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Objective Routine preoperative laboratory testing (RLT) is common practice in pediatric cardiothoracic surgery and is associated with significant cost burden to patients and families. We sought to examine the value of RLT in patients undergoing elective pediatric cardiothoracic surgery. Methods We conducted a retrospective study of all scheduled elective pediatric cardiothoracic surgery at our institution from 2012 to 2014. Inpatients were excluded. Patient charts were reviewed to obtain preoperative laboratory values and determine relationship to case cancellation. RLT includes complete blood count, prothrombin time, partial thromboplastin time, urinalysis, 7 chemistry metabolic panel, electrocardiogram, and 2-view chest radiograph. Results RLT was completed for 1106 scheduled elective cases. Six (0.5%) cancellations were related to abnormal preoperative laboratory test results: 5 complete blood counts and 1 urinalysis. Hospital charge for RLT averaged $2064 per patient. Based on this incidence, we estimated that 184 routine preoperative laboratory tests, which generated a total hospital charge of $379,776, were required to capture 1 abnormal test significant enough to cancel surgery. An estimated charge of $2,169,552 was generated on prothrombin time, partial thromboplastin time, 7 chemistry metabolic panel, electrocardiogram, and 2-view chest radiograph, and none of these tests resulted in a cancellation. Conclusions RLT does not significantly impact decision-making in elective pediatric cardiothoracic surgery. The decision to order a specific screening test should be clinically driven. Selective preoperative laboratory testing may have a positive impact on healthcare costs without affecting outcomes.

Objective Valve-sparing root replacement is an attractive alternative to composite mechanical or biologic prostheses for aortic root aneurysms in children. Data on outcomes in pediatric patients are limited. We present our institutional experience with 100 consecutive pediatric valve-sparing aortic root procedures. Methods All children who underwent valve-sparing root replacement at our institution from May 1997 to August 2017 were identified, and echocardiographic and clinical data were reviewed. The primary end point was mortality, and secondary end points included complications, further interventions, and subsequent valvular dysfunction. Results Median age at operation was 13.6 years (interquartile range, 9.42-15.9); 51 patients (51%) had Marfan syndrome, and 39 patients (39%) had Loeys–Dietz syndrome. Mean preoperative maximum sinus diameter was 4.4 ± 0.71 cm (z score 7.3 [5.7-9.3]). Most patients (n = 80, 80%) underwent reimplantation procedures with a Valsalva graft. Four patients (4%) underwent David I reimplantation with a straight-tube graft, 13 patients (13%) underwent a Yacoub remodeling procedure, and 3 patients (3%) underwent a Florida sleeve procedure. Perioperative valve-sparing root replacement mortality was 2% (n = 2). Six patients required late reintervention for development of pseudoaneurysms. Eight patients underwent additional aortic surgery. Average time to reoperation was 7.23 ± 4.56 years. Of the 84 patients undergoing a reimplantation procedure, 5 (5.9%) underwent late valve replacement versus 5 (33.3%) of the 15 patients who received a remodeling procedure (P = .001). Conclusions Valve-sparing root replacement is a safe and effective option for children with aortic root aneurysms in children. The reimplantation procedure is preferred. Late aortic insufficiency and pseudoaneurysm formation remain late concerns.

Bovine jugular vein (BJV) grafts (Medtronic, Inc, Minneapolis, Minn) are used to restore right ventricle-to-pulmonary artery continuity. Recent studies have associated these grafts with the development of infective endocarditis. The purpose of this study was to report the incidence of endocarditis in BJV grafts.All BJV grafts placed in the right ventricle-to-pulmonary artery position between 2001 and 2017 at our institution were included. Freedom from endocarditis was analyzed using the Kaplan-Meier method and parametric survival regression models.Overall, 228 patients underwent placement of 253 BJV grafts. The median duration of conduit follow-up was 6 years (5 months to 14 years). Twenty-five conduits developed endocarditis, yielding an incidence of 10% at a median of 7.5 years after surgery. Median duration of symptoms before the diagnosis of endocarditis was 21 days (3-180 days). The most common infectious agents were viridans streptococci (n = 13; 52%). Freedom from endocarditis at 5 and 10 years was 97% and 77%, respectively. After controlling for confounders, BJV grafts had a higher incidence of endocarditis compared with homografts (P .001). Twenty-three (92%) of the conduits that developed endocarditis were managed surgically, with no mortality.The incidence of late endocarditis affecting BJV is high. Increased surveillance and a high index of suspicion for endocarditis are warranted in patients who have undergone implantation of BJV grafts, especially if the graft has been in place for more than 7 years. When infective endocarditis has been diagnosed in these grafts, surgical replacement is recommended, with excellent outcomes.

