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Background:Caring for infants after the first-stage palliative surgery for single-ventricle heart disease bring challenges beyond the usual parenting responsibilities. Current studies fail to capture the nuances of caregivers’ experiences during the most critical “interstage” period between the first and second surgery.Objectives:To explore the perceptions of caregivers about their experiences while transitioning to caregiver roles, including the successes and challenges associated with caregiving during the interstage period.Methods:Constructivist Grounded Theory methodology guided the collection and analysis of data from in person or telephonic interviews with caregivers after their infants underwent the first-stage palliative surgery for single-ventricle heart disease, and were sent to home for 2–4 months before returning for their second surgery. Symbolic interactionism informed data analyses and interpretation.Results:Our sample included 14 parents, who were interviewed 1–2 times between November, 2019 and July, 2020. Most patients were mothers (71%), Latinx (64%), with household incomes Conclusions:Parents transitioned to caregiver roles by developing a sense of self-reliance and, in the process, gained self-confidence and decision-making skills. Our study responded to the key research priority from the AHA Scientific Statement to address the knowledge gap in home monitoring for interstage infants through qualitative research design.

Adolescents with single ventricle heart disease (SVHD) exhibit mood and cognitive deficits, which may result from injury to the basal ganglia structures, including the caudate nuclei. However, the integrity of the caudate in SVHD adolescents is unclear. Our aim was to examine the global and regional caudate volumes, and evaluate the relationships between caudate volumes and cognitive and mood scores in SVHD and healthy adolescents. We acquired two high-resolution T1-weighted images from 23 SVHD and 37 controls using a 3.0-Tesla MRI scanner, as well as assessed mood (Patient Health Questionnaire-9 [PHQ-9]; Beck Anxiety Inventory [BAI]) and cognition (Montreal Cognitive Assessment [MoCA]; Wide Range Assessment of Memory and Learning–2; General Memory Index [GMI]) functions. Both left and right caudate nuclei were outlined, which were then used to calculate and compare volumes between groups using ANCOVA (covariates: age, gender, and head-size), as well as perform 3D surface morphometry. Partial correlations (covariates: age, gender, and head-size) were used to examine associations between caudate volumes, cognition, and mood scores in SVHD and controls. SVHD subjects showed significantly higher PHQ-9 and BAI scores, indicating more depressive and anxiety symptoms, as well as reduced GMI scores, suggesting impaired cognition, compared to controls. SVHD patients showed significantly reduced caudate volumes (left, 3,198.8 ± 490.1 vs. 3,605.0 ± 480.4 mm(3), p < 0.004; right, 3,162.1 ± 475.4 vs. 3,504.8 ± 465.9 mm(3), p < 0.011) over controls, and changes were localized in the rostral, mid-dorsolateral, and caudal areas. Significant negative correlations emerged between caudate volumes with PHQ-9 and BAI scores and positive correlations with GMI and MoCA scores in SVHD and controls. SVHD adolescents show significantly reduced caudate volumes, especially in sites that have projections to regulate mood and cognition, which may result from developmental and/or hypoxia-/ischemia-induced processes.

Background: Adolescents with single ventricle heart disease (SVHD), who have undergone the Fontan procedure, show cognitive/memory deficits. Mammillary bodies are key brain sites that regulate memory; however, their integrity in SVHD is unclear. We evaluated mammillary body (MB) volumes and their associations with cognitive/memory scores in SVHD and controls. Methods: Brain MRI data were collected from 63 adolescents (25 SVHD; 38 controls) using a 3.0-Tesla MRI scanner. Cognition and memory were assessed using Montreal Cognitive Assessment (MoCA) and Wide Range Assessment of Memory and Learning 2. MB volumes were calculated and compared between groups (ANCOVA, covariates: age, sex, and total-brain-volume [TBV]). Partial correlations and linear regression were performed to examine associations between volumes and cognitive scores (covariates: age, sex, and TBV). Results: SVHD group showed significantly lower MoCA and WRAML2 scores over controls. MB volumes were significantly reduced in SVHD over controls. After controlling for age, sex, and TBV, MB volumes correlated with MoCA and delayed memory recall scores in SVHD and controls. Conclusion: Adolescents with SVHD show reduced MB volumes associated with cognitive/memory deficits. Potential mechanisms of volume losses may include developmental and/or hypoxic/ischemic-induced processes. Providers should screen for cognitive deficits and explore possible interventions to improve memory.

