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When a baby is born with single ventricular heart disease (SVHD), a condition where only one heart ventricle functions, it is often fatal without surgical intervention. This rare disorder affects 1 in 15,000 babies born in the U.S. and is caused by an underdeveloped lower heart chamber. Babies with this condition may experience rapid or labored breathing, bluish or ashen skin and difficulty feeding. Multiple surgeries are needed to ensure the child’s survival, beginning shortly after birth. These surgeries are lifesaving but lead to lifelong health complications, such as arrhythmias (irregular heartbeats) and liver disease, due to both the original defect and the additional surgeries required as the child grows.
To advance understanding of this complex condition, a new $13 million three-year research initiative will bring together scientists from multiple institutions to challenge current knowledge and explore potential cures for SVHD. Funded by the American Heart Association/Additional Ventures Collaborative Sciences Awards in Single Ventricle Heart Disease, the five teams involved will focus on different aspects of the disease and the complications caused by the numerous heart surgeries.
One of the teams will focus on broadening the knowledge and treatment of arrhythmias in patients with SVHD. Their project, funded by the Collaborative Science Award, is titled PULSE-SVA Network: Personalized Understanding from Linked Simulations & Electrophysiology in Single Ventricle Arrhythmia. The researchers include Patrick Boyle, associate professor of UW Bioengineering, Isabelle Deschênes of The Ohio State University College of Medicine, Stacey Rentschler of Washington University in St. Louis and Mingtao Zhao at Nationwide Children’s Hospital.
The team will investigate the electrical mechanisms behind arrhythmias at both the cellular and tissue levels, while also exploring the potential of cardiac radiotherapy as a novel potential treatment. Boyle, who leads the Cardiac Systems Simulation (CardSS) Lab, will contribute his expertise in using computer models of the heart to study heart rhythm disorders.
“We don’t know a lot about why these patients have arrhythmias,” Boyle said. “Often, they will go into heart failure at the same time as they start having clinical arrhythmias. The priority then becomes treating the heart failure rather than addressing the arrhythmias.” Boyle’s CardSS lab will examine data from research conducted on “induced” heart cells derived from stem cells of patients with SVHD and from siblings who don’t have the disorder. The lab will also obtain MRI scans of adult patients with SVHD.
“We can see what the heart geometry looks like for patients with SVHD, and we can analyze the patient-specific distribution of diseased tissue,” Boyle stated. “From there, we can make models and then incorporate the electrical properties from experiments conducted by our partners in the PULSE-SVA Network. The models will allow us to better understand where the arrhythmias come from and what we can do to treat them more effectively and safely. We are particularly excited to explore the feasibility of using the emergent technology of noninvasive cardiac radiation therapy in this context.”
The team’s work on arrhythmias in SVHD patients will play a key role in the larger research effort, helping to uncover insights and develop new treatments for this complex disease. Learn more about the project and the research other teams are conducting.