Contemporary Patterns of Care in Tetralogy of Fallot: Analysis of The Society of Thoracic Surgeons Data.

BACKGROUND: Against the background of earlier studies, recent patterns in surgical management of tetralogy of Fallot (TOF) were assessed. METHODS: A retrospective review of The Society of Thoracic Surgeons (STS) Congenital Database (2010-2020) was performed on patients aged <18 years with TOF or pulmonary stenosis and primary procedure TOF surgical repair or palliation. Procedural frequencies were examined by epoch. Demographics, clinical variables, and outcomes were compared between the initial palliation and primary repair groups. Among those operated on at 0 to 60 days of age, variation in palliation rates across hospitals was assessed. RESULTS: The 12,157 operations included 11,307 repairs (93.0%) and 850 palliations (7.0%); 68.5% of all palliations were modified Blalock-Taussig-Thomas shunts. Of 1105 operations on neonates, 45.4% (502) were palliations. Among neonates, palliations declined from 49.0% (331 of 675) in epoch 1 (2010-2015) to 39.8% (171 of 430) in epoch 2 (2016-2020; P = .0026). Overall, the most prevalent repair technique (5196 of 11,307; 46.0%) was ventriculotomy with transanular patch, which was also used in 520 of 894 (58.2%) of repairs after previous cardiac operations. Patients undergoing initial palliation demonstrated more preoperative STS risk factors (50.1% vs 24.3% respectively; P < .0001) and more major morbidity and mortality than patients undergoing primary repair (21.2% vs 7.46%; P < .0001). In the 0- to 60-day age group, risk factor-adjusted palliation rates across centers varied considerably, with 32 of 99 centers performing significantly more or significantly fewer palliations than predicted on the basis of their case mix. CONCLUSIONS: Surgical palliation rates have decreased across all age groups despite increasing prevalence of risk factors. Ventriculotomy with transanular patch remains the most prevalent repair type. The considerable center-level variation in rates of palliation was not completely explained by case mix.

Growth Differentiation Factor 15: A Novel Growth Biomarker for Children With Congenital Heart Disease.

BACKGROUND: Failure to thrive (FTT), defined as weight or height less than the lowest 2.5 percentile for age, is prevalent in up to 66% of children with congenital heart disease (CHD). Risk stratification methods to identify those who would benefit from early intervention are currently lacking. We aimed to identify a novel growth biomarker to aid clinical decision-making in children with CHD. METHODS: This is a cross-sectional study of patients 2 months to 10 years of age with any CHD undergoing cardiac surgery. Preoperative weight-for-age Z scores (WAZ) and height-for-age Z scores (HAZ) were calculated and assessed for association with preoperative plasma biomarkers: growth differentiation factor 15 (GDF-15), fibroblast growth factor 21, leptin, prealbumin, and C-reactive protein (CRP). RESULTS: Of the 238 patients included, approximately 70% of patients had WAZ/HAZ < 0 and 34% had FTT. There was a moderate correlation between GDF-15 and WAZ/HAZ. When stratified by age, the correlation of GDF-15 to WAZ and HAZ was strongest in children under 2 years of age and persisted in the setting of inflammation (CRP > 0.5 mg/dL). Diagnoses commonly associated with congestive heart failure had high proportions of FTT and median GDF-15 levels. Prealbumin was not correlated with WAZ or HAZ. CONCLUSIONS: GDF-15 represents an important growth biomarker in children with CHD, especially those under 2 years of age who have diagnoses commonly associated with CHF. Our data do not support prealbumin as a long-term growth biomarker.

Impact of obesity on post-operative arrhythmias after congenital heart surgery in children and young adults.

