Background: Investigations of ventricular dominance and outcomes after the Fontan procedure have shown conflicting results. This may be due to the inclusion of multiple modifications of the Fontan or the omission of recently identified complications of the procedure. We examined the association between right ventricular dominance (RVD) and morbidity/mortality in a contemporary cohort following the extracardiac (EC) Fontan. Methods: We studied all pediatric patients at our center who underwent a predominantly fenestrated EC Fontan from 2004 to 2016. Outcomes assessed were freedom from (1) Fontan failure (death, takedown, listing for transplantation) and (2) complication (arrhythmia requiring medication, postoperative pacemaker, or implantable cardioverter defibrillator requirement, stroke, thrombosis in the Fontan circuit, protein losing enteropathy, plastic bronchitis, New York Heart Association class >2). We defined the perioperative period as occurring before hospital discharge or within 30 days of the Fontan. Results: A total of 137 patients (median age: 34 months, 62% male, 60% RVD) underwent the EC Fontan. Median duration of follow-up was 5.8 years (interquartile range: 2.4-9.0). Freedom from any event was 82.5% (RVD = 77%, LVD = 91%, χ 2 (1) = 5.03, P = .025) and RVD was associated with reduced event-free survival (hazard ratio: 2.94, P = .02). No confounders were identified. In the perioperative period, RVD was associated with reduced complication-free survival ( P = .004). After this period, RVD was associated with reduced failure-free survival ( P = .003). Conclusions: In this contemporary, single-center cohort of EC Fontan patients, RVD was associated with inferior outcomes.
Extracorporeal membrane oxygenation (ECMO) circuit volume, patient size, and blood flow may influence coagulation and hemolysis complications. We performed a single-center retrospective analysis of ECMO patients over a 6.5 year period. In 299 ECMO runs, 13% required coagulation-associated circuit changes. Respiratory ECMO was associated with coagulation-associated circuit changes [odds ratio (O/R) 2.8, p < 0.05] and developed severe (plasma-free hemoglobin [pfHb] > 100 mg/dl) hemolysis (O/R 2.3, p < 0.05). Severe hemolysis and component changes were associated with hospital mortality (O/R 2.3 and 2.5, respectively, p < 0.05). The activated partial thromboplastin time (aPTT) to residence time (RT) ratio (aPTT/RT) was used as a surrogate for coagulation risk. We found that aPTT/RT > 2.5 more than doubled time to circuit change (3–8 days, p < 0.05), but aPTT/RT > 3 increased bleeding risks and hospital mortality (O/R 1.8; p < 0.1). Hemolysis was associated with patient weight and circuit to patient volume ratio (CPVR) ( p < 0.05), but not pump type. Hemolysis slightly increased with transfusion ( p = 0.08), and transfusion requirements increased for CPVR >50% ( p < 0.1).Our data suggest that pediatric respiratory ECMO patients are more likely to develop coagulation and hemolysis complications, which are associated with increased mortality. This may result from higher inflammatory processes, which affect coagulation and red cell fragility. Minimizing circuit volume, inflammation, and red cell stress may help to reduce these two complications and their associated mortality.
