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Introduction: Cardiac allograft vasculopathy (CAV) limits long-term survival in heart transplant (HTx) recipients. The use of biomarkers in CAV surveillance has been studied, but none are used in clinical practice. The predictive value of high-sensitivity troponin I (hsTnI) has not been extensively investigated in HTx recipients., Methods: HTx patients undergoing surveillance coronary angiograms and enrolled in the Emory Cardiovascular Biobank had plasma hsTnI measured. CAV grade was assessed using ISHLT nomenclature. Multivariable cumulative link mixed modeling was performed to determine association between hsTnI level and CAV grade. Patients were followed for adverse outcomes over a median 10-year period. Kaplan-Meier survival analysis and Cox proportional hazard modeling were performed., Results: Three hundred and seventy-two angiograms were analyzed in 156 patients at a median 8.9 years after transplant. hsTnI levels were positively correlated with concurrent CAV grade after adjustment for age, age at transplant, sex, BMI, hypertension, diabetes, hyperlipidemia, estimated glomerular filtration rate, and history of acute cellular rejection (p = .016). In an adjusted Cox proportional hazard model, initial hsTnI level above the median (4.9 pg/mL) remained a predictor of re-transplantation or death (hazard ratio 1.82; 95% confidence interval 1.16-2.90; p = .01)., Conclusion: An elevated hsTnI level reflects severity of CAV and is associated with poor long-term outcomes in patients with HTx., (© 2023 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Introduction: International Classification of Diseases (ICD) codes recorded in administrative data are often used to identify congenital heart defects (CHD). However, these codes may inaccurately identify true positive (TP) CHD individuals. CHD surveillance could be strengthened by accurate CHD identification in administrative records using machine learning (ML) algorithms., Methods: To identify features relevant to accurate CHD identification, traditional ML models were applied to a validated dataset of 779 patients; encounter level data, including ICD-9-CM and CPT codes, from 2011 to 2013 at four US sites were utilized. Five-fold cross-validation determined overlapping important features that best predicted TP CHD individuals. Median values and 95% confidence intervals (CIs) of area under the receiver operating curve, positive predictive value (PPV), negative predictive value, sensitivity, specificity, and F1-score were compared across four ML models: Logistic Regression, Gaussian Naive Bayes, Random Forest, and eXtreme Gradient Boosting (XGBoost)., Results: Baseline PPV was 76.5% from expert clinician validation of ICD-9-CM CHD-related codes. Feature selection for ML decreased 7138 features to 10 that best predicted TP CHD cases. During training and testing, XGBoost performed the best in median accuracy (F1-score) and PPV, 0.84 (95% CI: 0.76, 0.91) and 0.94 (95% CI: 0.91, 0.96), respectively. When applied to the entire dataset, XGBoost revealed a median PPV of 0.94 (95% CI: 0.94, 0.95)., Conclusions: Applying ML algorithms improved the accuracy of identifying TP CHD cases in comparison to ICD codes alone. Use of this technique to identify CHD cases would improve generalizability of results obtained from large datasets to the CHD patient population, enhancing public health surveillance efforts., (© 2023 Wiley Periodicals LLC.)

Background: Adolescent and young adult (AYA) solid organ transplant recipients experience worsening medical outcomes during transition to adult healthcare. Current understanding and definitions of transition success emphasize first initiation of appointment attendance in adult healthcare; however, declines in attendance over time after transfer remain possible, particularly as AYAs are further removed from their pediatric provider and assume greater independence in their care., Methods: The current study assessed health-care utilization, medical outcomes, and transition success among 49 AYA heart, kidney, or liver recipients recently transferred to adult healthcare. Differences in outcomes were examined along two transition success criteria: (1) initial engagement in adult healthcare within 6 or 12 months of last pediatric appointment and (2) retention in adult healthcare over 3 years following last pediatric appointment. Growth curve modeling examined change in attendance over time., Results: Successful retention in adult healthcare was significantly related to more improved clinical outcomes, including decreased number and duration of hospitalizations and greater medication adherence, as compared to initial engagement. Significant declines in appointment attendance over 3 years were noted, and individual differences in declines were not accounted for by age at transfer or time since transplant., Conclusions: Findings underscore support for AYAs after transfer, as significant declines in attendance were noted after initiating adult care. Clinical care teams should examine transition success longitudinally to address changes in health-care utilization and medical outcomes. Attention to interventions and administrative support aimed at maintaining or increasing attendance and identifying risk factors and intervention for unsuccessful transition is warranted., (© 2022 Wiley Periodicals LLC.)

Background: Little is known about patient choice in treatment of eosinophilic esophagitis (EoE)., Aim: Determine motivators and barriers to using common EoE therapies and describe patient-reported shared decision making (SDM) and satisfaction with treatment., Methods: We developed and administered a Web-based survey on factors influencing EoE treatment choice, SDM, and satisfaction. Adults with EoE and adult caregivers of pediatric EoE patients were recruited via patient advocacy groups and at two centers. Descriptive statistics of multiple response questions and multivariable logistic regression were performed to identify predictors of SDM and satisfaction with treatment., Results: A total of 243 adults (mean age 38.7 years) and 270 adult caregivers of children (mean age 9.5 years) completed the survey. Preventing worsening disease was the most common motivator to treat EoE. Barriers to topical steroids were potential side effects, cost, and preferring a medication-free approach. Inconvenience and quality of life were barriers to diet. Potential adverse events, discomfort, and cost were barriers to dilation. Nearly half (42%) of patients experienced low SDM, but those followed by gastroenterologists were more likely to experience greater SDM compared to non-specialists (OR 1.81; 95% CI 1.03-3.15). Patients receiving more SDM were more satisfied with treatment, regardless of provider or treatment type (OR 2.62, 95% CI 1.76-3.92)., Conclusions: Patients with EoE pursue treatment mostly to prevent worsening disease. Common barriers to treatment are inconvenience and financial costs. SDM is practiced most by gastroenterologists, but nearly half of patients do not experience SDM, indicating a substantial area of need in EoE.

Objectives: Clinical features of eosinophilic esophagitis (EoE) have been well-described in the literature, however, characterization of features experienced by patients with other eosinophilic gastrointestinal diseases (EGIDs) is lacking. Using data collected from a patient contact registry, we sought to characterize and contrast patient-reported gastrointestinal and extragastrointestinal symptoms and comorbidities in non-EoE EGIDs, including eosinophilic gastritis, gastroenteritis and colitis, relative to EoE., Methods: We conducted a cross-sectional study of contact registry data collected from 2015 to 2018. Statistical comparisons were made using chi-square (categorical measures) and the Mann-Whitney U test (continuous measures). Multivariable analyses were used to evaluate associations between treatment and feelings of isolation., Results: Of the 715 reporting an EGID diagnosis (n = 525 EoE; n = 190 non-EoE EGID), a higher proportion of those with a non-EoE EGID reported more frequent specific and nonspecific gastrointestinal symptoms, including nausea, abdominal pain, diarrhea, constipation, and bloating (P < 0.01 for all). Participants with a non-EoE EGID were more likely to report higher frequency of fatigue, isolation, and deep muscle or joint pain (P < 0.01 for all). Specific food elimination and elemental formula treatments were associated with increased odds of more frequent (at least weekly) feelings of isolation for participants with EoE (adjusted odds rtaio [aOR]: 2.4; 95% confidence interval [CI]: 1.5--4.1 for specific food elimination and adjusted OR: 1.9; 95% CI: 1.2--3.3 for elemental formula)., Conclusions: Significant differences exist in the symptoms and comorbidities experienced between those with EoE versus non-EoE EGIDs. Additional investigation is needed to elucidate the factors that may contribute to the high disease burden of these poorly understood conditions.

