Native T1 mapping detects both acute clinical rejection and graft dysfunction in pediatric heart transplant patients.

BACKGROUND: Cardiovascular magnetic resonance (CMR) is emerging as an important tool for cardiac allograft assessment. Native T1 mapping may add value in identifying rejection and in assessing graft dysfunction and myocardial fibrosis burden. We hypothesized that CMR native T1 values and features of textural analysis of T1 maps would identify acute rejection, and in a secondary analysis, correlate with markers of graft dysfunction, and with fibrosis percentage from endomyocardial biopsy (EMB). METHODS: Fifty cases with simultaneous EMB, right heart catheterization, and 1.5 T CMR with breath-held T1 mapping via modified Look-Locker inversion recovery (MOLLI) in 8 short-axis slices and subsequent quantification of mean and peak native T1 values, were performed on 24 pediatric subjects. A single mid-ventricular slice was used for image texture analysis using nine gray-level co-occurrence matrix features. Digital quantification of Masson trichrome stained EMB samples established degree of fibrosis. Markers of graft dysfunction, including serum brain natriuretic peptide levels and hemodynamic measurements from echocardiography, catheterization, and CMR were collated. Subjects were divided into three groups based on degree of rejection: acute rejection requiring new therapy, mild rejection requiring increased ongoing therapy, and no rejection with no change in treatment. Statistical analysis included student's t-test and linear regression. RESULTS: Peak and mean T1 values were significantly associated with acute rejection, with a monotonic trend observed with increased grade of rejection. Texture analysis demonstrated greater spatial heterogeneity in T1 values, as demonstrated by energy, entropy, and variance, in cases requiring treatment. Interestingly, 2 subjects who required increased therapy despite low grade EMB results had abnormal peak T1 values. Peak T1 values also correlated with increased BNP, right-sided filling pressures, and capillary wedge pressures. There was no difference in histopathological fibrosis percentage among the 3 groups; histopathological fibrosis did not correlate with T1 values or markers of graft dysfunction. CONCLUSION: In pediatric heart transplant patients, native T1 values identify acute rejection requiring treatment and may identify graft dysfunction. CMR shows promise as an important tool for evaluation of cardiac grafts in children, with T1 imaging outperforming biopsy findings in the assessment of rejection.

Characterization of Inhaled Nitric Oxide Use for Cardiac Indications in Pediatric Patients.

OBJECTIVES: Characterize the use of inhaled nitric oxide (iNO) for pediatric cardiac patients and assess the relationship between patient characteristics before iNO initiation and outcomes following cardiac surgery. DESIGN: Observational cohort study. SETTING: PICU and cardiac ICUs in seven Collaborative Pediatric Critical Care Research Network hospitals. PATIENTS: Consecutive patients, less than 18 years old, mechanically ventilated before or within 24 hours of iNO initiation. iNO was started for a cardiac indication and excluded newborns with congenital diaphragmatic hernia, meconium aspiration syndrome, and persistent pulmonary hypertension, or when iNO started at an outside institution. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Four-hundred seven patients with iNO initiation based on cardiac dysfunction. Cardiac dysfunction patients were administered iNO for a median of 4 days (2-7 d). There was significant morbidity with 51 of 407 (13%) requiring extracorporeal membrane oxygenation and 27 of 407 (7%) requiring renal replacement therapy after iNO initiation, and a 28-day mortality of 46 of 407 (11%). Of the 366 (90%) survivors, 64 of 366 patients (17%) had new morbidity as assessed by Functional Status Scale. Among the postoperative cardiac surgical group (n = 301), 37 of 301 (12%) had a superior cavopulmonary connection and nine of 301 (3%) had a Fontan procedure. Based on echocardiographic variables prior to iNO (n = 160) in the postoperative surgical group, right ventricle dysfunction was associated with 28-day and hospital mortalities (both, p < 0.001) and ventilator-free days (p = 0.003); tricuspid valve regurgitation was only associated with ventilator-free days (p < 0.001), whereas pulmonary hypertension was not associated with mortality or ventilator-free days. CONCLUSIONS: Pediatric patients in whom iNO was initiated for a cardiac indication had a high mortality rate and significant morbidity. Right ventricular dysfunction, but not the presence of pulmonary hypertension on echocardiogram, was associated with ventilator-free days and mortality.

Common carotid artery imaging after vessel sparing decannulation from Extracorporeal Membrane Oxygenation (ECMO) support.

