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Overall survival of patients with hypoplastic left heart syndrome (HLHS) has shown continued improvement. Right ventricular (RV) dysfunction, in the long term, adversely affects prognosis in these patients. This study examines changes in echocardiographic markers of RV function in a longitudinal cohort. We retrospectively reviewed patients with HLHS managed at our institution from 7/1994 to 1/2016. Follow-up included surgical and clinical data, and echocardiographic measures. Measures of RV function preceding and following all three stages of single ventricular palliation were collected. Freedom from transplant-free survival was assessed by Kaplan–Meier analysis. Multivariable associations with time to death or transplant were explored using the Cox proportional hazards model. A total of 120 patients with HLHS were identified. Norwood operation was performed in all patients. The probability of survival for the cohort was 71 ± 4.4%, 69 ± 4.5% and 66 ± 4.7% at 1, 2 and 5 years respectively after stage I Norwood operation. RV fractional area change (FAC), compared to post-Norwood was decreased at all subsequent stages with the greatest change noted post-superior cavo-pulmonary shunt from 40.7 ± 9.3% to 31.1 ± 8.3% (p 35% and TAPSE Z-score > -5, patients with RV FAC ≤ 35% and TAPSE Z-score ≤ -5 had a significantly lower transplant-free survival (p

The Fontan Outcomes Network was created to improve outcomes for children and adults with single ventricle CHD living with Fontan circulation. The network mission is to optimise longevity and quality of life by improving physical health, neurodevelopmental outcomes, resilience, and emotional health for these individuals and their families. This manuscript describes the systematic design of this new learning health network, including the initial steps in development of a national, lifespan registry, and pilot testing of data collection forms at 10 congenital heart centres.

Ventricular assist device (VAD) support for children with single ventricle (SV) heart disease remains challenging. We performed a single-center retrospective review of SV patients on VAD support and examined survival to transplant using the Kaplan-Meier method. Patients transplanted were compared with those who died on support. Between 2009 and 2017, there were 14 SV patients with 1,112 patient-days of VAD support. Stages of palliation included pre-Glenn (n = 5), Glenn (n = 5), and Fontan (n = 4). Eight patients (57%) were successfully bridged to transplant at a median 107 days. Deaths occurred early (n = 6, median 16 days) and in smaller patients (10.1 vs. 28.3 kg, P = 0.04). All Fontan patients survived to transplant, whereas only 20% of Glenn patients survived to transplant. Adverse events occurred in 79% (n = 11). Five patients met hospital discharge criteria, with two patients (one pre-Glenn, one Glenn) discharged and transplanted after 219 and 174 days of VAD support. All transplanted patients were discharged at a median 21 days posttransplant. SV patients in various stages of palliation can be successfully bridged to transplant with VAD support. With use of intracorporeal continuous-flow devices, longer-term support and hospital discharge are possible.

Objective:Infants with single ventricle physiology have arterial oxygen saturations between 75 and 85%. Home monitoring with daily pulse oximetry is associated with improved interstage survival. They are typically sent home with expensive, bulky, hospital-grade pulse oximeters. This study evaluates the accuracy of both the currently used Masimo LNCS and a relatively inexpensive, portable, and equipped with Bluetooth technology study device, by comparing with the gold standard co-oximeter.Design:Prospective, observational study.Setting:Single institution, paediatric cardiac critical care unit, and neonatal ICU.Interventions:none.Patients:Twenty-four infants under 12 months of age with baseline oxygen saturation less than 90% due to cyanotic CHD.Measurements and Results:Pulse oximetry with WristOx2 3150 with infant sensors 8008 J (study device) and Masimo LCNS saturation sensor connected to a Philips monitor (hospital device) were measured simultaneously and compared to arterial oxy-haemoglobin saturation measured by co-oximetry. Statistical analysis evaluated the performances of each and compared to co-oximetry with Schuirmann’s TOST equivalence tests, with equivalence defined as an absolute difference of 5% saturation or less. Neither the study nor the hospital device met the predefined standard for equivalence when compared with co-oximetry. The study device reading was on average 4.0% higher than the co-oximeter, failing to show statistical equivalence (p = 0.16). The hospital device was 7.4% higher than the co-oximeter and also did not meet the predefined standard for equivalence (p = 0.97).Conclusion:Both devices tended to overestimate oxygen saturation in this patient population when compared to the gold standard, co-oximetry. The study device is at least as accurate as the hospital device and offers the advantage of being more portable with Bluetooth technology that allows reliable, efficient data transmission. Currently FDA-approved, smaller portable pulse oximeters can be considered for use in home monitoring programmes.

ObjectiveThis study sets out to determine the influence of age at the time of surgery as a risk factor for post-operative length of stay after bidirectional cavopulmonary anastomosis.MethodsAll patients undergoing a Glenn procedure between January 2010 and July 2015 were included in this retrospective cohort study. Demographic data were examined. Standard descriptive statistics was used. A univariable analysis was conducted using the appropriate test based on data distribution. A propensity score for balancing the group difference was included in the multi-variable analysis, which was then completed using predictors from the univariable analysis that achieved significance of pResultsOver the study period, 50 patients met the inclusion criteria. Patients were separated into two cohorts of ⩾4 months (28 patients) and ConclusionWe found that Glenn procedures performed after 4 months of age had shorter post-operative length of stay when compared to a younger cohort. These findings suggest that balancing timing of surgery to decrease the inter-stage period should take into consideration differences in post-operative recovery with earlier operations.