BACKGROUND: Obesity increases the risk of post-operative arrhythmias in adults undergoing cardiac surgery, but little is known regarding the impact of obesity on post-operative arrhythmias after CHD surgery. METHODS: Patients undergoing CHD surgery from 2007 to 2019 were prospectively enrolled in the parent study. Telemetry was assessed daily, with documentation of all arrhythmias. Patients aged 2-20 years were categorised by body mass index percentile for age and sex (underweight <5, normal 5-85, overweight 85-95, and obese >95). Patients aged >20 years were categorised using absolute body mass index. We investigated the impact of body mass index category on arrhythmias using univariate and multivariate analysis. RESULTS: There were 1250 operative cases: 12% underweight, 65% normal weight, 12% overweight, and 11% obese. Post-operative arrhythmias were observed in 38%. Body mass index was significantly higher in those with arrhythmias (18.8 versus 17.8, p = 0.003). There was a linear relationship between body mass index category and incidence of arrhythmias: underweight 33%, normal 38%, overweight 42%, and obese 45% (p = 0.017 for trend). In multivariate analysis, body mass index category was independently associated with post-operative arrhythmias (p = 0.021), with odds ratio 1.64 in obese patients as compared to normal-weight patients (p = 0.036). In addition, aortic cross-clamp time (OR 1.007, p = 0.002) and maximal vasoactive-inotropic score in the first 48 hours (OR 1.03, p = 0.04) were associated with post-operative arrhythmias. CONCLUSION: Body mass index is independently associated with incidence of post-operative arrhythmias in children after CHD surgery.

Salvage of an epicardial lead in a pacemaker-dependent patient with Fontan palliation using an IS-1 extender.

We present a case report of severed epicardial atrial lead salvage using an IS-1 lead extender. A 37-year-old male with single ventricle physiology, Fontan palliation, sinus node dysfunction, recurrent atrial tachycardias, and atrial fibrillation resulting in failing Fontan physiology presented with failure of the atrial pacing lead. The patient was initially paced with an epicardial system that had to be removed due to pocket infection, and the epicardial leads were cut and abandoned. Given his significant sinus node dysfunction he required atrial pacing to allow for rhythm control. The failing Fontan physiology of the patient precluded him from undergoing surgery for epicardial lead placement or a complex intravascular lead placement procedure (although anatomically feasible). We considered the option of salvaging the existing epicardial atrial leads to provide atrial pacing, allowing for rhythm control and improvement of his failing Fontan physiology as a bridge to a more permanent pacing solution. This case report is important because it demonstrates how a lead extender can be used to salvage a severed pacemaker lead. This may be useful for patients in whom implantation of new leads is not promptly feasible due to patient anatomy and/or clinical status.

HeartMate 3 in a ccTGA patient.

A 49-year-old female with congenitally corrected (or levo-) transposition of the great arteries complicated by nonischemic cardiomyopathy presented for worsening heart failure despite guideline-directed medical therapy and was found to be in cardiogenic shock. She successfully underwent ventricular assist device placement with a HeartMate III to her systemic right ventricle as a bridge to transplantation.

Advanced Heart Failure in Adults With Congenital Heart Disease.

As a result of improvements in care for patients with congenital heart disease (CHD), >90% of children born with CHD are expected to survive to adulthood. For those adults, heart failure (HF) is the leading cause of death. Advances in recognition of, and treatments for, these patients continue to improve. Specifically, adults with CHD are candidates for both heart transplantation and mechanical circulatory support. However, challenges remain that require investigation to improve outcomes.

Disorders of Adjustment, Mood, and Anxiety in Children and Adolescents Undergoing Heart Transplantation and the Association of Ventricular Assist Device Support.

OBJECTIVE: To assess the prevalence of psychiatric disorders and associated therapies in children during their heart transplantation admission. STUDY DESIGN: All pediatric heart transplant recipients (1999-2016) were included from a linked administrative and clinical registry database. Psychiatric disorders and associated therapies were identified using International Classification of Diseases or billing codes during the transplant admission. Data were analyzed using standard descriptive statistics. Multivariable logistic regression assessed factors independently associated with psychiatric disorders or therapies. RESULTS: A total of 3073 pediatric heart transplant recipients were included. Psychiatric disorders were present in 434 (14.1%) patients during the heart transplant admission, with adjustment disorders being the most common. Antidepressant therapy was prescribed to 212 patients (6.9%) and selective serotonin reuptake inhibitors were most commonly used. Psychiatric diagnoses (8.4% vs 18.1%; P < .001) and the use of antidepressants (4.5% vs 8.9%; P < .001) increased over time (era 1, 1999-2009 vs era 2, 2010-2016). Psychiatric disorders were present in 39.8% of patients ≥8 years of age requiring ventricular assist device support at heart transplantation. The need for ventricular assist device support was independently associated with psychiatric diagnoses (aOR, 1.57; 95% CI, 1.18-2.1; P = .002) and antidepressant therapy (aOR, 2.11; 95% CI. 1.43-3.12; P < .001). CONCLUSIONS: Psychiatric disorders are common in pediatric heart transplant recipients, especially among those bridged with ventricular assist device support. Psychiatric diagnoses and the use of antidepressants has increased over time, likely representing improved recognition of psychiatric comorbidities in this vulnerable population. Access to psychiatric services represents an important component of the multidisciplinary team caring for pediatric heart transplant recipients.

