Outcomes of Neonates Born with Symptomatic Tetralogy of Fallot and Absent Ductus Arteriosus.

OBJECTIVE: To test the hypothesis that neonates with symptomatic tetralogy of Fallot (TOF) and absent ductus arteriosus (ADA) have worse clinical outcomes compared with those with a ductus arteriosus (DA), and that this difference is driven by those born with ADA and with critically deficient pulmonary blood flow (CDPBF). STUDY DESIGN: We performed a retrospective, multicenter cohort study of neonates who underwent intervention for symptomatic TOF comparing death and reintervention between subjects with and without a DA identified on fetal echocardiogram or on echocardiogram performed in the first postnatal day. Exclusion criteria were as follows: inability to define DA status, collaterals supplying pulmonary blood flow, atrioventricular septal defect, and absent pulmonary valve. We defined CDPBF as undergoing a procedure to augment pulmonary blood flow on the date of birth or extracorporeal membrane oxygenation prior to such a procedure. RESULTS: The study cohort included 519 patients, among whom 11% had ADA. Patients with ADA were more likely to have a genetic syndrome and had smaller branch pulmonary artery size. In analyses adjusting for center, interventional treatment strategy, genetic syndrome, and minimum branch pulmonary artery size, ADA was associated with higher mortality risk (adjusted hazard ratio of 2.37 (95% CI: 1.07,5.27; P = .034). Seven patients had CDPBF (1.3% of the entire cohort and 12% of patients with ADA). CONCLUSIONS: A minority of symptomatic TOF neonates have ADA, which is associated with higher adjusted mortality risk compared with those with a DA. CDPBF appears to be a rare but important entity in this population.

Neonatal heart transplantation in the United States: Trends and outcomes.

BACKGROUND: Heart transplantation in the neonatal period is associated with excellent survival. However, outcomes data are scant and have been obtained primarily from two single-center reports within the United States. We sought to analyze the outcomes of all neonatal heart transplants performed in the United States using the United Network for Organ Sharing (UNOS) dataset. METHODS: The UNOS dataset was queried for patients who underwent infant heart transplantation from 1987 to 2021. Patients were divided into two groups based on age - neonates (<=31 days), and older infants (32 days-365 days). Demographic and clinical characteristics were analyzed and compared, along with follow up survival data. RESULTS: Overall, 474 newborns have undergone heart transplantation in the United States since 1987. Freedom from death or re-transplantation for neonates was 63.5%, 58.8% and 51.6% at 5, 10, and 20 years, respectively. Patients in the newborn group had lower unadjusted survival compared to older infants (p < .001), but conditional 1-year survival was higher in neonates (p = .03). On multivariable analysis, there was no significant difference in survival between the two age groups (p = .43). Black race, congenital heart disease diagnosis, earlier surgical era, and preoperative mechanical circulatory support use were associated with lower survival among infant transplants (p < .05). CONCLUSIONS: Neonatal heart transplantation is associated with favorable long-term clinical outcomes. Neonates do not have a significant survival advantage over older infants. Widespread applicability is limited by the small number of available donors. Efforts to expand the donor pool to include non-standard donor populations ought to be considered.

Incidence and recovery of post-surgical heart block in children following cardiac surgery.

