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Neonates with symptomatic tetralogy of Fallot (sTOF) with hypoplastic pulmonary arteries (hPA) are considered high risk. Data are needed to inform the impact of hPA on outcomes, and the ideal management strategy. The objectives of this study were to quantify the impact of hPA on outcomes in neonates with sTOF and measure the impact of strategy on pulmonary artery (PA) growth in this population. Neonates with sTOF from 2005 to 2017 were reviewed from the Congenital Cardiac Research Collaborative. Criteria for hPA included a unilateral PA z score <−2.0 and contralateral PA z score <0. Primary outcome was mortality. Secondary outcomes included reintervention and PA growth. We included 542 neonates with sTOF, including 188 (35%) with hPA and 354 (65%) with normal PA, with median follow-up of 4.1 years. Median right and left hPA z scores were −2.19 (25th-75th percentile: −2.55 to −1.94) and −2.23 (25th-75th percentile: −2.64 to −1.91), respectively. Staged repair (vs primary TOF repair) was less common in the hPA cohort (36 vs 44%; P = 0.07). Survival was similar between groups (unadjusted P = 0.16; adjusted P = 0.25). Reintervention was more common in the hPA group (HR: 1.28; 95% CI: 1.01-1.63; P = 0.044); there was no difference after definitive repair (HR: 1.21; 95% CI: 0.93-1.58; P = 0.16). PA growth at 1 year was greater in the hPA cohort, particularly for the right PA (P < 0.001). Despite perception, the presence of hPA in neonates with sTOF conferred no increase in overall hazard of mortality or reintervention after definitive repair. PA growth was superior in the hPA cohort. These findings suggest that the presence of hPA does not adversely impact outcomes in sTOF. [Display omitted] [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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 Registrationhttps://clinicaltrials.gov/ct2/home NCT03406884, First posted January 23, 2018. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Recent data have demonstrated that overall mortality and adverse events are not significantly different for primary repair (PR) and staged repair (SR) approaches to management of neonates with symptomatic tetralogy of Fallot (sTOF). Cost data can be used to compare the relative value (cost for similar outcomes) of these approaches and are a potentially more sensitive measure of morbidity.Objectives: This study sought to compare the economic costs associated with PR and SR in neonates with sTOF.Methods: Data from a multicenter retrospective cohort study of neonates with sTOF were merged with administrative data to compare total costs and cost per day alive over the first 18 months of life in a propensity score-adjusted analysis. A secondary analysis evaluated differences in department-level costs.Results: In total, 324 subjects from 6 centers from January 2011 to November 2017 were studied (40% PR). The 18-month cumulative mortality (P = 0.18), procedural complications (P = 0.10), hospital complications (P = 0.94), and reinterventions (P = 0.22) did not differ between PR and SR. Total 18-month costs for PR (median $179,494 [IQR: $121,760-$310,721]) were less than for SR (median: $222,799 [IQR: $167,581-$327,113]) (P < 0.001). Cost per day alive (P = 0.005) and department-level costs were also all lower for PR. In propensity score-adjusted analyses, PR was associated with lower total cost (cost ratio: 0.73; P < 0.001) and lower department-level costs.Conclusions: In this multicenter study of neonates with sTOF, PR was associated with lower costs. Given similar overall mortality between treatment strategies, this finding suggests that PR provides superior value. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Neonates with tetralogy of Fallot and symptomatic cyanosis (sTOF) require early intervention.Objectives: This study sought to perform a balanced multicenter comparison of staged repair (SR) (initial palliation [IP] and subsequent complete repair [CR]) versus primary repair (PR) treatment strategies.Methods: Consecutive neonates with sTOF who underwent IP or PR at ≤30 days of age from 2005 to 2017 were retrospectively reviewed from the Congenital Cardiac Research Collaborative. The primary outcome was death. Secondary outcomes included component (IP, CR, PR) and cumulative (SR): hospital and intensive care unit lengths of stay; durations of cardiopulmonary bypass, anesthesia, ventilation, and inotrope use; and complication and reintervention rates. Outcomes were compared using propensity score adjustment.Results: The cohort consisted of 342 patients who underwent SR (IP: surgical, n = 256; transcatheter, n = 86) and 230 patients who underwent PR. Pre-procedural ventilation, prematurity, DiGeorge syndrome, and pulmonary atresia were more common in the SR group (p ≤0.01). The observed risk of death was not different between the groups (10.2% vs 7.4%; p = 0.25) at median 