Background: Retinoblastoma is a malignant tumor of the eye that typically presents in early childhood and occurs in approximately 1 in 20,000 births. While active treatment of the tumor is typically completed in childhood, survivors often suffer from long-term effects from treatment including visual impairment, facial deformities, and fear of recurrence or secondary cancer. However, little is known how these long-term effects affect their health-related quality of life (HRQOL). Purpose: To review the literature on HRQOL in retinoblastoma survivors. Method: We searched three electronic databases from January 2005 to December 2018 for original research articles reporting on HRQOL or individual domains such as function, cognition, and psychosocial outcomes in retinoblastoma survivors. Results: A total of 59 articles were reviewed and 15 were identified as eligible. Five of the studies reported worse HRQOL in retinoblastoma survivors than controls or general population norms. Parent-proxy ratings were worse than survivors’ self-reports. Conclusion: Our findings confirm the need for further HRQOL research to assess the factors influencing long-term outcomes associated with treatment in adolescent and young adult retinoblastoma survivors. By identifying any potential deficits in specific domains of HRQOL, early interventions might be developed to improve HRQOL in retinoblastoma survivors.

BACKGROUND Single ventricle heart disease (SVHD) adolescents show cognitive impairments and anxiety and depressive symptoms, indicating the possibility of brain injury in regions that control these functions. However, brain tissue integrity in cognition, anxiety, and depression regulatory sites in SVHD remains unclear. We examined brain tissue changes in SVHD compared to controls using T2-relaxometry procedures, which measure free water content and show tissue injury. METHODS Proton-density and T2-weighted images, using a 3.0-Tesla MRI, as well as anxiety (Beck anxiety inventory [BAI]), depressive symptoms (patient health questionnaire-9 [PHQ-9]), and cognition (wide range assessment of memory and learning 2 [WRAML2] and Montreal cognitive assessment [MoCA]) data were collected from 20 SVHD (age: 15.8 ± 1.1 years, male/female: 11/9) and 36 controls (age: 16.0 ± 1.1 years, male/female: 19/17). Whole-brain T2-relaxation maps were calculated, normalized to a common space, smoothed, and compared between groups and sexes (analysis of covariance; covariates: age, sex; p

Background Cognitive deficits are common, long-term sequelae in children and adolescents with congenital heart disease (CHD) who have undergone surgical palliation. However, there is a lack of a validated brief cognitive screening tool appropriate for the outpatient setting for adolescents with CHD. One candidate instrument is the Montreal Cognitive Assessment (MoCA) questionnaire. Objective The purpose of the research was to validate scores from the MoCA against the General Memory Index (GMI) of the Wide Range Assessment of Memory and Learning, 2nd Edition (WRAML2), a widely accepted measure of cognition/memory, in adolescents and young adults with CHD. Methods We administered the MoCA and the WRAML2 to 156 adolescents and young adults ages 14–21 (80 youth with CHD and 76 healthy controls who were gender and age matched). Spearman’s rank order correlations were used to assess concurrent validity. To assess construct validity, the Mann–Whitney U test was used to compare differences in scores in youth with CHD and the healthy control group. Receiver operating characteristic curves were created and area under the curve, sensitivity, specificity, positive predictive value, and negative predictive value were also calculated. Results The MoCA median scores in the CHD versus healthy controls were (23, range 15–29 vs. 28, range 22–30; p < .001), respectively. With the screening cutoff scores at

Neurodevelopmental disability is common after operations for congenital heart defects. We previously showed that patient and preoperative factors, center, and calendar year of birth explained less than 30% of the variance for the Psychomotor Development Index (PDI) and the Mental Development Index (MDI) of the Bayley Scales of Infant Development-Second Edition. Here we investigate how much additional variance in PDI and MDI is contributed by operative variables and postoperative events.We analyzed neurodevelopmental outcomes after operations with cardiopulmonary bypass at age 9 months or younger between 1996 and 2009. We used linear regression to investigate the effect of operative factors (age, weight, and cardiopulmonary bypass variables) and postoperative events on neurodevelopmental outcomes, adjusting for center, type of congenital heart defect, year of birth, and preoperative factors.We analyzed 1,770 children from 22 institutions with neurodevelopmental testing at age 13.3 months (range, 6 to 30 months). Among operative factors, longer total support time was associated with lower PDI and MDI (p0.05). When postoperative events were added, use of either extracorporeal membrane oxygenation or ventricular assist device support, and longer postoperative length of stay were associated with lower PDI and MDI (p0.05). Longer total support time was not a significant predictor in these models. After adjusting for patient, preoperative, intraoperative, and postoperative factors, measured intraoperative and postoperative factors accounted for 5% of the variances in PDI and MDI.Operative factors may be less important than innate patient and preoperative factors and postoperative events in predicting early neurodevelopmental outcomes after cardiac operations in infants. Neurodevelopmental outcomes improved over calendar time when adjusted for patient and medical variables.

