The long-term effects of the fenestration in patients with extracardiac Fontan circulation-a multicenter Korean cohort study based on national Fontan registry.

Introduction: The long-term effects of fenestration in patients with Fontan circulation remain unclear. We aim to evaluate the fenestration impact on early and late outcomes in patients with extracardiac Fontan (ECF) using a propensity score matching analysis. Methods: We performed an extensive retrospective multicenter clinical data review of the Korean Fontan registry and included 1,233 patients with surgical ECF (779 fenestrated, 454 non-fenestrated). Demographics, baseline, and follow-up data were collected and comprehensively analyzed. Patients were divided into two groups according to the baseline presence or absence of surgical fenestration. Subsequently, patients were sub-divided according to the fenestration status at the last follow-up. Propensity-score matching was performed to account for collected data between the 2 groups using a multistep approach. The primary outcomes were survival and freedom from Fontan failure (FFF). We also looked at postoperative hemodynamics, cardiopulmonary exercise test results, oxygen saturations, and functional status. Results: After propensity-score matching (454 matched pairs), there was no difference in survival or FFF between the 2 groups. However, ECF patients with baseline fenestration had significantly lower oxygen saturation (p = 0.001) and lower functional status (p < 0.001). Patients with fenestration had significantly longer bypass times, higher postoperative central venous pressure, higher postoperative left atrial pressure, and less prolonged pleural effusion in the early postoperative period. The propensity score matching according to the fenestration status at the last follow-up (148 matched pairs) showed that patients with a persistent fenestration had significantly lower oxygen saturation levels (p < 0.001). However there were no intergroup differences in the functional status, survival and FFF. Conclusions: Our results showed no long-term benefits of the Fenestration in terms of survival and FFF. Patients with persistent fenestration showed oxygen desaturation but no difference in exercise intolerance was shown between the 2 groups.

The Effect of Udenafil on Heart Rate and Blood Pressure in Adolescents With the Fontan Circulation.

The Fontan Udenafil Exercise Longitudinal (FUEL) trial showed that treatment with udenafil was associated with improved exercise performance at the ventilatory anaerobic threshold in children with Fontan physiology. However, it is not known how the initiation of phosphodiesterase 5 inhibitor therapy affects heart rate and blood pressure in this population. These data may help inform patient selection and monitoring after the initiation of udenafil therapy. The purpose of this study is to evaluate the effects of udenafil on vital signs in the cohort of patients enrolled in the FUEL trial. This international, multicenter, randomized, double-blind, placebo-controlled trial of udenafil included adolescents with single ventricle congenital heart disease who had undergone Fontan palliation. Changes in vital signs (heart rate [HR], systolic [SBP] and diastolic blood pressure [DBP]) were compared both to subject baseline and between the treatment and the placebo groups. Additional exploratory analyses were performed to evaluate changes in vital signs for prespecified subpopulations believed to be most sensitive to udenafil initiation. Baseline characteristics were similar between the treatment and placebo cohorts (n = 200 for each). The groups demonstrated a decrease in HR, SBP, and DBP 2 hours after drug/placebo administration, except SBP in the placebo group. There was an increase in SBP from baseline to after 6-min walk test in the treatment and placebo groups, and the treatment group showed an increase in HR (87.4 ± 15.0 to 93.1 ± 19.4 beats/min, p <0.01) after exercise. When comparing changes from baseline to the 26-week study visit, small decreases in both SBP (-1.9 ± 12.3 mm Hg, p = 0.03) and DBP (-3.0 ± 9.6 mm Hg, p <0.01) were seen in the treatment group. There were no clinically significant differences between treatment and placebo group in change in HR or blood pressure in the youngest age quartile, lightest weight quartile, or those on afterload-reducing agents. In conclusion, initiation of treatment with udenafil in patients with Fontan circulation was not associated with clinically significant changes in vital signs, implying that for patients similar to those enrolled in the FUEL trial, udenafil can be started without the requirement for additional monitoring after initial administration.

The impact of physical activity changes on exercise capacity and health-related quality of life in young patients with CHD: a 3-year follow-up study.