INTRODUCTION: In 2008, Children's National Hospital adopted a simple vessel sparing technique (VST) for neck extra corporeal membrane (ECMO) cannulation/decannulation that is technically simple and reproducible. In this study, we review a cohort of patients decannulated from venous-arterial (VA) ECMO using a VST with the goal of understanding flow dynamics and anatomic changes of the common carotid artery (CCA) after repair with a VST. METHODS: Patients supported with ECMO at a single, tertiary care center between 2008 and 2019 were identified. Patients were included in the analysis if they survived VA ECMO including VST decannulation and neck vessel imaging was completed with either magnetic resonance angiogram (MRA) or computerized tomography angiogram (CTA) post decannulation. The right CCA was assessed for patency and arteriopathy. Complications and feasibility of repeat ECMO cannulation via the neck vessels were also investigated. RESULTS: Three hundred and nineteen patients were identified as having received ECMO support in either the PICU or CICU between 2008 and 2019, of which 76 survived VA ECMO support via neck cannulation. Neck vessel imaging was obtained in 21 patients. Ten had imaging demonstrating a normal right CCA. The CCA was occluded in 3 and stenotic in 5. Vessel wall defects were present in 4. No definitive complication was associated with any of the arterial abnormalities. Repeat right CCA cannulation was achieved in 6/7 patients who needed additional VA ECMO support. CONCLUSIONS: Repair of the right CCA with a simple VST can be achieved safely and consistently during VA ECMO support in pediatric patients. Vascular imaging of the right CCA was normal in almost half and repeat cannulation was achieved in most when pursued. Stenosis and vessel wall defects were common, thus neck vessel imaging post decannulation may be warranted for all patients with a right CCA repair after ECMO support.

Plasma and Urinary FGF-2 and VEGF-A Levels Identify Children at Risk for Severe Bleeding after Pediatric Cardiopulmonary Bypass: A Pilot Study.

Severe bleeding after cardiothoracic surgery with cardiopulmonary bypass (CPB) is associated with increased morbidity and mortality in adults and children. Fibroblast Growth Factor-2 (FGF-2) and Vascular Endothelial Growth Factor-A (VEGF-A) induce hemorrhage in murine models with heparin exposure. We aim to determine if plasma and urine levels of FGF-2 and VEGF-A in the immediate perioperative period can identify children with severe bleeding after CPB. We performed a prospective, observational biomarker study in 64 children undergoing CPB for congenital heart disease repair from June 2015 - January 2017 in a tertiary pediatric referral center. Primary outcome was severe bleeding defined as ≥ 20% estimated blood volume loss within 24-hours. Independent variables included perioperative plasma and urinary FGF-2 and VEGF-A levels. Analyses included comparative (Wilcoxon rank sum, Fisher's exact, and Student's t tests) and discriminative (receiver operator characteristic [ROC] curve) analyses. Forty-eight (75%) children developed severe bleeding. Median plasma and urinary FGF-2 and VEGF-A levels were elevated in children with severe bleeding compared to without bleeding (preoperative: plasma FGF-2 = 16[10-35] vs. 9[2-13] pg/ml; urine FGF-2= 28[15-76] vs. 14.5[1.5-22] pg/mg; postoperative: plasma VEGF-A = 146[34-379] vs. 53 [0-134] pg/ml; urine VEGF-A = 132 [52-257] vs. 45[0.1-144] pg/mg; all p < 0.05). ROC curve analyses of combined plasma and urinary FGF-2 and VEGF-A levels discriminated severe postoperative bleeding (AUC: 0.73-0.77) with mean sensitivity and specificity above 80%. We conclude that the perioperative plasma and urinary levels of FGF-2 and VEGF-A discriminate risk of severe bleeding after pediatric CPB.

Cardiopulmonary Bypass Reduces Early Thrombosis of Systemic-to-Pulmonary Artery Shunts.

BACKGROUND: Shunt thrombosis is a significant cause of morbidity and mortality after systemic-to-pulmonary artery shunt (SPS) placement. Concurrent procedures with placement of SPS may require cardiopulmonary bypass (CPB). Cardiopulmonary bypass is known to cause bleeding and platelet dysfunction in infants, which may protect from early shunt thrombosis. We hypothesized that infants undergoing SPS placement on CPB have a lower incidence of early shunt thrombosis. METHODS: Retrospective cohort study of infants undergoing SPS placement from January 2008 to December 2014 was performed. Patients with and without early shunt thrombosis and on or off CPB were compared using the Mann-Whitney U test or Fisher exact test. Multivariable regression analysis was performed to identify independent predictors of early shunt thrombosis and to assess effect of CPB independent of other factors. RESULTS: Seventy-five infants underwent SPS placement during the study period (on CPB, n = 25; off CPB, n = 50). Operative mortality was 11% (8/75). Nine (12%) patients developed early shunt thrombosis, all of whom had shunt placement off CPB. Independent risk factors for early shunt thrombosis were identified to be SPS placement off CPB ( P = .011), prematurity ( P = .034), and competitive antegrade pulmonary blood flow ( P = .038). CONCLUSION: Prematurity, competitive antegrade pulmonary blood flow, and shunt placement off CPB lead to higher risk of early shunt thrombosis. We speculate that the protection offered by use of CPB may be accounted for by the associated complex coagulopathy and platelet dysfunction associated with CPB.

