Current Environment:

At the Boston Children’s Hospital Complex Biventricular Repair Program, we are always looking for innovative ways to improve our outcomes and to offer the option of complex biventricular repair or conversion to more children with borderline single ventricle defects. Our research and innovations are helping guide us to those goals. Close alignment with the Congenital Heart Valve Program has led to development of advanced valve repair and replacement techniques including expandable valve technology. Adjunctive therapies such as stem cell therapy to improve results of recruitment are being developed as well.

3-D printing of the heart

It can take anywhere between 30 minutes and an hour in the operating room to sort through a patient’s cardiac anatomy. During that time, the child is on cardiopulmonary bypass and anesthesia. To make the most of the time spent in surgery, pediatric cardiologists and surgeons at Boston Children’s have developed a program to use 3-D printing technology to create models of each child’s heart based on imaging tests. These models are used to aid surgical planning and to explain procedures to family members.

Sitaram Emani, MD, director the Complex Biventricular Repair Program at the Benderson Family Heart Center and his colleagues in the Department of Cardiac Surgery believe use of these models will reduce time in the OR and can help improve outcomes. The models can also be used by cardiologists and cardiac surgeons for education of families on their child’s specific anatomy and surgical repair. To study these assumptions, Emani and his colleagues are conducting a randomized clinical trial of 20 patients diagnosed with double outlet right ventricle (DORV). The intricately complex and highly variable cardiac anatomy of patients with DORV make it ideal for modeling with 3-D printing.

Mesoblast stem cell therapy for patients with single ventricle and borderline left ventricle

Emani is the principal investigator of this clinical trial to explore if mesenchymal precursor cells (MPCs) injected directly into the left ventricle can help promote growth of the ventricle.

The goal of the trial is to determine if injection of MPCs will help improve the chances of those patients with a single ventricle or borderline left ventricle (LV) of being converted to biventricular circulation, which could improve their quality of life and longevity over palliation.

This study is enrolling patients under the age of 5, with a diagnosis of hypoplastic left heart syndrome (HLHS), unbalanced atrioventricular canal (uAVC) or borderline left heart who are undergoing staged LV recruitment following single ventricle palliation.

Virtual surgery to model individual patient’s response to biventricular repair

Making the decision to undergo biventricular repair or conversion can be difficult given individual patient differences. We are developing complex computer modeling with our engineers to determine how each individual patient will respond to surgery in terms of blood pressure and heart function — factors that determine success of biventricular repair. Using data collected from catheterization, MRI and echocardiogram, “virtual surgery” is performed using computer simulation. The results can be used to assist clinicians in decision making.