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Background: As the life expectancy of patients with Down syndrome (DS) improves, the number of older patients with DS who require a cardiac surgical procedure for congenital heart disease will increase. Perioperative risk factors and outcomes in these patients are unknown., Methods: In a multicenter retrospective study, teenaged and adult patients with DS who underwent a cardiac surgical procedure between 2008 and 2018 were matched by age and surgical procedure with patients who did not have DS. Demographic, medical, and surgical characteristics were compared. Outcome measures were length of stay (LOS), duration of mechanical ventilation, need for noninvasive positive pressure ventilation and reintubation, additional cardiac interventions, postoperative infections, and early postoperative mortality. Risk factors for extended hospital LOS (>10 days) were explored using multivariable logistic regression., Results: The study compared 121 patients with DS with 121 patients who did not have DS. Patients with DS had a longer median LOS (7 days vs 5 days; P < .001), a longer duration of mechanical ventilation (12.5 hours vs 6.7 hours; P < .001), greater need for noninvasive positive pressure ventilation or reintubation (26% vs 4%; P < .001), and a higher likelihood of postoperative infections (10% vs 2%; P = .035). There was no early mortality. Preoperative risk factors for extended LOS for patients with DS included pulmonary medication use (odds ratio [OR], 4.0; P = .046), a history of immunodeficiency (OR, 10.4; P = .004), or moderate or greater tricuspid regurgitation (OR, 12.7; P < .001)., Conclusions: Teenaged and adult patients with DS who underwent congenital a cardiac surgical procedure had a longer hospital LOS and more postoperative respiratory and infectious complications compared with patients who did not have DS, without increased mortality. A cardiac surgical procedure can be performed safely in older patients with DS. Management of pulmonary disease, immunodeficiency, and tricuspid regurgitation may mitigate risk., Competing Interests: Disclosures The authors have no conflicts of interest to disclose., (Copyright © 2024 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Infants with complete heart block (CHB) require epicardial pacemaker (PM) insertion. Prior studies described epicardial pacing outcomes in infants and children, although they were limited by small or heterogeneous populations., Objective: This study aimed to explore patient- and procedure-level associations with device complications in infants with CHB who received a permanent PM., Methods: This was a multicenter, retrospective cohort study including infants receiving an epicardial PM between 2000 and 2021 for CHB. The primary outcome was time to device-related adverse event: lead failure requiring revision; pocket infection; exit block requiring increased pacing output; or lead-related coronary artery compression. Time-to-event analysis was performed by the Kaplan-Meier method with a multivariable Cox proportional hazards model., Results: There were 174 infants who received an epicardial PM (282 bipolar, 39 unipolar leads) for CHB. Median age and weight at PM were 93.5 days and 4.5 kg, respectively. Pacing indication was postoperative CHB in 63% and congenital CHB in 37%. The median follow-up was 2.1 years. The primary outcome occurred in 26 infants at a median time to event of 0.6 year. Age ≤90 days at PM implantation was the most significant risk factor for a device-related adverse event (hazard ratio, 7.02; P < .001), primarily driven by pocket infections. Lead failure occurred in 3% of leads with a 5- and 10-year freedom from failure of 93% and 83%, respectively., Conclusion: Device complications affect 15% of infants receiving a permanent PM for heart block. Age ≤90 days at PM implantation is especially associated with infectious complications. Epicardial lead durability appears similar to previously reported pediatric experiences., Competing Interests: Disclosures The authors have no conflicts of interest to disclose., (Copyright © 2024 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Many patients with repaired tetralogy of Fallot require pulmonary valve replacement (PVR) due to significant pulmonary regurgitation (PR). Transcatheter PVR (TPVR) is an equally effective and less invasive alternative to surgical PVR but many native right ventricular outflow tracts (RVOTs) are too large for TPVR at time of referral. Understanding the rate of growth of the RVOT may help optimize timing of referral. This study aims to examine the longitudinal growth of the native RVOT over time in repaired tetralogy of Fallot (TOF)., Methods: A retrospective review of serial cardiac MRI cardiovascular magnetic resonance (CMR) data from 121 patients with repaired TOF and a native RVOT (median age at first CMR 14.7 years, average interval between the first and last CMR of 8.1 years) was performed to measure serial changes in RVOT diameter, cross-sectional area, perimeter-derived diameter, and length., Results: All parameters of RVOT size continued to grow with increasing age but growth was more rapid in the decade after TOF repair (for minimum systolic diameter, mean increase of 5.7 mm per 10 years up to year 12, subsequently 2.3 mm per 10 years). The RVOT was larger with a transannular patch and in patients without pulmonary stenosis (p < 0.001 for both), but this was not associated with rate of growth. More rapid RVOT enlargement was noted in patients with larger right ventricular end-diastolic volume (RVEDV), higher PR fraction, and greater rates of increases in RVEDV and PR (p < 0.001 for all) CONCLUSIONS: in patients with repaired TOF, using serial CMR data, we found that RVOT size increased progressively at all ages, but the rate was more rapid in the first decade after repair. More rapid RVOT enlargement was noted in patients with a larger RV, more PR, and greater rates of increases in RV size and PR severity. These results may be important in considering timing of referral for transcatheter pulmonary valves, in planning transcatheter and surgical valve replacement, and in designing future valves for the native RVOT., Competing Interests: Declaration of Competing Interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests. Ashwin Prakash reports a relationship with Moderna Inc that includes: equity or stocks. If there are other authors, they declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Pediatric heart transplant (HT) candidates experience high waitlist mortality due to a limited donor pool that is constrained in part by anti-HLA sensitization. We evaluated the impact of CDC and Flow donor-specific crossmatch (XM) results on pediatric HT outcomes., Methods: All pediatric HTs between 1999 and 2019 in the OPTN database were included. Donor-specific XM results were sub-categorized based on CDC and Flow results. Primary outcomes were treated rejection in the first year and time to death or allograft loss. Propensity scores were utilized to adjust for differences in baseline characteristics., Results: A total of 4,695 pediatric HT patients with T-cell XM data were included. After propensity score adjustment, a positive T-cell CDC-XM was associated with 2 times higher odds of treated rejection (OR 2.29 (1.56, 3.37)) and shorter time to death/allograft loss (HR 1.50 (1.19, 1.88)) compared to a negative Flow-XM. HT recipients who were Flow-XM positive with negative/unknown CDC-XM did not have higher odds of rejection or shorter time to death/allograft loss. An isolated positive B-cell XM was also not associated with worse outcomes. Over the study period XM testing shifted from CDC- to Flow-based assays., Conclusions: A positive donor-specific T-cell CDC-XM was associated with rejection and death/allograft loss following pediatric HT. This association was not observed with a positive T-cell Flow-XM or B-cell XM result alone. The shift away from performing the CDC-XM may result in loss of important prognostic information unless the clinical relevance of quantitative Flow-XM results on heart transplant outcomes is systematically studied., (Copyright © 2024 International Society for the Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Background: Ventricular pacing can cause myocardial dysfunction, but how lead anchoring to the myocardium affects function has not been studied., Objective: The purpose of this study was to evaluate patterns of regional and global ventricular function in patients with a ventricular lead using cine cardiac computed tomography (CCT) and histology., Methods: This was a single-center retrospective study with 2 groups of patients with a ventricular lead: (1) those who underwent cine CCT from September 2020 to June 2021 and (2) those whose cardiac specimen was analyzed histologically. Regional wall motion abnormalities on CCT were assessed in relation to lead characteristics., Results: For the CCT group, 122 ventricular lead insertion sites were analyzed in 43 patients (47% female; median age 19 years; range 3-57 years). Regional wall motion abnormalities were present at 51 of 122 lead insertion sites (42%) in 23 of 43 patients (53%). The prevalence of a lead insertion-associated regional wall motion abnormality was higher with active pacing (55% vs 18%; P < .001). Patients with lead insertion-associated regional wall motion abnormalities had a lower systemic ventricular ejection fraction (median 38% vs 53%; P < .001) than did those without regional wall motion abnormalities. For the histology group, 3 patients with 10 epicardial lead insertion sites were studied. Myocardial compression, fibrosis, and calcifications were commonly present directly under active leads., Conclusion: Lead insertion site-associated regional wall motion abnormalities are common and associated with systemic ventricular dysfunction. Histopathological alterations including myocardial compression, fibrosis, and calcifications beneath active leads may explain this finding., (Copyright © 2023 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Outcomes after total anomalous pulmonary venous connection (TAPVC) repair remain suboptimal due to recurrent pulmonary vein (PV) obstruction requiring reinterventions. We sought to develop a clinical prediction rule for PV reintervention after TAPVC repair., Methods: Data from consecutive patients who underwent TAPVC repair at a single institution from January 1980 to January 2020 were retrospectively reviewed after Institutional Review Board approval. The primary outcome was postdischarge (late) unplanned PV surgical or transcatheter reintervention. Echocardiographic criteria were used to assess PV residual lesion severity at discharge (class 1: no residua; class 2: minor residua; class 3: major residua). Competing risk models were used to develop a weighted risk score for late reintervention., Results: Of 437 patients who met entry criteria, there were 81 (18.5%) reinterventions at a median follow-up of 15.6 (interquartile range, 5.5-22.2) years. On univariable analysis, minor and major PV residua, age, single-ventricle physiology, infracardiac and mixed TAPVC, and preoperative obstruction were associated with late reintervention (all P < .05). The final risk prediction model included PV residua (class 2: subdistribution hazard ratio [SHR], 4.8; 95% CI, 2.8-8.1; P < .001; class 3: SHR, 6.4; 95% CI, 3.5-11.7; P < .001), age <1 year (SHR, 3.3; 95% CI, 1.3-8.5; P = .014), and preoperative obstruction (SHR, 1.8; 95% CI, 1.1-2.8; P = .015). A risk score comprising PV residua (class 2 or 3: 3 points), age (neonate or infant: 2 points), and obstruction (1 point) was formulated. Higher risk scores were significantly associated with worse freedom from reintervention (P < .001)., Conclusions: A risk prediction model of late reintervention may guide prognostication of high-risk patients after TAPVC repair., (Copyright © 2023 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The natural history of the dilated truncal root in repaired truncus arteriosus (TA) is incompletely understood., Methods: A single-center review of patients who underwent TA repair between January 1984 and December 2018 was performed. Echocardiographically determined root diameters and derived z scores were measured at the annulus, sinus of Valsalva (SoV), and sinutubular junction (STJ) immediately before TA repair and throughout follow-up. Linear mixed-effects models assessed trends in root dimensions over time., Results: Of 193 patients who underwent TA repair at a median age of 12 days (interquartile range, 6-48 days) and survived to discharge, 34 (17.6%), 110 (57.0%), and 49 (25.4%) patients had bicuspid, tricuspid, and quadricuspid truncal valves, respectively. Median postoperative follow-up was 11.6 years (interquartile range, 4.4-22.0 years; range, 0.1-34.8 years). Truncal valve or root intervention was required in 38 patients (19.7%). The mean rates of annular, SoV, and STJ growth were 0.7 ± 0.3 mm/y, 0.8 ± 0.5 mm/y, and 0.9 ± 0.4 mm/y, respectively. Root z scores remained stable with time. At baseline, compared with patients with tricuspid leaflet anatomy, bicuspid patients had larger diameters at the SoV (P = .003) and STJ (P = .029), whereas quadricuspid patients had larger STJ diameters (P = .004). Over time, the bicuspid and quadricuspid cohorts demonstrated comparatively greater annular dilatation (both P < .05). Patients with ≥75th percentile root growth rates had a higher incidence of moderate-severe truncal regurgitation (P = .019) and truncal valve intervention (P = .002)., Conclusions: Root dilatation in TA persisted for up to 30 years after primary repair. Patients with bicuspid and quadricuspid truncal valves demonstrated greater root dilatation over time and required more valve interventions. Continued longitudinal follow-up is warranted in this higher-risk cohort., (Copyright © 2023 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Early postoperative catheterizations (EPOCs) within 6 weeks after a congenital heart surgical procedure can treat residual lesions and provide important clinical information. However, EPOCs are often assumed to impose additional risk on a vulnerable patient population. This study aimed to describe the EPOC population, evaluate procedural safety, compare EPOC patients with procedure-matched non-EPOC patients, and determine risk factors for poor outcomes using data from the Congenital Cardiac Catheterization Project on Outcomes registry., Methods: In a retrospective cohort, demographic, clinical, and procedural characteristics were analyzed for diagnostic and interventional catheterizations performed in 13 participating institutions from January 2014 to December 2017, excluding patients after heart transplant. The primary outcome was a high-severity adverse event (AE). Three distinct analyses included (1) describing the full cohort and EPOC patients, (2) comparing EPOC patients with and without a high-severity AE, and (3) comparing EPOC patients with controls matched on case type., Results: This study included 17,776 catheterizations, with 1399 EPOCs. The high-severity AE rate was 6.4% overall, 8.9% in the EPOC cohort, and 8.4% in matched controls (P = .74). The association between EPOC status and high-severity AE was not significant in a multivariable model (P = .17). In EPOCs with a high-severity AE, median procedure duration was 30 minutes longer (P < .001), and median time from surgical procedure to catheterization was 3 days longer (P = .05)., Conclusions: EPOC was not associated with additional risk. Individual patient characteristics of size and hemodynamic vulnerability may serve as informative predictors. Timely catheterization may preempt further clinical deterioration, and intraprocedure duration optimization may correlate with improved outcomes., (Copyright © 2023 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Direct oral anticoagulants use in pediatric cardiology is poorly defined., Objective: We present the largest experience of apixaban use in children with heart disease, using weight- and level-based dosing., Methods: Retrospective single-center analysis of cardiac patients ≤19 years treated with apixaban. Patients were evaluated for safety (clinically relevant non-major [CRNM] or major bleeding; thrombotic events) and effectiveness (thrombus improvement by imaging). Peak drug-specific anti-Xa chromogenic assay results ("apixaban levels") were analyzed., Results: Over 3 years (5/2018-9/2021), 219 children, median age 6.8 years (0.3-19), median weight 20.8 kg (4.8-160) received apixaban, totaling 50,916 patient days. Of them, 172 (79%) warranted thromboprophylaxis and 47 (21%) thrombosis treatment (with 10 arterial, 19 venous, 15 intracardiac, and 3 pulmonary). The median initial peak apixaban level was 165 ng/mL (23-474; n = 125) in the prophylaxis subgroup and 153 ng/mL (30-450; n = 33) in the treatment subgroup; dosage was adjusted in response to levels in 25% of the patients. There were 4 bleeding safety events (3 CRNM; 1 major, hemoptysis complicating empyema); the serious bleeding event rate was 2.9 per 100 patient-years of apixaban. Minor bleeding events (42) were noted in 18 patients, with an additional 2 having leukopenia, 1 transaminitis, and 3 rashes. An improvement in thrombosis was seen in 95% of the treated patients with available follow-up imaging (37/39 patients)., Conclusion: Apixaban use was feasible with a low rate of adverse events across a diverse pediatric cardiac population using commercially available tablets dosed to weight and adjusted based on peak apixaban levels., (Copyright © 2023 International Society on Thrombosis and Haemostasis. Published by Elsevier Inc. All rights reserved.)