Association Between Diastolic Blood Pressure During Pediatric In-Hospital Cardiopulmonary Resuscitation and Survival.

BACKGROUND: On the basis of laboratory cardiopulmonary resuscitation (CPR) investigations and limited adult data demonstrating that survival depends on attaining adequate arterial diastolic blood pressure (DBP) during CPR, the American Heart Association recommends using blood pressure to guide pediatric CPR. However, evidence-based blood pressure targets during pediatric CPR remain an important knowledge gap for CPR guidelines. METHODS: All children ≥37 weeks' gestation and <19 years old in Collaborative Pediatric Critical Care Research Network intensive care units with chest compressions for ≥1 minute and invasive arterial blood pressure monitoring before and during CPR between July 1, 2013, and June 31, 2016, were included. Mean DBP during CPR and Utstein-style standardized cardiac arrest data were collected. The hypothesis was that DBP ≥25 mm Hg during CPR in infants and ≥30 mm Hg in children ≥1 year old would be associated with survival. Primary outcome was survival to hospital discharge. Secondary outcome was survival to hospital discharge with favorable neurological outcome, defined as Pediatric Cerebral Performance Categories 1 to 3 or no worse than prearrest baseline. Multivariable Poisson regression models with robust error estimates were used to estimate the relative risk of outcomes. RESULTS: Blinded investigators analyzed blood pressure waveforms during CPR from 164 children, including 60% <1 year old, 60% with congenital heart disease, and 54% after cardiac surgery. The immediate cause of arrest was hypotension in 67%, respiratory decompensation in 44%, and arrhythmia in 19%. Median duration of CPR was 8 minutes (quartiles, 3 and 27 minutes). Ninety percent survived the event, 68% with return of spontaneous circulation and 22% by extracorporeal life support. Forty-seven percent survived to hospital discharge, and 43% survived to discharge with favorable neurological outcome. Maintaining mean DBP ≥25 mm Hg in infants and ≥30 mm Hg in children ≥1 year old occurred in 101 of 164 children (62%) and was associated with survival (adjusted relative risk, 1.7; 95% confidence interval, 1.2-2.6; P=0.007) and survival with favorable neurological outcome (adjusted relative risk, 1.6; 95% confidence interval, 1.1-2.5; P=0.02). CONCLUSIONS: These data demonstrate that mean DBP ≥25 mm Hg during CPR in infants and ≥30 mm Hg in children ≥1 year old was associated with greater likelihood of survival to hospital discharge and survival with favorable neurological outcome.

Risk Factors for Development of Ectopic Atrial Tachycardia in Post-operative Congenital Heart Disease.

Ectopic atrial tachycardia (EAT) is common in surgically repaired congenital heart disease (CHD) and carries the potential for significant hemodynamic compromise. Our objective was to determine the incidence, and risk factors of EAT after CHD surgery. Prospective study of patients that underwent surgery for CHD from February to October 2016 was performed. Demographic, perioperative and electrophysiologic data were collected. Sustained EAT (> 30 s) was documented by telemetry or electrocardiogram and confirmed by a pediatric electrophysiologist. All patients were followed through index hospitalization. During the study period, 17/204 (8%) of patients developed EAT with median time-to-event of 14 days. 15/17 (88%) received anti-arrhythmic therapy for sustained EAT. By univariate analysis, younger age (5 vs. 284 days, P < .001), lower weight (3.2 vs. 7.5 kg, P < .001), single ventricle physiology (P = .05), longer cardiopulmonary bypass time (176 vs. 94 min, P < .001), need for delayed sternal closure (P < .001), and higher STAT category (P < .001) were associated with EAT. Incidence among single ventricle patients was 7/44 (16%), and of those 7/13 (54%) were < 30 days of age. Multivariable Cox regression analysis confirmed age at surgery < 30 days (hazard ratio = 11.7, P = .002) and use of milrinone (hazard ratio = 4.4, P = .007) as independent predictors of EAT. Post-operative EAT is frequent following surgery for CHD especially in neonates. Further study is warranted, specifically in the single ventricle population, given the high potential risk for arrhythmia-induced hemodynamic compromise in this vulnerable population.

