Behavioral and Emotional Outcomes in Children with Congenital Heart Disease: Effects of Disease Severity, Family Life Stress, Disease-Related Chronic Stress, and Psychosocial Adaptation.

OBJECTIVE: To evaluate whether effects of congenital heart disease (CHD) severity and family life stress on behavioral and emotional functioning are mediated by disease-related chronic stress and psychosocial adaptation. STUDY DESIGN: A cross-sectional analysis of the Pediatric Cardiac Quality of Life Inventory Testing Study was performed. Relationships between CHD severity (comprising 3 groups: mild heart disease, moderate biventricular disease, and single ventricle) and family life stress, on patient- and parent disease-related chronic stress, psychosocial adaptation, and behavioral-emotional outcomes were assessed using structural equation modeling. Patient and parent models were reported separately. RESULTS: There were 981 patient-parent dyads: 22% had mild heart disease, 63% biventricular, and 15% single ventricle; 19% of families reported moderate to major family life stress. Path models revealed that CHD severity and family life stress were mediated by disease-related chronic stress and psychosocial adaptation factors (R2 = 0.18-0.24 for patient outcomes and R2 = 0.33-0.34 for parent outcomes, P < .001, respectively). CONCLUSIONS: The effects of greater CHD severity and family life stress on behavioral-emotional outcomes were mediated by worse disease-related chronic stress and psychosocial adaptation factors. Both disease-related chronic stress and psychosocial adaptation factors may be targets for interventions to improve behavioral and emotional outcomes.

Deployed Dimensions of the GORE® CARDIOFORM ASD Occluder as Function of Defect Size.

The GORE® CARDIOFORM ASD occluder (ASDO) is approved for closure of ASDs up to 35 mm diameter. With an adaptable central waist, each device size is suitable over a range of defect diameters. Understanding deployed dimensions across various defect sizes will assist operators. Therefore, this study investigates the deployed dimensions of the ASDO as a function of defect size. A 2-mm-thick ASD model with circular defects ranging from 5 to 35 mm was 3D printed. Diameter, width, and left-right disc diameter were measured by fluoroscopy after ASDO devices were deployed in applicable defects. Linear regression evaluated relationships between device size, defect size, and deployed dimensions. Six ASDOs of each size (27, 32, 37, 44, and 48 mm) were deployed in all applicable defects. There was significant ASDO size-defect size interaction in determining deployed ASDO diameter. Diameter was positively associated with defect size for 48-mm (B = 0.13, p < 0.001) and 44-mm (B = 0.11, p < 0.001) ASDOs, while no association was seen for 27-mm, 32-mm, or 37-mm ASDOs. No such interaction existed for deployed width or left-right disc difference. Controlling for ASDO size, width (B = - 0.12, p < 0.001) and left-right disc difference (B = - 0.06, p < 0.001) were negatively associated with defect size. In smaller defects, the 44-mm and 48-mm ASDOs display progressive diameter foreshortening, and all devices display progressive increase in width and left-right disc asymmetry. Anticipating the degree of diameter foreshortening may be critical when attempting closure of fenestrated lesions and/or in patients with limited total atrial septal length.

Aortic root dilation in a child with Marfan syndrome and mosaic Turner syndrome.

Patients with a known genetic cause of aortic root dilation usually have a single underlying aetiology, either a single gene defect as in Marfan syndrome or chromosomal anomaly as in Turner syndrome. However, it is possible, although unlikely, for a patient to inherit multiple independent risk factors for aortic root dilation. We describe such a patient, who inherited Marfan syndrome and a very unusual form of mosaic Turner syndrome. Long-term follow-up of this patient may provide insight into the natural history of this unique genetic combination.

Use of a Dilatable exGraft Conduit in Single-Ventricle Palliation.

Surgically paced prosthetic conduits are commonly used in the treatment of congenital heart disease. A major limitation of available prosthetic grafts is that they do not grow with the patient. We describe a human case of percutaneous balloon dilation of a surgically placed exGraft conduit (PECA Labs, Inc, Pittsburgh, PA) in a neonate with single-ventricle disease. The use of dilatable conduits could change the management of many congenital heart defects and greatly reduce both the morbidity of repeat cardiac reoperations and the deleterious effects of prolonged conduit dysfunction that accrue between surgical conduit revisions.

Decline in Ocular Toxoplasmosis over 40 Years at a Tertiary Referral Practice in the United States.

PURPOSE: To identify whether there has been a decline in ocular toxoplasmosis at a tertiary uveitis practice. METHODS: Retrospective review of new patients at the University of Illinois Uveitis Service from 1973 to 2012. RESULTS: There were 6820 patients with adequate records for inclusion; 323 (4.7%) were diagnosed with ocular toxoplasmosis. There was a 78.0% decline in prevalence of ocular toxoplasmosis from 2008 to 2012 compared with 1973 to 1977. Compared with the aggregate uveitis population, toxoplasmosis patients were more likely to be Hispanic (p<0.0001) and less likely to be African American (p<0.0001). Ocular toxoplasmosis in Hispanics commonly occurred in foreign-born patients (85.3%). CONCLUSIONS: The diagnosis of ocular toxoplasmosis at our clinic declined, with Hispanics accounting for an increasing proportion of cases. These trends are consistent with the decreasing toxoplasmosis seropositivity in the United States, but may also reflect decreased referrals due to improved management of ocular toxoplasmosis in primary clinics.

Risk of Second Seizure in Pediatric Patients With Idiopathic Autism.

PURPOSE: Epilepsy is a comorbidity of idiopathic autism spectrum disorder. The aim was to characterize the risk and time of second seizure in children with idiopathic autism spectrum disorder. METHODS: A retrospective review was performed at the University of Chicago and NorthShore University HealthSystem. Patients with idiopathic autism spectrum disorder, ≥1 seizure, and age 2 to 23 years were included. RESULTS: 153 patients were included; 141 (92%) had a second seizure. The average age at first seizure was 7.14 years (median: 5.08 years) and 8.12 years (median: 7.3 years) at second seizure. Average time between first and second seizure was 7.68 months. DISCUSSION: A high risk of seizure recurrence was found in this population. There was a short time to second seizure, with most having a recurrence within 1 year. These findings may be used to guide therapy in children with autism spectrum disorder and epilepsy.