three years bidirectional cavopulmonary shunt was performed as a first stage for univentricular repair. However, the patient was lost follow-up for four years, following which further evaluation showed that the right ventricle was reasonably adequate to support pulmonary circulation if the ventricular septal defects (VSDs) were closed using percutaneous techniques. Four VSDs were then closed using Amplatzer devices (AGA Medical Corporation, Plymouth, MN, USA). At the age of eight years she underwent complex biventricular repair in the form of arterial switch, closure of atrial septal defect, take down of Glenn shunt, and reanastomosis of the distal end of the superior vena cava to the distal superior vena cava stump on the right atrium. One year later the patient is alive and well. In conclusion; biventricular repair may be considered before completion of Fontan whenever cardiac anatomy allows.
