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

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

Introduction Adolescents with single ventricle congenital heart disease (SVHD) show functional deficits, particularly in memory and mood regulation. Hippocampi are key brain structures that regulate mood and memory; however, their tissue integrity in SVHD is unclear. Our study aim is to evaluate hippocampal volumes and their associations with memory, anxiety, and mood scores in adolescents with SVHD compared to healthy controls. Methods We collected brain magnetic resonance imaging data from 25 SVHD (age 15.9 ± 1.2 years; 15 male) and 38 controls (16.0 ± 1.1 years; 19 male) and assessed memory (Wide Range Assessment of Memory and Learning 2, WRAML2), anxiety (Beck Anxiety Inventory, BAI), and mood (Patient Health Questionnaire 9, PHQ‐9) functions. Both left and right hippocampi were outlined and global volumes, as well as three‐dimensional surfaces were compared between groups using ANCOVA and associations with cognitive and behavioral scores with partial correlations (covariates: age and total brain volume). Results The SVHD group showed significantly higher BAI (p = .001) and PHQ‐9 (p, Right hippocampal volume was significantly reduced in adolescents with SVHD compared to controls, localized in several sites (CA1, CA4, subiculum, and dentate gyrus), which are associated with memory deficits and trended toward significance in anxiety and mood.

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: In the SVR trial (Single Ventricle Reconstruction), 1-year transplant-free survival was better for the Norwood procedure with right ventricle-to-pulmonary artery shunt (RVPAS) compared with a modified Blalock–Taussig shunt in patients with hypoplastic left heart and related syndromes. At 6 years, we compared transplant-free survival and other outcomes between the groups. Methods: Medical history was collected annually using medical record review, telephone interviews, and the death index. The cohort included 549 patients randomized and treated in the SVR trial. Results: Transplant-free survival for the RVPAS versus modified Blalock–Taussig shunt groups did not differ at 6 years (64% versus 59%, P =0.25) or with all available follow-up of 7.1±1.6 years (log-rank P =0.13). The RVPAS versus modified Blalock–Taussig shunt treatment effect had nonproportional hazards ( P =0.009); the hazard ratio (HR) for death or transplant favored the RVPAS before stage II surgery (HR, 0.66; 95% confidence interval, 0.48–0.92). The effect of shunt type on death or transplant was not statistically significant between stage II to Fontan surgery (HR, 1.36; 95% confidence interval, 0.86–2.17; P =0.17) or after the Fontan procedure (HR, 0.76; 95% confidence interval, 0.33–1.74; P =0.52). By 6 years, patients with RVPAS had a higher incidence of catheter interventions (0.38 versus 0.23/patient-year, P Conclusions: By 6 years, the hazards of death or transplant and catheter interventions were not different between the RVPAS versus modified Blalock–Taussig shunt groups. Children assigned to the RVPAS group had 5% higher transplant-free survival, but the difference did not reach statistical significance, and they required more catheter interventions. Both treatment groups have accrued important complications. Clinical Trial Registration: URL: https://www.clinicaltrials.gov . Unique identifier: NCT00115934.

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

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

Background Children with single–right ventricle anomalies such as hypoplastic left heart syndrome (HLHS) have left ventricles of variable size and function. The impact of the left ventricle on the performance of the right ventricle and on survival remains unclear. The aim of this study was to identify whether left ventricular (LV) size and function influence right ventricular (RV) function and clinical outcome after staged palliation for single–right ventricle anomalies. Methods In the Single Ventricle Reconstruction trial, echocardiography-derived measures of LV size and function were compared with measures of RV systolic and diastolic function, tricuspid regurgitation, and outcomes (death and/or heart transplantation) at baseline (preoperatively), early after Norwood palliation, before stage 2 palliation, and at 14 months of age. Results Of the 522 subjects who met the study inclusion criteria, 381 (73%) had measurable left ventricles. The HLHS subtype of aortic atresia/mitral atresia was significantly less likely to have a measurable left ventricle (41%) compared with the other HLHS subtypes: aortic stenosis/mitral stenosis (100%), aortic atresia/mitral stenosis (96%), and those without HLHS (83%). RV end-diastolic and end-systolic volumes were significantly larger, while diastolic indices suggested better diastolic properties in those subjects with no left ventricles compared with those with measurable left ventricles. However, RV ejection fraction was not different on the basis of LV size and function after staged palliation. Moreover, there was no difference in transplantation-free survival to Norwood discharge, through the interstage period, or at 14 months of age between those subjects who had measurable left ventricles compared with those who did not. Conclusions LV size varies by anatomic subtype in infants with single–right ventricle anomalies. Although indices of RV size and diastolic function were influenced by the presence of a left ventricle, there was no difference in RV systolic function or transplantation-free survival on the basis of LV measures.