Assuring Sustainable Gains in Interdisciplinary Performance Improvement: Creating a Shared Mental Model During Operating Room to Cardiac ICU Handoff.

OBJECTIVE: To understand sustainability and assure long-term gains in multidisciplinary performance improvement using an operating room to cardiac ICU handoff process focused on creation of a shared mental model. DESIGN: Performance improvement cohort project with pre- and postintervention assessments spanning a 4-year period. SETTING: Twenty-six bed pediatric cardiac ICU in a tertiary care children's hospital. PATIENTS: Cardiac surgery patients admitted to cardiac ICU from the operating room following cardiac surgery. INTERVENTIONS: An interdisciplinary workgroup overhauled our handoff process in 2010. The new algorithm emphasized role delineation, standardized communication, and creation of a shared mental model. Our "I-5" mnemonic allowed validation and verification of a shared mental model between multidisciplinary teams. Staff orientation and practice guidelines were revised to incorporate the new process, visual aids were distributed and posted at each patient's bedside, and lapses/audit data were discussed in multidisciplinary forum. MEASUREMENTS AND MAIN RESULTS: Audits assessing equipment and information transfer during handoff were performed 8 weeks following implementation (n = 29), repeated at 1 year (n = 37), 3 years (n = 15), and 4 years (n = 50). Staff surveys prior to implementation, at 8 weeks, and 4 years postintervention assessed satisfaction. Comprehensiveness of information transfer improved in the 4 years following implementation, and staff satisfaction was maintained. At 4 years, discussion of all elements of information transfer was 94%, increased from 85% 8 weeks following implementation and discussion of four or more information elements was 100% increased from 93%. Of the 73% of staff who completed the survey at 4 years, 91% agreed that they received all necessary information, and 87% agreed that the handoff resulted in a shared mental model. CONCLUSIONS: Our methods were effective in creating and sustaining high levels of staff communication and adherence to the new process, thus achieving sustainable gains. Performance improvement initiatives require proactive interdisciplinary maintenance to be successful long term.

Morbidity and mortality prediction in pediatric heart surgery: Physiological profiles and surgical complexity.

OBJECTIVES: Outcome prediction for pediatric heart surgery has focused on mortality but mortality has been significantly reduced over the past 2 decades. Clinical care practices now emphasize reducing morbidity. Physiology-based profiles assessed by the Pediatric Risk of Mortality (PRISM) score are associated with new significant functional morbidity detected at hospital discharge. Our aims were to assess the relationship between new functional morbidity and surgical risk categories (Risk Adjustment for Congenital Heart Surgery [RACHS] and Society for Thoracic Surgery Congenital Heart Surgery Database Mortality Risk [STAT]), measure the performance of 3-level (intact survival, survival with new functional morbidity, or death) and 2-level (survival or death) PRISM prediction algorithms, and assess whether including RACHS or STAT complexity categories improves the PRISM predictive performance. METHODS: Patients (newborn to age 18 years) were randomly selected from 7 sites (December 2011-April 2013). Morbidity (using the Functional Status Scale) and mortality were assessed at hospital discharge. The most recently published PRISM algorithms were tested for goodness of fit, and discrimination with and without the RACHS and STAT complexity categories. RESULTS: The mortality rate in the 1550 patients was 3.2%. Significant new functional morbidity rate occurred in 4.8%, increasing from 1.8% to 13.9%, 1.7%, and 12.9% from the lowest to the highest RACHS and STAT categories, respectively. The 3-level and 2-level PRISM models had satisfactory goodness of fit and substantial discriminative ability. Inclusion of RACHS and STAT complexity categories did not improve model performance. CONCLUSIONS: Both mortality and new, functional morbidity are important outcomes associated with surgical complexity and can be predicted using PRISM algorithms. Adding surgical complexity to the physiologic profiles does not improve predictor performance.

Acquired von Willebrand Syndrome: An Under-Recognized Cause of Major Bleeding in the Cardiac Intensive Care Unit.

