Brief Summary
This presentation outlines the comprehensive management of hypoplastic left heart syndrome (HLHS), emphasizing a team-based approach from prenatal diagnosis to postnatal care. It covers prenatal detection, counseling, delivery planning, immediate post-natal stabilization, surgical interventions (Norwood procedure, Glenn, and Fontan), and long-term care strategies including home monitoring and outpatient support. The goal is to improve outcomes for babies with HLHS, focusing on both medical and family support.
- Prenatal diagnosis and counseling are crucial for preparing families.
- Postnatal care focuses on stabilizing the infant and promoting fetal circulation.
- Surgical interventions are staged, beginning with the Norwood procedure.
- Long-term care includes home monitoring and comprehensive outpatient support.
Introduction to Hypoplastic Left Heart Syndrome
The presentation introduces a case involving the management of hypoplastic left heart syndrome (HLHS), highlighting the importance of a team approach. HLHS is characterized by a severely underdeveloped left ventricle, often accompanied by aortic and mitral valve stenosis or atresia, leading to impaired systemic circulation. The condition necessitates the ductus arteriosus to maintain blood flow to the body.
Prenatal Diagnosis and Management
Most prenatal diagnoses of HLHS occur around 18-20 weeks during routine anatomy ultrasounds, though sometimes later. Prompt referral to a fetal echocardiogram is essential to confirm the diagnosis and detail the specific anatomy. This stage is critical for initiating family counseling and education. Regular fetal echocardiograms are conducted around 26 and 32-34 weeks to monitor the condition and plan for delivery.
Prenatal Goals and Comprehensive Counseling
Prenatal goals include comprehensive counseling to discuss surgical strategies, short and long-term outcomes, and prenatal options, including termination if appropriate. Follow-up appointments monitor the growth of left heart structures and the patency of the atrial septum. A comprehensive delivery plan is developed, and families are counseled on potential issues in infancy and long-term care. A comprehensive counseling visit around 32 weeks involves fetal echocardiogram, maternal-fetal medicine consult, visits with pediatric cardiac ICU physicians, neonatologists, palliative medicine, and a congenital cardiac surgeon, along with facility tours and a detailed delivery plan.
Delivery and Initial Postnatal Stabilization
Delivery should ideally occur at a center equipped to provide definitive care for the infant. The special delivery unit is designed for high-risk mothers and infants needing tertiary care. Post-delivery, neonatology and cardiology promptly evaluate the infant, allowing for initial bonding time with parents if the baby is stable before transfer to the pediatric cardiac ICU. Initial stabilization involves maintaining the ductus arteriosus with prostaglandin to promote fetal circulation, optimizing systemic output, and minimizing pulmonary blood flow.
Surgical Management: Norwood Procedure and Beyond
Surgical intervention aims to convert the right ventricle into the systemic ventricle through the Norwood procedure, which includes reconstructing the aortic arch and creating a shunt for pulmonary blood flow. Subsequent stages include the Glenn and Fontan procedures, typically performed at six months and three to four years, respectively. Heart transplantation is considered if conventional surgery is unsuccessful.
Postoperative Care and Home Monitoring Program
Postoperative care addresses the multiple medical needs of these infants, who still have a single ventricle heart and often face increased metabolic demands, extra cardiac anomalies, and feeding intolerance. A "journey board" is used to guide families and nursing staff through the steps needed before hospital discharge. A home monitoring program includes a baby scale, pulse oximeter, a red flag action plan, and a laminated card with the baby's diagnosis and emergency information. Parents enroll in MyChart to track and share daily weight, pulse oximetry, and feeding volume with the care team.
Outpatient Care and Goals
Comprehensive outpatient visits are scheduled with a cardiologist, nurse coordinator, and dietitian to provide ongoing support. The ultimate goal is to transition these high-risk babies to a healthy life at home, despite the challenges posed by their condition.
