Heart Institute at UPMC Children’s Single Ventricle Program: Supporting Patients and Families Through the Interstage Period
February 14, 2025
The Single Ventricle Program at the Heart Institute at UPMC Children’s Hospital of Pittsburgh was officially launched in March 2024 to address the complex and multidisciplinary needs of infants with single ventricle congenital heart defects during the high-risk interstage period.
This program, designed to allow patients to transition safely from hospital to home between their first and second surgeries, has supported 15 patients and their families as of this writing. By leveraging multidisciplinary expertise and family-centered strategies, the program has created a new standard of care at UPMC Children’s for this patient population.
Leading the development of the program and its ongoing work is a dedicated team, including pediatric cardiologists Lizabeth Lanford, MD, associate professor of Pediatrics; Evonne Morell, DO, associate professor of Pediatrics and director of Inpatient General Pediatric Cardiology, and pediatric cardiology advanced practice providers Stacey Green, PA-C, and Elizabeth Pratt, CRNP. The pediatric cardiology team also collaborates closely with colleagues in multiple rehabilitation disciplines, social work, complex care management, and others.
Navigating the High-Risk Interstage Period
Single ventricle congenital heart defects (SV), such as (the most common of SV) hypoplastic left heart syndrome (HLHS), are among the most complex congenital heart conditions faced by pediatric cardiology and surgery teams. These defects require multiple staged surgical interventions, with the interstage period — the time between the patient's first procedure (Norwood procedure) and second (Glenn procedure) surgeries — being particularly precarious.
“Historically the interstage period carried a significant mortality risk,” says Dr. Morell. “As a result, most of these patients remained hospitalized. Families were frustrated by prolonged hospital stays, but the lack of structured follow-up and technological advancements in care and remote monitoring made home care too risky in most cases in prior years. We knew we needed a better solution.”
The primary goal of the Single Ventricle Program is to ensure infants receive the necessary medical oversight and care while benefiting from the developmental and emotional advantages of being at home instead of the hospital. Families of single ventricle patients play a central role in this effort, and their empowerment through education and resources is a bedrock component of the program’s success.
Genesis of the Program
The idea for the Single Ventricle Patient Care Program evolved over many years. Historically, single ventricle patients were either discharged home without structured follow-up or transitioned to The Children’s Home in Pittsburgh, a facility that provided daily monitoring and therapies such as physical, occupational, and speech therapy. While this model worked well for some time, recent challenges such as the COVID-19 pandemic revealed limitations, including restricted visitation and staff shortages.
“COVID forced us to reevaluate our approach,” says Dr. Lanford. “Families couldn’t visit freely, and keeping patients in the hospital for extended periods often led to developmental delays and increased feeding issues. We know that in most instances babies generally do better at home so making that a reality has been a priority initiative for the Heart Institute.”
To develop the program, the team relied upon the vast expertise and resources available at the Heart Institute and the broader UPMC Children's Hospital and UPMC system, but also drew inspiration from similar programs in existence in other parts of the country, adapting some aspects of these models and best practices of care to meet the unique needs of the single ventricle patient population at UPMC Children’s and the broad geographic region from which patients live in and are referred from.
“The result is a robust, multidisciplinary program designed to provide safe, comprehensive home-based care during the interstage period,” says Dr. Morell.
Core Elements of the Program
The Single Ventricle Program is built around a multidisciplinary team that includes pediatric cardiologists, advanced practice providers (APPs), neurodevelopmental specialists, social workers, and therapists. Together, the team collaborates to ensure that each family entering the program is fully equipped and trained to care for their child at home while they prepare for their next surgery.
Families undergo extensive preparation for caring for their child at home, beginning weeks before discharge. The training process is rigorous, covering medication administration, feeding techniques for both oral and tube-fed patients, and CPR certification. Parents are also trained to identify critical red flags for when either an emergency room visit is necessary or a call to emergency medical services, such as weight loss, low oxygen saturation levels, and dehydration, ensuring they can respond promptly to potential complications.
“The training is intense but necessary,” says Dr. Lanford. “We ask a lot from these families. The preparation is comprehensive because the safety and well-being of the patient depend on their readiness and ability to respond to the needs of their child.”
Another part of the preparation family caregivers must go through and successfully navigate is a 24-hour live-in at the Heart Institute, during which families spend 24 hours providing, managing, and ordering all aspects of care for their child under hospital supervision. This experience ensures that parents are confident and capable of managing the complex care their child requires at home.
