Extracorporeal Membrane Oxygenation Support After Heart Transplantation in Children-Outcomes of a Single Center Cohort.

OBJECTIVES: Extracorporeal membrane oxygenation is used for postcardiotomy low cardiac output but is less established following heart transplantation. We characterized outcomes for children supported with extracorporeal membrane oxygenation after heart transplantation. DESIGN: Single-center retrospective study. SETTING: Large pediatric cardiac referral center. PATIENTS: All patients who received heart transplantation and were cannulated to extracorporeal membrane oxygenation between 1995 and 2016. INTERVENTIONS: Primary outcome measure was mortality 12 months postextracorporeal membrane oxygenation. Patient characteristics were analyzed for association with outcome according to early graft failure (extracorporeal membrane oxygenation ≤ 7 d after heart transplantation), or late graft failure. MEASUREMENTS AND MAIN RESULTS: There were 246 heart transplants during the study period and 50 extracorporeal membrane oxygenation runs in 44 patients. Median time from transplant to extracorporeal membrane oxygenation was 1 day (range, 0-11.7 yr), with early graft failure in 28 patients (median 1, range 0-2 d) and 22 extracorporeal membrane oxygenation runs in 20 late graft failure patients (median, 0.8 yr; range, 8 d to 11.7 yr), including four patients with prior extracorporeal membrane oxygenation for early graft failure. Twenty-six patients (59%) survived to hospital discharge, and survival 12 months postextracorporeal membrane oxygenation was 24 patients (55%), lower in those with late graft failure (40% vs 67%; p 0.02). Independent risk factors for 12-month mortality were congenital heart disease, higher pulmonary vascular resistance indexed to body surface area (> 2.2 Woods U/m), and higher creatinine. Higher panel reactive antibody levels were associated with 12-month mortality in the late graft failure group only. CONCLUSIONS: Extracorporeal membrane oxygenation can be effectively used to rescue patients with graft dysfunction after heart transplantation but is associated with high early mortality. Factors associated with mortality within 12 months include presence of congenital heart disease, renal dysfunction, elevated pulmonary vascular resistance indexed to body surface area and in those supported with extracorporeal membrane oxygenation late after heart transplantation, significant human leukocyte antigen sensitization.

Stroke in Acquired and Congenital Heart Disease Patients and Its Relationship to Hospital Mortality and Lasting Neurologic Deficits.

OBJECTIVE: To describe strokes in patients with acquired or congenital heart disease and investigate risk factors for in-hospital mortality and ongoing neurologic deficits. DESIGN: Single-center, retrospective review of cardiac, neurologic, and radiologic patient databases. SETTING: Tertiary care children's hospital. PATIENTS: All patients with acquired or congenital heart disease admitted from January 2010 to October 2014 identified with stroke. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Eighty-four stroke events were identified in 83 patients (median age, 5.9 mo; interquartile range, 0.8-33.4). Thirty-two patients (38%) had single ventricle congenital heart disease. Eight patients (9%) presented with symptoms at home, and the remainder was diagnosed while already admitted to the hospital. Forty patients (48%) presented with clinically evident neurologic deficits (e.g., weakness, seizures). Fifty-eight patients (69%) had arterial ischemic strokes, and 13 (15%) had parenchymal hemorrhages. At diagnosis, 54 patients (64%) were on inotropes. Twenty-nine patients (35%) had greater than or equal to 3 cardiac procedures during their hospitalization before stroke diagnosis. In-hospital mortality occurred in 28 patients (33%). Under multivariate analysis, inotropes, number of cardiac procedures, lack of seizure, and parenchymal hemorrhage were independently associated with in-hospital mortality (p < 0.05). Fifty-four percent of survivors with neurologic follow-up had ongoing neurologic deficits attributable to strokes (median follow-up, 15.3 mo; interquartile range, 7.0-29.9). With multivariate analysis, longer hospital stay (p = 0.02) was independently associated with ongoing deficits. CONCLUSIONS: A majority of patients with acquired or congenital heart disease who suffer stroke present while hospitalized and without focal neurologic findings. In-hospital mortality is associated with inotropes, cardiac procedures, lack of seizure, and parenchymal hemorrhage. The majority of survivors have lasting neurologic deficits associated with longer hospital stay.

Association of Extracorporeal Membrane Oxygenation Support Adequacy and Residual Lesions With Outcomes in Neonates Supported After Cardiac Surgery.

