Transcatheter Palliation With Pulmonary Artery Flow Restrictors in Neonates With Congenital Heart Disease: Feasibility, Outcomes, and Comparison With a Historical Hybrid Stage 1 Cohort.

BACKGROUND: Neonates with complex congenital heart disease and pulmonary overcirculation have been historically treated surgically. However, subcohorts may benefit from less invasive procedures. Data on transcatheter palliation are limited. METHODS: We present our experience with pulmonary flow restrictors (PFRs) for palliation of neonates with congenital heart disease, including procedural feasibility, technical details, and outcomes. We then compared our subcohort of high-risk single ventricle neonates palliated with PFRs with a similar historical cohort who underwent a hybrid Stage 1. Cox regression was used to evaluate the association between palliation strategy and 6-month mortality. RESULTS: From 2021 to 2023, 17 patients (median age, 4 days; interquartile range [IQR], 2-8; median weight, 2.5 kilograms [IQR, 2.1-3.3]) underwent a PFR procedure; 15 (88%) had single ventricle physiology; 15 (88%) were high-risk surgical candidates. All procedures were technically successful. At a median follow-up of 6.2 months (IQR, 4.0-10.8), 13 patients (76%) were successfully bridged to surgery (median time since PFR procedure, 2.6 months [IQR, 1.1-4.4]; median weight, 4.9 kilograms [IQR, 3.4-5.8]). Pulmonary arteries grew adequately for age, and devices were easily removed without complications. The all-cause mortality rate before target surgery was 24% (n=4). Compared with the historical hybrid stage 1 cohort (n=23), after adjustment for main confounding (age, weight, intact/severely restrictive atrial septum or left ventricle to coronary fistulae), the PFR procedure was associated with a significantly lower all-cause 6-month mortality risk (adjusted hazard ratio, 0.26 [95% CI, 0.08-0.82]). CONCLUSIONS: Transcatheter palliation with PFR is feasible, safe, and represents an effective strategy for bridging high-risk neonates with congenital heart disease to surgical palliation, complete repair, or transplant while allowing for clinical stabilization and somatic growth.

High-Fidelity Cleft Simulation Maintains Improvements in Performance and Confidence: A Prospective Study.

OBJECTIVE: High-fidelity simulation has a growing role in plastic surgical education. This study tests the hypothesis that cleft lip repair simulation followed by structured debriefing improves performance and self-confidence and that gains are maintained. DESIGN: Prospective, single-blinded interventional study with repeated measures. Trainees performed cleft lip repair on a high-fidelity simulator followed by debriefing, immediately completed a second repair, and returned 3 months later for a third session. Anonymized simulation videos were rated using the modified Objective Structured Assessment of Technical Skills (OSATS) and the Unilateral Cleft Lip Repair competency assessment tool (UCLR). Self-assessed cleft lip knowledge/confidence and procedural self-confidence were surveyed after each simulation. SETTING: Boston Children's Hospital, a tertiary care academic hospital in Boston, MA, USA. PARTICIPANTS: All trainees rotating through the study setting were eligible. Twenty-six participated; 21 returned for follow-up. RESULTS: Significant improvements (p < 0.05) occurred between the first and second simulations for OSATS, UCLR, and procedural self-confidence. Significant improvement occurred between the second and third simulations cleft lip knowledge/confidence. Compared to the first simulation, improvements were maintained at the third simulation for all variables. Training level moderately correlated with score for UCLR for the first simulation (r = 0.55, p < 0.01), deteriorated somewhat with the second (r = 0.35, p = 0.08), and no longer corelated by the third (r = 0.02, p = 0.92). CONCLUSIONS: Objective performance and subjective self-assessed knowledge and confidence improve with high-fidelity simulation plus structured debriefing and improvement is maintained. Differences in procedure-specific performance seen with increasing training level are reduced with simulation, suggesting it may accelerate knowledge and skill acquisition.

Acute coronary artery obstruction following surgical repair of congenital heart disease.

