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Context Chylothorax is a well-established acquired complication of thoracic surgery in infants. Current data suggest acquired chylothorax may affect infant growth and nutrition because of a loss of essential nutrients via chylous effusion. Objective The 3 objectives for this study were: (1) identify nutritional markers affected by the development of acquired chylothorax in infants; (2) highlight the variability in methods used to assess nutritional status and growth in this patient population; and (3) highlight nutritional deficits that can serve as treatment targets during postoperative feeding protocols. Data Sources A systematic literature search was conducted between May 31, 2021, and June 21, 2022, using the PubMed, Embase, CINAHL, and Web of Science databases. Search terms included, but were not limited to, “chylothorax,” “infants,” and “nutrition.” Data Extraction Inclusion criteria required studies that measured quantitative markers of nutrition in ≥10 participants aged Data Analysis The variation in methods, time points, interventional groups, and nutritional markers did not facilitate a meta-analysis. Risk of bias was assessed using the Cochrane Risk of Bias in Nonrandomized Studies assessment tool. Conclusion This review highlights the need for reliable quantitative markers of nutrition that will enable providers to assess the nutritional needs of infants with chylothorax. Future studies must focus on measuring markers of nutrition at regular intervals in larger study populations.

Spontaneous thrombosis in the aortic arch is a rare finding in the neonate. Often the thrombosis is initially interpreted as arch obstruction secondary to coarctation or interrupted aortic arch. Thus the obstruction is mechanical with no structural abnormality. We describe 2 newborns with coarctation and extensive thrombosis within the aortic arch. We report their successful surgical management, with a brief review of the literature and pertinent management principles.

BACKGROUND Hyperoxemia (arterial oxygen tension >100 mm Hg) may occur in critically ill patients and have effects on mixed venous saturation (SvO2 ) and on Fick-based estimates of cardiac output (CO). We investigated the effect of hyperoxemia on SvO2 and on assessments of CO using the Fick equation. METHODS Yorkshire swine (n = 14) were anesthetized, intubated, and paralyzed for instrumentation. SvO2 (co-oximetry) and tissue oxygen tension (tPO2 , implantable electrodes) in brain and myocardium were measured during systematic manipulation of arterial oxygen tension (PaO2 ) using graded hyperoxia (fraction of inspired oxygen 0.21 → 0.8). Secondarily, oxygen- and carbon dioxide-based estimates of CO (FickO2 and FickCO2 , respectively) were compared with measurements from a flow probe placed on the aortic root. RESULTS Independent of changes in measured oxygen delivery, cerebral and myocardial tPO2 increased in proportion to PaO2 , as did SvO2 (P

Efficient coupling of soft robotic cardiac assist devices to the external surface of the heart is crucial to augment cardiac function and represents a hurdle to translation of this technology. In this work, we compare various fixation strategies for local and global coupling of a direct cardiac compression sleeve to the heart. For basal fixation, we find that a sutured Velcro band adheres the strongest to the epicardium. Next, we demonstrate that a mesh-based sleeve coupled to the myocardium improves function in an acute porcine heart failure model. Then, we analyze the biological integration of global interface material candidates (medical mesh and silicone) in a healthy and infarcted murine model and show that a mesh interface yields superior mechanical coupling via pull-off force, histology, and microcomputed tomography. These results can inform the design of a therapeutic approach where a mesh-based soft robotic DCC is implanted, allowed to biologically integrate with the epicardium, and actuated for active assistance at a later timepoint. This strategy may result in more efficient coupling of extracardiac sleeves to heart tissue, and lead to increased augmentation of heart function in end-stage heart failure patients.

In the current era, coarctation repair is usually performed as soon as diagnosis is established. We sought to determine if neonatal isolated coarctation repair had a higher rate of intervention postdischarge when compared with older infants.We conducted a retrospective review of neonates (≤30 days of age) and infants (1 to 6 months of age) undergoing isolated coarctation repair between January 1, 2000, and March 31, 2016. Preoperative and postoperative Z scores of arch, aortic valve, and isthmus; rates of reintervention; and length of stay (LOS) were compared between groups. Linear or Cox regression was used to determine predictors of postoperative intensive care unit and hospital LOS during index surgery and postdischarge interventions for the entire cohort.There were 213 (71.5%) neonates and 85 (28.5%) infants. There was no difference in aortic valve morphology between groups. There was a trend but no statistical difference in postdischarge coarctation reintervention (neonates 10.3% versus infants 4.7%; p = 0.1) or left ventricular outflow tract, aortic valve, or mitral valve interventions (9.9% versus 9.4%; p = 0.9). Median postoperative intensive care LOS (3.01 versus 2.28 days; p 0.001) and postoperative hospital LOS (6.23 versus 4.85 days; p0.001) were significantly higher in neonates. Median follow-up was 3 (interquartile range, 0.2 to 9) years for the entire cohort. On multivariable modeling, preoperative hypoplastic arch was associated with arch reinterventions, particularly in infants (10%; p = 0.003). Preoperative left-sided catheter intervention, left-sided obstructive lesions, and age predicted postdischarge intervention on left-sided structures.Coarctation repair can be safely performed in infants and neonates with acceptable postdischarge intervention. Severity of aortic arch hypoplasia is an important discriminator for reinterventions on the arch, particularly in infants.

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.

Objective Superior vena cava pressure after the bidirectional Glenn operation usually is higher than that associated with the preceding shunt-dependent circulation. The aim of the present study was to determine whether the acute elevation in central venous pressure was associated with changes in cerebral oxygenation and perfusion. Design Single-center prospective, observational cohort study. Setting Academic children’s hospital. Participants Infants with single-ventricle lesions and surgically placed systemic-to-pulmonary artery shunts undergoing the bidirectional Glenn operation. Interventions Near-infrared spectroscopy and transcranial Doppler sonography were used to measure regional cerebral oxygen saturation and cerebral blood flow velocity. Measurements and Main Results Mean differences in regional cerebral oxygen saturation and cerebral blood flow velocity before anesthetic induction and shortly before hospital discharge were compared using the F-test in repeated measures analysis of variance. In the 24 infants studied, mean cerebral oxygen saturation increased from 49%±2% to 57%±2% (p = 0.007), mean cerebral blood flow velocity decreased from 57±4 cm/s to 47±4 cm/s (p = 0.026), and peak systolic cerebral blood flow velocity decreased from 111±6 cm/s to 99±6 cm/s (p = 0.046) after the bidirectional Glenn operation. Mean central venous pressure was 8±2 mmHg postinduction of anesthesia and 14±4 mmHg on the first postoperative day and was not associated with a change in cerebral perfusion pressure (p = 0.35). Conclusions The bidirectional Glenn operation in infants with a shunt-dependent circulation is associated with an improvement in cerebral oxygenation, and the lower cerebral blood flow velocity is likely a response of intact cerebral autoregulation.