PURPOSE: Single ventricle heart disease (SVHD) patients show injury in brain sites that regulate autonomic, mood, and cognitive functions. However, the nature (acute or chronic changes) and extent of brain injury in SVHD are unclear. Our aim was to examine regional brain tissue damage in SVHD over controls using DTI-based mean diffusivity (MD), axial diffusivity (AD), radial diffusivity (RD), and fractional anisotropy (FA) procedures. METHODS: We collected two DTI series (3.0–Tesla MRI), mood and cognitive data from 27 SVHD and 35 control adolescents. Whole-brain MD, AD, RD, and FA maps were calculated from each series, realigned and averaged, normalized to a common space, smoothed, and compared between groups using ANCOVA (covariates, age and sex; false-discovery-rate, p

Adolescents with single ventricle heart disease (SVHD) show autonomic, mood, and cognitive deficits, indicating aberrations in brain areas that regulate these functions. However, the gray matter integrity in autonomic, mood, and cognitive control sites is unclear. We examined regional brain gray matter changes, using high-resolution T1-weighted images (3.0-Tesla magnetic resonance scanner) with voxel based morphometry procedures, as well as mood and cognitive functions in SVHD (n = 18; age, 15.7±1.1 years; male, 10) and controls (n = 31; age, 16.0±1.1 years; male, 17). High-resolution T1-weighted images were realigned, gray matter tissue type partitioned, normalized to a common space, smoothed, and compared between groups (analysis of covariance; covariates, age and gender). The mood and cognitive scores were compared between groups using independent samples t-tests. SVHD subjects showed significantly altered mood and cognitive functions over controls. Significantly reduced gray matter emerged in multiple brain areas, including the thalamus, caudate nuclei, putamen, insula, prefrontal, post-central and precentral gyrus, occipital gyrus, para-hippocampal gyrus, temporal gyrus, and cerebellar sites in SVHD over controls. SVHD subjects show compromised gray matter integrity in autonomic, mood and cognitive control sites. The findings indicate that frequent deficits found in SVHD subjects have a brain structural basis in the condition.

ObjectiveChildren with hypoplastic left heart syndrome are at a risk for neurodevelopmental delays. Current guidelines recommend systematic evaluation and management of neurodevelopmental outcomes with referral for early intervention services. The Single Ventricle Reconstruction Trial represents the largest cohort of children with hypoplastic left heart syndrome ever assembled. Data on life events and resource utilisation have been collected annually. We sought to determine the type and prevalence of early intervention services used from age 1 to 4 years and factors associated with utilisation of services.MethodsData from 14-month neurodevelopmental assessment and annual medical history forms were used. We assessed the impact of social risk and geographic differences. Fisher exact tests and logistic regression were used to evaluate associations.ResultsAnnual medical history forms were available for 302 of 314 children. Greater than half of the children (52–69%) were not receiving services at any age assessed, whereas 20–32% were receiving two or more therapies each year. Utilisation was significantly lower in year 4 (31%) compared with years 1–3 (with a range from 40 to 48%) (pConclusionDespite significant neurodevelopmental delays, early intervention service utilisation was low in this cohort. As survival has improved for children with hypoplastic left heart syndrome, attention must shift to strategies to optimise developmental outcomes, including enrolment in early intervention when merited.

BACKGROUND A healthy work environment can improve patient outcomes and registered nurse (RN) turnover. Creating cultures of retention and fostering healthy work environments are 2 major challenges facing nurse leaders today. SPECIFIC AIMS Examine the effects of the healthy work environment (communication, collaboration, and leadership) on RN turnover from data collected from a research study. METHODS Descriptive, cross-sectional, correlational design. Pediatric critical care RNs from 10 pediatric intensive care units (PICU) completed the Practice Environment Scale of the Nursing Work Index Revised and a subscale of the Intensive Care Unit Nurse-Physician Communication Questionnaire. These staff nurses were asked whether they intend to leave their current job in the next 6 months. Statistical analysis included correlations, multiple linear regression, t tests (2-tailed), and 1-way analysis of variance. RESULTS A total of 415 RNs completed the survey. There was a statistically significant relationship between leadership and the intent to leave (P < .05). There was also an inverse relationship between years of experience and intent to leave. None of the communication variables between RNs and among RNs and MDs or collaboration were significantly associated with PICU nurses' intention to leave. CONCLUSION Effective leadership in the PICU is important to PICU RNs and significantly influences their decisions about staying in their current job.

