Improving Care for Infants with Single Ventricle Circulation
Infants with single ventricle heart defects are among the most complex and medically fragile patients cared for by the Heart Center. Hypoplastic left heart syndrome (HLHS) and other forms of single ventricle anatomy typically require three operations to palliate the heart. The first surgery, called the Stage I: Norwood procedure, takes place within the first several weeks of life. The second surgery, the Stage II: bi-directional Glenn shunt, is typically performed between 4-6 months of age. The period between Stage I and Stage II is known as the interstage period, and this is when infants are at the highest risk for adverse events and poor growth.
Two Heart Center initiatives, the National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC) and the Home Monitoring Program (HMP), are focused on reducing mortality and improving outcomes in these vulnerable infants and providing support to parents during this stressful period.
Boston Children’s Hospital was a founding member of the National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC), a national group of more than 56 pediatric cardiac centers whose aim is to improve survival and quality of life among infants discharged home during the interstage period. NPCQIC is based on a partnership between cardiologists, primary care providers, nurses, dietitians, social workers and parents/caretakers. The group developed a national data registry to identify and disseminate best practices in caring for interstage infants. David Brown, MD, and Teresa Saia, DNP, lead Heart Center clinicians in this effort, with data support from the Program for Patient Safety and Quality. Beginning in 2016, NPCQIC will expand its aims to include care of single ventricle infants from initial diagnosis through 1 year old. “This broader time frame will allow us to additionally focus on optimizing neurodevelopment and supporting families,” explains Dr. Brown.
The Home Monitoring Program is one example of a successful interstage care practice that has demonstrated improved outcomes for these infants. This program gives parents the education and tools to identify early signs of clinical changes at home, allowing for prompt assessment and treatment to avoid major adverse events. Prior to discharge, nurse practitioners provide extensive parent education about caring for infants with single ventricle, emphasizing clinical warning signs as well as growth and nutrition. Parents receive a binder with educational materials and daily log sheets to monitor their infants’ progress. At the time of hospital discharge, parents are also provided with an infant scale and a pulse oximeter.
Patricia O’Brien, CPNP, explains, “Every day, parents measure their child’s weight, monitor oxygen saturations and record their infants’ feedings in their log. This structure allows health care providers to note patterns over time.” Parents are also told to call their nurse or doctor if they observe certain “red flags,” such as faster or harder breathing, lower oxygen levels, cyanosis, fever, vomiting/diarrhea, low urine output or weight loss. In addition, families receive a weekly phone call from a Heart Center nurse practitioner who monitors the infant’s progress and addresses parent questions and concerns. Heart Center clinicians also maintain close communication with primary care providers, referring cardiologists and community resources, such as visiting nurses, to assure continuity of care during the interstage period.
Poor growth and feeding problems are significant issues for interstage patients. The addition of a registered dietitian in 2012 added a valuable resource to the Heart Center’s HMP care team. Erin Keenan, RD, evaluates every single ventricle patient prior to discharge and communicates with families on a weekly basis (at a clinic visit for patients followed in Boston or via phone for patients followed remotely). She collaborates with parents, pediatricians, cardiologists and nurse practitioners to ensure adequate caloric intake and make nutrition adjustments during the interstage period. Keenan’s involvement has helped the program achieve adequate interstage weight gain in 85 percent of patients. “I am deeply dedicated to proving these fragile babies can grow with close follow-up and individualized care,” she says.
Another important focus of NPCQIC and HMP efforts is active parent engagement during the interstage period. Saia observes that “parents are a crucial component of the care team, as they are in the best position to observe daily variations in their child’s condition. They are also able to advocate for care processes that optimize their child’s health and quality of life during the interstage period.” Shannon Jasper is one such parent who felt supported during the stressful interstage period. Says Jasper, "The HMP gave us a sense of security during a time full of unknowns. It was comforting to have our NP and dietitian checking in frequently to ensure our son was OK."
Margaret Schroeder, PNP, Home Monitoring Program coordinator, has been tracking the program’s progress. She reports that since 2009, “195 patients have been enrolled in our HMP: 99 with HLHS, 82 with other single ventricle types and 14 with other high-risk cardiac defects.”
Thus far, the Heart Center’s quality improvement efforts have resulted in positive change. Overall, interstage mortality has declined from a baseline of 11 percent in 2009 (when Boston Children’s Hospital joined NPCQIC) to 6 percent in 2014. Parents, cardiologists and referring providers have related increased satisfaction with interstage care coordination and communication.