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Background: Socioeconomic factors may lead to a disproportionate impact on health care usage and death among individuals with congenital heart defects (CHD) by race, ethnicity, and socioeconomic factors. How neighborhood poverty affects racial and ethnic disparities in health care usage and death among individuals with CHD across the life span is not well described., Methods and Results: Individuals aged 1 to 64 years, with at least 1 CHD-related International Classification of Diseases, Ninth Revision, Clinical Modification ( ICD-9-CM ) code were identified from health care encounters between January 1, 2011, and December 31, 2013, from 4 US sites. Residence was classified into lower- or higher-poverty neighborhoods on the basis of zip code tabulation area from the 2014 American Community Survey 5-year estimates. Multivariable logistic regression models, adjusting for site, sex, CHD anatomic severity, and insurance-evaluated associations between race and ethnicity, and health care usage and death, stratified by neighborhood poverty. Of 31 542 individuals, 22.2% were non-Hispanic Black and 17.0% Hispanic. In high-poverty neighborhoods, non-Hispanic Black (44.4%) and Hispanic (47.7%) individuals, respectively, were more likely to be hospitalized (adjusted odds ratio [aOR], 1.2 [95% CI, 1.1-1.3]; and aOR, 1.3 [95% CI, 1.2-1.5]) and have emergency department visits (aOR, 1.3 [95% CI, 1.2-1.5] and aOR, 1.8 [95% CI, 1.5-2.0]) compared with non-Hispanic White individuals. In high poverty neighborhoods, non-Hispanic Black individuals with CHD had 1.7 times the odds of death compared with non-Hispanic White individuals in high-poverty neighborhoods (95% CI, 1.1-2.7). Racial and ethnic disparities in health care usage were similar in low-poverty neighborhoods, but disparities in death were attenuated (aOR for non-Hispanic Black, 1.2 [95% CI=0.9-1.7])., Conclusions: Racial and ethnic disparities in health care usage were found among individuals with CHD in low- and high-poverty neighborhoods, but mortality disparities were larger in high-poverty neighborhoods. Understanding individual- and community-level social determinants of health, including access to health care, may help address racial and ethnic inequities in health care usage and death among individuals with CHD.

Background: Various bioprosthetic valves are used off-label for pulmonary valve replacement (PVR), but there is no consensus on whether a particular valve is best for this application. Recently, the Inspiris Resilia valve (Edwards Lifesciences Inc) was approved for aortic valve replacement, and surgeons have begun using it for PVR. There is limited evidence on the performance of the Inspiris valve compared with other valves in the pulmonary position., Methods: This study reviewed all patients who underwent PVR with a size 19- to 29-mm Inspiris valve or Mosaic valve (Medtronic Inc) from 2007 to 2022 at Lucile Packard Children's Hospital Stanford (Palo Alto, CA). Midterm outcomes included freedom from moderate or severe pulmonary regurgitation (PR), a maximum Doppler gradient ≥36 mm Hg, and freedom from reintervention., Results: A total of 225 consecutive patients who underwent PVR with a size 19- to 29-mm Mosaic (n = 163) or Inspiris (n = 62) valve were included. There was no difference in baseline characteristics. Early postoperative gradients were low in both groups but higher in the Mosaic cohort, and neither group had more than mild PR on discharge. On univariable and multivariable analysis, Inspiris valves were significantly more likely to develop moderate or greater PR over time. There was no significant difference between the valves in freedom from reintervention or from a maximum gradient ≥36 mm Hg., Conclusions: Early and short-term gradients were similar in patients undergoing PVR with Inspiris and Mosaic valves, but significant PR was more common in patients who received an Inspiris valve. These preliminary findings suggest that the durability of the Inspiris valve in the pulmonary position may not be superior to that of other bioprosthetic valves used for PVR., (Copyright © 2024 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Introduction: As the adult Fontan population with Fontan associated liver disease continues to increase, more patients are being referred for transplantation, including combined heart and liver transplantation., Methods: We report updated mortality and morbidity outcomes after