Anticoagulation-Free Pediatric Extracorporeal Membrane Oxygenation: Single-Center Retrospective Study.

OBJECTIVES: To analyze hemorrhage and thrombosis data related to anticoagulation-free pediatric extracorporeal membrane oxygenation (ECMO). DESIGN: Retrospective cohort study. SETTINGS: High-volume ECMO single institution data. PATIENTS: Children (0-18 yr) supported with ECMO (>24 hr) with initial anticoagulation-free period of greater than or equal to 6 hours. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Utilizing consensus American Thoracic Society definitions for hemorrhage and thrombosis on ECMO, we evaluated thrombosis and associated patient and ECMO characteristics during anticoagulation-free period. Thirty-five patients met inclusion criteria from 2018 to 2021 having a median age (interquartile range [IQR]) of 13.5 months (IQR, 3-91 mo), median ECMO duration of 135 hours (IQR, 64-217 hr), and 964 anticoagulation-free hours. Increased RBC transfusion needs were associated with longer anticoagulation-free periods ( p = 0.03). We identified 20 thrombotic events: only four during the anticoagulation-free period and occurring in three of 35 (8%) patients. Compared with those without thrombotic events, anticoagulation-free clotting events were associated with younger age (i.e., 0.3 mo [IQR, 0.2-0.3 mo] vs 22.9 mo [IQR, 3.6-112.9 mo]; p = 0.02), lower weight (2.7 kg [IQR, 2.7-3.25 kg] vs 13.2 kg [5.9-36.4 kg]; p = 0.006), support with lower median ECMO flow rate (0.5 kg [IQR, 0.45-0.55 kg] vs 1.25 kg [IQR, 0.65-2.5 kg]; p = 0.04), and longer anticoagulation-free ECMO duration (44.5 hr [IQR, 40-85 hr] vs 17.6 hr [IQR, 13-24.1]; p = 0.008). CONCLUSIONS: In selected high-risk-for-bleeding patients, our experience is that we can use ECMO in our center for limited periods without systemic anticoagulation, with lower frequency of patient or circuit thrombosis. Larger multicentered studies are required to assess weight, age, ECMO flow, and anticoagulation-free time limitations that are likely to pose risk for thrombotic events.

Paediatric cardiologist adherence to American Heart Association neurodevelopmental recommendations for CHD patients.

A 2012 American Heart Association statement concluded that children with CHD are at an increased risk for neurodevelopmental delays. Routine surveillance and evaluation throughout childhood are recommended. To assess paediatric cardiologist compliance with American Heart Association guidelines and developmental referral practices, a survey was distributed to paediatric cardiologists nationwide (n = 129). The majority of participants (69%) stated they were somewhat familiar or not familiar with the American Heart Association statement and were concerned about patients not being properly referred to specialists for developmental evaluation. Forty paediatric cardiologists (31%) indicated that their institution did not have a neurodevelopmental cardiology programme. Of these, 25% indicated they generally did not refer CHD patients for neurodevelopmental evaluation, 45% performed surveillance and referred if warranted, and 30% generally referred all patients for surveillance. Lastly, 43% of paediatric cardiologists did not feel responsible for developmental surveillance, and 11% did not feel responsible for referrals. To ensure all children with CHD are appropriately screened and referred, paediatricians and cardiologists must work together to address differing impressions of accountability for surveillance and screening of children with CHD.

Decision-Making, Ethics, and End-of-Life Care in Pediatric Extracorporeal Membrane Oxygenation: A Comprehensive Narrative Review.