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.

This study was conducted to determine the prevalence of fatigue and identify its demographic, clinical, and psychological correlates in 150 heart failure (HF) patients (73% men, 66% Caucasian, mean age 55.0 years, mean ejection fraction 26.7%+/-11%), from a single HF center, using the Profile of Mood States-Fatigue Subscale, the Minnesota Living With Heart Failure Questionnaire, and the Beck Depression Inventory. Sociodemographic and clinical data were obtained through self-report and chart abstraction. High levels of fatigue were reported in 50.4% of men and 51.2% of women. In a multivariate model, maximal workload, physical health, emotional health, and depression explained 51% of the variance in fatigue (P

BACKGROUND: Neurodevelopmental disability is the most common complication for survivors of surgery for congenital heart disease (CHD). METHODS: We analyzed individual participant data from studies of children evaluated with the Bayley Scales of Infant Development, second edition, after cardiac surgery between 1996 and 2009. The primary outcome was Psychomotor Development Index (PDI), and the secondary outcome was Mental Development Index (MDI). RESULTS: Among 1770 subjects from 22 institutions, assessed at age 14.5 ± 3.7 months, PDIs and MDIs (77.6 ± 18.8 and 88.2 ± 16.7, respectively) were lower than normative means (each P < .001). Later calendar year of birth was associated with an increased proportion of high-risk infants (complexity of CHD and prevalence of genetic/extracardiac anomalies). After adjustment for center and type of CHD, later year of birth was not significantly associated with better PDI or MDI. Risk factors for lower PDI were lower birth weight, white race, and presence of a genetic/extracardiac anomaly (all P ≤ .01). After adjustment for these factors, PDIs improved over time (0.39 points/year, 95% confidence interval 0.01 to 0.78; P = .045). Risk factors for lower MDI were lower birth weight, male gender, less maternal education, and presence of a genetic/extracardiac anomaly (all P < .001). After adjustment for these factors, MDIs improved over time (0.38 points/year, 95% confidence interval 0.05 to 0.71; P = .02). CONCLUSIONS: Early neurodevelopmental outcomes for survivors of cardiac surgery in infancy have improved modestly over time, but only after adjustment for innate patient risk factors. As more high-risk CHD infants undergo cardiac surgery and survive, a growing population will require significant societal resources.

Background Persistent pleural effusions after the Fontan procedure contribute to prolonged hospitalization and increased costs. We report our experience using a modified Wisconsin Fontan protocol to reduce chest tube drainage and hospital length of stay (LOS). Methods Single institutional retrospective chart review of 120 consecutive patients (60 before and 60 after initiation of our protocol) undergoing an extracardiac Fontan procedure from January 2004 to February 2007. Protocol influence was assessed by comparing group differences on duration of pleural drainage, requirement for nothing by mouth/total parenteral nutrition, hospital LOS, readmission for pleural effusion, and total hospital costs. Results Groups were similar in demographic characteristics, single ventricle morphology, preoperative hemodynamic parameters, and operative and immediate postoperative management. Median duration of pleural drainage and hospital LOS was reduced in the post- versus preprotocol groups: 4 days (interquartile range [IQR], 4-5 days) pre versus 6 days (IQR, 5-10 days) ( P P = .005), respectively. Pleural drainage lasting >1 week was also less common postprotocol: 23 (38%) before versus 7 (12%) after ( P = .001). Fewer postprotocol patients required nothing by mouth/total parenteral nutrition to control effusions: 5 pre versus 0 post ( P = .06), and fewer readmissions for effusions (14 before vs 7 after [ P = .1]). An average total cost savings of 22% and readmissions savings of 29% resulted in nearly $500,000 in institutional savings over the study period. Conclusions A modified Fontan protocol resulted in reduced time to chest tube removal, hospital LOS, and chest tube drainage lasting >1 week. There was a strong trend toward avoiding nothing by mouth/total parenteral nutrition to control pleural effusion and lower hospital costs.