combined heart and liver transplant in a retrospective cohort series of 40 patients (age 14 to 49 years) with Fontan circulation across two centers from 2006-2022., Results: The 30-day, 1-year, 5-year and 10-year survival rate was 90%, 80%, 73% and 73% respectively. Sixty percent of patients met a composite comorbidity of needing either post-transplant mechanical circulatory support, renal replacement therapy or tracheostomy. Cardiopulmonary bypass time > 283 min (4.7 h) and meeting the composite comorbidity were associated with mortality by Kaplan Meier analysis., Conclusion: Further study to mitigate early mortality and the above comorbidities as well as the high risk of bleeding and vasoplegia in this patient population is warranted., (© 2024 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Patients with severe heart disease may have coexisting liver disease from various causes. The incidence of combined heart-liver transplant (CHLT) is increasing as more patients with congenital heart disease survive to adulthood and develop advanced heart failure with associated liver disease from chronic right-sided heart or Fontan failure. However, the criteria for CHLT have not been established. To address this unmet need, a virtual consensus conference was organized on June 10, 2022, endorsed by the American Society of Transplantation. The conference represented a collaborative effort by experts in cardiothoracic and liver transplantation from across the United States to assess interdisciplinary criteria for liver transplantation in the CHLT candidate, surgical considerations of CHLT, current allocation system that generally results in the liver following the heart for CHLT, and optimal post-CHLT management. The conference served as a forum to unify criteria between the different specialties and to forge a pathway for patients who may need dual organ transplantation. Due to the continuing shortage of available donor organs, ethical issues related to multiorgan transplantation were also debated. The findings and consensus statements are presented., (Copyright © 2023 American Society of Transplantation & American Society of Transplant Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Altered coagulation is a striking feature of COVID-19. Adult patients with congenital heart disease (ACHD) are prone to thromboembolic (TE) and bleeding complications., Objectives: The purpose of this study was to investigate the prevalence and risk factors for COVID-19 TE/bleeding complications in ACHD patients., Methods: COVID-19-positive ACHD patients were included between May 2020 and November 2021. TE events included ischemic cerebrovascular accident, systemic and pulmonary embolism, deep venous thrombosis, myocardial infarction, and intracardiac thrombosis. Major bleeding included cases with hemoglobin drop >2 g/dl, involvement of critical sites, or fatal bleeding. Severe infection was defined as need for intensive care unit, endotracheal intubation, renal replacement therapy, extracorporeal membrane oxygenation, or death. Patients with TE/bleeding were compared to those without events. Factors associated with TE/bleeding were determined using logistic regression., Results: Of 1,988 patients (age 32 [IQR: 25-42] years, 47% male, 59 ACHD centers), 30 (1.5%) had significant TE/bleeding: 12 TE events, 12 major bleeds, and 6 with both TE and bleeding. Patients with TE/bleeding had higher in-hospital mortality compared to the remainder cohort (33% vs 1.7%; P  < 0.0001) and were in more advanced physiological stage ( P  = 0.032) and NYHA functional class ( P  = 0.01), had lower baseline oxygen saturation ( P  = 0.0001), and more frequently had a history of atrial arrhythmia ( P  < 0.0001), previous hospitalization for heart failure ( P  < 0.0007), and were more likely hospitalized for COVID-19 ( P  < 0.0001). By multivariable logistic regression, prior anticoagulation (OR: 4.92; 95% CI: 2-11.76; P  = 0.0003), cardiac injury (OR: 5.34; 95% CI: 1.98-14.76; P  = 0.0009), and severe COVID-19 (OR: 17.39; 95% CI: 6.67-45.32; P  < 0.0001) were independently associated with increased risk of TE/bleeding complications., Conclusions: ACHD patients with TE/bleeding during COVID-19 infection have a higher in-hospital mortality from the illness. Risk of coagulation disorders is related to severe COVID-19, cardiac injury during infection, and use of anticoagulants., Competing Interests: The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2023 The Authors.)