OBJECTIVES: Pediatric extracorporeal membrane oxygenation is associated with significant morbidity and mortality. We sought to summarize literature on communication and decision-making, end-of-life care, and ethical issues to identify recommended approaches and highlight knowledge gaps. DATA SOURCES: PubMed, Embase, Web of Science, and Cochrane Library. STUDY SELECTION: We reviewed published articles (1972-2020) which examined three pediatric extracorporeal membrane oxygenation domains: 1) decision-making or communication between clinicians and patients/families, 2) ethical issues, or 3) end-of-life care. DATA EXTRACTION: Two reviewers independently assessed eligibility using Preferred Reporting Items for Systematic Reviews and Meta-Analyses methodology. DATA SYNTHESIS: Of 2,581 publications screened, we identified one systematic review and 35 descriptive studies. No practical guides exist for communication and decision-making in pediatric extracorporeal membrane oxygenation. Conversation principles and parent/clinician perspectives are described. Ethical issues related to consent, initiation, discontinuation, resource allocation, and research. No patient-level synthesis of ethical issues or end-of-life care in pediatric extracorporeal membrane oxygenation was identified. CONCLUSIONS: Despite numerous ethical issues reported surrounding pediatric extracorporeal membrane oxygenation, we found limited patient-level research and no practical guides for communicating with families or managing extracorporeal membrane oxygenation discontinuation.

Physiologic diagnosis of congenital heart disease in cyanotic neonates.

PURPOSE OF REVIEW: We aim to improve diagnosis of congenital heart disease (CHD) with cyanosis by physiology for general practitioners to reduce time to appropriate treatment. RECENT FINDINGS: New implementation of the critical congenital heart disease (CCHD) pulse oximetry screen has improved rate of diagnosis of CHD in recent years. However, many infants with cyanotic heart lesions often decompensate before screening in the newborn nursery, or have lesions that are not amenable to pulse oximetry screening and that present later in the emergency room. Recent literature has shown preoperative acidosis because of delayed diagnosis of cyanotic CHD worsens outcomes postoperatively. Wide availability of prostaglandin therapy and catheter procedures help to preoperatively stabilize critical cardiac patients. With a firm grasp of the underlying physiology of neonatal cyanotic CHD, practitioners can appropriately implement these therapies more judiciously. This early recognition will subsequently improve overall outcomes. SUMMARY: Physiologic diagnosis of CHD with cyanosis by general practitioners will allow initiation of appropriate management more quickly and effectively. This may avoid progressive clinical decompensation and acidosis until cardiology consultation and potential intervention are available.

Examining the Utility of Coronary Artery Lack of Tapering and Perivascular Brightness in Incomplete Kawasaki Disease.

BACKGROUND: In 2017, the AHA published revised guidelines for the diagnosis of Kawasaki disease (KD). In the absence of compelling data supporting or refuting the utility of lack of tapering (LT) and perivascular brightness (PB), expert panel consensus removed LT and PB from consideration. We hypothesize that LT and PB are unreliable, subjective findings, non-specific to KD, which can be seen in systemic febrile illnesses without KD and in normal controls. METHODS: We performed a single-center retrospective study from 1/2008 to 12/2016. De-identified coronary artery (CA) echocardiographic clips from patients 0-10 years old were interpreted blindly by six pediatric cardiologists. Subjects were grouped as follows: (1) healthy: afebrile with benign murmur, (2) KD: IVIG treatment, 4-5 clinical criteria at presentation, (3) incomplete KD (iKD): IVIG, 1-3 clinical criteria, (4) Febrile: ≥3 days of fever, no IVIG, KD not suspected. The presence or absence of LT and PB was recorded. Inter-rater and intra-rater reliabilities were analyzed using intra-class correlation coefficient, Fleiss' Kappa and Cohen's Kappa coefficients. RESULTS: We interpreted 117 echocardiograms from healthy (27), KD (30), iKD (32), and febrile (28) subjects. Analysis showed moderate agreement in CA z score measurements. LT and PB were observed by most readers in control groups. LT exhibited fair inter-reader agreement (reliability coefficient 0.36) and PB slight inter-reader agreement (reliability coefficient 0.13). Intra-rater reliability was inconsistent for both parameters. CONCLUSIONS: LT and PB are subjective, poorly reproducible features that can be seen in febrile patients without KD and in healthy children.

Does Prophylactic Ibuprofen After Surgical Atrial Septal Defect Repair Decrease the Rate of Post-Pericardiotomy Syndrome?