Background: The utility of the intraoperative technical performance score (IO-TPS) in predicting outcomes after congenital cardiac surgery remains unknown., Methods: Data from patients undergoing surgery for congenital heart disease from January 2011 to December 2019 at a single institution were retrospectively reviewed. Intraoperative echocardiograms were used to assign IO-TPS for each index operation (class 1, no residua; class 2, minor residua; class 3, major residua). The primary outcome was a composite of in-hospital mortality, transplant, unplanned reintervention in the anatomic area of repair, and new permanent pacemaker implantation. Secondary outcomes included postdischarge (late) mortality or transplant and unplanned reintervention. Associations between IO-TPS and outcomes were assessed using logistic (primary) and Cox or competing risk (secondary) models, adjusting for preoperative patient- and procedure-related covariates., Results: The primary outcome was observed in 784 (11.5%) of 6793 patients who met entry criteria. On multivariable analysis, IO-TPS was a significant predictor of the primary outcome (class 2: odds ratio, 1.7 [95% CI, 1.4-2.0; P < .001]; class 3: odds ratio, 6.0 [95% CI, 4.0-8.9; P < .001]). Among 6661 transplant-free survivors of hospital discharge observed for up to 10.5 years, there were 185 (2.8%) deaths or transplants and 1171 (17.6%) reinterventions. Class 3 patients had a greater adjusted risk of late mortality or transplant (hazard ratio, 2.2; 95% CI, 1.2-4.2; P = .012) and late reintervention (subdistribution hazard ratio, 2.5; 95% CI, 1.8-3.3; P < .001) vs class 1 patients., Conclusions: IO-TPS is significantly associated with adverse early and late outcomes after congenital heart surgery and may serve as an important adjunct for self-assessment and quality improvement., (Copyright © 2023 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: We sought to validate the technical performance score (TPS) as a predictor of midterm outcomes after congenital aortic valve repair., Methods: This was a single-center, retrospective review of consecutive patients who underwent aortic valve repair between January 1, 2011, and December 31, 2019. Predischarge echocardiograms were used to assign a TPS for each index operation as class 1, no aortic valve residua; class 2, minor aortic valve residua; or class 3, major aortic valve residua or predischarge reintervention for major residua. The primary outcome was postdischarge (late) unplanned aortic valve reintervention. Secondary outcomes included late mortality and at least moderate aortic regurgitation or stenosis at the latest follow-up or before the earliest reintervention. Associations between TPS and outcomes were assessed using competing risk, Cox proportional hazards, or logistic regression models, adjusting for preoperative patient- and procedure-related covariates., Results: Of 507 patients, there were 110 (21.7%) reinterventions, 22 (4.3%) deaths, and 67 (13.2%) cases of at least moderate aortic regurgitation or stenosis at the latest follow-up or earliest reintervention. On multivariable analysis, class 3 patients had a greater risk of reintervention (subdistribution hazard ratio, 2.6; 95% CI, 1.3-5.1; P = .005) and mortality (hazard ratio, 5.3; 95% CI. 1.1-25.2; P = .038) compared with class 1 patients. Adjusting for duration of follow-up, class 3 patients also had a greater risk of at least moderate aortic regurgitation or stenosis at the latest follow-up or earliest reintervention (odds ratio, 7.7; 95% CI, 2.5-24.2; P < .001) vs class 1 patients., Conclusions: Patients with major residua after congenital aortic valve repair have significantly worse midterm outcomes compared with those with no residua, warranting closer follow-up., (Copyright © 2023 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: While the predischarge technical performance score (DC-TPS) is significantly associated with outcomes after congenital cardiac surgery, the utility of the intraoperative TPS (IO-TPS) remains unknown., Methods: This was a single-center retrospective review of consecutive patients who underwent congenital cardiac surgery from January 2011 to December 2019. Intraoperative and predischarge echocardiograms were used to assign IO-TPS and DC-TPS, respectively, for each index operation (class 1, no residua; class 2, minor residua; class 3, major residua). Anatomic modules identifying the principal residual lesion were assigned to all class 2/3 patients. Overall and module-specific TPS comparisons were made. Multivariable regression models with IO-TPS and DC-TPS as separate predictors of postoperative outcomes were compared., Results: Of 6201 patients, overall agreement between IO-TPS and DC-TPS was observed in 4251 patients (68.6%); scores were likelier to be worse at discharge (P < .001). Paired comparative analyses revealed that among patients with at least class 2 atrioventricular and semilunar valve residua, IO-TPS was likelier to worsen than improve (both P < .001). Class 3 patients had a higher risk of in-hospital/early mortality (IO-TPS: odds ratio, 7.5; 95% CI, 2.4-23; DC-TPS: odds ratio, 6.6; 95% CI, 3.0-15), postdischarge/late mortality (IO-TPS: hazard ratio [HR], 3.1, 95% CI, 1.3-7.1; DC-TPS: HR, 2.3; 95% CI, 1.2-4.4), and late unplanned reintervention (IO-TPS: HR, 2.8; 95% CI, 1.9-4.0; DC-TPS: HR, 3.4; 95% CI, 2.8-4.2) vs class 1 (all P < .05). IO- and DC-TPS models were equivalent fits for predicting early and late mortality; the latter was a marginally better fit for late reintervention., Conclusions: IO-TPS and DC-TPS are both important adjuncts for quality improvement in congenital cardiac surgery., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: In patients with congenitally corrected transposition of the great arteries (ccTGA) with an intact ventricular septum (IVS) or d-looped transposition of the great arteries (DTGA) with IVS after an atrial switch operation, left ventricular dysfunction often develops after anatomic repair despite previous retraining of the morphologically left ventricle (mLV) by using pulmonary artery banding (PAB). This study examined histopathologic changes in such mLVs., Methods: Capillary density, myocyte diameter, and interstitial fibrous area in the mLV were retrospectively evaluated in postmortem or explanted heart specimens obtained from patients with ccTGA with IVS or DTGA with IVS after atrial switch operations after PAB for retraining and were compared with those of patients with normal cardiac anatomy, ccTGA with IVS or DTGA with IVS without PAB, and ccTGA or DTGA with high mLV pressure by using generalized estimating equations models., Results: Adjusting for age, capillary density in 4 patients with ccTGA with IVS or DTGA with IVS after PAB was approximately 20% lower than that in 8 patients with normal cardiac anatomy (3149 ± 863/μm 2 vs 3978 ± 1206/μm 2 [mean, SD]; P = .039), whereas myocyte diameter was approximately 50% larger (16.2 ± 4.0 μm vs 11.7 ± 2.4 μm [mean, SD]; P < .001). The interstitial fibrous area did not differ between the 2 groups (803 ± 422 μm 2 vs 789 ± 480 μm 2 ; P = .92)., Conclusions: The study investigators observed significant cardiomyocyte hypertrophy but lower capillary density in patients with ccTGA with IVS or DTGA with IVS after PAB for retraining compared with normal control subjects. This finding suggests that inadequate capillary growth is a potential pathologic basis for mLV dysfunction occurring after retraining or anatomic repair., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: American Heart Association (AHA) guidelines for management of Kawasaki disease (KD) rely on coronary artery (CA) z-scores from echocardiograms. Compared with echocardiography, cardiac CT (CCT) offers better visualization of distal segments and evaluation for thrombosis and stenosis. Despite increasing use of CCT in KD, CA z-scores for CCT are not available and measurement concordance between imaging modalities is a critical knowledge gap., Methods: We retrospectively reviewed KD patients with CA aneurysms who had concurrent echocardiography and CCT between 2016 and 2020. Patients were included if they had history of CA z-scores of ≥3 on echocardiography during their clinical course. Agreement between CCT and echocardiography was assessed using Bland-Altman analysis., Results: Paired CCT and echocardiography studies were available in 18 patients (21 studies). The largest CA aneurysms were large/giant (z-score ≥10) in 14 studies, medium (z-score ≥5, <10) in 3 studies, and small (z score ≥2.5, <5) in 2 studies. Intra- and inter-observer reliability for CCT measurements were high for all CA segments (ICC 99.7% and 98.6%). For the LMCA, proximal LAD and proximal and distal RCA there was high correlation between echocardiogram and CCT absolute measurements with wider variation between modalities for the distal LAD and circumflex. Overall, CCT measurements tended to be smaller than echocardiogram measurements, and led to a lower AHA z-score risk classification in 24% of studies., Conclusion: CCT and echocardiography have high agreement for absolute measurements of proximal CA segments, but more measurement discrepancy exists for distal CA segments with bias toward lower dimensions on CCT., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2021 Society of Cardiovascular Computed Tomography. Published by Elsevier Inc. All rights reserved.)

