Survival, Morbidities, and Developmental Outcomes among Low Birth Weight Infants with Congenital Heart Defects.

OBJECTIVE: Prematurity and low birth weight (LBW) are risk factors for increased morbidity and mortality in infants with congenital heart defects (CHDs). We sought to describe survival, inhospital morbidities, and 2-year neurodevelopmental follow-up in LBW infants with CHD. STUDY DESIGN: We included infants with birth weight (BW) <2,500 g diagnosed with CHD (except isolated patent ductus arteriosus) admitted January 2013 to March 2016 to a single level-IV academic neonatal intensive care unit. We reported CHD prevalence by BW and gestational age; selected in-hospital morbidities and mortality by infant BW, CHD type, and surgical intervention; and developmental outcomes by Bayley's scales of infant and toddler development, third edition (BSID-III) scores at age 2 years. RESULTS: Among 420 infants with CHD, 28 (7%) underwent cardiac surgery. Median (25th and 75th percentiles) gestational age was 30 (range: 27-33) weeks and BW was 1,258 (range: 870-1,853) g. There were 134 of 420 (32%) extremely LBW (<1,000 g) infants, 82 of 420 (20%) were small for gestational age, and 51 of 420 (12%) multiples. Most common diagnosis: atrial septal defect (260/420, 62%), followed by congenital anomaly of the pulmonary valve (75/420, 18%). Most common surgical procedure: pulmonary artery banding (5/28, 18%), followed by the tetralogy of Fallot corrective repair (4/28, 14%). Survival to discharge was 88% overall and lower among extremely LBW (<1,000 g, 81%) infants and infants undergoing surgery (79%). Comorbidities were common (35%); retinopathy of prematurity and bronchopulmonary dysplasia were most prevalent. BSID-III scores were available on 148 of 176 (84%); any scores <85 were noted in 73 of 148 (49%), with language being most commonly affected. CONCLUSION: Among LBW infants with congenital heart disease, hospital mortality varied by BW and cardiac diagnosis. KEY POINTS: · In low birth weight infants with congenital heart disease, survival varied by birth weight and cardiac diagnosis.. · Overall survival was higher than previously reported.. · There were fewer morbidities than previously reported.. · Bayley's scale-III scores at 2 years of age were <85 for nearly half..

Proteomic Analysis of Infants Undergoing Cardiopulmonary Bypass Using Contemporary Ontological Tools.

BACKGROUND: Cardiopulmonary bypass (CPB) is essential for the repair of many congenital cardiac defects in infants but is associated with significant derangements in hemostasis and systemic inflammation. As a result, hemorrhagic complications and thrombosis are major challenges in the management of children requiring CPB or extracorporeal membrane oxygenation. Conventional clinical laboratory tests capture individual hemostatic derangements (low platelets, elevated fibrinogen) but fail to describe the complex, overlapping interactions among the various components of coagulation, including cellular interactions, contact activation, fibrinolysis, and inflammation. Given recent advances in analytic tools for identifying protein-protein interactions in the plasma proteome, we hypothesized that an unbiased proteomic analysis would help identify networks of interacting proteins for further investigation in pediatric CPB. MATERIALS AND METHODS: Infants up to 1 y of age were enrolled. Plasma samples were collected at 0, 1, 4, and 24 h after CPB. Mass spectrometry was used to identify proteins undergoing changes in concentration after CPB, and STRING and ToppGene tools were used to identify biological networks. Two-dimensional difference gel electrophoresis identified changes in protein concentrations. Inflammatory markers were assessed by enzyme-linked immunosorbent assay at the same time points. RESULTS: Ten infants with cardiac anomalies requiring surgery and CPB were enrolled; no major complications were recorded (median age, 127.5 d; interquartile range, 181.25 d). Using two-dimensional difference gel electrophoresis, >1400 individual protein spots were observed, and 89 proteins demonstrated change in concentration >30% with P < 0.02 when comparing 1, 4, or 24 h to baseline. Among protein spots with significant changes in concentration after CPB, 29 were identified with mass spectrometry (33%). In our interrogation of functional associations among these differentially expressed proteins, our results were dominated by the acute phase response, coagulation, and cell signaling functional categories. Among cytokines analyzed by enzyme-linked immunosorbent assay, IL-2, IL-8, and IL-10 were elevated at 4 h but normalized by 24 h, whereas IL-6 was persistently elevated. CONCLUSIONS: Infants manifest a robust response to CPB that includes overlapping, complex pathways. Further investigation of interactions among immune, coagulation, and cell signaling systems may lead to novel therapeutics or biomarkers useful in the management of infants requiring CPB.