With increasing survival trends for children and adolescents with congenital heart defects (CHD), there is a growing need to focus on transition from pediatric to adult specialty cardiac care. To better understand parental perspectives on the transition process, a survey was distributed to 451 parents of adolescents with CHD who had recent contact with the healthcare system in Georgia (GA) and New York (NY). Among respondents, 90.7% reported excellent, very good or good health-related quality of life (HRQoL) for their adolescent. While the majority of parents (77.8%) had been told by a provider about their adolescent's need to transition to adult specialty cardiac care, most reported concerns about transitioning to adult care. Parents were most commonly concerned with replacing the strong relationship with pediatric providers (60.7%), locating an appropriate adult provider (48.7%), and accessing adult health insurance coverage (43.6%). These findings may offer insights into transition planning for adolescents with CHD.

Importance: Guidelines endorse routine coronary angiography and percutaneous coronary intervention (PCI) to screen for and treat cardiac allograft vasculopathy in heart transplant recipients. However, the current Appropriate Use Criteria for Revascularization (AUC-R) do not recognize prior heart transplant as a unique PCI indication. Whether this affects rates of rarely appropriate (RA) PCIs is unknown., Objective: To assess the rate of RA PCI procedures in heart transplant recipients and how it pertains to hospital PCI appropriateness metrics and pay-for-performance scorecards., Design, Setting, and Participants: This observational study used National Cardiovascular Data Registry CathPCI Registry data on all patients undergoing elective PCIs from 96 Medicare-approved heart transplant centers from quarter 3 of 2009 to quarter 2 of 2017. The data were analyzed in July 2018., Exposures: Prior heart transplant., Main Outcomes and Measures: Rates of RA elective PCIs in heart transplant recipients compared with nonrecipients and hospital rates of RA PCI before vs after exclusion of heart transplant recipients using paired t tests. In a subset of heart transplant centers participating in the Anthem Blue Cross and Blue Shield's Quality-In-Sights Hospital Incentive Program (Q-HIP), we compared the change in Q-HIP scorecards before vs after excluding heart transplant recipients., Results: Of 168 802 participants, 123 124 (72.9%) were men, 137 457 were white, and the mean (SD) age was 66.3 (11.4) years. Of 168 802 elective PCIs performed in heart transplant centers, 1854 (1.1%) were for heart transplant recipients. Heart transplant recipients were less likely to have ischemic symptoms (14.6% vs 61.4%, P < .001), had lower rates of antecedent stress testing (15.0% vs 58.4%, P < .001), and had higher RA PCI rates (66.0% vs 16.9%, P < .001) compared with nonrecipients. In heart transplant centers, the absolute difference in RA rates (before vs after excluding transplant recipients) was directly associated with the proportion of PCIs performed in heart transplant recipients (r = 0.91; P < .001). In the subset of heart transplant centers participating in Q-HIP during the 2016 and 2017 calendar years, 8 of 20 (40%) and 8 of 16 centers (50%), respectively, could have benefited from a change in their Q-HIP scorecards if their RA PCI rates excluded transplant recipients., Conclusions and Relevance: Two-thirds of PCIs in heart transplant recipients were deemed RA by the AUC-R. The failure of the AUC-R to consider prior heart transplant as a unique PCI indication may lead to inflated RA PCI rates with the potential for affecting quality reporting and pay-for-performance metrics in heart transplant centers.

Background: As the population of adults with congenital heart disease (CHD) grows, cardiologists continue to encounter patients with complex anatomies that challenge the standard treatment of care. Single ventricle Fontan palliated patients are the most complex within CHD, with a high morbidity and mortality burden. Factors driving this early demise are largely unknown., Methods and Results: We analyzed biomarker expression in 44 stable Fontan outpatients (29.2 ± 10.7 years, 68.2% female) seen in the outpatient Emory Adult Congenital Heart Center and compared them to 32 age, gender and race matched controls. In comparison to controls, Fontan patients had elevated levels of multiple cytokines within the inflammatory pathway including Tumor Necrosis Factor-α (TNF-α) (p < 0.001), Interleukin-6 (IL-6) (p < 0.011), Growth Derived Factor-15 (GDF-15) (p < 0.0001), β2-macroglobulin, (p = 0.0006), stem cell mobilization: Stromal Derived Factor-1∝ (SDF-1α) (p = 0.006), extracellular matrix turnover: Collagen IV (p < 0.0001), neurohormonal activation: Renin (p < 0.0001), renal dysfunction: Cystatin C (p < 0.0001) and Urokinase Receptor (uPAR) (p = 0.022), cardiac injury: Troponin-I (p < 0.0004) and metabolism: Adiponectin (p = 0.0037). Within 1 year of enrollment 50% of Fontan patients had hospitalizations, arrhythmias or worsening hepatic function. GDF-15 was significantly increased in Fontan patients with clinical events (p < 0.0001). In addition, GDF-15 moderately correlated with longer duration of Fontan (r = 0.55, p = 0.01) and was elevated in atriopulmonary (AP) Fontan circulation. Finally, in a multivariate model, VEGF-D and Collagen IV levels were found to be associated with a change in MELDXI, a marker of liver dysfunction., Conclusion: Multiple clinical and molecular biomarkers are upregulated in Fontan patients, suggesting a state of chronic systemic dysregulation., Competing Interests: Declaration of competing interest There is no conflict of interest associated with the study findings reported in this manuscript., (Copyright © 2020 Elsevier B.V. All rights reserved.)

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., (Copyright © 2020 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

The prevalence, co-morbidities, and healthcare utilization in adolescents with congenital heart defects (CHDs) is not well understood. Adolescents (11 to 19 years old) with a healthcare encounter between January 1, 2008 (January 1, 2009 for MA) and December 31, 2010 with a CHD diagnosis code were identified from multiple administrative data sources compiled at 3 US sites: Emory University, Atlanta, Georgia (EU); Massachusetts Department of Public Health (MA); and New York State Department of Health (NY). The estimated prevalence for any CHD was 4.77 (EU), 17.29 (MA), and 4.22 (NY) and for severe CHDs was 1.34 (EU), 3.04 (MA), and 0.88 (NY) per 1,000 adolescents. Private or commercial insurance was the most common insurance type for EU and NY, and Medicaid for MA. Inpatient encounters were more frequent in severe CHDs. Cardiac co-morbidities included rhythm and conduction disorders at 20% (EU), 46% (MA), and 9% (NY) as well as heart failure at 3% (EU), 15% (MA), and 2% (NY). Leading noncardiac co-morbidities were respiratory/pulmonary (22% EU, 34% MA, 16% NY), infectious disease (17% EU, 22% MA, 20% NY), non-CHD birth defects (12% EU, 23% MA, 14% NY), gastrointestinal (10% EU, 28% MA, 13% NY), musculoskeletal (10% EU, 32% MA, 11% NY), and mental health (9% EU, 30% MA, 11% NY). In conclusion, this study used a novel approach of uniform CHD definition and variable selection across administrative data sources in 3 sites for the first population-based CHD surveillance of adolescents in the United States. High resource utilization and co-morbidities illustrate ongoing significant burden of disease in this vulnerable population., (Copyright © 2019 The Authors. Published by Elsevier Inc. All rights reserved.)