BACKGROUND: A subset of patients who develop post-surgical heart block have recovery of atrioventricular node function. Factors predicting recovery are not understood. We investigated our centre's incidence of post-surgical heart block and examine factors associated with recovery of atrioventricular node function. METHODS: We conducted a single-centre retrospective study of patients 0 - 21 years who underwent cardiac surgery between January 2010 and December 2019 and experienced post-operative heart block. Data including patient and clinical characteristics and operative variables were collected and analysed. RESULTS: Of 6333 surgical hospitalisations, 128 (2%) patients developed post-operative heart block. Of the 128 patients, 90 (70%) had return of atrioventricular node function, and 38 (30%) had pacemaker placement. Of the 38 patients who underwent pacemaker placement, 6 (15.8%) had recovery of atrioventricular node function noted on long-term follow-up. Median time from onset of heart block to late atrioventricular node recovery was 13 days (Interquartile range: 5 - 117). Patients with single-ventricle physiology (p = 0.04), greater weight (p = 0.03), and shorter cardiopulmonary bypass time (p = 0.015) were more likely to have recovery. The use of post-operative steroids was similar between all groups (p = 0.445). Infectious or wound complications were similar between pacemaker groups (p = 1). CONCLUSIONS: Two per cent of patients who underwent congenital cardiac surgery developed post-operative heart block, and 0.6% underwent pacemaker placement. Early recovery of atrioventricular node was associated with greater weight at the time of surgery, single-ventricle physiology, and shorter cardiopulmonary bypass time. Late recovery of atrioventricular node conduction following pacemaker placement occurred in 15.8% of patients.

Impact of Management Strategy on Feeding and Somatic Growth in Neonates with Symptomatic Tetralogy of Fallot: Results from the Congenital Cardiac Research Collaborative.

OBJECTIVE: To evaluate early growth following primary or staged repair of neonatal symptomatic tetralogy of Fallot (sTOF). STUDY DESIGN: We performed a retrospective, multicenter cohort study of consecutive infants with sTOF who underwent initial intervention at age ≤30 days, from 2005 to 2017. Management strategies were either primary repair or staged repair (ie, initial palliation followed by complete repair). The primary outcome was change in weight-for-age z-score (ΔWAZ) from the initial intervention to age 6 ± 2 months. Secondary outcomes included method and mode of feeding, feeding-related medications, and feeding-related readmissions. Propensity score adjustment was used to account for baseline differences between groups. A secondary analysis was performed comparing patients stratified by the presence of adequate growth (6-month ΔWAZ > -0.5) or inadequate growth (6-month ΔWAZ ≤ -0.5), independent of treatment strategy. RESULTS: The study cohort included 143 primary repair subjects and 240 staged repair subjects. Prematurity was more common in the staged repair group. After adjustment, median ΔWAZ did not differ between treatment groups over the first 6 months of life (primary: -0.43 [IQR, -1.17 to 0.50]; staged: -0.31 [IQR, -1.31 to 0.71]; P = .55). For the entire cohort, ΔWAZ was negative (-0.36; IQR, -1.21 to 0.63). There were no between-group differences in the secondary outcomes. Secondary analysis revealed that the subjects with adequate growth were more likely to be orally fed at initial hospital discharge (P = .04). CONCLUSIONS: In neonates with sTOF, growth trajectory over the first 6 months of life was substandard, irrespective of treatment strategy. Those patients with adequate growth were more likely to be discharged from the index procedure on oral feeds.

Autologous Cardiac Stem Cell Injection in Patients with Hypoplastic Left Heart Syndrome (CHILD Study).

Mortality in infants with hypoplastic left heart syndrome (HLHS) is strongly correlated with right ventricle (RV) dysfunction. Cell therapy has demonstrated potential improvements of RV dysfunction in animal models related to HLHS, and neonatal human derived c-kit+ cardiac-derived progenitor cells (CPCs) show superior efficacy when compared to adult human cardiac-derived CPCs (aCPCs). Neonatal CPCs (nCPCs) have yet to be investigated in humans. The CHILD trial (Autologous Cardiac Stem Cell Injection in Patients with Hypoplastic Left Heart Syndrome) is a Phase I/II trial aimed at investigating intramyocardial administration of autologous nCPCs in HLHS infants by assessing the feasibility, safety, and potential efficacy of CPC therapy. Using an open-label, multicenter design, CHILD investigates nCPC safety and feasibility in the first enrollment group (Group A/Phase I). In the second enrollment group, CHILD uses a randomized, double-blinded, multicenter design (Group B/Phase II), to assess nCPC efficacy based on RV functional and structural characteristics. The study plans to enroll 32 patients across 4 institutions: Group A will enroll 10 patients, and Group B will enroll 22 patients. CHILD will provide important insights into the therapeutic potential of nCPCs in patients with HLHS.Clinical Trial Registration https://clinicaltrials.gov/ct2/home NCT03406884, First posted January 23, 2018.