Background Our institutional policy is to continue centrifugal-flow ventricular assist device support for 3 months or more without activation on the transplant wait-list for physical recovery and assessment of possible myocardial recovery. We evaluated our single-institutional outcomes with centrifugal-flow ventricular assist device support in children. Methods Prospectively collected outcomes data in consecutive patients aged 18 years or less with centrifugal-flow ventricular assist device support were reviewed. Results There were 40 implantations in 39 patients (28 with cardiomyopathy, 11 with congenital heart disease, including 3 with univentricular physiology). The median support was 8 months (range, 1-79), with 13 patients (33%) supported for 12 months or more and a cumulative duration of 41 patient-years. The median age and weight at implantation were 11 (4-18) years and 35 (14-98) kg, respectively. The median body surface area was 1.1 (0.7-2.2) m2, with 16 patients (40%) having a body surface area less than 1.0 m2. Thirty-four patients (85%) had Interagency Registry for Mechanically Assisted Circulatory Support 1 or 2. Children with congenital heart disease were significantly smaller (P Conclusions This study demonstrates favorable outcomes of centrifugal-flow ventricular assist device support in children, including those with congenital heart disease, with an increased incidence of cardiac recovery.

Objectives Neonates undergoing complex congenital heart surgery have a significant incidence of neurologic problems. Erythropoietin has antiapoptotic, antiexcitatory, and anti-inflammatory properties to prevent neuronal cell death in animal models, and improves neurodevelopmental outcomes in full-term neonates with hypoxic ischemic encephalopathy. We designed a prospective phase I/II trial of erythropoietin neuroprotection in neonatal cardiac surgery to assess safety and indicate efficacy. Methods Neonates undergoing surgery for D-transposition of the great vessels, hypoplastic left heart syndrome, or aortic arch reconstruction were randomized to 3 perioperative doses of erythropoietin or placebo. Neurodevelopmental testing using the Bayley Scales of Infant and Toddler Development III was performed at age 12 months. Results Fifty-nine patients received the study drug. Safety profile, including magnetic resonance imaging brain injury, clinical events, and death, was not different between groups. Three patients in each group died. Forty-two patients (22 in the erythropoietin group and 20 in the placebo group; 79% of survivors) returned for 12-month follow-up. In the group receiving erythropoietin, mean Cognitive Scale scores were 101.1 ± 13.6, Language Scale scores were 88.5 ± 12.8, and Motor Scale scores were 89.9 ± 12.3. In the group receiving placebo, Cognitive Scale scores were 106.3 ± 10.8 ( P = .19), Language Scores were 92.4 ± 12.4 ( P = .33), and Motor Scale scores were 92.6 ± 14.1 ( P = .51). Conclusions Safety profile for erythropoietin administration was not different than placebo. Neurodevelopmental outcomes were not different between groups; however, this pilot study was not powered to definitively address this outcome. Lessons learned suggest optimized study design features for a larger prospective trial to definitively address the utility of erythropoietin for neuroprotection in this population.

Background The Infant Jarvik 2015 is an implantable axial-flow ventricular assist device (VAD) that has undergone the major evolutionary design modifications to improve hemocompatibility. This study was conducted in anticipation of data submission to the US Food and Drug Administration to obtain Investigational Device Exemption approval. Methods The VAD was implanted via a left thoracotomy in Barbado sheep (n = 10, 26 (19-34] kg). Anticoagulation was maintained with coumadin, with a target international normalized ratio of greater than the individual sheep's baseline values. The VAD was managed at the highest possible speed as clinically tolerable. Complete necropsy was performed at the end of the study. Results There were 2 early mortalities: tension pneumothorax (n = 1) and shower emboli of the fragmented myocardium (n = 1). The remaining 8 sheep (2 with 30-day and 6 with 60-day protocols) completed the anticipated study duration in excellent condition, with the 6 completing 60-day sheep showing appropriate weight gain during support. There were no signs of clinically significant hemolysis, with the final plasma-free hemoglobin of 2 (1-17) mg/dL. Necropsy showed old renal infarction in 7 sheep. Although thromboembolism can be the potential etiology, given the mild anticoagulation regimen, other sources of emboli were identified in 2 sheep (graft coating material and fragmented myocardium). Flow study demonstrated favorable increase in flow (up to 3.0 L/min) in proportion to change in pump speed. Conclusions This study has demonstrated that the Infant Jarvik 2015 VAD is capable of maintaining its functionality for an extended period of time with minimal hemolysis.