Overall survival of patients with hypoplastic left heart syndrome (HLHS) has shown continued improvement. Right ventricular (RV) dysfunction, in the long term, adversely affects prognosis in these patients. This study examines changes in echocardiographic markers of RV function in a longitudinal cohort. We retrospectively reviewed patients with HLHS managed at our institution from 7/1994 to 1/2016. Follow-up included surgical and clinical data, and echocardiographic measures. Measures of RV function preceding and following all three stages of single ventricular palliation were collected. Freedom from transplant-free survival was assessed by Kaplan-Meier analysis. Multivariable associations with time to death or transplant were explored using the Cox proportional hazards model. A total of 120 patients with HLHS were identified. Norwood operation was performed in all patients. The probability of survival for the cohort was 71 ± 4.4%, 69 ± 4.5% and 66 ± 4.7% at 1, 2 and 5 years respectively after stage I Norwood operation. RV fractional area change (FAC), compared to post-Norwood was decreased at all subsequent stages with the greatest change noted post-superior cavo-pulmonary shunt from 40.7 ± 9.3% to 31.1 ± 8.3% (p 0.001). Similarly, tricuspid valve annular systolic excursion (TAPSE) Z-score declined from -2.9 ± 1.3 to -9.7 ± 1.3 (p 0.001) with a decrement at every stage of evaluation. In comparison to patients with post-Norwood RV FAC 35% and TAPSE Z-score -5, patients with RV FAC ≤ 35% and TAPSE Z-score ≤ -5 had a significantly lower transplant-free survival (p 0.0001). In patients with HLHS undergoing staged palliation, decrement in RV function manifests longitudinally. Post-Norwood RV FAC and TAPSE Z-score appear to be early markers of poor outcome in this population.

The accuracy of liver biopsy to stage fibrosis due to Fontan-associated liver disease (FALD) remains unclear. We compared the results of biopsy pre-combined heart and liver transplantation (CHLT) to the results of whole liver explant. Liver biopsy and explants from 15 Fontan patients (ages 16-49, median 28 years) were retrospectively reviewed. Staging was as follows: stage 0: no fibrosis, stage 1: pericellular fibrosis, stage 2: bridging fibrosis, and stage 3: regenerative nodules. There is no stage 4. Clinical characteristics including Model of End-stage Liver Disease eXcluding INR and Varices, Ascites, Splenomegaly, and Thrombocytopenia (VAST) scores were collected, and descriptive statistics and Mann-Whitney U tests were used to analyze the data. All patients had biopsies with at least bridging fibrosis, and all had nodularity on explant; transjugular biopsy never overestimated fibrosis. Explant showed higher-grade fibrosis (stage 3) than pre-CHLT biopsy (stage 2) in 6 of 15 patients and equal grade of fibrosis (stage 3) in 9 of 15 patients. Though clinical characteristics varied significantly, VAST score was ≥2 in all but two patients. Transjugular liver biopsy does not overestimate and can underestimate fibrosis in Fontan patients undergoing CHLT, likely due to the patchy nature of fibrosis in FALD.

s Because of remarkable advances in survival over the past 40 years, the worldwide population of individuals with single ventricle heart disease living with Fontan circulation has grown to ≈70 000, with nearly half aged >18 years. Survival to at least 30 years of age is now achievable for 75% of Fontan patients. On the other hand, single ventricle patients account for the largest group of the 6000 to 8000 children hospitalized with circulation failure, with or without heart failure annually in the United States, with the highest in‐hospital mortality. Because there is little understanding of the underlying mechanisms of heart failure, arrhythmias, pulmonary and lymphatic vascular abnormalities, and other morbidities, there are no specific treatments to maintain long‐term myocardial performance or to optimize overall patient outcomes.

Clinicians' words have a profound impact on a patient's attitude and outlook on life. Positive language, which acknowledges the uniqueness of each patient and highlights personal triumphs, can relieve anxiety and empower individuals to pursue their passions. Not only does this enhance emotional health, but an increasing body of literature indicates that positivity and mindful awareness improve aspects of physical health critical to Fontan optimization as well. There are still many unknown factors influencing long-term outcomes and quality of life. Thus, by acknowledging that today's “best available evidence” may not be “truth” over time for an individual, clinicians may open up possibilities for patients to define their own meaningful lives. Through small shifts in language, clinicians may change the narrative to the positive for individuals with Fontan circulation and in doing so offer patients the gift of “Yes” to the fullness of life.