OBJECTIVE: This study examined the relationship between changes in physical activity and their impact on exercise capacity and health-related quality of life over a 3-year span in patients with CHD. METHODS: We evaluated 99 young patients with CHD, aged 13-18 years at the outset. Physical activity, health-related quality of life, and exercise capacity were assessed via questionnaires and peak oxygen uptake measurements at baseline and after 3 years; changes in measures were estimated between the two time points and categorised into quartiles. Participants were stratified according to achieved (active) or not-achieved (inactive) recommended levels of physical activity (≥150 minutes/week) at both time points. RESULTS: Despite increases in physical activity, exercise capacity, and health-related quality of life over 3 years, the changes were not statistically significant (all p > 0.05). However, a positive association was found between physical activity changes and exercise capacity (ß = 0.250, p = 0.040) and health-related quality of life improvements (ß = 0.380, p < 0.001). Those with the most pronounced physical activity increase showed notable exercise capacity (p < 0.001) and health-related quality of life increases (p < 0.001) compared with patients with the largest decline in physical activity. The active-inactive category demonstrated a notable decline in exercise capacity compared to the active-active group, while the inactive-active group showed health-related quality of life improvements. CONCLUSIONS: Over 3 years, increased physical activity was consistently linked to increases in exercise capacity and health-related quality of life in patients with CHD, highlighting the potential of physical activity augmentation as an intervention strategy.

Impact of Pulmonary Arterial Elastance on Right Ventricular Mechanics and Exercise Capacity in Repaired Tetralogy of Fallot.

BACKGROUND AND OBJECTIVES: Pathophysiological changes of right ventricle (RV) after repair of tetralogy of Fallot (TOF) are coupled with a highly compliant low-pressure pulmonary artery (PA) system. This study aimed to determine whether pulmonary vascular function was associated with RV parameters and exercise capacity, and its impact on RV remodeling after pulmonary valve replacement. METHODS: In a total of 48 patients over 18 years of age with repaired TOF, pulmonary arterial elastance (Ea), RV volume data, and RV-PA coupling ratio were calculated and analyzed in relation to exercise capacity. RESULTS: Patients with a low Ea showed a more severe pulmonary regurgitation volume index, greater RV end-diastolic volume index, and greater effective RV stroke volume (p=0.039, p=0.013, and p=0.011, respectively). Patients with a high Ea had lower exercise capacity than those with a low Ea (peak oxygen consumption [peak VO2] rate: 25.8±7.7 vs. 34.3±5.5 mL/kg/min, respectively, p=0.003), while peak VO2 was inversely correlated with Ea and mean PA pressure (p=0.004 and p=0.004, respectively). In the univariate analysis, a higher preoperative RV end-diastolic volume index and RV end-systolic volume index, left ventricular end-systolic volume index, and higher RV-PA coupling ratio were risk factors for suboptimal outcomes. Preoperative RV volume and RV-PA coupling ratio reflecting the adaptive PA system response are important factors in optimal postoperative results. CONCLUSIONS: We found that PA vascular dysfunction, presenting as elevated Ea in TOF, may contribute to exercise intolerance. However, Ea was inversely correlated with pulmonary regurgitation (PR) severity, which may prevent PR, RV dilatation, and left ventricular dilatation in the absence of significant pulmonary stenosis.

The Outcome of Long QT Syndrome, a Korean Single Center Study.

BACKGROUND AND OBJECTIVES: Although long QT syndrome (LQTS) is a potentially life-threatening inherited cardiac channelopathy, studies documenting the long-term clinical data of Korean patients with LQTS are scarce. METHODS: This retrospective cohort study included 105 patients with LQTS (48 women; 45.7%) from a single tertiary center. The clinical outcomes were analyzed for the rate of freedom from breakthrough cardiac events (BCEs), additional treatment needed, and death. RESULTS: LQTS was diagnosed at a median age of 11 (range, 0.003-80) years. Genetic testing was performed on 90 patients (yield, 71.1%). The proportions of genetically confirmed patients with LQTS types 1, 2, 3, and others were 34.4%, 12.2%, 12.2%, and 12.2%, respectively. In the symptomatic group (n=70), aborted cardiac arrest was observed in 30% of the patients. Treatments included medications in 60 patients (85.7%), implantable cardioverter-defibrillators in 27 (38.6%; median age, 17 years; range, 2-79 years), and left cardiac sympathetic denervation surgery in 7 (10%; median age, 13 years; range, 2-34). The 10-year BCE-free survival rate was 73.2%. By genotype, significant differences were observed in BCEs despite medication (p<0.001). The 10-year BCE-free survival rate was the highest in patients with LQTS type 1 (81.8%) and the lowest in those with multiple LQTS-associated mutations (LQTM). All patients with LQTS survived, except for one patient who had LQTM. CONCLUSIONS: Good long-term outcomes can be achieved by using recently developed genetically tailored management strategies for patients with LQTS.

