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Background: Infants born with single ventricle heart disease require in-home medicalized care during the interstage period (time between the first and second staged heart surgery). These caregivers rely on extended family, friends, and hired caretakers to provide respite time. However, the coronavirus pandemic removed these families’ options due to stay-at-home and social distancing directives. We explored the caregivers’ experiences during the interstage period, including impacts on their lifestyle, as they managed their infants’ critical needs during the coronavirus disease 2019 pandemic. Method: In-person or telephonic interviews of 14 caregivers interviewed once or twice were conducted between November 2019 and July 2020. Constructivist Grounded Theory methodology guided both data collection and analysis for the inductive and abductive exploration of caregivers’ experiences. Results: Data analysis led to the development of 2 concepts: Accepting and adapting to a restrictive home environment and Reconciling what is and what is yet to come. Refinement of the relationship between the 2 concepts led to the development of a theory grounded in the words and experiences of the participants called: A Continuous Process of Compromise. Conclusions: Our findings increase understanding of caregivers’ experiences related to psychosocial and lifestyle impacts and the need for additional support during the interstage period.

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.

OBJECTIVES Neurodevelopmental disability is the most common complication among congenital heart surgery survivors. The Bayley scales are standardized instruments to assess neurodevelopment. The most recent edition (Bayley Scales of Infant and Toddler Development 3rd Edition, Bayley-III) yields better-than-expected scores in typically developing and high-risk infants than the second edition (Bayley Scales of Infant Development 2nd Edition, BSID-II). We compared BSID-II and Bayley-III scores in infants undergoing cardiac surgery. METHODS We evaluated 2198 infants who underwent operations with cardiopulmonary bypass between 1996 and 2009 at 26 institutions. We used propensity score matching to limit confounding by indication in a subset of patients (n = 705). RESULTS Overall, unadjusted Bayley-III motor scores were higher than BSID-II Psychomotor Development Index scores (90.7 ± 17.2 vs 77.6 ± 18.8, P CONCLUSIONS The Bayley-III yielded higher scores than the BSID-II and classified fewer children as severely impaired. The systematic bias towards higher scores with the Bayley-III precludes valid comparisons between early and contemporary cardiac surgery cohorts.

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.

Background:Survivors of single ventricle heart disease must cope with the physical, neurodevelopmental, and psychosocial sequelae of their cardiac disease, which may also affect academic achievement and social relationships. The purpose of this study was to qualitatively examine the experiences of school and social relationships in adolescents with single ventricle heart disease.Methods:A descriptive phenomenological methodology was employed, utilising semi-structured interviews. Demographic and clinical characteristics were obtained via chart review.Results:Fourteen adolescents (aged 14 to 19 years) with single ventricle heart disease participated. Interviews ranged from 25 to 80 minutes in duration. Four themes emerged from the interviews, including “Don’t assume”: Pervasive ableism; “The elephant in the room”: Uncertain future; “Everyone finds something to pick on”: Bullying at school; “They know what I have been through”: Social support. The overall essence generated from the data was “optimism despite profound uncertainty.”Conclusions:Adolescents with single ventricle heart disease identified physical limitations and school challenges in the face of an uncertain health-related future. Despite physical and psychosocial limitations, most remained optimistic for the future and found activities that were congruent with their abilities. These experiences reflect “optimism despite profound uncertainty.”

This scientific statement summarizes the current state of knowledge related to interstage home monitoring for infants with shunt‐dependent single ventricle heart disease. Historically, the interstage period has been defined as the time of discharge from the initial palliative procedure to the time of second stage palliation. High mortality rates during the interstage period led to the implementation of in‐home surveillance strategies to detect physiologic changes that may precede hemodynamic decompensation in interstage infants with single ventricle heart disease. Adoption of interstage home monitoring practices has been associated with significantly improved morbidity and mortality. This statement will review in‐hospital readiness for discharge, caregiver support and education, healthcare teams and resources, surveillance strategies and practices, national quality improvement efforts, interstage outcomes, and future areas for research. The statement is directed toward pediatric cardiologists, primary care providers, subspecialists, advanced practice providers, nurses, and those caring for infants undergoing staged surgical palliation for single ventricle heart disease.

