Values Beneath the Surface: Ethical Considerations in Interpreting Clinical Data for Children with Trisomy 13 and 18 Requiring Cardiac Surgery and Respiratory Support

Abstract

In the last decade, there has been a notable shift in the care of children with trisomy 13 (T13) and trisomy 18 (T18) that challenges the predominant view of nonintervention due to high early mortality rates in these cohorts. Recent literature suggests that the increased frequency of surgical interventions, increased parental involvement in decision-making, disability advocacy, family support groups, and social networks have influenced this paradigm shift. Given these factors, more children with T13 and T18 are receiving life-sustaining interventions and living longer. However, the heterogeneity of these genetic conditions and limitations of retrospective studies makes it difficult to determine the extent to which certain factors cause early mortality. Common factors associated with early mortality in patients with T13 and T18 are lung and airway diseases, abnormal respiratory control, and structural cardiac conditions. When congenital cardiac malformations and pulmonary disorders coincide, these pulmonary conditions can increase the risk of morbidity and mortality following cardiac surgery. Respiratory support is frequently mentioned as a negative predictor of postoperative survival, but few studies focus on the effect of respiratory support on survival outcomes. The goals of this analysis were to (1) characterize current morbidity and mortality in pediatric patients with T13 and T18 undergoing cardiac surgery, focusing on ventilation as a risk factor for survival, and (2) explore ethical considerations surrounding cardiac surgery through a role-based lens. This analysis considers how survival data and their interpretations influence judgments towards providing interventions to children with T13 and T18 who are dependent on respiratory technology. The findings from this analysis indicate that a majority of children who received preoperative MV or postoperative tracheostomy survived to hospital discharge. From examining the clinical manuscripts, quality-of-life assessments and perceptions of dis/ability, futility, and successful outcomes are compared among the papers’ conclusions. This analysis emphasizes how one’s values and relationship with the child influence clinical decision-making, underscoring the need for shared decision-making among parents and clinicians with regard to cardiac surgery in children with T13 and T18 receiving respiratory support.