Surgical and Electrical Anatomy of the Inter-Nodal and Intra-Atrial Conduction System in the Heart.

An anatomical understanding of the atrial myocardium is crucial for surgeons and interventionists who treat atrial arrhythmias. We reviewed the anatomy of the inter-nodal and intra-atrial conduction systems. The anterior inter-nodal route (#1) arises from the sinus node and runs through the ventral wall of the atrial chambers. The major branch of route #1 approaches the atrioventricular node from the anterior aspect. Other branches of route #1 are Bachmann's bundle and a vestibular branch around the tricuspid valve. The middle inter-nodal route (#2) begins with a broad span of fibers at the sinus venarum and extends to the superior limbus of the oval fossa. The major branch of route #2 joins with the branch of route #1 at the anterior part of the atrioventricular node. The posterior inter-nodal route (#3) is at the terminal crest and gives rise to many branches at the pectinate muscles of the right atrium and then approaches the posterior atrioventricular node after joining with the vestibular branch of route #1. The branches of the left part of Bachmann's bundle and the branches of the second inter-nodal route form a thin myocardial network at the posterior wall of the left atrium. These anatomical structures could be categorized into major routes and side branches. There are 9 or more anatomical circles in the atrial chambers that could be structural sites for macro re-entry. The implications of normal and abnormal structures of the myocardium for the pathogenesis and treatment of atrial arrhythmias are discussed.

Long-term Outcome of Fontan-Associated Protein-Losing Enteropathy: Treatment Modality and Predictive Factor of Mortality.

BACKGROUND AND OBJECTIVES: Protein-losing enteropathy (PLE) is a devastating complication after the Fontan operation. This study aimed to investigate the clinical characteristics, treatment response, and outcomes of Fontan-associated PLE. METHODS: We reviewed the medical records of 38 patients with Fontan-associated PLE from 1992 to 2018 in 2 institutions in Korea. RESULTS: PLE occurred in 4.6% of the total 832 patients after the Fontan operation. After a mean period of 7.7 years after Fontan operation, PLE was diagnosed at a mean age of 11.6 years. The mean follow-up period was 8.9 years. The survival rates were 81.6% at 5 years and 76.5% at 10 years. In the multivariate analysis, New York Heart Association Functional classification III or IV (p=0.002), low aortic oxygen saturation (<90%) (p=0.003), and ventricular dysfunction (p=0.032) at the time of PLE diagnosis were found as predictors of mortality. PLE was resolved in 10 of the 38 patients after treatment. Among medical managements, an initial heparin response was associated with survival (p=0.043). Heparin treatment resulted in resolution in 4 patients. We found no evidence on pulmonary vasodilator therapy alone. PLE was also resolved after surgical Fontan fenestration (2/6), aortopulmonary collateral ligation (1/1), and transplantation (1/1). CONCLUSIONS: The survival rate of patients with Fontan-associated PLE has improved with the advancement of conservative care. Although there is no definitive method, some treatments led to the resolution of PLE in one-fourth of the patients. Further investigations are needed to develop the best prevention and therapeutic strategies for PLE.

Clinical Outcomes of Implantable Cardioverter-Defibrillator in Pediatric Patients　- A Korean Multicenter Study.