BACKGROUND: Acquired von Willebrand syndrome (AvWS) in the setting of congenital heart disease is an under-recognized cause of bleeding in the pediatric cardiac critical care unit. METHODS: Fourteen patients diagnosed with AvWS admitted to the cardiac intensive care unit at the Children's National Health System between December 2009 and September 2015 were identified with subsequent chart review and case analysis. RESULTS: Of the 14 patients included in this study, 4 patients were on ventricular-assist devices, 6 patients were on extracorporeal membrane oxygenation, and 4 were patients with congenital heart disease not receiving any mechanical circulatory support. All patients identified manifested persistent severe bleeding, despite appropriate management of anticoagulation and blood product administration based on the established protocols. Detailed hemostatic testing including quantitative von Willebrand factor (vWF) multimer analysis revealed decreased high-molecular-weight multimers (HMWMs) and absent ultra-HMWM, consistent with AvWS in all patients. Eight patients received treatment with vWF concentrate, one patient with desmopressin, and five recovered without specific treatment. Bleeding ceased in all but one patient. CONCLUSIONS: Acquired von Willebrand syndrome is an uncommon but important cause of bleeding in pediatric patients with cardiac disease. A high index of clinical suspicion with knowledge of the characteristic clinical scenario in addition to low levels of vWF multimers is required to manage and diagnose AvWS. Although the optimal management of AvWS in this patient population is unclear, vWF concentrates are available and appear to be efficacious for controlling life-threatening bleeding.

Outcomes of Patients with Pulmonary Atresia and Major Aortopulmonary Collaterals Without Intervention in Infancy.

Treatment of pulmonary atresia with major aortopulmonary collaterals (PA MAPCAs) remains a challenge. Despite variations in surgical technique, contemporary strategies all include initial intervention in the first year of life. However, a subset of patients presents later in life, and contemporary outcomes of this group have not been reported previously. We performed a retrospective case series of consecutive cases of PA MAPCAs who were seen at our center between January 2001 and February 2016, who had not undergone surgery before the age of 1 year. We describe their presenting characteristics, operative and transcatheter interventions, and outcomes. A total of eight cases were identified from 76 children with PA MAPCAs treated over the study period. Median age at presentation was 5.9 years. Seventy-five percent had confluent pulmonary arteries with a median Nakata index of 113 mm(2)/m(2). Operative intervention was performed in 5/6 cases. Two are awaiting intervention. The combination of operative and transcatheter interventions allowed for ventricular septal defect closure in 60 % of cases, all of whom had subsystemic right ventricular pressures. Operative intervention is possible in some older cases with PA and MAPCAs. Though multiple operations and transcatheter therapies are necessary, some can achieve operative correction of serial circulation with tolerable physiology. Subjects with ventricular hypoplasia and those without confluent pulmonary arteries are more challenging.

Postoperative Abdominal NIRS Values Predict Low Cardiac Output Syndrome in Neonates.

BACKGROUND: The development of low cardiac output syndrome (LCOS) after cardiopulmonary bypass (CPB) occurs in up to 25% of neonates and is associated with increased morbidity. Invasive cardiac output monitors such as pulmonary artery catheters have limited availability and are costly. Near-infrared spectroscopy (NIRS) is a noninvasive tool for monitoring regional oxygenation in neonates in the cardiac intensive care unit (CICU). We hypothesize that anterior abdominal NIRS may aid in the early identification of LCOS after cardiac surgery. METHODS: Prospective observational study from October 2013 to October 2014 of all neonates with congenital heart disease admitted to the CICU following CPB. Abdominal NIRS values were continuously recorded upon CICU admission and for the subsequent 24-hour period. The primary outcome was the development of LCOS. Low cardiac output syndrome was defined as the presence of metabolic lactic acidosis (pH < 7.3 and lactate > 4) or addition of a new vasoactive agent or a vasoactive inotropic score > 15. Autoregressive time series models were constructed for each patient based on the continuously recorded NIRS values, and patients were stratified by development of LCOS. RESULTS: Twenty-seven neonates met inclusion criteria, of whom 11 developed LCOS. Neonates who developed LCOS had lower constant NIRS values (49% vs. 66%, P < .001). Constant NIRS values less than 58% best predicted development of LCOS with a sensitivity of 100% and specificity of 69%. CONCLUSION: Lower constant anterior abdominal NIRS values in the early postoperative period may allow early identification of neonates at risk for LCOS.

Use of Pulmonary Hypertension Medications in Patients with Tetralogy of Fallot with Pulmonary Atresia and Multiple Aortopulmonary Collaterals.