Palliation Strategy to Achieve Complete Repair in Symptomatic Neonates with Tetralogy of Fallot.

Neonates with symptomatic tetralogy of Fallot (sTOF) may undergo palliations with varying physiology, namely systemic to pulmonary artery connections (SPC) or right ventricular outflow tract interventions (RVOTI). A comparison of palliative strategies based on the physiology created is lacking. Consecutive sTOF neonates undergoing SPC or RVOTI from 2005-2017 were reviewed from the Congenital Cardiac Research Collaborative. The primary outcome was survival with successful complete repair (CR) by 18 months. A variety of secondary outcomes were assessed including overall survival, hospitalization-related comorbidities, and interstage reinterventions. Propensity score adjustment was utilized to compare treatment strategies. The cohort included 252 SPC (surgical shunt = 226, ductus arteriosus stent = 26) and 68 RVOTI (balloon pulmonary valvuloplasty = 48, RVOT stent = 11, RVOT patch = 9) patients. Genetic syndrome (29 [42.6%] v 75 [29.8%], p = 0.04), weight < 2.5 kg (28 [41.2%] v 68 [27.0%], p = 0.023), bilateral pulmonary artery Z-score < - 2 (19 [28.0%] v 36 [14.3%], p = 0.008), and pre-intervention antegrade flow (48 [70.6%] v 104 [41.3%], p < 0.001) were more common in RVOTI. Significant center differences were noted (p < 0.001). Adjusted survival to CR by 18 months (HR = 0.87, 95% CI = 0.63-1.21, p = 0.41) and overall survival (HR = 2.08, 95% CI = 0.93-4.65, p = 0.074) were similar. RVOTI had increased interstage reintervention (HR = 2.15, 95% CI = 1.36-3.99, p = 0.001). Total anesthesia (243 [213, 277] v 328 [308, 351] minutes, p < 0.001) and cardiopulmonary bypass times (117 [103, 132] v 151 [143, 160] minutes, p < 0.001) favored RVOTI. In this multicenter comparison of physiologic palliation strategies for sTOF, survival to successful CR and overall survival were similar; however, reintervention burden was significantly higher in RVOTI.

Weight impacts 1-year congenital heart surgery outcomes independent of race/ethnicity and payer.

Body mass index, race/ethnicity, and payer status are associated with operative mortality in congenital heart disease (CHD). Interactions between these predictors and impacts on longer term outcomes are less well understood. We studied the effect of body mass index, race/ethnicity, and payer on 1-year outcomes following elective CHD surgery and tested the degree to which race/ethnicity and payer explained the effects of body mass index. Patients aged 2-25 years who underwent elective CHD surgery at our centre from 2010 to 2017 were included. We assessed 1-year unplanned cardiac re-admissions, re-interventions, and mortality. Step-wise, multivariable logistic regression was performed.Of the 929 patients, 10.4% were underweight, 14.9% overweight, and 8.5% obese. Non-white race/ethnicity comprised 40.4% and public insurance 29.8%. Only 0.5% died prior to hospital discharge with one additional death in the first post-operative year. Amongst patients with continuous follow-up, unplanned re-admission and re-intervention rates were 14.7% and 12.3%, respectively. In multivariable analyses adjusting for surgical complexity and surgeon, obese, overweight, and underweight patients had higher odds of re-admission than normal-weight patients (OR 1.40, p = 0.026; OR 1.77, p < 0.001; OR 1.44, p = 0.008). Underweight patients had more than twice the odds of re-intervention compared with normal weight (OR 2.12, p < 0.001). These associations persisted after adjusting for race/ethnicity, payer, and surgeon.Pre-operative obese, overweight, and underweight body mass index were associated with unplanned re-admission and/or re-intervention 1-year following elective CHD surgery, even after accounting for race/ethnicity and payer status. Body mass index may be an important modifiable risk factor prior to CHD surgery.

