Atrial septal defect closure is associated with improved clinical status in patients ≤ 10 kg with bronchopulmonary dysplasia.

Patients with bronchopulmonary dysplasia (BPD) have shown clinical improvement after secundum atrial septal defect (ASD) closure. We sought to determine if this post-ASD closure improvement is secondary to the expected course in BPD patients or related to the closure itself. A novel BPD-ASD score was created to assess patients' clinical status (higher score = worse disease) and applied to 10 BPD-ASD inpatients weighing ≤ 10 kg who underwent ASD closure. The score and its subcomponents were retrospectively calculated serially ranging from 8 weeks pre- to 8 weeks post-intervention, and pre- and post-intervention score slopes were created. These slopes were compared using mixed regression modeling with an interaction term. There was a significant difference in pre- versus post-intervention slope with the most score drop the first week post-intervention (-2.1 + /- 0.8, p = 0.014). The mean score also dropped through weeks 2 (slope -0.8 + /- 0.8, p = 0.013) and 4 (slope -1.0 + /- 0.5, p = 0.001) post-intervention. There was a significant difference in pre- and post-intervention slopes for diuretics (p = 0.018) and the combined score of respiratory support, FiO2 need, and respiratory symptoms (p = 0.018). This study demonstrated significant improvement in BPD-ASD score, diuretic need, and respiratory status after ASD closure in BPD-ASD patients ≤ 10 kg that was outside of the natural course of BPD. Our study was limited by its small, single-center, retrospective nature. Future studies should be performed in a larger multicenter population to both validate the scoring system and compare to non-intervention infants.

Use of Berlin EXCOR cannulas in both venovenous and venoarterial central extracorporeal membrane oxygenation configurations overcomes the problem of cannula instability while bridging infants and young children to lung transplant.

Objectives: Infants and young children awaiting lung transplantation present challenges that often preclude successful extracorporeal membrane oxygenation support as a bridge to transplantation. Instability of neck cannulas often results in the need for intubation, mechanical ventilation, and muscle relaxation creating a worse transplant candidate. With the use of Berlin Heart EXCOR cannulas (Berlin Heart, Inc) in both venoarterial and venovenous central cannulation configurations, 5 pediatric patients were successfully bridged to lung transplant. Methods: We performed a single-center retrospective case review of central extracorporeal membrane oxygenation cannulation used as a bridge to lung transplantation cases performed at Texas Children's Hospital between 2019 and 2021. Results: Six patients, 2 with pulmonary veno-occlusive disease (15-month-old male and 8-month-old male), 1 with ABCA3 mutation (2-month-old female), 1 with surfactant protein B deficiency (2-month-old female), 1 with pulmonary arterial hypertension in the setting of D-transposition of the great arteries after repair as a neonate (13-year-old male), and 1 with cystic fibrosis and end-stage lung disease, were supported for a median of 56.3 days on extracorporeal membrane oxygenation while awaiting transplantation. All patients were extubated after initiation of extracorporeal membrane oxygenation, participating in rehabilitation until transplant. No complications due to central cannulation and use of the Berlin Heart EXCOR cannulas were observed. One patient with cystic fibrosis developed fungal mediastinitis and osteomyelitis resulting in discontinuation of mechanical support and death. Conclusions: Novel use of Berlin Heart EXCOR cannulas for central cannulation eliminates the problem of cannula instability allowing extubation, rehabilitation, and bridge to lung transplant for infants and young children.

Characterization of Suboptimal Responses to Fetoscopic Endoluminal Tracheal Occlusion in Congenital Diaphragmatic Hernia.