The Fontan Outcomes Network aims to dramatically improve longevity and quality of life for people living with Fontan circulation. This mission will be achieved by improving physical health, resilience and emotional health, and neurodevelopment for these individuals and their families. Co-produced by patients, parents, clinicians, and researchers, this national initiative has been strategically designed and built on a scalable learning health network platform to address key gaps in the field, support discovery, and accelerate improvements in outcomes.

Background There are conflicting reports regarding the importance of mitral stenosis and aortic atresia as a risk factor for Norwood mortality. This study reviews outcomes of this anatomic subgroup at our institution and examines the utility of preoperative cardiac catheterization and its correlation with clinical outcomes and pathology findings. Methods This is a single-center, retrospective review of hypoplastic left heart syndrome patients who underwent modified Norwood operation between October 2005 and May 2013. Results Fourteen of 74 hypoplastic left heart syndrome patients (19%) had mitral stenosis and aortic atresia. Operative mortality for MS/AA was 29% versus 7% for all other hypoplastic left heart syndrome anatomic subgroups ( p = 0.04). Although only 19% of the entire cohort, the mitral stenosis and aortic atresia subgroup constituted 50% of the total operative mortality and the only interstage deaths. Autopsies support myocardial ischemia as the mechanism of death. Although preoperative angiography defined the presence of ventriculo-coronary connections, it did not clearly risk stratify patients in regard to operative mortality. Conclusions Mitral stenosis and aortic atresia is a risk factor for perioperative myocardial ischemia and mortality. Further exploration of myocardial reserve is warranted.

Background Hypertrophic cardiomyopathy (HCM) in childhood is a rare diagnosis, and associations with adrenocortical tumors (ACTs) have been rarely reported in the pediatric literature. Case presentation We present a case of a 5-month-old who presented with HCM and during the evaluation for hypertension was found to have elevated glucocorticoids, mineralocorticoids, androgens and urine metanephrines. During preoperative evaluation, he developed shock followed by cardiogenic collapse requiring extracorporeal membrane oxygenation (ECMO); however, he did not survive. Pathology revealed an ACT with hormone production that contributed to his demise. Conclusions Adrenocortical tumors associated with hypertrophic cardiomyopathy can be life-threatening. We discuss the complex interplay of unrestricted cortical hormone production in the setting of hypertrophic cardiomyopathy that may lead to rapid decline and poor clinical outcomes.

More children with single ventricle heart disease are surviving after Fontan surgery. This circulation has pervasive effects on multiple organ systems and has unique modes of failure. Many centers have created multidisciplinary programs to care for these patients. Our aim was to survey such programs to better understand current approaches to care. We hypothesized that significant variability in surveillance testing strategy would be present. Eleven academic institutions with established Fontan care programs performing a combined estimated 300 Fontan surgeries per year, with a total population of 1500–2000 Fontan patients, were surveyed using a REDCap survey regarding surveillance testing and basic practice philosophies. Fontan care programs were structured both as consultative services (64%) and as the primary clinical team (9%). Electrocardiograms (73%) and echocardiograms (64%) were most commonly obtained annually. Serum studies, including complete blood count (73%), complete metabolic panel (73%), and Brain-type natriuretic peptide (54%), were most commonly obtained annually. Hepatic testing consisted of liver ultrasound in most centers, obtained biennially (45%) or > every 2 years (45%). Liver biopsy was not routinely recommended (54%). Neurodevelopmental outcomes were assessed at most institutions (54%), with a median frequency of every 3–4 years. There is considerable variability in the surveillance testing regimen and management strategy after a Fontan procedure at surveyed programs. There is an urgent need for surveillance guidelines to reduce variability, define quality metrics, streamline collaborative practice, and prospective research to better understand the complex adaptations of the body to Fontan physiology.

It is recognized that there are numerous anatomic variants that result in hypoplastic left heart physiology. One such variant includes critical aortic stenosis or atresia, a hypoplastic aortic arch, and a reasonably well-developed left ventricle due to the presence of a ventricular septal defect. These patients are candidates for 1 of 3 surgical options: (1) a Norwood procedure followed by a single-ventricle pathway; (2) a Norwood procedure followed by a Rastelli procedure (2-stage Yasui); or (3) a single-stage Yasui procedure. Because 2 of the 3 options include a Norwood procedure as the initial step, the purpose of this study was to evaluate the contemporaneous results of the Yasui and Norwood procedures at a single institution.This was a retrospective review of patients who underwent a Yasui or Norwood procedure at Lucile Packard Children's Hospital between 2004 and 2013. Eighteen patients underwent a Yasui, of whom 15 had a single-stage procedure and 3 had a 2-stage procedure. During this time frame, 113 patients underwent a Norwood procedure. Kaplan-Meier survival curves and freedom from reoperation were compared for the 2 procedures.The operative mortality (using the Society of Thoracic Surgeons definition) for the single-stage Yasui was 6.7% compared with 16% for the Norwood procedure (P.05); survival was 85% versus 62% at 1 year, 85% versus 60% at 3 years, and 85% versus 58% at 5 years, respectively (log-rank P = .06). The average interval to first reoperation was 13.5 ± 3 months versus 4.5 ± 1 months for the Yasui and Norwood procedures, respectively (P.001).The Yasui procedure had a significantly lower operative mortality compared with the Norwood procedure. Early and midterm survival was also higher in the Yasui group versus the Norwood followed by a single ventricle pathway. These results indicate that the Yasui procedure has significant midterm benefits compared with the Norwood procedure and should be pursued when the anatomy is amenable for this approach.

