Contemporary Management of the Failing Fontan.

Adult patients with congenital heart disease have now surpassed the pediatric population due to advances in surgery and improved survival. One such complex congenital heart disease seen in adult patients is the Fontan circulation. These patients have complex physiology and are at risk for several complications, including thrombosis of the Fontan pathway, pulmonary vascular disease, heart failure, atrial arrhythmias, atrioventricular valve regurgitation, and protein-losing enteropathy. This review discusses the commonly encountered phenotypes of Fontan circulatory failure and their contemporary management.

Hypertrophic Cardiomyopathy After Heart Transplantation: A Single-Center Case Series.

We present 3 heart transplant recipients who developed hypertrophic cardiomyopathy years after transplantation. In all 3 cases, the diagnosis was initially made based on echocardiography and confirmed using cardiac magnetic resonance imaging. (Level of Difficulty: Advanced.).

Comparison of Uveitis Incidence by Medication in Juvenile Idiopathic Arthritis and Implications for Screening.

PURPOSE: To determine uveitis incidence in juvenile idiopathic arthritis (JIA) patients treated with disease-modifying antirheumatic drugs (DMARD) medications, and to evaluate uveitis risk-stratification protocols. DESIGN: Retrospective clinical cohort study. METHODS: Medical records of patients with JIA seen by ophthalmology at a single institution from April 2014 to April 2022 and ≥18 months' follow-up were reviewed. Exclusion criteria included uveitis history prior to study period, Still disease, or <18 months' follow-up. Patient characteristics, medications, and uveitis status were recorded. Factors associated with uveitis development were analyzed and statistically significant metrics used to determine empiric risk-stratification criteria. These criteria and American College of Rheumatology (ACR) risk-stratification guidelines were applied retroactively to determine predictive power. RESULTS: One hundred eighty-four patients met inclusion criteria and were included. Twenty-one new cases of uveitis developed during the study period. There were no statistically significant differences between no DMARD treatment, methotrexate (MTX), and etanercept (ETA) groups in uveitis incidence, whereas the adalimumab (ADA) and other biologics groups had no uveitis cases. Under the empirically determined criteria, the ratio of uveitis incidence between high- and low-risk groups was 8.21 (2.68-33.55; P < .0001), whereas it was 1.90 (0.72-4.93; P = .15) under the ACR criteria. CONCLUSION: Patients on MTX, ETA, and no DMARD treatment were comparable in JIA-associated uveitis incidence, whereas there were no new cases with ADA or other biologics. Further, we found increased predictive power in the empiric criteria in comparison to current ACR risk stratification.

Sex- and race-specific associations of bone mineral density with incident heart failure and its subtypes in older adults.

BACKGROUND: Previous studies have suggested an association between bone mineral density (BMD) and heart failure (HF) risk that may be race-dependent. METHODS: We evaluated the relationship between BMD and incident HF in a cohort of older adults, the Health, Aging, and Body Composition (Health ABC) study (n = 2835), and next performed a pooled analysis involving a second older cohort, the Cardiovascular Health Study (n = 1268). Hip BMD was measured by dual-energy X-ray absorptiometry in both cohorts and spine BMD by computed tomography in a subset from Health ABC. RESULTS: In Health ABC, lower BMD at the total hip was associated with higher incident HF in Black women after multivariable adjustment. Similar associations were found for BMD at the femoral neck and spine. In both cohorts, pooled analysis again revealed an association between lower total hip BMD and increased risk of HF in Black women (HR = 1.41 per 0.1-g/cm2 decrement [95% CI = 1.23-1.62]), and showed the same to be true for White men (HR = 1.12 [1.03-1.21]). There was a decreased risk of HF in Black men (HR 0.80 [0.70-0.91]), but no relationship in White women. The associations were numerically stronger with HFpEF for Black women and White men, and with HFrEF for Black men. Findings were similar for femoral neck BMD. Sensitivity analyses delaying HF follow-up by 2 years eliminated the association in Black men. CONCLUSIONS: Lower BMD was associated with higher risk of HF and especially HFpEF in older Black women and White men, highlighting the need for additional investigation into underlying mechanisms.

Prediction for Cycloplegic Refractive Error in Chinese School Students: Model Development and Validation.