Robots have been employed in cardiovascular therapy as surgical tools and for automation of hospital systems. Soft robots are a new kind of robot made of soft deformable materials, that are uniquely suited for biomedical applications because they are inherently less likely to injure body tissues and more likely to adapt to biological environments. Awareness of the soft robotic systems under development will help promote clinician involvement in their successful clinical translation. Areas covered: The most advanced soft robotic systems, across the size scale from nano to macro, that have shown the most promise for clinical application in cardiovascular therapy because they offer solutions where a clear therapeutic need still exists. We discuss nano and micro scale technology that could help improve targeted therapy for cardiac regeneration in ischemic heart disease, and soft robots for mechanical circulatory support. Additionally, we suggest where the gaps in the technology currently lie. Expert commentary: Soft robotic technology has now matured from the proof-of-concept phase to successful animal testing. With further refinement in materials and clinician guided application, they will be a useful complement for cardiovascular therapy.

Tricuspid valve (TV) performance is critical for palliation of hypoplastic left heart syndrome. We will review current TV repair techniques, outcomes, and novel approaches.

ObjectiveIsolated pulmonary artery of ductal origin (IPADO) is a rare disease with diverse presentations. Diagnostic and therapeutic approaches vary widely given the low incidence. Reporting additional cases may help inform medical decision making.MethodsWe reviewed diagnostic data—including echocardiography, catheterization, and MRI—and outcomes for all patients with IPADO at our institution.ResultsThirty-seven patients who met inclusion criteria were identified. The mean age at diagnosis was 3 months (range, 1 day-45 years). In 11 patients, the duct supplying the IPADO was patent (ie, patent ductus arteriosis [PDA]); the duct had closed (ligamentum) in the other 26 patients. When performed, catheterization delineated the anatomy in 90% (100% if PDA) versus 54% with magnetic resonance imaging. Patients with a PDA were more likely to undergo intervention (100% vs 58%, P = .02) and had earlier first intervention (1 vs 20 months; P

Recognition of late problems following repair of tetralogy of Fallot (TOF) with a transannular patch has stimulated modifications to preserve pulmonary valve (PV) function. This study assesses the ability of technical performance score (TPS) to determine the need for post-discharge reinterventions (RIs) in valve-sparing TOF repair. We retrospectively reviewed 157 patients following valve-sparing repair of TOF from 2007-2012. We assigned TPS as Class 1 (optimal), Class 2 (adequate), or Class 3 (inadequate) based on discharge echo and clinical criteria. Preoperative, discharge, and follow-up PV Z scores and post-discharge RIs were documented. Reasons for Class 2 or 3 designation were right ventricular outflow tract (RVOT) gradient in 52, pulmonary regurgitation in 13, residual ventricular septal defects in 7, both RVOT gradient and ventricular septal defects in 13, and both RVOT gradient and pulmonary regurgitation in 37 patients. Median follow-up was 19.6 (range: 0.1-86.1) months. Class 3 patients had a significantly longer median intensive care unit and hospital stay compared with Class 1 (3 vs 2 days [P = 0.015] and 7 vs 5 days [P < 0.001], respectively). Post-discharge RIs were significantly lower in Class 1 vs Class 2 and Class 3 (P = 0.003). Class 1 patients had significantly larger PV Z scores compared with Class 2 or Class 3 patients (P < 0.001). TPS is associated with post-discharge RI rate after valve-sparing TOF repair. Preoperative PV Z score is highly correlated with Class I TPS. Patient selection based on preoperative PV Z scores may help determine if valve-sparing approach is appropriate, thus minimizing the need for RIs.

Thrombosis contributes to morbidity and mortality in neonates following cardiac surgery. Alterations in hemostatic factors following cardiac surgery have been described, but there is no data correlating these changes with risk of thrombosis in neonates. The aim of this study is to predict thrombosis in neonates undergoing cardiac surgery by assessment of a panel of hypercoagulability markers. Neonates undergoing cardiac surgery were enrolled preoperatively and prospectively followed. Preoperative hypercoagulability panel testing included thrombin generation assay (TGA), immunoassays for antithrombin III, protein C, protein S, factor VIII, thrombin-activatable fibrinolytic inhibitor (TAFI), plasminogen activator inhibitor-1 (PAI-1), and cardiolipin antibody. Postoperative thrombosis was defined by clinical events (shunt thrombosis, limb ischemia, and stroke) or imaging (intravascular or intracardiac thrombus). Risk factors for thrombosis were assessed. One hundred neonates were enrolled in the study over a two-year period. The incidence of postoperative in-hospital thrombosis was 20%. The only significant clinical risk factor associated with thrombosis was the single ventricle physiology. Hypercoagulability factors associated with increased risk of thrombosis by univariate analysis were elevated PAI-1, TAFI, and TGA, and presence of anticardiolipin antibodies. Multivariable logistic regression analysis demonstrated that elevated PAI-1 (P = 0.015), TAFI (P = 0.028), and TGA (P = 0.007) were independent predictors of thrombosis. Hypercoagulability panel testing may help identify neonates at high risk for thrombosis following cardiac surgery. Future studies are warranted to determine if high risk patients benefit from targeted anticoagulation therapies.

Left ventricular outflow tract obstruction after neonatal repair of interrupted aortic arch with ventricular septal defect may warrant reintervention. We sought to identify clinical and preoperative echocardiographic predictors of reintervention for postoperative left ventricular outflow tract obstruction.Retrospective data were collected on neonates with interrupted aortic arch with ventricular septal defect who underwent single-stage repair from 1995 to 2009. Univariate and multivariate analyses were performed to identify predictors of reintervention.Seventy patients underwent repair, with 16 patients requiring reintervention: 8 underwent surgical reintervention, 5 underwent percutaneous reintervention, and 3 underwent both. The median time to reintervention was 1.2 years (range, 0.2 to 7.7). All surgical reoperations involved subaortic resection, and all percutaneous reinterventions included balloon aortic valve dilation. Several preoperative echocardiographic measurements were significant by univariate analysis; however, smaller preoperative aortic root size was an independent predictor (p = 0.02) by multivariate analysis. Patients with an aortic root size less than 6.5 mm were at greater risk for reintervention compared with patients with a root size greater than 6.5 mm (reintervention rate 44% and 12%, respectively; p0.001). Postoperative left ventricular outflow tract gradient by echocardiogram before discharge was significantly higher in the reintervention group.Preoperative aortic root size predicts reintervention for postoperative left ventricular outflow tract obstruction after single-stage repair of interrupted aortic arch with ventricular septal defect. Patients with elevated left ventricular outflow tract gradients at discharge are at higher risk of having progressive obstruction and require closer follow-up to ensure early identification and management.