Objective: Transfer of congenital heart disease care from the pediatric to adult setting has been identified as a priority and is associated with better outcomes. Our objective is to determine what percentage of patients with congenital heart disease transferred to adult congenital cardiac care., Design: A retrospective cohort study., Setting: Referrals to a tertiary referral center for adult congenital heart disease patients from its pediatric referral base., Patients: This resulted in 1514 patients age 16-30, seen at least once in three pediatric Georgia health care systems during 2008-2010., Interventions: We analyzed for protective factors associated with age-appropriate care, including distance from referral center, age, timing of transfer, gender, severity of adult congenital heart disease, and comorbidities., Outcome Measures: We analyzed initial care by age among patients under pediatric care from 2008 to 2010 and if patients under pediatric care subsequently transferred to an adult congenital cardiologist in this separate pediatric and adult health system during 2008-2015., Results: Among 1514 initial patients (39% severe complexity), 24% were beyond the recommended transfer age of 21 years. Overall, only 12.1% transferred care to the referral affiliated adult hospital. 90% of these adults that successfully transferred were seen by an adult congenital cardiologist, with an average of 33.9 months between last pediatric visit and first adult visit. Distance to referral center contributed to delayed transfer to adult care. Those with severe congenital heart disease were more likely to transfer (18.7% vs 6.2% for not severe)., Conclusion: Patients with severe disease are more likely to transfer to adult congenital heart disease care than nonsevere disease. Most congenital heart disease patients do not transfer to adult congenital cardiology care with distance to referral center being a contributing factor. Both pediatric and adult care providers need to understand and address barriers in order to improve successful transfer., (© 2019 Wiley Periodicals, Inc.)

Purpose: Although congenital heart defects (CHD) are one of the most common types of birth defects in the United States, subnational prevalence estimates beyond early childhood are limited., Methods: We used capture-recapture methodology and logistic regression to estimate CHD prevalence per 1000 residents as of January 1, 2010, separately for adolescents and adults treated and living within five metropolitan Atlanta, Georgia counties, during 2008-2010., Results: Data sources differed by age. Adolescents (n = 1621, aged 11-20 years) and adults (n = 3176, aged 21-64 years) were captured from at least one source. We estimated CHD prevalence to be 7.85 per 1000 adolescents (estimated n = 3718 [95% CI: 3471-4004]) and 6.08 per 1000 adults (estimated n = 12,969 [95% CI: 13,873-18,915]). When we included persons found in age-inappropriate sources, prevalence estimates increased to 11 per 1000 adolescents and 6.5 per 1000 adults., Conclusions: This method for obtaining subnational prevalence estimates provided reasonable prevalence results and identified needs for service improvement. Only one half of adolescents and one-quarter of adults with CHD were in health care within a 3-year time frame, suggesting need for better access to health insurance, transition care, and an increased number of physicians specializing in CHD care., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Heart failure remains the most common cause of morbidity and mortality in adults with congenital heart disease (CHD). Although gender-specific outcomes are not robust, it seems that women with CHD may be more affected by late heart failure (HF) than men. A specialized and experienced adult CHD team is required to care for these women as they age, including assessment for reversible causes of HF and in the management of pregnancy, labor, and delivery., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: The prevalence of moderate or complex (moderate-complex) congenital heart defects (CHDs) among adults is increasing due to improved survival, but many patients experience lapses in specialty care or their CHDs are undocumented in the medical system. There is, to date, no efficient approach to identify this population., Objective: To develop and assess the performance of a risk score to identify adults aged 20-60 years with undocumented specific moderate-complex CHDs from electronic health records (EHR)., Methods: We used a case-control study (596 adults with specific moderate-complex CHDs and 2384 controls). We extracted age, race/ethnicity, electrocardiogram (EKG), and blood tests from routine outpatient visits (1/2009 through 12/2012). We used multivariable logistic regression models and a split-sample (4: 1 ratio) approach to develop and internally validate the risk score, respectively. We generated receiver operating characteristic (ROC) c-statistics and Brier scores to assess the ability of models to predict the presence of specific moderate-complex CHDs., Results: Out of six models, the non-blood biomarker model that included age, sex, and EKG parameters offered a high ROC c-statistic of 0.96 [95% confidence interval: 0.95, 0.97] and low Brier score (0.05) relative to the other models. The adult moderate-complex congenital heart defect risk score demonstrated good accuracy with 96.4% sensitivity and 80.0% specificity at a threshold score of 10., Conclusions: A simple risk score based on age, sex, and EKG parameters offers early proof of concept and may help accurately identify adults with specific moderate-complex CHDs from routine EHR systems who may benefit from specialty care.

Background: Improved treatment of congenital heart defects (CHDs) has increased survival of persons with CHDs; however, no U.S. population-based systems exist to assess prevalence, healthcare utilization, or longer-term outcomes among adolescents and adults with CHDs., Methods: Novel approaches identified individuals aged 11-64 years who received healthcare with ICD-9-CM codes for CHDs at three sites: Emory University in Atlanta, Georgia (EU), Massachusetts Department of Public Health (MA), New York State Department of Health (NY) between January 1, 2008 (2009 for MA) and December 31, 2010. Case-finding sources included outpatient clinics; Medicaid and other claims data; and hospital inpatient, outpatient, and emergency visit data. Supplemental information came from state vital records (EU, MA), and birth defects registries (EU, NY). Demographics and diagnostic and procedural codes were linked, de-duplicated, and shared in a de-identified dataset. Cases were categorized into one of five mutually exclusive CHD severity groups; non-cardiac comorbidity codes were grouped into broad categories., Results: 73,112 individuals with CHD codes in healthcare encounters were identified. Primary data source type varied: clinics (EU, NY for adolescents), claims (MA), hospital (NY for adults). There was a high rate of missing data for some variables and data varied in format and quality. Some diagnostic codes had poor specificity for CHD ascertainment., Conclusions: To our knowledge, this is the first population-based, multi-site CHD surveillance among adolescents and adults in the U.S. Identification of people living with CHDs through healthcare encounters using multiple data sources was feasible, though data quality varied and linkage/de-duplication was labor-intensive., (© 2018 Wiley Periodicals, Inc.)