Background: Transvenous permanent pacemaker (PPM) implantation is an available option for Fontan patients with sinus node dysfunction. However, the thrombogenic potential of leads within the Fontan baffle is unknown., Objective: The purpose of this study was to compare the clot burden in Fontan patients with a transvenous atrial PPM to those without a PPM and those with an epicardial PPM., Methods: This was a retrospective cohort study of all transvenous PPM implantations in Fontan patients followed at our institution (2000-2018). We performed frequency matching on Fontan type and age group. Primary outcome was identification of intracardiac clot, pulmonary embolus, or embolic stroke., Results: Of 1920 Fontan patients, 58 patients (median age 23 years; interquartile range [25th-75th percentiles] 14-33) at the time of transvenous PPM implantation and 174 matched subjects formed our cohort. The type of Fontan performed in case subjects was right atrium-pulmonary artery or right atrium-right ventricle conduit (54%), lateral tunnel (43%), and extracardiac (3%). The cumulative incidence of clot was highest in patients with transvenous PPM, followed by patients with epicardial PPM and no PPM (1.2 vs 0.87 vs 0.67 per 100 person-years of follow-up, respectively). In multivariable analysis, anticoagulation and/or antiplatelet therapy were protective against clot and resulted in reduction of clot risk by 3-fold (incidence rate ratio 0.33; 95% confidence interval 0.21-0.53; P <.001)., Conclusion: In a large cohort of Fontan patients matched for age and Fontan type, patients with transvenous PPM had a higher but not statistically significant incidence of clot compared to those with no PPM and epicardial PPM. Patients treated with warfarin/aspirin had lower clot risk., (Copyright © 2021 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Catheter ablation of accessory pathways (APs) in Ebstein anomaly (EA) has been associated with a high recurrence risk., Objective: The purpose of this study was to compare outcomes of AP ablation in EA in an early (1990-2004) vs a recent (2005-2019) era and identify variables associated with recurrence., Methods: A retrospective review of all catheter ablations for supraventricular tachycardia in EA at our institution was performed., Results: We identified 76 patients with median (25th-75th quartiles) age 9 (2.6-13.3) years. Of these patients, 52 had AP alone, 12 had atrial flutter, 3 had atrioventricular nodal reentrant tachycardia, and 9 had AP plus at least 1 additional arrhythmia. Of the 61 patients with APs, a total of 78 separate APs were identified: 40 right-sided, 37 septal, and 1 left-sided. Acute success for AP first procedure was 89% and did not differ between early and recent eras (89% vs 88%; P = .48). However, 19 patients (31%) required repeat procedures (average 1.4 per patient) due to AP recurrence or ablation failure at first attempt. In comparison to early era, recent era ablations had significantly lower recurrence rates at 1 year (62% vs 19%; P = .005). At median follow-up of 2.5 (0.2-7) years, ultimate AP elimination after all procedures was 93%. Younger age at time of electrophysiological study (<2 vs 12-47 years: hazard ratio [HR] 7.3; P = .003) and ablation era (early era vs recent era: HR 3.65; P = .009) predicted recurrence., Conclusion: Outcomes for AP ablation in patients with EA have improved, but there is still a relatedly high recurrence risk requiring repeat procedures., (Copyright © 2021 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: The Technical Performance Score (TPS) can predict outcomes after congenital cardiac surgery. We sought to validate TPS as a predictor of both short- and long-term outcomes of the Norwood procedure., Methods: We conducted a retrospective review of patients who underwent the Norwood procedure from 1997 to 2017. We assigned TPS (class 1, no residua; class 2, minor residua; class 3, major residua or reintervention for major residua before discharge) based on subcomponent scores from discharge echocardiograms or unplanned reinterventions, or both. Multivariable Cox or competing risk analysis, adjusted for preoperative patient- and procedure-related covariates, examined the association of TPS with postoperative hospital length of stay, transplant-free survival, and postdischarge reinterventions., Results: Among 500 patients, 319 (64%) were male, 54 (11%) were premature, 56 (11%) had noncardiac anomalies/syndromes, 146 (29%) had preoperative risk factors, and 480 (96%) were assigned TPS. On multivariable analysis, class 3 had greater hazard for reinterventions in transplant-free survivors (class 3: subdistribution hazard ratio [HR], 2.06; 95% confidence interval [CI] 1.34-3.16; P = .001) and was associated with increased hospital length of stay vs class 1 (HR, 0.25; 95% CI, 0.18-0.34; P < .001). Transplant-free survival after Norwood surgery was shorter for both class 2 (HR, 2.48; 95% CI, 1.68-3.66; P < .001) and class 3 (HR, 3.29; 95% CI, 2.18-4.95; P < .001)., Conclusions: TPS predicts early and late outcomes after Norwood. Absence of residual lesions results in improved long-term prognosis for single-ventricle patients. TPS may improve outcomes after Norwood by identifying patients warranting closer follow-up and potentially earlier reintervention., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Several factors predict reintervention for subaortic stenosis (SubAS): age, preoperative left ventricular outflow tract gradient, distance from the obstructive subaortic ridge to the aortic valve, and peeling of membrane from the aortic/mitral valves. We sought to develop a prediction rule to categorize risk of reintervention for recurrent SubAS and guide follow-up in patients with discrete SubAS., Methods: We retrospectively reviewed patients who underwent SubAS resection between 1984 and 2016. Our primary outcome was reintervention for recurrent SubAS after discharge. Kaplan-Meier estimates were used for time-to-event analysis of any reintervention. Multivariable models were used to create a prediction rule. We excluded patients without 3 years of follow-up., Results: Of 172 patients, 21 (12.2%) required reintervention. The characteristics predicting reintervention were age younger than 2 years (P < .001), preoperative left ventricular outflow tract gradient of 65 mm Hg or more (P = .011), peeling of membrane from the mitral valve (P < .001), distance from the membrane to the aortic valve of less than 5 mm (P < .001), prior complex operation (P = .035), other left-sided heart lesions (P = .008), and aortic annulus z-score of -2.5 or less (P < .001). Our final prediction rule includes age, membrane to aortic valve distance, and other left-sided heart lesions each scored as 1 point. For patients with a score of 1 or less, 4% required a reintervention compared with 34% with a score of 2 or more., Conclusions: A prediction rule that incorporates the patient's age at the index operation, membrane to aortic valve distance, and associated left-sided heart lesions can determine the likelihood of reintervention for recurrent SubAS., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Outcomes after the arterial switch operation (ASO) for dextro-transposition of the great arteries have improved significantly since its inception in the 1980s. This study reviews contemporaneous outcomes and predictors for late reinterventions after ASO., Methods: We retrospectively reviewed patients who underwent ASO for dextro-transposition of the great arteries from 1997 to 2017. Technical performance score (TPS) class (class 1, trivial or no residua; class 2, minor residua; class 3, major residua or reintervention) was assigned at discharge based on echocardiographic evaluation of components of the ASO. Multivariable Cox regression identified patient- and procedure-specific factors associated with postdischarge reinterventions., Results: Among 598 patients, 410 (69%) underwent ASO and 188 (31%) underwent ASO with ventricular septal defect repair. Median age at surgery was 5 days (interquartile range, 3 to 7); median follow-up time was 8.2 years; 408 (68%) were male; 50 (8.3%) were premature; and 10 (1.7%) had noncardiac anomalies or syndromes. Survival to hospital discharge was 98% (n = 591). Among 349 patients with follow-up, freedom from unplanned reintervent2ion at 5 years was 99% for TPS class 1, compared with 84% for class 2 and 30% for class 3. On multivariable Cox regression, classes 2 and 3 had significantly higher hazard for reintervention (class 2 hazard ratio 10.6; 95% confidence interval, 2.5 to 44.2; P = .001; class 3 hazard ratio 58.2, 95% confidence interval, 13.1 to 259; P < .001)., Conclusions: At our center, ASO was associated with relatively low mortality. Class 2 and class 3 TPS were the most important independent predictors of reinterventions after discharge. Therefore, TPS can serve as a tool for identifying high-risk patients who warrant closer follow-up., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The aim of this study was to evaluate early and mid-term outcomes (mortality and prosthetic valve reintervention) after mitral valve replacement with 15- to 17-mm mechanical prostheses., Methods: A multicenter, retrospective cohort study was performed among patients who underwent mitral valve replacement with a 15- to 17-mm mechanical prosthesis at 6 congenital cardiac centers: 5 in The Netherlands and 1 in the United States. Baseline, operative, and follow-up data were evaluated., Results: Mitral valve replacement was performed in 61 infants (15 mm, n = 17 [28%]; 16 mm, n = 18 [29%]; 17 mm, n = 26 [43%]), of whom 27 (47%) were admitted to the intensive care unit before surgery and 22 (39%) required ventilator support. Median age at surgery was 5.9 months (interquartile range [IQR] 3.2-17.4), and median weight was 5.7 kg (IQR, 4.5-8.8). There were 13 in-hospital deaths (21%) and 8 late deaths (17%, among 48 hospital survivors). Major adverse events occurred in 34 (56%). Median follow-up was 4.0 years (IQR, 0.4-12.5) First prosthetic valve replacement (n = 27 [44%]) occurred at a median of 3.7 years (IQR, 1.9-6.8). Prosthetic valve endocarditis was not reported, and there was no mortality related to prosthesis replacement. Other reinterventions included permanent pacemaker implantation (n = 9 [15%]), subaortic stenosis resection (n = 4 [7%]), aortic valve repair (n = 3 [5%], and aortic valve replacement (n = 6 [10%])., Conclusions: Mitral valve replacement with 15- to 17-mm mechanical prostheses is an important alternative to save critically ill neonates and infants in whom the mitral valve cannot be repaired. Prosthesis replacement for outgrowth can be carried out with low risk., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Supravalvular aortic stenosis (SVAS) represents a heterogeneous group, including Williams syndrome, familial elastin arteriopathy, sporadic cases, and others. This study sought to evaluate long-term outcomes of SVAS repair., Methods: A total of 87 patients underwent surgical repair of congenital SVAS at Boston Children's Hospital in Boston, Massachusetts, between 1997 and 2017. A total of 41 patients had Williams syndrome, and 46 did not. Of the 46 patients who did not have Williams syndrome, 23 had sporadic SVAS, and 13 had familial elastin arteriopathy. Demographic data and outcomes were reviewed and analyzed from medical records., Results: The median age at operation was 2.9 years. Mean z score of the sinotubular junction was -3.29 ± 1.42 and of the aortic root was -0.09 ± 1.19. A total of 26% (n = 22) patients had coronary ostium stenosis, and 41% (n = 9) of them required patch plasty. Survival rates at 5, 10, and 20 years were all 94.3%. Freedom from left ventricular outflow tract reoperation at 5, 10, and 20 years was 78.5%, 70.3%, and 70.3%, respectively. Freedom from aortic arch reintervention at 5, 10, and 20 years was 98.6%, 94.3%, 89.3%, respectively. In risk factors analysis, age younger than 1 year, z scores of the aortic valve and aortic root, and concomitant right ventricular outflow tract surgical repair were predictive of the need for reoperation and reintervention for left or right ventricular outflow tract obstruction., Conclusions: Excellent long-term survival rates can be achieved with surgical repair of SVAS. Age younger than 1 year, small aortic valve and aortic root z scores, and concomitant right ventricular outflow tract surgical repair were predictors of reoperation and reintervention., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Successful repair of anomalous origin of coronary artery from the pulmonary artery (ACAPA) is generally associated with a good prognosis. However, risk factors for poor postoperative outcomes have not been well characterized. This study used a multicenter data set to determine predictors of mortality after ACAPA repair., Methods: A retrospective analysis was performed using The Society of Thoracic Surgeons Congenital Heart Surgery Database's Participant User File. After identification of all patients with ACAPA who underwent repair from 2007 to 2016, demographics, preoperative and intraoperative variables, and postoperative complications were compared between survivors and nonsurvivors. The primary outcomes included (1) in-hospital mortality and (2) the need for postoperative extracorporeal membrane oxygenation (ECMO) support. Multivariable logistic regression was used to determine preoperative and intraoperative risk factors for these outcomes., Results: Of the 703 patients who underwent ACAPA repair, 20 (2.8%) died during the same hospitalization. The odds of mortality were increased if preoperative shock was present (odds ratio [OR], 4.6; 95% confidence interval [CI], 1.4 to 15.1; P = .01) and if postoperative ECMO was required (OR, 11.8; 95% CI, 3.6 to 38.4; P < .001). The odds of postoperative ECMO use were increased if preoperative shock was present (OR, 3.6; 95% CI, 1.6 to 7.6; P = .001). Lower weight was also a risk factor for both mortality and postoperative ECMO., Conclusions: Lower weight, preoperative shock, and postoperative ECMO use were identified as risk factors for in-hospital mortality in patients undergoing ACAPA repair. These important perioperative factors likely reflect the clinical severity of presentation and suggest a role for early consideration of postoperative mechanical circulatory support to improve outcomes., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Fasciculoventricular fibers (FVFs) are responsible for 1%-5% of cases of asymptomatic preexcitation on the surface electrocardiogram (ECG). Unlike ventricular preexcitation seen in Wolff-Parkinson-White (WPW) syndrome, FVFs are not associated with sudden cardiac death from preexcited atrial fibrillation., Objective: The purpose of this study was to identify surface ECG variables that differentiate FVFs from true WPW syndrome., Methods: This is a retrospective case-control study comparing surface ECG characteristics of patients diagnosed with FVFs (cases) with those of patients with WPW syndrome and anteroseptal accessory pathways (controls) via intracardiac electrophysiology testing at a single institution from 2005 to 2017., Results: Twenty-four cases of FVFs confirmed by intracardiac electrophysiology testing were identified and compared with 48 consecutive controls with WPW syndrome and anteroseptal accessory pathways. Patients with WPW syndrome were found to have significantly higher delta wave amplitudes (4.8 ± 2.0 mm vs 1.9 ± 1.3 mm; P < .001), shorter PR intervals (94.6 ± 12.5 ms vs 106.8 ± 13.2 ms; P < .001), and longer QRS intervals (133.6 ± 19.0 ms vs 118.7 ± 24.7 ms; P = .006) than did those with FVFs. Multivariable logistic regression analysis identified the delta wave amplitude as the only independent predictor of WPW syndrome (odds ratio 3.1 per 1-mm increase; bootstrapped 95% confidence interval 1.5-6.4; c statistic 0.90; P = .002)., Conclusion: The etiology of preexcitation in patients with an anteroseptal preexcitation pattern, whether because of a benign FVF or because of potentially serious WPW syndrome, can be noninvasively deduced using the surface ECG. A higher delta wave amplitude is an independent risk factor for the presence of WPW syndrome and can accurately distinguish WPW syndrome from a FVF with good test accuracy characteristics., (Copyright © 2019 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Previous reports of primary graft dysfunction (PGD) in pediatric heart transplant (HT) recipients are limited to descriptive series of children who required extracorporeal membrane oxygenation (ECMO) support shortly after HT. In this study we sought to determine the incidence, risk factors, and survival after severe PGD in pediatric HT recipients., Methods: We identified all children <18 years old who underwent HT in the United States during 1996 to 2015 using the Organ Procurement and Transplant Network database and then identified those who developed severe PGD by linking patient variables to Extracorporeal Life Support Organization registry data. Logistic regression models were used to assess risk factors for developing severe PGD., Results: The overall incidence of severe PGD was 4.7% over 20 years (95% confidence interval 4.2% to 5.3%). The incidence was 4.1%, 4.5%, 5.3%, and 4.6%, respectively, in consecutive 5-year periods (p for trend = 0.48). Independent risk factors for developing severe PGD were younger age, congenital heart disease, HT while supported on ECMO, higher serum bilirubin, and graft ischemic time ≥4 hours. Ventricular assist device support as bridge to HT and available donor variables were not associated. Death (or graft loss) before discharge occurred in 40.6% of children with PGD (105 deaths, 7 re-transplants) and in 5.6% of children without PGD., Conclusions: Severe PGD remains an important clinical morbidity in pediatric HT recipients in the current era and is associated with high mortality. These findings highlight the need for research in preventing and treating PGD in pediatric HT recipients for improving overall post-transplant survival., (Copyright © 2019. Published by Elsevier Inc.)