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.

We have shown previously that technical performance score (TPS) is strongly associated with early mortality and major postoperative adverse events in a diverse group of patients. We now report evaluation of the validity of TPS in predicting late outcomes in the same group of patients.Patients who underwent surgery between June 1, 2005 and June 30, 2006 were included. The TPS were assigned based on discharge echocardiograms and certain clinical criteria as previously described. Follow-up data for up to 4 years were retrospectively collected. Cox proportional hazards models were used for analysis.A total of 679 patients were included in the analysis. One hundred twenty-three (18%) were neonates, 213 (31%) infants, 291 (435) children, and 52 (8%) adults. Four hundred ninety-one (72%) were in low-risk adjustment in congenital heart surgery (RACHS; 1 to 3), 109 (16%) in high risk (4 to 6), and 27 (4%) were less than 18 years and could not be assigned a RACHS score. Three hundred thirty-one (48%) had an optimal TPS, 283 (42%) adequate, 61 (9%) inadequate, and 4 (1%) could not be scored. There were 34 (5%) late deaths and 149 (22%) late unplanned reinterventions. By univariate analysis, age, RACHS-1 categories, and TPS were all significantly associated with late reintervention (p0.001 for all), while TPS and RACHS-1 were significant factors for mortality (p0.001). On multivariable modeling, inadequate TPS was strongly associated with both late mortality (p = 0.001; HR [hazard ratio] 3.8, CI [confidence interval] 1.7 to 8.4) and late reintervention (p = 0.002, HR 2.1, CI 1.3 to 3.3) after controlling for RACHS-1 and age.The TPS has a strong association with late outcomes across a wide range of age and disease complexity and may serve as a tool to identify patients who are at a higher risk for late reintervention or mortality.

Background Management of unbalanced common atrioventricular canal (UCAVC) defect by a single-ventricle (SV) approach frequently results in poor outcomes, especially in trisomy 21 patients. In this report we describe our results with conversion to biventricular circulation in UCAVC patients with SV palliation. Methods Retrospective review of patients with UCAVC undergoing biventricular conversion from prior SV palliation between 2003 and 2011 was conducted. Mortality and freedom from reinterventions were analyzed using nonparametric methods. Results Sixteen children with UCAVC (8 patients [50%] were left dominant) and prior SV palliation underwent conversion to biventricular circulation between 2003 and 2011. Median follow-up was 18 months (range, 3 to 94 months). Surgical indications included worsening cyanosis, severe atrioventricular valve regurgitation, or failing bidirectional Glenn or Fontan physiology. All patients had either unequal distribution of the common atrioventricular valve of greater than 60% or one hypoplastic ventricle. By magnetic resonance imaging or computed tomography, 8 patients with right dominant atrioventricular canal had a median left ventricular end-diastolic volume of 32 mL/m 2 (range, 22 to 35 mL/m 2 ). Eight patients with a left dominant atrioventricular canal had a median right ventricular end-diastolic volume of 42 mL/m 2 (range, 26 to 64 mL/m 2 ). Eleven patients (69%) had trisomy 21, and 3 patients (19%) had heterotaxy. Stages of palliation included stage I in 2 patients, bidirectional Glenn in 10 patients, hemi-Fontan in 2 patients, and Fontan in 2 patients. There was 1 (6%) operative (right ventricle dominant) and 1 (6%) late death (left ventricle dominant). Eight patients required reinterventions, 3 (19%) surgical and 6 (38%) catheter-based. On follow-up, all had improvement in cyanosis and symptoms. Conclusions Biventricular conversion from failing SV palliation in UCAVC can be accomplished with an acceptable early and late morbidity and mortality, although need for reintervention was not uncommon.

Background Systemic vasodilation using α‐receptor blockade has been shown to decrease the incidence of postoperative cardiac arrest following stage 1 palliation (S1P), primarily when utilizing the modified Blalock‐Taussig shunt. We studied the effects of a protocol in which milrinone was primarily used to lower systemic vascular resistance ( SVR ) following S1P using the right ventricular to pulmonary artery shunt, measuring its effects on oxygen delivery ( DO 2 ) profiles and clinical outcomes. We also correlated Fick‐based assessments of DO 2 with commonly used surrogate measures. Methods and Results Neonates undergoing S1P were treated according to best clinical judgment prior to (n=32) and following (n=24) implementation of a protocol that guided operative, anesthetic, and postoperative management, particularly as it related to SVR . A majority of the subjects (n=51) received a modified right ventricular to pulmonary artery shunt. In a subset of these patients (n=21), oxygen consumption ( VO 2 ) was measured and used to calculate SVR , DO 2 , and oxygen debt. Neonates treated with the protocol had significantly lower SVR ( P =0.02), serum lactate ( P O 2 difference ( P CPR requiring extracorporeal membrane oxygenation (E‐ CPR , P =0.02) within the first 72 postoperative hours. DO 2 was closely associated with SVR (r 2 =0.78) but correlated poorly with arterial (SaO 2 ) and venous (SvO 2 ) oxyhemoglobin concentrations, the Sa‐v O 2 difference, and blood pressure. Conclusions A vasodilator protocol utilizing milrinone following S1P effectively decreased SVR , improved serum lactate, and decreased postoperative cardiac arrest. DO 2 correlated more closely with SVR than with Sa‐v O 2 difference, highlighting the importance of measuring VO 2 in this population. Clinical Trial Registration URL : http://www.clinicaltrials.gov . Unique identifier: NCT 02184169.

Objectives Although pseudoaneurysm is an uncommon complication after right ventricle–to–pulmonary artery conduit placement, it has the potential to cause significant morbidity and mortality. Methods We performed a review of patients with pseudoaneurysms diagnosed at our institution in a 20-year period (from 1995 through 2015) and compared their clinical characteristics with a group of age- and sex-matched control patients. Results We found that younger age, smaller size, the diagnosis of tetralogy of Fallot, the use of a pulmonary homograft conduit, the presence of an unrestrictive ventricular septal defect after conduit placement, and having at least systemic right ventricular pressure were all more common in patients who had pseudoaneurysms develop. Conclusions This study is unique in identifying both patient and surgical factors that may predispose to pseudoaneurysm development and can help inform optimal strategies to monitor and evaluate this patient population.