Background We sought to characterize growth between birth and age 3 years in infants with hypoplastic left heart syndrome who underwent the Norwood procedure. Methods and Results We performed a secondary analysis using the Single Ventricle Reconstruction Trial database after excluding patients z score ( LAZ ) and weight‐for‐age z score ( WAZ ) at birth and age 3 years and change in WAZ over 4 clinically relevant time periods. We identified correlates of change in WAZ and LAZ using multivariable linear regression with bootstrapping. Mean WAZ and LAZ were below average relative to the general population at birth ( P P =0.05, respectively) and age 3 years ( P WAZ occurred between birth and Norwood discharge; the greatest gain occurred between stage II and 14 months. At age 3 years, WAZ and LAZ were WAZ differed among time periods. Shunt type was associated with change in WAZ only in the Norwood discharge to stage II period; subjects with a Blalock‐Taussig shunt had a greater decline in WAZ than those with a right ventricle‐pulmonary artery shunt ( P =0.002). Conclusions WAZ changed over time and the predictors of change in WAZ varied among time periods. By age 3 years, subjects remained small and three times as many children were short as were underweight (>2 SD below normal). Failure to find consistent risk factors supports the strategy of tailoring nutritional therapies to patient‐ and stage‐specific targets. Clinical Trial Registration URL: http://clinicaltrials.gov/ . Unique identifier: NCT00115934.

To assess variation in feeding practice at hospital discharge after the Norwood procedure, factors associated with tube feeding, and associations among site, feeding mode, and growth before stage II.From May 2005 to July 2008, 555 subjects from 15 centers were enrolled in the Pediatric Heart Network Single Ventricle Reconstruction Trial; 432 survivors with feeding data at hospital discharge after the Norwood procedure were analyzed.Demographic and clinical variables were compared among 4 feeding modes: oral only (n = 140), oral/tube (n = 195), nasogastric tube (N-tube) only (n = 40), and gastrostomy tube (G-tube) only (n = 57). There was significant variation in feeding mode among sites (oral only 0%-81% and G-tube only 0%-56%, P.01). After adjusting for site, multivariable modeling showed G-tube feeding at discharge was associated with longer hospitalization, and N-tube feeding was associated with greater number of discharge medications (R(2) = 0.65, P.01). After adjusting for site, mean pre-stage II weight-for-age z-score was significantly higher in the oral-only group (-1.4) vs the N-tube-only (-2.2) and G-tube-only (-2.1) groups (P = .04 and .02, respectively).Feeding mode at hospital discharge after the Norwood procedure varied among sites. Prolonged hospitalization and greater number of medications at the time of discharge were associated with tube feeding. Infants exclusively fed orally had a higher weight-for-age z score pre-stage II than those fed exclusively by tube. Exploring strategies to prevent morbidities and promote oral feeding in this highest risk population is warranted.

