Guidance for Pediatric End-of-Life Care.

The final hours, days, and weeks in the life of a child or adolescent with serious illness are stressful for families, pediatricians, and other pediatric caregivers. This clinical report reviews essential elements of pediatric care for these patients and their families, establishing end-of-life care goals, anticipatory counseling about the dying process (expected signs or symptoms, code status, desired location of death), and engagement with palliative and hospice resources. This report also outlines postmortem tasks for the pediatric team, including staff debriefing and bereavement.

Ethical considerations of maternal-fetal intervention in a twin pregnancy discordant for anomalies.

BACKGROUND: Recent evidence suggests prenatal fetoscopic tracheal occlusion (FETO) may improve the survival and long-term morbidity of neonates with congenital diaphragmatic hernia, yet little guidance exists in the medical literature as to the ethical permissibility of performing a maternal-fetal surgical intervention in a twin pregnancy discordant for a structural abnormality. CASE: Here, we present a case of a twin pregnancy with an unaffected twin (Twin A) and a twin diagnosed with severe congenital diaphragmatic hernia (Twin B). A proposed fetoscopic tracheal occlusion (FETO) procedure may improve the likelihood of survival and postnatal outcome of Twin B; however, balloon placement may also initiate very preterm birth at 28 weeks of gestation. The Fetal Ethics Advisory Board was asked to provide guidance on the permissibility of FETO in this pregnancy. DISCUSSION: A literature review identified one brief mention of FETO in a 34-week dichorionic twin pregnancy in the medical literature, which resulted in the rupture of fetal membranes in the sac of the nonsurgical twin. Only one paper specifically addressed the question of whether it would be ethically permissible to subject a healthy twin to the risks of maternal-fetal surgery for the benefit of a compromised twin, finding that any risk to the unaffected twin would be an ethical contraindication. We offer our own analysis of moral weight and risk/benefit considerations of this proposed intervention, and present our findings on the circumstances in which it may be ethically permissible to perform a maternal-fetal intervention in a twin pregnancy. CONCLUSION: While FETO was not ethically advisable in this pregnancy, we find that in limited circumstances, certain maternal-fetal surgical interventions may be ethically permissible in a twin pregnancy discordant for a structural abnormality if the risks to the unaffected twin are minimal and the procedure would improve the likelihood of survival and postnatal outcome of a critically compromised co-twin.

Innovative 2-Step Management Strategy Utilizing EXIT Procedure for a Fetus With Hypoplastic Left Heart Syndrome and Intact Atrial Septum.

Hypoplastic left heart syndrome (HLHS) with intact atrial septum (HLHS-IAS) carries a high risk of mortality and affects about 6% of all patients with HLHS. Fetal interventions, postnatal transcatheter interventions, and postnatal surgical resection have all been used, but the mortality risk continues to be high in this subgroup of patients. We describe a novel, sequential approach to manage HLHS-IAS and progressive fetal hydrops. A 28-year-old, gravida 4 para 2 mother was referred to Mayo Clinic for fetal HLHS. Fetal echocardiography at 28 weeks of gestation demonstrated HLHS-IAS with progressive fetal hydrops. The atrial septum was thick and muscular with no interatrial communication. Ultrasound-guided fetal atrial septostomy was performed with successful creation of a small atrial communication. However, fetal echocardiogram at 33 weeks of gestation showed recurrence of a pleural effusion and restriction of the atrial septum. We proceeded with an Ex uteroIntrapartum Treatment (EXIT) delivery and open atrial septectomy. This was performed successfully, and the infant was stabilized in the intensive care unit. The infant required venoarterial extracorporeal membrane oxygenator support on day of life 1. The patient later developed hemorrhagic complications, leading to his demise on day of life 9. This is the first reported case of an EXIT procedure and open atrial septectomy performed without cardiopulmonary bypass for an open-heart operation and provides a promising alternative strategy for the management of HLHS-IAS in select cases.

Attitudes of paediatric and obstetric specialists towards prenatal surgery for lethal and non-lethal conditions.