Background: Anomalous aortic origin of the coronary arteries (AAOCA) has an estimated prevalence of 0.1%-0.3%. Data on risks and benefits of surgery vs. observation to guide management decisions are limited. Methods: In a single center, we included patients (pts) with AAOCA with right (R) and/or left (L) intramural (IM), interarterial IA) or intraconal (IC) course from 1996-2014. We excluded pts with benign CA anomalies or major structural heart disease. Results: Among 154 pts, median age at diagnosis (dx) was 8.5 (range 0.1-50) yrs, median f/u was 1.92 (0.1-12.8) yrs, and 65% were male. The AAOCA course was IA in 116 (75%), IM in 52 (34%) and IC in 3 (2%). AAORCA was present in 126 (82%), of whom (51) 40% had repair. AAOLCA was present in 28 (18%), of whom 20 (71%) had repair; of the remaining 8, 2 had intraconal course; 2 were second opinions, 1 had Friederich’s ataxia, and 3 are awaiting surgery. In the surgical group, all had IA/IM CAs, and CA unroofing was performed in 90%, of whom 50% also had resuspension of the intercoronary commissure (table). Major perioperative complications occurred in 4 pts (6%: 1 ECMO, 1 mediastinitis, 2 early reoperation), 1 pt had late AoV repair. In the surgical group, no pts died; in the observed group, 2 pts with AAORCA (2.3%) died of severe non-cardiac comorbidities. Pts with surgery vs. observation were more likely to have AAOLCA (28% vs.10%, p=0.003), and, at time of dx, symptoms of chest pain/syncope (63% vs.13%, p10 yrs (median 11 vs. 6 yrs, p

There is much interest in form-fitting, low-modulus, implantable devices or soft robots that can mimic or assist in complex biological functions such as the contraction of heart muscle. We present a soft robotic sleeve that is implanted around the heart and actively compresses and twists to act as a cardiac ventricular assist device. The sleeve does not contact blood, obviating the need for anticoagulation therapy or blood thinners, and reduces complications with current ventricular assist devices, such as clotting and infection. Our approach used a biologically inspired design to orient individual contracting elements or actuators in a layered helical and circumferential fashion, mimicking the orientation of the outer two muscle layers of the mammalian heart. The resulting implantable soft robot mimicked the form and function of the native heart, with a stiffness value of the same order of magnitude as that of the heart tissue. We demonstrated feasibility of this soft sleeve device for supporting heart function in a porcine model of acute heart failure. The soft robotic sleeve can be customized to patient-specific needs and may have the potential to act as a bridge to transplant for patients with heart failure.

Parachute left atrioventricular (AV) valve can complicate repair of common atrioventricular canal (CAVC), and single-ventricle palliation is sometimes preferred. The goal of this study is to review our single institutional experience in biventricular repair in this patient group.The demographic, procedural and outcome data were obtained for all children who underwent biventricular repair for complete CAVC with parachute [single left ventricular (LV) papillary muscle] or forme fruste parachute left AV valve (closely spaced LV papillary muscles) from 2001 to 2012. Primary outcomes were survival, freedom from left AV valve stenosis (defined as an inflow gradient ≥7 mmHg and post-capillary pulmonary hypertension) and freedom from left AV valve replacement.A total of 24 patients were included (21 parachutes, 3 forme frustes). There was 1 early death (4.2%). At discharge, no patient had more-than-mild regurgitation and 1 had stenosis. During a median follow-up of 3.7 years (IQR 4 months to 5 years), there were 2 late deaths (8.3%), 6 patients (25%) presented significant left AV valve stenosis and 2 patients (8.3%) required valve replacement. Freedom from stenosis was 95 ± 4.9% at 1 year, 83.1 ± 8.9% at 3 years, 64.7 ± 13.5% at 5 years and 51.7 ± 15.8% at 10 years. Complete cleft closure was not associated with a significantly different freedom from left AV valve reoperation (log-rank test, P = 0.89) or significant stenosis (P = 0.47).Biventricular repair in parachute left AV valve and CAVC is feasible with acceptable mortality and freedom from stenosis. The burden of reoperation remains significant in this patient group.

ObjectiveRisk factors for mortality after neonatal cardiac surgery have been seldom studied. We sought to identify contemporary risk factors for mortality and the impact of surgical technical performance on surgical outcomes after neonatal cardiac surgery.MethodsWe conducted a matched case-control study comparing 56 neonates who died after cardiac surgery (2002-2008) with 56 survivors matched by surgical procedure and year of surgery. Surgical efficacy for repair or palliation was graded using a reliable simple surgical technical score. Patient and surgical characteristics were compared for the survivors and nonsurvivors using paired analyses.ResultsThere was no significant difference between patients who died and their matched controls in terms of age, Aristotle score, Risk Adjustment in Congenital Heart Surgery-1 category, and single versus biventricular repair. When compared with survivors, patients who died were more likely to be premature (41% vs 5%, P

ObjectivesThe goal of this study was to review results of a novel management strategy intended to rehabilitate the left heart (LH) in patients with LH hypoplasia who have undergone single-ventricle palliation (SVP).BackgroundManagement of patients with hypoplastic LH syndrome and borderline left ventricle (LV) involves 2 options: SVP or biventricular repair. We hypothesized that staged LV recruitment and biventricular conversion may be achieved after SVP by using a strategy consisting of relief of inflow and outflow tract obstructions, resection of endocardial fibroelastosis, and promotion of flow through the LV.MethodsPatients with hypoplastic LH and borderline LV who underwent traditional SVP (n = 34) or staged LV recruitment (n = 34) between 1995 and 2010 were retrospectively analyzed and compared with a control SVP group.ResultsMean initial z-scores for LH structures before stage 1 SVP were not significantly different between groups. Mortality occurred in 4 of 34 patients after LV recruitment and in 7 of 34 after traditional SVP. LH dimension z-scores increased significantly over time after LV recruitment, whereas they declined after traditional SVP, with significant interaction between stage of palliation and treatment group. Restriction of the atrial septum (conducted in 19 of 34 patients) was the only predictor of increase in left ventricular end-diastolic volume (p < 0.001). Native biventricular circulation was achieved in 12 patients after staged LV recruitment; all of these patients had restriction at the atrial septum.ConclusionsIn these patients with borderline LH disease who underwent SVP, it is possible to increase LH dimensions by using an LV recruitment strategy. In a subset of patients, this strategy allowed establishment of biventricular circulation.