OBJECTIVES: Acute coronary artery obstruction is a rare complication of congenital heart disease surgery but imposes a high burden of morbidity and mortality. Previous case series have described episodes in specific congenital heart lesions or surgical repairs but have not examined the complication in all-comers to congenital heart surgery. We hypothesize that shorter time from a clinically recognized postoperative sentinel event suggestive of coronary ischemia to diagnosis of coronary obstruction is associated with improved clinical outcomes. METHODS: This was a single-center, retrospective review of patients diagnosed with acute coronary artery obstruction by angiography following surgical repair of congenital heart disease between January 2000 and June 2016. RESULTS: In total, 34 patients were identified. The most common procedures associated with coronary artery obstruction were the Norwood procedure, arterial switch operation, and aortic valve repair/replacement. In total, 79% required mechanical circulatory support, 41% died, and 27% were listed for heart transplant. Patients who died or were listed for heart transplant had longer median sentinel-event-to-cardiac-catheterization time (28 [6-168] hours vs 10 [3-56] hours, P = .001), and longer median sentinel-event-to-intervention time (32 [11-350] hours vs 13 [5-59] hours, P = .003). Patients with hypoplastic left heart syndrome were at greater risk of death or transplant listing (odds ratio, 9.23, P = .03). CONCLUSIONS: Time from clinically relevant postoperative sentinel event to diagnosis of coronary artery obstruction by angiography was associated with transplant-listing-free survival. Clinicians should maintain a high index of suspicion for coronary obstruction and consider early catheterization and coronary angiography for patients in whom post-operative coronary compromise is suspected.

Applying Educational Theory to Interdisciplinary Education in Pediatric Cardiac Critical Care.

At the 14th Annual International Meeting for the Pediatric Cardiac Intensive Care Society, the authors presented a simulation workshop for junior multidisciplinary providers focused on cardiopulmonary interactions. We provide an overview of educational theories of particular relevance to curricular design for simulation-based or enhanced activities. We then demonstrate how these theories are applied to curriculum development for individuals to teams and for novice to experts. We review the role of simulation in cardiac intensive care education and the education theories that support its use. Finally, we demonstrate how a conceptual framework, SIMZones, can be applied to design effective simulation-based teaching.

Accurate Prediction of Congenital Heart Surgical Length of Stay Incorporating a Procedure-Based Categorical Variable.

OBJECTIVES: There is increasing demand for the limited resource of Cardiac ICU care. In this setting, there is an expectation to optimize hospital resource use without restricting care delivery. We developed methodology to predict extended cardiac ICU length of stay following surgery for congenital heart disease. DESIGN: Retrospective analysis by multivariable logistic regression of important predictive factors for outcome of postoperative ICU length of stay greater than 7 days. SETTING: Cardiac ICU at Boston Children's Hospital, a large, pediatric cardiac surgical referral center. PATIENTS: All patients undergoing congenital heart surgery at Boston Children's Hospital from January 1, 2010, to December 31, 2015. INTERVENTIONS: No study interventions. MEASUREMENTS AND MAIN RESULTS: The patient population was identified. Clinical variables and Congenital Heart Surgical Stay categories were recorded based on surgical intervention performed. A model was built to predict the outcome postoperative ICU length of stay greater than 7 days at the time of surgical intervention. The development cohort included 4,029 cases categorized into five Congenital Heart Surgical Stay categories with a C statistic of 0.78 for the outcome ICU length of stay greater than 7 days. Explanatory value increased with inclusion of patient preoperative status as determined by age, ventilator dependence, and admission status (C statistic = 0.84). A second model was optimized with inclusion of intraoperative factors available at the time of postoperative ICU admission, including cardiopulmonary bypass time and chest left open (C statistic 0.87). Each model was tested in a validation cohort (n = 1,008) with equivalent C statistics. CONCLUSIONS: Using a model comprised of basic patient characteristics, we developed a robust prediction tool for patients who will remain in the ICU longer than 7 days after cardiac surgery, at the time of postoperative ICU admission. This model may assist in patient counseling, case scheduling, and capacity management. Further examination in external settings is needed to establish generalizability.

Impact of empiric nesiritide or milrinone infusion on early postoperative recovery after Fontan surgery: a randomized, double-blind, placebo-controlled trial.