Extracorporeal membrane oxygenation use in the first 24 hours following pediatric heart transplantation: Incidence, risk factors, and outcomes.

Primary graft dysfunction following HTx is associated with significant morbidity and mortality. This study aimed to assess the incidence of, risk factors for, and outcomes of children requiring ECMO within 24 hours of HTx. This study utilized a linked PHIS/SRTR database of pediatric HTx recipients (2002-2016). Post-HTx ECMO was identified using inpatient billing data. Logistic regression assessed risk factors for post-HTx ECMO. Kaplan-Meier analyses assessed in-hospital mortality and post-discharge survival. A total of 2820 patients were included with 224 (7.9%) requiring ECMO. Independent risk factors for post-HTx ECMO include age <1 year (aOR: 2.2, 95% CI: 1.3-3.7, P = 0.006) or 1-5 years (aOR: 2.1, 95% CI: 1.3-3.4, P = 0.002), and ECMO support at HTx (aOR: 27.4, 95% CI: 15.2-49.6, P < 0.001). Survival to discharge decreased with increasing duration of post-HTx ECMO support; 89% for 1-3 days, 79.1% for 4-6 days, 63.2% for 7-9 days, and 18.8% for ≥10 days. There was no difference in long-term survival for patients requiring post-HTx ECMO who survived to hospital discharge (P = 0.434). There are identifiable risk factors associated with the need for ECMO in the post-HTx period. Length of time on ECMO post-HTx is strongly associated with the risk of in-hospital mortality. Patients who require ECMO early post-HTx and survive to discharge have comparable outcomes to patients who did not require ECMO.

Center Variation in Hospital Costs for Pediatric Heart Transplantation: The Relationship Between Cost and Outcomes.

There are limited published data addressing the costs associated with pediatric heart transplantation and no studies evaluating the variation in costs across centers. We aimed to describe center variation in pediatric heart transplant costs and assess the association of transplant hospitalization costs with patient outcomes. Using a linkage between the Pediatric Health Information System and Scientific Registry of Transplant Recipients databases, hospital costs were assessed for patients (< 18 years of age) undergoing heart transplantation (2007-2016). Severity-adjusted patient costs were calculated using generalized linear mixed-effects models with a random hospital intercept. Center variation in hospital cost was described after adjusting for the predicted risk of in-hospital mortality. Post-transplant survival was compared between low- and high-cost centers using Cox proportional hazard models. A total of 2156 patients were included from 24 centers. There was 3.7-fold variation in transplant hospitalization costs across centers, ranging from $329,477 to $1,226,507. Patients transplanted at high-cost centers have a higher predicted risk of in-hospital mortality (8.1% vs. 6.1%, p < 0.001). Both early (p = 0.008) and long-term (p = 0.003) post-transplant survival were better in patients transplanted at low-cost centers. Transplant at low-cost centers was associated with improved post-transplant survival, independent of patient-specific risk (adjusted hazard ratio 0.72; 95%CI 0.57-0.92, p = 0.008). There is wide variation in cost for pediatric heart transplant inpatient care among U.S. centers with low-cost centers demonstrating the best patient survival. Differences in patient populations likely contribute to these findings, but cannot account for all the variation seen. This suggests that variability in the delivery of care across centers may influence post-transplant survival.

Mechanical circulatory support costs in children bridged to heart transplantation - analysis of a linked database.