The specialty of pediatric cardiac critical care has undergone rapid scientific and clinical growth in the last 25 years. The Board of Directors of the Pediatric Cardiac Intensive Care Society assembled an updated list of sentinel references focused on the critical care of children with congenital and acquired heart disease. We encouraged board members to select articles that have influenced and informed their current practice or helped to establish the standard of care. The objective of this article is to provide clinicians with a compilation and brief summary of these updated 100 useful references.The list of 'One Hundred Useful References for Pediatric Cardiac Intensive Care' (2004) and relevant literature to the practice of cardiac intensive care.A subset of Pediatric Cardiac Intensive Care Society board members compiled the initial list of useful references in 2004, which served as the basis of the new updated list. Suggestions for relevant articles were submitted by the Pediatric Cardiac Intensive Care Society board members and selected pediatric cardiac intensivists with an interest in this project following the Society's meeting in 2010. Articles were considered for inclusion if they were named in the original list from 2004 or were suggested by Pediatric Cardiac Intensive Care Society board members and published before December 31, 2011.Following submission of the complete list by the Pediatric Cardiac Intensive Care Society board and contributing Society members, articles were complied by the two co-first authors (D.A., D.K.). The authors also performed Medline searches to ensure comprehensive inclusion of all relevant articles. The final list was then submitted to the Pediatric Cardiac Intensive Care Society board members, who ranked each publication.Rankings were compiled and the top 100 articles with the highest scores were selected for inclusion in this publication. The two co-first authors (D.A., D.K.) reviewed all existing summaries and developed summaries of the newly submitted articles.An updated compilation of 100 useful references for the critical care of children with congenital and acquired heart disease has been compiled and summarized here. Clinicians and trainees may wish to use this document as a reference for education in this complex and challenging subspecialty.

Background— The use of ventricular assist devices (VADs) to bridge pediatric patients to heart transplantation has increased dramatically over the last 15 years. In this report, we present the largest US single-center report of pediatric VAD use to date. We present detailed descriptions of morbidity and mortality associated with VAD support, using standard Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) criteria for pediatrics to facilitate the comparison of these results to other studies. Methods and Results— We retrospectively identified 25 patients younger than 18 years with 27 episodes of mechanical circulatory support using VADs as bridge to heart transplantation from January 1998 to December 2007. Survival to transplant for the entire cohort was 74%. The most common major morbidities, as defined by INTERMACS criteria for a pediatric population, were respiratory failure, major localized infections, major bleeding events, hepatic dysfunction, and right heart failure. Major neurological events occurred in 48% of the study population. The median time to the first occurrence of an adverse event was less than 14 days for respiratory failure, right heart failure, major localized infection, and major bleeding. Patients who died before transplantation had significantly more adverse events per day of support than did those who were successfully transplanted. Episodes of major bleeding, tamponade, acute renal failure, respiratory failure, and right heart failure were all associated with increased risk of mortality. Conclusions— INTERMACS criteria can be successfully used to analyze pediatric VAD outcomes. These data serve as a baseline for future studies of VAD support in children and indicate good survival rates but considerable morbidity.

Objective Palliation of hypoplastic left heart syndrome with a standard nonvalved right ventricle to pulmonary artery conduit results in an inefficient circulation in part due to diastolic regurgitation. A composite right ventricle pulmonary artery conduit with a homograft valve has a hypothetical advantage of reducing regurgitation, but may differ in the propensity for stenosis because of valve remodeling. Methods This retrospective cohort study included 130 patients with hypoplastic left heart syndrome who underwent a modified stage 1 procedure with a right ventricle to pulmonary artery conduit from 2002 to 2015. A composite valved conduit (cryopreserved homograft valve anastomosed to a polytetrafluoroethylene tube) was placed in 100 patients (47 aortic, 32 pulmonary, 13 femoral/saphenous vein, 8 unknown), and a nonvalved conduit was used in 30 patients. Echocardiographic functional parameters were evaluated before and after stage 1 palliation and before the bidirectional Glenn procedure, and interstage interventions were assessed. Results On competing risk analysis, survival over time was better in the valved conduit group ( P = .040), but this difference was no longer significant after adjustment for surgical era. There was no significant difference between groups in the cumulative incidence of bidirectional Glenn completion ( P = .15). Patients with a valved conduit underwent more interventions for conduit obstruction in the interstage period, but this difference did not reach significance ( P = .16). There were no differences between groups in echocardiographic parameters of right ventricle function at baseline or pre-Glenn. Conclusions In this cohort of patients with hypoplastic left heart syndrome, inclusion of a valved right ventricle to pulmonary artery conduit was not associated with any difference in survival on adjusted analysis and did not confer an identifiable benefit on right ventricle function.