The goal of this study was to use decision analysis to evaluate the impact of varying uncertainties on the outcomes of patients with anomalous aortic origin of a coronary artery.Two separate decision analysis models were created: one for anomalous left coronary artery (ALCA) and one for anomalous right coronary artery (ARCA). Three strategies were compared: observation, exercise restriction, and surgery. Probabilities and health utilities were estimated on the basis of existing literature. Deterministic and probabilistic sensitivity analyses were performed.Surgery was the optimal management strategy for patients30 years of age with ALCA. As age increased, observation became an equivalent strategy and eventually surpassed surgery as the treatment of choice. The advantage on life expectancy for surgery over observation ranged from 2.6 ± 1.7 years for a 10-year-old patient to -0.03 ± 0.1 for a 65-year old patient. In patients with ARCA, observation was the optimal strategy for most patients with a life expectancy advantage over surgery of 0.1 ± 0.1 years to 0.2 ± 0.4 years, depending on age. Surgery was the preferred strategy only for patients25 years of age when the perceived risk of sudden cardiac death was high and the perioperative mortality was low. Exercise restriction was a suboptimal strategy for both ALCA and ARCA in all scenarios.The optimal management in anomalous aortic origin of a coronary artery depends on multiple factors, including individual patient characteristics. Decision analysis provides a tool to understand how these characteristics affect the outcomes with each management strategy and thus may aid in the decision making process for a particular patient.

Objective The purpose of this study was to prospectively analyze the outcomes of patients with anomalous aortic origin of a coronary artery undergoing surgical intervention according to a standardized management algorithm. Methods All patients aged 2 to 18 years undergoing surgical intervention for anomalous aortic origin of a coronary artery between December 2012 and April 2017 were prospectively included. Patients underwent stress nuclear perfusion imaging, stress cardiac magnetic resonance imaging, and retrospectively electrocardiogram-gated computed tomography angiography preoperatively. Patients were cleared for exercise at 3 months postoperatively if asymptomatic and repeat stress nuclear perfusion imaging, stress cardiac magnetic resonance imaging, and computed tomography angiography showed normal results. Results A total of 44 patients, with a median age of 14 years (8-18 years), underwent surgical intervention: 9 (20%) for the anomalous left coronary artery and 35 (80%) for the anomalous right coronary artery. Surgical procedures included unroofing in 35 patients (80%), translocation in 7 patients (16%), ostioplasty in 1 patient (2%), and side-side-anastomosis in 1 patient (2%). One patient who presented with aborted sudden cardiac death from an anomalous left coronary and underwent unroofing presented 1 year later with a recurrent episode and was found to have an unrecognized myocardial bridge and persistent compression of the coronary requiring reintervention. At last follow-up, 40 patients (91%) are asymptomatic and 4 patients have nonspecific chest pain; 42 patients (95%) have returned to full activity, and 2 patients are awaiting clearance. Conclusions Surgical treatment for anomalous aortic origin of a coronary artery is safe and should aim to associate the coronary ostium with the correct sinus, away from the intercoronary pillar. After surgery, the majority of patients are cleared for exercise and remain asymptomatic. Longer follow-up is needed to assess the true efficacy of surgery in the prevention of sudden cardiac death.

ObjectiveTight glycemic control improves outcomes in critically ill adults. There are limited data regarding the effect of glycemic profiles in infants after cardiac operations. The aim of this study was to evaluate the association of hyperglycemia and hypoglycemia on adverse events in infants undergoing the arterial switch operation.MethodsFrom 2000 through 2005, 93 infants underwent the arterial switch operation (mean age, 2.5 ± 5.9 weeks; mean weight, 3.4 ± 0.8 kg). All serum glucose values during the first 24 postoperative hours were documented. The effect of time spent in specific glycemic bands on adverse events was determined.ResultsTwenty-three (25%; group 1) infants spent more than 50% of the time with glucose values between 80 and 110 mg/dL, and 13 (14%; group 2) spent more than 50% of the time with glucose values of greater than 200 mg/dL. A total of 71 adverse events was documented in 45 (48%) of 93 infants. Group 1 infants were more likely to have any adverse event (P = .001) and renal insufficiency (P < .001). Group 2 infants were not more likely to have adverse events. When controlling for preoperative and operative factors, being in group 1 was an independent predictor of postoperative adverse events (P = .004).ConclusionHyperglycemia does not appear to be detrimental in postoperative infants with congenital heart disease. Infants who spent the majority of the time with glucose values between 80 and 110 mg/dL were at increased risk for adverse events. The ideal glycemic profile in the postoperative cardiac infant has yet to be defined.

Objective Real-time information about regional spinal cord ischemia can guide intraoperative management and reduce the risk of paraplegia after thoracic aortic surgery. We hypothesized that near-infrared spectroscopy could provide such information during intercostal and lumbar artery ligation in pigs. Methods Transcutaneous near-infrared spectroscopic sensors were placed in the midline over the upper and lower thoracic vertebrae of 4 progressively larger pigs (weight range 21-70 kg). After the entire aorta was exposed, segmental arteries from T6 through L1 were sequentially ligated while regional oxygen saturation was monitored. Decreases in regional oxygen saturation were calculated as percentage changes from baseline. The degrees of ischemia in the upper and lower spinal cord were compared histopathologically. Results Baseline regional oxygen saturations were similar in the upper (68.8% ± 9.0%) and lower (68.0% ± 11.5%, P = .82) cord. After ligation, however, regional oxygen saturation levels were significantly lower in the lower cord (41.3% ± 10.1%) than in the upper cord (64.8% ± 9.3%, P = .037). The regional oxygen saturation had decreased by 39.0% ± 11.5% in the lower cord but only by 6.3% ± 7.6% in the upper cord ( P = .026). This difference was confirmed microscopically: upper-cord sections had fewer ischemic neurons (8.8 ± 9.4) than did lower-cord sections (21.3 ± 13.6, P = .002). Conclusion Intraoperative spinal cord ischemia was detectable with near-infrared spectroscopy in pigs weighing as much as 70 kg. The potential utility of this technique in patients undergoing thoracic aortic surgery warrants investigation.