Background: The number of adult congenital heart disease (ACHD) patients requiring heart transplantation (HT) continues to grow, and if they survive the first year after transplant, their long-term survival is at least equivalent to non-ACHD patients. The 1-year survival of ACHD patients with HT remains lower than non-ACHD patients. We evaluated the affect of transplant center volume on 1-year survival of ACHD patients. We analyzed United Network of Organ Sharing patients (age ≥18 years) who underwent their first orthotopic HT between January 1, 2000, and December 31, 2015, to assess the association between transplant center volume and 1-year survival of ACHD patients., Results: We identified 827 ACHD patients at 113 centers who underwent HT during the study period. The average age of the recipients and donors was 36 ± 13 years (60% men and 84% Caucasian) and 28 ± 11 (63% men and 66% Caucasian), respectively. Of the ACHD patients undergoing HT, 27% (n = 60) were done at low-volume centers, 30% (n = 10) were reported at high-volume centers, and the remaining (n = 43) were at medium-volume centers. A total of 96 patients died within 30 days, including 37 (16.7%) at low-volume, 37 (10.2%) at medium-volume, and 22 (9.0%) at high-volume centers (p = 0.019). The average unadjusted Kaplan-Meier 30-day survival at low-volume centers was 83% ± 2%, which was significantly lower than medium-volume (90% ± 1%) and high-volume (91% ± 2%) centers (log-rank p < 0.05). Within 1 year, 154 patients had died, including 56 (36.4%) at low-volume, 60 (38.9%) at medium-volume, and 38 (24.7%) at high-volume centers (p = 0.011). Average unadjusted Kaplan-Meier 1-year survival at low-volume centers was 75% ± 3%, which was significantly lower than medium-volume (83% ± 2%) and high-volume (84% ± 2%) centers (log-rank p < 0.05)., Conclusions: The 30-day and 1-year survival of ACHD patients undergoing HT is partly influenced by overall transplant center volume and, potentially, volume of ACHD HTs, with low-volume centers performing poorly relative to medium-volume and high-volume centers. The role of peri-operative care and multidisciplinary management in improving survival at low-volume centers required further investigations., (Copyright © 2018 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Introduction: Eosinophilic gastrointestinal disorders (EGIDs) are a rare but emerging healthcare problem. Patient advocacy groups (PAGs) have an important role in representing the EGID community, and serve as valuable research partners. By leveraging the partnership between medical researchers and PAGs, we examined the unmet needs and barriers to care perceived by individuals affected by EGIDs. Next, we examined if these varied between adult EGID patients and adult caregivers of children with EGID., Methods: Adult EGID patients and adult caregivers of children (<18 years) with EGIDs participated in this study. PAGs conducted focus groups comprised of individuals affected by EGIDs to identify domains and questions meaningful to the EGID community and this information was used to develop an online REDCap survey. The survey consisted of 58 questions across medical, healthcare, social, and emotional impact domains. It was distributed via the PAGs' web-based platforms. Demographic data, and responses to questions on a six-point Likert scale were collected and analyzed., Results: Of the 361 responses analyzed, 90 (25%) were from adult EGID patients and 271 (75%) were from adult caregivers. Of the applicable responses, in the medical domain only 19% of participants indicated that repeated endoscopies to monitor response to treatment was convenient. In the healthcare domain, 67% indicated that lack of insurance coverage for elemental formula was a barrier. In the social domain, only 5% of respondents reported adequate awareness of EGIDs in schools. In the emotional domain, 64% had experienced significant stress due to EGID related out-of-pocket costs. Multivariate logistic regression revealed that some of these responses varied between adult EGID patients and adult caregivers of children with EGID. The respondents indicated highest priority for improvement in the medical domain compared to other domains., Conclusions: Individuals affected by EGIDs have a constellation of complex unmet needs and perceived barriers across medical, healthcare, social and emotional domains. Addressing unmet needs in the medical domain is relatively more important for the EGID community. Understanding unmet needs and barriers will likely help design improved patient-centered EGID care paradigms., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Background: Despite improvements in outcomes after heart transplantation, black recipients have worse survival compared with non-black recipients. The source of such disparate outcomes remains largely unknown. We hypothesize that a propensity to generate de-novo donor-specific antibodies (dnDSA) and subsequent antibody-mediated rejection (AMR) may account for racial differences in sub-optimal outcomes after heart transplant. In this study we aimed to determine the role of dnDSA and AMR in racial disparities in post-transplant outcomes., Methods: This study was a single-center, retrospective analysis of 137 heart transplant recipients (81% male, 48% black) discharged from Emory University Hospital. Patients were classified as black vs non-black for the purpose of our analysis. Kaplan-Meier and Cox regression analyses were used to evaluate the association between race and selected outcomes. The primary outcome was the development of dnDSA. Secondary outcomes included treated AMR and a composite of all-cause graft dysfunction or death., Results: After 3.7 years of follow-up, 39 (28.5%) patients developed dnDSA and 19 (13.8%) were treated for AMR. In multivariable models, black race was associated with a higher risk of developing dnDSA (hazard ratio [HR] 3.65, 95% confidence interval [CI] 1.54 to 8.65, p = 0.003) and a higher risk of treated AMR (HR 4.86, 95% CI 1.26 to 18.72, p = 0.021) compared with non-black race. Black race was also associated with a higher risk of all-cause graft dysfunction or death in univariate analyses (HR 2.10, 95% CI 1.02 to 4.30, p = 0.044). However, in a multivariable model incorporating dnDSA, black race was no longer a significant risk factor. Only dnDSA development was significantly associated with all-cause graft dysfunction or death (HR 4.85, 95% CI 1.89 to 12.44, p = 0.001)., Conclusion: Black transplant recipients are at higher risk for the development of dnDSA and treated AMR, which may account for racial disparities in outcomes after heart transplantation., (Copyright © 2018 International Society for the Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Objective: Although the ICD-9-CM code 745.5 is widely used to indicate the presence of a secundum atrial septal defect (ASD), it is also used for patent foramen ovale (PFO) which is a normal variant and for "rule-out" congenital heart disease (CHD). The ICD-10-CM code Q21.1 perpetuates this issue. The objective of this study was to assess whether code 745.5 in isolation or in combination with unspecified CHD codes 746.9 or 746.89 miscodes for CHD, and if true CHD positives decrease with age., Design: Echocardiograms of patients with an ICD-9-CM code of 745.5 in isolation or in combination with unspecified CHD codes 746.9 or 746.89 were reviewed to validate the true incidence of an ASD. This observational, cross-sectional record review included patients between 11 and 64 years of age., Results: Medical charts and echocardiograms of 190 patients (47.9% males) were reviewed. The number of falsely coded patients with 745.5 (no ASD) was high (76.3%). Forty-five (23.7%) patients had a true ASD. Among the 145 patients without an ASD, 100 (52.6%) were classified as having a PFO, 37 (19.5%) had a normal non-CHD echocardiogram, and 8 (4.2%) had some other CHD anomaly. The likelihood that 745.5 coded for a true ASD was higher in children aged 11-20 (64.3%) than adults aged 21-64 years (20.6%)., Conclusions: This validation study demonstrates that 745.5 performed poorly across all ages. As 745.5 is widely used in population-level investigations and ICD-10-CM perpetuates the problem, future analyses utilizing CHD codes should consider separate analysis of those identified only through code 745.5., (© 2017 Wiley Periodicals, Inc.)

Over the past decade, as the majority of patients with single ventricle anatomy who have undergone the Fontan operation reach adulthood, a newly recognized disease process, Fontan-associated liver disease (FALD), has emerged. FALD is an extracardiac complication that may lead to substantial comorbid disease and premature mortality. The risk factors, pathophysiology, longitudinal consequences, and therapeutic options related to FALD remain poorly defined. Although we recognize that Fontan circulatory properties are associated with extracardiac organ dysfunction, numerous gaps in our understanding of the nature of this relationship exist. Such extracardiac manifestations, in addition to other late complications of the circulation, can significantly affect quality of life and healthcare use. Therefore, to initiate a formal evaluation of FALD, the American College of Cardiology (ACC) sponsored a stakeholders meeting on October 1 to 2, 2015, in Washington, DC. The goal of the meeting was to bring together subspecialty experts in the fields of adult and pediatric hepatology, congenital cardiology (adult congenital and pediatric cardiology), heart failure/transplant, epidemiology, and cardiothoracic surgery, as well as patient advocates, patients, parents of children and young adults who have had the Fontan procedure, and research organizations and societies to discuss the current state of FALD. Topics included gaps in knowledge, optimal care, research opportunities and barriers, and sound practices to guide providers, patients, and families. This report summarizes findings from the stakeholders meeting and seeks to establish a platform for understanding and addressing FALD., (Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Antibody-mediated rejection (AMR) resulting from de novo donor-specific antibodies (dnDSA) leads to adverse outcomes following heart transplantation (HTx). It remains unclear what role dnDSA to specific HLA antigens play in adverse outcomes. This study compares outcomes in patients developing dnDSA to DQ antigens with those developing non-DQ dnDSA and those free from dnDSA., Methods: The present study was a single-center, retrospective analysis of 122 consecutive HTx recipients. The primary outcome was a composite of death or graft dysfunction., Results: After 3.3 years of follow-up, 31 (28%) patients developed dnDSA. Mean time to dnDSA was 539 days. Of 31 patients, 19 developed DQ antibodies and 12 developed non-DQ antibodies. Compared to non-DQ dnDSA, DQ antibodies presented with higher MFI values (P=.001) were more likely persistent (P=.001) and appeared later post-HTx (654 vs 359 days, P=.035). In a multivariable analysis, DQ dnDSA was associated with increased risk of the primary endpoint (HR 6.15, 95% CI 2.57-14.75, P=.001), whereas no increased risk was seen with non-DQ dnDSA (P=.749)., Conclusions: dnDSA to DQ antigens following HTx are associated with increased risk of death and graft dysfunction., (© 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Objective: Adults with congenital heart disease (CHD) are at increased risk of psychological disorders and cognitive deficiencies due to structural/acquired neurological abnormalities and neurodevelopmental disorders as children. However, limited information is known about the neuropsychological functioning of adults with CHD. This study screened neuropsychological abilities and explored group differences related to cardiac disease severity and neurological risk factors in adults with CHD., Design: Participants completed brief neuropsychological testing. Information about neurobehavioral and psychological symptoms, employment, education, and disability were also collected from the patient and a family member., Results: Forty-eight participants with adult CHD completed neuropsychological testing. Visuospatial skills and working memory were worse than expected compared to the typical population. Frequency of neurological comorbidities (e.g., stroke, seizures) was higher in those with more severe heart disease (e.g., single ventricle or cyanotic disease), and executive functioning was weaker in those with neurological comorbidities. Those with more severe heart disease were more likely to be unemployed and to receive disability benefits, but educational attainment did not differ. Those who received disability performed worse on tasks of executive functioning., Conclusions: Findings suggest concerns about neuropsychological functioning that need to be more comprehensively assessed in adults with CHD. Understanding the cognitive limitations of this aging population can help guide access to resources, transition of care, and medical care engagement, thus improving quality of care and quality of life., (© 2016 Wiley Periodicals, Inc.)

