Complex Biventricular Repair Program

The following are heart conditions we treat, but please keep in mind your child might not be a candidate for biventricular or one-and-a-half ventricle repair.

• Borderline small left and right heart 
• Pulmonary atresia with intact ventricular septum
• Hypoplastic left heart syndrome (HLHS) with mitral and aortic stenosis
• Shones complex
• Unbalanced atrioventricular (AV) canal
• Double-outlet right ventricle (DORV)
• Complex transposition of the great arteries
• Upstairs-downstairs ventricles
• Double inlet right or left ventricle
• Straddling or crisscross mitral or tricuspid valves

Each year, we evaluate and treat hundreds of children and dozens of adults from all over the world for possible biventricular or one-and-a-half ventricle repair, helping those with complex congenital heart disease start a journey to improved heart function.

If your child is a candidate for such treatment, we will offer a customized second opinion evaluation and care plan. Our highly specialized team of pediatric cardiologists, cardiac surgeons, cardiac anesthesiologists, cardiac critical care specialists, and nurse practitioners uses innovative methods — including advanced imaging and three-dimensional (3-D) modeling — to evaluate, diagnose, and treat your child.

Care for your child extends beyond procedures and hospitalization. After you return home, we stay in close contact with you and your local care team to monitor your child and determine next steps in the pathway of care. Our team is available to ensure long-term follow-up care.

Over the years, we have developed many ways to study the heart through imagery and, in turn, better plan surgery. We have also developed several new surgical techniques and strategies to gradually “grow” small heart structures. Better ways to assess and manage the heart’s electrical system have reduced complication rates, and protocols for post-operative management have improved the chances of surgical success.

Several years ago, we discovered that small heart structures can be stimulated to grow by promoting blood flow into the small side of the heart using surgical techniques — a process called staged ventricular recruitment. This must be done gradually and carefully, however, to avoid stressing the heart too quickly. The type of staged ventricular recruitment surgery needed to promote growth of the small heart depends on the type of congenital heart defect. If the heart grows as anticipated, then we can convert from single ventricle to biventricular or one-and-a-half ventricle circulation (biventricular conversion). If the heart does not respond adequately, then single ventricle management is still an option.

Boston Children’s cardiac surgeons and cardiologists are aiming to drastically reduce the number of children who need a pacemaker. In separate initiatives, they’ve created new technological approaches — an electrophysiology tool and a confocal microscope — that attempt to reduce the risk of postoperative heart block during biventricular repair and other surgeries, and, in turn, reduce the need for pacemakers. The advancements are showing promising results in helping our heart surgeons locate and avoid injuring the hard-to-see cells and electrical signals of conduction tissue.

We collect health information about patients who received biventricular repair treatment at Boston Children’s so we can assess how they are doing in the long run. This helps us better understand the effectiveness of our approach to patient care, and it lets us provide the most accurate information about treatment to families who ask us for second opinions.

If your child has biventricular surgical repair at Boston Children’s, you may be asked to participate in the outcomes survey BIV-PRO. The survey takes about 10 minutes to complete, and asks questions about your child’s quality of life and health status. If you would like to participate, please contact Sri Sreepada (sri.sreepada@childrens.harvard.edu).