Initial Application of a Bicuspid Aortic Annuloplasty Ring in Pediatric Cardiac Surgery.

Aortic stenosis and aortic insufficiency (AI) are common valvular conditions that may necessitate repair or replacement of the aortic valve. Aortic valve replacement is associated with higher long-term complications and thus, a consistent, reliable method of repair is needed. This is especially true in the pediatric population where lifelong anticoagulation and development of recurrent aortic stenosisor aortic insufficiency are especially problematic. The Hemispherical Aortic Annuloplasty Reconstruction Technology ring has been developed and used for annular stabilization in adults with success, though its efficacy in the pediatric population has yet to be demonstrated. Herein, we discuss the use of a geometric ring in aortic valve repair for the pediatric patient.

Implantation of a HeartMate 3 ventricular assist device in a 21-kg pediatric patient with Fontan failure.

AIMS: The HeartMate 3 (HM3) ventricular assist device (VAD) is gaining popularity in adults due to a favorable risk profile. However, reports of HM3 use in children are limited, potentially due to concerns with device size. MATERIALS AND METHODS: Here we report the successful use of an HM3-VAD as a bridge to transplantation in a 21 kg (BSA 0.84), an 8-year-old male child with Fontan failure on home inotropes. RESULTS: Urgent VAD implantation was performed. The standard adult sewing ring was used. The tricuspid valve and papillary muscles were completely excised from the ventricular cavity to prevent inflow obstruction. The pump was placed in the left pleural space. Outflow graft and driveline implantation were routine. VAD function appeared excellent with a reduction in Fontan pressures and improved kidney and liver function. Reoperation was required once to rule out tamponade and twice to evacuate a recurrent right hemothorax. The patient was discharged 3 months later in good condition and underwent successful heart transplantation 10 months after VAD placement. DISCUSSION: This report demonstrates the feasibility of HM3-VAD implantation in a 21-kg Fontan patient, suggesting HM3 size is not a prohibitive limitation at this weight.

Double-Blind, Randomized, Placebo-Controlled Trial Comparing the Effects of Antithrombin Versus Placebo on the Coagulation System in Infants with Low Antithrombin Undergoing Congenital Cardiac Surgery.

OBJECTIVES: To determine whether precardiopulmonary bypass (CPB) normalization of antithrombin levels in infants to 100% improves heparin sensitivity and anticoagulation during CPB and has beneficial effects into the postoperative period. DESIGN: Randomized, double-blinded, placebo-controlled prospective study. SETTING: Multicenter study performed in 2 academic hospitals. PARTICIPANTS: The study comprised 40 infants younger than 7 months with preoperative antithrombin levels <70% undergoing CPB surgery. INTERVENTIONS: Antithrombin levels were increased with exogenous antithrombin to 100% functional level intraoperatively before surgical incision. MEASUREMENTS AND MAIN RESULTS: Demographics, clinical variables, and blood samples were collected up to postoperative day 4. Higher first post-heparin activated clotting times (sec) were observed in the antithrombin group despite similar initial heparin dosing. There was an increase in heparin sensitivity in the antithrombin group. There was significantly lower 24-hour chest tube output (mL/kg) in the antithrombin group and lower overall blood product unit exposures in the antithrombin group as a whole. Functional antithrombin levels (%) were significantly higher in the treatment group versus placebo group until postoperative day 2. D-dimer was significantly lower in the antithrombin group than in the placebo group on postoperative day 4. CONCLUSION: Supplementation of antithrombin in infants with low antithrombin levels improves heparin sensitivity and anticoagulation during CPB without increased rates of bleeding or adverse events. Beneficial effects may be seen into the postoperative period, reflected by significantly less postoperative bleeding and exposure to blood products and reduced generation of D-dimers.