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

Objective Apolipoprotein E ( APOE ) genotype is a determinant of neurologic recovery after brain ischemia and traumatic brain injury. The APOE e2 allele has been associated with worse neurodevelopmental (ND) outcome after repair of congenital heart defects (CHD) in infancy. Replication of this finding in an independent cohort is essential to validate the observed genotype-phenotype association. Methods The association of APOE genotype with ND outcomes was assessed in a combined cohort of patients with single-ventricle CHD enrolled in the Single Ventricle Reconstruction and Infant Single Ventricle trials. ND outcome was assessed at 14 months using the Psychomotor Development Index (PDI) and Mental Development Index (MDI) of the Bayley Scales of Infant Development-II. Stepwise multivariable regression was performed to develop predictive models for PDI and MDI scores. Results Complete data were available for 298 of 435 patients. After adjustment for preoperative and postoperative covariates, the APOE e2 allele was associated with a lower PDI score ( P = .038). Patients with the e2 allele had a PDI score approximately 6 points lower than those without the risk allele, explaining 1.04% of overall PDI variance, because the e2 allele was present in only 11% of the patients. There was a marginal effect of the e2 allele on MDI scores ( P = .058). Conclusions These data validate the association of the APOE e2 allele with adverse early ND outcomes after cardiac surgery in infants, independent of patient and operative factors. Genetic variants that decrease neuroresilience and impair neuronal repair after brain injury are important risk factors for ND dysfunction after surgery for CHD.

Background— In the Single Ventricle Reconstruction (SVR) trial, 1-year transplantation-free survival was better for the Norwood procedure with right ventricle–to–pulmonary artery shunt (RVPAS) compared with a modified Blalock-Taussig shunt (MBTS). At 3 years, we compared transplantation-free survival, echocardiographic right ventricular ejection fraction, and unplanned interventions in the treatment groups. Methods and Results— Vital status and medical history were ascertained from annual medical records, death indexes, and phone interviews. The cohort included 549 patients randomized and treated in the SVR trial. Transplantation-free survival for the RVPAS versus MBTS groups did not differ at 3 years (67% versus 61%; P =0.15) or with all available follow-up of 4.8±1.1 years (log-rank P =0.14). Pre-Fontan right ventricular ejection fraction was lower in the RVPAS group than in the MBTS group (41.7 ± 5.1% versus 44.7 ± 6.0%; P =0.007), and right ventricular ejection fraction deteriorated in RVPAS ( P =0.004) but not MBTS ( P =0.40) subjects (pre-Fontan minus 14-month mean, −3.25±8.24% versus 0.99±8.80%; P =0.009). The RVPAS versus MBTS treatment effect had nonproportional hazards ( P =0.004); the hazard ratio favored the RVPAS before 5 months (hazard ratio=0.63; 95% confidence interval, 0.45–0.88) but the MBTS beyond 1 year (hazard ratio=2.22; 95% confidence interval, 1.07–4.62). By 3 years, RVPAS subjects had a higher incidence of catheter interventions ( P P =0.005): 1 year, 2.48 (95% confidence interval, 1.28–4.80). Conclusions— By 3 years, the Norwood procedure with RVPAS compared with MBTS was no longer associated with superior transplantation-free survival. Moreover, RVPAS subjects had slightly worse right ventricular ejection fraction and underwent more catheter interventions with increasing hazard ratio over time. Clinical Trial Registration— URL: http://www.clinicaltrials.gov . Unique identifier: NCT00115934.