Journal of Cardiovascular Nursing Vol. 00, No. 0, pp 00Y00 x Copyright B 2012 Wolters Kluwer Health | Lippincott Williams & Wilkins Prevalence, Awareness, Treatment, and Control of Hypertension Among Arab Americans Ayman Tailakh, PhD, RN; Janet C. Mentes, PhD, APRN, BC, FGSA; Donald E. Morisky, ScD, MSPH, ScM; Nancy A. Pike, PhD, RN, CPNP-AC, FAHA; Linda R. Phillips, PhD, RN, FAAN; Lorraine S. Evangelista, PhD, RN, FAHA, FAAN Background: Hypertension (HTN) is a major risk factor for heart disease, which is the leading cause of death in the United States. Hypertension detection and blood pressure (BP) control are critically important for reducing the risk of myocardial infarction and strokes. Although there are more than 3.5 million Arab Americans in the United States, there are no national or regional data on HTN prevalence among Arab Americans. Objective: This study aims to estimate the prevalence of HTN in a community sample of Arab Americans; assess levels of awareness, treatment, and control in hypertensive patients; and describe and compare lifestyle behaviors (eg, physical activity, nutrition, and weight control). Methods: In this cross-sectional, descriptive study, 126 participants completed a self-administered questionnaire to measure physical activity, nutrition, and medical history. Height and weight were measured. Three BP measurements were obtained at 60-second intervals after resting for 5 minutes. Hypertension was defined as a mean systolic BP of 140 mm Hg or higher, or a diastolic BP 90 mm Hg or higher, and/or taking antihypertensive medications. Results: Overall, 36.5% of participants had HTN and 39.7% had pre-HTN. Among hypertensive participants, only 67.4% were aware of their high BP, and 52.2% were taking antihypertensive medication. Among those taking medication, 46% had controlled BP. The prevalence of HTN was higher in men than in women (45.9% and 23.2%, respectively; P = .029) and increased with age (P = .01). Hypertensive participants also had higher body mass index (mean, 31.55 kg/m 2 ) compared with normotensive participants (mean, 28.37 kg/m 2 ; P = .01). Conclusion: Our results indicate that HTN and pre-HTN are highly prevalent in Arab Americans. Hypertension awareness and control rates were inadequate and low compared with national data. These results emphasize the urgent need to develop public health strategies to improve the prevention, detection, and treatment of HTN among Arab Americans. KEY WORDS: adherence, Arab Americans, hypertension, prevalence Ayman Tailakh, PhD, RN Assistant Professor, College of Nursing, Sultan Qaboos University, Muscat, Oman. Janet C. Mentes, PhD, APRN, BC, FGSA Associate Professor, School of Nursing, University of California, Los Angeles. Donald E. Morisky, ScD, MSPH, ScM Professor, Department of Community Health Sciences, School of Public Health, University of California, Los Angeles. Nancy A. Pike, PhD, RN, CPNP-AC, FAHA Assistant Professor, School of Nursing, University of California, Los Angeles. Linda R. Phillips, PhD, RN, FAAN Professor, School of Nursing, University of California, Los Angeles. Lorraine S. Evangelista, PhD, RN, FAHA, FAAN Associate Professor, Program of Nursing Science, University of California, Irvine. The authors have no funding or conflicts of interest to disclose. Correspondence Ayman Tailakh, PhD, RN, College of Nursing, Sultan Qaboos University, PO Box 66 Al-Khodh, 123 Muscat, Oman (atailakh@squ.edu.om). DOI: 10.1097/JCN.0b013e31825638ae Background Hypertension (HTN) is a major risk factor for heart disease, which is the leading cause of death in the United States. 1 Hypertension is the third leading cause of death in the world, with a mortality rate of 13%. 2 Almost 30% of American adults live with HTN. 3 The estimated cost of HTN in the United States for 2010 was $174 billion. 4 Hypertension prevention and treatment have been addressed as national health goals for Healthy People 2020, which include reducing the prevalence of HTN from a baseline of 29.9% to 26.9% and increasing HTN control rate from 43.7% to 61.2%. 5 Racial/ethnic disparities in awareness and treat- ment of HTN persist in the United States; this raises a concern, as minority ethnic groups are more likely to develop HTN before the age of 50 years. 1 There are more than 3.5 million Arab Americans in the United States, 6 which represents a new minority ethnic Copyright © 2012 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.

Although previously described in patients undergoing staged palliation for univentricular heart disease, the mechanism by which hepatic venous flow prevents development of pulmonary arteriovenous malformations is still not completely understood. We present a case in which successful H-type Fontan revision with rerouting of hepatic venous flow through a hemiazygous vein successfully reversed the progression of severe left pulmonary arteriovenous malformations.

Background: Treatment for most children with cancer includes the use of a central venous catheter (CVC). CVCs provide reliable venous access for delivery of chemotherapy and supportive care. This advantage is mitigated by an increased risk of bloodstream infections (BSIs). Despite the ubiquitous use of CVCs, few prospective studies have been conducted to address infection prevention strategies in pediatric oncology patients. Design: Prospective, crossover pilot study of a CVC team intervention versus standard care. Setting: Two inpatient oncology units in a metropolitan children’s hospital. Patients: A total of 41 patients/135 admissions for the experimental unit (EU) and 41/129 admissions for the control unit (CU). Methods: Patients received a CVC blood draw bundle procedure by a CVC registered nurse (RN) team member (experimental intervention: EU) for 6 months and by the assigned bedside RN (standard care: CU) for 6 months. Feasibility of implementing a CVC RN team; a significant difference in CVC-related BSIs between the team intervention versus standard care and risk factors associated in the development of CVC-related BSIs were determined. Results: There were 7 CVC-related BSIs/1238 catheter days in the EU group (5.7/1000 catheter days) versus 3 CVC-related BSIs/1419 catheter days in the CU group (2.1/1000 catheter days; P = .97). Selected risk factors were not significantly associated with the development of a CVC-related BSI. Conclusions: A CVC team in the care of pediatric oncology patients is feasible; however, a larger cohort will be required to adequately determine the effectiveness of the team reducing CVC-related BSIs.