BACKGROUND: Implantable cardioverter-defibrillator (ICD) therapy is important for the prevention of sudden cardiac death, but data on clinical outcomes of ICD therapy in Asian pediatric patients are scarce. The aim of this Korean multicenter study was to evaluate the current state and elucidate the clinical outcomes of ICD therapy in children.Methods and Results:Data from 5 pediatric cardiology centers were retrospectively collected from 2007 to 2019. Altogether, 99 patients were enrolled (mean age 13.9±4.1 years). The most common underlying disease was a primary electrical disease (56%). An ICD was implanted for primary prevention in 19%. Appropriate shock occurred in 44% of patients at a median of 1.6 years after implantation. There was no significant difference in the appropriate shock rate between patients with primary and secondary prevention indications (32% vs. 48%, respectively). A total of 33 patients (33%) experienced inappropriate shock, which was associated with primary electrical disease and follow-up duration on multivariate analysis. 17% of patients had ICD-related complications. CONCLUSIONS: The utilization rate of ICD for primary prevention was still low in the pediatric population in Korea, but there was a substantial rate of appropriate shock in these patients. Efforts to increase ICD usage to save the lives of high-risk patients and reduce the incidence of inappropriate shock are required.

Mediating effects of exercise capacity on the association between physical activity and health-related quality of life among adolescents with complex congenital heart disease.

OBJECTIVES: There is little evidence on interrelationships between physical activity, sedentary behaviors, and health-related quality of life (HRQOL) among adolescents with congenital heart disease (CHD). We hypothesized that exercise capacity would have a mediating effect on the associations of either physical activity or sedentary behavior with HRQOL. METHODS: Adolescents with complex CHD (n = 111) were consecutively recruited from an outpatient clinic in a general hospital in South Korea. Physical activity and sedentary behavior were assessed using the global physical activity questionnaire. Exercise capacity was directly measured by peak oxygen uptake using a symptom-limited maximal treadmill exercise test. HRQOL was evaluated by both adolescents and their parents using the Pediatric Quality of Life Inventory questionnaire. RESULTS: The self-reported and parent proxy-reported HRQOL were positively associated with physical activity (ß = 0.16, P = .003; ß = 0.12, P = .049) and exercise capacity (ß = 0.63, P < .001; ß = 0.66, P < .001), but not with sedentary behavior in adjusted regression models. When both variables were entered in the same regression models, only exercise capacity remained significantly associated with the self-reported (ß = 0.50, P = .008) and parent proxy-reported HRQOL (ß = 0.62, P = .003). Exercise capacity acted as a full mediator variable on the relationship between physical activity and HRQOL (P < .05 for both). CONCLUSIONS: The present findings suggest that exercise capacity mediates the association between physical activity and HRQOL, highlighting the importance of improving exercise capacity to potentially enhance HRQOL in adolescents with complex CHD.

Hybrid Pulmonary Vein Stenting in Infants with Refractory to Surgical Pulmonary Vein Stenosis Repair.

Pulmonary vein stenosis (PVS) is still a frustrating disease with extremely high mortality, especially in children with multiple severe PVS. Hybrid pulmonary vein stenting (HPVS) is a rescue treatment for recurrent and malignant PVS. The aim of this study is to share our successful experience with intraoperative HPVS for recurrent PVS after total anomalous pulmonary venous connection (TAPVC) repair in infant. Six patients were identified between 2013 and January 2018, who were diagnosed with recurrent PVS and underwent HPVS in the operating room. The mean age at the time of the HPVS was 10.3 ± 2.7 months (range 7-14 months) and the mean body weight was 7.9 ± 2.6 kg (range 4.1-10.5 kg). Prior pulmonary vein surgery had been performed on average 2.7 times (range 2-3) in all patients. We used a bare-metal stent (BMS) of 6-8 mm diameter in 15 veins of five patients and a drug-eluting coronary stent (DES) in two veins of one patient. All patients had undergone several elective further pulmonary vein in-stent balloon dilatations or another stent insertion after HPVS. Over a mean follow-up of 17.3 ± 13.7 months (range 6-44 months), all patients maintained patency of stents although two patients died due to respiratory failure not associated with PVS. HPVS is a useful treatment modality for recurrent PVS patient that could save the life and achieve longer freedom from restenosis than repetitive surgical pulmonary vein widening only. Even though the prognosis of severe multiple PVS is very poor, planned HPVS could be a good palliation in this patients group.

The outcome of percutaneous stent implantation in congenital heart disease: experience of a single institute.