BACKGROUND: We sought to determine whether empirical nesiritide or milrinone would improve the early postoperative course after Fontan surgery. We hypothesized that compared with milrinone or placebo, patients assigned to receive nesiritide would have improved early postoperative outcomes. METHODS AND RESULTS: In a single-center, randomized, double-blinded, placebo-controlled, multi-arm parallel-group clinical trial, patients undergoing primary Fontan surgery were assigned to receive nesiritide, milrinone, or placebo. A loading dose of study drug was administered on cardiopulmonary bypass followed by a continuous infusion for ≥12 hours and ≤5 days after cardiac intensive care unit admission. The primary outcome was days alive and out of the hospital within 30 days of surgery. Secondary outcomes included measures of cardiovascular function, renal function, resource use, and adverse events. Among 106 enrolled subjects, 35, 36, and 35 were randomized to the nesiritide, milrinone, and placebo groups, respectively, and all were analyzed based on intention to treat. Demographics, patient characteristics, and operative factors were similar among treatment groups. No significant treatment group differences were found for median days alive and out of the hospital within 30 days of surgery (nesiritide, 20 [minimum to maximum, 0-24]; milrinone, 18 [0-23]; placebo, 20 [0-23]; P=0.38). Treatment groups did not significantly differ in cardiac index, arrhythmias, peak lactate, inotropic scores, urine output, duration of mechanical ventilation, intensive care or chest tube drainage, or adverse events. CONCLUSIONS: Compared with placebo, empirical perioperative nesiritide or milrinone infusions are not associated with improved early clinical outcomes after Fontan surgery. CLINICAL TRIAL REGISTRATION URL: http://www.clinicaltrials.gov. Unique identifier: NCT00543309.

An extracorporeal membrane oxygenation cannulation curriculum featuring a novel integrated skills trainer leads to improved performance among pediatric cardiac surgery trainees.

INTRODUCTION: American Heart Association guidelines recommend timely extracorporeal membrane oxygenation (ECMO) cannulation during cardiopulmonary resuscitation for pediatric cardiac arrest refractory to conventional resuscitation. Traditional cannulation training relies on the apprenticeship model. We hypothesized that a simulation-based ECMO cannulation curriculum featuring a novel integrated skills trainer would improve ECMO cannulation during cardiopulmonary resuscitation performance by cardiothoracic surgery trainees. METHODS: An embedded surgical neck cannulation trainer, designed in collaboration with expert surgeons, formed the focus for a simulation-based cannulation curriculum. The course included a didactic presentation and 2 neck cannulations during cardiopulmonary resuscitation with video-assisted expert feedback with a further cannulation at 3 months. Primary outcome was time to cannulation on the trainer. Secondary outcomes were performance on a validated Global Rating Scale (GRS) of surgical technique and a novel Composite ECMO Cannulation Score (CECS). RESULTS: Ten cardiothoracic surgery trainees participated. The trainer was rated as authentic, and sessions was rated as highly useful. Median time to cannulation decreased between cannulation 1 and 2 (15 minutes 24 seconds vs. 12 minutes 15 seconds, P = 0.002). Improvement was sustained at 3 months (13 minutes 36 seconds, P = 0.157 vs. attempt 2). Likewise, GRS increased significantly at attempt 2 versus 1 (77% vs. 62%, P = 0.003) as did CECS (88% vs. 52%, P = 0.002). No deterioration in GRS or CECS was measured at 3 months. CONCLUSIONS: Cardiothoracic surgery trainees found a contextualized ECMO cannulation during cardiopulmonary resuscitation cannulation curriculum to be highly useful and demonstrated sustained improvement in time to cannulation, CECS, and GRS. Further work will focus on determining the clinical impact of this training and defining the optimal interval and number of training sessions.

Outcomes and risk factors for mortality in premature neonates with critical congenital heart disease.