In the Single Ventricle Reconstruction trial, infants with hypoplastic left heart syndrome (HLHS) who received a right-ventricle-to-pulmonary-artery shunt (RVPAS) versus a modified Blalock-Taussig shunt (MBTS) had lower early postoperative mortality, but more complications at 14 months. We explored the effect of shunt type and other patient, medical, and surgical factors on postoperative length of stay (LOS) after the Fontan operation.Fontan postoperative course was ascertained from medical record review. Cox proportional hazards modeling was used to identify factors associated with LOS.Of 327 subjects who underwent Fontan, 323 were analyzed (1 death, 1 biventricular repair, 2 with missing data). Median age and weight at Fontan were 2.8 years (interquartile range [IQR]: 2.3, 3.4) and 12.7 kg (IQR: 11.4, 14.1), respectively. Fontan type was extracardiac in 55% and lateral tunnel in 45%; 87% were fenestrated. The RVPAS and MBTS subjects had similar LOS (median 11 days [IQR: 9, 18] vs 10 days [IQR: 9, 13]; P = .23). Independent risk factors for longer LOS were treatment center (P.01), LOS at stage II (hazard ratio [HR] 1.02 for each additional day; P.01), and pre-Fontan complications (HR 1.03 for each additional complication; P = .04). Use of deep hypothermic circulatory arrest at Fontan (HR 0.64; P = .02) was independently associated with shorter LOS. When center was excluded from the model, pre-Fontan complications and use of circulatory arrest were no longer significant; instead, older age at stage II (HR 1.08 for each additional month; P = .01) predicted longer LOS. In 254 subjects who had a pre-Fontan echocardiogram, at least moderate tricuspid regurgitation was independently associated with longer LOS, both with center (HR 1.72; P.01) and without center in the model (HR 1.49; P = .02).In this multicenter prospective cohort of subjects with HLHS, Norwood shunt type was not associated with Fontan LOS. Rather, global measures of earlier medical complexity indicate greater likelihood of longer LOS after the Fontan operation.

Advances in treatment of congenital heart disease (CHD) have enabled nearly 1.3 million children in the United States with significant heart defects to survive into adulthood.1 At present, adult survivors of CHD are equal in number to children with this disease.2,3 Single ventricle congenital heart disease (SVCHD) is considered 1 of the most severe forms of CHD and requires multiple surgical procedures in most cases.4 Although the Fontan procedure provides satisfactory surgical palliation,5 it leaves the patient with a single right or left ventricle and increased risk of late cardiac failure, exercise intolerance, and arrhythmias.5,6 Along with the medical residua of CHD, there is the potential for psychosocial difficulties.7–13 Depressive symptoms, anxiety, and behavioral and social adjustment issues have been identified in the general CHD population.7–14 However, conflicting reports exist regarding the level of psychological distress in CHD when compared with healthy peers. To our knowledge, there are no reports evaluating depression in adolescents or adults who have undergone the Fontan procedure. Few studies have addressed health status14–16 and QOL17–19 in adolescents or adults who have undergone the Fontan procedure. Unfortunately, many of these studies had small sample sizes, included young children, and used a variety of health status instruments to evaluate QOL.17–19 The meaning ascribed to QOL is subjective and differs among individuals, thus making it difficult to compare or contrast the literature on QOL when different conceptualizations and measurements are used. As SVCHD mortality declines related to surgical palliation, the focus of clinical care and surgical modifications has turned to morbidity and QOL outcomes.14 However, studies have shown inconsistencies in parent proxy reports with both overestimations and underestimations of QOL, health status, and functional abilities.20–24 The current adolescent and adult Fontan survivors are from a previous surgical era. They have now reached an appropriate age to contribute meaningful self-report data on this phenomenon. This information is imperative for future comparisons of QOL, health status, and psychosocial issues in the current surgical era of single ventricle palliation. The purpose of this study was to compare QOL, health status, and prevalence of depression in adolescents and adults with SVCHD who have undergone the Fontan procedure with those of age-matched, healthy control group and identify predictors of QOL in the Fontan group.

Single-ventricle congenital heart disease (SVCHD) requires multiple palliative surgical procedures that leave visible surgical scars and physical deficits, which can alter body-image and self-esteem. This study aimed to compare sex and age differences in body-image, selfesteem, and body mass index (BMI) in adolescents and adults with SVCHD after surgical palliation with those of a healthy control group. Using a comparative, cross-sectional design, 54 adolescent and adult (26 male and 28 female) patients, age 15-50 years, with SVCHD were compared with 66 age-matched healthy controls. Body-image and self-esteem were measured using the Multidimensional Body-Self Relations Questionnaire-Appearance Scale and Rosenberg Self-Esteem Scale. Height and weight were collected from retrospective chart review, and BMI was calculated. Female adolescents and adult patients with SVCHD reported lower body image compared with males patients with SVCHD and healthy controls (p = 0.003). Specific areas of concern were face (p = 0.002), upper torso or chest (p = 0.002), and muscle tone (p = 0.001). Patients with SVCHD who were \21 years of age had lower body image compared with healthy controls (p = 0.006). Self-esteem was comparable for both patients with SVCHD and healthy peers. There were no sex differences in BMI; BMI was higher in subjects[21 years of age (p = 0.01). Despite the similarities observed in selfesteem between the two groups, female patients with SVCHD\21 years of age reported lower perceived bodyimage. Our findings support the need to recognize poor psychological adjustment related to low self-esteem in patients with SVCHD; female patients warrant increased scrutiny. Strategies to help patients with SVCHD cope with nonmodifiable aspects of body-image during the difficult adolescent-to-young adult years may potentially enhance self-esteem and decrease psychological distress. © Springer Science+Business Media, LLC 2012.

