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Introduction: Single ventricle heart disease (SVHD) adolescents with a single right ventricle (RV) have worse cognition and mood function compared to single left ventricle (LV) which may result from variability in ventricular function or structure related sequela after Fontan completion. However, it is unclear whether RV SVHD has worse cerebral blood flow (CBF) in cognitive and mood regulatory areas over LV SVHD compared to healthy controls. Methods: Cross-sectional, comparative design, 14 adolescents with RV SVHD (age 16.1±1.5 years; 7 male), 6 LV SVHD (age, 16.3±1.0 years; 4 male), and 25 healthy controls (age, 15.9±1.4 years; 13 male) were studied. SVHD participants were recruited who have undergone surgical palliation with Fontan completion from local pediatric cardiology clinics. Self-reported healthy controls were recruited from the community. Brain MRI studies were performed using a 3.0-Tesla MRI scanner and 3D pseudo-continuous arterial spin labelling data were collected. We calculated whole-brain CBF maps, normalized to a common space, and assessed brain changes between RV and LV SVHD and controls [ANCOVA; covariates, age and sex; p Results: Regional brain CBF was reduced in single RV over LV SVHD. Multiple brain sites showed more widespread reduced CBF values in RV over LV SVHD compared to controls (Figure 1, p Conclusion: Single RV adolescents show more widespread reduced CBF than single LV in cognitive and mood regulatory sites, which may result from variable function or structure related sequela between ventricle types. The findings indicate that the therapeutic approach should recognize the differences in CBF based on ventricle type and investigate interventions to optimize CBF in single RV SVHD.

It has been 50 years since Francis Fontan pioneered the operation that today bears his name. Initially designed for patients with tricuspid atresia, this procedure is now offered for a vast array of congenital cardiac lesions when a circulation with 2 ventricles cannot be achieved. As a result of technical advances and improvements in patient selection and perioperative management, survival has steadily increased, and it is estimated that patients operated on today may hope for a 30-year survival of >80%. Up to 70 000 patients may be alive worldwide today with Fontan circulation, and this population is expected to double in the next 20 years. In the absence of a subpulmonary ventricle, Fontan circulation is characterized by chronically elevated systemic venous pressures and decreased cardiac output. The addition of this acquired abnormal circulation to innate abnormalities associated with single-ventricle congenital heart disease exposes these patients to a variety of complications. Circulatory failure, ventricular dysfunction, atrioventricular valve regurgitation, arrhythmia, protein-losing enteropathy, and plastic bronchitis are potential complications of the Fontan circulation. Abnormalities in body composition, bone structure, and growth have been detected. Liver fibrosis and renal dysfunction are common and may progress over time. Cognitive, neuropsychological, and behavioral deficits are highly prevalent. As a testimony to the success of the current strategy of care, the proportion of adults with Fontan circulation is increasing. Healthcare providers are ill-prepared to tackle these challenges, as well as specific needs such as contraception and pregnancy in female patients. The role of therapies such as cardiovascular drugs to prevent and treat complications, heart transplantation, and mechanical circulatory support remains undetermined. There is a clear need for consensus on how best to follow up patients with Fontan circulation and to treat their complications. This American Heart Association statement summarizes the current state of knowledge on the Fontan circulation and its consequences. A proposed surveillance testing toolkit provides recommendations for a range of acceptable approaches to follow-up care for the patient with Fontan circulation. Gaps in knowledge and areas for future focus of investigation are highlighted, with the objective of laying the groundwork for creating a normal quality and duration of life for these unique individuals. ispartof: CIRCULATION vol:140 issue:6 pages:E234-E284 ispartof: location:United States status: published

Background— Survivors of the Norwood procedure may experience neurodevelopmental impairment. Clinical trials to improve outcomes have focused primarily on methods of vital organ support during cardiopulmonary bypass. Methods and Results— In the Single Ventricle Reconstruction trial of the Norwood procedure with modified Blalock-Taussig shunt versus right-ventricle-to-pulmonary-artery shunt, 14-month neurodevelopmental outcome was assessed by use of the Psychomotor Development Index (PDI) and Mental Development Index (MDI) of the Bayley Scales of Infant Development-II. We used multivariable regression to identify risk factors for adverse outcome. Among 373 transplant-free survivors, 321 (86%) returned at age 14.3±1.1 (mean±SD) months. Mean PDI (74±19) and MDI (89±18) scores were lower than normative means (each P R 2 =26%) were clinical center ( P =0.003), birth weight P =0.023), longer Norwood hospitalization ( P P R 2 =34%) included center ( P P =0.04), genetic syndrome/anomalies ( P =0.04), lower maternal education ( P =0.04), longer mechanical ventilation after the Norwood procedure ( P P Conclusions— Neurodevelopmental impairment in Norwood survivors is more highly associated with innate patient factors and overall morbidity in the first year than with intraoperative management strategies. Improved outcomes are likely to require interventions that occur outside the operating room. Clinical Trial Registration— URL: http://www.clinicaltrials.gov . Unique identifier: NCT00115934.

Background: Adolescents with single ventricle heart disease (SVHD), who have undergone staged surgical palliation, have increased risk for neurocognitive deficits. However, the type and location of brain injury in SVHD adolescents remains unclear. Purpose: Examine brain injury, using Diffusion Tensor Imaging (DTI) based axial (gray matter injury) and radial diffusivity (white matter injury) procedures in adolescents with SVHD who have undergone surgical palliation compared to controls. Methods: Six SVHD (age 16.2±1 years; 4 males; BMI 20±3.5 kg/m2), and 8 controls (age 16.2±1 years; 4 males; BMI 25.4±8.1 kg/m2) were examined via brain DTI on a 3T MRI. No subject had a history of prematurity, cardiac arrest, ECMO use, genetic syndrome, head trauma or severe developmental delays. Whole-brain axial/radial diffusivity maps were calculated. Statistics consisted of ANCOVA, with age and gender as covariates (uncorrected threshold, p < 0.005). Results: Increased axial diffusivity in SVHD compared to controls were found in several brain sites that include frontal gyrus (p=.03), temporal gyrus (p=.01), parahippocampal gyrus (p=.008), occipital gyrus (p=.02), insular cortex (p=.04), cerebellar cortex (p=.004), pons (p=.02), and cingulate gyrus (p=.006) (Fig1). Increased radial diffusivity appeared in frontal gyrus (p=.01), cingulate gyrus (p=.001), corpus callosum (p=.04), cerebellum (p=.004), temporal gyrus (p=.004), parahippocampal gyrus (p=.004), and pons (p=.004) (Fig2). Conclusions/Implications: Brain injury was more extensive in gray matter than white matter and was located in autonomic and cognitive areas of the brain in adolescents with SVCHD. These findings indicate that the neurocognitive deficits commonly reported in SVCHD are likely related to brain injury. Further investigation is needed to clarify these relationships and to evaluate potential interventions to repair and/or protect these brain regions in this high risk patient population.