The Emory Adult Congenital Heart (Emory University, Atlanta, GA) program was founded in 2001. In 2004, the surgical component transitioned from a pediatric facility to an adult facility. The aim of this article is characterize the program as a whole, outline changes in the program, and discuss the challenges of the transition process. Between 2001 and 2015, changes in program structure and personnel were evaluated. There has been significant growth of the program between 2001 and 2015. There are currently 19 half-day clinics per week, with 2,700 clinic visits per year. There are six cardiologists, three congenital cardiac surgeons, two sonographers, one advanced practice provider, and one social worker dedicated to the program. There are Accreditation Council for Graduate Medical Education-accredited adult congenital cardiology and congenital cardiac surgery fellowships. One thousand forty-four operations were performed between 2001 and 2015. There were 828 open-heart operations, of which 581 (70%) were re-operations. Over the study period, the number of yearly operations increased from 30 to 119, and the mean age at surgery increased from 22 to 35 years. Over time, more of the operations were performed at the adult hospital: increasing from 3% in 2001 to 82% in 2015, and more of the operations were performed by congenital cardiac surgeons: 87% (114 of 131) before the 2004 transition to 97% (881 of 913) afterward. The Emory Adult Congenital Heart program has undergone significant growth and change, including transition of the surgical component from the pediatric to the adult facility. While numerous obstacles have been overcome and great progress has been made, additional challenges remain., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Adult patients with CHD often require complex operations, and indications for particular aspects of the operation are sometimes unclear. The aims of our study were as follows: to characterise concomitant procedures performed during adult congenital cardiac surgery, and to better define the risk involved with performing concomitant procedures during a single operation., Methods: We retrospectively studied 458 adult congenital cardiac surgical patients. Major procedures were characterised as aortic, mitral, pulmonary, tricuspid, septal defect, single ventricle, transplant, and others. We constructed logistic regression models to assess the risk for mortality, major adverse event, and prolonged length of stay., Results: A total of 362 operations involved a single major procedure, whereas 96 involved concomitant procedures. Performing concomitant procedures increased mortality (7.3 versus 2.5%), major adverse events (21.8 versus 14.9%), and prolonged length of stay (29.2 versus 17.1%). The added risks of concomitant procedures on mortality, major adverse event, and prolonged length of stay were 2.9 (95% CI 1.0-8.5, p=0.05), 1.9 (95% CI 1.1-3.3, p=0.02), and 2.4 (95% CI 1.4-4.1, p=0.003), respectively. There were 200 patients with conotruncal anomalies who underwent pulmonary valve surgery. In this subset, the added risks of concomitant procedures in addition to pulmonary valve surgery on mortality, major adverse events, and prolonged length of stay were 6.6 (95% CI 1.2-37.3, p=0.03), 2.8 (95% CI 1.2-6.6, p=0.03), and 3.3 (95% CI 1.5-7.4, p=0.005), respectively., Conclusion: Concomitant procedures performed during adult congenital heart surgery increase risk. Awareness of this risk may improve surgical decision making and outcomes.

Patients with single ventricle heart disease often undergo staged surgical palliation, ultimately resulting in Fontan anatomy and physiology. Long-term consequences include cirrhosis of the liver, protein-losing enteropathy, and premature death. Elevated central venous pressure and venous congestion transmitted to the abdominal viscera have been implicated in the aetiology of many of these complications. We present a novel operation directed at protecting the liver and intestines by excluding the splanchnic venous return from the Fontan pathway. Instead of exposure to elevated Fontan pressures, the liver and intestines will be exposed to lower common atrial pressures. We hope that this modification will minimise the abdominal complications of Fontan anatomy and physiology.

Background: Adults with congenital heart defects (ACHD) face long-term complications related to prior surgery, abnormal anatomy, and acquired cardiovascular conditions. Although self-care is an important part of chronic illness management, few studies have explored self-care in the ACHD population. The purpose of this study is to describe self-care and its antecedents in the ACHD population., Methods: Persons with moderate or severe ACHD (N=132) were recruited from a single ACHD center. Self-care (health maintenance behaviors, monitoring and management of symptoms), and potential antecedents including sociodemographic and clinical characteristics, ACHD knowledge, behavioral characteristics (depressive symptoms and self-efficacy), and family-related factors (parental overprotection and perceived family support) were collected via self-report and chart review. Multiple regression was used to identify antecedents of self-care maintenance, monitoring, and management., Results: Only 44.7%, 27.3%, and 23.3% of participants performed adequate levels of self-care maintenance, monitoring and management, respectively. In multiple regression analysis, self-efficacy, education, gender, perceived family support, and comorbidities explained 25% of the variance in self-care maintenance (R(2)=.248, F(5, 123)=9.44, p<.001). Age, depressive symptoms, self-efficacy, and NYHA Class explained 23% of the variance in self-care monitoring (R(2)=.232, F(2, 124)=10.66, p<.001). Self-efficacy and NYHA Class explained 9% of the variance in self-care management (R(2)=.094, F(2, 80)=5.27, p=.007)., Conclusions: Low levels of self-care are common among persons with ACHD. Multiple factors, including modifiable factors of self-efficacy, depressive symptoms, and perceived family support, are associated with self-care and should be considered in designing future interventions to improve outcomes in the ACHD population., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Background: Cardiac transplantation in adult patients with congenital heart disease poses numerous challenges. The optimal operative and postoperative management strategies remain unclear. The purpose of our study was to (1) characterize the adult patient with a congenital heart condition undergoing transplantation, the operation, and the postoperative course; (2) report the survival after heart transplantation at our center; and (3) discuss issues surrounding the unique setting we provide for the operative and postoperative care of this complex patient cohort., Methods: We performed a retrospective cohort study of 12 consecutive adult patients with a prior history of congenital heart disease who underwent heart transplantation at a single, large, academic center between September 1, 2005, and September 1, 2013. The operations were performed by a surgeon specializing in congenital heart disease in an adult hospital. Postoperative care was provided jointly by that surgeon and the adult cardiac transplantation team., Results: At operation, the median age and weight were 41 years (range, 16 to 72 years) and 65 kg (range, 45 to 104 kg), and 100% of patients had undergone previous operations. The median donor ischemic time was 197 minutes (range, 137 to 282 minutes). The median cardiopulmonary bypass time was 210 minutes (range, 175 to 457 minutes), and the median total operating time was 582 minutes (range, 389 to 968 minutes). Three patients required mechanical support to be weaned from cardiopulmonary bypass. Postoperatively, 3 patients required the addition of mechanical support in the intensive care unit, and 3 patients required tracheostomy for prolonged ventilation. The majority of patients had a complicated postoperative course (66%). The median number of noncardiac consultants required to help care for these patients was four (range, two to 12). The mortality was 0%., Conclusions: Cardiac transplantation in adults with congenital cardiac disease is challenging, is fraught with adverse events, and requires meticulous care and teamwork for success. A surgeon specializing in congenital heart conditions may be best to handle the operative challenges, and an adult hospital with access to certain technology and consultant services may be best to handle the postoperative challenges in this difficult patient population., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