BACKGROUND: Pediatric mechanical circulatory support (MCS) has evolved considerably over the past decade. Though marked improvements in waitlist survival have been realized, costs have not been reassessed. This project aimed to assess contemporary MCS costs in children bridged to heart transplant (HT). METHODS: All pediatric HT recipients (2002-2016) were identified from a unique, linked PHIS/SRTR dataset. Costs were calculated from hospital charges, inflated to 2016 Dollars and adjusted for patient-specific characteristics using generalized linear mixed-effects models. Costs and length of stay (LOS) were compared across support strategies at the time of HT (no MCS, VAD, or ECMO) with select subgroup analyses. RESULTS: A total of 2873 pediatric HT recipients were included; no MCS: 2268 (78.9%), VAD: 470 (16.4%), and ECMO: 135 (4.7%). Both VAD and ECMO were associated with greater total hospitalization costs compared to no MCS ($755,345 and $808,771 vs. $457,086; P < .001). Total costs and LOS were similar between VAD and ECMO groups; however, costs and LOS were greatest for VAD-supported patients in the pre-HT period and greatest for ECMO-supported patients post-HT. Post-HT costs and LOS were similar between patients who did not require MCS and those supported with a VAD ($324,887 and 18 days vs. $329,198 and 18 days respectively, p = NS). Outpatients with VAD support at HT demonstrated significantly lower total costs compared to those who were inpatient with continuous flow devices ($552,222 vs. $663,071, P = .003). CONCLUSIONS: MCS as a bridge to HT in children is associated with greater total costs. While costs are similar between VAD and ECMO groups, the majority of costs associated with VAD support is incurred pre-HT while ECMO costs are incurred primarily post-HT. Discharging patients on VAD support awaiting HT may represent a strategy to reduce costs in this population.

Changes in Pediatric Heart Transplant Hospitalization Costs Over Time.

BACKGROUND: Despite significant changes in the past decade for children undergoing heart transplantation, including the evolution of mechanical circulatory support and increasing patient complexity, costs and resource utilization have not been reassessed. We sought to use a novel linkage of clinical-registry and administrative data to examine changes in hospitalization costs over time in this population. METHODS: We identified all pediatric heart transplant recipients in a unique linked Pediatric Health Information System/Scientific Registry of Transplant Recipients data set (2002-2016). Hospital costs were estimated from charges using cost-to-charge ratios, inflated to 2016 dollars. Severity-adjusted costs were calculated using generalized linear mixed-effects models. Costs were compared across 3 eras (era 1, 2002-2006; era 2, 2007-2011; and era 3, 2012-2016). RESULTS: A total of 2896 pediatric heart transplant recipients were included: era 1, 649 (22.4%); era 2, 1028 (35.5%); and era 3, 1219 (42.1%). Extracorporeal membrane oxygenation support at transplant decreased over time, concurrent with an increase in ventricular assist device-supported patients. Between era 1 and era 2, there was an increase in pretransplant hospitalization costs (US $343 692 vs US $435 554; P < 0.001). However, between era 2 and era 3, there was a decline in total (US $906 454 vs US $767 221; P < 0.001), pretransplant (US $435 554 vs US $353 364; P < 0.001), and posttransplant (US $586 133 vs US $508 719; P = 0.002) hospitalization costs. CONCLUSIONS: Concurrent with the increase in utilization of ventricular assist device support, there has been an increase in pretransplant costs associated with pediatric heart transplantation. However, in the most recent era, costs have declined. These findings suggest the evolution of more cost-effective management strategies, which may be related to shifts in the approach to pediatric mechanical circulatory support.

Geographic Distance From Transplant Center Does Not Impact Pediatric Heart Transplant Outcomes.

BACKGROUND: Many pediatric heart transplant recipients live a significant distance from their transplant center. This results in families either traveling long distances or relying on outside physicians to assume aspects of their care. Distance has been implicated to play a role in congenital heart disease outcomes, but its impact on heart transplantation has not been reported. The aim of this study was to assess the impact of distance on pediatric heart transplant outcomes. METHODS: The Scientific Registry of Transplant Recipients database was queried for all pediatric heart transplant recipients from large US children's hospitals (1987-2014). Patients were stratified into 4 groups (<20, 20-50, 50-100, and >100 miles) based on distance. Survival curves were generated and compared using the log-rank test. Cox proportional hazards regression was performed to adjust for differences between groups. RESULTS: A total of 4768 patients were included in the analysis, of which 1435 (30.1%) were <20 miles, 940 (19.7%) were 20 to 50 miles, 806 (16.9%) were 50 to 100 miles, and 1587 (33.3%) were >100 miles from their transplant center. There was no difference in posttransplant survival based on distance after adjusting for patient age, gender, ethnicity, blood type, diagnosis, listing status, and the need for pretransplant ventricular assist device, extracorporeal membrane oxygenation, or ventilator support. CONCLUSION: There is no significant difference in graft survival after pediatric heart transplantation based on patient distance from their transplant center. Our data suggest the current strategy of transitioning some aspects of transplant care to local physicians or management from a distance does not increase posttransplant mortality risk.

