Management advances for congenital diaphragmatic hernia: integrating prenatal and postnatal perspectives.

In congenital diaphragmatic hernia (CDH), abdominal organs are displaced into the chest, compress the lungs, and cause mediastinal shift. This contributes to development of pulmonary hypoplasia and hypertension, which is the primary determinant of morbidity and mortality for affected newborns. The severity is determined using prenatal imaging as early as the first trimester and is related to the laterality of the defect, extent of lung compression, and degree of liver herniation. Comprehensive evaluation of fetal CDH includes imaging-based severity assessment, severity assessment, and evaluation for structural or genetic abnormalities to differentiate isolated from complex cases. Prenatal management involves multispecialty counseling, consideration for fetal therapy with fetoscopic endoluminal tracheal occlusion (FETO) for severe cases, monitoring and intervention for associated polyhydramnios or signs of preterm labor if indicated, administration of antenatal corticosteroids in the appropriate setting, and planned delivery to optimize the fetal condition at birth. Integrated programs that provide a smooth transition from prenatal to postnatal care produce better outcomes. Neonatal care involves gentle ventilation to avoid hyperinflation and must account for transitional physiology to avoid exacerbating cardiac dysfunction and decompensation. Infants who have undergone and responded to FETO have greater pulmonary capacity than expected, but cardiac dysfunction seems unaffected. In about 25-30% of CDH neonates extracorporeal life support is utilized, and this provides a survival benefit for patients with the highest predicted mortality, including those who underwent FETO. Surgical repair after initial medical management for the first 24-48 hours of life is preferred since later repair is associated with delayed oral feeding, increased need for tube feeds, and increased post-repair ventilation requirement and supplemental oxygen at discharge. With overall survival rates >70%, contemporary care involves management of chronic morbidities in the context of a multidisciplinary clinic setting.

Update on Perioperative Pediatric Pulmonary Hypertension Management.

Pediatric pulmonary hypertension is a disease that has many etiologies and can present anytime during childhood. Its newly revised hemodynamic definition follows that of adult pulmonary hypertension: a mean pulmonary artery pressure >20 mmHg. However, the pediatric definition stipulates that the elevated pressure must be present after the age of three months. The definition encompasses many different etiologies, and diagnosis often involves a combination of noninvasive and invasive testing. Treatment often is extrapolated from adult studies or based on expert opinion. Moreover, although general anesthesia may be required for pediatric patients with pulmonary hypertension, it poses certain risks. A thoughtful, multidisciplinary approach is needed to deliver excellent perioperative care.

Transforming a PICU Into an Adult ICU During the Coronavirus Disease 2019 Pandemic: Meeting Multiple Needs.

We describe the process converting half of our 40-bed PICU into a negative-pressure biocontainment ICU dedicated to adult coronavirus disease 2019 patients within a 1,003-bed academic quaternary hospital. We outline the construction, logistics, supplies, provider education, staffing, and operations. We share lessons learned of working with a predominantly pediatric staff blended with adult expertise staff while maintaining elements of family-centered care typical of pediatric critical care medicine. Critically ill coronavirus disease 2019 adult patients may be cared for in a PICU and care may be augmented by implementing elements of holistic, family-centered PICU practice.

A National Survey on Interhospital Transport of Children in Cardiac Arrest.

OBJECTIVES: To describe the U.S. experience with interhospital transport of children in cardiac arrest undergoing cardiopulmonary resuscitation. DESIGN: Self-administered electronic survey. SETTING: Pediatric transport teams listed with the American Academy of Pediatrics Section on Transport Medicine. SUBJECTS: Leaders of U.S. pediatric transport teams. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Sixty of the 88 teams surveyed (68%) responded. Nineteen teams (32%) from 13 states transport children undergoing cardiopulmonary resuscitation between hospitals. The most common reasons for transfer of children in cardiac arrest are higher level-of-care (70%), extracorporeal life support (60%), and advanced trauma resuscitation (35%). Eligibility is typically decided on a case-by-case basis (85%) and sometimes involves a short interhospital distance (35%), or prompt institution of high-quality cardiopulmonary resuscitation (20%). Of the 19 teams that transport with ongoing cardiopulmonary resuscitation, 42% report no special staff safety features, 42% have guidelines or protocols, 37% train staff on resuscitation during transport, 11% brace with another provider, and 5% use mechanical cardiopulmonary resuscitation devices for patients less than 18 years. In the past 5 years, 18 teams report having done such cardiopulmonary resuscitation transports: 22% did greater than five transports, 44% did two to five transports, 6% did one transport, and the remaining 28% did not recall the number of transports. Seventy-eight percent recall having transported by ambulance, 44% by helicopter, and 22% by fixed-wing. Although patient outcomes were varied, eight teams (44%) reported survivors to ICU and/or hospital discharge. CONCLUSIONS: A minority of U.S. teams perform interhospital transport of children in cardiac arrest undergoing cardiopulmonary resuscitation. Eligibility criteria, transport logistics, and patient outcomes are heterogeneous. Importantly, there is a paucity of established safety protocols for the staff performing cardiopulmonary resuscitation in transport.

Spectrum of Current Management of Pediatric Pulmonary Hypertensive Crisis.