Evolution of pediatric ventricular assist devices and their neurologic and renal complications-A 24-year single-center experience.

Utilization of ventricular assist devices (VADs) in adult populations with severe heart failure as a bridge to transplant has become the standard of care over the past two decades. Analogously, the use of VADs in pediatric populations has become more commonplace as pediatric heart transplantation has become more prevalent. We still have much to learn, however, about the complications after VAD placement in pediatric patients, their impact on transplantation and, in particular, how outcomes have changed over time. The objectives of this study were to (a) review the experience of a single pediatric VAD center, (b) identify risk factors that could lead to poor outcomes in patients on the transplant waitlist after VAD implantation and (c) demonstrate changes in outcomes over time. A retrospective cohort analysis was performed comparing death as a primary outcome and stroke and acute kidney injury (AKI) as secondary outcomes, across the study period divided into three timed eras. We analyzed 88 patients supported by a VAD over a 24-year timeframe. The duration, age at implant and indication for VAD support did not change significantly across the eras. We found that the incidence of stroke decreased over the study period and, while the rates of AKI did not change over the study period, those who developed AKI, while supported on VAD, had an increased risk of death.

Transcatheter stenting of superior vena cava obstruction after pediatric heart transplantation: A single-center experience assessing risk factors and outcomes.

Transcatheter stent implantation for SVC obstruction following OHT has been well described, particularly in pediatric patients. This study describes a large single-center pediatric heart transplant experience that investigates the risk factors for SVC stenosis requiring stent implantation and its associated outcomes. All pediatric OHTs between January 1, 2000, and December 12, 2016, were examined for risk factors. Twelve of 349 (3.4%) OHTs required SVC stent implantation. Patients who required stents were younger (2.5 years vs 10.0 years, P = 0.0097), weighed less (8.6 kg vs 26.0 kg, P = 0.0024), and were more likely to have had CHD (83% vs 32%, P = 0.001) or previous SCPA (58% vs 18%, P = 0.002). Bicaval anastomosis was not associated with subsequent SVC stent implantation. Symptoms included SVC syndrome and chylous effusions. All 12 patients had evidence of significant SVC obstruction by both echocardiographic Doppler interrogation and transcatheter angiography. There were no acute procedural complications; however, reinterventions were common (four of 12 patients) and occurred at a mean of 5.7 ± 3.6 months poststent implantation. In conclusion, transcatheter SVC stent implantation is safe and effective after OHT. There were significant associations between SVC stent implantation and younger age, smaller weight, CHD, and history of SCPA, but not with bicaval anastomosis or donor-recipient weight ratio.

Delirium in Children After Cardiac Bypass Surgery.

OBJECTIVES: To describe the incidence of delirium in pediatric patients after cardiac bypass surgery and explore associated risk factors and effect of delirium on in-hospital outcomes. DESIGN: Prospective observational single-center study. SETTING: Fourteen-bed pediatric cardiothoracic ICU. PATIENTS: One hundred ninety-four consecutive admissions following cardiac bypass surgery, 1 day to 21 years old. INTERVENTIONS: Subjects were screened for delirium daily using the Cornell Assessment of Pediatric Delirium. MEASUREMENTS AND MAIN RESULTS: Incidence of delirium in this sample was 49%. Delirium most often lasted 1-2 days and developed within the first 1-3 days after surgery. Age less than 2 years, developmental delay, higher Risk Adjustment for Congenital Heart Surgery 1 score, cyanotic disease, and albumin less than three were all independently associated with development of delirium in a multivariable model (all p < 0.03). Delirium was an independent predictor of prolonged ICU length of stay, with patients who were ever delirious having a 60% increase in ICU days compared with patients who were never delirious (p < 0.01). CONCLUSIONS: In our institution, delirium is a frequent problem in children after cardiac bypass surgery, with identifiable risk factors. Our study suggests that cardiac bypass surgery significantly increases children's susceptibility to delirium. This highlights the need for heightened, targeted delirium screening in all pediatric cardiothoracic ICUs to potentially improve outcomes in this vulnerable patient population.