We sought to describe contemporary outcomes and identify risk factors for hospital mortality in premature neonates with critical congenital heart disease who were referred for early intervention. Neonates who were born before 37 weeks' gestation with critical congenital heart disease and admitted to our institution from 2002 to 2008 were included in this retrospective cohort study. Critical congenital heart disease was defined as a defect requiring surgical or transcatheter cardiac intervention or a defect resulting in death within the first 28 days of life. Logistic regression analyses were performed to identify risk factors for mortality before hospital discharge. The study included 180 premature neonates, of whom 37 (21%) died during their initial hospitalization, including 6 (4%) before cardiac intervention and 31 (17%) after cardiac intervention. For the 174 patients undergoing cardiac intervention, independent risk factors for mortality were a 5 min Apgar score ≤ 7, need for preintervention mechanical ventilation, and Risk Adjustment in Congenital Heart Surgery category ≥ 4 or not assignable. Mortality for premature infants with critical congenital heart disease who are referred for early intervention remains high. Patients with lower Apgar scores who receive preintervention mechanical ventilation and undergo more complex procedures are at greatest risk.

Risky business: human factors in critical care.

Remarkable achievements have occurred in pediatric cardiac critical care over the past two decades. The specialty has become well defined and extremely resource intense. A great deal of focus has been centered on optimizing patient outcomes, particularly mortality and early morbidity, and this has been achieved through a focused and multidisciplinary approach to management. Delivering high-quality and safe care is our goal, and during the Risky Business symposium and simulation sessions at the Eighth International Conference of the Pediatric Cardiac Intensive Care Society in Miami, December 2010, human factors, systems analysis, team training, and lessons learned from malpractice claims were presented.

The relationship between inflammatory activation and clinical outcome after infant cardiopulmonary bypass.

BACKGROUND: Cardiopulmonary bypass (CPB) induces a systemic inflammatory response. The magnitude and consequences in infants remain unclear. We assessed the relationship between inflammatory state and clinical outcomes in infants undergoing CPB. METHODS: Plasma concentrations of interleukin (IL)-6, IL-8, IL-10, tumor necrosis factor α, IL-1ß, and C-reactive protein (CRP) were measured pre-CPB and immediately post-CPB, and at 6, 12, and 24 hours post-CPB in infants ≤9 months old. Perioperative clinical data were collected prospectively. RESULTS: Diagnoses of 93 patients included transposition of the great arteries (40), tetralogy of Fallot (28), ventricular septal defect (21), truncus arteriosus (2), and complete atrioventricular canal (2). The median age was 37 days (range = 2 to 264). Pre-CPB IL-6 and CRP were higher in younger infants but were not associated with postoperative inflammatory mediator concentrations or measured clinical outcomes. IL-6 increased post-CPB (median 3.2 pg/mL pre-CPB, 24.2 post-CPB, 95.4 at 6 hours, and 90.3 at 24 hours; all P < 0.001). CRP increased post-CPB, peaking at 24 hours (median 27.5 at 24 hours, 0.3 pre-CPB; P < 0.001). IL-10 and IL-8 increased immediately post-CPB. After adjusting for age and diagnosis, postoperative IL-6 and IL-8 correlated with intensive care unit length of stay and postoperative blood product administration and, for IL-8, 24-hour lactate. CONCLUSIONS: Greater preoperative cytokine and CRP production in younger infants did not correlate with postoperative outcomes; correlation between postoperative inflammatory mediator production and clinical course was statistically significant but clinically modest. We conclude that in infants undergoing low-to-moderate-complexity cardiac surgery in a single high-volume center, the contribution of inflammatory mediator production to postoperative morbidity is relatively limited.

Management of suspected myocarditis during critical-care transport.

Myocarditis and malignant dysrhythmias are unusual presentations in pediatric patients. We report a series of 4 patients with myocarditis and arrhythmia who presented to community emergency departments and were transported to a pediatric tertiary-care center. Three of the patients required extracorporeal life support. We discuss considerations for stabilization and transport: airway and ventilation, hemodynamic support, induction and sedation medication choices, transport decisions, and the traits of an ideal receiving center.

Simulation-based training delivered directly to the pediatric cardiac intensive care unit engenders preparedness, comfort, and decreased anxiety among multidisciplinary resuscitation teams.