Objective The Contegra conduit was developed for right ventricular outflow tract reconstruction. This report evaluates the Contegra conduit, with focus on certain subpopulations in which conduits are known to perform poorly (ie, patients with previous homograft conduits and infants). Methods A retrospective review of 76 patients who had 77 Contegra conduits placed for right ventricular outflow tract reconstruction (January 2001 through August 2005) was completed. Characteristics include the following: median age of 1.6 years (range, 17 days–15.1 years), weight of 9.8 kg (range, 2.5–64.0 kg), and conduit diameter of 16 mm (range, 12–22 mm). Operations performed include right ventricular outflow tract reconstruction for pulmonary atresia–stenosis (n = 33), conduit exchange (n = 28), truncus repair (n = 7), primary conduit placement (n = 6), and the Ross procedure (n = 3). Seventy-nine percent were reoperations. Results There was no hospital mortality. Mean follow-up was 20 ± 14 months. One-, 2-, and 3- year freedom from severe conduit regurgitation was 97%, 86%, and 81%, respectively, and freedom from severe conduit stenosis was 100%. Freedom from reoperation for conduit failure at 1 and 3 years is 98.3% and 93.1%, respectively. All conduit failures (n = 3) were for asymptomatic conduit pseudoaneurysms in the setting of multiple-level pulmonary branch stenoses. Survival at 3 years is 96%. Infants (n = 26) had a freedom from Contegra conduit failure at 3 years of 100%. Patients with previous homograft conduits (n = 26) had a freedom from Contegra conduit failure at 3 years of 100%. Conclusion At midterm follow-up, the Contegra conduit remains a reliable, accessible, and easily implantable conduit for right ventricular outflow tract reconstruction. It appears to be the most promising conduit option for patients with previous homograft conduits and for infants.

Background It has previously been suggested that significant dilatation of the coronary sinus can contribute to left ventricular inflow obstruction and is amenable to surgical correction. The purpose of this study was to review our experience with this rare condition. Methods Since 1995, 6 patients have undergone coronary sinus reduction for concerns of obstruction with other concomitant intracardiac repairs. Preoperative echocardiography identified a significantly dilated left superior vena cava to the coronary sinus in 5 patients (83%) and an abnormal mitral valve in 4 patients (67%); these resulted in abnormal Doppler inflow patterns. Preoperative cardiac catheterization was performed in 5 patients and revealed increased atrial "a" waves, with a gradient to the left ventricular end-diastolic pressure in each case. At the time of surgery, coronary sinus angioplasty was performed in all patients. Results There were no deaths, and there was no major morbidity. Follow-up imaging revealed no significant left ventricular inflow obstruction in any patient. Conclusions We conclude that dilatation of the coronary sinus can become hemodynamically significant and that coronary sinus angioplasty is a safe and effective technique.

Objectives: Patients undergoing cardiopulmonary bypass frequently manifest generalized systemic inflammation and occasionally manifest serious multiorgan failure. Inflammatory responses of bypass are triggered by contact of blood with artificial surfaces of the bypass circuits, surgical trauma, and ischemia-reperfusion injury. We studied the effects of specific inhibition of the alternative complement cascade by using an anti-factor D monoclonal antibody (166-32) in extracorporeal circulation of human whole blood used as a simulated model of cardiopulmonary bypass. Methods: Five healthy blood donors were used in the study. Monoclonal antibody 166-32 was added to freshly collected, heparinized human blood recirculated in a pediatric cardiopulmonary bypass circuit at a final concentration of 18 μg/mL. An irrelevant monoclonal antibody was used as a negative control with the same donor blood in a parallel bypass circuit on the same day. Blood samples were collected at different time points during recirculation for measurement of activation of complement, neutrophils, and platelets by immunofluorocytometric methods and enzyme-linked immunosorbent assays. Results: Monoclonal antibody 166-32 inhibited the alternative complement activation and the production of Bb, C3a, sC5b-9, and C5a. Upregulation of CD11b on neutrophils and CD62P on platelets was also significantly inhibited by monoclonal antibody 166-32. This is consistent with the inhibition of the release of neutrophil-specific myeloperoxidase and elastase and platelet thrombospondin. The production of proinflammatory cytokine interleukin 8 was also suppressed by the antibody. Conclusions: The alternative complement cascade is predominantly activated during extracorporeal circulation. Anti-factor D monoclonal antibody 166-32 is effective in inhibiting the activation of complement, neutrophils, and platelets. Inhibition of the alternative complement pathway by targeting factor D could be useful in reducing systemic inflammation in patients undergoing cardiopulmonary bypass. J Thorac Cardiovasc Surg 2001;122:113-22

