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Adolescents and young adults with surgically treated congenital heart disease (CHD) have been shown to exhibit difficulties with executive functions; however, the neural underpinnings of these impairments have not been previously examined with functional magnetic resonance imaging (fMRI). The current study employed fMRI to examine the neural mechanisms during a letter n-back task of working memory compared to vigilance. Seventeen participants with CHD (Mage = 17.76 ± 1.72 years; 88% Caucasian; 30% female; mean IQ = 104.12 ± 15.15) were compared to 17 controls (Mage = 18.40 ± 1.74 years; 70% Caucasian; 30% female; mean IQ = 110.59 ± 5.28) with similar declining performance as the n-back became more challenging. Overall, both groups activated similar frontal-parietal working-memory networks as seen in previous literature; however, some significant differences were detected between the groups. Specifically, the participants with CHD demonstrated differences within the left precuneus and the right inferior frontal gyrus. Secondary analyses indicated that this difference appeared to be due to less task-induced deactivation (TID) in the CHD group during working memory and greater working-memory TID in the control group. In CHD, prefrontal fMRI deactivation on working-memory tasks correlated with improved working-memory performance. Future complementary neuroimaging research with functional connectivity is warranted to further examine the neural underpinnings of disrupted executive function in the long-term outcomes of CHD.

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White matter disruptions have been identified in individuals with congenital heart disease (CHD). However, no specific theory-driven relationships between microstructural white matter disruptions and cognition have been established in CHD. We conducted a two-part study. First, we identified significant differences in fractional anisotropy (FA) of emerging adults with CHD using Tract-Based Spatial Statistics (TBSS). TBSS analyses between 22 participants with CHD and 18 demographically similar controls identified five regions of normal appearing white matter with significantly lower FA in CHD, and two higher. Next, two regions of lower FA in CHD were selected to examine theory-driven differential relationships with cognition: voxels along the left uncinate fasciculus (UF; a tract theorized to contribute to verbal memory) and voxels along the right middle cerebellar peduncle (MCP; a tract previously linked to attention). In CHD, a significant positive correlation between UF FA and memory was found, r(20)=.42, p=.049 (uncorrected). There was no correlation between UF and auditory attention span. A positive correlation between MCP FA and auditory attention span was found, r(20)=.47, p=.027 (uncorrected). There was no correlation between MCP and memory. In controls, no significant relationships were identified. These results are consistent with previous literature demonstrating lower FA in younger CHD samples, and provide novel evidence for disrupted white matter integrity in emerging adults with CHD. Furthermore, a correlational double dissociation established distinct white matter circuitry (UF and MCP) and differential cognitive correlates (memory and attention span, respectively) in young adults with CHD.

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We report the case of an acutely ill 3-year-old female, with a previous medical history of Kawasaki disease, who presented to care with an acute myocardial infarction. We describe the coordinated therapies employed by pediatric and adult cardiologists aimed to establish coronary revascularization.

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BACKGROUND: Chest pain is a frequent chief complaint among the pediatric population. To date, limited data exist on the full spectrum of emergent cardiac disease among such patients; and existing data have been limited to relatively small cohorts. OBJECTIVES: The aims of the study were to investigate the emergent cardiac etiologies of chest pain in a large cohort of patients presenting to a tertiary care pediatric emergency department (PED) and to examine the use of resources (electrocardiogram, chest radiograph, echocardiogram, and laboratories) in those with and without cardiac-related chest pain. METHODS: Patient visits to 2 tertiary care PEDs were evaluated over a 3 and half-year period. Records of patients less than 19 years of age with a chief complaint of chest pain and no history of cardiovascular disease were reviewed. Patients were categorized as having cardiac or noncardiac etiologies or history of cardiovascular disease at the time of discharge, based on PED attending's final diagnoses. Final diagnoses classified as emergent cardiac etiologies were determined a priori. RESULTS: Four thousand four hundred thirty-six patients reported a chief complaint of chest pain during the study period. Three percent were excluded secondary to a history of heart disease. Only 24 (0.6%) of the remaining 4288 were determined to have chest pain of cardiac origin. Those with cardiac-related chest pain had a rate of admission of 50% compared to those without cardiac disease at 4% (P < .001). Nine patients had an arrhythmia, 6 had pericarditis, 4 had myocarditis, 3 had acute myocardial infarction, and 1 had pulmonary embolism and pneumopericardium. Ninety-two percent of the cardiac-related chest pain cohort received electrocardiograms compared to those without cardiac-related chest pain at 27% (P < .01). Only 1 (4%) of 24 subjects with cardiac-related chest pain had a prior emergency department visit within 72 hours suggesting a high detection rate upon initial presentation. The most common noncardiac etiologies for the chest pain were 56% musculoskeletal disorders; 12% related to wheezing, asthma, and cough; 8% infectious causes; 6% gastrointestinal; and 4% related to sickle cell anemia. CONCLUSION: Cardiac-related chest pain in pediatric patients is rare but potentially serious. Arrhythmia was the most common cardiac-related etiology among this cohort. Those with myocarditis and myocardial infarction were the most acutely ill. An electrocardiogram in addition to history and physical examination was most useful in detecting relatively uncommon but significant cardiac-related chest pain. Using a thorough physical examination and potentially an electrocardiogram evaluation by a pediatric emergency care physician has an excellent rate of detection of cardiac-related causes.