Aortic arch reconstruction in children with single ventricle lesions may predispose to circulatory inefficiency and maladaptive physiology leading to increased myocardial workload. We sought to describe neoaortic anatomy and physiology, risk factors for abnormalities, and impact on right ventricular function in patients with single right ventricle lesions after arch reconstruction.Prestage II aortic angiograms from the Pediatric Heart Network Single Ventricle Reconstruction trial were analyzed to define arch geometry (Romanesque [normal], crenel [elongated], or gothic [angular]), indexed neoaortic dimensions, and distensibility. Comparisons were made with 50 single-ventricle controls without prior arch reconstruction. Factors associated with ascending neoaortic dilation, reduced distensibility, and decreased ventricular function on the 14-month echocardiogram were evaluated using univariate and multivariable logistic regression.Interpretable angiograms were available for 326 of 389 subjects (84%). Compared with controls, study subjects more often demonstrated abnormal arch geometry (67% vs 22%, P .01) and had increased ascending neoaortic dilation (Z score 3.8 ± 2.2 vs 2.6 ± 2.0, P .01) and reduced distensibility index (2.2 ± 1.9 vs 8.0 ± 3.8, P .01). Adjusted odds of neoaortic dilation were increased in subjects with gothic arch geometry (odds ratio [OR], 3.2 vs crenel geometry, P .01) and a right ventricle-pulmonary artery shunt (OR, 3.4 vs Blalock-Taussig shunt, P .01) but were decreased in subjects with aortic atresia (OR, 0.7 vs stenosis, P .01) and those with recoarctation (OR, 0.3 vs no recoarctation, P = .04). No demographic, anatomic, or surgical factors predicted reduced distensibility. Neither dilation nor distensibility predicted reduced right ventricular function.After Norwood surgery, the reconstructed neoaorta demonstrates abnormal anatomy and physiology. Further study is needed to evaluate the longer-term impact of these features.

ObjectiveFor infants with single ventricle malformations undergoing staged repair, interstage mortality is reported at 2% to 20%. The Single Ventricle Reconstruction trial randomized subjects with a single morphologic right ventricle undergoing a Norwood procedure to a modified Blalock–Taussig shunt (MBTS) or a right ventricle-to-pulmonary artery shunt (RVPAS). The aim of this analysis was to explore the associations of interstage mortality and shunt type, and demographic, anatomic, and perioperative factors.MethodsParticipants in the Single Ventricle Reconstruction trial who survived to discharge after the Norwood procedure were included (n = 426). Interstage mortality was defined as death postdischarge after the Norwood procedure and before the stage II procedure. Univariate analysis and multivariable logistic regression were performed adjusting for site.ResultsOverall interstage mortality was 50 of 426 (12%)—13 of 225 (6%) for RVPAS and 37 of 201 (18%) for MBTS (odds ratio [OR] for MBTS, 3.4; P

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”.

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

Introduction Adolescents and young adults with congenital heart disease (CHD) show a range of memory deficits, which can dramatically impact their clinical outcomes and quality of life. However, few studies have identified predictors of these memory changes. The purpose of this investigation was to identify predictors of memory deficits in adolescents and young adults with CHD after surgical palliation compared to healthy controls. Method One hundred fifty-six adolescents and young adults (80 CHD and 76 controls; age 14–21 years) were recruited and administered an instrument to assess memory [Wide Range Assessment of Memory and Learning Second Edition – general memory index (GMI) score] and completed questionnaires that measure anxiety, depression, sleepiness, health status, and self-efficacy. Descriptive and non-parametric statistics were used to assess group differences, and logistic regression to identify predictors of memory deficits. Results CHD subjects consisted of 58% males, median age 17 years, 43% Hispanic, and medians of 2 previous heart surgeries and 14 years since last surgery. Memory deficits (GMI ≤ 85) were identified in 50% CHD compared to 4% healthy controls (median GMI 85 vs. 104, p

Sleep disorders, autonomic dysfunction, and abnormal cognition are important comorbidities in adult patients with heart failure and are associated with disease progression, morbidity, and mortality. The clinical incidence of these conditions is unknown in children with heart failure. We sought to determine the incidence of symptoms that may be attributable to autonomic dysfunction among children with dilated cardiomyopathy and heart failure. We performed a retrospective chart review of patients with dilated cardiomyopathy seen at our institution between 1999 and 2005. We reviewed charts for symptoms of dysautonomia, sleep problems, or abnormal cognition. From the records of 204 pediatric patients, we identified 69 patients aged 7-18 years with severe dilated cardiomyopathy. Of these, 55 (80%) had symptoms attributable to dysautonomia, 20 (29%) had evidence of sleep disturbance, and 3 (4%) had abnormal cognition. Dysautonomia and sleep disturbances are prevalent in children with heart failure, congruent with studies of adult patients. Based on our data, it is not possible to draw conclusions about any cognitive deficits in this population. Because relatively few subjects' charts explored symptoms of sleep disturbance, we speculate that sleep symptoms may be underappreciated.