Patients with right heart obstructive lesions develop residual or recurrent right ventricle outflow tract pathology as a result of native or implanted pulmonary valve (PV) dysfunction. Until recently, the standard of care has been surgical placement of a PV or valved right ventricle to pulmonary artery conduit. Catheter-based options are being increasingly applied in patients with PV dysfunction. The purpose of our study was to evaluate outcomes of surgical pulmonary valve/conduit replacement (PVR) at a large pediatric hospital to provide contemporary benchmark data for comparison with developing technologies.Retrospective review of patients undergoing PVR not associated with complex concomitant procedures from July 1995 to December 2010 was completed. Inclusion criteria were designed to generally match those applied to patients promoted for catheter-based valve replacement based on age and weight (age≥5 years and weight≥30 kg).There were 148 PVRs with all patients having undergone ≥1 previous interventions (tetralogy of Fallot [53%] and pulmonary atresia [17%]). Surgical indications were PV insufficiency (60%), PV stenosis (26%), and both (13%). Valves used included bioprosthetic (n=108; 73%) and homografts (n=40; 27%). Time-to-extubation, intensive care unit stay, and hospital length of stay were1 day (interquartile range, 0-1 day), 2 days (interquartile range, 1-2 days), and 5 days (interquartile range, 4-6 days), respectively, with no hospital deaths. Freedom from PV reintervention at 1, 3, and 5 years was 99%, 99%, and 94%, respectively. Multivariable analysis showed age13 years (P=.003), and smaller valve size (P=.025) were associated with increased risk of valve reintervention. Patient survival at follow-up (mean, 5.0±3.9 years) was 99%.Surgical PVR is safe with low in-hospital and midterm follow-up mortality and reoperation rates. These outcomes provide a useful benchmark for treatment strategy comparisons.

ObjectiveThere is limited information regarding the true incidence of and risk factors for chylothorax after pediatric cardiac surgery. The objective of this study was to determine, from a large multi-institution database, incidence, associated factors, and treatment strategy in patients undergoing pediatric cardiac surgery.MethodsAll patients younger than 18 years in the Pediatric Health Information System (PHIS) database who underwent congenital heart surgery or heart transplant from 2004 to 2011 were included. Procedure complexity was assessed by Risk Adjustment for Congenital Heart Surgery-1.ResultsIn all, 77,777 patients (55% male) of median age 6.7 months were included. Overall incidence of chylothorax was 2.8% (n = 2205), significantly associated with increased procedure complexity, younger age, genetic syndromes, vein thrombosis, and higher annual hospital volume. Patients with multiple congenital procedures had the highest incidence. Incidence increased with time, from 2% in 2004 to 3.7% in 2011 (P

Objective To determine the incidence and risk factors for endocarditis and reintervention in patients undergoing placement of right ventricle-to-pulmonary artery valve conduits. Methods All right ventricle–to–pulmonary artery valved conduits placed between 1995 and 2014 were included. Freedom from endocarditis, reintervention, and replacement were analyzed using the Kaplan-Meier method and parametric survival regression models. Results A total of 586 patients underwent placement of a total of 792 valved conduits, including 289 (36%) pulmonary homografts, 121 (15%) aortic homografts, 245 (31%) bovine jugular grafts, and 137 (17%) porcine heterografts. There were 474 (60%) primary placements and 318 (40%) replacements. The median duration of conduit follow-up was 7 years; 23 conduits developed endocarditis at a median of 5 years after surgery. The use of bovine jugular grafts was the sole significant risk factor associated with endocarditis (hazard ratio, 9.05; 95% confidence interval, 2.6–31.8 compared with homografts). The hazard was greater for bovine jugular grafts compared with the other conduit types and increased with time; however, bovine jugular grafts were associated with a lower risk for reintervention ( P P = .0002). Factors associated with greater risk of both reintervention and replacement were younger age and smaller conduit size. In addition, a diagnosis of truncus arteriosus was associated with a greater risk for replacement ( P = .03). Conclusions Bovine jugular grafts are associated with a significantly greater risk of late endocarditis but with lower reintervention rates compared with other valved conduits. The risk of endocarditis and durability must be balanced during conduit selection. Antibiotic prophylaxis and a high index of suspicion for endocarditis are warranted in patients with bovine jugular grafts.