Background The surgical repair of aortic coarctation in infants has evolved over time. This study evaluates our current approach utilizing extended end-to-end anastomosis without prosthetic material to enlarge all areas of aortic arch hypoplasia. Methods The Michigan Congenital Heart Center database was reviewed for infants who underwent repair of isolated aortic coarctation from January 1, 1990, to January 1, 2000. Results Eighty-three infants underwent surgical repair of isolated coarctation during this decade. Median age at repair was 21 days (range, 2 to 365). Repair was performed through thoracotomy in 72 patients. Because of severe transverse arch hypoplasia, the remaining 11 infants underwent median sternotomy with circulatory arrest. There were 2 deaths: 1 due to pulmonary hypertension in a patient with alveolar capillary dysplasia and 1 late death due to pneumonia in a patient with noncardiac anomalies. Neither patient had residual coarctation. Technique-related complications of bronchial compression, chylothorax, and vocal cord paralysis were noted in 4 patients. Follow-up data were available for 66 patients (80%) with mean follow-up duration of 4.5 years (SD ± 3.1). Reintervention was required in 4 patients (6%). One underwent reoperation after 1 month, and 3 underwent balloon angioplasty within 7 months of initial repair. The remaining 61 patients are asymptomatic, on no antihypertensive medications, and have aortic arch gradients less than 15 mm Hg. One developed subaortic stenosis necessitating resection. Conclusions Tailored surgical repair for aortic coarctation has a low rate (6%) of residual and recurrent coarctation even when performed in infants. Mortality and morbidity are low.

complications of surgical and Amplatzer device closure of atrial septal defects. J Thorac Cardiovasc Surg 1999;118:674–680. 3. Sievert H. Interventional versus surgical closure of atrial and ventricular septal defects: advantages and limitations of the catheter-based approach. J Intervent Cardiol 2000;87:116–118. 4. Cao QL, Du ZD, Joseph A, Koenig P, Heitschmidt M, Rhodes J, Hijazi ZM. Immediate and six-month results of the profile of the Amplatzer septal occluder as assessed by transesophageal echocariography. Am J Cardiol 2001;88:754–759. 5. Masura J, Gavora P, Formanek A, Hijazi ZM. Transcatheter closure of secundum atrial septal defects using the new self-centering Amplatzer septal occluder: initial human experience. Cathet Cardiovasc Diagn 1997;42:388–393. 6. Rhodes J, Hijazi ZM, Marx GR, Fulton DR. Aerobic exercise function of patients with persistent coronary artery aneurysms secondary to Kawasaki disease. Pediatr Cardiol 1996;17:226–230. 7. Beaver WL, Wasserman K, Whipp BJ. A new method for detecting anaerobic threshold by gas exchange. J Appl Physiol 1986;60:2020–2027. 8. Jones NL. Clinical Exercise Testing. Philadelphia, PA: WB Saunders, 1988: 174. 9. Clark AL, Poole-Wilson PA, Coats AJS. Effects of motivation on indices of exercise capacity in chronic heart failure. Br Heart J 1994;71:162–165. 10. Wasserman K, Hansen JE, Sue DY, Whipp BJ, Casaburin R. Prinicples of Exercise Testing and Interpretation. 2nd Edition. Philadelphia, PA: Lea & Febiger, 1994:66–133. 11. Cumming GR. Maximal exercise capacity of children with heart defects. Am J Cardiol 1978;42:613–620. 12. Goldberg SJ, Mendes F, Hurwitz R. Maximal exercise capability of children as a function of specific cardiac defects. Am J Cardiol 1969;23:349–353. 13. Schofield PM, Barber PV, Kingston T. Preoperative and postoperative pulmonary function tests in patients with atrial septal defect. Br Heart J 1985;54: 577–582. 14. Porter CJ, Feldt RH, Edwards WD, Seward JB, Schaff HV. Atrial septal defects. In: Emmanouilides GC, Riemenschneider TA, Allen HD, Gutgessell HP, eds. Heart Disease in Infants, Children and Adolescents. 5th Edition. Baltimore, MD: Williams and Wilkins, 1995:687–703. 15. Wasserman K. New concepts in assessing cardiovascular function. Circulation 1988;78:1060–1071.

Objective. To examine the effects of stress management training on pain behavior exhibited by persons with rheumatoid arthritis (RA) and the relationship of change in pain behavior with certain patient characteristics as well as change in self-reported levels of pain. Methods. Patients with RA (n 5 131) were randomly assigned to 1 of 3 groups: a stress management group, an attention control group, or a standard care control group. The stress management and attention control groups received a 10-week intervention followed by a 15-month maintenance phase. Results. The 3 groups did not differ significantly in the change in pain behavior at any of the assessment periods. However, persons with RA who had less disease activity tended to exhibit positive changes in pain behavior over time. Changes in self-reported pain were not significantly related to changes in pain behavior. Conclusion. The results indicate that stress management interventions do not reduce total pain behaviors exhibited by persons with RA. Changes in pain behaviors appear to be related to disease activity, age, and disease duration, but not to changes in self-reported measures of pain.