Summary Background: The routine use of ketorolac after congenital heart surgery in infants and children is limited by concerns for postoperative bleeding complications. The object of this study was to determine if the use of ketorolac is associated with an increased risk of significant postoperative bleeding after congenital heart surgery in infants and children. Methods: A retrospective chart review was performed. The exposure of interest was postoperative use of ketorolac after congenital heart surgery in infants and children. The outcome measured was postoperative bleeding requiring surgical exploration. The patients who received ketorolac were compared with an age- and diagnosis-matched comparison group who did not receive ketorolac. Results: Records of 842 infants and children who underwent congenital heart surgery between July 2001 and October 2002 were reviewed. 94 (11.1%) patients were treated with ketorolac postoperatively. The comparison group consisted of 94 matched subjects selected from the patients that did not receive ketorolac. The mean age of patient in the ketorolac group was 8.5 (±6.1) years. No (0%) patients in the ketorolac group and four (4.2%) patients in the nonketorolac group developed postoperative bleeding requiring surgical exploration. The relative risk for postoperative bleeding that required surgical exploration in the ketorolac group compared with the nonketorolac group was 0.2 (95% CI 0.02–1.67). Conclusions: The use of ketorolac after congenital heart surgery in infants and children does not significantly increase the risk of bleeding complications requiring surgical exploration.

Objective: Ketorolac is a potent nonsteroidal analgesic agent used to treat postoperative pain. It produces excellent analgesia without the sedating side effects of opioid analgesics. Routine use of ketorolac after cardiac surgery is limited by concerns of bleeding complications. The purpose of this study was to evaluate the risk of bleeding complications of ketorolac for treatment of pain after congenital heart surgery in infants and children. Design: Prospective randomized, controlled trial. Setting: Pediatric cardiac intensive care unit in tertiary teaching hospital. Participants: Seventy infants and children, median age 10 months (range 2.5–174), who underwent congenital heart surgery requiring cardiopulmonary bypass were randomized in the trial. Intervention: Pain control was performed with ketorolac and opioid analgesics in one arm of the study and opioid analgesics alone in the other arm. Outcome Measures: The main outcome evaluated was bleeding complications measured by chest-tube drainage and wound and gastrointestinal bleeding. Results: Thirty-five patients were randomized to each treatment arm. In the ketorolac group, the median chest-tube drainage was 13.3 (range 4–22) mL/kg/d, no patient had significant wound bleeding, and 1 (0.03%) patient had gastrointestinal bleeding. In the control group, the median chest-tube drainage was 16.5 (range 3–24) mL/kg/d, 1 (0.03%) patient had wound bleeding, and no patient had gastrointestinal bleeding. Conclusion: Ketorolac can be used to treat pain after congenital heart surgery without an increased risk of bleeding complications.

Objectives: Aortic arch reconstruction in patients with single ventricle lesions may predispose to circulatory inefficiency and maladaptive physiology leading to increased myocardial workload. We sought to describe potentially maladaptive aortic anatomy and physiology, risk factors, and impact on right ventricle (RV) function in patients with single RV lesions after Norwood. Methods: Pre-stage II angiograms for subjects from the Single Ventricle Reconstruction (SVR) Trial were compared to 50 single left ventricle controls with no prior arch reconstruction. Arch geometry was defined as normal, crenel (elongated), or gothic (angular). Aortic index (ascending /descending aorta diameter) and distensibility index (systolic aortic area - diastolic area) / (diastolic area x pulse pressure) were calculated. Univariable tests were used for comparisons, and Spearman’s rho was used for correlation. Results: Interpretable angiograms were available for 326/389 (84%) SVR subjects. Table 1 summarizes findings. Age at catheterization was similar for SVR subjects and controls. SVR subjects more often demonstrated abnormally elongated (crenel) arch geometry with dilated ascending and transverse aortae tapering to a small isthmus. Distensibility was reduced in both the ascending and descending aorta. Risk factors for ascending aortic dilation (aortic index ≥ 2.5) included native aortic stenosis (OR = 2.2 [95% CI: 1.2, 3.8] vs. aortic atresia) and receipt of a RV-pulmonary artery shunt (OR = 2.6 [1.47, 4.5] vs. Blalock-Taussig shunt). There was no association between reduced distensibility and any demographic, anatomic, or reported surgical factors. Aortic dilation and reduced distensibility did not correlate with RV function at 14-month echocardiogram (p=0.5). Conclusions: After Norwood single RV surgery, the reconstructed aorta demonstrates abnormal anatomy and physiology. Further study is needed to evaluate the longer-term impact of these findings.