Association of Shunt Type With Arrhythmias After Norwood Procedure.

BACKGROUND: Transplant-free survival for single right ventricle (RV) lesions remains less than 70% at 3 years. Arrhythmia burden, influence of shunt type at Norwood procedure (RV-to-pulmonary artery shunt [RVPAS] versus Blalock-Taussig shunt [BTS]), and implications for mortality risk are not well defined. METHODS: The authors performed a single-center retrospective analysis of patients with single RV lesions enrolled in a prospective study of arrhythmias after congenital heart surgery. RESULTS: Fifty-eight patients received a RVPAS and 62 received a BTS, with a median follow-up of 773 days. Overall arrhythmia incidence was 78%, two-thirds of which prompted intervention. Among all types of arrhythmias, only ventricular arrhythmias (VAs) differed by shunt type, which were more common in patients receiving an RVPAS (29% RVPAS versus 14% BTS; p = 0.049). The majority of VAs were transient (69% less than 1 minute), and typically occurred early post-Norwood procedure (median 12 days). No additional variables were associated with development of VAs. Shunt type did not influence transplant-free survival. Within the entire cohort, there was a trend toward increased mortality with prior history of VA (odds ratio, 2.90; 95% confidence interval, 0.99 to 8.90; p = 0.052). For interstage survivors to Glenn palliation, any VA associated with a 14-fold increased risk of death or transplant (hazard ratio, 14.00; 95% confidence interval, 3.66 to 53.40; p < .001). No other tachyarrhythmia or bradyarrhythmia was associated with mortality. CONCLUSIONS: In this cohort with single RV lesions and prospective rhythm surveillance, patients receiving an RVPAS at Norwood surgery had an increased incidence of VAs compared with patients with a BTS. VAs correlated with late mortality in patients who survived the interstage period.

Percutaneous Removal of Intravascular Pellet Following Penetrating Cardiac Trauma.

There is controversy regarding the management of projectile embolization, a rare complication of penetrating trauma. We present the case of a 5-year-old, 20 kg male with retrograde venous projectile embolization following traumatic injury with a pellet gun. The projectile was successfully removed utilizing a novel, percutaneous approach.

Left main coronary artery stent placement in a 7.0 kg infant with Williams Syndrome.

We report the case of a 9-month-old male with Williams syndrome who underwent patch augmentation of supravalvar aortic stenosis and pulmonary artery stenosis, and required emergent drug-eluting left coronary artery stenting on post-operative day 1 for severe left ventricular dysfunction related to myocardial ischemia.

A unique linkage of administrative and clinical registry databases to expand analytic possibilities in pediatric heart transplantation research.

BACKGROUND: Large clinical, research, and administrative databases are increasingly utilized to facilitate pediatric heart transplant (HTx) research. Linking databases has proven to be a robust strategy across multiple disciplines to expand the possible analyses that can be performed while leveraging the strengths of each dataset. We describe a unique linkage of the Scientific Registry of Transplant Recipients (SRTR) database and the Pediatric Health Information System (PHIS) administrative database to provide a platform to assess resource utilization in pediatric HTx. METHODS: All pediatric patients (1999-2016) who underwent HTx at a hospital enrolled in the PHIS database were identified. A linkage was performed between the SRTR and PHIS databases in a stepwise approach using indirect identifiers. To determine the feasibility of using these linked data to assess resource utilization, total and post-HTx hospital costs were assessed. RESULTS: A total of 3188 unique transplants were identified as being present in both databases and amenable to linkage. Linkage of SRTR and PHIS data was successful in 3057 (95.9%) patients, of whom 2896 (90.8%) had complete cost data. Median total and post-HTx hospital costs were $518,906 (IQR $324,199-$889,738), and $334,490 (IQR $235,506-$498,803) respectively with significant differences based on patient demographics and clinical characteristics at HTx. CONCLUSIONS: Linkage of the SRTR and PHIS databases is feasible and provides an invaluable tool to assess resource utilization. Our analysis provides contemporary cost data for pediatric HTx from the largest US sample reported to date. It also provides a platform for expanded analyses in the pediatric HTx population.