INTRODUCTION: The aim of the study was to characterize the changes in fetal lung volume following fetoscopic endoluminal tracheal occlusion (FETO) that are associated with infant survival and need for extracorporeal membrane oxygenation (ECMO) in congenital diaphragmatic hernia (CDH). METHODS: Fetuses with CDH who underwent FETO at a single institution were included. CDH cases were reclassified by MRI metrics [observed-to-expected total lung volume (O/E TLV) and percent liver herniation]. The percent changes of MRI metrics after FETO were calculated. ROC-derived cutoffs of these changes were derived to predict infant survival to discharge. Regression analyses were done to determine the association between these cutoffs with infant survival and ECMO need, adjusted for site of CDH, gestational age at delivery, fetal sex, and CDH severity. RESULTS: Thirty CDH cases were included. ROC analysis demonstrated that post-FETO increases in O/E TLV had an area under the curve of 0.74 (p = 0.035) for the prediction of survival to hospital discharge; a cutoff of less than 10% was selected. Fetuses with a <10% post-FETO increase in O/E TLV had lower survival to hospital discharge [44.8% vs. 91.7%; p = 0.018] and higher ECMO use [61.1% vs. 16.7%; p = 0.026] compared to those with an O/E TLV increase ≥10%. Similar results were observed when the analyses were restricted to left-sided CDH cases. A post-FETO <10% increase in O/E TLV was independently associated with lower survival at hospital discharge (aOR: 0.073, 95% CI: 0.008-0.689; p = 0.022) and at 12 months of age (aOR: 0.091, 95% CI: 0.01-0.825; p = 0.036) as well as with higher ECMO use (aOR: 7.88, 95% CI: 1.31-47.04; p = 0.024). CONCLUSION: Fetuses with less than 10% increase in O/E TLV following the FETO procedure are at increased risk for requiring ECMO and for death in the postnatal period when adjusted for gestational age at delivery, CDH severity, and other confounders.

Provider Perceptions for Withdrawing Life Sustaining Therapies at a Large Pediatric Hospital.

CONTEXT: More than 74% of pediatric deaths occur in an intensive care unit (ICU), with 40% occurring after withdrawal of life-sustaining therapies (WOLST). No needs assessment has described provider needs or suggestions for improving the WOLST process in pediatrics. OBJECTIVES: This study aims to describe interdisciplinary provider self-reported confidence, needs, and suggestions for improving the WOLST process. METHODS: A convergent parallel mixed-methods design was used. An online survey was distributed to providers involved in WOLSTs in a quaternary children's hospital between January and December 2018. The survey assessed providers' self-reported confidence in their role, in providing guidance to families about the WOLST, experiences with the WOLST process, areas for improvement, and symptom management. Kruskal-Wallis testing was used for quantitative data analysis with P values <0.05 considered significant. Analysis was performed with SPSS v27. Qualitative data were thematically analyzed using Atlas.ti.8 and NVivo. RESULTS: A total of 297 surveys were received (48% survey completion) that consisted of multiple choice, Likert-type, and yes/no questions with options for open-ended responses. Mean provider self-rated confidence was high and varied significantly between disciplines. Qualitative analysis identified four areas for refining communication: 1) between the primary team and family, 2) within the primary team, 3) between the primary team and consulting providers, and 4) logistical challenges. CONCLUSIONS: While participants' self-rated confidence was high, it varied between disciplines. Participants identified opportunities for improved communication and planning before a WOLST. Future work includes development and implementation of a best practice guideline to address gaps and standardize care delivery.

Extracorporeal Membrane Oxygenation Support for Antineutrophil Cytoplasmic Antibody-associated Vasculitides: An ELSO Registry Analysis.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides with pulmonary involvement include granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis, and can present with life-threatening pulmonary hemorrhage in up to 40% of patients. Mortality in those patients who require intubation and mechanical ventilation can reach 77%. Extracorporeal membrane oxygenation (ECMO) can be used to support these patients through definitive diagnosis and treatment, although minimizing the risk of ventilator-induced lung injury. We aimed to determine factors associated with favorable outcomes in patients with (ANCA)-associated vasculitides supported on ECMO. We performed a retrospective observational study using the Extracorporeal Life Support Organization registry of pediatric and adult patients with ANCA-associated vasculitis supported on ECMO from 2010 to 2020. One hundred thirty-five patients were included for analysis. Many patients had renal involvement (39%) in addition to pulmonary involvement (93%). Survival was 73% in AAV patients supported on ECMO. The presence of pulmonary hemorrhage was not associated with worse outcomes in our cohort. Older age, the use of venoarterial ECMO, ECMO-cardiopulmonary resuscitation, or sustaining a cardiac arrest before ECMO was associated with decreased survival. In conclusion, venovenous ECMO should be considered as a supportive bridge to definitive diagnosis and treatment in (ANCA)-associated vasculitides, regardless if pulmonary hemorrhage is present.