Recent Advances in Congenital Heart Surgery: Alternative Perfusion Strategies for Infant Aortic Arch Repair.

PURPOSE OF REVIEW: This paper will discuss current cannulation strategies for infant aortic arch repair and compare them to more traditionally used techniques. RECENT FINDINGS: Aortic arch reconstruction in infants has traditionally involved deep hypothermic circulatory arrest which results in total body ischemia. This has been associated with an increased risk of morbidity including bleeding, renal dysfunction, and neurologic injury. Advances in perfusion techniques have allowed for preserved perfusion to the brain during arch repair. Current techniques have further evolved that allow for continuous perfusion of the heart and even the lower body during arch reconstruction. With current techniques, aortic arch reconstruction in infants can be performed with continuous perfusion to the brain, heart, and lower body. Further technical refinements will be helpful, and study is necessary to evaluate the benefit of these strategies.

Clinical pharmacology considerations for children supported with ventricular assist devices.

The ventricular assist device is being increasingly used as a "bridge-to-transplant" option in children with heart failure who have failed medical management. Care for this medically complex population must be optimised, including through concomitant pharmacotherapy. Pharmacokinetic/pharmacodynamic alterations affecting pharmacotherapy are increasingly discovered in children supported with extracorporeal membrane oxygenation, another form of mechanical circulatory support. Similarities between extracorporeal membrane oxygenation and ventricular assist devices support the hypothesis that similar alterations may exist in ventricular assist device-supported patients. We conducted a literature review to assess the current data available on pharmacokinetics/pharmacodynamics in children with ventricular assist devices. We found two adult and no paediatric pharmacokinetic/pharmacodynamic studies in ventricular assist device-supported patients. While mechanisms may be partially extrapolated from children supported with extracorporeal membrane oxygenation, dedicated investigation of the paediatric ventricular assist device population is crucial given the inherent differences between the two forms of mechanical circulatory support, and pathophysiology that is unique to these patients. Commonly used drugs such as anticoagulants and antibiotics have narrow therapeutic windows with devastating consequences if under-dosed or over-dosed. Clinical studies are urgently needed to improve outcomes and maximise the potential of ventricular assist devices in this vulnerable population.

Natural history of nonimmune-mediated thrombocytopenia and acute kidney injury in pediatric open-heart surgery.

OBJECTIVE: Thrombocytopenia and acute kidney injury (AKI) are common following pediatric cardiac surgery with cardiopulmonary bypass (CPB). However, the relationship between postoperative nadir platelet counts and AKI has not been investigated in the pediatric population. Our objective was to investigate this relationship and examine independent predictors of AKI. DESIGN: After IRB approval, we performed a retrospective review of the institution's medical records and database. SETTING: This study was performed at a single institution over a 5-year period. PATIENTS: We included patients <21 years of age undergoing cardiac surgery with CPB. INTERVENTIONS: Demographics, laboratory, and surgical characteristics were captured, and clinical event rates were recorded. MEASUREMENTS: Descriptive statistics were used to evaluate platelet and creatinine distributions. T-tests and chi-squared tests were used to compare characteristics among Acute Kidney Injury Network groups. Multivariable logistic and ordinal logistic regression models were used to determine the association of our predictor of interest, postoperative nadir platelet count and AKI. RESULTS: Eight hundred and fourteen patients (23% infants and 23% neonates) were included in the analysis. Postoperative platelet counts decreased 48% from baseline reaching a mean nadir value of 150 × 109 ·l-1 on postoperative day 3. AKI occurred in 37% of patients including 13%, 17%, and 6% with Acute Kidney Injury Network stages 1, 2, and 3, respectively. The magnitude of nadir platelet counts correlated with the severity of AKI. Independent predictors of severity of AKI include nadir platelet counts, CPB time, Aristotle score, patient weight, intra-operative packed red blood cell transfusion, and having a heart transplant procedure. CONCLUSIONS: In pediatric open-heart surgery, thrombocytopenia and AKI occur commonly following CPB. Our findings show a strong association between nadir platelet counts and the severity of AKI.