Background: In the current era, coarctation repair is usually performed as soon as diagnosis is established. We sought to determine if neonatal isolated coarctation repair had a higher rate of intervention postdischarge when compared with older infants., Methods: We conducted a retrospective review of neonates (≤30 days of age) and infants (1 to 6 months of age) undergoing isolated coarctation repair between January 1, 2000, and March 31, 2016. Preoperative and postoperative Z scores of arch, aortic valve, and isthmus; rates of reintervention; and length of stay (LOS) were compared between groups. Linear or Cox regression was used to determine predictors of postoperative intensive care unit and hospital LOS during index surgery and postdischarge interventions for the entire cohort., Results: There were 213 (71.5%) neonates and 85 (28.5%) infants. There was no difference in aortic valve morphology between groups. There was a trend but no statistical difference in postdischarge coarctation reintervention (neonates 10.3% versus infants 4.7%; p = 0.1) or left ventricular outflow tract, aortic valve, or mitral valve interventions (9.9% versus 9.4%; p = 0.9). Median postoperative intensive care LOS (3.01 versus 2.28 days; p < 0.001) and postoperative hospital LOS (6.23 versus 4.85 days; p < 0.001) were significantly higher in neonates. Median follow-up was 3 (interquartile range, 0.2 to 9) years for the entire cohort. On multivariable modeling, preoperative hypoplastic arch was associated with arch reinterventions, particularly in infants (10%; p = 0.003). Preoperative left-sided catheter intervention, left-sided obstructive lesions, and age predicted postdischarge intervention on left-sided structures., Conclusions: Coarctation repair can be safely performed in infants and neonates with acceptable postdischarge intervention. Severity of aortic arch hypoplasia is an important discriminator for reinterventions on the arch, particularly in infants., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