Background: The use of transcatheter edge-to-edge repair (TEER) in patients with advanced heart failure has been shown to reduce hospitalizations and increase survival. As patients with Fontan circulations grow older, a significant proportion of them will develop severe atrioventricular (AV) valve regurgitation in the systemic ventricle. Conventional surgical repair and transplant carry high mortality risk for the adult Fontan patient with progressive heart failure., Case Summary: A 51-year-old female extracardiac Fontan patient developed severe AV valve regurgitation and progressive functional decline. Based on her operative risk for conventional surgical intervention or transplant, TEER using the Abbott MitraClip device was performed. The degree of mitral regurgitation was decreased from severe to moderate regurgitation., Discussion: This is the first known case describing the use of a successful TEER in an adult patient with an extracardiac Fontan. Given the increasing numbers of patients surviving into adulthood with a Fontan circulation, transcatheter interventions may provide an alternative treatment option to conventional surgeries and medical therapies., Competing Interests: Conflict of interest: Dr. Haeffele is a consultant and speaker for Edward Lifesciences. Dr. Rahul Sharma is a proctor and consultant for Abbott., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Background We sought to characterize health care usage for adolescents with congenital heart defects (CHDs) using population-based multisite surveillance data. Methods and Results Adolescents aged 11 to 18 years with ≥1 CHD-related diagnosis code and residing in 5 US sites were identified in clinical and administrative data sources for the years 2011 to 2013. Sites linked data on all inpatient, emergency department (ED), and outpatient visits. Multivariable log-binomial regression models including age, sex, unweighted Charlson comorbidity index, CHD severity, cardiology visits, and insurance status, were used to identify associations with inpatient, ED, and outpatient visits. Of 9626 eligible adolescents, 26.4% (n=2543) had severe CHDs and 21.4% had Charlson comorbidity index >0. At least 1 inpatient, ED, or outpatient visit was reported for 21%, 25%, and 96% of cases, respectively. Cardiology visits, cardiac imaging, cardiac procedures, and vascular procedures were reported for 38%, 73%, 10%, and 5% of cases, respectively. Inpatient, ED, and outpatient visits were consistently higher for adolescents with severe CHDs compared with nonsevere CHDs. Adolescents with severe and nonsevere CHDs had higher health care usage compared with the 2011 to 2013 general adolescent US population. Adolescents with severe CHDs versus nonsevere CHDs were twice as likely to have at least 1 inpatient visit when Charlson comorbidity index was low (Charlson comorbidity index =0). Adolescents with CHDs and public insurance, compared with private insurance, were more likely to have inpatient (adjusted prevalence ratio, 1.5 [95% CI, 1.3-1.7]) and ED (adjusted prevalence ratio, 1.6 [95% CI, 1.4-1.7]) visits. Conclusions High resource usage by adolescents with CHDs indicates a substantial burden of disease, especially with public insurance, severe CHDs, and more comorbidities.

There is limited information about durability of large diameter porcine bioprostheses implanted for pulmonary valve replacement (PVR). We studied patients who underwent surgical PVR from 2002-2019 with a stented porcine bioprosthetic valve (BPV) with a labeled size ≥27 mm. The primary outcome was freedom from reintervention. During the study period, 203 patients underwent PVR using a porcine BPV ≥27 mm, 94% of whom received a Mosaic valve (Medtronic Inc., Minneapolis, MN). Twenty patients underwent reintervention from 3.4-12.0 years after PVR: 5 surgical and 15 transcatheter PVR procedures. The indication for reintervention was regurgitation in 13 patients, stenosis in 2, mixed disease in 4, and endocarditis in 1. Estimated freedom from reintervention was 97±1% at 5 years and 82±4% at 10 years, and freedom from prosthesis dysfunction (moderate or severe regurgitation and/or a maximum Doppler gradient ≥50 mm Hg) over time was 91±2% at 5 years and 74±4% at 10 years. Younger age and smaller true valve diameter were associated with shorter freedom from reintervention, but valve oversizing was not. The durability of large stented porcine bioprostheses in the pulmonary position is generally excellent, particularly in adolescents and adults, similar to various