OBJECTIVES: Resuscitation of pediatric cardiac patients involves unique and complex physiology, requiring multidisciplinary collaboration and teamwork. To optimize team performance, we created a multidisciplinary Crisis Resource Management training course that addressed both teamwork and technical skill needs for the pediatric cardiac intensive care unit. We sought to determine whether participation improved caregiver comfort and confidence levels regarding future resuscitation events. METHODS: We developed a simulation-based, in situ Crisis Resource Management curriculum using pediatric cardiac intensive care unit scenarios and unit-specific resuscitation equipment, including an extracorporeal membrane oxygenation circuit. Participants replicated the composition of a clinical team. Extensive video-based debriefing followed each scenario, focusing on teamwork principles and technical resuscitation skills. Pre- and postparticipation questionnaires were used to determine the effects on participants' comfort and confidence regarding participation in future resuscitations. RESULTS: A total of 182 providers (127 nurses, 50 physicians, 2 respiratory therapists, 3 nurse practitioners) participated in the course. All participants scored the usefulness of the program and scenarios as 4 of 5 or higher (5 = most useful). There was significant improvement in participants' perceived ability to function as a code team member and confidence in a code (P < .001). Participants reported they were significantly more likely to raise concerns about inappropriate management to the code leader (P < .001). CONCLUSIONS: We developed a Crisis Resource Management training program in a pediatric cardiac intensive care unit to teach technical resuscitation skills and improve team function. Participants found the experience useful and reported improved ability to function in a code. Further work is needed to determine whether participation in the Crisis Resource Management program objectively improves team function during real resuscitations.

Preoperative extracorporeal membrane oxygenation as a bridge to cardiac surgery in children with congenital heart disease.

BACKGROUND: The efficacy of extracorporeal membrane oxygenation (ECMO) in bridging children with unrepaired heart defects to a definitive or palliative surgical procedure has been rarely reported. The goal of this study is to report our institutional experience with ECMO used to provide preoperative stabilization after acute cardiac or respiratory failure in patients with congenital heart disease before cardiac surgery. METHODS: A retrospective review of the ECMO database at Children's Hospital Boston was undertaken. Children with unrepaired congenital heart disease supported with ECMO for acute cardiac or respiratory failure as bridge to a definitive or palliative cardiac surgical procedure were identified. Data collection included patient demographics, indication for ECMO, details regarding ECMO course and complications, and survival to hospital discharge. RESULTS: Twenty-six patients (18 male, 8 female) with congenital heart disease were bridged to surgical palliation or anatomic repair with ECMO. Median age and weight at ECMO cannulation were, respectively, 0.12 months (range, 0 to 193) and 4 kg (range, 1.8 to 67 kg). Sixteen patients (62%) survived to hospital discharge. Variables associated with mortality included inability to decannulate from ECMO after surgery (p = 0.02) and longer total duration of ECMO (p = 0.02). No difference in outcomes was found between patients with single and biventricular anatomy. CONCLUSIONS: Extracorporeal membrane oxygenation, used as a bridge to surgery, represents a useful modality to rescue patients with failing circulation and unrepaired complex heart defects.

Causes of death after congenital heart surgery.

BACKGROUND: There has been little research about the causes of death after congenital heart surgery. METHODS: To determine whether mode of death differs after congenital heart surgery, we evaluated the cause of death for 100 consecutive postoperative deaths at our institution. Mode of death was determined based on retrospective chart review including available autopsy reports. Low output states were categorized into ventricular failure; inadequate postoperative physiology (technically adequate surgery and ventricular function, but persistent low cardiac output); pulmonary hypertension; and atrioventricular valve regurgitation. RESULTS: There was considerable anatomic diversity among patients who died; 46 patients had single-ventricle physiology. The vast majority of patients (n = 79) were in the intensive care unit before surgery. Surgical repairs were revised at initial operation in 22 cases; 7 patients died in the operating room. Seventy-three patients had technically adequate surgical procedures, 23 had residual anatomic defects, and 4 were indeterminate. Thirty patients underwent additional surgical and 9 catheter-based procedures, although some were classified as rescue procedures performed to address minor anatomic or physiologic abnormalities as a last hope to rescue the patient from impending demise. Of 100 deaths, most (n = 52) were due to low cardiac output: 24 inadequate postoperative physiology, 19 ventricular failure, 8 pulmonary hypertension, and 1 valvar regurgitation. Other significant causes of death included sudden cardiac arrest (n = 11), sepsis (n = 11), and procedural complications (n = 8). CONCLUSIONS: More than half of the deaths were due to low cardiac output, but not exclusively ventricular failure.