Objective: To review the short and medium term outcome of transcatheter pulmonary valve perforation (PVP) in patients with pulmonary atresia-intact ventricular septum and non-right ventricular dependant coronary circulation (PA/IVS non-RVDCC).Background: PVP in patients with PA/IVS non-RVDCC has become more common in the past two decades. However, data on outcomes with this strategy are mixed.Methods: Data were reviewed retrospectively for all patients with PA/IVS non-RVDCC treated from 1996- 2010 at our institution. Patients who had severe neonatal Ebstein malformation, or initial interventional management at another institution were excluded.Results: PVP was attempted in 30 of 50 patients (60%); 26 (87%) of these had a successful procedure. Twenty-four patients (48%) had surgery without PVP. There were no deaths in the cohort. Complications of PVP included 5 (17%) myocardial perforations. Of those with successful PVP, 10 (38%) did not have surgery (PVP-NS) and 16 (62%) had surgery (PVP-S) prior to discharge. Tricuspid valve (TV)Z-score was larger in the PVP-NS than in PVP-S patients, with median TV diameterZ-scores of10.7 (20.9, 1.7) and21.1 (-2.8, 2), respectively (P50.01). Time from PVP to either hospital discharge (PVP-NS group) or surgery (PVP-S group) was significantly different between groups: 15 (7, 22) and 8 days (0, 46), respectively (P50.01). There were no differences in the number of trials or lowest arterial PaO2 off prostaglandins between groups. All patients in the PVP-NS group had a biventricular circulation at a median follow-up of 4.3 years. Conclusions: The results of a collaborative approach to treating neonates with PA/IVS non-RVDCC are excellent. Smaller TV size is associated with greater likelihood of surgery prior to discharge, and may serve as a surrogate for early RV inadequacy. V C 2012 Wiley Periodicals Inc.

Background In some studies, tight glycemic control with insulin improved outcomes in adults undergoing cardiac surgery, but these benefits are unproven in critically ill children at risk for hyperinsulinemic hypoglycemia. We tested the hypothesis that tight glycemic control reduces morbidity after pediatric cardiac surgery. Methods In this two-center, prospective, randomized trial, we enrolled 980 children, 0 to 36 months of age, undergoing surgery with cardiopulmonary bypass. Patients were randomly assigned to either tight glycemic control (with the use of an insulin-dosing algorithm targeting a blood glucose level of 80 to 110 mg per deciliter [4.4 to 6.1 mmol per liter]) or standard care in the cardiac intensive care unit (ICU). Continuous glucose monitoring was used to guide the frequency of blood glucose measurement and to detect impending hypoglycemia. The primary outcome was the rate of health care–associated infections in the cardiac ICU. Secondary outcomes included mortality, length of stay, orga...

Background Long-term outcomes of repair of tetralogy of Fallot associated with complete atrioventricular septal defect are seldom reported. We report our survival and reintervention outcomes over a 29-year time period. Methods Between March 1979 and April 2008, 61 patients with the combined cardiac defect of atrioventricular septal defect and tetralogy of Fallot were surgically managed. Trisomy 21 was present in 49 (80%) patients. Primary repair was performed in 36 patients at a median age of 9 months (range, 1 month to 16 years), whereas 25 patients had initial palliation by systemic–pulmonary shunt at a median age of 21 months (range, 0 days to 36 years). Thirty-one (51%) patients had a transannular patch. Fifty-three patients required right ventriculotomy for relief of the right ventricular outflow tract obstruction. Four patients had a right ventricle–pulmonary artery conduit with a homograft. Relationships between patient characteristics and outcome variables were examined using Kaplan-Meier survival curves; comparisons were performed using the log–rank test. Results Median follow-up was 4.7 years. A total of 12 patients died during the course of follow-up: 4 (7%)deaths within 30 days of surgery and 8 late deaths (range, 4 months to 9.9 years after repair). Since 2000, there have been no early deaths and 1 late death, 5 months after the operation. The estimated survival at 5 years after definitive repair was 82% (95% confidence interval, 69%, 90%). Time to death was not associated with any patient or surgical variables examined. Overall, 30% of the survivors required a reoperation. The type of reoperations was on the mitral valve (4 repairs, 4 replacements) and 7 pulmonary valve replacements. We did not find an effect of era on mortality (P = .23 for comparison of 1979-1989, 1990-1999, and 2000-2008). The percentage of patients with primary repair did not change during the different quartiles. The estimated freedom from reoperation at 5 years was 80% (65%, 90%). Time to reoperation was shorter for patients with a conduit (P = .01). Conclusions Excellent long-term survival was achieved after repair of tetralogy of Fallot associated with complete atrioventricular septal defect. Palliation and primary repair resulted in comparable outcomes; as such, primary repair is favored. The choice of right ventricular outflow tract reconstruction affects the need for reoperation.

Objectives. We report our analysis of conventional surgery and the cone procedure for Ebstein's malformation (EM) of the tricuspid valve at a single institution. Previous conventional surgery for EM, including use of bioprosthetic valves, has inherent problems especially in pediatrics. The newer cone procedure aims to construct a funnel-like valve out of native leaflets, obviating problems with artificial valves. Methods. This is a retrospective cohort study to examine short-term outcomes of both surgeries for EM. Results. Nineteen patients (our initial cohort) had the cone procedure, and 13 had conventional tricuspid valve repair or replacement. No early deaths occurred in either group. Three cone and one conventional repair patients required reoperation. Two of 19 patients in the cone and one of 13 in the conventional group died suddenly >30 days after operation, assumed secondary to dysrhythmias. At discharge, by two-dimensional echocardiography, the cone group had 85% reduction in tricuspid valve regurgitation (TVR), and the conventional group had 56% reduction, P= .004. This decrease of TVR persisted to a greater extent in the cone group. Discussion. Short-term results for the cone procedure are similar to conventional surgery. The cone procedure uses autologous tissue; hypothetically, early favorable improvement in reduction of TVR should persist.

Background Clinical indications and outcomes for children requiring multiple extracorporeal membrane oxygenation (ECMO) runs during a single hospital admission are rarely reported. We review indications, cannulation techniques, and outcomes for infants requiring multiple ECMO runs after congenital heart surgery. Methods Demographic and ECMO-related data for all children requiring multiple ECMO runs during a single hospital admission from1995 to 2008 were extracted from our institution's ECMO database. Survival was defined as survival to hospital discharge. Demographic and ECMO data were compared between survivors and nonsurvivors using the Mann-Whitney test for continuous and Fisher's exact test for categorical data. Results Twenty children (5% of 391 ECMO runs) with a median age of 5 days (range, 4 to 180 days) and weight of 3.22 kg required repeat ECMO support for persistent cardiopulmonary dysfunction despite successful weaning from an index ECMO run. Of the two-ventricle repair group, tetralogy of Fallot, truncus arteriosus, and transposition constituted the majority of cardiac diagnoses (75%), whereas single-ventricle physiology made up the rest (25%). The median total ECMO duration was 191 hours (range, 77 to 485 hours), and median hospital length of stay was 41 days (range, 6 to 297 days). Five (25%) patients survived to discharge. When survivors were compared with nonsurvivors, there were no significant differences in demographics, single or biventricular repair, indication for ECMO support, or need for surgical reoperation while on ECMO. However, the incidence of renal failure was higher in nonsurvivors than in survivors after multiple ECMO runs (12 versus 2.0; p = 0.03). Conclusions Survival in children undergoing congenital heart surgery requiring multiple ECMO runs is low, but 1 in 4 patients will survive to discharge. If a correctable lesion or ventricular impairment is present, repeat ECMO support may be justified. Development of renal failure is associated with nonsurvival in these patients.

