Single Ventricle Cardiac Home Monitoring Program
The single ventricle cardiac home monitoring program at Children’s Minnesota provides comprehensive care for patients born with single ventricle heart disease. Led by a team of dedicated experts in single ventricle care, we are committed to improving outcomes through specialized treatment, evidence-based practices, multidisciplinary care, and family support.
What is single ventricle heart disease?
Single ventricle heart disease encompasses a group of heart defects in which the heart has only one viable pumping chamber (ventricle) instead of two. These severe congenital heart defects have an extremely high mortality rate if left untreated. The introduction of “staged palliation”, a series of heart surgeries that allow the body’s circulation to function with just one ventricle—has drastically improved survival rates.
More about single ventricle heart disease
- Single ventricle heart disease is rare, occurring in about 2-5 births per 10,000
- The cause is complex and not well understood. Genetics may play a role
- Diagnosis typically occurs prenatally (before birth) through ultrasound imaging
- There are several diagnoses that encompass single ventricle heart disease. These are some examples:
- HLHS
- Tricuspid atresia
- DILV
- Pulmonary atresia/ Intact ventricular septum (some types)
- Heterotaxy syndrome (some types)
- AVSD (unbalanced)
- DORV (some types)
Treatment approach and surgical stages
The current standard treatment for single ventricle heart disease involves a series of two or three open-heart surgeries. These surgeries enable the single pumping chamber to effectively distribute high-oxygen blood to the body and redirect low-oxygen blood directly to the lung vessels. This approach is palliative, as there is no cure for single ventricle heart disease.
The initial open-heart surgery is typically performed in the first week of life. The goal of this stage is to ensure proper blood flow to the body and lungs. The type of surgery (Norwood procedure, modified Blalock-Taussig-Thomas shunt, others) depends on the underlying heart anatomy.
The time between the first and second surgeries is called the interstage period. This is a high-risk time for infants with single ventricle heart disease. Babies are closely monitored through our single ventricle cardiac home monitoring program to detect and address any potential issues.
Around 4-6 months of age, the bidirectional Glenn procedure is performed. This surgery redirects low-oxygen blood from the upper body directly to the lung vessels. This allows for more stable circulation. Patients are discharged from the interstage monitoring program after this surgery.
Around 3-4 years of age, the Fontan procedure is performed. This is typically the last anticipated operation in the staged palliation treatment strategy. The Fontan procedure redirects low-oxygen blood from the lower body directly to the lungs. This surgery helps achieve near-normal oxygen saturation levels by separating low and high-oxygen blood circuits in the body.
Post-surgical care and follow-up
Patients require lifelong cardiology follow-up care after the Fontan procedure. This includes routine testing like echocardiograms, electrocardiograms, exercise stress tests, blood draws, heart rhythm monitors and cardiac catheterizations as recommended by your cardiologist.
Regular exercise is important for patients with single ventricle heart disease. Families will receive guidance from their cardiologist to ensure safe participation in physical activities. Most patients with single ventricle heart disease are encouraged to maintain an active lifestyle.
Children with single ventricle heart disease are at higher risk for neurodevelopmental and psychological challenges. Our clinic offers evaluation and care through the intensive care follow-up clinic to specifically support our cardiac families.
Despite ongoing medical advancements in the care of patients with single ventricle heart disease, there is an increased risk of heart failure and organ dysfunction. This may eventually require a heart transplant. Our heart failure and transplant team can help to deliver optimal care to patients showing signs of heart failure.
The Children’s Minnesota Difference
The Children’s Minnesota cardiovascular program is one of the largest and most well-established pediatric cardiovascular programs in the region. Our team consistently achieves treatment results that are among the best in the nation.
Our multidisciplinary team includes pediatric cardiologists, nurse practitioners, occupational therapists, nurses, and social workers. We collaborate locally, regionally, and internationally to improve patient care and outcomes.
- Mayo Clinic – Children’s Minnesota Cardiovascular Collaborative shares program resources and clinical talent to provide the highest-quality pediatric cardiology and cardiovascular surgery services to patients throughout the country.
- National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) is a multicenter network whose mission is to decrease mortality and improve quality of life for infants with single ventricle congenital heart disease and their families.
- Fontan Outcomes Network (FON) is a lifespan learning network made up of a community of patients, families, clinicians, and researchers who together seek to transform outcomes for all people affected by single ventricle heart disease by leveraging data to facilitate research, drive improvement, and spark innovation.
- Cardiac Neurodevelopmental Outcome Collaborative is a multicenter network whose mission is to determine and implement best practices of neurodevelopmental services for every individual with pediatric and congenital heart disease and their families through clinical, quality improvement, and research initiatives.
- Pediatric Acute Care Cardiology Collaborative (PAC3) is an international collaboration of centers with a mission to improve cardiac acute care outcomes.
- Pediatric Cardiac Critical Care Consortium (PC4) is an international consortium with a mission that aims to improve the quality of care to patients with critical pediatric and congenital cardiovascular disease.