4.3 years. After adjustment, the hazard of death remained similar between groups (hazard ratio: 0.82; 95% confidence interval: 0.49 to 1.38; p = 0.456), but it favored SR during early follow-up (<4 months; p = 0.041). Secondary outcomes favored the SR group in component analysis, whereas they largely favored PR in cumulative analysis. Reintervention risk was higher in the SR group (p = 0.002).Conclusions: In this multicenter comparison of SR or PR for management of neonates with sTOF, adjusted for patient-related factors, early mortality and neonatal morbidity were lower in the SR group, but cumulative morbidity and reinterventions favored the PR group, findings suggesting potential benefits to each strategy. [ABSTRACT FROM AUTHOR]

Introduction: The Ross procedure remains the gold standard surgical therapy for severe aortic valve dysfunction in children and young adults. With the emergence of evolving surgical techniques for aortic valve disease (e.g. Ozaki reconstruction) and transcatheter-based valve technology, defining true long-term outcomes and surgical benchmarks for the Ross procedure remain critical for future comparisons. Methods: All patients undergoing the Ross procedure at a single institution for severe aortic valve dysfunction were evaluated over a 25-year study period (1993-2018). Patients were stratified into age categories: I (< 1y, 14%), II (1-10y, 26%), III (11-17y, 27%) and IV (>18y, 33%). Univariate and Kaplan-Meier analyses evaluated operative and long-term outcomes. Results: A total of 201 patients underwent the Ross (73%) +/- Konno (27%) procedure. Mean patient age was 16±14y (range <1-56y). Long-term follow-up was 100% (mean 5±7y, range <1-21y). Isolated aortic insufficiency (45%) was the most common surgical indication among all patients, while isolated aortic stenosis (52%) was most common in Age I. A pulmonary homograft RV-PA conduit was utilized for the majority (89%) of RVOT reconstructions. Overall mortality was 1.5% and was highest among infants (Age I: 7%). Importantly, Kaplan-Meier long-term survival was high across age groups: I (93% at 18 years), II (98% at 20 years), III (100% at 19 years), and IV (100% at 20 years). Freedom from autograft and RVOT reinterventions (Figure) were also lowest for infants, with strong durability of 10-15 years in older cohorts. Importantly, the overall incidence of moderate-severe neo-aortic insufficiency after Ross was 11% and not associated with underlying aortic valve pathology (P =0.25). Conclusions: Operative and long-term results for the Ross procedure remain promising with excellent survival and long-term durability of the neo-aortic autograft. Neonates and infants remain the highest risk cohort with the earliest need for RVOT re-intervention. Primary aortic valve pathology was not associated with development of autograft dysfunction after Ross. These long-term data provide critical benchmarks to which prosthetic AVR as well as future surgical and catheter-based therapy should be compared. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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·m²; P=0.04), and increased echocardiographic volumes (end-diastolic volume, 126±39 versus 112±33 mL/BSA1.3, 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. Clinical Trial Registration--URL: http://www.clinicaltrials.gov. Unique identifier: NCT00115934. [ABSTRACT FROM AUTHOR]

Background. Human cardiac-derived progenitor cells (hCPCs) have shown promise in treating heart failure (HF) in adults. The purpose of this study was to describe derivation of hCPCs from pediatric patients with end-stage HF. Methods. At surgery, discarded right atrial tissues (hAA) were obtained from HF patients (n = 25; hAA-CHF). Minced tissues were suspended in complete (serum-containing) DMEM. Cells were selected for their tissue migration and expression of stem cell factor receptor (hc-kit). Characterization of hc-kitpositive cells included immunohistochemical screening with a panel of monoclonal antibodies. Results. Cells, including phase-bright cells identified as hc-kitpositive, spontaneously emigrated fromhAA-CHF in suspended explant cultures (SEC) after Day 7. When cocultured with tissue, emigrated hc-kitpositive cells proliferated, first as loosely attached clones and later as multicellular clusters. At Day 21∼5% of cells were hc-kitpositive. Between Days 14 and 28 hc-kitpositive cells exhibited mesodermal commitment (GATA-4positive and NKX2.5positive); then after Day 28 cardiac lineages (flk-1positive, smooth muscle actinpositive, troponin-Ipositive, and myosin light chainpositive). Conclusions. C-kitpositive hCPCs can be derived from atrial tissue of pediatric patients with end-stage HF. SEC is a novel culture method for derivation ofmigratory hc-kitpositive cells that favors clinical translation by reducing the need for exogenously added factors to expand hCPCs in vitro. [ABSTRACT FROM AUTHOR]