Background and Methods. As the first multicenter quality improvement collaborative in pediatric cardiology, the Joint Council on Congenital Heart Disease National Pediatric Cardiology Quality Improvement Collaborative registry collects information on the clinical care and outcomes of infants discharged home after first-stage palliation of single-ventricle heart disease, the Norwood operation, and variants. We sought to describe the preoperative and intraoperative characteristics of the first 100 patients enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry. Results. From 21 contributing centers, 59% of infants were male, with median birth weight of 3.1 kg (1.9–5.0 kg); the majority had hypoplastic left heart syndrome (71%). A prenatal diagnosis of congenital heart disease was made in 75%; only one had fetal cardiac intervention. Chromosomal anomalies were present in 8%, and major noncardiac organ system anomalies were present in 9%. Preoperative risk factors were common (55%) but less frequent in those with prenatal cardiac diagnosis (P= .001). Four patients underwent a preoperative transcatheter intervention. Substantial variation across participating sites was demonstrated for choice of initial palliation for the 93 patients requiring a full first-stage approach, with 50% of sites performing stage I with right ventricle to pulmonary artery conduit as the preferred operation; 89% of hybrid procedures were performed at a single center. Significant intraoperative variation by site was noted for the 83 patients who underwent traditional surgical stage I palliation, particularly with use of regional perfusion and depth of hypothermia. Conclusions. In summary, there is substantial variation across surgical centers in the successful initial palliation of infants with single-ventricle heart disease, particularly with regard to choice of palliation strategy, and intraoperative techniques including use of regional perfusion and depth of hypothermia. Further exploration of the relationship of such variables to subsequent outcomes after hospital discharge may help reduce variability and improve long-term outcomes.

Background We sought to identify risk factors for surgical site infections (SSI) in children undergoing cardiac surgery. Methods A matched case-control study was conducted in the Children's Hospital Boston Cardiovascular Program. Surgical site infections were identified for 3 years (2004 to 2006). We identified two randomly selected control patients who underwent cardiac surgery within 7 days of each index case. Univariate and multivariate conditional logistic regression analyses were used to identify risk factors for SSI. In a secondary analysis, risk factors for organ space SSI (mediastinitis) were sought. Secondary analyses were also conducted using only those variables known preoperatively. Results Seventy-two SSI and 144 controls were included. Independent risk factors for any type of SSI were age younger than 1 year (adjusted odds ratio, 2.28; 95% confidence interval, 1.18 to 4.39) and duration of cardiopulmonary bypass greater than 105 minutes (adjusted odds ratio, 1.92; 95% confidence interval, 1.02 to 3.62). Independent risk factors for organ space SSI were aortic cross-clamp time greater than 85 minutes (adjusted odds ratio, 5.61; 95% confidence interval, 1.06 to 29.67) and postoperative exposure to at least three separate red blood cell transfusions (adjusted odds ratio, 7.87; 95% confidence interval, 1.63 to 37.92). When only those potential risk factors known preoperatively were considered, age younger than 1 year independently predicted the subsequent development of any type of SSI, and preoperative hospitalization independently predicted the subsequent development of organ space SSI. Conclusions Younger patients undergoing longer surgical procedures and those requiring more postoperative blood transfusions are at greatest risk for SSI. Additional preventive strategies, including restrictive blood transfusion policies, warrant further investigation.

Background— Near-infrared spectroscopy monitoring of cerebral oxygen saturation (rS o 2 ) has become routine in many centers, but no studies have reported the relationship of intraoperative near-infrared spectroscopy to long-term neurodevelopmental outcomes after cardiac surgery. Methods and Results— Of 104 infants undergoing biventricular repair without aortic arch reconstruction, 89 (86%) returned for neurodevelopmental testing at 1 year of age. The primary near-infrared spectroscopy variable was the integrated rS o 2 (area under the curve) for rS o 2 ≤45%; secondary variables were the average and minimum rS o 2 by perfusion phase and at specific time points. Psychomotor and mental development indexes of the Bayley scales, head circumference, neurological examination, and abnormalities on brain magnetic resonance imaging did not differ between subjects according to a threshold level for rS o 2 of 45%. Lower Psychomotor Development Index scores were modestly associated with lower average ( r =0.23, P =0.03) and minimum ( r =0.22, P =0.04) rS o 2 during the 60-minute period after cardiopulmonary bypass but not with other perfusion phases. Hemosiderin foci on brain magnetic resonance imaging were associated with lower average rS o 2 from postinduction to 60 minutes post cardiopulmonary bypass (71±10% versus 78±6%, P =0.01) and with lower average rSO 2 during the rewarming phase (72±12% versus 83±9%, P =.003) and during the 60-minute period following cardiopulmonary bypass (65±11% versus 75±10%, P =0.009). In regression analyses that adjusted for age ≤30 days, Psychomotor Development Index score ( P =0.02) and brain hemosiderin ( P =0.04) remained significantly associated with rS o 2 during the 60-minute period following cardiopulmonary bypass. Conclusions— Perioperative periods of diminished cerebral oxygen delivery, as indicated by rS o 2 , are associated with 1-year Psychomotor Development Index and brain magnetic resonance imaging abnormalities among infants undergoing reparative heart surgery. Clinical Trial Registration— URL: http://clinicaltrials.gov. Unique identifier: NCT00006183.

Background We sought to identify risk factors for surgical site infections (SSI) in children undergoing cardiac surgery. Methods A matched case-control study was conducted in the Children's Hospital Boston Cardiovascular Program. Surgical site infections were identified for 3 years (2004 to 2006). We identified two randomly selected control patients who underwent cardiac surgery within 7 days of each index case. Univariate and multivariate conditional logistic regression analyses were used to identify risk factors for SSI. In a secondary analysis, risk factors for organ space SSI (mediastinitis) were sought. Secondary analyses were also conducted using only those variables known preoperatively. Results Seventy-two SSI and 144 controls were included. Independent risk factors for any type of SSI were age younger than 1 year (adjusted odds ratio, 2.28; 95% confidence interval, 1.18 to 4.39) and duration of cardiopulmonary bypass greater than 105 minutes (adjusted odds ratio, 1.92; 95% confidence interval, 1.02 to 3.62). Independent risk factors for organ space SSI were aortic cross-clamp time greater than 85 minutes (adjusted odds ratio, 5.61; 95% confidence interval, 1.06 to 29.67) and postoperative exposure to at least three separate red blood cell transfusions (adjusted odds ratio, 7.87; 95% confidence interval, 1.63 to 37.92). When only those potential risk factors known preoperatively were considered, age younger than 1 year independently predicted the subsequent development of any type of SSI, and preoperative hospitalization independently predicted the subsequent development of organ space SSI. Conclusions Younger patients undergoing longer surgical procedures and those requiring more postoperative blood transfusions are at greatest risk for SSI. Additional preventive strategies, including restrictive blood transfusion policies, warrant further investigation.

