Ethical considerations for cardiac surgical interventions in children with trisomy 13 and trisomy 18

Abstract

Medical and surgical approaches to children with trisomy 13 and 18 are evolving, and an increasing number of patients are being considered for simple and complex cardiac procedures. This review describes how the shifts in medical and social considerations for children with trisomy 13 and 18 mirror the shifts that occurred 50 years ago for children with trisomy 21. Yet the variability in cardiac lesions, and variability in non‐cardiac comorbidities, is much greater for patients with trisomy 13 and 18 than for those with trisomy 21. That variability, combined with the severe neurologic impairment in survivors, complicates the current risk: benefit balance of surgical intervention. Consistent approaches to care for these patients should be built on an evidence base, and should include contributions from specialists in medical ethics and palliative care.

Interdisciplinary care of children with trisomy 13 and 18

“…Cardiac surgical intervention offerings for children with trisomy 18 and 13 remain controversial in this decade (Janvier, Okah, Farlow, & Lantos, 2011; Lantos, 2016; Pallotto & Lantos, 2017), as did cardiac surgical interventions for children with trisomy 21 until the late 1970s (Champagne, Lewis, & Gilchrist, 2014; Kett, 2020; Neubauer & Boss, 2020). Geography and health systems impact the approach to cardiac surgery, as 37% of children with trisomy 13 and 18 referred to palliative care in the United States had accessed cardiac surgical interventions while none of these patients had in the United Kingdom (Mullin et al, 2019).…”

“…The ITTP members exchange knowledge grounded in each member's unique specialty insight and familiarity with the growing trisomy‐specific evidence base from both institutional experience and medical literature. Recognizing that technology and surgical interventions for children with trisomy 13 and 18 remain controversial within and across medical disciplines based on historic stigma (Harahsheh & Cohen, 2019; Neubauer & Boss, 2020), the ITTP team has benefited from inclusivity of diverse perspectives for open discussion and academic dialogue.…”

Discipline-Specific Perspectives on Caring for Babies with Trisomy 13 or 18 in the Neonatal Intensive Care Unit

“…27,32,46 The message of inevitable early death and poor quality of life is no longer a definitive black-and-white message for parents or providers, leaving both to engage with evolving care models. 4,29 Interview staff depicted a clear sense of uncertainly in prognostic outcomes, complicated by genetic and phenotypic variability and a shifting biomedical landscape. One quarter of all interviewed NICU staff (22% nurses, 21% APPs, and 44% neonatologists) mentioned that they grapple with uncertainty regarding biomedical interventions and technology escalations 1 ; citing professional desire to care without causing harm and to provide meaningful and quality care sensitive to family experiences as their actual black-andwhite principles, although the phenotypic variability and prognostic outcomes remained less clear.…”

The state of congenital heart disease

“…An important consideration in genetic diagnoses with a poor prognosis is the decision to repair structural heart defects. Neubauer and Boss address the ethics issue in correcting CHD surgically in individuals affected by Trisomies 13 and 18 (Neubauer & Boss, 2020). Improved care, especially surgical interventions, has resulted in most children with CHD in developed countries surviving to adulthood, resulting in more adults with CHD than children.…”

“…An important consideration in genetic diagnoses with a poor prognosis is the decision to repair structural heart defects. Neubauer and Boss address the ethics issue in correcting CHD surgically in individuals affected by Trisomies 13 and 18 (Neubauer & Boss, ).…”