ObjectivesSurgical treatment of aortic valve (AoV) disease in childhood involves complex decisions particularly in very small patients. There is no consensus regarding the optimum surgical option. The objective of this review was to analyze a contemporary experience of AoV surgery in a large children's hospital.MethodsA retrospective review of children (aged ≤18 years) undergoing AoV repair or replacement from June 1995 to December 2011 was carried out.ResultsA total of 285 AoV operations (97 repairs, 188 replacements) were performed on 241 patients. Hospital survival for repair was 98% and for replacements was 97%. At follow-up of repairs, there were 16 (17%) reoperations and 3 (3%) late deaths. Follow-up of AoV replacements demonstrated 31 (16%) reoperations (homograft 27, autograft 3, mechanical 1) and 8 (4%) late deaths (homograft 5, autograft 2, mechanical 1). Freedom from reintervention or death (FRD) was found to be lower in repairs for infants (P = .048) and truncal valves (P

Objectives Infective endocarditis is rare in children but potentially carries high mortality and morbidity. Few data exist regarding surgical therapy and the associated outcomes in children with infective endocarditis. The aim of the present study was to describe the characteristics and outcomes of children undergoing surgery for infective endocarditis. Methods A retrospective review of all patients aged 21 years or younger diagnosed with definitive infective endocarditis at a single center from 1996 to 2010 was performed. Results Of 76 identified patients with infective endocarditis (median age, 8.3 years; 73.9% boys), 46 patients (61%) required surgical intervention. Staphylococcus aureus was most commonly isolated (18 patients, 24%) followed by Streptococcus (17 patients, 22%). Common surgical indications included severe valvular insufficiency in 13 patients, septic embolization in 12, concomitant severe valvular insufficiency and ventricular dysfunction in 9, persistent vegetations in 9, and persistent bacteremia in 3. Although early surgery was performed within 7 days of diagnosis in 35 patients (76%), 25 (54%) underwent surgery within 3 days or less. The factors associated with surgery included the presence of ventricular dysfunction, left-sided vegetation, severe valvular insufficiency, septic embolization, and S aureus . Surgery within 3 days or less was associated with the presence of ventricular dysfunction and S aureus . Native valve repair was performed in 50% of patients with native-valve disease. Postoperatively, no septic embolization events occurred and recurrence was low (2%). The 1-, 5-, and 10-year survival was 98% ± 2%, 90% ± 8%, and 81% ± 11%, respectively. Conclusions Children with infective endocarditis can undergo successful early surgical therapy with a low risk of septic embolization, recurrence, and operative mortality.

Objective With improving operative mortality for staged palliation of hypoplastic left heart syndrome, interstage death accounts for an increasing proportion of hypoplastic left heart syndrome mortality. We investigated risk factors for death or cardiac transplantation during the interstage period between bidirectional Glenn and Fontan procedures in children with hypoplastic left heart syndrome. Methods Patients with hypoplastic left heart syndrome who underwent bidirectional Glenn between August 1995 and June 2007 were screened. Standard risk patients, defined by having been discharged after both Norwood and bidirectional Glenn, were included for analysis. Patient demographic, echocardiographic, cardiac catheterization, and operative data were reviewed. Interstage attrition was defined as death or cardiac transplantation more than 30 days after bidirectional Glenn and before the Fontan procedure. Statistical analysis was carried out using the Student t test, Pearson chi-square correlation, and Cox proportional hazard modeling for multivariable analysis. Results Ninety-two patients with hypoplastic left heart syndrome were alive at 30 days after bidirectional Glenn. Of these patients, 8 died and 3 underwent cardiac transplantation at a median of 391 days (range, 59–1175 days) after bidirectional Glenn, yielding an interstage attrition rate of 12%. Removing the 7 patients who are still awaiting Fontan (but all of whom are at least 3.5 years after bidirectional Glenn) adjusts the attrition rate to 13%. Interstage attrition did not correlate with hemodynamic data obtained at cardiac catheterization, aortic arch obstruction, or right ventricular dysfunction. Multivariable analysis demonstrated that the presence of moderate or severe tricuspid valve regurgitation (hazard ratio, 6.02; 95% confidence interval, 1.56–23.24; P z score (hazard ratio, 0.38; 95% confidence interval, 0.16–0.88; P = .02) were independent preoperative risk factors for interstage attrition. Conclusions Interstage attrition between bidirectional Glenn and Fontan procedures occurred in 12% of our study population. Moderate or greater tricuspid valve regurgitation and low weight z score at the time of bidirectional Glenn are important risk factors for interstage attrition between the bidirectional Glenn and Fontan procedures in children with hypoplastic left heart syndrome.