The official analysis of posttransplant survival considers only recently transplanted patients and suggests absence of outcome differences among pediatric heart transplant (HT) centers. We sought to compare posttransplant survival among currently active pediatric HT centers in the United States over 15 years of activity. We identified all children <18 years old who underwent their first HT during 2000-2014 at US centers active during 2013-14. Recipients were followed until March 2016. A mixed-effects survival model with center as a random effect was used to assess center differences in patient and graft survival. Center case-mix and standardized mortality ratio (SMR) for 90-day mortality were assessed by applying an internally validated risk-model. Overall, 4271 children transplanted at 46 centers were analyzed. There was a significant center effect on risk-adjusted patient (P = .01) and graft survival (P < .001). Adjusting for 90-day SMR or center-volume, but not for case-mix, was associated with a significant reduction in center effect on long-term survival. There was no center effect on conditional survival in 90-day survivors. In conclusion, there are significant differences in posttransplant survival among pediatric HT centers in the United States. Centers with better short-term performance (lower SMR for 90-day mortality) maintain their outcome advantage on follow-up and have superior longer-term outcomes., (© 2018 The American Society of Transplantation and the American Society of Transplant Surgeons.)

Background: Isolated reports from low- and middle-income countries (LMICs) for surgical results in tetralogy of Fallot (TOF) are available. The International Quality Improvement Collaborative for Congenital Heart Disease (IQIC) seeks to improve surgical results promoting reductions in infection and mortality in LMICs., Methods: All cases of TOF in the IQIC database performed between 2010 and 2014 at 32 centers in 20 LMICs were included. Excluded from the analysis were TOF with any associated lesions. A logistic regression analysis was performed to identify risk factors for in-hospital mortality after surgery for TOF., Results: A total of 2,164 patients were identified. There were 1,839 initial primary repairs, 200 with initial systemic-to-pulmonary artery shunt, and 125 underwent secondary repair after initial palliation. Overall mortality was 3.6% (78 of 2,164), initial primary repair was 3.3% (60 of 1,839), initial systemic-to-pulmonary artery shunt was 8.0% (16 of 200), and secondary repair was 1.6% (2 of 125; p = 0.003). Major infections occurred in 5.9% (128 of 2,164) of the entire cohort. Risk factors for death after the initial primary repair were oxygen saturation less than 90% and weight/body mass index for age below the fifth percentile (p < 0.001). The initial primary repair occurred after age 1 year in 54% (991 of 1,839). Older age at initial primary repair was not a risk factor for death (p = 0.21)., Conclusions: TOF patients are often operated on after age 1 year in LMICs. Unlike in developed countries, older age is not a risk factor for death. Nutritional and hypoxemic status were associated with higher mortality and infection. This information fills a critical knowledge gap for surgery in LMIC., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Repair of partial or transitional atrioventricular septal defects (P/TAVSDs) has excellent outcomes; however, late reinterventions remain a concern. Technical performance score (TPS) measures residua after repair and has been associated with early/mid-term outcomes after congenital cardiac operation. Our study investigates TPS as a predictor of outcomes after P/TAVSD repair., Methods: This was a single-center retrospective review of P/TAVSD repair from July 2000 to November 2015. Intraoperative and discharge TPS were assigned based on echocardiographic criteria: class 1, no residua; class 2, minor residua; and class 3, major residua or reintervention for major residua. Intensive care unit (ICU) hospital length of stay and reintervention after discharge were analyzed with Cox regression., Results: In our cohort, 124 partial (68%) and 59 transitional (32%) AVSDs underwent repair. Median age was 1.5 years (interquartile range [IQR]: 0.6 to 3.8 years), median weight was 9.7 kg (IQR: 6.6 to 14.1 kg), and 96 (52%) were female. Twenty patients (11%) required reintervention after discharge. On multivariable modeling, patients with TPS class 3 spent more days in the ICU (hazard ratio [HR] 0.33, 95% confidence interval [CI]: 0.19 to 0.58, p < 0.001) and hospital (HR 0.33, 95% CI: 0.19 to 0.57, p < 0.001) and had shorter time to reintervention after discharge (HR 8.76, 95% CI: 1.03 to 74.7, p = 0.047)., Conclusions: Major residua, that is TPS class 3, were a predictor of in-hospital outcomes and unplanned reinterventions after discharge, with left atrioventricular valve regurgitation being the strongest predictor. Use of TPS as a tool for residual lesions may identify patients predisposed to prolonged ICU hospital stay and reinterventions after discharge, providing feedback on areas in need of improvement in technique and identification of patients who warrant closer follow-up., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Technical performance score (TPS) has been associated with both early and late outcomes across a wide range of congenital cardiac procedures. We sought to validate TPS as predictor of outcomes for complete atrioventricular septal defect (CAVSD) repair., Methods: This was a single-center retrospective review of patients after balanced CAVSD repair between January 1, 2000, and March 1, 2016. We assigned TPS (class 1, no residua; class 2, minor residua; class 3, major residua or reintervention before discharge for residua) based on summation of subcomponent scores from discharge echocardiograms. Outcomes of interest were in-hospital complications, postoperative days on ventilator, and postdischarge reintervention., Results: Among 350 patients, median age was 3.2 months (interquartile range [IQR], 2.4 to 4.2 months). Fifty-four patients (16%) had class 1 TPS, 218 (62%) class 2, 63 (18%) class 3, and 15 (4%) were unscorable. There were 36 complications (10%), and median postoperative days on ventilator were 2 (IQR, 1 to 3) days. There were 34 postdischarge reinterventions (10%). Median follow-up was 2.6 years (IQR, 0.09 to 7.9) years. On multivariable modeling, class 3 TPS was associated with complications (odds ratio 5.45, 95% confidence interval [CI]: 1.06 to 28.1, p = 0.04), prolonged postoperative ventilator days (hazard ratio [HR] 0.54, 95% CI: 0.37 to 0.80, p = 0.002), and postdischarge reintervention (HR 5.61, 95% CI: 1.28 to 24.5, p = 0.02) after adjusting for covariates such as age, weight, genetic abnormality, concomitant procedure, prematurity, and second bypass run., Conclusions: At our center, CAVSD repair was associated with low morbidity. TPS may identify patients with complications, prolonged days on ventilator, and who require postdischarge reinterventions; thus, it provides feedback on areas of improvement and allows identification of patients who warrant closer follow-up., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Hepatic fibrosis is highly prevalent in individuals with Fontan circulation. FibroSure (LabCorp, Burlington, NC) and hyaluronic acid (HA) have been validated for assessment of hepatic fibrosis in several forms of liver disease. We sought to determine whether these tests could identify Fontan patients with advanced hepatic fibrosis or cirrhosis., Methods: Subjects who had liver biopsy and FibroSure or HA testing within 6 months of biopsy were identified from the Alliance for Adult Research in Congenital Cardiology Fontan Liver Health study. Biopsy specimens were scored for degree of sinusoidal and portal fibrosis on a 3- and 5-point scale, respectively. Histologic findings were correlated with FibroSure and HA results., Results: The study included 27 subjects. Median age was 26.8 years (range, 17.4-59.8 years), and the median time since the Fontan surgery was 20.4 years (range, 12.0-31.3 years). FibroSure scores were elevated (>0.21) in 21 of 23 subjects (91%), and the scores for 3 (13%) suggested cirrhosis (>0.74). HA suggested cirrhosis (>46 ng/mL) in 3 of 17 subjects (18%). One subject died during the collection period. Eleven of 26 subjects (42%) had 4/5 or 5/5 portal fibrosis, consistent with cirrhosis; 17 (63%) had 3/3 sinusoidal fibrosis involving >66% of sinusoids. The FibroSure score and HA levels did not correlate with the degree of hepatic fibrosis and did not predict cirrhosis., Conclusions: Abnormal biomarkers of hepatic fibrosis and specimen-proven hepatic fibrosis are common in adults with Fontan circulation. However, FibroSure and HA do not accurately predict the degree of histologic hepatic fibrosis. Further studies are needed to guide strategies for surveillance of liver disease in this population., (Copyright © 2016 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Background: Previous studies have demonstrated the effect of adult nursing skill mix, staffing ratios, and level of education on patient deaths, complication rates, and failure to rescue (FTR). To date, only one known study had examined the effect of nursing experience and education on postoperative pediatric cardiac operations., Methods: Nursing survey data were linked to The Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database for patients undergoing cardiac operations (2010 to 2011). Logistic regression models were used to estimate associations of nursing education and years of clinical experience with in-hospital mortality rates, complication rates, and FTR. Generalized estimating equations and robust standard error estimates were used to account for within-center correlation of outcomes., Results: Among 15,463 patients (29 hospitals), the in-hospital mortality rate was 2.8%, postoperative complications occurred in 42.4%, and the FTR rate was 6.4%. After covariate adjustment, pediatric critical care units with a higher proportion of nurses with a Bachelor of Science degree or higher had lower odds of complication (odds ratio for 10% increase, 0.85; 95% confidence interval, 0.76 to 0.96; p = 0.009). Units with a higher proportion of nurses with more than 2 years of experience had lower mortality rates (odds ratio for 10% increase, 0.92; 95% confidence interval, 0.85 to 0.99; p = 0.025)., Conclusions: This is the first study to demonstrate that higher levels of nursing education and experience are significantly associated with fewer complications after pediatric cardiac operations and aligns with our previous findings on their association with reduced deaths. These results provide data for pediatric hospital leaders and reinforce the importance of organization-wide mentoring strategies for new nurses and retention strategies for experienced nurses., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: To examine the feasibility, effectiveness, and safety of the cranial cup for the correction of visible head shape deformities in premature infants., Design: A prospective descriptive research design., Setting: Two study sites, an urban Level 4 NICU and a suburban Level 2 special care nursery., Participants: The study sample consisted of 23 neonates and infants with deformational plagiocephaly who were born at less than or equal to 35 weeks gestation, weighed greater than 1 kg, and were in the convalescent phase (stable) of their hospitalizations., Methods: All participants received the experimental treatment with the cranial cup. Infants were positioned on the cranial cup for a minimum of 12 hours per day. They also received routine position changes at least every 3 to 4 hours. Demographic data and baseline and discharge cranial measurements were obtained for each infant., Results: All participants (N = 23) had visible deformational plagiocephaly on study entry, and 86% (n = 19) had corresponding abnormal cranial measurements. The median hours per day on the cranial cup was 12.7 (range = 6.3 to 18.0). At hospital discharge, 83% (n = 19) of participants had normal cranial measurements. Furthermore, there were no reported safety concerns associated with use of the cranial cup., Conclusion: Use of the cranial cup during the convalescent phase of hospitalization is feasible and safe, and it provides effective correction of DP for premature neonates and infants., (Copyright © 2016 AWHONN, the Association of Women's Health, Obstetric and Neonatal Nurses. Published by Elsevier Inc. All rights reserved.)

Background: In response to societal pressure to reduce expenditures and increase quality, we sought to develop a methodology to predict hospital charges related to congenital heart surgery., Methods: Patients undergoing congenital heart surgery at Boston Children's Hospital in fiscal years 2007 to 2009 comprised the derivation cohort. Clinical data, including Current Procedural Terminology coding of the primary surgical intervention, were collected prospectively and linked to total hospital charges for an episode of care. Surgical charge categories were developed to group surgical procedure types using empiric data and expert consensus. A multivariable model was built using surgical charge categories and additional patient and procedural characteristics to predict the outcome, total hospital charges. A contemporary cohort for fiscal years 2010 to 2012 was used to validate surgical charge categories and the multivariable model., Results: In the derivation cohort, 2,105 cases met inclusion criteria. One hundred three surgical procedure types were categorized into seven surgical charge categories, yielding a grouper variable with an R(2) explanatory value of 47.3%. Explanatory value increased with consideration of patient age, admission status, and preoperative ventilator dependence (R(2) = 59.4%), as well as weight category, noncardiac abnormality, and genetic syndrome other than trisomy 21 (R(2) = 61.5%). Additional variability in charge was explained when extracorporeal membrane oxygenation utilization and greater than one operating room visit during the episode of care were added (R(2) = 74.3%). The contemporary cohort yielded an R(2) explanatory value of 67.7%., Conclusions: The combination of clinical data with resource utilization information resulted in a statistically valid predictive model for total hospital charges in congenital heart surgery., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Although early results of pulmonary valve replacement (PVR) after tetralogy of Fallot repair have been described, information about late postoperative ventricular size and function is lacking. This study was designed to characterize right ventricular (RV) remodeling up to 10 years after PVR., Methods: Retrospective analysis was conducted of cardiovascular magnetic resonance (CMR) data from 2002 to 2011 in 101 patients (244 studies) who underwent PVR and had one or more post-PVR CMR studies at five post-PVR time intervals up to 10 years., Results: Compared with pre-PVR values, in the 0- to 1-year post-PVR group, pulmonary regurgitation (PR) fraction decreased from 49 ± 11% to 3 ± 2% (p < 0.001), RV end-diastolic volume index (EDVi) decreased by 39% (p < 0.001), RV end-systolic volume index (ESVi) decreased by 33% (p < 0.001), and RV ejection fraction (EF) decreased from 48 ± 8% to 44 ± 8% (p = 0.01). These values remained unchanged through the sixth post-PVR year. However, by 7 to 10 years after PVR (n = 15), RVEDVi and RVESVi were significantly increased and had returned to 84% and 104% of pre-PVR volumes, respectively, and RV EF had declined further. Increasing RV EDVi correlated with increasing grades of PR (rs = 0.36, p < 0.001), tricuspid regurgitation (rs = 0.33, p < 0.001), and RV pressure (rs = 0.32, p = 0.03)., Conclusions: In this cohort, early reduction in RV size showed a gradual return toward preoperative values by 7 to 10 years after PVR. The late adverse RV remodeling was associated with increased RV volume and pressure loads. These findings highlight the palliative nature of PVR and the importance of continued surveillance., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: To measure the feasibility, safety, and efficacy of the cranial cup device in a sample of hospitalized infants at risk for deformational plagiocephaly (DP)., Design: A multisite, stratified, and randomized single-blinded study., Setting: Neonatal intensive care units (NICU) from three urban and one suburban hospital participated., Participants: Subjects included 62 infants with lengths of stay ≥ 14 days., Methods: Nurses caring for infants in study group 1 used the moldable positioner. In study group 2, nurses rotated the moldable positioner and cranial cup devices using the cranial cup for a target goal of 12 hours/day. Both study groups received routine position changes. Outcome measures included hours of device use (feasibility), cardiorespiratory and emesis events (safety), and cranial measurements obtained at discharge (efficacy) by one of four, licensed orthotists who were blinded to the study., Results: A total of 35 infants were randomized to study group 1 (moldable positioner) and 27 infants to study group 2 (moldable positioner and cranial cup). The median hours per day on the cranial cup was 10.7 (range 4.5-15.3). Emesis and cardiorespiratory events were equally distributed for the moldable positioner and cranial cup devices in study group 2. At discharge, more infants in study group 1 (46%, n = 16) exhibited abnormal cranial measurements than those in study group 2 (19%, n = 5) (p = .03)., Conclusion: Rotating the cranial cup with the moldable positioner provides a feasible, safe, and potentially efficacious therapy for prevention of DP., (© 2015 AWHONN, the Association of Women's Health, Obstetric and Neonatal Nurses.)