The study aims to describe the clinical profile of the adult Fontan survivor and identify the worries, symptoms, and the impact of cardiac surveillance most commonly experienced.A descriptive, cross-sectional design was used.The study was performed in outpatient adult and pediatric cardiology clinics in university-affiliated and private practice offices.Fifty-four adolescent and adult patients with single ventricle congenital heart disease who have undergone the Fontan procedure participated in the study. The mean age was 26 ± 9 years with 52% female and 63% Caucasian.Demographic and clinical data were obtained by a standard intake form and retrospective chart reviews. The Congenital Heart Disease TNO/AZL Adult Quality Of Life questionnaire was completed to assess worries, symptoms, and the impact of cardiac surveillance.The majority were single (73%), employed or full-time students (93%), with health insurance (94%), had a single left ventricle (78%), the diagnosis of tricuspid atresia or double inlet left ventricle (59%), lateral tunnel Fontan type (44%), history of arrhythmias (76%), left ventricle ejection fraction percentage50 (66%), oxygen saturations90% (70%), frequent headaches (50%), scoliosis (22%), varicose veins, ascites, and liver cirrhosis (46%), normal body mass index (59%), and New York Heart Association class I (48%) and II-III (52%). Primary worries related to current health (83%), job/employment (69%), ability to work, (61%) and living independently (54%). The most bothersome symptoms were shortness of breath with ambulation (69%), dizziness (61%), and palpitations (61%).Fontan survivors experience residua and sequelae from multiple surgical procedures and the natural disease course. Our results support the need for ongoing assessment of both physical symptoms and psychosocial concerns, and suggest the need for multiple risk factor intervention strategies that improve physical and emotional health in Fontan survivors.

W ITH THE INTRODUCTION of minimally invasive surgical techniques for patent ductus arteriosus (PDA) ligation, the length of hospital stay after the surgery has been substantially reduced.1,2 Regional anesthesia combined with light general anesthesia may further facilitate rapid recovery from surgery and anesthesia, allowing for same-day discharge, or fast-tracking.3 The authors report their initial experience of fast-tracking PDA ligation.

The Norwood procedure with a modified Blalock-Taussig (MBT) shunt, the first palliative stage for single-ventricle lesions with systemic outflow obstruction, is associated with high mortality. The right ventricle-pulmonary artery (RVPA) shunt may improve coronary flow but requires a ventriculotomy. We compared the two shunts in infants with hypoplastic heart syndrome or related anomalies.Infants undergoing the Norwood procedure were randomly assigned to the MBT shunt (275 infants) or the RVPA shunt (274 infants) at 15 North American centers. The primary outcome was death or cardiac transplantation 12 months after randomization. Secondary outcomes included unintended cardiovascular interventions and right ventricular size and function at 14 months and transplantation-free survival until the last subject reached 14 months of age.Transplantation-free survival 12 months after randomization was higher with the RVPA shunt than with the MBT shunt (74% vs. 64%, P=0.01). However, the RVPA shunt group had more unintended interventions (P=0.003) and complications (P=0.002). Right ventricular size and function at the age of 14 months and the rate of nonfatal serious adverse events at the age of 12 months were similar in the two groups. Data collected over a mean (+/-SD) follow-up period of 32+/-11 months showed a nonsignificant difference in transplantation-free survival between the two groups (P=0.06). On nonproportional-hazards analysis, the size of the treatment effect differed before and after 12 months (P=0.02).In children undergoing the Norwood procedure, transplantation-free survival at 12 months was better with the RVPA shunt than with the MBT shunt. After 12 months, available data showed no significant difference in transplantation-free survival between the two groups. (ClinicalTrials.gov number, NCT00115934.)