BACKGROUND: While prenatal surgery historically was performed exclusively for lethal conditions, today intrauterine surgery is also performed to decrease postnatal disabilities for non-lethal conditions. We sought to describe physicians' attitudes about prenatal surgery for lethal and non-lethal conditions and to elucidate characteristics associated with these attitudes. METHODS: Survey of 1200 paediatric surgeons, neonatologists and maternal-fetal medicine specialists (MFMs). RESULTS: Of 1176 eligible physicians, 670 (57%) responded (range by specialty, 54%-60%). In the setting of a lethal condition for which prenatal surgery would likely result in the child surviving with a severe disability, most respondents either disagreed (59%) or strongly disagreed (19%) that they would recommend the surgery. Male physicians were twice as likely to recommend surgery for the lethal condition, as were physicians who believe that abortion is morally wrong (OR 1.75; 95%CI 1.0 to 3.05). Older physicians were less likely to recommend surgery (OR 0.57; 95%CI 0.36 to 0.88). For non-lethal conditions, most respondents agreed (66% somewhat, 4% strongly) that they would recommend prenatal surgery, even if the surgery increases the risk of prematurity or fetal death. Compared with MFMs, surgeons were less likely to recommend such surgery, as were physicians not affiliated with a fetal centre, and physicians who were religious (ORs range from 0.45 to 0.64). CONCLUSION: Physician's attitudes about prenatal surgery relate to physicians' beliefs about disability as well as demographic, cultural and religious characteristics. Given the variety of views, parents are likely to receive different recommendations from their doctors about the preferable treatment choice.

Weighing the Social and Ethical Considerations of Maternal-Fetal Surgery.

OBJECTIVES: The ethics of maternal-fetal surgery involves weighing the importance of potential benefits, risks, and other consequences involving the pregnant woman, fetus, and other family members. We assessed clinicians' ratings of the importance of 9 considerations relevant to maternal-fetal surgery. METHODS: This study was a discrete choice experiment contained within a 2015 national mail-based survey of 1200 neonatologists, pediatric surgeons, and maternal-fetal medicine physicians, with latent class analysis subsequently used to identify groups of physicians with similar ratings. RESULTS: Of 1176 eligible participants, 660 (56%) completed the discrete choice experiment. The highest-ranked consideration was of neonatal benefits, which was followed by consideration of the risk of maternal complications. By using latent class analysis, we identified 4 attitudinal groups with similar patterns of prioritization: "fetocentric" (n = 232), risk-sensitive (n = 197), maternal autonomy (n = 167), and family impact and social support (n = 64). Neonatologists were more likely to be in the fetocentric group, whereas surgeons were more likely to be in the risk-sensitive group, and maternal-fetal medicine physicians made up the largest percentage of the family impact and social support group. CONCLUSIONS: Physicians vary in how they weigh the importance of social and ethical considerations regarding maternal-fetal surgery. Understanding these differences may help prevent or mitigate disagreements or tensions that may arise in the management of these patients.

Left cardiac sympathetic denervation for the treatment of long QT syndrome and catecholaminergic polymorphic ventricular tachycardia using video-assisted thoracic surgery.

BACKGROUND: Long QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia (CPVT) are two of the most common, potentially lethal, cardiac channelopathies. Treatment strategies for the primary and secondary prevention of life-threatening polymorphic ventricular tachycardia/fibrillation include pharmacotherapy with beta-blockers, implantable cardioverter defibrillators, and left cardiac sympathetic denervation (LCSD). OBJECTIVES: This study sought to report our institutional experience with LCSD using video-assisted thoracic surgery (VATS). METHODS: From November 2005 through November 2008, 20 patients (8 female, average age at surgery 9.1 +/- 9.7 years, range 2 months to 42 years) underwent LCSD via either a traditional approach (N = 2) or VATS (N = 18). A total of 12 patients had genotype-positive LQTS (7 LQT1, 2 LQT2, 1 LQT3, 2 LQT1/LQT2), 2 had JLNS, 4 had genotype-negative LQTS, and 2 had CPVT1. Electronic medical records were reviewed for patient selection, perioperative complications, and short-term outcomes. RESULTS: LCSD was performed as a secondary prevention strategy in 11 patients (8 LQTS patients, average QTc 549 ms) and as primary prevention in 9 patients (average QTc 480 ms). There were no perioperative complications, including no intraoperative ectopy, no uncontrolled hemorrhage, and no VATS cases requiring conversion to a traditional approach. The average length of available follow-up was 16.6 +/- 9.5 months (range 4 to 40 months). Among the 18 patients who underwent VATS-LCSD, the average time from operation to dismissal was 2.6 days (range 1 day to 15 days), the majority being next-day dismissals. Among those receiving LCSD as secondary prevention, there has been a marked reduction in cardiac events. CONCLUSIONS: We present a series of 20 patients with LQTS and CPVT who underwent LCSD, 18 using VATS. The minimally invasive VATS surgical approach was associated with minimal perioperative complications, including no intraoperative ectopy and excellent immediate and short-term outcomes. Videoscopic denervation surgery, in addition to traditional LCSD, offers a safe and effective treatment option for the personalized medicine required for patients with LQTS/CPVT.