At 6 years of age, patients with hypoplastic left heart syndrome had mean age-adjusted z -scores for weight and height below the normative population, and body mass index was similar to the normative population. Males had the greatest increase in z -scores for body mass index. Trial registration ClinicalTrials.gov: NCT00115934.

Growth faltering is common in children with hypoplastic left heart syndrome (HLHS) after the Norwood operation. We previously demonstrated that transplant-free survivors remained small at 3 years of age with mean weight-for-age z-scores (WAZ) and height-for-age z-scores (HAZ) below average. We

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.

Dilated cardiomyopathy (DCM) is the final common phenotypic pathway for multiple pathological processes that are characterized by left ventricular (LV) chamber dilation and systolic dysfunction, and clinically manifested by congestive heart failure [(1)][1]. Etiologies may include antecedent viral

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

Takayasu's arteritis is a granulomatous giant-cell arteritis of the aorta and its major branches. It primarily affects East Asian women in their second or third decade of life but is well known to affect all ethnicities across the world. Given its systemic nature, Takayasu's arteritis has multiorgan involvement, with the majority of disease morbidity related to the cardiovascular, central nervous, and renal systems. This report describes an unusual presentation of Takayasu's arteritis in a 2½-year-old boy. This is the first report in the literature describing the presence of both severe aortic regurgitation and diffuse coronary artery involvement in a patient so young.

The evaluation of left ventricular systolic performance in infants undergoing extracorporeal membrane oxygenation (ECMO) using traditional ejection-phase indices is hampered by significant alterations in preload and afterload. Therefore, a load-independent index, which relates heart-rate-corrected mean velocity of circumferential fiber shortening (VCFc) to afterload, measured as end-systolic wall stress (ESS), was used to assess left ventricular function in 18 term neonates undergoing ECMO. The mean age at the onset of ECMO was 75.5 h and the duration of therapy was 171 +/- 106 h. Left ventricular performance was highest before the onset of ECMO (VCFc = 1.65 +/- 0.49 circ/s) and decreased toward normal during (1.38 +/- 0.33 circ/s) and following ECMO (1.29 +/- 0.16 circ/s). Initially, nine of 17 (53%) patients had enhanced performance for the degree of afterload but in only 16 of 48 (33%) studies during ECMO and none following ECMO was VCFc elevated beyond the normal range predicted for ESS. These changes in left ventricular performance may be the result of variations in exogenous, as well as endogenous, catecholamines rather than intrinsic alterations in myocardial contractility. It is concluded that the VCFc/ESS relation permits a meaningful assessment of ventricular performance in critically ill neonates undergoing ECMO.

To assess the utility of Doppler echocardiography in evaluation of aortic homograft valved conduit function, 10 consecutive pediatric patients had Doppler examination of aortic homograft valved conduits associated with follow-up cardiac catheterization. General correlation was found between the Doppler-derived peak systolic pressure gradient across the aortic homograft valved conduit and cardiac catheterization (r = 0.65, P less than 0.05, SEE = 20.5). One patient with multilevel obstruction had significant underestimation of gradient by Doppler due to incomplete segmental interrogation of the aortic homograft valved conduit. For the other nine patients with complete segmental conduit interrogation, correlation between Doppler and cardiac catheterization was excellent (r = 0.93, P less than 0.01, SEE = 10). Conduit insufficiency in nine out of ten patients was graded qualitatively using Doppler color flow mapping with excellent correlation between color flow mapping and cardiac catheterization (r s = 98). Aortic homograft valved conduit function can be accurately noninvasively assessed by Doppler but proximal, valve, and distal conduit segmental interrogation is necessary to localize site of obstruction if present, rule out multilevel obstruction, and avoid underestimation of systolic gradient.

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.