Tetralogy of Fallot (TOF) with pulmonary atresia (PA) and multiple aortopulmonary collaterals (MAPCAs) is a rare and severe form of congenital heart disease with poor prognosis. Aortopulmonary collaterals expose pulmonary arterioles to systemic pressure resulting in pulmonary hypertension (PH). To date, reports regarding the role of PH medications in this population are sparse. The objective of this study was to assess the effect of PH medications in patients with TOF, PA and MAPCAs or similar anatomy, with emphasis on symptoms, echocardiography and invasive hemodynamics. A retrospective review was performed for patients at a single tertiary care pediatric center. Twelve of 66 patients were treated with PH medications (18 %), and eight of these patients had adequate follow-up for further analysis. Median age at last follow-up was 6 years (range 1.4-21 years). Median length of therapy with PH medication was 4 years (range 0.3-17 years). PH medications included sildenafil, bosentan, ambrisentan, inhaled treprostinil and prostacyclin infusion. PH therapy was associated with improvement in symptoms in all patients and improvement in PH by hemodynamic measures in the majority of patients. All patients underwent at least one cardiac intervention by catheterization or surgery while taking PH medication. Two patients died from non-PH-related causes. The remaining six patients are alive and remain on PH medication. This review indicates that PH medications are well tolerated by this patient group and provide symptomatic improvement. Further studies are required to determine whether PH medications provide long-term survival benefit for patients with complex congenital heart disease.

The Pediatric Risk of Mortality Score: Update 2015.

OBJECTIVES: Severity of illness measures have long been used in pediatric critical care. The Pediatric Risk of Mortality is a physiologically based score used to quantify physiologic status, and when combined with other independent variables, it can compute expected mortality risk and expected morbidity risk. Although the physiologic ranges for the Pediatric Risk of Mortality variables have not changed, recent Pediatric Risk of Mortality data collection improvements have been made to adapt to new practice patterns, minimize bias, and reduce potential sources of error. These include changing the outcome to hospital survival/death for the first PICU admission only, shortening the data collection period and altering the Pediatric Risk of Mortality data collection period for patients admitted for "optimizing" care before cardiac surgery or interventional catheterization. This analysis incorporates those changes, assesses the potential for Pediatric Risk of Mortality physiologic variable subcategories to improve score performance, and recalibrates the Pediatric Risk of Mortality score, placing the algorithms (Pediatric Risk of Mortality IV) in the public domain. DESIGN: Prospective cohort study from December 4, 2011, to April 7, 2013. MEASUREMENTS AND MAIN RESULTS: Among 10,078 admissions, the unadjusted mortality rate was 2.7% (site range, 1.3-5.0%). Data were divided into derivation (75%) and validation (25%) sets. The new Pediatric Risk of Mortality prediction algorithm (Pediatric Risk of Mortality IV) includes the same Pediatric Risk of Mortality physiologic variable ranges with the subcategories of neurologic and nonneurologic Pediatric Risk of Mortality scores, age, admission source, cardiopulmonary arrest within 24 hours before admission, cancer, and low-risk systems of primary dysfunction. The area under the receiver operating characteristic curve for the development and validation sets was 0.88 ± 0.013 and 0.90 ± 0.018, respectively. The Hosmer-Lemeshow goodness of fit statistics indicated adequate model fit for both the development (p = 0.39) and validation (p = 0.50) sets. CONCLUSIONS: The new Pediatric Risk of Mortality data collection methods include significant improvements that minimize the potential for bias and errors, and the new Pediatric Risk of Mortality IV algorithm for survival and death has excellent prediction performance.

Immunologic and Infectious Diseases in Pediatric Cardiac Critical Care: Proceedings of the 10th International Pediatric Cardiac Intensive Care Society Conference.

Since the inception of the Pediatric Cardiac Intensive Care Society (PCICS) in 2003, remarkable advances in the care of children with critical cardiac disease have been developed. Specialized surgical approaches, anesthesiology practices, and intensive care management have all contributed to improved outcomes. However, significant morbidity often results from immunologic or infectious disease in the perioperative period or during a medical intensive care unit admission. The immunologic or infectious illness may lead to fever, which requires the attention and resources of the cardiac intensivist. Frequently, cardiopulmonary bypass leads to an inflammatory state that may present hemodynamic challenges or complicate postoperative care. However, inflammation unchecked by a compensatory anti-inflammatory response may also contribute to the development of capillary leak and lead to a complicated intensive care unit course. Any patient admitted to the intensive care unit is at risk for a hospital acquired infection, and no patients are at greater risk than the child treated with mechanical circulatory support. In summary, the prevention, diagnosis, and management of immunologic and infectious diseases in the pediatric cardiac intensive care unit is of paramount importance for the clinician. This review from the tenth PCICS International Conference will summarize the current knowledge in this important aspect of our field.

Risk-stratified postnatal care of newborns with congenital heart disease determined by fetal echocardiography.