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.

OBJECTIVES Despite aggressive measures to miniaturize the cardiopulmonary bypass (CPB) circuit in neonates and infants, the CPB prime volume is often at least as large as the patients' blood volume. We conducted an observational study to characterize the hemostatic consequences of a CPB prime consisting of either non-fresh or reconstituted whole blood. METHODS Hematocrit, fibrinogen, platelet count, plasminogen, anti-thrombin III (AT-III), and factors (F) II, V, VII, IX, and X of 30 neonates and infants undergoing cardiac surgery with CPB utilizing either a non-fresh or reconstituted whole blood prime were prospectively evaluated at eight time points. Following protamine administration, microvascular bleeding was treated by protocol. RESULTS The hemostatic composition of the CPB prime was the same following the use of either non-fresh or reconstituted whole blood. The CPB prime platelet count (mean +/- SD) was 5.87 +/- 2.84 x 10(3) microl(-1) when compared to a preoperative platelet count of 298 +/- 142 x 10(3) microl(-1) (P < 0.0001). Twenty patients received 17.3 +/- 9.2 ml x kg(-1) (0.86 +/- 0.46 units x kg(-1)) of platelets with significant improvement in platelet count. Nine patients received 16.7 +/- 13.4 ml x kg(-1) (0.84 +/- 0.67 units x kg(-1)) of cryoprecipitate with significant improvements in FVIII and fibrinogen. CONCLUSIONS Non-fresh or reconstituted whole blood as a component of a small volume CPB prime in neonates and infants induces clinically significant dilutional thrombocytopenia in conjunction with less significant reductions in fibrinogen, FII, FV, FVII, FVIII, FIX, FX, plasminogen, and AT-III.

Objective Perioperative stroke and periventricular leukomalacia have been reported to occur commonly in infants with congenital heart disease. We aimed to determine the incidence and type of brain injury in infants undergoing 2-ventricle repair in infancy and to determine risk factors associated with such injury. Methods Forty-eight infants enrolled in a trial comparing 2 different hematocrits during surgical repair of congenital heart disease underwent brain magnetic resonance imaging scans and neurodevelopmental testing at 1 year of age. Results Eighteen (38%) of our subjects had tiny foci of hemosiderin by susceptibility imaging, without evidence of abnormalities in corresponding regions on conventional magnetic resonance imaging sequences. Subjects with foci of hemosiderin had a significantly lower Psychomotor Developmental Index at 1 year of age (79.6 ± 16.5, mean ± standard deviation) compared with subjects without these foci (89.5 ± 15.3; P = .04). Older age at surgery and diagnostic group were significantly associated with the presence of hemosiderin foci. Only 1 subject had a small stroke (2%), and 2 subjects had periventricular leukomalacia (4%). Conclusion Foci of hemosiderin without radiologic evidence of ischemic brain injury are an abnormality associated with adverse neurodevelopmental outcome not previously described in magnetic resonance imaging studies of children with surgically repaired congenital heart disease. The association of hemosiderin foci with older age at surgery and cardiac diagnosis, and not with risk factors associated with brain injury, in previous studies suggests that the cause and pathogenesis of this abnormality are different from ischemic brain lesions reported previously.

A healthy 9-month-old boy presented with a 1-month history of cough. A chest x-ray showed a linear metallic foreign body (FB) lying transversely in the posterior mediastinum. Computed tomographic scan confirmed the location and size of the metallic FB and also revealed a large pericardial effusion that was corroborated by echocardiogram. The patient underwent a right thoracotomy revealing a normal esophagus without mediastinitis and a 12-mm needle in the posterior mediastinum embedded in the pericardium with the sharp end abutting the left atrium. The needle was extracted uneventfully, and his postoperative course was unremarkable. The diagnosis and treatment for this case are discussed along with a review of the literature.

We examined changes in cerebral oxygen saturation during infant heart surgery and its relationship to anatomic diagnosis and early outcome.Regional cerebral oxygen saturation (rSO(2)) was measured by near-infrared spectroscopy in 104 infants undergoing biventricular repair without aortic arch obstruction as part of a randomized trial of hemodilution to a hematocrit of 25% vs 35%.Before cardiopulmonary bypass (CPB), infants with tetralogy of Fallot had higher rSO(2) values compared to those with D-transposition of the great arteries (D-TGA) or ventricular septal defect (P0.001). During CPB cooling, low flow, and at the termination of CPB, D-TGA subjects had the highest rSO(2) values (P0.001). There were no significant associations between intraoperative rSO(2) and early postoperative outcomes after adjustment for diagnosis. In 39 D-TGA subjects withor =5 min of deep hypothermic circulatory arrest (DHCA), there was no correlation between the rSO(2) (91% +/- 6%) or hematocrit (29.2% +/- 5.5%) at the onset of arrest and the rate of decline in rSO(2) during arrest.Intraoperative rSO(2) varies according to anatomic diagnosis but accounts for very little of the variance in early outcome. As measured by frontal near-infrared spectroscopy, higher levels of hematocrit and current perfusion techniques appear to provide an adequate oxygen reservoir prior to relatively short periods of DHCA.

ObjectiveTwo randomized trials of hematocrit strategy during hypothermic cardiopulmonary bypass in infant heart surgery have been performed. The first suggested worse outcomes were concentrated in patients with lower hematocrit levels (approximately 20%), whereas the second suggested there was little benefit to increasing the hematocrit level above 25%. The form of the relationship between continuous hematocrit levels and outcomes requires further study.MethodsIn the two trials, 271 infants who underwent biventricular repair not involving the aortic arch were enrolled. Analysis was undertaken of the effects of hematocrit level, as a continuous variable, at the onset of low-flow cardiopulmonary bypass.ResultsPsychomotor Development Index scores at age 1 year varied nonlinearly with hematocrit levels, with increasing scores up to 23.5% hematocrit (P < .001) and a plateau effect beyond 23.5% (P = .42), based on a piecewise linear model. Lower hematocrit levels were associated with more positive intraoperative fluid balance (P < .001 for linear trend) and marginally associated with higher serum lactate levels at 60 minutes after bypass (P = .08 for linear trend), but not with blood products given, nadir of cardiac index in the first 24 hours, or Mental Development Index scores.ConclusionsA hematocrit level at the onset of low-flow cardiopulmonary bypass of approximately 24% or higher is associated with higher Psychomotor Development Index scores and reduced lactate levels. Because the effects of hemodilution may vary according to diagnosis, age at operation, bypass variables such as pH strategy and flow rate, and other perioperative factors, this study cannot ascertain a universally “safe” hemodilution level.