PURPOSE: The efficacy of percutaneous stent implantation for congenital heart disease (CHD) in Korea, where stent availability is limited, has not been determined. This study evaluated the acute and midterm results of stent implantation in different CHD subgroups. METHODS: Stents were implanted in 75 patients with 81 lesions: (1) pulmonary artery stenosis (PAS) group, 56 lesions in 51 patients; (2) coarctation of the aorta (CoA) group, 5 lesions in 5 patients; (3) Fontan group, 13 lesions in 12 patients; (4) ductal stent group, 3 lesions in 3 patients; and (5) other CHD group, 4 lesions in 4 patients. Mean follow-up duration was 2.1 years (0.1-4 years). Medical records were reviewed retrospectively. RESULTS: The minimum lumen diameter (MLD) in PAS and CoA increased from 5.0±1.9 mm and 8.4±1.6 mm to 10.1±3.6 mm and 12.3±2.5 mm, respectively (P<0.01). In the PAS group, pressure gradient decreased from 25.7±15.6 mmHg to 10.4±10.1 mmHg, and right ventricular to aortic pressure ratio from 0.56±0.21 to 0.46±0.19. In the CoA group, the pressure gradient decreased from 50±33 mmHg to 17±8 mmHg. In the ductal stent group, the MLD of the ductus increased from 2.3 mm to 4.3 mm and arterial oxygen saturation from 40%-70% to 90%. No deaths were associated with stent implantation. Stent migration occurred in 3 patients, but repositioning was successful in all. Stent redilation was performed successfully in 26 cases after 29±12 months. CONCLUSION: Percutaneous stent implantation was safe and effective, with acceptable short and mid-term outcomes in Korean CHD patients.

Locoregionally advanced oral cavity cancer: A propensity-score matched analysis on overall survival with emphasis on the impact of adjuvant radiotherapy.

BACKGROUND: The purpose of this study was to determine the impact of adjuvant radiotherapy (RT) in locoregionally advanced oral cavity cancer. METHODS: Data were extracted from the National Cancer Data Base, of which overall survival (OS) is the only outcome variable available. The chi-square test and Cox regression models were used. RESULTS: A total of 6654 patients were identified. The utilization of adjuvant RT has increased over time. A propensity matched cohort included 3946 patients, exactly one-half of whom received adjuvant RT. Independent predictors associated with receipt of adjuvant RT included age, Charlson/Deyo comorbidity score, extracapsular extension, surgical margins, and T and N classifications. On multivariable analysis, adjuvant RT remained an independent prognosticator for OS. CONCLUSION: Receipt of adjuvant RT is a prognostic factor associated with improved OS, its utilization has increased over time, and it should be considered for clinically suitable patients who have undergone resection for the disease.

Progress in the Treatment and Outcomes for Early-Stage Non-Small Cell Lung Cancer.

PURPOSE: The purpose of this study is to assess temporal trends in population-based treatment and survival rates in patients with early-stage non-small cell lung cancer (NSCLC). METHODS: Data were extracted from the Surveillance, Epidemiology, and End Results (SEER) database. Chi-square test, Kaplan-Meier method, and Cox regression models were employed in SPSS 23.0. RESULTS: Fifty-seven thousand and eighty-eight NSCLC patients with early-stage disease from 1988 to 2014 were identified. 6409 (11.2%) were diagnosed in 1988-1994, 5800 (10.2%) 1995-1999, 13,031 (22.8%) 2000-2004, 15,786 (27.7%) 2005-2009, and 16,062 (28.1%) 2010-2014. We observed a significant increase in the proportion of older patients, adenocarcinoma histology, and rate of wedge resection over the study period. The five-year overall survival (OS) for the entire cohort was 63.3%. Those undergoing resection without adjuvant therapy had the highest outcomes. Lobectomy was associated with better outcomes compared to wedge resection or pneumonectomy. A significant difference in five-year OS by year of diagnosis (1988-1994: 58.8% vs. 1995-1999: 60.6% vs. 2000-2004: 63.2% vs. 2005-2009: 66.1%; p < 0.001) was observed. This significant OS difference was also observed regardless of age, surgery type, and T stage, but also only in those with adenocarcinoma. On multivariable analysis, year of diagnosis, age, gender, race, treatment and surgery type, histology, T stage, and tumor grade remained independent prognostic factors for OS. CONCLUSIONS: Overall survival for early-stage NSCLC has significantly improved over the recent decades despite an increasing proportion of older patients and those undergoing sublobar resection or SBRT. This finding may be limited to those with adenocarcinoma.