Objective Ductal origin of a pulmonary artery (DOPA) is commonly misdiagnosed as agenesis of a pulmonary artery (PA), which may result in inadequate treatment. The objective is to describe the results of resuscitation of unilateral DOPA. Methods This study is a retrospective review of all patients with unilateral DOPA who underwent PA resuscitation at Texas Children's Hospital from 1993 to 2012. Patients with other cardiac or contralateral lung anomalies were excluded. Results Ten patients, median age 2 years (range, 3 days to 9 years), with unilateral DOPA were included. Symptoms were present in 6 patients. Cardiac catheterization was performed in all and showed a patent duct or a ductal stump in most patients and a small PA on wedge angiography of the pulmonary veins. Two patients underwent single-stage centralization. The other 8 underwent ductal stenting (n = 2) or a systemic-to-PA shunt (n = 6) as the first stage before centralization. The 2 patients with ductal stenting developed pulmonary edema. The 2 patients with a cryopreserved vein shunt developed early thrombosis requiring reintervention. Nine patients have undergone centralization. Six patients have required further interventional procedures. There have been no deaths. Symptoms and lung hypoplasia have improved in all patients. Median relative lung perfusion at follow-up was 26% (range, 12%-46%) with significant improvement in the size of the affected PA. Conclusions PA resuscitation is effective at restoring flow to the affected lung resulting in improved diameter of the PA, lung growth, and resolution of symptoms. PA resuscitation should be considered in all children with DOPA, including those beyond infancy.

J Thorac Cardiovasc Surg 2002;124:413-6

Objective Fenestration during Fontan palliation has traditionally been used to decrease surgical morbidity and mortality, particularly in high-risk cases. Potential limitations include oxygen desaturation, risk of paradoxic embolism, and need for late intervention. Our practice has evolved away from routine fenestration with increased extracardiac conduit use. We reviewed our experience with Fontan palliation and retrospectively assessed outcomes with decreased fenestration. Methods Between January 2002 and April 2008, 226 patients underwent primary Fontan palliation. Outcomes were assessed by hospital stay, chest drain duration, short- and long-term survivals, and late interventions. Results Anatomic subtypes were single left ventricle (n = 88, 38.9%), single right ventricle (n = 78, 34.5%), common ventricle (n = 19, 8.4%), and heterotaxy syndrome (n = 41, 18.1%). Lateral tunnel connection was created in 69 patients (30.5%); extracardiac connection was created in 157 (69.5%). Mean age and weight at surgery were 4.3 ± 3.8 years and 17.2 ± 9 kg, respectively. In 2002, 14 of 16 patients (87.5%) had fenestrated Fontan circulations, versus 2 of 32 (6.3%) in 2008. Mean hospital stay was 10.8 ± 8.8 days. Survival to discharge or 30 days was 98.7%. There were 2 (0.9%) late deaths during mean follow-up of 2.0 ± 1.7 years. Outcomes were equivalent between fenestrated and nonfenestrated procedures across anatomic subtypes. Conclusions Highly selective use of Fontan fenestration is achievable while maintaining excellent outcomes without increased surgical morbidity or mortality, irrespective of anatomic subtype. Risks of hypoxia, systemic embolism, and late instrumentation can be avoided in most cases.

ObjectivesWe sought to characterize factors and outcomes associated with postoperative acute kidney injury in infants undergoing cardiac surgery.MethodsWe retrospectively studied 430 infants (

ObjectiveIn 1984, the first successful infant heart transplant was performed at Texas Children’s Hospital. This study analyzes the 21-year experience with pediatric heart transplantation at Texas Children’s Hospital to assess whether and how survival has changed over time.MethodsBetween November 1, 1984, and October 3, 2005, 164 consecutive orthotopic heart transplants were performed on 154 patients. Characteristics: mean age 7.1 ± 6.0 years, mean body surface area 0.8 ± 0.5 m2. Diagnosis at transplant: cardiomyopathy 53.0% (n = 87), congenital heart defect 39.0% (n = 64), retransplant 7.9% (n = 13). Multivariate risk factor analysis of 32 variables was completed by Cox proportional hazards regression models.ResultsMean follow-up was 5.9 ± 4.8 years. Overall Kaplan–Meier survival was 82% at 1 year, 65% at 5 years, and 54% at 10 years. After 1995, Kaplan–Meier survival (91% at 1 year and 71% at 5 years) was significantly improved over pre-1995 survival (71% at 1 year, 57% at 5 years, and 48% at 10 years; P =.026). Hospital survival improved in the post-1995 era (96%) compared with the pre-1995 era (77%; P < .001). Life-table analysis by yearly increments demonstrates only an improved survival (pre-1995, 71% →post-1995, 91%) in the first posttransplant year (P = .001); every subsequent year the mortality rates are the same (P = .92). Risk factors for overall mortality are prolonged postoperative intubation (>5 days) and longer cardiopulmonary bypass time.ConclusionsPrimarily attributable to an increase in early survival, overall pediatric heart transplant survival is improved. However, after the first posttransplant year, the rate of mortality has not changed in 21 years. This highlights the need for new therapies to treat children both with or in need of a heart transplant.