Pulmonary hypertension is a growing pediatric problem and children may present with pulmonary hypertensive crisis-a life-threatening emergency requiring acute interventions. The aim of this study was to characterize the broad spectrum of care provided in North American PICUs for children who present with pulmonary hypertensive crisis. DESIGN: Electronic cross-sectional survey. Survey questions covered the following: demographics of the respondents, institution, and patient population; pulmonary hypertension diagnostic modalities; pulmonary hypertension-specific pharmacotherapies; supportive therapies, including sedation, ventilation, and inotropic support; and components of multidisciplinary teams. SETTING: PICUs in the United States and Canada. SUBJECTS: Faculty members from surveyed institutions. INTERVENTIONS: None. MEASUREMENT AND MAIN RESULTS: The response rate was 50% of 99 identified institutions. Of the respondents, 82.2% were pediatric intensivists from large units, and 73.9% had over a decade of experience beyond training. Respondents provided care for a median of 10 patients/yr with acute pulmonary hypertensive crisis. Formal echocardiography protocols existed at 61.1% of institutions with varying components reported. There were no consistent indications for cardiac catheterization during a pulmonary hypertensive crisis admission. All institutions used inhaled nitric oxide, and enteral phosphodiesterase type 5 inhibitor was the most frequently used additional targeted vasodilator therapy. Milrinone and epinephrine were the most frequently used vasoactive infusions. Results showed no preferred approach to mechanical ventilation. Fentanyl and dexmedetomidine were the preferred sedative infusions. A formal pulmonary hypertension consulting team was reported at 51.1% of institutions, and the three most common personnel were pediatric cardiologist, pediatric pulmonologist, and advanced practice nurse. CONCLUSIONS: The management of critically ill children with acute pulmonary hypertensive crisis is diverse. Findings from this survey may inform formal recommendations - particularly with regard to care team composition and pulmonary vasodilator therapies - as North American guidelines are currently lacking. Additional work is needed to determine best practice, standardization of practice, and resulting impact on outcomes.

Perioperative events in children with pulmonary hypertension undergoing non-cardiac procedures.

Prior limited research indicates that children with pulmonary hypertension (PH) have higher rates of adverse perioperative outcomes when undergoing non-cardiac procedures and cardiac catheterizations. We examined a single-center retrospective cohort of children with active or pharmacologically controlled PH who underwent cardiac catheterization or non-cardiac surgery during 2006-2014. Preoperative characteristics and perioperative courses were examined to determine relationships between the severity or etiology of PH, type of procedure, and occurrence of major and minor events. We identified 77 patients who underwent 148 procedures at a median age of six months. The most common PH etiologies were bronchopulmonary dysplasia (46.7%), congenital heart disease (29.9%), and congenital diaphragmatic hernia (14.3%). Cardiac catheterizations (39.2%), and abdominal (29.1%) and central venous access (8.9%) were the most common procedures. Major events included failed planned extubation (5.6%), postoperative cardiac arrest (4.7%), induction or intraoperative cardiac arrest (2%), and postoperative death (1.4%). Major events were more frequent in patients with severe baseline PH ( P = 0.006) and the incidence was associated with procedure type ( P = 0.05). Preoperative inhaled nitric oxide and prostacyclin analog therapies were associated with decreased incidence of minor events (odds ratio [OR] = 0.32, P = 0.046 and OR = 0.24, P = 0.008, respectively), but no change in the incidence of major events. PH etiology was not associated with events ( P = 0.24). Children with PH have increased risk of perioperative complications; cardiac arrest and death occur more frequently in patients with severe PH and those undergoing thoracic procedures. Risk may be modified by using preoperative pulmonary vasodilator therapy and lends itself to further prospective studies.

Pediatric Critical Care Transport as a Conduit to Terminal Extubation at Home: A Case Series.

OBJECTIVES: To present our single-center's experience with three palliative critical care transports home from the PICU for terminal extubation. DESIGN: We performed a retrospective chart review of patients transported between January 1, 2012, and December 31, 2014. SETTING: All cases were identified from our institutional pediatric transport database. PATIENTS: Patients were terminally ill children unable to separate from mechanical ventilation in the PICU, who were transported home for terminal extubation and end-of-life care according to their families' wishes. INTERVENTIONS: Patients underwent palliative care transport home for terminal extubation. MEASUREMENTS AND MAIN RESULTS: The rate of palliative care transports home for terminal extubation during the study period was 2.6 per 100 deaths. The patients were 7 months, 6 years, and 18 years old and had complex chronic conditions. The transfer process was protocolized. The families were approached by the PICU staff during multidisciplinary goals-of-care meetings. Parental expectations were clarified, and home hospice care was arranged pretransfer. All transports were performed by our pediatric critical care transport team, and all terminal extubations were performed by physicians. All patients had unstable medical conditions and urgent needs for transport to comply with the families' wishes for withdrawal of life support and death at home. As such, all three cases presented similar logistic challenges, including establishing do-not-resuscitate status pretransport, having limited time to organize the transport, and coordinating home palliative care services with available community resources. CONCLUSIONS: Although a relatively infrequent practice in pediatric critical care, transport home for terminal extubation represents a feasible alternative for families seeking out-of-hospital end-of-life care for their critically ill technology-dependent children. Our single-center experience supports the need for development of formal programs for end-of-life critical care transports to include patient screening tools, palliative care home discharge algorithms, transport protocols, and resource utilization and cost analyses.