Indication for initiation of mechanical circulatory support impacts survival of infants with shunted single-ventricle circulation supported with extracorporeal membrane oxygenation.

OBJECTIVES: The use of extracorporeal membrane oxygenation to support patients with shunted single-ventricle physiology has been controversial. Variable survivals are reported in a number of small case series. We sought to evaluate outcomes and identify predictors of survival for patients with shunted single-ventricle physiology who require extracorporeal membrane oxygenation support. METHODS: We retrospectively reviewed the medical records of all patients aged less than 1 year with shunted single-ventricle physiology who were supported with extracorporeal membrane oxygenation at Children's Hospital Boston between 1996 and 2005. Survivors and nonsurvivors were compared with respect to demographics, diagnosis, operative variables, indication for extracorporeal membrane oxygenation, and extracorporeal membrane oxygenation variables. RESULTS: Forty-four infants with shunted single-ventricle physiology were supported with extracorporeal membrane oxygenation. Diagnoses included hypoplastic left heart syndrome (24), other single-ventricle lesions (12), and pulmonary atresia/intact ventricular septum or a variant (8). Overall survival to discharge was 48%. Indication for extracorporeal membrane oxygenation was the strongest predictor of survival to discharge, with 81% of patients cannulated for hypoxemia but only 29% of those cannulated for hypotension surviving to discharge. Specifically, patients cannulated for shunt obstruction had the highest survival (83%). CONCLUSIONS: Overall survival to discharge for patients with shunted single-ventricle physiology is similar to survival reported in the Extracorporeal Life Support Organization registry for all infants supported with cardiac extracorporeal membrane oxygenation. Thus, shunted single-ventricle physiology should not be considered a contraindication to extracorporeal membrane oxygenation. Patients cannulated for hypoxemia, particularly shunt thrombosis, had markedly improved survival compared with those supported primarily for hypotension/cardiovascular collapse. Survival did not differ depending on anatomic diagnosis.

Initial experience with fenoldopam after cardiac surgery in neonates with an insufficient response to conventional diuretics.

OBJECTIVE: Fenoldopam, a selective dopamine-1 receptor agonist, causes systemic vasodilation and increased renal blood flow and tubular sodium excretion. We hypothesized that urine output would improve when fenoldopam was added to conventional diuretic therapy after neonatal cardiopulmonary bypass. DESIGN: Retrospective cohort study using a time-series design. SETTING: Pediatric cardiac intensive care unit. PATIENTS: All neonates who received fenoldopam to promote diuresis after cardiac surgery requiring cardiopulmonary bypass from February 2002 through December 2004. INTERVENTIONS: Fenoldopam infusion for inadequate urine output despite conventional diuretics. MEASUREMENTS: Demographics, diagnostic information, and surgical procedures were recorded. Urine output, fluid balance, inotrope scores, diuretic doses, and other clinical variables that may influence diuresis were recorded for the 24-hr period immediately preceding fenoldopam initiation and during the initial 24 hrs of drug administration. MAIN RESULTS: A total of 25 neonates received fenoldopam to promote diuresis after the modified Norwood (n = 14), arterial switch (n = 4), or other operations (n = 7). Heart rate, conventional diuretic dosing, and fluid intake were similar during the 24-hr periods of conventional therapy and fenoldopam use (p = not significant for all), whereas inotrope scores decreased during the study (p = .021). There was a small but statistically significant increase in blood pressure during the 48-hr study period. Median urine output was 3.6 mL x kg(-1) x hr(-1) (range, 0.2-7.2 mL x kg(-1) x hr(-1)) during the 24-hr period of conventional therapy and 5.8 mL x kg(-1) x hr(-1) (range, 1.6-11.7 mL x kg(-1) x hr(-1)) during the initial 24 hrs of fenoldopam administration (Wilcoxon's signed-rank test, p = .001). CONCLUSIONS: Fenoldopam may improve urine output in neonates who are failing to achieve an adequate negative fluid balance despite conventional diuretic therapy after cardiac surgery and cardiopulmonary bypass. This study is limited by its retrospective design and the possibility that urine output improved spontaneously during the treatment period. A randomized, placebo-controlled clinical trial will be required to confirm these findings.