Background: Subpulmonary ventricular outflow conduits are utilized routinely to repair complex congenital cardiac abnormalities, but are limited by the inevitable degeneration and need for reintervention. Data on conduit durability and propensity to dysfunction in the adult population are limited., Methods: The study included 288 consecutive patients ≥18 years of age who were evaluated between 1991 and 2010 after placement of a ≥18 mm conduit. Freedom from hemodynamic conduit dysfunction served as our primary outcome. Freedom from reintervention, overall mortality and heart transplantation were also evaluated., Results: Median age at conduit implant was 19 years and median follow-up duration was 13 years. Probabilities of survival without conduit dysfunction and reintervention at 5, 10 and 15 years were 87%, 63%, and 49%, and 95%, 81%, and 56%, respectively. Smaller conduit diameter (18-20mm) was associated with lower probability of survival without dysfunction in the entire study cohort, with prominent effects in patients in both the lowest and the highest age quartiles. Other parameters with similar associations were higher BMI, native anatomy of tetralogy of Fallot or truncus arteriosus, and active smoking., Conclusions: Adult congenital heart disease patients with conduit diameter ≥18 mm had an approximately 50% chance of developing hemodynamic conduit dysfunction and undergoing conduit reintervention by 15 years of post-implant, and a 30% likelihood of undergoing conduit reoperation in the same time frame. The importance of these data is underscored by the increasing number of adults with congenital heart diseases seeking care and the recent advances in transcatheter valve replacement for dysfunctional conduits., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

Background: Most transplant centers perform serial cardiac biopsies for rejection surveillance in pediatric heart transplant (HT) recipients. We sought to assess tissue Doppler imaging (TDI) findings during biopsy specimen-proven rejection in pediatric HT recipients and to develop TDI criteria for absence of rejection with high predictive accuracy., Methods: We included the 122 HT recipients in follow-up at our center (median age at HT, 8.7 years). We identified all echocardiograms with adequate TDI data performed within 24 hours of a cardiac biopsy during 2005 to 2011. Rejection was defined as Grade ≥ 2R cellular rejection or antibody-mediated rejection. Paired comparisons of TDI velocities were made using patients' baseline velocities as the control., Results: Overall, 647 specimen-pairs were identified where there was no rejection at baseline. In 24 of these, the second biopsy specimen demonstrated rejection. Using receiver operating characteristic curve analysis of percentage change from baseline, we identified < 15% decline in left ventricular (LV) S' velocity and < 5% decline in LV A' velocity to individually predict non-rejection with > 99% accuracy. When joint criteria were used, the predictive accuracy was 100%, and no rejection event was misclassified. More than 75% of TDI pairs met these criteria for non-rejection., Conclusions: Biopsy specimen-proven rejection is associated with a significant decline in biventricular TDI velocities from baseline in pediatric HT recipients. By using well-defined TDI criteria to predict non-rejection, a substantial proportion of planned biopsies may be deferred or avoided at minimal risk to pediatric HT recipients., (Published by Elsevier Inc.)

Background: Medication non-adherence (NA) can result in life-threatening illness in children after solid-organ transplantation. Little is known about the incidence, risk factors and outcomes of NA in large numbers of pediatric heart transplant (HT) recipients., Methods: Organ Procurement Transplant Network (OPTN) data were used to identify all children <18 years of age in the U.S.A. who underwent HT from October 1999 to January 2007. Cox proportional hazards analysis was used to identify risk factors for NA and the effect on graft survival., Results: Of 2,070 pediatric heart transplants performed the median age at transplant was 6 years (interquartile range [IQR] 0 to 13 years); 40% had congenital heart disease (CHD), 7% were re-transplants, 42% were non-white and 43% had Medicaid insurance. Overall, 186 (9%) children had a report of NA at a median age of 15 years with more than two-thirds of NA episodes occurring after 12 years of age. Factors independently associated with NA were: adolescent age at transplant (hazard ratio [HR] 7.0, 95% confidence interval [CI] 4.1 to 12, compared with infants); black race (HR 2.3, 95% CI 1.7 to 3.3, compared with white); Medicaid insurance (HR 2.0, 95% CI 1.5 to 2.7, compared with non-Medicaid insurance); and ventilator or ventricular assist device (VAD) support at transplant. The risk of mortality conditional upon report of NA was 26% at 1 year and 33% at 2 years., Conclusions: Medication NA is an important problem in pediatric HT recipients and is associated with high mortality. Adolescent age, black race, Medicaid insurance and invasive hemodynamic support at transplant were associated with NA, whereas time on the wait list and gender were not. Targeted interventions among at-risk populations may be warranted., (Copyright © 2013 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Background: We have shown previously that technical performance score (TPS) is strongly associated with early mortality and major postoperative adverse events in a diverse group of patients. We now report evaluation of the validity of TPS in predicting late outcomes in the same group of patients., Methods: Patients who underwent surgery between June 1, 2005 and June 30, 2006 were included. The TPS were assigned based on discharge echocardiograms and certain clinical criteria as previously described. Follow-up data for up to 4 years were retrospectively collected. Cox proportional hazards models were used for analysis., Results: A total of 679 patients were included in the analysis. One hundred twenty-three (18%) were neonates, 213 (31%) infants, 291 (435) children, and 52 (8%) adults. Four hundred ninety-one (72%) were in low-risk adjustment in congenital heart surgery (RACHS; 1 to 3), 109 (16%) in high risk (4 to 6), and 27 (4%) were less than 18 years and could not be assigned a RACHS score. Three hundred thirty-one (48%) had an optimal TPS, 283 (42%) adequate, 61 (9%) inadequate, and 4 (1%) could not be scored. There were 34 (5%) late deaths and 149 (22%) late unplanned reinterventions. By univariate analysis, age, RACHS-1 categories, and TPS were all significantly associated with late reintervention (p < 0.001 for all), while TPS and RACHS-1 were significant factors for mortality (p < 0.001). On multivariable modeling, inadequate TPS was strongly associated with both late mortality (p = 0.001; HR [hazard ratio] 3.8, CI [confidence interval] 1.7 to 8.4) and late reintervention (p = 0.002, HR 2.1, CI 1.3 to 3.3) after controlling for RACHS-1 and age., Conclusions: The TPS has a strong association with late outcomes across a wide range of age and disease complexity and may serve as a tool to identify patients who are at a higher risk for late reintervention or mortality., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

We sought to develop and validate a quantitative risk-prediction model for predicting the risk of posttransplant in-hospital mortality in pediatric heart transplantation (HT). Children <18 years of age who underwent primary HT in the United States during 1999-2008 (n = 2707) were identified using Organ Procurement and Transplant Network data. A risk-prediction model was developed using two-thirds of the cohort (random sample), internally validated in the remaining one-third, and independently validated in a cohort of 338 children transplanted during 2009-2010. The best predictive model had four categorical variables: hemodynamic support (ECMO, ventilator support, VAD support vs. medical therapy), cardiac diagnosis (repaired congenital heart disease [CHD], unrepaired CHD vs. cardiomyopathy), renal dysfunction (severe, mild-moderate vs. normal) and total bilirubin (≥ 2.0, 0.6 to <2.0 vs. <0.6 mg/dL). The C-statistic (0.78) and the Hosmer-Lemeshow goodness-of-fit (p = 0.89) in the model-development cohort were replicated in the internal validation and independent validation cohorts (C-statistic 0.75, 0.81 and the Hosmer-Lemeshow goodness-of-fit p = 0.49, 0.53, respectively) suggesting acceptable prediction for posttransplant in-hospital mortality. We conclude that this risk-prediction model using four factors at the time of transplant has good prediction characteristics for posttransplant in-hospital mortality in children and may be useful to guide decision-making around patient listing for transplant and timing of mechanical support., (© Copyright 2012 The American Society of Transplantation and the American Society of Transplant Surgeons.)

We sought to evaluate trends in overall and race-specific pediatric heart transplant (HT) wait-list mortality in the United States (US) during the last 20 years. We identified all children <18 years old listed for primary HT in the US during 1989-2009 (N = 8096, 62% White, 19% Black, 13% Hispanic and 6% Other) using the Organ Procurement and Transplant Network database. Wait-list mortality was assessed in four successive eras (1989-1994, 1995-1999, 2000-2004 and 2005-2009). Overall wait-list mortality declined in successive eras (26%, 23%, 18% and 13%, respectively). The decline across eras remained significant in adjusted analysis (hazard ratio [HR] 0.70 in successive eras, 95% confidence interval [CI], 0.67-0.74) and was 67% lower for children listed during 2005-2009 versus those listed during 1989-1994 (HR 0.33; CI, 0.28-0.39). In models stratified by race, wait-list mortality decreased in all racial groups in successive eras. In models stratified by era, minority children were not at higher risk of wait-list mortality in the most recent era. We conclude that the risk of wait-list mortality among US children listed for HT has decreased by two-thirds during the last 20 years. Racial gaps in wait-list mortality present variably in the past are not present in the current era., (©Copyright 2011 The American Society of Transplantation and the American Society of Transplant Surgeons.)