Post-pericardiotomy syndrome (PPS) is an inflammatory process involving the pleura, pericardium, or both and occurs after cardiothoracic surgery. Surgical atrial septal defect (ASD) closure is associated with higher incidence of PPS post-operatively as compared to other operations. Reported incidence of PPS varies from 1 to 40%. NSAIDs are often used to treat PPS and in our center, some practitioners have prescribed ibuprofen prophylactically. This study sought to investigate the impact of prophylactic treatment with ibuprofen on the development and severity of PPS following surgical ASD closure, with particular attention to secundum-type ASDs. We retrospectively reviewed clinical and operative data of all surgical ASD repairs in our center from 1/2007 to 7/2017. ASDs were grouped by subtype. PPS was considered positive if the primary cardiologist diagnosed and documented clinical signs of PPS on post-operative outpatient follow-up. Records were reviewed to confirm documented diagnosis of PPS. A total of 245 cases were reviewed with 207 having sufficient data. Median age was 2 years (range 4 months-27 years), female 57%. Overall incidence of PPS was 10%. There was no difference in incidence of PPS in those prescribed ibuprofen as compared to those who were not. This was true for both the entire cohort and the subgroup analysis (P = 1.0). Four patients overall required pericardiocentesis, none of whom received prophylactic ibuprofen. Prophylactic ibuprofen prescription following surgical ASD repair did not reduce the rate of PPS in our cohort.

Single Ventricular Assist Device Care and Outcomes for Failed Stage I Palliation: A Single-Center Decade of Experience.

Single ventricular assist device (SVAD) use before and after stage I palliation (S1P) is increasing with limited data on outcomes. To address this knowledge gap, we conducted a single-center retrospective review to assess pre- and post-SVAD clinical status, complications, and outcomes. We leveraged a granular, longitudinal, local database that captures end-organ support, procedural interventions, hematologic events, laboratory data, and antithrombotic strategy. We identified 25 patients between 2013 and 2023 implanted at median age of 53 days (interquartile range [IQR] = 16-130); 80% had systemic right ventricles and underwent S1P. Median SVAD days were 54 (IQR = 29-86), and 40% were implanted directly from ECMO. Compared to preimplant, there was a significant reduction in inotrope use ( p = 0.013) and improved weight gain ( p = 0.008) post-SVAD. Complications were frequent including bleeding (80%), stroke (40%), acute kidney injury (AKI) (40%), infection (36%), and unanticipated catheterization (56%). Patients with in-hospital mortality had significantly more bleeding complications ( p = 0.02) and were more likely to have had Blalock-Thomas-Taussig shunts pre-SVAD ( p = 0.028). Survival to 1 year postexplant was 40% and included three recovered and explanted patients. At 1 year posttransplant, all survivors have technology dependence or neurologic injury. This study highlights the clinical outcomes and ongoing support required for successful SVAD use in failed single-ventricle physiology before or after S1P.

Poor Reliability of Common Measures of Anticoagulation in Pediatric Extracorporeal Membrane Oxygenation.

Anticoagulation management in pediatric extracorporeal membrane oxygenation (ECMO) is challenging with multiple laboratory measures utilized across institutions without consensus guidelines. These include partial thromboplastin time (PTT), thromboelastography (TEG), and antifactor Xa (aXa). We aimed to evaluate the consistency of TEG R-time, PTT, and aXa correlation to bivalirudin and heparin dosing. We conducted a single-center restrospective review of pediatric ECMO cases from 2018 to 2020 anticoagulated with bivalirudin or heparin. We collected up to 14 serial simultaneous TEG R-time, PTT, and aXa measurements over a 7 day ECMO course with corresponding bivalirudin or heparin dosing. We analyzed the correlation between bivalirudin, heparin, and the three measurements of anticoagulation. A total of 67 ECMO runs, 32 bivalirudin, and 35 heparin, and more than 1,500 laboratory values, of which >80% simultaneous, were analyzed. When assessing correlations at the individual patient level, there was no consistent correlation between dosing and at least one laboratory parameter in the majority of patients. Furthermore, 44% of the bivalirudin cohort and 37% of the heparin cohort exhibited no correlation with any parameters. There were statistically significant correlations only between bivalirudin and heparin dosing and the sum total of the different laboratory tests. These inconsistencies highlight the importance of multimodality testing of anticoagulation in the management of pediatric ECMO anticoagulation and cannot be relied on in isolation from bedside clinical judgment.