Neonatal Aortic Arch Reconstruction With Direct Splanchnic Perfusion Avoids Deep Hypothermia.

BACKGROUND: Neonatal aortic arch reconstruction, typically performed with deep hypothermia and selective cerebral perfusion, leaves splanchnic organ protection dependent on hypothermia alone. A simplified method of direct in-field descending aortic perfusion during neonatal arch reconstruction permits the avoidance of deep hypothermia. We hypothesize that direct splanchnic perfusion at mild hypothermia provides improved or equivalent safety compared with deep hypothermia and may contribute to postoperative extracardiac organ recovery. METHODS: Included were 138 biventricular patients aged younger than 90 days undergoing aortic arch reconstruction with cardiopulmonary bypass. Patients were grouped according to perfusion method A (selective cerebral perfusion with deep hyperthermia at 18° to 20°C) or method B (selective cerebral perfusion and splanchnic perfusion at 30° to 32°C). Patient characteristics and perioperative clinical and serologic data were analyzed. Significance was assigned for p of less than 0.05. RESULTS: Of the 138 survivors, 63 underwent method A and 75 underwent method B. The median age at operation was 8.5 days (range, 6 to 15 days), and median weight was 3.2 kg (range, 2.8 to 3.73 kg), with no significant differences between groups. Cardiopulmonary bypass times were comparable between the two perfusion methods (p = 0.255) as were the ascending aortic cross-clamp times (p = 0.737). The postoperative glomerular filtration rate was significantly different between our groups (p = 0.028 to 0.044), with method B achieving a higher glomerular filtration rate. No significant differences were seen in ventilator time, postoperative length of stay, fractional increase of postoperative serum creatinine over preoperative serum creatinine, and postoperative lactate. CONCLUSIONS: A simplified method of direct splanchnic perfusion during neonatal aortic arch reconstruction avoids the use of deep hypothermia and provides renal protection at least as effective as deep hypothermia.

Regional variation in the use of 1A status exceptions for pediatric heart transplant candidates: is this equitable?

The use of status exceptions (SE) was recently publicized as a strategy to reduce waitlist times for children awaiting heart transplant (HTx). The aim of this study was to assess SE use across UNOS regions and compare survival in patients listed using a SE to those listed by standard criteria. The OPTN database was queried for all pediatric patients listed for HTx (2000-2014). SE use was compared across UNOS regions. Survival curves were generated and compared using the log-rank test. 1A SE use is uncommon, being utilized in 108 of 4587 pediatric 1A listings (2.4%). There is significant variability in SE use across UNOS regions (0.7%-16.4% of 1A listings, P < .001). Waitlist survival is significantly higher in candidates listed using a 1A SE compared to those listed by standard criteria (P = .001) and is similar to 1B listings. Regional variation in 1A SE use has the potential to introduce bias into a system designed to be equitable. Waitlist survival in patients listed using a SE is similar to those listed status 1B, suggesting these patients may not require 1A status. Careful review of pediatric heart allocation policies is needed to optimize patient outcomes and ensure a fair and unbiased allocation system.

Expanding the donor pool: regional variation in pediatric organ donation rates.

There are limited published data on pediatric organ donation rates. The aim of this study was to describe the trends in pediatric organ donation over time and to assess the regional variation in pediatric deceased organ donation. OPTN data were utilized to assess the trends in pediatric organ donation over time. The number of deceased pediatric organ donors was indexed using regional mortality data obtained from the National Center for Health Statistics and compared across UNOS regions and two different eras. The number of pediatric deceased organ donors has declined in the recent era, largely driven by fewer adolescent donors. For all age groups, there is significant regional variation in organ donation rates, with identifiable high- and low-performing regions. Expansion of the donor pool may be possible by optimizing organ donation in regions demonstrating lower recruitment of pediatric donors. Using the region with the highest donation rate for each age group as the gold standard, we estimate a potential 24% increase in the number of donors if all regions performed comparably, equating to 215 new pediatric donors annually.