Objective To test whether change in cognitive–behavioral variables (such as self-efficacy, coping strategies, and helplessness) is a mediator in the relation between cognitive behavior therapy and reduced pain and depression in persons with rheumatoid arthritis (RA). Methods A sample of patients with RA who completed a stress management training program (n = 47) was compared to a standard care control group (n = 45). A path analysis testing a model including direct effects of comprehensive stress management training on pain and depression and indirect effects via change in cognitive–behavioral variables was conducted. Results The path coefficients for the indirect effects of stress management training on pain and depression via change in cognitive–behavioral variables were statistically significant, whereas the path coefficients for the direct effects were found not to be statistically significant. Conclusion Decreases in pain and depression following stress management training are due to beneficial changes in the arenas of self-efficacy (the belief that one can perform a specific behavior or task in the future), coping strategies (an individual's confidence in his or her ability to manage pain), and helplessness (perceptions of control regarding arthritis). There is little evidence of additional direct effects of stress management training on pain and depression.

Objective To examine the factor structure of the Center for Epidemiologic Studies Depression Scale (CES-D) in a sample of patients with rheumatoid arthritis (RA), testing all of the alternative models suggested by the previous evidence Methods The CES-D was administered to a group of RA patients (n = 685) during a structured telephone interview. The telephone interview was repeated 6 months later (n = 537) and 12 months later (n = 453). Confirmatory factor analyses were conducted to test alternative models. Results The correlated 4-factor model and the second-order 4-factor model were the best fitting models. Conclusion The factor structure of the CES-D previously found in the general population was replicated in an RA sample. The results are consistent with previous evidence of criterion contamination in the CES-D when used in an RA sample and provide support for the view that a single summary score may not be the most informative index of the CES-D.

The Veterans Health Administration under the direction of Kenneth Kizer, M.D., has faced the challenge of providing high-quality health care while controlling costs. Under Dr. Kizer's direction, VA hospitals are moving toward managed patient centered primary care. Hospital organization, funding, and clinical practices have been changed. Reorganization has meant a variety of possibilities for psychologists employed in the VA system.

Objective To examine the relationship between age and depression in persons with rheumatoid arthritis (RA). Methods Two separate outpatient cohorts of persons with RA were studied. In both studies, the Center for Epidemiological Studies Depression Scale was administered to all subjects, and the prevalence of depressive symptoms was determined by age group. In the second study, data on additional measures of disease activity, pain, life stress, and coping were collected for use in multiple linear regression analyses. Results In both samples, a significant correlation between age and depression was found; younger persons (age ≤45 years) with RA were significantly more depressed, even after controlling for potentially confounding variables such as sex, marital status, antidepressant medication, arthritis medication, functional class, and disease duration. Conclusion The findings show that younger persons with RA are at higher risk for depressive symptoms than their older counterparts.

Objective. To examine relationships among changes in self-efficacy and changes in other clinically relevant outcome measures. Method. Subjects (n = 44) were participants in a prospective, randomized stress-management study followed over 15 months. Outcome measures included self-efficacy, depression, pain, health status, and disease activity. Results. Correlational analyses revealed significant associations between changes in self-efficacy (particularly total self-efficacy) and changes in selected measures of depression, pain, health status, and disease activity. The observed associations were not due to changes in medication regimen or to nonadherence to the stress-management program. Conclusions. Evidence is provided that induced changes in self-efficacy following a stress-management program were significantly related to other clinically important outcome measures.

Objective. To test and cross-validate a model using disease activity, pain, and helplessness to predict future psychological and physical disability in persons with rheumatoid arthritis (RA) across time. Methods. Measures of disease activity, pain, helplessness, psychological function, and physical function were collected from 63 males with RA at baseline, 3 months, and 6 months. Path analytic methods were used to examine longitudinal relationships among these variables. Results. Path analysis revealed that pain and helplessness were significant mediators of the relationship between disease activity and future disability in RA; the predictive model withstood two cross-validations. Conclusion. The findings suggest that pain and helplessness are key biopsychosocial variables that affect the development of disability in RA.

Objective. To identify risk factors for the development of depression in persons with rheumatoid arthritis (RA). Methods. Subjects were divided into depressed versus nondepressed groups on the basis of the Center for Epidemiologic Studies-Depression Scale; a range of psychological, pain-related, disease-related, and demographic variables were analyzed to predict depression. Both cross-sectional and longitudinal predictive models were examined. Results. A series of analyses, including multiple logistic regression, found that the optimal predictors of depression in RA were average daily stressors, confidence in one's ability to cope, and degree of physical disability. The model was successfully cross-validated on separate data sets (i.e., same subjects at different time points). Conclusion. All of the identified risk factors for depression in RA are preventable to some extent and, therefore, should be addressed in comprehensive, rheumatology team care.