BACKGROUND: Advances in fetal echocardiography have improved recognition of congenital heart disease (CHD). Imaging protocols have been developed that predict delivery room (DR) risk and anticipated postnatal level of care (LOC). The aim of this study was to determine the utility of fetal echocardiography in the perinatal management of CHD. METHODS: A retrospective analysis of fetal and postnatal records was conducted. The anticipated LOC was assigned by fetal echocardiography (LOC 1, nursery consult/outpatient follow-up; LOC 2, stable in DR with transfer to cardiac hospital; LOC 3 or 4, DR instability/urgent intervention needed). Prenatal diagnoses and LOC assignment were compared with postnatal diagnoses, treatment, and short-term outcomes. RESULTS: From 2004 to 2012, 8,101 fetuses were evaluated; 7,405 were normal. Of 696 with CHD, 101 terminated, 40 died in utero, and 37 received palliative care. LOC was assigned in the remaining 518. Of 219 LOC 1, 195 (89%) had postnatal follow-up. Only two required transfer for intervention (LOC 1 sensitivity, 0.9; LOC 1 positive predictive value, 0.99). Of 260 assigned LOC 2, 229 (88%) had follow-up. Of these, 200 (87%) were transferred for surgery or intervention. The median time to admission was 195 min. Twenty-two patients (10%) assigned LOC 2 did not require intervention; however, seven (all with D-transposition of the great arteries) required catheter intervention before surgery. Hospital survival was 86% (LOC 2 sensitivity, 0.97; LOC 2 positive predictive value, 0.87). All LOC 3 and 4 patients had follow-up. Thirty-four (87%) needed urgent intervention, with 100% DR and 87% hospital survival (LOC 3 and 4 sensitivity, 0.83; LOC 3 and 4 positive predictive value, 0.87). CONCLUSIONS: Fetal echocardiography enables accurate postnatal risk stratification in CHD, with the exception of D-transposition of the great arteries. LOC 1 assignment facilitated outpatient follow-up; LOC 2 assignment facilitated transfer for intervention. LOC 3 and 4 patients underwent stabilizing intervention or surgery with good short-term outcomes. Given the inability to predict need for intervention in D-transposition of the great arteries, all such patients should be assigned as LOC 3 or 4. Fetal echocardiography with LOC assignment should be used in the planning of postnatal care in CHD.

Single center experience on dosing and adverse events of recombinant factor seven use for bleeding after congenital heart surgery.

There are limited data on the relationship between the administered dose of recombinant factor seven (rFVIIa) and the development of adverse clinical outcomes after congenital heart surgery. This single institution case series reports on dosing, adverse events, and blood product usage after the administration of rFVIIa in the congenital heart surgery patient population. A retrospective review identified 16 consecutive pediatric patients at an academic, free-standing, children's hospital who received rFVIIa to curtail bleeding following congenital heart surgery between April 2004 and June 2012. Patients were assessed for survival to hospital discharge versus in-hospital mortality and the presence or absence of a major neurological event during inpatient hospitalization. The median age at surgery was 6.8 months (range: 3 days-42 years). Seven patients (44%) survived to hospital discharge and nine patients (56%) died. The cause of mortality included major neurological events (44%), uncontrolled bleeding (33%), and sepsis (23%). Eight patients (50%) required extracorporeal membrane oxygenation support following congenital heart surgery. The median cumulative rFVIIa dose administered was 97 mcg/kg, and the median cumulative amount of blood products administered was 452 ml/kg. In conclusion, this case series underscores the need to prospectively evaluate the effect that rFVIIa has on patient survival and the incidence of adverse events, including thrombotic and major neurological events, in congenital heart surgery patients. Ideally, a randomized, multicenter study would provide the sufficient numbers of patients and events to test these relationships.

Twenty-four hour in-hospital congenital cardiac surgical coverage improves perioperative ECMO support outcomes.