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

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

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.

Advances in paediatric healthcare are often the result of randomised clinical trials.1,2 Notable examples include trials on vaccines,3 surfactant in preterm infants,4 and oncology.5 Researchers, healthcare providers, and parents acknowledge the importance of research, but effective recruitment into paediatric clinical trials remains a difficult task.6-9 Barriers to participation in paediatric research have been well described in the literature,10-16 but the literature on strategies for recruiting critically ill infants for clinical trials is limited.17-19 The concept of equipoise or the “uncertainty principle” can present challenges to recruitment in randomised controlled trials, especially when the study drug is routinely used in clinical practice.20 The ethical principle is upheld if there is true uncertainty about which trial arm is most likely to benefit the patient.20 Preconceived notions of efficacy on the part of the investigators should be discussed in advance to eliminate the lack of equipoise as a potential barrier to recruitment. The purpose of this report is to describe the key challenges for recruitment, the strategies implemented to address these challenges, and lessons learned from this experience. In the case of neonates with complex congenital cardiac diseases and functionally univentricular hearts, parents are under extreme stress when approached to participate in research, especially if the diagnosis was not made prenatally. Parents are mourning the loss of an expected “healthy child” and adjusting to the new realities of a child who will require multiple surgical and other procedures before 3 years of age. Appropriate practices and strategies of recruitment during this emotional time are key to engaging parents and their medical providers in a conversation about clinical research and securing successful enrolment of infants. In 2001, the Pediatric Heart Network was established by the National Heart, Lung, and Blood Institute, National Institutes of Health to conduct multi-centre clinical research in children with cardiovascular disease.21 After successful recruitment to an observational study22 and a randomised placebo-controlled drug trial in Kawasaki disease,23 the Pediatric Heart Network launched the “Angiotensin-Converting Enzyme Inhibition in Infants with Single Ventricle Trial” or the “Infant Single Ventricle Trial”.24,25 This double-blind, randomised placebo-controlled trial compared the effects of enalapril with placebo on somatic growth in 230 neonates with functionally univentricular hearts. After the “Infant Single Ventricle Trial” began, recruitment was more challenging than in previous studies and enrolment began to lag. Efforts to understand and remediate the problems were further complicated by the launch of the Pediatric Heart Network’s “Single Ventricle Reconstruction Trial”, a randomised surgical trial comparing two types of surgical shunts, in May, 2005,26,27 which recruited shortly after birth a subset of infants eligible for the “Infant Single Ventricle Trial”.

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.

ObjectiveThe purpose of this analysis was to evaluate a novel strategy for reporting adverse events in the Pediatric Heart Network’s randomized surgical trial of systemic–pulmonary artery shunt versus right ventricle–pulmonary artery conduit in infants with hypoplastic left heart syndrome. The strategy was developed to align the reporting process with the needs of a surgical trial while maintaining participant safety.MethodsAdverse event reporting was analyzed for 2 groups of study subjects: those randomized to a trial arm during a period in which a standard adverse event reporting system was used (period 1) and those randomized after institution of a system that focused serious adverse event reporting on 6 sentinel events (period 2). The analysis encompassed the period from randomization (Norwood surgery) to hospital discharge from stage II surgery. Adverse event rates were compared using a Poisson regression model for the number of events per subject.ResultsFrom period 1 to period 2, the rate of serious adverse events requiring expedited reporting decreased as expected (0.42 vs 0.14/subject/month of follow-up; P