Traumatic Gerbode ventricular septal defect and third-degree heart block.

We present an interesting and rare case of traumatic Gerbode ventricular septal defect and complete heart block. The multimodality images illustrate the diagnosis well. This case is an excellent demonstration of the diagnostic utility of multimodality imaging.

Surgical approach to a rare case of double-outlet right ventricle and aortopulmonary window.

Although the spectrum of physiology seen in infants and children with double-outlet right ventricle is broad, this anatomy in combination with an aortopulmonary window is extremely rare. We present an interesting case of an infant prenatally diagnosed with this rare CHD. To our knowledge, this is the first report of complete repair in the neonatal period for such a combination of defects.

Reduction in blood transfusion in a cohort of infants having cardiac surgery with cardiopulmonary bypass after instituting a goal-directed transfusion policy.

BACKGROUND: Current trends in pediatric cardiac surgery and anesthesiology include goal-directed allogeneic blood transfusion, but few studies address the transfusion of platelets and cryoprecipitate. We report a quality improvement initiative to reduce the transfusion of platelets and cryoprecipitate in infants having cardiac surgery with cardiopulmonary bypass (CPB). METHODS: Data from 50 consecutive patients weighing four to ten kilograms having cardiac surgery with CPB were prospectively collected after the institution of a policy to obtain each patient's platelet and fibrinogen levels during the rewarming phase of CPB. Data from 48 consecutive patients weighing four to ten kilograms having cardiac surgery with CPB prior to the implementation of the policy change were retrospectively collected. Demographics, laboratory values and blood product transfusion data were compared between the groups, using the Chi-square/Fisher's exact test or the T-Test/Wilcoxon Rank-Sum test, as appropriate. RESULTS: The results showed more total blood product exposures in the control group during the time from bypass through the first twenty-four post-operative hours (median of 2 units versus 1 unit in study group, p=0.012). During the time period from CPB separation through the first post-operative day, 67% of patients in the control group received cryoprecipitate compared to 32% in the study group (p=0.0006). There was no difference in platelet exposures between the groups. CONCLUSION: Checking laboratory results during the rewarming phase of CPB reduced cryoprecipitate transfusion by 50%. This reproducible strategy avoids empiric and potentially unnecessary transfusion in this vulnerable population.

A change in anticoagulation monitoring improves safety, reduces transfusion, and reduces costs in infants on cardiopulmonary bypass.

BACKGROUND: An immature coagulation system coupled with the hypothermia and hemodilution associated with cardiopulmonary bypass (CPB) in infants makes the activated clotting time (ACT) an ineffective monitor for anticoagulation in this population. The Medtronic HMS Plus Hemostasis Management System (HMS; Medtronic, Inc., Minneapolis, MN, USA) is shown to decrease thrombin generation and blood product requirements. AIM: We conducted a quality improvement initiative to test our hypothesis that the use of HMS results in reduced incidence of subtherapeutic ACT values, blood product usage, and operating room time for infants undergoing cardiac surgery. METHODS: Fifty consecutive patients weighing <10 kg having cardiac surgery requiring CPB had anticoagulation managed by the HMS. Data were compared to that of 50 consecutive patients weighing <10 kg having cardiac surgery who had their anticoagulation monitored by the ACT alone. Comparisons between categorical variables were performed with chi-square tests. Comparisons between continuous variables were performed with the Wilcoxon rank-sum test. Statistical significance was defined as two-tailed P value < 0.05. RESULTS: The HMS group had a 61% decrease in incidence of ACT values <480 s and elimination of ACT values < 400 s at any time on bypass. The HMS group received fewer blood products and spent fewer minutes in the operating room after protamine administration, translating to fewer donor exposures and a savings of $403 in transfusion costs and $440 in operating room time costs. CONCLUSION: Our findings highlight the benefits of individualized heparinization for pediatric patients undergoing CPB with a monitored heparinization system.