To support families, the program provides each with a personalized binder tailored to their child’s specific needs and plan of care. The binder includes essential contact information, detailed care instructions, and emergency protocols. It also contains guidelines for managing specific issues, such as the cautious use of oxygen in interstage patients, which can be harmful if not properly managed or administered. This binder serves as a comprehensive resource that families can reference at any time, bolstering their confidence and preparedness.
The program’s multidisciplinary approach extends beyond the initial training and discharge. Weekly clinic visits alternate between pediatric cardiologists and APPs to provide consistent monitoring and early identification of any issues. Neurodevelopmental evaluations are integrated into the care plan, along with speech, occupational, and physical therapy services. Social workers play an essential role in addressing logistical and other challenges that may arise or affect a family, challenges such as transportation and financial assistance, often coordinating temporary housing through the local Ronald McDonald House or similar resources.
“Our approach ensures that families receive not only the medical support they need but also the resources they need to navigate the challenges of interstage care,” says Dr. Morell. “Many of our families live hours away from the Heart Institute, so we are cognizant of the extra demands this can bring about.”
Community integration is another element of the program that makes it possible for single ventricle patients and families to be at home instead of the hospital. Upon discharge, local emergency medical services (EMS) and emergency departments (EDs) are notified about the presence of these high-risk patients in their area.
“This proactive communication ensures that first responders and local health care providers are familiar with the specific care protocols required for these interstage patients, further enhancing their safety," says Dr. Lanford. "We make it a priority that these groups know what the plans of action are should their services be needed, and it's specific to the individual case."
Improve Quality of Life and Outcomes Through Care at Home
The benefits of home care for interstage single ventricle patients are profound. Infants who transition to home often demonstrate better feeding and developmental outcomes than those who remain hospitalized for the entirety of the interstage period.
At home, babies can bond with their families, interact with siblings, and experience a more natural environment for growth. For such a complex patient population, it may sound counterintuitive, but all things being equal the hospital is the least optimal place for them to be during this time.
“At home, babies receive the individualized attention and stimulation that are critical for their optimal development, says Dr. Morell. “The improvements in feeding that we see in patients living at home alone can make a significant difference in their overall health and ability to tolerate their subsequent surgical needs.”
Evidence in the literature and also from data tracking at UPMC Children’s suggests that sending patients home during the interstage period may also extend the time between the Norwood and Glenn procedures.
“By allowing patients to grow stronger and healthier at home, we’ve been able to delay the Glenn procedure until the baby is more developed, often closer to five or six months of age,” says Dr. Lanford. “This additional time can improve surgical outcomes and overall recovery and its one of the areas in which we hope to study rigorously.”
Challenges and Family Feedback
Despite the many benefits, the program is not without its challenges for patients and families. Families must manage the stress of providing complex medical care while remaining alert for potential complications.
The program’s APPs play a vital and ongoing role in supporting families, often fielding multiple daily calls from the same family to provide reassurance, answer questions, and address concerns. This ongoing communication stream between providers and families is necessary to assist families in navigating the demands, complexities, and challenges of interstage care for a single ventricle patient.
Feedback from families reflects the dual nature of the experience. While parents appreciate the opportunity to bring their child home, the responsibility can feel overwhelming.
“Our team works tirelessly to support families and alleviate their concerns,” says Dr. Morell. “We recognize that the demands of interstage care are significant, and we strive to provide the resources and reassurance they need.”
Expanding the Program’s Reach
As the Single Ventricle Patient Program continues to grow, the team is exploring ways to expand its services to other patient populations with complex congenital heart conditions.
Advances in surgical techniques and interstage monitoring have dramatically improved survival rates for single ventricle patients, leading to a growing population transitioning into adolescence and adulthood.
“The integration of neurodevelopmental services and therapies has been particularly impactful,” says Dr. Lanford. “We hope to extend these resources to more patients, ensuring that all children with complex heart conditions receive the care and support they need.”
The Single Ventricle Program at the Heart Institute at UPMC Children’s exemplifies how a multidisciplinary, family-centered approach can transform outcomes for a complex and high-risk patient population. By addressing the known and anticipated challenges of the interstage period, the program ensures that patients receive the best possible care while empowering families to play an active role in their child’s health care journey.
“As our program evolves, its impact will continue to grow,” says Dr. Morell “We’re helping to set a new standard for excellence in congenital heart care, locally and nationally."
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