Hypoplastic Left Heart Syndrome: Parent Support for Early Decision Making

Abstract

No extramural funding has been provided for this manuscript. No previous presentations of this manuscript have been made. No commercial financial support has been provided for this manuscript.Hypoplastic Left Heart Syndrome is a life threatening congenital cardiac anomaly. After a child has been diagnosed with hypoplastic left heart syndrome, parents must make life or death decisions within days of birth. Healthcare providers must provide appropriate education so that parents are able to make informed, timely decisions. Information regarding the diagnosis, treatment options, and parental decision making process for initial decision making for hypoplastic left heart syndrome are provided to guide nurses who work with these families. The challenging decision making process which parents must go through after diagnosis of hypoplastic left heart syndrome will be described.Rachel and Evan, a young couple expecting their first child, received unexpected and devastating news at the completion of a routine screening ultrasound at 20 weeks. The ultrasound revealed that their unborn son had a congenital heart defect. After further testing, the diagnosis of hypoplastic left heart syndrome (HLHS) was confirmed. Rachel and Evan received information about HLHS from several physicians, made an appointment with a pediatric cardiothoracic surgeon to discuss surgical options, and found information from pediatric hospitals and HLHS online support groups. Ultimately, Rachel and Evan had decisions to make. After several sleepless nights of reviewing information, statistics, options, and prognosis, they elected to continue with the pregnancy and give their son a chance at life.

Palliative Care Across the Life Span for Children With Heart Disease: A Scientific Statement From the American Heart Association

“…Educating families with appropriate diagnostic and prognostic information while acknowledging uncertainty in individual outcomes is required at the time of fetal diagnosis to guide shared decision-making regarding multiple treatment options. 55,131,132 Duration and location of counseling along with type of counseling materials affect knowledge transfer. Understanding family values and beliefs may improve family-centered decision support.…”

Parental decision-making for medically complex infants and children: An integrated literature review

“…Medically complex children have chronic complex conditions (e.g., cardiovascular disease, congenital abnormalities), potential dependence on technology (e.g., tracheostomy, cerebral spinal fluid shunts), frequent inpatient admissions, parental administration of multiple medications, multiple specialists involved in care (Srivastava et al, 2005), and potentially an early death (Morris, 2009; Ortenstrand et al, 2010). Decision-making for medically complex children begins at diagnosis and continues throughout the child’s life with each prior decision effecting the next decision (Toebbe et al, 2012). …”