Gliosarcoma in septuagenarians and octogenarians: What is the impact of adjuvant chemoradiation?

The objective of our study is to determine the impact of adjuvant chemoradiation on overall survival (OS) for gliosarcoma in septuagenarians and octogenarians. Data were extracted from the National Cancer Data Base (NCDB). Chi-square test, Kaplan-Meier method, and Cox regression models were employed in SPSS 23.0 (Armonk, NY: IBM Corp.) for data analyses. 210 patients with gliosarcoma who underwent resection were identified. 168 (80.0%) patients received adjuvant chemoradiation, and 42 (20.0%) received adjuvant RT alone. Patients were more likely to receive adjuvant chemoradiation if they were male vs. female (85.3% vs. 71.6%, p=0.016). There was no significant difference in receipt of adjuvant therapy by year of diagnosis, age at diagnosis, race, Charlson/Deyo Score, treatment facility type, tumor size, or extent of surgery. Those who received adjuvant chemoradiation had significantly better one-year OS than those who received adjuvant radiation alone (35.3% vs. 16.2%, p<0.001). On subset analysis, this significant one-year OS benefit was observed in septuagenarians, those with Charlson/Deyo Score of 0, and in those with tumor size ≤5cm. On multivariate analysis, receipt of adjuvant chemoradiation and greater extent of resection were independent prognostic factors for improved OS. Our data suggests that adjuvant chemoradiation is an independent prognostic factor for improved OS in elderly patients with gliosarcoma, and the results of our study can serve as estimated benchmarks for outcome in this growing and important patient population. Its benefit, however, may be limited to septuagenarians and those with lower comorbidity burden.

External Validation of the New International Association for the Study of Lung Cancer Tumor, Node, and Metastasis 8th Edition Staging System and Updated T Descriptors in Determining Prognosis for Patients With Non-Small Cell Lung Cancer Patients With N3 Disease.

OBJECTIVE: The objective of this study is to externally validate the 8th Edition of the Tumor, Node, and Metastasis staging system and its updated T descriptors in patients with non-small cell lung cancer with N3 disease. METHODS: Data were extracted from the Surveillance, Epidemiology, and End Results database. Chi-square test, Kaplan-Meier method, and Cox regression models were used in SPSS 23.0 (IBM Corp, Armonk, NY). RESULTS: A total of 7732 patients with non-small cell lung cancer with T1-4N3M0 disease from 1988 to 2013 were identified. A total of 1410 patients (18.2%) had T1N3 disease, 2491 patients (32.2%) had T2N3 disease, 1563 patients (20.2%) had T3N3 disease, and 2268 patients (29.3%) had T4N3 disease. The 5-year overall survival (OS) for the entire cohort was 8.4%. There was a significant difference in OS concerning T stage (T1N3: 10.8% vs. T2N3: 8.3% vs. T3N3: 8.1% vs. T4N3: 7.3%; P < .001). When stratified by the median age of patients (66 years), a significant difference in OS by stage of disease (IIIB vs. IIIC) was still observed in both the younger (P < .001) and older (P < .001) patient populations. A significant difference in disease-specific survival (DSS) was observed by T stage (T1N3: 14.7% vs. T2N3: 11.6% vs. T3N3: 11.3% vs. T4N3: 9.7%; P < .001). On multivariate analysis, T stage, year of diagnosis, age, gender, histology, and receipt of radiotherapy remained independent prognostic factors for both OS and DSS. CONCLUSIONS: The 8th Edition of the Tumor, Node, and Metastasis staging system significantly stratifies both overall and DSS between stages IIIB and IIIC among those with N3 disease. However, small absolute differences in 5-year outcomes between T stage may suggest limited clinical relevance.

Association of Insurance and Community-Level Socioeconomic Status With Treatment and Outcome of Squamous Cell Carcinoma of the Pharynx.