Cardiopulmonary bypass is associated with hemolysis and acute kidney injury in neonates, infants, and children*.

OBJECTIVES: This pilot study assesses the degree of hemolysis induced by cardiopulmonary bypass and determines its association with acute kidney injury in pediatric patients. Further, it evaluates the degree to which the use of urinary biomarkers neutrophil gelatinase-associated lipocalin and cystatin C correlate with the presence of acute kidney injury and hemolysis following cardiopulmonary bypass. DESIGN: Prospective observational study. SETTING: A 13-bed pediatric cardiac ICU in a university hospital. PATIENTS: Children undergoing cardiac surgery with the use of cardiopulmonary bypass. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Blood and urine samples were obtained at multiple time points before and after cardiopulmonary bypass. Hemolysis was assessed by measuring levels of plasma hemoglobin and haptoglobin. Acute kidney injury was defined as a doubling in serum creatinine from preoperative baseline and by using the pediatric-modified RIFLE criteria. Urinary neutrophil gelatinase-associated lipocalin and cystatin C levels were measured. A total of 40 patients (range, 3 d to 4.8 yr) were enrolled. Plasma hemoglobin levels increased markedly on separation from cardiopulmonary bypass with a concurrent decrease in haptoglobin. This was associated with an increase in protein oxidation in the plasma. Hemolysis was more evident in younger patients with a longer duration of bypass and in those requiring a blood-primed circuit. Forty percent of patients had a doubling in serum creatinine and acute kidney injury was developed in 88% of patients when defined by the pediatric-modified RIFLE criteria. Controlling for cardiopulmonary bypass time, persistently elevated levels of plasma hemoglobin were associated with a five-fold increase in acute kidney injury. Further, urinary neutrophil gelatinase-associated lipocalin measured 2 hours after separation from cardiopulmonary bypass was associated with acute kidney injury and with elevations in plasma hemoglobin. CONCLUSIONS: Cardiopulmonary bypass in pediatric patients results in significant hemolysis, which is associated with the development of acute kidney injury. The biomarker neutrophil gelatinase-associated lipocalin correlates with both acute kidney injury and hemolysis in this population.

Corynebacterium endocarditis of a percutaneously placed transcatheter pulmonary valve.

We report a case of endocarditis of a transcatheter pulmonary valve-in-valve in a 14-year-old boy with tetralogy of Fallot. He presented with recurrent low-grade fevers, lethargy, and anorexia. Multiple blood cultures grew a gram-positive rod, Corynebacterium pseudodiphtheriticum. He was taken to the operating room for removal of the vegetative endocarditis and pulmonary valve replacement.

A strategy for atrial septal defect closure in small children that eliminates long-term wall erosion risk.