Abstract Introduction: Approximately 30% of youth present with diabetic ketoacidosis (DKA) at the time of diagnosis of type 1 diabetes mellitus (DM). DKA severity can be mild to severe, impacting hospitalization duration as severity worsens, or even result in death. A delay in care from onset of symptoms results in greater illness severity. Travel has been a proposed risk factor for illness severity in general; one study identified ruptured appendicitis as a more frequent complication in children vacationing from their hometown. Identifying travel as a potential risk factor for severe DKA is necessary. There is no research highlighting the association between vacation and severity of DKA in new onset diabetics. Central Florida is one of the major vacation destinations and is home to a diverse population, so offers a unique study population. The goal of this novel study was to investigate the association between travel and severity of DKA in new onset diabetics. Methods: A retrospective chart review of children admitted to a children’s hospital in Central Florida with both new onset diabetes and DKA from October 2012-March 2020 was conducted. Patients that did not meet criteria for DKA based on venous pH (pH) < 7.3 or bicarbonate (HCO3) < 15 mmol/l were excluded. Patients were divided into two groups by primary residence: locals versus tourists. The severity of DKA was determined as either mild (pH < 7.3 or HCO3 10 to < 15), moderate (pH < 7.2 or HCO3 5–9), or severe (pH < 7.1 or HCO3 < 5), and compared between local versus tourist children with Chi-squared testing. Results: There was no significant difference in DKA severity between locals and tourists. 33% of local children presented in severe DKA compared to 29% of tourist children (p=0.809). The percentage of children overall presenting with moderate or severe DKA was about 70%. Gender and admission status had a statistically significant correlation to DKA severity. More females presented in severe DKA than males (56 % versus 44%, p=0.029), and patients transferred from an outside facility versus directly admitted from the emergency department had a higher percentage of severe DKA (76% versus 24%, p=0.002). Conclusion: This study is the first in the literature to report that travel does not seem to be a contributing risk factor to the severity of DKA. We did find that more than half of all new onset diabetics presented in moderate or severe DKA, indicating that there is currently a frequent delay in diagnosis of children with new onset diabetes. A higher rate of severe DKA was noted in patients transferred from an outside facility versus children admitted directly to the ICU from our emergency department, which may likely be due to challenging access to a specialty pediatric hospital among patients living in remote areas. We propose that future studies investigate this relationship further to guide interventions that can significantly reduce the rate of severe DKA on presentation.
Abstract Background New drugs may further decrease the need for lung transplant (LTx) in pediatric patients with cystic fibrosis (CF), but few studies highlight pediatric non‐CF LTx characteristics and outcomes. Methods The ISHLT registry was used to report morbidity, graft failure, and survival for primary pediatric (<18 years) LTx performed 1990–2017. Recipient/donor characteristics and long‐term outcomes were analyzed for CF and non‐CF recipients. Survival was assessed using Kaplan–Meier curves. Results Of 2232 primary LTx, (43% in males), 918 (41%) were performed for non‐CF indications; most commonly pulmonary hypertension (43%). Non‐CF patients were younger (median age 11 vs. 15, p < .001), and more frequently on inotropes and/or extracorporeal membrane oxygenation (15% vs. 2.4%, p < .001) at transplant, compared to CF recipients. In‐hospital major complications more commonly affected CF LTx recipients (57% vs. 48%, p = .003), but 30‐day mortality was higher in the non‐CF group (9% non‐CF vs. 5% CF, p < .001). One‐, five‐, and ten‐year mortality was 18%, 50%, and 65% for CF recipients, respectively, and 21%, 45%, and 58% for non‐CF recipients ( p = .01 at 10 years). Five‐year survival was significantly better for non‐CF females versus CF females (56% vs. 48%, p = .013), but was similar between groups for males (55% vs. 54%, p = .305). While age was a late outcomes risk factor, pulmonary hypertension and later transplants eras were protective. Conclusions Early mortality is higher and late mortality is lower in non‐CF LTx. Current non‐CF LTx outcomes leave room for improvement. Further study is needed to evaluate the effects of center volume and pediatric‐specific experience on outcomes.
Mechanical cardiovascular assist devices must be properly designed to avoid damage to the blood they contact. The factors that affect the hemocompatibility of a cardiovascular assist device include three major non-physiological components – the material, fluid flow paths, and flow related stresses – as well as the device interaction with the native vasculature. Furthermore, the interaction of the device with the blood is not static. Foreign surfaces activate blood components including platelets, leukocytes and the coagulation cascade. Thrombus formation on the surface of the device can alter the fluid dynamics in a manner that causes erythrocyte damage ranging from significant hemolysis to sub-lethal trauma that can take many days to weeks to develop into a significant clinical problem. This sub-lethal blood trauma is not easily detectable without special equipment, which is typically unavailable in routine clinical practice. Surveillance for blood damage is often suboptimal in the clinical setting, but once clinically relevant hemolysis occurs, crucial decisions – device removal, replacement, or additional medical therapies including surgery or plasmapheresis – that take into account the risk/benefit of intervention must be quickly evaluated. The various pre-clinical designs and testing, surgical considerations, available surveillance techniques, and clinical consequences will be discussed using recent and historical case reports to highlight key points.