Clinical Profile of the Adolescent/Adult Fontan Survivor chd_475 Nancy A. Pike, RN, PhD,* Lorraine S. Evangelista, RN, PhD,* Lynn V. Doering, RN, PhD,* Deborah Koniak-Griffin, RN, EdD,* Alan B. Lewis, MD, † and John S. Child, MD ‡ *School of Nursing, University of California, † Division of Pediatric Cardiology, Childrens Hospital Los Angeles, Ahmanson—UCLA Adult Congenital Heart Disease Center, Los Angeles, Calif, USA ABSTRACT Objectives. 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. Design. A descriptive, cross-sectional design was used. Setting. The study was performed in outpatient adult and pediatric cardiology clinics in university-affiliated and private practice offices. Patients. 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. Outcome Measures. 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. Results. 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 percentage >50 (66%), oxygen saturations >90% (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 Conclusions. 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 Key Words. Single Ventricle; Congenital Heart Disease; Fontan; Health-related Quality of Life Introduction C ongenital heart disease (CHD) not only is a disease of pediatrics but also represents a chronic adult disease population. The clinical profile of the general CHD population is changing related to advanced age, improved surgical tech- niques, and the survival of more complex con- genital heart defects. Approximately 1.3 million children in the United States with significant heart defects have survived into adulthood. 1 At the © 2011 Copyright the Authors Congenital Heart Disease © 2011 Wiley Periodicals, Inc. present time, there are as many adults with CHD as there are children with this disease. 2 Furthermore, this population is estimated to grow at a rate of 5% per year. 3 The current and future resources required to provide optimal care for this growing population reflect the increased size and number of adult CHD programs across the United States. 4 Over the past two decades, life expec- tancy for children with single ventricle CHD has increased significantly. This is related to Congenit Heart Dis. 2011;6:9–17

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

Purpose of review Advancements in surgical technique and perioperative care have significantly improved the survival of infants born with hypoplastic left heart syndrome. A recent modification to the Norwood procedure is being adopted by many centers to improve postoperative hemodynamic stability and survival to stage II palliation. The late effects of this modification, however, are speculated and have not been investigated. Recent findings Center-specific improved short-term outcomes have been reported in a few small, nonrandomized studies of a new approach to the Norwood procedure, which utilizes a right ventricle to pulmonary artery shunt or Sano modification to provide pulmonary blood flow rather than the standard modified Blalock-Taussig shunt. Summary The classic Norwood procedure and Sano modification each have specific advantages and disadvantages in both the short and long term. Data comparing the two techniques are nonrandomized, contradictory, and utilize historical controls. The optimal shunt to improve survival to the second-stage palliation is unknown. A multicenter randomized clinical trial comparing the Sano with the modified Blalock-Taussig shunt in hypoplastic left heart syndrome or variants is currently in progress and should hopefully provide future guidelines for shunt selection based on clinical presentation.

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.

Minimally invasive pediatric cardiac surgery, although still evolving, lacks much of the corporate "thunder" directed to wards the adult patient. Because the majority of pediatric procedures remain "open" requiring cardiopulmonary bypass, innovations and/or modifications of already established tech niques might have important adult medical applications. The care of children born with congenital heart disease at our institution presently incorporates cardioscopy, active venous suction, epidural/spinal anesthesia, and, most recently, robotic video assistance. This mufti-disciplinary combination has not only optimized the cosmetics of the operation and use of hospital resources but also has maintained the safety and reproducibility of the surgical interventions.

The use of regional anesthesia in combination with general anesthesia for children undergoing cardiac surgery is the subject of a growing number of publications and presenta tions. Benefits of regional anesthesia in patients having car diac surgery include attenuation of the neuroendocrine response to surgical stress, improved postoperative pulmo nary function, enhanced cardiovascular stability, and im proved postoperative analgesia. To the extent that regional anesthesia facilitates early tracheal extubation in cardiac surgical patients, complications and costs associated with postoperative mechanical ventilation may be reduced. These benefits must, however, be weighed against the ad verse effects that may accompany the use of regional anes thesia, including hypotension, postoperative respiratory de pression, and epidural hematoma formation. In this article, the benefits and risks of regional anesthesia in infants and children undergoing open heart surgery are reviewed. In addition, specific spinal and epidural techniques currently in use are described, including management of side effects. Copyright © 2001 by W.B. Saunders Company.