BACKGROUND: Extracorporeal membrane oxygenation (ECMO) support is often required in the management of perioperative congenital heart surgery (CHS) patients. However, 24-hour in-hospital congenital cardiac surgical coverage (24-CCSC) is not available at all institutions. The purpose of this study is to evaluate the effect of 24-CCSC on perioperative ECMO outcomes in CHS patients. METHODS: An institutional review board approved, retrospective review of 128 perioperative CHS ECMO patients at a single, quaternary care children's hospital between January 2003 and December 2012 was performed. Primary endpoints evaluated were mortality in children supported with ECMO after undergoing cardiac surgery and ECMO-related morbidity after initiation of 24-CCSC with advanced congenital cardiac surgical fellows. Patients were divided into 2 groups based on whether 24-CCSC was absent (cohort 1: January 2003 to July 2007) or present (cohort 2: August 2007 to December 2012) at the time of ECMO management. RESULTS: The surgical procedures performed were similar in both cohorts based on STAT Mortality Categories (5 Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery Congenital Heart Surgery Mortality Categories). The overall mortality rate in children supported with ECMO after undergoing cardiac surgery was 53%. This mortality was significantly reduced from 68% to 43% (p = 0.007) with 24-CCSC. Multivariate logistic regression analysis revealed that 24-CCSC (p = 0.009) and lower STAT Mortality Category (p = 0.042) were independent predictors of operative survival. Cardiac arrhythmias (36% to 16%; p = 0.012) and pulmonary complications (32% to 8%; p < 0.001) were significantly reduced with 24-CCSC. CONCLUSIONS: The presence of 24-CCSC significantly decreased the rate of mortality in children supported with ECMO after undergoing cardiac surgery, as well as cardiac arrhythmias and pulmonary complications for perioperative CHS patients receiving ECMO support. This study demonstrates that CHS programs would benefit from 24-CCSC in the care of this critically ill patient population.

Pediatric intensive care outcomes: development of new morbidities during pediatric critical care.

OBJECTIVE: To investigate significant new morbidities associated with pediatric critical care. DESIGN: Randomly selected, prospective cohort. SETTING: PICU patients from eight medical and cardiac PICUs. PATIENTS: This was a randomly selected, prospective cohort of PICU patients from eight medical and cardiac PICUs. MEASUREMENTS AND MAIN RESULTS: The main outcomes measures were hospital discharge functional status measured by Functional Status Scale scores and new morbidity defined as an increase in the Functional Status Scale of more than or equal to 3. Of the 5,017 patients, there were 242 new morbidities (4.8%), 99 PICU deaths (2.0%), and 120 hospital deaths (2.4%). Both morbidity and mortality rates differed (p < 0.001) among the sites. The worst functional status profile was on PICU discharge and improved on hospital discharge. On hospital discharge, the good category decreased from a baseline of 72% to 63%, mild abnormality increased from 10% to 15%, moderate abnormality status increased from 13% to 14%, severe status increased from 4% to 5%, and very severe was unchanged at 1%. The highest new morbidity rates were in the neurological diagnoses (7.3%), acquired cardiovascular disease (5.9%), cancer (5.3%), and congenital cardiovascular disease (4.9%). New morbidities occurred in all ages with more in those under 12 months. New morbidities involved all Functional Status Scale domains with the highest proportions involving respiratory, motor, and feeding dysfunction. CONCLUSIONS: The prevalence of new morbidity was 4.8%, twice the mortality rate, and occurred in essentially all types of patients, in relatively equal proportions, and involved all aspects of function. Compared with historical data, it is possible that pediatric critical care has exchanged improved mortality rates for increased morbidity rates.

Intermediate-term results of extracorporeal membrane oxygenation support following congenital heart surgery.

BACKGROUND: Although there are considerable data regarding in-hospital results of congenital heart surgery (CHS) patients requiring postoperative extracorporeal membrane oxygenation (ECMO) support, there is limited information on intermediate-term outcomes. METHODS: A single-institution retrospective review of 25 consecutive postoperative CHS patients who required ECMO and survived to hospital discharge between January 2003 and June 2008. Survival was estimated by the Kaplan-Meier method. RESULTS: At a median follow-up of 3.3 years (interquartile range: 1.2-5.9 years), there was one death which occurred at six months postsurgery. Kaplan-Meier-estimated survival at three years was 95% (95% confidence interval: 90%-100%). Indications for ECMO included extracorporeal cardiopulmonary resuscitation (48%), systemic hypoxia (4%), postoperative low-cardiac output syndrome (28%), and intraoperative failure to wean off cardiopulmonary bypass (20%). Following ECMO support, 65% of patients had unplanned cardiac reinterventions (three requiring operative interventions, six requiring percutaneous interventions, and four requiring both), and 47% of patients required unplanned hospitalizations. In all, 29% of patients developed neurological deficits and 12% of patients developed chronic respiratory failure. No patients developed renal failure. Overall, systemic ventricular (SV) function normalized in 83% of patients, whereas 17% of patients had persistent mild-to-moderate SV dysfunction. CONCLUSIONS: Intermediate-term patient survival of ECMO following CHS is encouraging. However, neurological impairment and unplanned cardiac reinterventions remain significant concerns. Further delineation of risk factors to improve patient outcomes is warranted.