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OBJECTIVE: Pulmonary vein stenosis is a rare, although often lethal, anomaly. Risk factors for the diagnosis of pulmonary vein stenosis are poorly characterized. In this study we sought to identify factors associated with pulmonary vein stenosis, paying particular attention to preterm birth. METHODS: By review of the cardiac database we identified all of the subjects with pulmonary vein stenosis over a 10-year period at our institution. Those children with anomalous pulmonary venous connection were not included. Patient-related variables were analyzed for their association with pulmonary vein stenosis. Pulmonary vein stenosis was diagnosed by spectral Doppler interrogation of the pulmonary veins (continuous, turbulent flow with calculated mean gradient > 5 mm Hg) and confirmed by cardiac catheterization in nearly all of the cases. RESULTS: Twenty-six patients with pulmonary vein stenosis were identified. The median age at diagnosis was 7.4 months; range: 1 day to 35 months. Congenital heart defects were present in the majority of subjects. Associated genetic syndromes were present in 8 subjects (31%). The 2-year survival rate from diagnosis was 43%. The majority of subjects (16 [61%]) were preterm. Gestational ages ranged from 24.2 to 41.0 weeks, and birth weights ranged from 460 to 4445 g. Preterm birth was strongly associated with the diagnosis of pulmonary vein stenosis, odds ratio 10.2 (95% CI 4.7-22.6), p < .001. Eleven (42%) of the 26 subjects were treated for bronchopulmonary dysplasia before being diagnosed with pulmonary vein stenosis. CONCLUSIONS: Prematurity is associated with the diagnosis of pulmonary vein stenosis. It is interesting to note that many of these patients also have intracardiac shunt lesions, which may act in concert with preterm endothelium to produce pulmonary vein stenosis.

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Pulmonary valve stenosis (PVS) is a common congenital cardiac lesion, 1/1000 live-births, the majority of patients having mild transvalvar gradients. In the present study, we sought to determine the outcome of mild PVS diagnosed by echocardiography and to propose a management algorithm that would identify patients at risk for progression of PVS, yet reduce health care expenditures. In this single-center retrospective study, we included all patients who met the following criteria: first diagnosed with PVS at <10 years of age, an initial peak systolic Doppler gradient of < or =40 mm Hg, no additional congenital heart lesions, and at least two clinical evaluations. There were 146 subjects who met these criteria. The median age at initial diagnosis was 3.9 months, with a range of 1 day to 9.9 years. The average initial peak systolic gradient (PSG) was 23.3 mm Hg (+/-7.8) and final PSG 17.1 mm Hg (+/-10.3). Over a mean length of follow-up of 4.0 years, 107/146 (73%) were later reported to have very mild PVS (PSG < or = 25 mm Hg, no clinical change or resolution of murmur). Only 3 of 146 subjects progressed to have a PSG above 40 mm Hg, with 1 undergoing a balloon valvuloplasty. In conclusion, mild PVS diagnosed in early childhood is a benign lesion, with most children essentially demonstrating resolution in the first years of life. Mild PVS identified in infancy requires a brief period of close observation. Based on these data, our management recommendations are that infants be followed closely within the first year of life. Young infants diagnosed at birth that maintain a PSG < or = 25 mm Hg at more than 6 months of age as well as those who are older than 1 year of age with a PSG < or = 40 mm Hg have a benign course and the utility of ongoing cardiology follow-up is questionable.

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