In this article, we review both acute and chronic liver diseases that occur as a result of heart or circulatory system failure. Ischemic hepatitis, congestive hepatopathy, cardiac cirrhosis, and Fontan liver disease are reviewed. We review clinical presentation, diagnostic data, prognosis, and available therapeutic strategies for these entities. We aim to increase awareness about cardio-hepatic disease as the prevalence of this disorder in adults is increasing. Due to advances in medical and surgical care, patients with heart disease are living longer and thus exposing long-term effects on the liver that are clinically relevant. There may be a role for dual organ transplantation in some cases, but this is a very challenging endeavor, and newer ideas about treatment or prevention are needed., (Copyright © 2014. Published by Elsevier Inc.)

Background: Sudden cardiac death is a major cause of late mortality in adults with congenital heart disease (ACHD). While data exist for adults with repaired Tetralogy of Fallot (TOF), little is known about those with non-TOF lesions. We examined the relative rates in implantable cardioverter defibrillator (ICD) therapy according to congenital lesion type in a large-volume adult congenital heart center., Methods: A cohort of 59 individuals (median follow up time, 3.2 years range 0-10) with ACHD and ICDs was stratified according to underlying congenital lesion and implant indication. Appropriate therapies were defined as any therapy for a physician-adjudicated ventricular arrhythmia. Rates of inappropriate and appropriate ICD therapies were analyzed according to several relevant clinical variables., Results: Thirty-three (56%) TOF, 15 (25.4%) L- or D-transposition of great arteries, and 11 (18.6%) with other lesions were included in the analysis. Approximately half (52.5%) were implanted for primary prevention indications. During follow-up, 12 (20.3%) patients received appropriate ICD therapies and 13 (22%) patients received inappropriate therapies. The incidence of appropriate shocks among patients with TOF was 27.3% (9/33) compared to 11.5% (3/26) among non-TOF diagnoses during the follow-up time (p = 0.043)., Conclusions: ACHD patients with non-TOF congenital lesions are significantly less likely to receive appropriate ICD therapy than those with TOF. Our analysis calls into question the validity of traditional ICD implantation guidelines in this growing and diverse patient population., (©2014 Wiley Periodicals, Inc.)

Objective: Patients with congenital heart disease frequently survive into adulthood, and many of them will require repeat surgery. Often, the unique anatomy can make reoperative sternotomy and the conduct of cardiopulmonary bypass challenging. We evaluated the utility of preoperative 3-dimensional imaging and presternotomy femoral cutdown in reoperative adult congenital heart disease surgery., Methods: We retrospectively studied 205 adult patients, who had undergone reoperative cardiac surgery for congenital heart disease from 2006 to 2011. Using the operative history and 3-dimensional preoperative imaging findings, an algorithm was created to determine whether femoral cutdown or cannulation should be performed before sternal reentry. Analyses were performed to determine the benefits of this strategy. In addition, analyses were performed to identify adverse outcomes related to this strategy., Results: Presternotomy femoral intervention was performed in 112 of 205 patients (55%)-femoral cutdown alone in 69 (34%) and femoral cutdown, cannulation, and institution of cardiopulmonary bypass in 43 (21%). Of the 19 patients (9%) with a cardiac injury, femoral cutdown had already been performed in 17, of whom 10 had also undergone cannulation. Only 2 patients required urgent femoral cutdown or cannulation. A strong correlation was found between the site of injury predicted by the preoperative algorithm and the actual site of cardiac injury (88%). In both univariate and multivariate models, the risk factors for cardiac injury included a history of cardiac injury during sternal reentry (18% vs 1%, P = .0001), proximity of the right ventricular outflow tract to the posterior chest wall (35% vs 14%, P = .04), and increased reoperative sternotomy incidence (P = .01). In 31 patients, despite safe reentry, the femoral vessels were used as a preferential site of venous (n = 6), arterial (n = 9), or venous and arterial cannulation (n = 16) because of anatomic constraints within the chest cavity. Three patients experienced groin complications (pseudoaneurysm, abscess, ischemia) requiring surgery., Conclusions: Cardiac injury during reoperative surgery in adults with congenital heart disease is not uncommon. The preoperative history and imaging findings could be predictive of certain cardiac injury patterns. Using the preoperative history and 3-dimensional imaging findings, a more selective algorithm for presternotomy femoral intervention might be warranted., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Background: Many adults with transposition of the great arteries have an anatomic right ventricle functioning as the systemic ventricle and are known to develop congestive heart failure, premature cardiac death, and need for cardiac transplantation. Predictors of poor clinical outcome and functional status in patients with left ventricular failure do not always apply to these patients. We aimed to identify predictors of poor functional status in those patients with a systemic right ventricle., Methods: We performed a prospective study of 51 adults with transposition of the great arteries and systemic right ventricles. Demographic, clinical, laboratory, and imaging data were collected, and patients completed a Minnesota Living with Heart Failure Questionnaire (MLHFQ). Comparisons were made between those patients with d-type transposition of the great arteries (dTGA) who have undergone prior atrial switch and those with congenitally corrected transposition (ccTGA). A correlation analysis was performed to identify predictors of poor functional status, as determined by a 6-minute walk distance test., Results: Median age was 30 years (range 19-65). Median B-type natriuretic peptide was 48 pg/mL (range 16-406). There were 27 patients (53%) with moderate-severe right ventricular dysfunction and 10 (20%) with moderate-severe tricuspid valve regurgitation. The median MLHFQ score was 9 (range 0-78) and 6-minute walk test was 510 m (range 231-703). Forty-one patients had a diagnosis of dTGA atrial switch and 11 patients had ccTGA. Patients with ccTGA were significantly older (40 vs. 28 years, P =.004) and had more tricuspid valve regurgitation (P =.02). Despite this, their MLHFQ scores were significantly lower (2.5 vs. 17, P =.04) and they walked further (635 vs. 504 m, P =.02). Predictors of a short 6-minute walk distance included short stature (P =.009) and dTGA (P =.002). The patient's self-assessment of poor health, as measured by an increased New York Heart Association class (P =.003) and a decreased MLHFQ score (P >.0001) also correlated. B-type natriuretic peptide levels, right ventricular dysfunction, severity of tricuspid valve regurgitation, need for pacemaker, and clinical signs of heart failure did not correlate with exercise tolerance., Conclusions: Traditional parameters used to predict outcomes in patients with left ventricular failure are not predictive in patients with a systemic right ventricle. Instead, patient's self-assessment of functional status did correlate with objective functional status., (© 2013 Wiley Periodicals, Inc.)