Thirty-one employers viewed tapes of job interviews of six upper-level college students with physical disabilities. Three interviewees were judged by graduate student raters to have good nonverbal skills and the other three to have poor nonverbal skills. Transcripts of the interviews were read and rated by graduate students in psychology, and the results were used to adjust statistically for the content of the interviews. Persons with disabilities who exhibited good nonverbal communication were rated as significantly more employable and rated higher on personal qualities considered in employment interviews than persons who exhibited poor nonverbal communication. Implications for further research and designing interventions for improving nonverbal communicati on of the physically disabled are discussed.

The authors describe a teaching activity using a poster-session format to teach first-year counseling psychology doctoral students the history of counseling. Evaluative data suggest that the format was an effective and powerful tool to teach beginning counseling psychology students the history of the profession. The implications of the evaluative feedback are discussed in terms of teaching and preparing the next generation of counselors.

This study empirically examined underlying dimensions from actual supervisory statements taken from interventions occurring during live supervision of counseling interviews. Multidimensional scaling revealed 6 dimensions: (a) directing-instructing vs. deepening, (b) cognitive clarification vs. emotional encouragement, (c) confronting vs. encouraging the client, (d) didactic-distant vs. emotionally involved, (e) joining with vs. challenging the trainee, (f) providing direction vs. resignation.

Background Young children supported on a ventricular assist device (VAD) can have prolonged hospitalizations awaiting heart transplantation. The adult VAD literature demonstrates that comprehensive programs optimize transplantation outcomes. The goal of this intervention was to create an interdisciplinary program to optimize care coordination and delivery in young children requiring a VAD. Method This study was a case review. Results We have supported 8 infants and young children with Berlin Heart VAD. These children's hospitalizations have been more complex than those of our older VAD patients, and they have required intensive care for prolonged periods. An interdisciplinary group evaluated our practices and identified areas for potential improvement. The focus group from our intensive care unit introduced multiple interventions to optimize interdisciplinary care coordination and consistency of practice. These included (1) interdisciplinary care guidelines for chronically, critically ill patients; (2) institution of a primary cardiovascular intensive care unit (CVICU) physician and nurse practitioner system; (3) introduction of a psychological intervention for families to minimize the impact of their extended CVICU stay; (4) implementation of early jejunal feeds; (5) a focused developmental and rehabilitation protocol, 6) implementation of a structured approach to minimizing blood transfusions; (7) increased structure for our interdisciplinary rounds to optimize team communication; (8) comprehensive nursing education including wound care, anticoagulation management, and laboratory draws; and (9) a protocol for off-unit rehabilitative/developmental interventions. These changes in both practice and approach for young VAD patients have also been of benefit to other chronically, critically ill children in the CVICU, as well as to our CVICU team. Conclusion Comprehensive interdisciplinary care coordination and standardized practice can be achieved in the critical care setting for complex pediatric heart failure patients who require long-term VAD support.

Background Mechanical circulatory support with the Berlin Heart EXCOR pediatric ventricular assist device (VAD) has been used successfully in Europe for children with cardiac failure. Eighty-seven devices have been placed in North America through February 2007. We describe our single-center experience in 8 children. Methods Eight children (ages 4 to 55 months), with median weight of 9.6 kg and body surface area of 0.48 m 2 , received the Berlin Heart VAD as a bridge to transplantation. All patients were in cardiogenic shock requiring multiple inotropes. Primary diagnoses were idiopathic dilated cardiomyopathy ( n = 4), congenital heart disease ( n = 3) and restrictive cardiomyopathy ( n = 1). After device insertion, all patients were treated with an anti-coagulant (heparin or coumadin) and one or more platelet inhibitors (aspirin with clopidogrel or dipyridamole). Results Five patients received support with a left ventricular assist device (LVAD) and 3 with a biventricular device (BiVAD). Duration of support ranged from 2 to 234 days (median 57 days). Five patients (63%) were successfully bridged to transplantation; of these, 4 were discharged home and 1 died from early graft failure. Five patients developed post-operative neurologic events. Of these 5 events, 4 could be explained by embolism or hemorrhage. Device exchange was performed in 4 patients in the intensive care unit. Conclusions In selected children, the Berlin Heart VAD can be used as a bridge to transplantation. In contrast to the published European experience, neurologic events occur frequently. Anti-coagulation and platelet inhibition strategies continue to evolve. Device exchange is technically feasible at the bedside and should be considered at the earliest visualization of thrombus formation.

Background— The use of a right ventricle to pulmonary artery (RV-PA) conduit in the Norwood procedure has been proposed to increase postoperative hemodynamic stability. A valve within the conduit should further decrease RV volume load. We report our clinical experience with this modification. Methods and Results— From February 2002 through August 2005, we performed 88 consecutive Norwood procedures using RV-PA conduits. We used composite valved conduits made from cryopreserved homograft and polytetrafluoroethylene (PTFE) in 66 cases (54 pulmonary, 12 aortic homografts), other valved conduits in 14, and unvalved PTFE in 8 cases. Hospital survival was 88.6% overall and increased to 93.1% after the initial year. Early interventions were required in 18 patients (16 for cyanosis). Prestage II cardiac catheterization was performed at a mean age of 126 days. Mean Qp/Qs was 1, with mean aortic saturation 71%, mean O 2 extraction 24%, and mean right ventricular end-diastolic pressure 9 mm Hg. Patient weight, use of an aortic homograft valve in the conduit, stage I palliation within the first year of our experience, and low O 2 extraction and high transpulmonary gradient prestage II were risk factors for overall death. Early interventions were more frequent in aortic valve conduits compared with all other conduits. Conclusions— The valved RV-PA conduit was associated with low early mortality after the Norwood procedure. The majority of these patients had normal cardiac output and well-maintained RV function. There may be a higher risk for early conduit interventions and death when aortic valve homografts are used in the RV-PA conduit.