OBJECTIVES: There is a paucity of data regarding the impact of extracorporeal membrane oxygenation support, adequacy of surgical repair, and timing of intervention for residual structural lesions in neonates cannulated to extracorporeal membrane oxygenation after cardiac surgery. Our goal was to determine how these factors were associated with survival. DESIGN: Retrospective study. SETTING: Cardiovascular ICU. SUBJECTS: Neonates (≤ 28 d old) with congenital heart disease cannulated to extracorporeal membrane oxygenation after cardiac surgery during 2006-2013. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Eighty-four neonates were cannulated to venoarterial extracorporeal membrane oxygenation after cardiac surgery. Survival to discharge was 50%. There was no difference in survival based on surgical complexity and those with single or biventricular congenital heart disease. Prematurity (≤ 36 wk gestation; odds ratio, 2.33; p = 0.01), preextracorporeal membrane oxygenation pH less than or equal to 7.17 (odds ratio, 2.01; p = 0.04), need for inotrope support during extracorporeal membrane oxygenation (odds ratio, 3.99; p = 0.03), and extracorporeal membrane oxygenation duration greater than 168 hours (odds ratio, 2.04; p = 0.04) were all associated with increased mortality. Although preextracorporeal membrane oxygenation lactate was not significantly different between survivors and nonsurvivors, unresolved lactic acidosis greater than or equal to 72 hours after cannulation (odds ratio, 2.77; p = 0.002) was associated with increased mortality. Finally, many patients (n = 70; 83%) were noted to have residual lesions after cardiac surgery, and time to diagnosis or correction of residual lesions was significantly shorter in survivors (1 vs 2 d; p = 0.02). CONCLUSIONS: Our data suggest that clearance of lactate is an important therapeutic target for patients cannulated to extracorporeal membrane oxygenation. In addition, timely identification of residual lesions and expedient interventions on those lesions may improve survival.

Perioperative Family Updates Reduce Anxiety and Improve Satisfaction: A Randomized Controlled Trial.

PURPOSE: This study aimed to determine if standardized updates at specific perioperative events affect anxiety and satisfaction of the family members and if the length of surgical procedure affects the satisfaction with updates. METHODS: This study was a randomized control trial. In the control group, surgeons communicated with the family only once near the completion of the procedure. In the intervention group, families received electronic updates at 3 significant perioperative events during the procedure. A postoperative survey rating family member satisfaction and anxiety levels, using a Likert scale of 0-5, was administered. RESULTS: Mean level of overall satisfaction did not differ between groups (intervention: 4.68 ± 0.69 [95% CI: 4.50, 4.87]; control: 4.61 ± 0.78 [95% CI: 4.40, 4.82]; P=0.69). Mean anxiety levels were lower in the intervention group (2.48 ± 1.43 [2.10, 2.86]) than in the control group (3.12 ± 1.32 [2.77, 3.47]; P=0.01). Mean satisfaction with perioperative updates was higher in the intervention (4.48 ± 0.83 [4.26, 4.70]) versus control group (3.16 ± 1.89 [2.67, 3.65]; P=0.0001). For all subjects, there was positive correlation between procedure time and anxiety (Spearman's rho: 0.34; P=0.0002) and negative correlation between procedure time and overall satisfaction (Spearman's rho: -0.23; P=0.01). CONCLUSIONS: Anxiety and satisfaction with perioperative updates were significantly improved by additional perioperative updates. These findings indicate that updating families during significant standardized strategic perioperative events can reduce the anxiety of loved ones and are preferred by most families.

Abnormalities in cerebral hemodynamics and changes with surgical intervention in neonates with congenital heart disease.

OBJECTIVE: To use novel optical techniques to measure perioperative cerebral hemodynamics of diverse congenital heart disease (CHD) groups (two-ventricle, d-transposition of the great arteries [TGA], and single ventricle [SV]) and (1) compare CHD groups with healthy controls preoperatively and (2) compare preoperative and postoperative values within each CHD group. METHODS: Frequency-domain near-infrared spectroscopy and diffuse correlation spectroscopy were used to measure cerebral oxygen saturation, cerebral blood volume, cerebral blood flow index, cerebral oxygen extraction fraction (OEF, calculated using arterial oxygen saturation and cerebral oxygen saturation), and an index of cerebral metabolic rate of oxygen consumption in control and CHD neonates. Preoperative CHD measures were compared with controls. Preoperative and postoperative measures were compared within each CHD group. RESULTS: In total, 31 CHD neonates (7 two-ventricle, 11 TGA, 13 SV) and 13 controls were included. Only neonates with SV CHD displayed significantly lower preoperative cerebral blood flow index (P < .04) than controls. TGA and SV groups displayed greater OEF (P < .05) during the preoperative period compared with controls. Compared with the preoperative state, postoperative neonates with TGA had a greater arterial oxygen saturation with lower OEF. CONCLUSIONS: Differences in cerebral hemodynamics and oxygen metabolism were observed in diverse CHD groups compared with controls. Increased OEF appears to be a compensatory mechanism in neonates with TGA and SV. Studies are needed to understand the relationship of these metrics to outcome and their potential to guide interventions to improve outcome.

Post-arrest therapeutic hypothermia in pediatric patients with congenital heart disease.

