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Fontan surgery is performed at 2-4 years of age and is the third planned surgical intervention for children with a univentricular heart. Major challenges for children and parents after Fontan include (a) psychological distress, (b) prolonged pleural drainage, and (c) the need for postoperative anticoagulation. The aim of this study was to evaluate a pre-Fontan video-based intervention for parents to address these challenges.

First, to compare QOL and illness perceptions between patients with a Fontan circulation and patients with anatomically simple defects (ie, atrial septal defects [ASD] or ventricular septal defects [VSD]). Second, to explore illness perceptions as a mediator of the association between congenital heart disease (CHD) diagnosis and QOL. Cross‐sectional observational study. Twenty‐four cardiology centers from 15 countries across five continents. Four hundred thirty‐five adult patients with congenital heart disease (177 Fontan and 258 ASD/VSD) ages 18‐83 years. QOL and illness perceptions were assessed by the Satisfaction With Life Scale and the Brief Illness Perceptions Questionnaire, respectively. Patients with a Fontan circulation reported lower QOL (Wald Z =−3.59, p= <.001) and more negative perceptions of their CHD (Wald Z= −7.66, p< .001) compared with patients with ASD/VSD. After controlling for demographics, anxiety, depressive symptoms, and New York Heart Association functional class, path analyses revealed a significant mediation model, αβ= 0.15, p= .002, 95% CI = 0.06‐0.25, such that CHD diagnosis was indirectly related to QOL through illness perceptions. The Fontan sample's more negative perceptions of CHD were likely a reflection of life with a more complex defect. Illness perceptions appear to account for unique differences in QOL between groups of varying CHD complexity. Psychosocial screening and interventions may be important treatment components for patients with CHD, particularly those with Fontan circulations.

The population of young adults with congenital heart disease (CHD) or a heart transplant (HTx) is growing rapidly. These survivors require lifelong cardiology care and must assume self‐management responsibilities with respect to their health. Accordingly, we sought to assess psychosocial maturity and validity of the Transition Readiness Assessment Questionnaire (TRAQ) in this population. The study was designed as a cross‐sectional observational study. The study was set at tertiary‐care pediatric and adult cardiology clinics in Edmonton and Toronto, Canada. The patients were 18‐ to 25‐year‐olds with moderate or complex CHDor a HTxin childhood. Participants completed validated instruments including the TRAQ, Erickson's Psychosocial Stage Inventory (EPSI), and Kenny's Parental Attachment Questionnaire (PAQ). We enrolled 188 participants (41% women), 109 (58%) having moderate CHD, 64 (34%) complex CHD, and 15 (8%) with a HTx. Mean age for those followed in a pediatric clinic was 21.3 ± 2.3 years, compared with 21.8 ± 2.3 years for those attending an adult clinic (P= 0.20). All questionnaire scores were similar among participants with moderate CHDvs. complex CHDvs. an HTx, and among participants followed in a pediatric clinic vs. an adult clinic. EPSIand PAQscores were similar to those of healthy populations, though perception of maternal overprotection was common. TRAQscores increased with age. A response of “not needed for my care” to 5 or more of the 29 TRAQitems was provided by 110 (75%) participants. Age, but not disease severity should be a factor when considering the transition needs of young adults with heart disease. The TRAQhas important limitations in the adult CHD/HTxpopulation and a cardiac‐specific measure of transition readiness is needed.

AbstractBackgroundImprovements in long-term survival of children undergoing the Norwood staged procedure and the arterial switch operation have resulted in the need to prepare these at-risk children for each stage of their developmental trajectory, including school readiness. This study describes and compares functional outcomes following the Norwood staged procedure and arterial switch operations.MethodsThis prospective inception cohort study comprised a sample of 73 children (71% boys) who had the Norwood staged procedure (n = 28) or the arterial switch operation (n = 45) at the age of 6 weeks or younger at the Stollery Children?s Hospital, Edmonton, Alberta, between 2002 and 2005. We excluded children who had chromosomal abnormalities or cerebral palsy. When children were 18?24 months of age, parents completed the Adaptive Behavioral Assessment System II. Standard scores for the domains are mean 100, standard deviation (15); skill area scaled scores, 10 (3). Student?s t-test with Bonferonni correction was used to compare groups.ResultsThis population has greater than four times the number of children delayed on the General Adaptive Composite than the normative group. Functional outcomes were similar in the two groups other than those of home living (Norwood: 8.8 (2.8) compared with arterial switch: 11.2 (3.1), t= 3.389, p = 0.001) and self-care (Norwood: 5.9 (3.5) versus arterial switch: 8.1 (2.6), t= 3.140, p = 0.002).ConclusionThese survivors are at increased risk for delayed functional abilities. Self-care, necessary for independence and confidence as children reach school age, was particularly low in the Norwood group. Reasons for low self-care abilities require further study.

Parents of children diagnosed with a complex congenital heart anomaly such as hypoplastic left heart syndrome HLHS are faced with the challenge of choosing the best treatment option for their children. Parents must decide among options of compassionate care, palliative reconstructive surgery, or neonatal cardiac transplantation, and additionally with antenatal diagnosis must decide regarding continuation of pregnancy. With improved survival rates yet persistent morbidities following the Norwood surgical approach for HLHS, controversy remains whether parents should be counseled on all treatment options as evidenced by research that articulates the varied opinions of health care providers. The parents' perspective is less well researched. In this study, interview data from parents who chose the Norwood-Sano surgical option were analyzed to provide insight into the various treatment options they were given following diagnosis of HLHS in their children and how they came to choose the surgical palliation option.