Plasma B-type natriuretic peptide (BNP) has been reported to be helpful in differentiating cardiac from pulmonary etiologies of dyspnea in adults. We investigated whether BNP concentration could be applied similarly in children with respiratory distress. BNP levels were measured using a rapid immunoassay in 49 infants and children presenting with acute respiratory distress. The patient's history, symptoms, physical exam, chest x-ray, and an echocardiogram were used to identify patients with congestive heart failure (CHF) from noncardiac causes of respiratory distress. Results are reported as mean+/-SD. Patients with CHF (n = 23) had BNP levels of 693.0+/-501.6 pg/ml, significantly higher than those of the group of patients with lung disease (n = 26), whose BNP was 45.2+/-64.0 pg/ml (p < 0.001). There was no significant difference in age between the two groups (29.7+/-59.3 vs 13.1+/-22.6 months; p = 0.12). A BNP level of 40 pg/ml was 84% accurate in differentiating CHF from pulmonary disease. Fifteen of 23 CHF patients had ventricular volume overload from left-to-right shunting congenital heart defects and 8/23 had left ventricular systolic dysfunction. Age-adjusted comparison of the two subgroups of CHF patients revealed that children with left ventricular systolic dysfunction had significantly higher mean BNP levels than those with left-to-right shunts (1181+/-487 vs 433+/-471 pg/ml, p = 0.0074). We conclude that BNP level is of value in differentiating cardiac from pulmonary causes of respiratory distress in children.

To measure neurodevelopment at 3 years of age in children with single right-ventricle anomalies and to assess its relationship to Norwood shunt type, neurodevelopment at 14 months of age, and patient and medical factors.All subjects in the Single Ventricle Reconstruction Trial who were alive without cardiac transplant were eligible for inclusion. The Ages and Stages Questionnaire (ASQ, n = 203) and other measures of behavior and quality of life were completed at age 3 years. Medical history, including measures of growth, feeding, and complications, was assessed through annual review of the records and phone interviews. The Bayley Scales of Infant Development, Second Edition (BSID-II) scores from age 14 months were also evaluated as predictors.Scores on each ASQ domain were significantly lower than normal (P.001). ASQ domain scores at 3 years of age varied nonlinearly with 14-month BSID-II. More complications, abnormal growth, and evidence of feeding, vision, or hearing problems were independently associated with lower ASQ scores, although models explained30% of variation. Type of shunt was not associated with any ASQ domain score or with behavior or quality-of-life measures.Children with single right-ventricle anomalies have impaired neurodevelopment at 3 years of age. Lower ASQ scores are associated with medical morbidity, and lower BSID-II scores but not with shunt type. Because only a modest percentage of variation in 3-year neurodevelopmental outcome could be predicted from early measures, however, all children with single right-ventricle anomalies should be followed longitudinally to improve recognition of delays.

BackgroundThe single-ventricle reconstruction trial randomized patients with single right ventricle lesions to a modified Blalock-Taussig or right ventricle-to-pulmonary artery shunt at the Norwood. This analysis describes outcomes at the stage 2 procedure and factors associated with a longer hospital length of stay (LOS).MethodsWe examined the association of shunt type with stage 2 hospital outcomes. Cox regression and bootstrapping were used to evaluate risk factors for longer LOS. We also examined characteristics associated with in-hospital death.ResultsThere were 393 subjects in the analytic cohort. Median stage 2 procedure hospital LOS (8 days; interquartile range [IQR], 6-14 days), hospital mortality (4.3%), transplantation (0.8%), median ventilator time (2 days; IQR, 1-3 days), median intensive care unit LOS (4 days; IQR, 3-7 days), number of additional cardiac procedures or complications, and serious adverse events did not differ by shunt type. Longer LOS was associated (R2 = 0.26) with center, longer post-Norwood LOS (hazard ratio [HR], 1.93 per log day; P

Objective. Angiographic visualization of systemic to pulmonary collaterals (SPC) has been documented in premature infants needing prolonged ventilatory support. Noninvasive identification of such communications in premature infants was reported recently. The purpose of this study was to describe: 1) incidence, 2) clinical findings and implications, and 3) short-term follow-up of SPC diagnosed by echocardiography in very low birth weight (VLBW) infants admitted to the neonatal intensive care unit. Methods. From December 1, 1994 to August 31, 1996, 196 infants with birth weight Results. SPC were demonstrated in 88 patients (66%) at 1 to 90 days of life (mean 28 days). In most cases, the SPC originated at the distal aortic arch or the proximal descending aorta. Ten patients (11%) were treated for congestive heart failure. The symptoms improved and anticongestive therapy was discontinued in 9. One patient with persistent congestive heart failure underwent therapeutic cardiac catheterization and 1 prominent SPC was embolized. Conclusions. The incidence of SPC in VLBW infants is much higher than previously reported. We postulate that SPC are bronchopulmonary communications that enlarge and/or proliferate in response to a given stimulus. These communications are associated with increased time on positive pressure ventilation and length of stay in the hospital. SPC may lead to pulmonary edema and should be searched for in VLBW infants with a more complicated course. Echocardiographic examination with color Doppler performed in premature infants to evaluate left to right shunts should include careful search for systemic to pulmonary collaterals. echocardiography, systemic to pulmonary collaterals, aortopulmonary collaterals, prematurity, pulmonary edema.

