Ethical Principles and Parental Choice: Treatment Options for Neonates With Hypoplastic Left Heart Syndrome

Vicki L. Zeigler


Pediatr Nurs. 2003;29(1) 

In This Article


HLHS is a developmental cardiac malformation that involves the left ventricle, aorta, and the mitral valve (Lev, 1952; Noonan & Nadas, 1958). It is the fourth most common critical congenital heart defect and occurs in 0.267 per 1,000 live births (Ferencz et al., 1985). Without treatment, 95% of neonates diagnosed with HLHS will die within 1 month, with none surviving beyond 4 months of life (Stuart, Wren, & Staples, 1991). Due to the severity of the defect and the "time-sensitivity" of specific treatment modalities, an option must be chosen and initiated quickly.

The treatment options available for the neonate with HLHS include medical termination of pregnancy (if detected early enough); palliative care, also known as comfort care or compassionate care (Zahka, Spector, & Hanisch, 1993); cardiac transplantation; or three stages of surgical palliation to reconstruct the heart and its supporting vasculature. Medical termination of pregnancy is an option if the diagnosis has been made by fetal ultrasound or echocardiography prior to a gestational age of 6 months. Palliative care, initiated when parents opt not to pursue surgical treatment, results in the baby dying, usually within the first week of life. Cardiac transplantation offers a greater chance of survival if performed in the first month of life, while the first stage of surgical palliation (known as the Norwood procedure) must be performed within the first few days to weeks of life. Table 1 offers resources for each of the options. This article will address the three options the parents face when a live child is born.

Parents generally must choose one of three available treatment options for their child: palliative care (the initiation of pain and symptom management provided with dignity and compassion for the baby and cultural, spiritual, and practical support for the parents [Catlin & Carter, 2002]), cardiac transplantation, or three stages of reconstructive surgery. Each of these choices has advantages and disadvantages that must be considered in the decision-making process. While the options are being considered, the infant may be managed with mechanical ventilation and various medications commonly used to keep the infant alive such as prostaglandin E1. If palliative care is chosen, these treatments will be tapered off and pain and symptom management will be the focus of care. Pearson (1997) described palliative care for the non-viable infant in the hospital setting, recounting the parents' wishes for a supportive team and a pain free death for their infant. Some parents may wish to take their infant home, and this is arranged with support from social services in conjunction with the local hospice team (Catlin & Carter, 2002).

Cardiac transplantation offers the advantage of providing the child with a structurally normal heart. Transplantation has been shown to be most successful when performed in the first month of life (Neglsen-Cannarella & Chang, 1992) with survival rates reported between 82% and 89% (Bailey & Gundry, 1990). Approximately 61% of children are alive at 1 year after transplant and 55% at 5 years (Jenkins et al., 2000). Although the organ recipient gains normal cardiac function if the transplant is successful, the child's physical and social activities may be restricted. Fleisher and colleagues (2002) also found cognitive performance and other developmental benchmarks in children with heart transplants below that of normal children. One of the disadvantages of transplantation is the potential need to transfer the neonate to a transplant center, which often means the family must relocate as well. This move adds to the significant stress and financial constraints already faced by the family. Another disadvantage to transplantation is the life-long need to balance preventing rejection with immunosuppressive therapy against the risk of succumbing to infection. The shortage of donors presents a significant limitation as well. In a recent study comparing 1-year survival rates between patients undergoing transplantation versus the staged surgical reconstruction, 63% of patients in the transplant group died while waiting for a donor heart (Jenkins et al., 2000).

Staged surgical palliation offers benefits but with important limitations as well. More pediatric cardiac centers perform the Norwood procedure versus cardiac transplantation. Moreover, advances in surgical technique have improved survival rates for these children; however, surgical palliation presents several limitations. The first surgical stage, the Norwood procedure, may not be successful, and the hospitalization may be very prolonged while feeding and other medical issues are resolved. Recent data indicates that the majority of deaths occur in hospitalized children after Stage I of reconstruction, but survival increased significantly over the 15-year study period (Mahle, Spray, Wernovsky, Gaynor, & Clark, 2000). In addition, surgical complications may occur at any stage. Ultimately, after all three surgical procedures, the child will be left with single ventricle physiology and, as a result, limitations in physical activity. Studies also indicate that many of these children later exhibit significant neurocognitive and neurodevelopmental impairment (Mahle, Clancy, et al., 2000).

In summary, parents with a neonate with HLHS are faced with treatment choices that offer no "cure" for this serious heart defect. The consequences of each of these options must be weighed by the family in order for them to make the best decision for their individual situation. Unfortunately, often the health care team, so earnest in their desire to help save the child, does not always know or discuss with families the realities of these treatment options as well as the problematic future developmental sequelae The urgency of making a treatment decision may not allow for extended time to discuss these future realities, yet parents must be made aware of them in order to truly make an informed decision.


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