Purpose: To predict cycloplegic refractive error using measurements obtained under noncycloplegic conditions. Method: Refractive error was measured in 5- to 18-year-old Chinese students using a NIDEK autorefractor before and after administration of 0.5% tropicamide. Spherical equivalent (SER) in diopters (D) was calculated as sphere plus half cylinder. A multivariable prediction model for cycloplegic SER was developed using data from students in Jinyun (n = 1938) and was validated using data from students in Hangzhou (n = 1498). The performance of the prediction model was evaluated using R2, mean difference between predicted and measured cycloplegic SER, and sensitivity and specificity for predicting myopia (cycloplegic SER ≤ -0.5 D). Results: Among 3436 students (mean age, 9.7 years; 51% female), the mean (SD) noncycloplegic and cycloplegic SER values were -1.12 (1.97) D and -0.20 (2.19) D, respectively. The prediction model that included demographics, noncycloplegic SER, axial length/corneal curvature radius ratio, uncorrected visual acuity (UCVA), and intraocular pressure predicted cycloplegic SER with R2 of 0.93 in the development dataset and 0.92 in the validation dataset. The mean (SD) differences between predicted and measured cycloplegic SER were 0.0 (0.55) D in the development dataset and 0.06 (0.64) D in the validation dataset. In both the development and validation datasets, the combination of predicted SER and UCVA yielded high sensitivity (91.4% and 91.9%, respectively) and specificity (95.0% and 90.1%, respectively) for detecting myopia. Conclusions: Cycloplegic refractive error can be predicted using measurements obtained under noncycloplegic conditions. The prediction model could potentially be used to correct the myopia prevalence in epidemiological studies in which administering cycloplegic agent on all participants is not feasible. Translational Relevance: The prediction model may provide a tool for correcting the overestimation of myopia from noncycloplegic refractive error in future epidemiological studies in which administering cycloplegic agent on all participants is not feasible.

Effect of Cycloplegia on Refractive Error Measure in Chinese School Students.

PURPOSE: To determine differences in cycloplegic vs. non-cycloplegic refractive error and factors associated with these differences in Chinese school students. METHOD: In this cross-sectional school-based study, refractive error was measured in school students using a NIDEK autorefractor before and after administration of 0.5% tropicamide. Spherical equivalent (SER) in diopters (D) was calculated as sphere plus half cylinder. SER differences before vs. after cycloplegia were evaluated using mean, standard deviation (SD), 95% limits of agreement. Univariable and multivariable regression models were used to determine factors associated with SER differences. RESULTS: Among 3604 students, 3450 (95.7%) provided data for analysis. Mean age (SD) was 9.7 (3.6) years. The mean SER (SD) was -1.12 (1.97) D before cycloplegia, and -0.20 (2.19) D after cycloplegia, with a mean difference of 0.92 D (95% limits of agreement: -0.93 to 2.78 D). Among 196 eyes with non-cycloplegic SER -6.0 D or worse (e.g., met high myopia definition), 71.4% had cycloplegic SER -6.0 D or worse, and among 3607 eyes with non-cycloplegic SER -0.5 D or worse (e.g., met myopia definition), 62.1% eyes had cycloplegic SER -0.5 D or worse. Cycloplegic SER was more correlated with axial length than non-cycloplegic SER (Pearson r = 0.82 vs. 0.72, p < .0001). In multivariable analysis, larger SER differences were associated with more hyperopic refractive error and smaller axial length (all p < .0001). CONCLUSION: Non-cycloplegic refractive error overestimates myopia by approximately one diopter. This overestimation increases with more hyperopic refractive error and smaller axial length. Non-cycloplegic refractive error should not be used for evaluating pediatric myopia. ABBREVIATIONS: BCVA = best corrected visual acuity; D = diopter; SD = standard deviation; SE = standard error; SER = spherical equivalent; CI = confidence interval.

Predictive Value of Clinician "Gestalt" in Pediatric Community-Acquired Pneumonia.

OBJECTIVES: Validated prognostic tools for pediatric community-acquired pneumonia (CAP) do not exist. Thus, clinicians rely on "gestalt" in management decisions for children with CAP. We sought to determine the ability of clinician gestalt to predict severe outcomes. METHODS: We performed a prospective cohort study of children 3 months to 18 years old presenting to a pediatric emergency department (ED) with lower respiratory infection and receiving a chest radiograph for suspected CAP from 2013 to 2017. Clinicians reported the probability that the patient would develop severe complications of CAP (defined as respiratory failure, empyema or effusion, lung abscess or necrosis, metastatic infection, sepsis or septic shock, or death). The primary outcome was development of severe complications. RESULTS: Of 634 children, 37 (5.8%) developed severe complications. Of children developing severe complications after the ED visit, 62.1% were predicted as having <10% risk by the ED clinician. Sensitivity was >90% at the <1% predicted risk threshold, whereas specificity was >90% at the 10% risk threshold. Gestalt performance was poor in the low-intermediate predicted risk category (1%-10%). Clinicians had only fair ability to discriminate children developing complications from those who did not (area under the receiver operator characteristic curve 0.747), with worse performance from less experienced clinicians (area under the receiver operator characteristic curve 0.693). CONCLUSIONS: Clinicians have only fair ability to discriminate children with CAP who develop severe complications from those who do not. Clinician gestalt performs best at very low or higher predicted risk thresholds, yet many children fall in the low-moderate predicted risk range in which clinician gestalt is limited. Evidence-based prognostic tools likely can improve on clinician gestalt, particularly when risk is low-moderate.