The prognosis for children with idiopathic dilated cardiomyopathy (IDC) is variable. Patients who fail to exhibit improvement in left ventricular (LV) function have a high 1-year mortality rate, whereas improvement in LV fractional shortening (LVFS) to15% is associated with better survival. However, complete recovery of LV performance to normal has not been examined.The clinical features and echocardiograms of 63 children with IDC were reviewed. Sixteen patients (group 1) were identified who demonstrated progressive improvement in LVFS, ultimately recovering to within the normal range. They were compared with 47 patients (group 2) in whom LVFS remained depressed. Group 1 LVFS at first examination was 13.6% +/- 5.1%, z = -10.8 +/- 4.0, and improved to within the normal range (33.7% +/- 3.4%, z = -0.9 +/- 1. 4, P.001). Group 2 initial LVFS was 13.6 +/- 2.3, z = -8.9 +/- 3.2 and did not change significantly (15.7% +/- 7.3%, z = -7.3 +/- 1.6). The LV was dilated at initial examination in all patients (z = 6.9 +/- 3.0). Recovery in group 1 was associated with a decrease in LV dimension to within the normal range (z = 1.3 +/- 1.6, P.001), whereas the LV dimension in group 2 patients remained increased (z = 6.2 +/- 3.4). The mean follow-up time at which LV function was noted to be normal was 4.5 +/- 3.6 years (range 0.3 to 14 years). The total duration of follow-up was 6.5 +/- 5.2 years (range 1 to 16 years).Complete recovery of LV function is possible in children with IDC. Recovery may occur within the first year after initial examination in some patients, but longer periods are needed in the majority of patients in whom LV function ultimately returned to normal.

Background. The optimal surgical treatment of complex (multiple level or recurrent) left ventricular outflow tract obstruction (LVOTO) in infancy is controversial. Staged procedures expose the children to the need for reoperation, and currently available techniques of aortoventriculoplasty are associated with the morbidities of biological and mechanical prostheses. Methods. Between July 1992 and January 1996, we have performed 24 aortic root replacements with the pulmonary autograft in pediatric patients ( Results. There were no perioperative or late deaths at follow-up (range, 2 to 25 months; mean, 13.5 ± 8 months). Mean hospital stay was 15 ± 17 days (range, 4 to 53 days). Three children had the following complications: diaphragmatic paresis (1), delayed pericardial effusion (1), and atrioventricular block requiring a pacemaker (1). In follow-up, echocardiographic findings showed absent aortic regurgitation in 3 and trivial aortic regurgitation in 5, and no significant LVOTO (mean peak gradient, 6.2 ± 7.6 mm Hg; range, 0 to 16 mm Hg). Pulmonary homograft regurgitation was absent in 5, trivial in 2, and moderate in 1. Peak right ventricular outflow tract gradient by echocardiogram was trivial in 7, and a significant gradient of 55 mm Hg has developed in 1 infant. There were no infective or embolic complications during follow-up. Conclusions. Our experience shows that aortic root replacement with the pulmonary autograft can be performed in children with excellent clinical results. The technique of root replacement combined with ventriculoplasty allows definitive and simultaneous relief of complex and multiple-level obstructive lesions. Considering the growth potential of the pulmonary autograft, this should be regarded as the optimal treatment modality in infants with complex LVOTO.