Background: Previous studies have found no association between graft ischemic time (IT) and survival in pediatric heart transplant (HTx) recipients. However, previous studies were small or analyzed risk only at the extremes of IT, where observations are few. We sought to determine whether graft IT is independently associated with graft survival in a large cohort of children with no a priori assumptions about where the risk threshold may lie., Methods: All children aged <18 years in the U.S. undergoing primary HTx (1987 to 2008) were included. The primary end point was graft loss (death or retransplant) within 6 months. Multivariate analysis was performed to analyze the association between graft IT and graft loss within 6 months after transplant. A secondary end point of longer-term graft loss was assessed among recipients who survived the first 6 months after transplant., Results: Of 4,716 pediatric HTxs performed, the median IT was 3.5 hours (interquartile range, 2.7-4.3 hours). Adjusted analysis showed that children with an IT > 3.5 hours were at increased risk of graft loss within 6 months after transplant (hazard ratio, 1.3; 95% confidence interval, 1.1-1.5; p = 0.002). Among 6-month survivors, IT was not associated with longer-term graft loss., Conclusions: IT beyond 3.5 hours is associated with a 30% increase in risk of graft loss within 6 months in pediatric HT recipients. Although the magnitude of risk associated with IT is small compared with the risk associated with recipient factors, these findings may be important during donor assessment for high-risk transplant candidates., (2011 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Objectives: This study sought to develop a method to adjust for case mix complexity in catheterization for congenital heart disease to allow equitable comparisons of adverse event (AE) rates., Background: The C3PO (Congenital Cardiac Catheterization Project on Outcomes) has been prospectively collecting data using a Web-based data entry tool on all catheterization cases at 8 pediatric institutions since 2007., Methods: A multivariable logistic regression model with high-severity AE outcome was built using a random sample of 75% of cases in the multicenter cohort; the models were assessed in the remaining 25%. Model discrimination was assessed by the C-statistic and calibration with Hosmer-Lemeshow test. The final models were used to calculate standardized AE ratios., Results: Between August 2007 and December 2009, 9,362 cases were recorded at 8 pediatric institutions of which high-severity events occurred in 454 cases (5%). Assessment of empirical data yielded 4 independent indicators of hemodynamic vulnerability. Final multivariable models included procedure type risk category (odds ratios [OR] for category: 2 = 2.4, 3 = 4.9, 4 = 7.6, all p < 0.001), number of hemodynamic indicators (OR for 1 indicator = 1.5, ≥2 = 1.8, p = 0.005 and p < 0.001), and age <1 year (OR: 1.3, p = 0.04), C-statistic 0.737, and Hosmer-Lemeshow test p = 0.74. Models performed well in the validation dataset, C-statistic 0.734. Institutional event rates ranged from 1.91% to 7.37% and standardized AE ratios ranged from 0.61 to 1.41., Conclusions: Using CHARM (Catheterization for Congenital Heart Disease Adjustment for Risk Method) to adjust for case mix complexity should allow comparisons of AE among institutions performing catheterization for congenital heart disease., (Copyright © 2011 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: We hypothesize that a measure of the immediate postoperative severity of illness after the stage I Norwood operation reflects technical performance or the adequacy of anatomic repair and can serve as a predictor of hospital mortality, reinterventions, and clinical outcomes., Methods: One hundred thirty-five patients undergoing stage I were retrospectively studied (2004 to 2007). The severity of illness on postoperative day 1 (POD1) was measured using the Pediatric Risk of Mortality III (PRISM) scoring system. Technical performance scores (optimal, adequate, inadequate) were calculated before hospital discharge. Hospital mortality, postoperative reinterventions, and complications were recorded. Postoperative reintervention was defined as need for cardiac catheterization laboratory or operating room based procedure that included balloon dilation or repair of arch obstruction, shunt revision, reoperations for bleeding, and extracorporeal membrane oxygenation support., Results: Hospital mortality was 14.1% (n=19). The rate of complications and reinterventions was, respectively, 28.1% (n=38) and 26.7% (n=36). The POD1 PRISM score was associated with technical performance (p=0.003). Higher POD1 PRISM scores were associated with mortality (p<0.001), complications (p<0.001), and reinterventions (p=0.001). The POD1 PRISM score had high discrimination for mortality, complications, reinterventions, and inadequate technical performance (areas under the receiver operating characteristic curve were 0.835, 0.776, 0.773, and 0.710, respectively; p≤0.001 for all)., Conclusions: The severity of illness as measured by PRISM score on POD1 after the stage I Norwood operation has strong association and discrimination with hospital mortality, postoperative reinterventions, inadequate technical performance, and major postoperative complications. It may be used as an early surrogate of technical performance to initiate a search for and correction of technical deficiencies., (Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

We assessed whether the improvement in posttransplant survival in pediatric heart transplant (HT) recipients during the last two decades has benefited the major racial groups in the United States equally. We analyzed all children <18 years of age who underwent their first HT in the US during 1987-2008. We compared trends in graft loss (death or retransplant) in white, black and Hispanic children in five successive cohorts (1987-1992, 1993-1996, 1997-2000, 2001-2004, 2005-2008). The primary endpoint was early graft loss within 6 months posttransplant. Longer-term survival was assessed in recipients who survived the first 6 months. The improvement in early posttransplant survival was similar (hazard ratio [HR] for successive eras 0.80, 95% confidence interval [CI] 0.7, 0.9, p = 0.24 for black-era interaction, p = 0.22 for Hispanic-era interaction) in adjusted analysis. Longer-term survival was worse in black children (HR 2.2, CI 1.9, 2.5) and did not improve in any group with time (HR 1.0 for successive eras, CI 0.9, 1.1, p = 0.57; p = 0.19 for black-era interaction, p = 0.21 for Hispanic-era interaction). Thus, the improvement in early post-HT survival during the last two decades has benefited white, black and Hispanic children equally. Disparities in longer-term survival have not narrowed with time; the survival remains worse in black recipients., (©2010 The Authors Journal compilation©2010 The American Society of Transplantation and the American Society of Transplant Surgeons.)

Background: Uncertainty exists whether hearts from infants who have died of sudden infant death syndrome (SIDS) are acceptable for transplantation because the mechanism of death in SIDS remains unclear. We analyzed post-transplant outcomes in infants who received a heart from a donor where SIDS was the primary cause of brain death., Methods: This retrospective multicenter cohort study used data from the Organ Procurement and Transplant Network (OPTN). All infants aged < 12 months undergoing heart transplant between 1994 and 2008 were included. A Cox proportional hazards model was used to determine whether donor SIDS was independently associated with post-transplant graft loss (death or retransplant)., Results: During the study period, 66 of 1033 infants (6.4%) who underwent heart transplant received an allograft from a SIDS donor. These infants were similar to the remaining infants with respect to age, diagnosis, blood type, and invasive support. In multivariable analysis, graft loss was associated with congenital heart disease (hazard ratio [HR], 1.6; 95% confidence interval [CI], 1.2-2.1), ventilator (HR, 1.4; 95% CI, 1.1-1.9), and extracorporeal membrane oxygenation support (HR, 3.0; 95% CI, 2.2-4.3), but not donor SIDS (HR, 1.0; 95% CI, 0.6-1.5), suggesting graft survival in SIDS-donor heart recipients was similar to the remaining infants. Primary causes of post-transplant death in infants receiving SIDS-donor hearts and the remaining infants were similar., Conclusions: Graft survival was similar in infants who received SIDS-donor hearts compared with those who received hearts from donors who died of other causes. There was no increase in incidence of non-rejection-related cardiac deaths after transplant in these children., (Copyright © 2010 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Background: Infants awaiting heart transplantation (HT) face the highest wait-list mortality among all children and adults listed for HT in the USA. We sought to determine the risk of death for infants <12 months old while awaiting HT in the current era, and to identify the principle risk factors associated with wait-list mortality., Methods: We analyzed outcomes for all infants listed for HT in the USA from January 1999 to July 2006, using data reported to the U.S. Scientific Registry of Transplant Recipients., Results: Of the 1,133 listed infants, 61% were <3 months of age, 80% were listed as Status 1A, 64% had a congenital heart disease (CHD) and 31% had cardiomyopathy. Of 724 infants with CHD, 25% were on prostaglandin (PG) and 27% had a history of prior surgery. By 6 months after listing, 23% died on the wait-list and 54% were transplanted. Multivariate factors associated with wait-list mortality were weight <3 kg (hazard ratio [HR] 1.4, 95% confidence interval [CI] 1.0 to 1.9), extracorporeal membrane oxygenation (ECMO) support (HR 5.6, CI 4.0 to 7.9), ventilator support (HR 2.1, 95% CI 1.6 to 2.8), CHD with PG support (HR 2.8, 95% CI 1.8 to 4.3), CHD without prior surgery (HR 2.8, 95% CI 1.9 to 3.9) and non-white race/ethnicity (HR 1.8, 95% CI 1.4 to 2.3)., Conclusions: One in four infants listed for HT in the USA die before a donor heart can be identified. Wait-list mortality is associated with weight <3 kg, level of invasive support and CHD, but not listing status, which captures medical urgency poorly. Measures to expand infant organ donation, especially among neonates, are urgently needed.

Racial differences in outcomes are well known in children after heart transplant (HT) but not in children awaiting HT. We assessed racial and ethnic differences in wait-list mortality in children <18 years old listed for primary HT in the United States during 1999-2006 using multivariable Cox models. Of 3299 listed children, 58% were listed as white, 20% as black, 16% as Hispanic, 3% as Asian and 3% were defined as 'Other'. Mortality on the wait-list was 14%, 19%, 21%, 17% and 27% for white, black, Hispanic, Asian and Other children, respectively. Black (hazard ratio [HR] 1.6, 95% confidence interval [CI] 1.3, 1.9), Hispanic (HR 1.5, CI 1.2, 1.9), Asian (HR, 2.0, CI 1.3, 3.3) and Other children (HR 2.3, CI 1.5, 3.4) were all at higher risk of wait-list death compared to white children after controlling for age, listing status, cardiac diagnosis, hemodyamic support, renal function and blood group. After adjusting additionally for medical insurance and area household income, the risk remained higher for all minorities. We conclude that minority children listed for HT have significantly higher wait-list mortality compared to white children. Socioeconomic variables appear to explain a small fraction of this increased risk.