Importance: Community-level socioeconomic status, particularly insurance status, is increasingly becoming important as a possible determinant in patient outcomes. Objective: To determine the association of insurance and community-level socioeconomic status with outcome for patients with pharyngeal squamous cell carcinoma (SCC). Design, Setting, and Participants: This study extracted data from more than 1500 Commission on Cancer-accredited facilities collected in the National Cancer Database. A total of 35 559 patients diagnosed with SCC of the pharynx from 2004 through 2013 were identified. The χ2 test, Kaplan-Meier method, and Cox regression models were used to analyze data from April 1, 2016, through April 16, 2017. Main Outcomes and Measures: Overall survival was defined as time to death from the date of diagnosis. Results: Among the 35 559 patients identified (75.6% men and 24.4% women; median age, 61 years [range, 18-90 years]), 15 146 (42.6%) had Medicare coverage; 13 061 (36.7%), private insurance; 4881 (13.7%), Medicaid coverage; and 2471 (6.9%), no insurance. Uninsured patients and Medicaid recipients were more likely to be younger, black, or Hispanic; to have lower median household income and lower educational attainment; to present with higher TNM stages of disease; and to start primary treatment at a later time from diagnosis. Those with private insurance (reference group) had significantly better overall survival than uninsured patients (hazard ratio [HR], 1.72; 95% CI, 1.59-1.87), Medicaid recipients (HR, 1.99; 95% CI, 1.88-2.12), or Medicare recipients (HR, 2.07; 95% CI, 1.99-2.16), as did those with median household income of at least $63 000 (reference) vs $48 000 to $62 999 (HR, 1.19; 95% CI, 1.13-1.26), $38 000 to $47 999 (HR, 1.31; 95% CI, 1.24-1.38), and less than $38 000 (HR, 1.51; 95% CI, 1.43-1.59). On multivariable analysis, insurance status and median household income remained independent prognostic factors for overall survival even after accounting for educational attainment, race, Charlson/Deyo comorbidity score, disease site, and TNM stage of disease. Conclusions and Relevance: Insurance status and household income level are associated with outcome in patients with SCC of the pharynx. Those without insurance and with lower household income may significantly benefit from improving access to adequate, timely medical care. Additional investigations are necessary to develop targeted interventions to optimize access to standard medical treatments, adherence to physician management recommendations, and subsequently, prognosis in these patients at risk.

Racial disparities in anaplastic oligodendroglioma: An analysis on 1643 patients.

The objective of our study is to determine the influence of race on overall survival (OS) for anaplastic oligodendroglioma (AO). Data were extracted from the National Cancer Data Base (NCDB). Chi-square test, Kaplan-Meier method, and Cox regression models were employed in SPSS 22.0 (Armonk, NY: IBM Corp.) for data analyses. 1643 patients with AO were identified. 1386 (84.3%) were White, 83 (5.0%) Black, 133 (8.1%) Hispanic, and 41 (2.5%) were Asian. White and Black patients were significantly older than Hispanic and Asian patients (49.3% vs. 49.4% vs. 33.1% vs. 39.0%, p=0.003). Black patients were significantly less likely to be insured than White patients (12.8 vs. 7.2%, p<0.001) and significantly more likely to have lower income than other races (p<0.001). A trend towards higher comorbidity burden and lower rate of gross total resection was seen in Black patients. Black patients had significantly worse five-year OS compared to White, Hispanic, and Asian patients (40.3% vs. 52.3% vs. 67.8% vs. 67.7%, p=0.028). Of those who received adjuvant chemoRT, Black patients still had significantly worse OS compared to White patients (p=0.021). On multivariate analysis, Black race, older age at diagnosis, and not receiving adjuvant chemoradiotherapy were independent prognostic factors for worse OS in anaplastic oligodendroglioma. Future studies are warranted to help determine predictors for unfavorable molecular status, ways to optimize management of comorbidities, and interventions to help ensure adequate access to medical care for all patients to better care for those who may be at more risk for poorer outcome.

Influence of insurance status and income in anaplastic astrocytoma: an analysis of 4325 patients.