OBJECTIVES: To evaluate feasibility, efficacy, and safety of an approach to atrial septal defect (ASD) occlusion in children ≤20 kg that eliminates cardiac wall erosion risk. BACKGROUND: Cardiac wall erosion is a potentially catastrophic complication of ASD device closure. The HELEX Septal Occluder (HSO) is a compliant device with no reports of erosion. The HSO is technically difficult to deploy in smaller children and cannot be used to close larger defects. To eliminate wall erosion risk, we use the HSO when feasible and surgery for larger defects. METHODS: Retrospective review of ASD procedures performed in children ≤20 kg. RESULTS: Between January 2006 and January 2011, 60 children underwent ASD closure. HSO placement was successful in 32 of 34 patients, and surgical closure was successful in all of 28 patients. Surgical patients were younger (35.1 ± 12.6 vs. 47.4 ± 15.3 months, P < 0.01) and smaller (15.3 ± 3.2 vs. 12.6 ± 4.3 kg; P < 0.01) with larger ASDs (15.8 ± 4.5 vs. 9.8 ± 3.0 mm; P < 0.01). No surgical patients demonstrated residual leak. Residual leak was seen in 14 of 32 (44%) HSO patients on postprocedure day #1 and in 1 of 26 (3.8%) with ≥6 months follow-up. Indications for surgery included: deficient inferior/superior rims (n = 17), provider preference (n = 2), and HSO device not feasible (n = 9). Serious adverse events included device embolization with percutaneous retrieval (n = 1) and postpericardiotomy syndrome without intervention (n = 1). CONCLUSIONS: The HSO can be safely used in most children ≤20 kg. Our approach to ASD closure is associated with minimal morbidity and good short-term results. This approach requires no more than a 15% increase in surgical referrals and eliminates risk of cardiac wall erosion.

Contemporary outcomes for functional single ventricle with total anomalous pulmonary venous connection.

OBJECTIVE: In 2004, we reported improved early survival for patients with functional single ventricle anatomy and total anomalous pulmonary venous connection. This study sought to discover if outcomes have been ameliorated in the contemporary era. METHODS: This was a single-center review of patients with single ventricle anatomy and total anomalous pulmonary venous connection who were admitted from 1984 to 2021. The cohort was divided into similarly sized groups by date of admission: Era 1: 1984 to 1992, Era 2: 1993 to 2007, and Era 3: 2008 to 2021. Survival was compared, and Cox proportional hazards models were used to evaluate the likelihood of mortality. RESULTS: We included 190 patients with single ventricle anatomy and total anomalous pulmonary venous connection. Unbalanced atrioventricular canal defect (70%) was the most common primary diagnosis. The most common type of total anomalous pulmonary venous connection was supracardiac (49%). Approximately one-third (32%) of patients had pulmonary venous obstruction. There were no significant differences in patient characteristics across eras. Early survival after initial palliative operation improved between Eras 1 and 2, and then remained stable in Era 3. Overall survival improved from Era 1 to Eras 2 and 3 (P < .001), but not between Era 2 and 3. Survival to 10 years by Eras 1 to 3 was 15%, 51%, and 54%, respectively. The anatomic features associated with worse survival were hypoplastic left heart syndrome diagnosis (hazard ratio, 1.60; 1.04-2.57) and pulmonary venous obstruction (hazard ratio, 1.80; 1.24-2.69). CONCLUSIONS: Overall survival for patients with single ventricle anatomy and total anomalous pulmonary venous connection has plateaued since the early 2000s. Even in the most recent era, survival to age 10 years remains less than 60%. Risk factors for mortality include the diagnosis of hypoplastic left heart syndrome and pulmonary venous obstruction. Further studies should focus on identification of the pathophysiological factors underlying the increased mortality.

Atrial septal defect repair by inversion of a juxtaposed left atrial appendage.

A 7-year-old child was noted to have dextrojuxtaposition of the left atrial appendage at the time of surgical atrial septal defect repair. Given the favourable anatomic location and size of the atrial appendage, it was inverted and used to close the atrial defect. This is the first report of atrial septal defect repair using a juxtaposed atrial appendage. The cardiac anatomy and theoretical benefits of this repair are discussed.

Corticosteroids and outcome in children undergoing congenital heart surgery: analysis of the Pediatric Health Information Systems database.

