Ethical Considerations and Multidisciplinary Care for Pediatric Patients with Hypoplastic Left Heart Syndrome: A Narrative Review with a Systematic Search

Mojca Zeleznik^1*Urh Groselj^2,3,4,5Petja Fister^5,6,7

• ¹Department of Neonatology, University Children's Hospital, University Medical Centre Ljubljana, Ljubljana, Slovenia
• ²Department of Endocrinology, Diabetes and Metabolism, University Children's Hospital, University Medical Centre Ljubljana, Ljubljana, Slovenia, Ljubljana, Slovenia
• ³Department of Medical Ethics, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia, Ljubljana, Slovenia
• ⁴Department of Pediatrics, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia, Ljubljana, Slovenia
• ⁵National Medical Ethics Committee of Slovenia, Ljubljana, Slovenia
• ⁶Department of Pediatric Critical Care, University Children’s Hospital, University Medical Centre Ljubljana, Ljubljana, Slovenia, Ljubljana, Slovenia
• ⁷Department of Pediatrics, Faculty of Medicine, University of Ljubljana, Ljubjana, Slovenia

Background: Hypoplastic left heart syndrome (HLHS) was a fatal congenital heart defect (CHD) until the 1980s. Introduction of the Norwood procedure and subsequent Fontan operation significantly improved survival by creating a single-ventricle circulation. Due to the high mortality associated with the Norwood operation, neonatal orthotopic heart transplantation emerged as an alternative, despite challenges such as lifelong immunosuppressive treatment and uncertain longevity of transplanted hearts. Methods: A narrative review with a systematic literature search was conducted in the PubMed, following PRISMA guidelines and included studies of ethical and medical considerations, decision-making, counseling and treatment planning in children with HLHS. In addition, we manually screened reference lists to identify further relevant literature. We aimed to explore: how do ethical considerations and decision-making processes influence the management and outcomes of fetuses and neonates with HLHS and their families across prenatal, postnatal, and long-term care? Results: Of the 115 studies, 56 met the inclusion criteria. Early diagnosis through prenatal fetal ultrasound has markedly improved survival rates by enabling better parental awareness, counseling, and decision-making. Managing HLHS requires urgent, extensive, and costly medical interventions, with outcomes influenced by the healthcare system's expertise, the experience of cardiologists and surgeons, ethical, legal, and religious considerations of the parents and medical team. The prenatal phase is crucial for optimal management, with advanced fetal ultrasound facilitating early detection. Postnatal care involves a multidisciplinary approach, including stage palliation physiology/surgery tailored to each patient. Despite surgical advancements, HLHS patients face higher morbidity and mortality rates than other patients with CHDs, with long-term survival and quality of life remaining key concerns. Ethical considerations play a significant role in the management of HLHS, encompassing the autonomy of families, the best medical interests of the child, societal, and cultural factors. Decision-making must balance full disclosure with sensitivity to parents' values and beliefs. Conclusions: Management of HLHS involves multidisciplinary approach with complex medical and ethical considerations, but the current literature lacks high-quality studies or consensus guidelines on ethical decision-making. Therefore, the influence of ethical considerations on clinical management and patient care remains unclear, highlighting the need for further research.