When Is It Better to Wait? Surgical Timing and Recurrence Risk for Children Undergoing Repair of Subaortic Stenosis.

Recurrence of subaortic stenosis (SAS) after surgery is common in children. The effects of patient characteristics and surgical timing on disease recurrence are largely unknown. We performed a retrospective study, assessing the relative effects of patient age and left ventricular outflow tract (LVOT) gradient on the need for reoperation for recurrent SAS. We included all children <20 years of age who underwent initial surgical resection of SAS at our center, January 2003-December 2013. Stratified logistic regression was performed, considering the effects of patient demographics, clinical characteristics, echocardiographic parameters, and operative technique, and clustering standard errors by surgeon. The multivariable model was used to simulate predicted probabilities of recurrent SAS for children at varying ages and baseline LVOT gradients. Sixty-three patients (38 males) underwent initial operation for SAS. Patients were followed for a median of 3.7 years (IQR 1.2-7.1). Twenty-one percent of patients (n = 13) underwent reoperation for SAS. Twelve were male. For every 10 mmHg increase in preoperative peak gradient in boys, the odds of reoperation for SAS doubled (OR 2.01, CI 1.5-2.72, p < 0.001), and for every additional 6 months of age, the odds of reoperation decreased by 14% (OR 0.86, CI 0.84-0.88, p < 0.001). Both younger age and higher preoperative outflow tract gradient are independently associated with risk of reoperation. Clinicians should consider the age and rate of LVOT gradient change-and not just the absolute gradient-in determining initial surgical timing.

Outcomes of Adult Patients With Congenital Heart Disease After Heart Transplantation: Impact of Disease Type, Previous Thoracic Surgeries, and Bystander Organ Dysfunction.

BACKGROUND: Adults with congenital heart disease (CHD) are at increased risk for adverse outcomes after heart transplantation (HT). However, small cohorts have constrained the identification of factors associated with poor prognosis. We hypothesized that number of sternotomies and bystander organ dysfunction would be associated with an increased risk for early death after HT. METHODS AND RESULTS: We performed a retrospective observational study of all adult CHD patients who underwent HT at our institution from January 1997 to January 2014. Forty-eight adult CHD patients were followed for a mean of 5 years. Diagnoses included tetralogy of Fallot/pulmonary atresia/double-outlet right ventricle in 15 (31%), D-transposition of the great arteries (TGA) in 10 (21%), tricuspid atresia/double-inlet left ventricle in 9 (19%), ventricular or atrial septal defect in 4 (8%), heterotaxy in 3 (6%), congenitally corrected TGA in 2 (4%), and other diagnoses in 5 (10%). Survival at both 1 and 5 years was 77%. According to multivariate analysis, ≥3 sternotomies (hazard ratio [HR] 8.5; P = .02) and Model for End-Stage Liver Disease Excluding International Normalized Ratio (MELD-XI) score >18 (HR 6.2; P = .01) were significant predictors of mortality. Failed Fontan surgery was not a significant predictor of death (P = .19). CONCLUSIONS: In our cohort of adult CHD patients undergoing HT, ≥3 sternotomies and MELD-XI score >18 were significantly associated with death. These findings may be important in patient selection and decision regarding tolerable number of CHD surgeries before considering HT.

Intervention for recoarctation in the single ventricle reconstruction trial: incidence, risk, and outcomes.