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.

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.

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 advancements in surgical technique and perioperative care have significantly improved the survival of children with single ventricle (SV) congenital heart disease (CHD) over the past decade. The population who have undergone the Fontan operation are growing into adulthood and facing many unique challenges. Past research has focused on functional and neurodevelopmental outcomes with inferences made to health-related quality of life (HRQOL). With the population who have undergone the Fontan operation surviving into adulthood, little research has been directed toward the self-report of HRQOL in adolescents and young adults after surgical palliation. Questions still remain on how these patients will transition into adulthood and whether they will live normal productive lives. This article reviews the literature related to HRQOL in the SV subgroup of CHD. In addition, an overview of newly developed disease-specific HRQOL instruments is presented as well as limitations and future research in HRQOL of the SV Fontan population.

Over the past decade, advancements in cardiac surgery occurred secondary to improvements in technology and the desire for a less invasive approach to operations in general. Minimally invasive cardiac surgery has progressed from partial sternotomy incisions to totally endoscopic open-heart procedures with robotic-assistance. There are 2 major companies that produce robotic equipment for use in cardiac surgery. These companies must undergo Food and Drug Association (FDA) mandated clinical trials on each cardiac surgical procedure, before it can be approved for public use. The surgeon must demonstrate clinical proficiency to operate the robotic equipment per FDA approved company testing. The use of computer (robotic) enhancement is well documented for coronary artery bypass grafting and selected cardiac valve procedures. Recent advancements are now being directed at congenital heart disease. The use of robotic-assisted totally endoscopic atrial septal defect closure is a tremendous advancement in congenital cardiac surgery. The future of robotic cardiac surgery will hopefully expand to cover more advanced valve procedures, congenital heart defects, and other procedures once robots are further modified for pediatric use.