ObjectiveWe sought to identify and characterize a subgroup of patients with hypoplastic left heart syndrome who might be at higher risk for stage I failure.MethodsFrom January 2001 through December 2006, all patients with hypoplastic left heart syndrome who underwent stage I palliation at Children’s Hospital Boston were retrospectively reviewed. The subgroup with the mitral stenosis–aortic atresia variant was studied separately. We evaluated preoperative echocardiographic data, operative characteristics, and postoperative factors associated with death or the need for transplantation. The Kaplan–Meier method was used to assess survival.ResultsThirty-eight (23%) of 165 patients had mitral stenosis–aortic atresia. Hospital mortality or need for transplantation for patients with mitral stenosis–aortic atresia was significantly higher than for other anatomic subgroups (29% vs 7.9%, P = .002). Left ventricle–subepicardial coronary artery communications were present in 20 (53%) patients with mitral stenosis–aortic atresia and were associated with a significantly higher hospital mortality (50% vs 6%, P = .004). No difference in outcome was demonstrated between different sources of pulmonary blood flow. A longer cardiopulmonary bypass time (P = .02) and the need for postoperative extracorporeal membrane oxygenation support (P < .001) were associated with a higher mortality rate.ConclusionsWith improved outcomes in the management of neonates with hypoplastic left heart syndrome, those with the mitral stenosis–aortic atresia variant and left ventricle–subepicardial coronary artery fistulae have emerged as a higher-risk subgroup for failure of stage I palliation. Further investigation is required, and a change in clinical management strategy for this particular subgroup might be warranted.

The presence of an intact or highly restrictive atrial septum (I/HRAS) has long been recognized as a predictor of poor outcome among patients with hypoplastic left heart syndrome (HLHS), although the rarity of this condition has precluded conclusive study. The purpose of this review is to summarize recent surgical outcomes for these patients at our center and to identify predictors.We retrospectively identified all neonates with a diagnosis of HLHS and I/HRAS who underwent stage I palliation at Children's Hospital Boston between January 2001 and December 2006. Chart review enabled analysis of patient and procedural variables.All 32 patients underwent left atrial decompression in utero or postnatally before surgery. Fourteen patients (44%) underwent fetal intervention, either atrial septoplasty (n = 9) or aortic valvuloplasty (n = 5). Twenty-nine of the 32 patients had postnatal left atrial hypertension and underwent transcatheter atrial septoplasty as neonates before surgery; 3 did not require postnatal atrial septoplasty after successful fetal atrial septoplasty. After stage I, hospital survival was 69% (22 of 32). Need for shunt revision (p = 0.02) and for extracorporeal membrane oxygenation use (p0.001) were associated with hospital mortality. Survival at 6 months was 69% for patients who had fetal intervention, and 38% for those who were treated only postnatally (p = 0.2).Surgical outcome for patients with HLHS and I/HRAS continues to improve. Prenatal decompression of the left atrium may be associated with greater hospital survival. Proposed effects of fetal intervention on lung pathology and longer-term survival are subjects for future study in this unique group of patients.

Anticoagulation during pediatric extracorporeal membrane oxygenation (ECMO) is accomplished by titrating heparin administration to maintain an activated clotting time (ACT) of between 180 and 220 seconds. We hypothesized that an ACT of 180 to 220 seconds results in inadequate anticoagulation during pediatric ECMO and that increased heparin levels will lead to increased survival.A retrospective review was conducted of 604 consecutive pediatric ECMO patients at a single institution between 1980 and 2001. Multiple logistic regressions were used to assess the impact on survival of ACT, heparin, age, weight, diagnosis, and previous surgery.There were 349 survivors (57.8%), and 255 (42.2%) nonsurvivors. Mean hours on ECMO were 182 +/- 134 (range, 3 to 957 hours), mean ACT was 227 +/- 50 seconds (range, 158 to 620 seconds), and the mean hourly heparin dose was 45 +/- 21 U/kg (range, 6 to 134 U/kg). Regression analysis indicated that increased heparin administration was predictive of survival (p0.0001), independent of all other variables. The ACT was not a predictor of survival (p = 0.096). Although previous surgery was independently associated with an increased likelihood of ECMO death (p0.001), increased heparin administration again exerted a survival advantage (p = 0.012).Adherence to the recommended ACT of 180 to 220 seconds in pediatric ECMO patients may result in inadequate anticoagulation. Survival is improved by increased heparin administration independent of the ACT. The ACT may be too insensitive to maintain adequate long-term systematic anticoagulation, and other methods, such as heparin levels or functional parameters such as anti-Factor Xa activity or thrombin generation, should be investigated.

The technical performance score (TPS), which measures technical adequacy of repair in congenital cardiac surgery, has been associated with early outcomes, such as mortality, adverse events, prolonged ventilation and hospital length of stay, and midterm outcomes such as post-discharge mortality

Objectives Although mitral valve repair is rarely required in neonates, this population is considered to be at high risk for adverse outcomes. The aim of this study was to review the indications for surgery, mechanisms, repair techniques, and mid-term outcomes of neonatal mitral valve repair. Methods The demographic, procedural, and outcome data were obtained for all neonates who underwent mitral valve repair from 2005 to 2012. The primary endpoints included mortality, transplantation, and mitral valve reoperation. Results Twenty patients were included during the study period. Median age at operation was 11 days (range: 3–25). Eleven patients (55%) presented with mitral stenosis, three had regurgitation (15%), and six had mixed mitral disease (30%). Nineteen of 20 patients had mild or less regurgitation on immediate postoperative imaging. During a median follow-up of 5 months (1 month–4.8 years), six patients died at a median of 33 months (7–41 months) from repair and one patient required orthotopic heart transplantation. Six patients required mitral valve reoperation, five for mitral valve re-repair, and one for mitral valve replacement. Freedom from death, transplantation, or mitral valve replacement was 84.2 ± 8.4% at 1 month, 71.3 ± 11% at 6 months, 64.1 ± 12% at 1 year, and 51.3 ± 15% at 2 years and was worse for patients presenting with mitral regurgitation compared to stenosis or mixed mitral valve disease. Conclusion Although mitral valve repair can be performed with acceptable immediate postoperative result, this procedure carries a high burden of late death and mitral valve reoperations.