Mortality of pediatric patients with idiopathic dilated cardiomyopathy (IDC) is highest during the first 1 to 2 years the child is first seen. The echocardiograms of 72 infants and children with IDC were reviewed to determine whether left ventricular shortening fraction (LVSF), end-diastolic dimension, and wall mass were helpful in predicting outcome. Patients who were alive at last follow-up (group 1) were compared to those who died or required heart transplant (group 2). None of the indexes obtained at first examination were predictive of survival. Shortening fraction had improved significantly at follow-up 1 to 6 months after first examination in group 1 (15.1 +/- 0.9 to 24.4 +/- 1.8, p0.001) but remained unchanged in group 2 (14.1 +/- 1.5 to 15.3 +/- 3.2). No significant trends were noted in left ventricular end-diastolic dimension or wall mass. The 1- and 5-year actuarial survival for all patients was 75% and 60%, respectively. Persistent depression of LVSF15% was associated with a significantly lower survival rate (1-year 46%; 5-year 29%) compared to patients in whom follow-up LVSF was15% (1-year 97%; 5-year 90%; p0.05). It is concluded that the follow-up LVSF obtained at least 1 month after first examination is helpful in identifying pediatric patients who would benefit from early cardiac transplantation.

Outcome in 81 pediatric patients with dilated cardiomyopathy was reviewed to assess whether treatment with angiotensin-converting enzyme (ACE) inhibitors affected survival. Age at onset was 3.6 +/- 0.6 years. Twenty-seven children (group 1) were treated with ACE inhibitors. Conventional therapy was used in the remaining 54 patients (group 2). There were no significant differences between the two groups in age at onset, left ventricular shortening fraction, left ventricular end-diastolic pressure, or mean pulmonary artery pressure. Patients treated with ACE inhibitors had a significantly better survival during the first year (p0.05) with continuation of this trend throughout the second year (p = 0.06). Beyond 2 years there was a tendency toward better survival in ACE inhibitor-treated patients, but the differences were no longer significant (p = 0.14). These data, along with observations in adult patients with chronic cardiac failure, indicate that converting enzyme inhibitors have a beneficial effect on prolonging survival of infants and children with severe left ventricular dysfunction from dilated cardiomyopathy.

A review of 81 infants and children with dilated, poorly contracting left ventricles without associated structural abnormalities was undertaken to identify risk factors for poor outcome, which could be used in selecting candidates for cardiac transplantation. Significant atrial or ventricular dysrhythmias, or both, were detected on presentation or during follow-up in 24 patients. Arrhythmias were present in only 8 of 51 survivors (16%) but were detected in 16 of 30 patients (53%) who died (p less than 0.05). Patients dying suddenly were even more likely to have had documented dysrhythmias (8 of 11, p less than 0.05). Left ventricular shortening fraction was similar in survivors and nonsurvivors (14.9 +/- 1.0% vs 15.3 +/- 1.7%). Left ventricular end-diastolic pressure in 44 patients who had cardiac catheterization averaged 20.8 +/- 1.6 mm Hg. Left ventricular end-diastolic pressure was significantly higher in patients who died than in those who survived (29.5 +/- 2.2 vs 15.0 +/- 1.6 mm Hg, p less than 0.001). Analysis of actuarial survival revealed that mortality was highest during the first 6 months after presentation (19% mortality). Survival declined more gradually thereafter and was 70% at 2 years, 64% at 5 years and 52% after 11.5 years. Age at initial presentation did not have any significant impact on survival. However, left ventricular end-diastolic pressure greater than 25 torr was associated with a significantly increased mortality rate (p less than 0.05). Early cardiac transplantation should be considered in patients with markedly elevated left ventricular end-diastolic pressure or complex atrial or ventricular arrhythmias.

The interaction between the plasma catecholamine response to hypoxemia and endogenous opiate receptor blockade was investigated in 9 fetal lambs. The animals were randomly assigned to receive either normal saline (control group 1) or two doses of naloxone (group 2 = 0.5 mg/kg; group 3 = 1.0 mg/kg) by bolus injection followed by continuous infusion. Arterial PO2 decreased from 21 to 12 Torr and remained < 14 Torr for 1 h. Arterial pH declined from 7.40 to a nadir of 7.32 at 1 h. Mean plasma catecholamine concentrations rose promptly from a baseline of 524–546 pg/ml for norepinephrine (NE) and 156–235 pg/ml for epinephrine (E) to reach a peak at 15 min (NE = 2,476–3,276, p < 0.01; E = 1,856–4,065 pg/ ml, p < 0.01) and remained elevated thereafter. Neither dose of naloxone significantly altered plasma NE or E concentrations. Therefore, we conclude that endogenous opiates do not modulate the sympathoadrenal response to moderately long periods of hypoxemia unaccompanied by acidemia.

Is Perivascular Echo Brightness a Reliable Marker of Coronary Arteritis in Acute Kawasaki Syndrome?