BACKGROUND: While therapeutic hypothermia (TH) is an effective neuroprotective therapy for neonatal hypoxic-ischemic encephalopathy, TH has not been demonstrated to improve outcome in other pediatric populations. Patients with acquired or congenital heart disease (CHD) are at high risk of both cardiac arrest and neurodevelopmental impairments, and therapies are needed to improve neurologic outcome. The primary goal of our study was to compare safety/efficacy outcomes in post-arrest CHD patients treated with TH versus controls not treated with TH. METHODS: Patients with CHD treated during the first 18 months after initiation of a post-arrest TH protocol (temperature goal: 33.5 °C) were compared to historical and contemporary post-arrest controls not treated with TH. Post-arrest data, including temperature, safety measures (e.g. arrhythmia, bleeding), neurodiagnostic data (EEG, neuroimaging), and survival were compared. RESULTS: Thirty arrest episodes treated with TH and 51 control arrest episodes were included. The groups did not differ in age, duration of arrest, post-arrest lactate, or use of ECMO-CPR. The TH group's post-arrest temperature was significantly lower than control's (33.6 ±â€¯0.2 °C vs 34.7 ±â€¯0.5 °C, p < 0.001). There was no difference between the groups in safety/efficacy measures, including arrhythmia, infections, chest-tube output, or neuroimaging abnormalities, nor in hospital survival (TH 61.5% vs control 59.1%, p = NS). Significantly more controls had seizures than TH patients (26.1% vs. 4.0%, p = 0.04). Almost all seizures were subclinical and occurred more than 24 h post-arrest. CONCLUSION: Our data show that pediatric CHD patients who suffer cardiac arrest can be treated effectively and safely with TH, which may decrease the incidence of seizures.

Fractures Related to Metabolic Bone Disease in Children with Congenital Heart Disease.

OBJECTIVE: Critically ill children with congenital heart disease (CHD) are at risk for metabolic bone disease (MBD) and bone fractures. Our objective was to characterize a cohort of CHD patients with fractures and describe a Fragile Bone Protocol (FBP) developed to reduce fractures. DESIGN/SETTING: Patients who developed fractures in the Cardiac Intensive Care Unit (CICU) of Boston Children's Hospital from 3/2008 to 6/2014 were identified via quality improvement and radiology databases. The FBP (initiated July 2011) systematically identifies patients at risk for MBD and prescribes special handling precautions. RESULTS: Twenty-three fractures were identified in 15 children. Median age at fracture identification was 6.2 months, with a median duration of hospitalization before fracture diagnosis of 2.7 months. Six patients (40%) had single ventricle CHD. Hyperparathyroidism and low 25-OH vitamin D levels were present in 77% and 40% of those tested, respectively. Compared with patients not diagnosed with fractures, fracture patients had increased exposure to possible risk factors for MBD and had elevated parathyroid and decreased calcitriol levels.Six patients (40%) did not survive to hospital discharge, compared with an overall CICU mortality rate of 2.6% (P < .01). The fracture case rate before implementation of the FBP was 2.6 cases/1000 admissions and was 0.7/1000 after implementation of the FBP (P = .04). CONCLUSIONS: Critically ill CHD patients are at risk for fractures. They represent a complex group who frequently has hyperparathyroidism and decreased calcitriol levels, and each may predispose to fractures. FBPs consisting of identification and careful patient handling should be considered in at-risk patients.

Perioperative cerebral hemodynamics and oxygen metabolism in neonates with single-ventricle physiology.

Congenital heart disease (CHD) patients are at risk for neurodevelopmental delay. The etiology of these delays is unclear, but abnormal prenatal cerebral maturation and postoperative hemodynamic instability likely play a role. A better understanding of these factors is needed to improve neurodevelopmental outcome. In this study, we used bedside frequency-domain near infrared spectroscopy (FDNIRS) and diffuse correlation spectroscopy (DCS) to assess cerebral hemodynamics and oxygen metabolism in neonates with single-ventricle (SV) CHD undergoing surgery and compared them to controls. Our goals were 1) to compare cerebral hemodynamics between unanesthetized SV and healthy neonates, and 2) to determine if FDNIRS-DCS could detect alterations in cerebral hemodynamics beyond cerebral hemoglobin oxygen saturation (SO 2). Eleven SV neonates were recruited and compared to 13 controls. Preoperatively, SV patients showed decreased cerebral blood flow (CBFi ), cerebral oxygen metabolism (CMRO 2i ) and SO 2; and increased oxygen extraction fraction (OEF) compared to controls. Compared to preoperative values, unstable postoperative SV patients had decreased CMRO 2i and CBFi , which returned to baseline when stable. However, SO 2 showed no difference between unstable and stable states. Preoperative SV neonates are flow-limited and show signs of impaired cerebral development compared to controls. FDNIRS-DCS shows potential to improve assessment of cerebral development and postoperative hemodynamics compared to SO 2 alone.