other types of BPV. In the current study, relative valve size was not associated with valve longevity, although the low event-rate in this population was a limiting factor., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background The Centers for Disease Control and Prevention's Surveillance of Congenital Heart Defects Across the Lifespan project uses large clinical and administrative databases at sites throughout the United States to understand population-based congenital heart defect (CHD) epidemiology and outcomes. These individual databases are also relied upon for accurate coding of CHD to estimate population prevalence. Methods and Results This validation project assessed a sample of 774 cases from 4 surveillance sites to determine the positive predictive value (PPV) for identifying a true CHD case and classifying CHD anatomic group accurately based on 57 International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) codes. Chi-square tests assessed differences in PPV by CHD severity and age. Overall, PPV was 76.36% (591/774 [95% CI, 73.20-79.31]) for all sites and all CHD-related ICD-9-CM codes. Of patients with a code for complex CHD, 89.85% (177/197 [95% CI, 84.76-93.69]) had CHD; corresponding PPV estimates were 86.73% (170/196 [95% CI, 81.17-91.15]) for shunt, 82.99% (161/194 [95% CI, 76.95-87.99]) for valve, and 44.39% (83/187 [95% CI, 84.76-93.69]) for "Other" CHD anatomic group ( X 2 =142.16, P <0.0001). ICD-9-CM codes had higher PPVs for having CHD in the 3 younger age groups compared with those >64 years of age, ( X 2 =4.23, P <0.0001). Conclusions While CHD ICD-9-CM codes had acceptable PPV (86.54%) (508/587 [95% CI, 83.51-89.20]) for identifying whether a patient has CHD when excluding patients with ICD-9-CM codes for "Other" CHD and code 745.5, further evaluation and algorithm development may help inform and improve accurate identification of CHD in data sets across the CHD ICD-9-CM code groups.

We present the case of a 61-year-old man with tetralogy of Fallot postrepair and mechanical aortic valve replacement with an aortic root/ascending/arch aneurysm with chronic type A aortic dissection. He underwent uncomplicated aortic root and total arch replacement. Continued surveillance for aortic aneurysm is necessary in the tetralogy of Fallot population. ( Level of Difficulty: Intermediate. )., Competing Interests: The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2022 Published by Elsevier on behalf of the American College of Cardiology Foundation.)

Background: Morbidity and mortality increase as Fontan patients age into adulthood. Limited studies have examined cardiac magnetic resonance and echocardiographic parameters to predict death and transplantation in children after the Fontan operation. The aim of this study was to investigate echocardiographic parameters in adolescents and adults after Fontan operation, including myocardial mechanics such as classic-pattern dyssynchrony (CPD), as predictors of transplantation or death., Methods: In a cross-sectional retrospective study, strain analysis was performed on echocardiographic studies performed between 2001 and 2015 on 110 patients with single-ventricle physiology after the Fontan procedure. Strain curves were measured and visually assessed for the presence of CPD. The primary end point was death or transplantation after a follow-up period of 85 ± 35 months after echocardiography., Results: The median age at the date of echocardiography was 20 years (range, 3-45 years). Of 110 patients, 28 had undergone transplantation. During the study period, three patients died after transplantation and seven patients died without undergoing transplantation. CPD was seen in 16 and protein-losing enteropathy in 21 of 110 patients. On multivariate analysis, CPD (hazard ratio [HR], 9.4; 95% confidence interval [CI], 2.6-34.6), protein-losing enteropathy (HR, 10.6; 95% CI, 3.4-33.2), systolic blood pressure (HR, 0.954; 95% CI, 0.913-0.996), systolic/diastolic duration ratio (HR, 6.83; 95% CI, 1.33-35.0), and E-wave deceleration time (HR, 0.98; 95% CI, 0.97-0.99) were independently associated with the primary end point., Conclusions: CPD, protein-losing enteropathy, and systolic and diastolic ventricular dysfunction are significantly associated with transplantation or death in Fontan-operated patients. In selected patients, the presence of CPD may be a basis to investigate cardiac resynchronization therapy as a treatment strategy., (Copyright © 2022 American Society of Echocardiography. All rights reserved.)