The Journal of Heart and Lung Transplantation Volume 25, Number 2S Abstracts Reasons for Declining Hearts (n Inotropic Support Haemodynamic Instability ECG Changes Age Past Medical History Abnormal CXR g Smoking Other Primary Reason% Secondary Reason% S117 groups was comparable (5.4%/year; 4.0%/year; 4.5%/year; all P⬎0.05). Conclusion: Donor screening without coronary angiogram over- looks significant atherosclerotic lesions in a considerable number of cases (7.0%). Therefore, angiographic screening should be performed in all donors ⱖ40 years old. Donor grafts with single-vessel CAS may be accepted as marginal hearts; however, in our opinion revascular- ization (CABG, PTCA) should be considered. Grafts with diffuse CAS have a serious risk for early graft failure. Beyond the first year the outcome of healthy grafts and grafts with donor-transmitted coronary atherosclerosis are comparable. CORRELATES OF FATIGUE IN ADVANCED HEART FAILURE PATIENTS L.S. Evangelista, 1 N. Pike, 1 A. Ter-Galstanyan, 1 M.A. Hamilton, 2 G.C. Fonarow, 2 1 School of Nursing, University of California, Los Angeles, Los Angeles, CA; 2 School of Medicine, University of California, Los Angeles, Los Angeles, CA Conclusions: This study demonstrates that hearts declined on medical grounds by one centre can safely be transplanted and should be offered out nationally. CORONARY ATHEROSCLEROSIS OF THE DONOR HEART O. Grauhan, 1 J. Pazurek, 1 H. Siniawski, 1 M. Dandel, 1 H. Lehmkuhl, 1 C. Knosalla, 1 M. Pasic, 1 M. Huebler, 1 Y. Weng, 1 R. Hetzer, 1 1 Thoracic and Cardiovascular Surgery, German Heart Institute, Berlin, Germany Objectives: Due to the shortage of donor hearts, the criteria for organ acceptability have been considerably extended and donor grafts with coronary atherosclerosis are among those offered. This study evalu- ated whether and to what degree pre-existing coronary atherosclero- sis may be acceptable. Methods: A total of 1253 consecutive HTx recipients were investi- gated retrospectively for donor-transmitted coronary atherosclerosis (DCAS). DCAS was defined as focal atherosclerosis with stenosis of at least 50%. Inclusion criteria were absence of pre-HTx angiogram but performance of angiogram or autopsy within 6 months after HTx. Hazard function and Kaplan-Meier analysis were used. Results: Eight-five out of 1253 (6.8%) cases were excluded, since coronary evaluation was not performed within 6 months (n⫽45) or hearts had undergone pre-transplant angiography (n⫽40). In 1086 patients no DCAS was found (NDCAS group) and in 82 patients (7%) DCAS was diagnosed by angiography (n⫽49) or autopsy (n⫽33). Single-vessel DCAS was found in 53/82 patients (DCAS1 group) and double- or triple-vessel DCAS in 26/82 patients (DCAS2/3 group). Three of the 82 patients with DCAS were excluded since the autopsy report was unclear regarding degree of DCAS. Early after HTx the instantaneous risk of death in the NDCAS and DCAS1 groups was 0.05 vs. 0.07 (P⬍0.05) whereas in the DCAS2/3 group it was 0.42 (P⬎0.05). However, beyond the first year the annual decrease in all Background: Fatigue is one of the most common symptoms associ- ated with heart failure (HF), and is an important impediment to maintain an active lifestyle. Unfortunately, factors associated with fatigue are poorly understood. We conducted this study to identify the significant demographic, clinical, and psychological correlates of fatigue in HF patients. Methods: One hundred and fifty HF patients (73% male, 68% Caucasian, mean age 55.4 years), from a single HF center, completed the Profile of Mood States – Fatigue Subscale, the Minnesota Living with Heart Failure, and the Beck Depression Inventory to assess levels of fatigue, quality of life (QOL), physical health, emotional health, and depression. Descriptive statistics and step-wise multiple regression were used to analyze data. Level of significance was set at 0.05. Results: Reported mean scores were as follows: fatigue, 2.4 ⫾ 1.0; QOL, 42.2 ⫾ 27.0; physical health, 18.0 ⫾ 12.0; emotional health, 8.4 ⫾ 7.0; and depression 9.9 ⫾ 8.2. Ergometry work rate (watts), lipid-lowering medication, QOL, physical health, emotional health, and depression scores were highly correlated with fatigue (p ⬍ 0.01). Additional demographic and clinical variables were not significantly related to levels of fatigue. In a multivariate model, watts, physical health, emotional health, and depression explained 51% of the variance in fatigue (p ⬍ 0.001). Conclusions: Fatigue significantly impacts QOL in physical and emotional domains. Our findings suggest the need for multiple risk factor interventions to decrease fatigue and possibly improve physical and emotional health; patients on lipid-lowering medications and depression warrant increased scrutiny and may be at high risk for this debilitating symptom of HF. ANXIETY AND QUALITY OF LIFE IN CHF PATIENTS AND THEIR CAREGIVERS D.M. Weisshaar, 1 E.L. Heiges, 1 1 CHF/Transplant Service, Kaiser Permanente Santa Teresa, San Jose, CA Background: Caregivers of CHF patients face difficult issues includ- ing concern for the patient’s physical symptoms, financial stress, and disruption in their social life. These challenges contribute to anxiety, stress, and a decrease in the perception of quality of life (QOL). Many studies have focused on depression in CHF patients and their caregivers, but few have looked into anxiety. Methods: Patients and their primary caregivers were recruited by mail from a private insured advanced outpatient CHF clinic. 65 patient and 52 caregiver respondents produced 48 patient/caregiver