Background Sudden death, remote from surgery, in patients with hypoplastic left heart syndrome (HLHS) after Norwood palliation is an important problem. The episodic nature of this syndrome has made its cause(s) difficult to ascertain. Observations made in hospitalized Norwood patients may afford insight into the pathophysiology of sudden death among these patients. Methods We conducted a retrospective chart review. Results Five patients with HLHS experiencing unremarkable recoveries from Norwood palliation, still hospitalized but extubated (only 1 in intensive care), had unexpected, acute decompensation 8 to 15 days postoperatively. All had acutely decreased peripheral perfusion; severe metabolic acidosis (mean HCO 3 = 9 mEq/L, range 6 to 11 mEq/L; mean arterial lactate=16 mmol/L, range 10 to 20 mmol/L, normal less than 2 mmol/L); relatively high arterial pO 2 , especially considering their low systemic perfusion (mean = 57 mm Hg, range 50 to 66 mm Hg on fraction of inspired oxygen (FiO 2 ) less than 0.3 in 4 of 5 patients); and relatively high systolic blood pressure (mean systolic blood pressure=91 mm Hg, range 78 to 116 mm Hg). During the preceding 24 hours, all had had systolic blood pressures of more than 85 mm Hg at multiple times. All were resuscitated with mechanical ventilation and administration of HCO 3 and intravenous inotropic agents or vasodilators (1 also required extracorporeal membrane oxygenation), with rapid resolution of their acidosis. After decompensating, all were treated with oral antihypertensive agents; 1 had an early hemi-Fontan. All survived to discharge. Conclusions Increased systemic vascular resistance may be especially pernicious in Norwood patients—even remote from operation—as the condition increases myocardial work and O 2 consumption while diminishing systemic perfusion. Chronic and acutely increased systemic vascular resistance may account for some cases of sudden unexpected death in Norwood patients.

Depression in rheumatoid arthritis (RA) affects many, but by no means all, persons with the disease. There is evidence that depression bears a significant relationship to pain in RA, although the causal direction is not entirely clear. Likewise, there is strong evidence that depression is a major contributor to the RA disability. Therefore, prevention, early diagnosis, and aggressive treatment of depression are needed to minimize the disability associated with RA.

Objective. To examine the effects of stress-management training on clinical outcomes in persons with rheumatoid arthritis (RA). Methods. Patients with RA (n = 141) were randomly assigned to 1 of 3 groups: a stress management group, an attention control group, or a standard care control group. The stress management and the attention control groups received a 10-w eek intervention followed by an additional 15-m onth maintenance phase. Results. The stress management group showed statistically significant improvements on measures of helplessness, self-efficacy, coping, pain, and health status. Selected beneficial effects were still detectable at the 15-m onth followup evaluation. Conclusion. The data indicated that stress management interventions are capable of producing important clinical benefits for persons with RA.

Background From 2002 to 2005, the interstage mortality after a modified Norwood procedure was 7% in our program. An interstage home monitoring program (HMP) was established to identify Norwood procedure patients at increased risk of decompensation and to reduce interstage mortality. Methods Results of the first 5 years of the Norwood HMP were reviewed retrospectively. Interstage was defined as the time between Norwood hospital discharge and admission for second stage surgical palliation. In the HMP, families documented oxygen saturation, heart rate, weight, and feedings daily. Nurse practitioners called each family at least weekly, and when issues arose, action plans were determined based on symptom severity. Results Between October 2005 and October 2010 there were 46 Norwood procedure patients who survived to hospital discharge. All were enrolled in the HMP. Forty-five patients had a Norwood procedure with right ventricle to pulmonary artery conduit, and 1 patient had a modified Blalock-Taussig shunt. Interstage survival was 100%. Nineteen patients (41%) were admitted interstage; 5 patients were admitted twice, 1 patient was admitted 4 times. Seventeen patients (37%) required interstage interventions. Eight patients (17%) required major interventions: conduit stenting, aortic arch balloon angioplasty, emergent shunt, or early Glenn surgery. Minor interventions included supplemental oxygen, blood transfusion, intravenous hydration, diuresis, anti-arrhythmic therapy, or feeding adjustments. Conclusions In the first 5 years of the HMP, all infants discharged after a modified Norwood procedure survived the interstage period. The HMP altered clinical management in 37% of patients. Home monitoring of oxygen saturation, heart rate, weight, and feedings, along with comprehensive care coordination, allowed timely interventions and reduced interstage mortality from 7% to 0%.