Background: Patients with congenital heart disease are frequently surviving into adulthood, and many of them will require surgery. Unfortunately, outcome data in this patient population are limited. We aimed to identify risk factors associated with adverse events in adults with congenital heart disease undergoing cardiac surgery and establish long-term survival data., Methods: We retrospectively studied 458 adult patients who underwent cardiac surgery for congenital heart disease between 2000 and 2010. We constructed logistic models to assess risk factors for mortality, major adverse event (stroke, renal failure, prolonged ventilation, prolonged coma, deep sternal infection, reoperation, and operative mortality), and prolonged length of stay (>7 days). Long-term, all-cause mortality was also measured., Results: Sixteen patients died (3.49%). Risk factors for mortality included a history of cerebrovascular disease (odds ratio [OR]: 4.51), New York Heart Association (NYHA) class 3 or 4 (OR: 8.88), and surgery on the aorta or the aortic valve (OR: 5.74). Ninety-four patients suffered a major adverse event (20.5%). Significant risk factors were male gender (OR: 2.28), NYHA class of 3 or 4 (OR 2.58), 2 concomitant major operations (OR: 2.15), and cardiopulmonary bypass time of greater than 100 minutes (OR: 3.18). Last, 90 patients (19.7%) remained in the hospital longer than 7 days. Significant risk factors for a prolonged length of stay included chronic lung disease (OR: 3.05), NYHA class of 3 or 4 (OR: 3.69), surgery by an adult cardiac surgeon (OR 2.58), 2 concomitant major operations (OR: 3.28), and cardiopulmonary bypass time of greater than 100 minutes (OR: 2.41). Survival at 1, 5, and 10 years was 97.6%, 95.2%, and 93.4%, respectively., Conclusions: Surgery in adults with congenital heart disease can be performed with low morbidity and mortality. Nonetheless, there remain important risk factors for adverse events. Awareness and modification of risk factors may help improve outcomes., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Eosinophils are blood cells that are often found in high numbers in the tissues of allergic conditions and helminthic parasite infections. The pathophysiologic roles that eosinophils may serve in other human "eosinophil-associated" diseases remain obscure., Objective: National Institutes of Health (NIH) Institutes and the Office of Disease Prevention assembled an international taskforce of clinical and basic scientists with the charge to propose and prioritize unmet research needs in eosinophil-associated diseases., Methods: The taskforce used an organ system approach to identify the different and common themes of eosinophil cell involvement in these diseases. In early 2012, a draft document was circulated for review. The document was amended and the prioritizations were set at a NIH-organized workshop in June 2012., Results: The taskforce identified significant research needs. These needs cross disease entities but some are disease specific. There are substantial shortcomings to the various preclinical animal models, as well as significant gaps in our epidemiologic, pathophysiologic, diagnostic, prognostic, and therapeutic knowledge. The taskforce recognized that recent efforts by patient advocacy groups have played instrumental roles in improving the identification and characterization of these disorders. However, communications among the eosinophil-interested communities, for example, governmental funding and regulatory agencies, and industry and clinician scientists need to be more comprehensive., Conclusions: Significant efforts are required to address our knowledge gaps to improve the outcomes of eosinophil-associated diseases. NIH Institutes, other federal agencies, lay organizations, and the pharmaceutical industry should consider the taskforce's recommendations in their future research activities., (Copyright © 2012 American Academy of Allergy, Asthma & Immunology. Published by Mosby, Inc. All rights reserved.)

Patients with palliated tetralogy of Fallot (TOF) often require pulmonary valve replacement in adulthood, yet the data regarding their outcomes are scarce. This study aimed to identify risk factors associated with postoperative complications in these patients and to establish long-term survival data for this patient group. A retrospective cohort study investigated 153 consecutive patients with a history of TOF repair who underwent pulmonary valve replacement at a single large academic center between March 1996 and March 2010. In part 1 of the study, logistic models were constructed to assess demographic, medical, and surgical risk factors for operative mortality; occurrence of a major adverse event (stroke, renal failure, prolonged ventilation, deep sternal infection, reoperation, or operative mortality); and prolonged hospital stay (>7 days). Risk factors with a p value less than 0.10 by univariate analysis were included in the subsequent multivariate analysis. In part 2 of the study, long-term, all-cause mortality was determined by construction of a Kaplan-Meier curve for the cohort. Seven patients died (4.5%). Significant risk factors for mortality in the multivariable analysis included age older than 40 years (odds ratio (OR) 9.89) and concomitant surgery (OR 6.65). A major adverse event occurred for 22 patients (14.4%). The only significant risk factor in the multivariable analysis for an adverse event was concomitant surgery (OR 6.42). The hospital stay was longer than 7 days for 31 patients (20.3%). The significant risk factors for a prolonged hospital stay included the presence of preoperative arrhythmias (OR 4.17), New York Heart Association class 3 (OR 4.35), and again, concomitant surgery (OR 4.2). Among the 146 hospital survivors, only 5 patients died in the intervening period. The predicted survival rates were 98.5% at 1 year, 96.7% at 5 years, and 93.5% at 10 years. Pulmonary valve replacement in adults with palliated TOF is a safe procedure with excellent long-term survival, but there remain important risk factors for postoperative mortality, prolonged hospital stay, and major adverse events. Awareness and modification of important risk factors may help to improve outcomes.

Mitral valve replacement surgery after previous aortic valve surgery can be extremely challenging. The presence of heavy calcification and scarring in the aortic and/or mitral annulus can make suture placement nearly impossible. Although novel techniques have been described, even these alternatives might not be feasible. We present a case of nonanatomical mitral valve replacement in the pulmonary venous confluence as a salvage operation. This can provide adequate hemodynamics and survival until a subsequent care plan can be established., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Many congenital heart defects require reconstruction of the right ventricular outflow tract utilizing a right ventricle to pulmonary artery conduit. One of the challenges with these conduits is the development of conduit stenosis. This phenomenon is quite common and typically results from a combination of progressive calcification, fibrosis, and/or the relative size mismatch that occurs with patient growth. However, extrinsic compression is much less common and a much more difficult problem to address. Chest wall resection and reconstruction is an option for alleviating external conduit compression that provides good results., (© 2011 Wiley Periodicals, Inc.)

Congenital heart defects with a component of pulmonary stenosis are often palliated in childhood by disrupting the pulmonary valve. Patients often undergo subsequent pulmonary valve replacement (PVR) to protect the heart from the consequences of pulmonary regurgitation. In the presence of associated moderate functional tricuspid valve (TV) regurgitation, it is unclear what factors contribute to persistent TV regurgitation following PVR. In particular, it is unknown whether PVR alone will reduce the right ventricular (RV) size and restore TV function or whether concomitant TV annuloplasty is required as well. Thirty-five patients were analyzed. Each patient underwent initial palliation of congenital pulmonary stenosis or tetralogy of Fallot, underwent subsequent PVR between 2002 and 2008, and had at least moderate TV regurgitation at the time of valve replacement. Serial echocardiograms were analyzed. Pulmonary and TV regurgitation, along with RV dilation and dysfunction, were scored (0, none; 1, mild; 2, moderate; 3, severe). RV volume and area were also calculated. Potential risk factors for persistent postoperative TV regurgitation were evaluated. One month following PVR, there was a significant reduction in pulmonary valve regurgitation (mean, 3 vs. 0.39; P < 0.0001) and TV regurgitation (mean, 2.33 vs. 1.3; P < 0.0001). There were also significant reductions in RV dilation, volume, and area. There were no significant further improvements in any of the parameters at 1 and 3 years. There was no difference in the degree of TV regurgitation postoperatively between those patients who underwent PVR alone and those who underwent concomitant tricuspid annuloplasty (mean, 1.29 vs. 1.31; P = 0.81). We conclude that following PVR, improvement in TV regurgitation and RV size occurs primarily in the first postoperative month. TV function improved to a similar degree with or without annuloplasty.

Many modifications of the Bentall technique have been described since its introduction in 1968. The crucial phases of the operation include the re-establishment of coronary flow and control of intraoperative hemorrhage. Based on their experience with Blalock-Taussig shunts, the proximal take-off of which is at the innominate/right subclavian artery junction, the present authors have developed a novel technique of coronary reconstruction. The details of a new technique of coronary reconstruction to minimize the duration of cardiopulmonary bypass, myocardial ischemia and bleeding, are reported.