Pulmonary-to-Systemic Arterial Shunt to Treat Children With Severe Pulmonary Hypertension.

BACKGROUND: The placement of a pulmonary-to-systemic arterial shunt in children with severe pulmonary hypertension (PH) has been demonstrated, in relatively small studies, to be an effective palliation for their disease. OBJECTIVES: The aim of this study was to expand upon these earlier findings using an international registry for children with PH who have undergone a shunt procedure. METHODS: Retrospective data were obtained from 110 children with PH who underwent a shunt procedure collected from 13 institutions in Europe and the United States. RESULTS: Seventeen children died in-hospital postprocedure (15%). Of the 93 children successfully discharged home, 18 subsequently died or underwent lung transplantation (20%); the mean follow-up was 3.1 years (range: 25 days to 17 years). The overall 1- and 5-year freedom from death or transplant rates were 77% and 58%, respectively, and 92% and 68% for those discharged home, respectively. Children discharged home had significantly improved World Health Organization functional class (P < 0.001), 6-minute walk distances (P = 0.047) and lower brain natriuretic peptide levels (P < 0.001). Postprocedure, 59% of children were weaned completely from their prostacyclin infusion (P < 0.001). Preprocedural risk factors for dying in-hospital postprocedure included intensive care unit admission (hazard ratio [HR]: 3.2; P = 0.02), mechanical ventilation (HR: 8.3; P < 0.001) and extracorporeal membrane oxygenation (HR: 10.7; P < 0.001). CONCLUSIONS: A pulmonary-to-systemic arterial shunt can provide a child with severe PH significant clinical improvement that is both durable and potentially free from continuous prostacyclin infusion. Five-year survival is comparable to children undergoing lung transplantation for PH. Children with severely decompensated disease requiring aggressive intensive care are not good candidates for the shunt procedure.

Outcomes of pediatric patients with oncologic disease or following hematopoietic stem cell transplant supported on extracorporeal membrane oxygenation: The PEDECOR experience.

BACKGROUND: Outcomes for patients with oncologic disease and/or after hematopoietic stem cell transplant (HSCT) requiring intensive care unit admission have improved, but indications for and outcomes after extracorporeal membrane oxygenation (ECMO) support in this population are poorly characterized. PROCEDURE: We analyzed data from consecutive patients < 18 years with oncologic disease and/or after HSCT reported to a pediatric ECMO registry by nine pediatric centers in the United States between 2011 and 2018. RESULTS: We identified 18 ECMO patients with oncologic disease and/or HSCT, and 415 ECMO controls matched with a propensity score algorithm based on age, gender, race, severity of illness at admission, and reason for ECMO. The primary indication for ECMO was respiratory failure in 66.7% in the oncologic disease and/or HSCT group, and in 70.7% in the matched ECMO control group. Eleven of 18 patients survived to hospital discharge (61.1%), similar to the matched control group (60.8%), P = 0.979. Children with oncologic disease and/or HSCT had lower mean platelet counts during ECMO and received higher volumes of platelets compared with the control group, mean 14.6 mL/kg/day (standard deviations [SD], 9.8) versus mean 9.3 mL/kg/day (SD, 10.4), P = 0.001. Of the 11 surviving children with oncologic disease and/or HSCT, five sustained new neurologic disorders (45.5%) versus 45 of 222 (20.3%) in the control group, P = 0.061. Bleeding complications were similar in the two groups. CONCLUSIONS: Outcomes of patients with oncologic disease and/or HSCT supported on ECMO in the current era are not significantly different compared with matched ECMO controls and are improved from previously published reports.

Extracorporeal Membrane Oxygenation Mortality in High-Risk Populations: An Analysis of the Pediatric Health Information System Database.

The number of children receiving extracorporeal membrane oxygenation (ECMO) has increased substantially, and includes a growing population of children with complex underlying conditions who previously may not have been considered ECMO candidates. However, it remains unclear to what extent the underlying disease impacts the risk of death in these patients, particularly related to malignancy, bone marrow transplantation, complex congenital heart disease (CHD), or chromosomal abnormalities. A retrospective study was performed using the Pediatric Health Information System database of all children placed on ECMO more than a 10 year period between 2004 and 2013. Patients with diagnoses of bone marrow transplant, leukemia, lymphoma, neutropenia, immune system abnormalities, genetic abnormalities, neoplastic disorders, and complex CHD were selected as "high risk" and their outcomes were compared with overall outcomes. Extracorporeal membrane oxygenation was used in 9,194 children. Two thousand two hundred (24%) were identified as high risk. Bone marrow transplant (81% mortality; odds ratio [OR] 3.49), leukemia (66% mortality; OR 1.88), and neutropenia (58% mortality; OR 1.62) were associated with higher odds of mortality. Complex CHD (52% mortality) and genetic syndromes (48%) were not associated with higher mortality. These findings are pertinent for clinicians and families when considering ECMO candidacy in these children.