BACKGROUND: Recoarctation after the Norwood procedure increases risk for mortality. The Single Ventricle Reconstruction (SVR) trial randomized subjects with a single right ventricle undergoing a Norwood procedure to a modified Blalock-Taussig shunt or a right ventricle-pulmonary artery shunt. We sought to determine the incidence of recoarctation, risk factors, and outcomes in the SVR trial. METHODS AND RESULTS: Recoarctation was defined by intervention, either catheter based or surgical. Univariate analysis and multivariable Cox proportional hazard models were performed with adjustment for center. Of the 549 SVR subjects, 97 (18%) underwent 131 interventions (92 balloon aortoplasty, 39 surgical) for recoarctation at a median age of 4.9 months (range, 1.1-10.5 months). Intervention typically occurred at pre-stage II catheterization (n=71, 54%) or at stage II surgery (n=38, 29%). In multivariable analysis, recoarctation was associated with the shunt type in place at the end of the Norwood procedure (hazard ratio, 2.0 for right ventricle-pulmonary artery shunt versus modified Blalock-Taussig shunt; P=0.02), and Norwood discharge peak echo-Doppler arch gradient (hazard ratio, 1.07 per 1 mm Hg; P<0.01). Subjects with recoarctation demonstrated comorbidities at pre-stage II evaluation, including higher pulmonary arterial pressures (15.4±3.0 versus 14.5±3.5 mm Hg; P=0.05), higher pulmonary vascular resistance (2.6±1.6 versus 2.0±1.0 Wood units·m(2); P=0.04), and increased echocardiographic volumes (end-diastolic volume, 126±39 versus 112±33 mL/BSA(1.3), where BSA is body surface area; P=0.02). There was no difference in 12-month postrandomization transplantation-free survival between those with and without recoarctation (P=0.14). CONCLUSIONS: Recoarctation is common after Norwood and contributes to pre-stage II comorbidities. Although with intervention there is no associated increase in 1-year transplantation/mortality, further evaluation is warranted to evaluate the effects of associated morbidities.

Reoperations for pediatric and congenital heart disease: an analysis of the Society of Thoracic Surgeons (STS) congenital heart surgery database.

In this descriptive analysis, data in the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database pertaining to patients who underwent reoperative cardiac surgery were analyzed. Practice patterns and outcomes are described. Reoperative cardiac surgery for congenital heart disease is common, with one third of index operations in the database occurring subsequent to prior cardiothoracic operation(s) performed on cardiopulmonary bypass. This analysis suggests that a history of previous cardiac surgery does not independently confer a significant incremental risk of operative mortality, but that patients with greater number of previous operations appear to be at higher risk.

Patient and procedural characteristics for successful and failed immediate tracheal extubation in the operating room following cardiac surgery in infancy.

BACKGROUND: Immediate extubation in the operating room after congenital heart surgery is practiced with rising frequency at many cardiac institutions to decrease costs and complications. Infants less than one year of age are also increasingly selected for this 'fast track'. However, factors for patient selection, success, or failure of this practice have not been well defined in this population, yet are critical for patient safety. OBJECTIVE: To identify selection criteria, patient and procedural characteristics for successful or failed very early endotracheal extubation in the operating room immediately following infant heart surgery. METHODS: A retrospective analysis was performed for 326 consecutive patients undergoing neonatal and infant heart surgery from 2009 to 2012. Extubation and reintubation data were taken from the institutional Society of Thoracic Surgeons database and patients' charts. Patient characteristics were derived using multivariable logistic regression models. RESULTS: Very early extubation in the operating room was performed for 130 of 326 neonates and infants (40%). Weight >4 kg, lesser procedural complexity, and absence of trisomy 21 were identified as significant predictors for attempted very early extubation. Of these patients, 12% required reintubation within 48 h following surgery, predominantly due to respiratory failure or for mediastinal re-exploration. Greater procedural complexity was associated with failed extubations. Reintubation was associated with prolonged hospitalization. CONCLUSIONS: Extubation immediately after infant heart surgery in the operating room can be safely achieved. However, our data suggest that patients undergoing more complex procedures should be selected more conservatively for immediate early extubation.