To determine the impact of insurance status and income for anaplastic astrocytoma (AA). Data were extracted from the National Cancer Data Base. Chi square test, Kaplan-Meier method, and Cox regression models were employed in SPSS 22.0 (Armonk, NY: IBM Corp.) for data analyses. 4325 patients with AA diagnosed from 2004 to 2013 were identified. 2781 (64.3%) had private insurance, 925 (21.4%) Medicare, 396 (9.2%) Medicaid, and 223 (5.2%) were uninsured. Those uninsured were more likely to be Black or Hispanic versus White or Asian (p < 0.001), have lower median income (p < 0.001), less educated (p < 0.001), and not receive adjuvant chemoradiation (p < 0.001). 1651 (38.2%) had income ≥$63,000, 1204 (27.8%) $48,000-$62,999, 889 (20.5%) $38,000-$47,999, and 581 (13.4%) had income <$38,000. Those with lower income were more likely to be Black or Hispanic versus White or Asian (p < 0.001), uninsured (p < 0.001), reside in a rural area (p < 0.001), less educated (p < 0.001), and not receive adjuvant chemoradiation (p < 0.001). Those with private insurance had significantly higher overall survival (OS) than those uninsured, on Medicaid, or on Medicare (p < 0.001). Those with income ≥$63,000 had significantly higher OS than those with lower income (p < 0.001). On multivariate analysis, age, insurance status, income, and adjuvant therapy were independent prognostic factors for OS. Being uninsured and having income <$38,000 were independent prognostic factors for worse OS in AA. Further investigations are warranted to help determine ways to ensure adequate medical care for those who may be socially disadvantaged so that outcome can be maximized for all patients regardless of socioeconomic status.

Idiopathic midaortic syndrome with malignant hypertension in 3-year-old boy.

Midaortic syndrome (MAS) is a rare vascular disease that commonly causes renovascular hypertension. The lumen of the abdominal aorta narrows and the ostia of the branches show stenosis. MAS is associated with diminished pulses in the lower extremities compared with the upper extremities, severe hypertension with higher blood pressure in the upper rather than lower extremities, and an abdominal bruit. The clinical symptoms are variable, and recognition in children with hypertension can aid early diagnosis and optimal treatment. Hypertension with MAS is malignant and often refractory to several antihypertensive drugs. Recently, radiologic modalities have been developed and have led to numerous interventional procedures. We describe the case of a 3-year-old boy presenting with left ventricular hypertrophy whose severely elevated blood pressure led to the diagnosis of idiopathic MAS. This case highlights the importance of measuring blood pressure and conducting a detailed physical examination to diagnose MAS. This is the first reported case of idiopathic MAS diagnosed in childhood in Korea.

Alagille syndrome and a JAG1 mutation: 41 cases of experience at a single center.

PURPOSE: Alagille syndrome is a complex hereditary disorder that is associated with cardiac, hepatic, skeletal, ocular, and facial abnormalities. Mutations in the Notch signaling pathway, such as in JAG1 and NOTCH2, play a key role in embryonic development. A cardiac or hepatic presentation is a critical factor for determining the prognosis. METHODS: We conducted a retrospective study of 41 patients with Alagille syndrome or a JAG1 mutation between 1983 and 2013. RESULTS: The first presentations were jaundice, murmur, cyanosis, and small bowel obstruction at a median age of 1.0 months (range, 0-24 months). The JAG1 mutation was found in 27 of the 28 genetically-tested patients. Cardiovascular anomalies were identified in 36 patients, chronic cholestasis was identified in 34, and liver transplantation was performed in 9. There was no significant correlation between the severity of the liver and cardiac diseases. The most common cardiovascular anomaly was peripheral pulmonary stenosis (83.3%), with 13 patients having significant hemodynamic derangement and 12 undergoing surgical repair. A total bilirubin level of >15 mg/dL with a complex surgical procedure increased the surgical mortality (P=0.022). Eight patients died after a median period of 2.67 years (range, 0.33-15 years). The groups with fetal presentation and with combined severe liver and heart disease had the poorest survival (P<0.001). CONCLUSION: The group with combined severe liver and heart disease had the poorest survival, and a multidisciplinary approach is necessary to improve the outcome.