Fenestration during Fontan palliation: now the exception instead of the rule.

OBJECTIVE: Fenestration during Fontan palliation has traditionally been used to decrease surgical morbidity and mortality, particularly in high-risk cases. Potential limitations include oxygen desaturation, risk of paradoxic embolism, and need for late intervention. Our practice has evolved away from routine fenestration with increased extracardiac conduit use. We reviewed our experience with Fontan palliation and retrospectively assessed outcomes with decreased fenestration. METHODS: Between January 2002 and April 2008, 226 patients underwent primary Fontan palliation. Outcomes were assessed by hospital stay, chest drain duration, short- and long-term survivals, and late interventions. RESULTS: Anatomic subtypes were single left ventricle (n = 88, 38.9%), single right ventricle (n = 78, 34.5%), common ventricle (n = 19, 8.4%), and heterotaxy syndrome (n = 41, 18.1%). Lateral tunnel connection was created in 69 patients (30.5%); extracardiac connection was created in 157 (69.5%). Mean age and weight at surgery were 4.3 +/- 3.8 years and 17.2 +/- 9 kg, respectively. In 2002, 14 of 16 patients (87.5%) had fenestrated Fontan circulations, versus 2 of 32 (6.3%) in 2008. Mean hospital stay was 10.8 +/- 8.8 days. Survival to discharge or 30 days was 98.7%. There were 2 (0.9%) late deaths during mean follow-up of 2.0 +/- 1.7 years. Outcomes were equivalent between fenestrated and nonfenestrated procedures across anatomic subtypes. CONCLUSIONS: Highly selective use of Fontan fenestration is achievable while maintaining excellent outcomes without increased surgical morbidity or mortality, irrespective of anatomic subtype. Risks of hypoxia, systemic embolism, and late instrumentation can be avoided in most cases.

Selective cerebral perfusion: real-time evidence of brain oxygen and energy metabolism preservation.

BACKGROUND: Deep hypothermic circulatory arrest (DHCA) is commonly used for complex cardiac operations in children, often with selective cerebral perfusion (SCP). Little data exist concerning the real-time effects of DHCA with or without SCP on cerebral metabolism. Our objective was to better define these effects, focusing on brain oxygenation and energy metabolism. METHODS: Piglets undergoing cardiopulmonary bypass were assigned to either 60 minutes of DHCA at 18 degrees C (n = 9) or DHCA with SCP at 18 degrees C (n = 8), using pH-stat management. SCP was administered at 10 mL/kg/min. A cerebral microdialysis catheter was implanted into the cortex for monitoring of cellular ischemia and energy stores. Cerebral oxygen tension and intracranial pressure also were monitored. After DHCA with or without SCP, animals were recovered for 4 hours off cardiopulmonary bypass. RESULTS: With SCP, brain oxygen tension was preserved in contrast to DHCA alone (p < 0.01). Deep hypothermic circulatory arrest was associated with marked elevations of lactate (p < 0.01), glycerol (p < 0.01), and the lactate to pyruvate ratio (p < 0.001), as well as profound depletion of the energy substrates glucose (p < 0.001) and pyruvate (p < 0.001). These changes persisted well into recovery. With SCP, no significant cerebral microdialysis changes were observed. A strong correlation was demonstrated between cerebral oxygen levels and cerebral microdialysis markers (p < 0.001). CONCLUSIONS: Selective cerebral perfusion preserves cerebral oxygenation and attenuates derangements in cerebral metabolism associated with DHCA. Cerebral microdialysis provides real-time metabolic feedback that correlates with changes in brain tissue oxygenation. This model enables further study and refinement of strategies aiming to limit brain injury in children requiring complex cardiac operations.