Perinatal Decision Making for Preterm Infants with Congenital Heart Disease: Determinable Risk Factors for Mortality.

For premature infants with congenital heart disease (CHD), it may be unclear when the burdens of treatment outweigh potential benefits. Parents may thus have to choose between comfort care at birth and medical stabilization until surgical repair is feasible. Better defined outcome data, including risk factors for mortality, are needed to counsel expectant parents who are considering intensive care for premature infants with CHD. We sought to evaluate outcomes in this population to inform expectant parents considering intensive versus palliative care at birth. We performed a retrospective cohort study of infants born <34 weeks who received intensive care with critical or moderately severe CHD predicted to require surgery in the neonatal period or the first 6 months of life. 46 % of 54 infants survived. Among non-survivors, 74 % died prior to surgery (median age 24 days). Of the infants that underwent surgery, 75 % survived. Survival was lower among infants <32 weeks gestational age (GA) (p = 0.013), with birth weight (BW) <1500 g (p = 0.011), or with extra-cardiac anomalies (ECA) (p = 0.015). GA and ECA remained significant risk factors for mortality in multiple logistic regression analysis. In summary, GA < 32 weeks, BW < 1500 g, and ECA are determinable prenatally and were significant risk factors for mortality. The majority of infants who survived to cardiac intervention survived neonatal hospitalization, whereas most of the infants who died did so prior to surgery. For some expectant parents, this early declaration of mortality may support a trial of intensive care while avoiding burdensome interventions.

Beyond the Clinical Trials: Off-Protocol Therapeutic Hypothermia.

Mild therapeutic hypothermia has been extensively studied and validated as an effective and safe treatment for term and near-term infants with moderate and severe hypoxic encephalopathy meeting narrow inclusion criteria. Unanswered questions remain about whether cooling treatment can be optimized to improve outcomes even further, and whether it is reasonable to offer treatment to infants excluded from the foundational studies. Consideration of "off-protocol" cooling practices requires methodical review of available evidence and analysis using both a clinical and a research ethical framework.

Conjoined twins: an obstetrician's guide to prenatal care and delivery management.

OBJECTIVE: Obstetricians infrequently encounter conjoined twins. Much of the clinical care literature focuses on postnatal management from a neonatology and pediatric surgery perspective; guidance on obstetrical management is limited. We outline steps for prenatal evaluation, obstetrical care, and delivery planning. STUDY DESIGN: Experiences with two cases of conjoined twins. RESULTS: We identified several points throughout the planning, delivery, and postnatal process that are important to highlight for optimizing clinical outcome, patient safety, and parental satisfaction. CONCLUSION: After diagnosis, patients should be referred to a center experienced in the management of conjoined twins. Specialists in fields including maternal fetal medicine, pediatric surgery, neonatology, and radiology play a vital role in the management of these patients. Early referral allows for timely family counseling and decision-making. Prenatal evaluation beyond the first trimester should include a detailed ultrasound, fetal echocardiogram, and fetal MRI. 3D printed life-sized models can improve delivery planning and patient understanding.

Impact of standardized prenatal documentation among newborns with ductal-dependent heart disease.

INTRODUCTION: Absent risk stratification, variable physiology of ductal-dependent congenital heart disease (DD-CHD) imparts risk of over-medicalization and unnecessary exclusion from early parental bonding. METHODS: Single-center retrospective cohort study of term infants with isolated prenatally diagnosed DD-CHD expected to require immediate advanced resuscitation/intervention (2005-2017). Standardized documentation (2015 onward) included diagnosis, expected saturations, and allowability of postnatal parental bonding. RESULTS: The study cohort (n = 386) included 289 patients in the pre-standardized documentation era and 97 in the post-era; the groups had similar characteristics. Fewer infants were born by cesarean in the later era (32% vs. 22%; p = 0.049). Decrease in any respiratory intervention (38-26%; p = 0.03) followed standardized documentation. More patients had any bonding time (22-74%; p < 0.0001), without increase in CPAP or intubation in the first two hours of life (6.9% vs. 7.2%; p = 0.92). CONCLUSION: Standardized predelivery documentation for neonates with DD-CHD decreased unnecessary respiratory intervention and increased parental bonding.

When Do Pediatricians Call the Ethics Consultation Service? Impact of Clinical Experience and Formal Ethics Training.