Objective: This study was undertaken to describe the combined measurement of cerebral blood flow velocity and cerebral oxygen saturation as a guide to bypass flow rate for regional low-flow perfusion during neonatal aortic arch reconstruction. Methods: Data were prospectively collected from 34 patients undergoing neonatal aortic arch reconstruction with regional low-flow perfusion. Cerebral oxygen saturation and blood flow velocity were measured by near-infrared spectroscopy and transcranial Doppler ultrasonography, respectively, throughout cardiopulmonary bypass. After cooling to 17°C to 22°C, baseline values of cerebral oxygen saturation and blood flow velocity were recorded during full-flow bypass. Regional low-flow perfusion was instituted for aortic arch reconstruction, and bypass flow rate was adjusted to maintain cerebral oxygen saturations and blood flow velocities within 10% of baseline recorded during cold full-flow bypass. Cerebral oxygen saturations and blood flow velocities were recorded again after repair during full-flow hypothermic bypass. Bypass flow during regional low-flow perfusion was recorded, as were arterial pressure and blood gas data. One-way repeated measures analysis of variance was used to determine differences in values during regional low-flow perfusion relative to baseline and after perfusion. Results: A mean bypass flow of 63 mL/(kg × min) was required to maintain cerebral oxygen saturations and blood flow velocities within 10% of baseline. Mean arterial pressure had a poor correlation with the required bypass flow rate ( r 2 = 0.006 by linear regression analysis). Fourteen of 34 patients had a cerebral oxygen saturation of 95% during regional low-flow perfusion, placing them at risk for cerebral hyperperfusion if the cerebral oxygen saturation had been used alone to guide bypass flow. Pressure was detected in the umbilical or femoral artery catheter (mean 12 mm Hg) in all patients during regional low-flow perfusion. Conclusions: Cerebral blood flow velocity, as determined by transcranial Doppler ultrasonography, adds valuable information to cerebral oxygen saturation data in guiding bypass flow during regional low-flow perfusion. Its most important use may be prevention of cerebral hyperperfusion during periods with high near-infrared spectroscopic saturation values. J Thorac Cardiovasc Surg 2003;125:491-9

Objective The aim of this study was to measure cerebral oxygenation, cerebral blood volume index, and cerebral blood flow velocity values in both cerebral hemispheres before, during, and after regional low-flow cerebral perfusion for neonatal aortic arch reconstruction and to test the hypothesis that cerebral blood volume index measured by near infrared spectroscopy correlates with cerebral blood flow velocity measured by transcranial Doppler ultrasonography. Methods Bilateral near infrared spectroscopy and transcranial Doppler ultrasonography sensors were placed, and values were recorded immediately before, during, and after regional low-flow cerebral perfusion at 18°C. Cerebral oxygen saturations, cerebral blood flow velocities, and cerebral blood volume index values were compared by Mann-Whitney U test. Correlations between values of cerebral blood volume index and cerebral blood flow velocity were tested with Spearman rank order correlation. Results Twenty patients were studied. Median cerebral oxygen saturations for the right and left sides were 95% and 95% before regional low-flow cerebral perfusion, 95% and 87% during regional low-flow cerebral perfusion ( P = .054), and 93% and 94% after regional low-flow cerebral perfusion. Median cerebral blood flow velocity values did not change during regional low-flow cerebral perfusion. Cerebral blood volume index exhibited a poor correlation with cerebral blood flow velocity. Conclusions Regional low-flow cerebral perfusion provides comparable blood flows and oxygenation to both cerebral hemispheres. Transcranial Doppler ultrasonography is recommended as a corroborative method with near-infrared spectroscopy to guide flow during regional low-flow cerebral perfusion, because cerebral blood volume index does not correlate with cerebral blood flow velocity.

Objective There has been considerable improvement in survival after the first stage of palliation for single-ventricle heart disease. Yet, interstage mortality continues to plague this population. Home monitoring has been proposed to reduce interstage mortality. We review our experience after creation of a Single Ventricle Program. Methods All infants with a single ventricle heart defect who were admitted to Texas Children's Hospital from the inception of the Single Ventricle Program on September 1, 2007, to January 1, 2010, were included in the Single Ventricle Program cohort. Infants with a single ventricle presenting between January 1, 2002, and August 31, 2007, comprised the pre-Single Ventricle Program group. Anatomic, operative, and postoperative details were noted for all patients. End points included in-hospital death after the first stage of palliation, interstage death (defined as after discharge from the first stage of palliation and before the second stage of palliation), and death or heart transplantation by 1 year of age. Interstage weight gain was also compared. Results A total of 137 infants with a single ventricle were included in the pre-Single Ventricle Program cohort, and 93 infants were included in the Single Ventricle Program cohort. Anatomic subtypes were similar between groups. There was significant improvement in rate of interstage weight gain, whereas age at the second stage of palliation was significantly reduced in the Single Ventricle Program group. In-house mortality decreased during the Single Ventricle Program era ( P = .021). Interstage mortality did not significantly decrease in the Single Ventricle Program group. However, 1-year transplant-free survival improved during the Single Ventricle Program era ( P = .002). Conclusions The Single Ventricle Program improved interstage weight gain, thereby allowing for early second-stage palliation at an equivalent patient weight. Interstage mortality was not significantly reduced by our program. However, 1-year transplant-free survival was significantly improved in patients in the Single Ventricle Program.