Systemic Hypertension in Pediatric Veno-Venous Extracorporeal Membrane Oxygenation.

Systemic hypertension (HTN) is a recognized complication of veno-venous (VV) extracorporeal membrane oxygenation (ECMO) in children. We sought to determine the prevalence and associated features of HTN in a retrospective cohort of children (>1 year old) supported with VV ECMO from January 2015 to July 2019 at our institution. Patient and ECMO-related characteristics were reviewed, including intensive care unit (ICU) length of stay (LOS), ECMO duration, corticosteroids and nephrotoxic medication exposure, acute kidney injury (AKI), overall fluid balance, and transfusion data. We analyzed 23 children (43% female) with a median age of 8.5 years (interquartile range [IQR] = 4-14.5). Median ICU LOS was 26 days (IQR = 15-47) with a median ECMO duration of 288 hours (IQR = 106-378) and a mortality rate of 35%. HTN was diagnosed in 87% subjects at a median of 25 ECMO hours (IQR = 9-54) of whom 55% were hypertensive >50% of their ECMO duration. AKI and fluid overload were documented in >50% of cohort. All but two subjects received at least one nephrotoxic medication, and nearly all received corticosteroids. Our data demonstrate that HTN is present in a preponderance of children supported with VV ECMO and appears within the first 3 days of cannulation. Underlying etiology is likely multifactorial.

A Case of Persistent Left Superior Vena Cava and Left Pulmonary Venous Drainage to the Coronary Sinus.

We report a case of a persistent left superior vena cava draining to the right atrium via the coronary sinus in conjunction with partial anomalous pulmonary venous return of the left pulmonary veins to the coronary sinus. Although a persistent left superior vena cava is typically of little clinical consequence, in this case, it complicated surgical repair of the congenital heart disease. Successful repair of this unusual combination of systemic and pulmonary venous anomalies required a combination of two well-described surgical techniques.

Promoting Pulmonary Arterial Growth via Right Ventricle-to-Pulmonary Artery Connection in Children With Pulmonary Atresia, Ventricular Septal Defect, and Hypoplastic Pulmonary Arteries.

BACKGROUND: Complete repair of pulmonary atresia (PA) ventricular septal defect (VSD) with hypoplastic or absent native pulmonary arteries, often with major aortopulmonary collateral arteries (MAPCAs), involves construction of an adequate sized pulmonary arterial tree. We report our results with a previously described staged strategy using initial right ventricle (RV)-to-reconstructed pulmonary arterial tree (RV-PA) connection to promote pulmonary arterial growth and facilitate later ventricular septation. METHODS: We retrospectively reviewed data for all patients (N = 10) with initial echocardiographic diagnosis of PA-VSD and hypoplastic pulmonary arteries operated in our center from October 2008 to August 2016. Pulmonary arterial vessel size measured on preoperative and postoperative angiography was used to calculate Nakata index. RESULTS: Seven patients had PA-VSD, three had virtual PA-VSD, and seven had MAPCAs. All underwent creation of RV-PA connection at a median age of 7.5 days and weight 3.6 kg. Eight patients had RV-PA conduits, two had a transannular patches, and seven had major pulmonary artery reconstruction simultaneously. There were no deaths or serious morbidity; one conduit required revision prior to complete repair. Complete repair with ventricular septation and RV pressure less than half systemic was achieved in all patients at a median age of 239 days. Nakata index in neonatal period was 54 mm2/m2 (range 15-144 mm2/m2) and at time of septation 184 mm2/m2 (range 56-510 mm2/m2; P = .004). Growth rates of right and left branch pulmonary arteries were similar. The 10 patients underwent 28 catheterizations with 13 interventions in 8 patients prior to full repair. CONCLUSION: Early palliative RV-PA connection promotes pulmonary arterial growth and facilitates eventual full repair with VSD closure with low RV pressure and operative risk.