Background: Previous research shows that pediatricians inconsistently utilize the ethics consultation service (ECS). Methods: Pediatricians in two suburban, Midwestern academic hospitals were asked to reflect on their ethics training and utilization of ECS via an anonymous, electronic survey distributed in 2017 and 2018, and analyzed in 2018. Participants reported their clinical experience, exposure to formal and informal ethics training, use of formal and informal ethics consultations, and potential barriers to formal consultation. Results: Less experienced pediatricians were more likely to utilize formal ethics consultation and more likely to have formal ethics training. The most commonly reported reasons not to pursue formal ECS consultation were inconvenience and self-reported expertise in pediatric ethics. Conclusions: These results inform ongoing discussions about ethics consultation among pediatricians and the role of formal ethics training in both undergraduate and graduate medical education.

Ethical decision-making for children with neuromuscular disorders in the COVID-19 crisis.

The sudden appearance and proliferation of coronavirus disease 2019 has forced societies and governmental authorities across the world to confront the possibility of resource constraints when critical care facilities are overwhelmed by the sheer numbers of grievously ill patients. As governments and health care systems develop and update policies and guidelines regarding the allocation of resources, patients and families affected by chronic disabilities, including many neuromuscular disorders that affect children and young adults, have become alarmed at the possibility that they may be determined to have less favorable prognoses due to their underlying diagnoses and thus be assigned to lower priority groups. It is important for health care workers, policymakers, and government officials to be aware that the long-term prognoses for children and young adults with neuromuscular disorders are often more promising than previously believed due to a better understanding of the natural history of these diseases, benefits of multidisciplinary supportive care, and novel molecular therapies that can dramatically improve the disease course. Although the realities of a global pandemic have the potential to require a shift from our usual, highly individualistic standards of care to crisis standards of care, shifting priorities should nonetheless be informed by good facts. Resource allocation guidelines with the potential to affect children and young adults with neuromuscular disorders should take into account the known trajectory of acute respiratory illness in this population and rely primarily on contemporary long-term outcome data.

Ethical considerations in the care of complicated twin pregnancies.

Twin gestations are increasing in prevalence worldwide, and are potentially subject to medical complications which present uniquely complex ethical and psychosocial challenges for the pregnant patient and obstetrician to navigate. In this article, we explore these issues as they relate to medical decision-making in cases of discordant growth and discordant anomalies in both monochorionic and dichorionic twin pregnancies, including those affected by twin-twin transfusion syndrome, with particular attention to scenarios in which the individual fetuses hold competing interests. For each of these scenarios, we consider how decisions may positively or negatively impact one or both fetuses, and how familiarity with population outcomes, as well as sensitivity to the complex psychosocial circumstances surrounding these pregnancies, can support and inform shared decision-making.

Fetal tachyarrhythmia associated with vibroacoustic stimulation.

BACKGROUND: Vibroacoustic stimulation is commonly used in antepartum fetal testing and has known benefits. Although the procedure has been deemed safe, questions have been raised as to potentially adverse effects, and the adult cardiology literature identifies a link between abrupt sound stimulation and severe tachyarrhythmias. CASE: A fetus with premature atrial contractions converted to supraventricular tachycardia immediately after vibroacoustic stimulation. The tachyarrhythmia spontaneously reverted to baseline after 4 minutes. The infant was treated for junctional reentry tachycardia at birth. CONCLUSION: Although the cardiac effects of vibroacoustic stimulation have previously been characterized as benign, the use of this modality may warrant restriction in the setting of known fetal arrhythmias.

Warning about warnings: weighing risk and benefit when information is in a state of flux.

In 2010, new data about the safety of recombinant human growth hormone (rhGH) resulted in warnings and subsequent pronouncements by the US Food and Drug Administration (FDA) and its European counterpart [the European Medicines Agency (EMA)] regarding its use in children and adolescents as an elective treatment for short stature. However, opinions about these new data are divergent: the FDA did not change the label of the drug and experts have argued for further research on the safety of rhGH. In this situation of an evolving scientific controversy, it is unclear how questions about benefit and risk are communicated to patients and their parents. Social biases and misperceptions about the deleterious effects of short stature and the benefits of added height influence decisions to prescribe rhGH and may affect discussions of the warnings by regulators. Fully supporting a model of shared decision-making involving children and adolescents requires sharing risk-benefit information, including evolving information from drug regulators, with patients and parents.