Surgical volume-to-outcome relationship and monitoring of technical performance in pediatric cardiac surgery.

A significant inverse relationship of surgical institutional and surgeon volumes to outcome has been demonstrated in many high-stakes surgical specialties. By and large, the same results were found in pediatric cardiac surgery, for which a more thorough analysis has shown that this relationship depends on case complexity and type of surgical procedures. Lower-volume programs tend to underperform larger-volume programs as case complexity increases. High-volume pediatric cardiac surgeons also tend to have better results than low-volume surgeons, especially at the more complex end of the surgery spectrum (e.g., the Norwood procedure). Nevertheless, this trend for lower mortality rates at larger centers is not universal. All larger programs do not perform better than all smaller programs. Moreover, surgical volume seems to account for only a small proportion of the overall between-center variation in outcome. Intraoperative technical performance is one of the most important parts, if not the most important part, of the therapeutic process and a critical component of postoperative outcome. Thus, the use of center-specific, risk-adjusted outcome as a tool for quality assessment together with monitoring of technical performance using a specific score may be more reliable than relying on volume alone. However, the relationship between surgical volume and outcome in pediatric cardiac surgery is strong enough that it ought to support adapted and well-balanced health care strategies that take advantage of the positive influence that higher center and surgeon volumes have on outcome.

The American Association for Thoracic Surgery (AATS) 2024 expert consensus document: Management of neonates and infants with Ebstein anomaly.

OBJECTIVES: Symptomatic neonates and infants with Ebstein anomaly (EA) require complex management. A group of experts was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic focusing on risk stratification and management. METHODS: The EA Clinical Congenital Practice Standards Committee is a multinational and multidisciplinary group of surgeons and cardiologists with expertise in EA. A citation search in PubMed, Embase, Scopus, and Web of Science was performed using key words related to EA. The search was restricted to the English language and the year 2000 or later and yielded 455 results, of which 71 were related to neonates and infants. Expert consensus statements with class of recommendation and level of evidence were developed using a modified Delphi method, requiring 80% of members votes with at least 75% agreement on each statement. RESULTS: When evaluating fetuses with EA, those with severe cardiomegaly, retrograde or bidirectional shunt at the ductal level, pulmonary valve atresia, circular shunt, left ventricular dysfunction, or fetal hydrops should be considered high risk for intrauterine demise and postnatal morbidity and mortality. Neonates with EA and severe cardiomegaly, prematurity (<32 weeks), intrauterine growth restriction, pulmonary valve atresia, circular shunt, left ventricular dysfunction, or cardiogenic shock should be considered high risk for morbidity and mortality. Hemodynamically unstable neonates with a circular shunt should have emergent interruption of the circular shunt. Neonates in refractory cardiogenic shock may be palliated with the Starnes procedure. Children may be assessed for later biventricular repair after the Starnes procedure. Neonates without high-risk features of EA may be monitored for spontaneous closure of the patent ductus arteriosus (PDA). Hemodynamically stable neonates with significant pulmonary regurgitation at risk for circular shunt with normal right ventricular systolic pressure should have an attempt at medical closure of the PDA. A medical trial of PDA closure in neonates with functional pulmonary atresia and normal right ventricular systolic pressure (>20-25 mm Hg) should be performed. Neonates who are hemodynamically stable without pulmonary regurgitation but inadequate antegrade pulmonary blood flow may be considered for a PDA stent or systemic to pulmonary artery shunt. CONCLUSIONS: Risk stratification is essential in neonates and infants with EA. Palliative comfort care may be reasonable in neonates with associated risk factors that may include prematurity, genetic syndromes, other major medical comorbidities, ventricular dysfunction, or sepsis. Neonates who are unstable with a circular shunt should have emergent interruption of the circular shunt. Neonates who are unstable are most commonly palliated with the Starnes procedure. Neonates who are stable should undergo ductal closure. Neonates who are stable with inadequate pulmonary flow may have ductal stenting or a systemic-to-pulmonary artery shunt. Subsequent procedures after Starnes palliation include either single-ventricle palliation or biventricular repair strategies.