A 33-year-old woman with a history of gestational trophoblastic disease presented for investigation of a right atrial mass. She had been receiving chemotherapy administered via a Port-a-Cath system for 2 months prior to presentation. On transesophageal echocardiography and magnetic resonance imaging, she was found to have a mass attached to the right atrial free wall, with a segment projecting across a patent foramen ovale. Because of the risk for an embolic event, the mass was surgically removed and the patent foramen ovale repaired. Pathology showed an organized thrombus. This case emphasizes the need for high suspicion for thrombus when a right atrial mass is found in a patient with a hypercoagulable state due to underlying malignancy who has a central venous catheter., (Copyright 2010 American Society of Echocardiography. Published by Mosby, Inc. All rights reserved.)

Many congenital heart defects with pulmonary valve pathology are repaired or palliated in childhood. It is anticipated that these patients will need subsequent pulmonary valve replacement due to the pulmonary regurgitation or right ventricular dilation/failure that results from their original operation. Interestingly, some patients with prior congenital heart surgery and no congenital pulmonary valve pathology require pulmonary valve replacement in adulthood. The current study analyzed this subset of patients. The pediatric and adult congenital cardiac surgical databases at a large academic center were reviewed from 2001 to 2008 for pulmonary valve replacements. Patients without congenital pulmonary valve disease were identified. Preoperative, operative, and postoperative data were analyzed. Between 2001 and 2008, five patients with congenital heart disease but no pulmonary valve pathology underwent late pulmonary valve replacement. The initial congenital diagnoses were coarctation/ventricular septal defect (n = 3), complete atrioventricular septal defect (n = 1), and anomalous left coronary artery from the pulmonary artery (PA) (n = 1). All five patients had undergone main PA manipulation during their previous operations. Four of these patients had received PA banding and debanding, and one had received a Takeuchi tunnel repair and revision. All the patients underwent pulmonary valve replacement secondary to pulmonary regurgitation and right ventricular dilation. The average time from the last previous operation to the time of pulmonary valve replacement was 20.8 +/- 9.1 years. Bioprosthetic valves were used for all the pulmonary valve replacements, and there were no mortalities. Patients who have undergone previous PA manipulation, regardless whether congenital pulmonary pathology is present, may be at risk for pulmonary valve replacement in adulthood. This report describes five cases of patients with a history of congenital heart surgery but no congenital pulmonary valve pathology who required pulmonary valve replacement due to the consequences of prolonged pulmonary regurgitation. Although pulmonary regurgitation may be well tolerated for many years, it is further evidence for the importance of close follow-up assessment and monitoring of young adults with congenital heart disease.

Background: Incremental value of echocardiography over clinical parameters for outcome prediction in advanced heart failure (HF) is not well established., Methods and Results: We evaluated 223 patients with advanced HF receiving optimal therapy (91.9% angiotensin-converting enzyme inhibitor/angiotensin receptor blocker, 92.8% beta-blockers, 71.8% biventricular pacemaker, and/or defibrillator use). The Seattle Heart Failure Model (SHFM) was used as the reference clinical risk prediction scheme. The incremental value of echocardiographic parameters for event prediction (death or urgent heart transplantation) was measured by the improvement in fit and discrimination achieved by addition of standard echocardiographic parameters to the SHFM. After a median follow-up of 2.4 years, there were 38 (17.0%) events (35 deaths; 3 urgent transplants). The SHFM had likelihood ratio (LR) chi(2) 32.0 and C statistic 0.756 for event prediction. Left ventricular end-systolic volume, stroke volume, and severe tricuspid regurgitation were independent echocardiographic predictors of events. The addition of these parameters to SHFM improved LR chi(2) to 72.0 and C statistic to 0.866 (P < .001 and P=.019, respectively). Reclassifying the SHFM-predicted risk with use of the echocardiography-added model resulted in improved prognostic separation., Conclusions: Addition of standard echocardiographic variables to the SHFM results in significant improvement in risk prediction for patients with advanced HF.

Objective: Congenital heart defects with a component of pulmonary stenosis are often palliated in childhood by disrupting the pulmonary valve, either by means of dilation or excision. It is unclear what factors affect a patient's ability to tolerate long-term pulmonary insufficiency before requiring pulmonary valve replacement. We analyze potential factors that are related to the interval between pulmonary valve disruption and pulmonary valve replacement., Methods: One hundred seven patients were analyzed. They had a congenital diagnosis of pulmonary stenosis or tetralogy of Fallot, had their first pulmonary valve replacement between 2002 and 2008, and had a known interval between pulmonary valve disruption and pulmonary valve replacement. The median age at the time of surgical intervention was 2 years for pulmonary valve disruption (range, 0-56 years) and 26 years for pulmonary valve replacement (range, 1-72 years). The median interval was 23 years (range, 0-51 years). Potential related factors were sex, race, initial diagnosis and procedure, age at pulmonary valve disruption, prior shunt operation, presence of branch pulmonary artery stenosis, and degree of pulmonary regurgitation., Results: As determined by using univariate analysis, male patients had a shorter interval than female patients (median, 16 vs 26 years; P = .01), and African American patients had a shorter interval than white patients (median, 16 vs 25 years; P = .049). A significant correlation was also identified between age at the time of pulmonary valve disruption and the subsequent interval to pulmonary valve replacement. Overall, the interval tended to increase as age at disruption increased (P < .0001). Although the presence of branch pulmonary artery stenosis determined by the need for concomitant pulmonary arterioplasty was associated with a significantly shorter interval to pulmonary valve replacement (21 vs 24 years, P = .02), stenosis determined based on small branch pulmonary artery diameter was correlated to a prolonged interval to pulmonary valve replacement (P = .009). Initial diagnosis, prior palliative shunt operation, and degree of pulmonary regurgitation had no effect on the interval between pulmonary valve disruption and subsequent pulmonary valve replacement. As determined by using multivariate analysis, only male sex and small pulmonary artery diameter remained significant factors., Conclusions: Male sex appears to shorten the interval between pulmonary valve disruption and pulmonary valve replacement, whereas small branch pulmonary artery diameter appears to lengthen the interval. Knowing which factors are detrimental and which are protective might help identify patients who are prone to a more rapid progression of right heart failure from free pulmonary insufficiency, possibly steering them toward more frequent follow-up or more aggressive heart failure medical regimens.

Background: One of the current controversies in the field of adult congenital heart disease is whether patients should be cared for at an adult or pediatric facility and by an adult or pediatric heart surgeon. After transitioning our program from the children's hospital to the adult hospital, we analyzed our experience with each system., Methods: Between 2000 and 2007, 303 operations were performed on adults (age >or= 18 years) with congenital heart disease. One hundred eighty-five operations were performed in an adult hospital and 118 in a pediatric hospital. Forty-six operations were performed by an adult heart surgeon and 257 by a congenital heart surgeon., Results: Mean age, coexisting medical problems, and preoperative risk factors were higher in both the adult hospital group and adult surgeon group compared with the respective pediatric groups. Mortality was similar at the adult and pediatric hospitals (4.3% versus 5.1%), but was markedly higher in the adult surgeon group compared with the pediatric surgeon group (15.2% versus 2.7%; p = 0.0008). By multivariate analysis, risk factors for mortality included older age at the time of surgery (p = 0.028), surgery performed at a children's hospital (p = 0.013), and surgery performed by an adult heart surgeon (p = 0.0004)., Conclusions: Congenital heart surgery can be performed in adults with reasonable morbidity and mortality. Caring for an anticipated aging adult congenital population with increasingly numerous coexisting medical problems and risk factors is best facilitated in an adult hospital setting. Also, when surgery becomes necessary, these adult patients are best served by a congenital heart surgeon.