BACKGROUND: Children undergoing congenital heart surgery often receive corticosteroids with the aim of reducing the inflammatory response after cardiopulmonary bypass; however, the value of this approach is unclear. METHODS AND RESULTS: The Pediatric Health Information Systems Database was used to evaluate outcomes associated with corticosteroids in children (0 to 18 years of age) undergoing congenital heart surgery at 38 US centers from 2003 to 2008. Propensity scores were constructed to account for potential confounders: age, sex, race, prematurity, genetic syndrome, type of surgery (Risk Adjustment in Congenital Heart Surgery [RACHS-1] category), center, and center volume. Multivariable analysis, adjusting for propensity score and individual covariates, was performed to evaluate in-hospital mortality, postoperative length of stay, duration of ventilation, infection, and use of insulin. A total of 46 730 children were included; 54% received corticosteroids. In multivariable analysis, there was no difference in mortality among corticosteroid recipients and nonrecipients (odds ratio, 1.13; 95% confidence interval, 0.98 to 1.30). Corticosteroids were associated with longer length of stay (least square mean difference, 2.18 days; 95% confidence interval, 1.62 to 2.74 days), greater infection (odds ratio, 1.27; 95% confidence interval, 1.10 to 1.46), and greater use of insulin (odds ratio, 2.45; 95% confidence interval, 2.24 to 2.67). There was no difference in duration of ventilation. In analysis stratified by RACHS-1 category, no significant benefit was seen in any group, and the association of corticosteroids with increased morbidity was most prominent in RACHS-1 categories 1 through 3. CONCLUSION: In this observational analysis of children undergoing congenital heart surgery, we were unable to demonstrate a significant benefit associated with corticosteroids and found that corticosteroids may be associated with increased morbidity, particularly in lower-risk patients.

Arrhythmias in patients with hypoplastic left heart syndrome.

BACKGROUND: Mortality between stage I and II palliation for hypoplastic left heart syndrome (HLHS) has been associated with arrhythmias. The stage-related proportion, associations, and clinical impact of arrhythmias in patients with HLHS have not been evaluated. In addition, arrhythmia subtypes have not been described in this patient group. METHODS: We performed a retrospective analysis of all patients at Duke University Medical Center who received one or more palliative stages for HLHS from September 2000 to October 2008. RESULTS: Overall, 49 (57%) of 86 patients had 63 arrhythmias. The majority of arrhythmias occurred between stage I and II, with 44 (51%) of 86 patients manifesting a new arrhythmia. Arrhythmias occurring in this interval tended to be associated with a higher mortality compared with arrhythmias occurring after stage II (odds ratio = 3.2 [95% CI 0.84-12.0], P = .09). Overall mortality was similar in patients with and without arrhythmias (P = .99). Supraventricular tachycardia was the most common arrhythmia (16/63; 25%), but persistent bradycardias (sinus node dysfunction or high-grade atrioventricular block) had the worst clinical outcome with 73% mortality (8/11). There was no association between arrhythmia occurrence and degree of tricuspid regurgitation, left ventricular hypertension, genetic syndrome, type of stage I operation, or need for extracorporeal membrane oxygenation. CONCLUSIONS: A large proportion of patients with HLHS experience serious arrhythmias requiring therapy, especially between stage I and II. Persistent bradycardia following stage I is associated with a high mortality rate. Considering all arrhythmia patients, overall mortality was not different compared with the arrhythmia-free group.

Successful extracorporeal membrane oxygenation for respiratory failure in an infant with DiGeorge anomaly, following thymus transplantation.

We report the first successful use of venovenous extracorporeal membrane oxygenation (ECMO) for refractory respiratory failure in an infant with DiGeorge anomaly, following thymus transplantation. A 23-month-old female with complete immune-incompetent DiGeorge anomaly 65 days after allogenic thymus transplantation was treated in our pediatric intensive care unit for acute respiratory failure secondary to bacterial sepsis. She subsequently developed acute hypercarbic respiratory failure unresponsive to conventional medical therapy. She was successfully managed with venovenous ECMO for 4 days, with complete resolution of her respiratory symptoms. This case demonstrates the complex decision making process regarding initiation of ECMO in patients with severe immunodeficiency.