Upper body central venous catheters in pediatric cardiac surgery.

BACKGROUND: A central venous catheter located in the jugular or subclavian vein provides rapid, reliable vascular access for pediatric heart surgery. However, intravascular catheters are associated with vessel injury. Stenosis or thrombosis of central veins in the upper body can lead to 'superior vena cava syndrome' with markedly elevated venous pressures in the head and neck, causing facial swelling and headaches. This complication may be especially serious for patients with superior cavopulmonary (Glenn) or total cavopulmonary (Fontan) circulation. The authors hypothesized that upper body central line placement would be associated with a low risk of venous thrombosis or stenosis. METHODS: A three-year retrospective review of infant and univentricular cardiac procedures at a single institution was performed. Two hundred and thirty-five consecutive cardiac surgical patients <1 year of age or undergoing palliation for univentricular cardiac anatomy up to five years of age during January 2010 to December 2012 were included in this study. Upper body central lines are routinely placed by the anesthesiologist after induction of anesthesia for pediatric cardiac surgery at the study institution. The major exception is existing central venous access via an umbilical vein or femoral vein. Patients <2 years of age received a 4.0-French, 5-cm double-lumen central line [Cook Medical polyurethane, no antibiotic or heparin coating]. Those over two years of age received a 5.0-French, 8-cm triple lumen central line [Cook Medical polyurethane, no antibiotic or heparin coating]. A retrospective review of charts, hospital reports, echocardiographic studies, and cardiac catheterization studies was performed. RESULTS: The combined population of infants <1 year of age and patients <5 years of age with functional univentricular hearts totaled 235 patients who underwent 261 cardiac surgical operations. In this cohort of 261 cases, 171 size 4.0 or 5.0-French upper body central lines were inserted. A total of 158 right internal jugular vein catheters were placed. Two left internal jugular lines, two left subclavian lines, and nine right subclavian lines were placed in this population after failure to obtain right internal jugular access. Due to the small sample size (N = 13), the central lines not placed in the right internal jugular vein were excluded from further review. Two cases with right internal jugular venous lines were excluded due to death (without known stenosis or thrombosis) with the line in place. Twenty-three size 4.0- or 5.0-French right internal jugular central venous lines were placed in patients over one year of age (range 1.1-4.3 years) having modified Glenn- or Fontan-type surgery. The central lines were removed with a median of 1.4 days after insertion (range 0.7-8.2 days) for these older children, compared with a median of 4.2 days of age (range 0.3-19.3 days) for the 133 children <1 year of age. Retrospective chart review of nursing notes, progress notes, cardiology notes, discharge summaries, echocardiographic reports, and cardiac catheterization reports for all patients who received an upper body central venous line (internal jugular or subclavian) showed no definitive diagnosis of an upper body venous stenosis or thrombosis related to the central venous line. A further targeted review of echocardiographic and cardiac catheterization studies for univentricular cardiac patients failed to show stenosis or thrombosis of a vessel associated with upper body central line placement. CONCLUSIONS: This study describes one institution's experience with routine upper body central venous catheter placement for neonatal and infant cardiac surgery as well as univentricular cardiac palliation (Glenn and Fontan procedures) with minimal risk of clinically significant catheter-associated vessel thrombosis or stenosis. No upper body central venous stenosis or thrombosis was detected in association with perioperative catheter placement in the upper body central venous system, primarily the right internal jugular vein in 156 cases. Right internal jugular central line placement for infant cardiac surgery can be utilized with a low risk of direct venous thrombosis or stenosis.