Exercise training improves activity in adolescents with congenital heart disease Six-minute walking test to assess exercise tolerance and cardiorespiratory responses during training program in children with congenital heart disease. Design and rationale for the PREVAIL study: effect of e-Health individually tailored encouragements to physical exercise on aerobic fitness among adolescents with congenital heart disease--a randomized clinical trial. Efficacy of exercise training in pulmonary arterial hypertension associated with congenital heart disease. The diagnostic and prognostic value of brain natriuretic peptides in adults with a systemic morphologically right ventricle or Fontan-type circulation. Failure of remote ischemic preconditioning to reduce the risk of postoperative acute kidney injury in children undergoing operation for complex congenital heart disease: a randomized single-center study. Marked changes in platelet count and function following pediatric congenital heart surgery. Palivizumab prophylaxis reduces hospitalization due to respiratory syncytial virus in young children with hemodynamically significant congenital heart disease. Impact of Anesthesia and Surgery for Congenital Heart Disease on the Vitamin D Status of Infants and Children: A Prospective Longitudinal Study Exercise Training for Heart Failure Patients with and without Systolic Dysfunction: An Evidence-Based Analysis of How Patients Benefit Relationship between Social-Emotional and Neurodevelopment of 2-Year-Old Children with Congenital Heart Disease. A Randomized Controlled Trial of Motavizumab Versus Palivizumab for the Prophylaxis of Serious Respiratory Syncytial Virus Disease in Children With Hemodynamically Significant Congenital Heart Disease Performance of 2-year-old children after early surgery for congenital heart disease on the Bayley Scales of Infant and Toddler Development, Third Edition. Adolescents With d-Transposition of the Great Arteries Corrected With the Arterial Switch Procedure: Neuropsychological Assessment and Structural Brain Imaging Screening for Rheumatic Heart Disease among Peruvian Children: A Two-Stage Sampling Observational Study Spectrum of steroid-resistant and congenital nephrotic syndrome in children: the PodoNet registry cohort. Severe Congenital Heart Defects Are Associated with Global Reduction of Neonatal Brain Volumes. A 4 year prospective study to determine risk factors for severe community acquired pneumonia in children in southern China† Effect of modified ultrafiltration on pulmonary function after cardiopulmonary bypass. Reproducibility of Maximal and Submaximal Exercise Testing in “Normal Ambulatory” and “Community Ambulatory” Children and Adolescents With Spina Bifida: Which Is Best for the Evaluation and Application of Exercise Training? Effect of a cardiac rehabilitation program on left ventricular diastolic function and its relationship to exercise capacity in patients with coronary heart disease: experience from a randomized, controlled study. Prediction of Coronary Heart Disease Risk in a General, Pre-Diabetic, and Diabetic Population During 10 Years of Follow-up: Accuracy of the Framingham, SCORE, and UKPDS Risk Functions Real-time transthoracic three-dimensional echocardiographic assessment of left ventricular volume and ejection fraction in congenital heart disease.
Objectives To ascertain if motivational techniques and a structured exercise programme can increase activity in adolescents afflicted with congenital heart disease ( CHD ) . Design Prospect i ve r and omised controlled trial . Setting One hundred and forty-three patients aged 12–20 years attending the tertiary centre for paediatric cardiology in Northern Irel and . Main outcome measures Increase in exercise capacity as assessed by duration of exercise stress test , and number of minutes spent in moderate to vigorous physical activity ( MVPA ) per day . Results Eighty-six patients were men ( 60 % ) , mean age was 15.60±2.27 years . Seventy-three percent were considered to have major CHD . Seventy-two participants were r and omised to the intervention group . Following intervention , duration of exercise test increased by 1 min 5 s for the intervention group ( p value 0.02 ) along with increase in predicted VO2Max ( p value 0.02 ) . There was a significant increase in minutes of MVPA per day for the intervention group from baseline to re assessment ( p value < 0.001 ) while MVPA remained much the same for the control group . Fourteen patients met the current recommendation for more than 60 min MVPA per day at baseline . This doubled to 29 participants at re assessment . There were no adverse effects or mortalities reported . Conclusions Exercise training  is safe , feasible and beneficial in adolescents with CHD . Psychological techniques can be employed to maximise the impact of interventions . Trial Registration Number IS RCT N27986270 This study assessed the exercise tolerance and the cardiorespiratory responses to a training program by the six-minute walk test ( 6'WT ) in children with congenital heart disease ( CHD ) . Seventeen cardiac and 14 healthy children performed maximal cardiopulmonary exercise test ( CPET ) and 6'WT . Reliability of 6'WT was assessed in all subjects ( test-retest ) by Bl and -Altman plots . Cardiac subjects were r and omly divided in training ( T-CHD ) and control groups ( C-CHD ) . T-CHD underwent an individualized training exercise at the ventilatory threshold ( VT ) intensity during 12 weeks . We found that the 6'WT is a reliable and reproducible test . CHD children  walked a lower distance than healthy children before training ( 472.5 + /- 18.1 vs. 548.8 + /- 7.7 m , respectively , p < 0.001 ) . Likewise , power output , oxygen uptake ( V.O ( 2 ) ) , and heart rate ( HR ) at the maximum and the VT levels , were significantly lower in patients ( p < 0.001 ) . After training , a significant improvement of walking distance ( WD ) was shown in T-CHD ( 529.6 + /- 15.3 vs. 467.7 + /- 17.1 m , p < 0.001 ) . The power output , VO2 , HR , and V.E increased slightly ( 6 to 10 % , p > 0.05 ) at peak exercise and significantly at ventilatory threshold level ( p < 0.05 ) in T-CHD . Significant relationships between WD and VO2max as well as VO2 at VT were founded ( p < 0.05 ) . We concluded that the 6'WT is a useful and reliable tool in the assessment and follow-up of functional capacity during rehabilitation program in children with CHD Intensive exercise may be an important part of rehabilitation in patients with congenital heart disease ( CHD ) . However , performing regular physical exercise is challenging for many adolescent patients . Consequently , effective exercise encouragements may be needed . Little is known on the effect of e-Health encouragements on physical fitness , physical activity , and health-related quality of life in adolescents . This trial is a nationwide interactive e-Health rehabilitation study lasting 1 year , centered on interactive use of mobile phone and Internet technology . We hypothesize that e-Health encouragements and interactive monitoring of intensive exercise for 1 year can improve physical fitness , physical activity , and health-related quality of life .  Two hundred sixteen adolescents ( age , 13 - 16 years ) with surgically corrected complex CHD but without significant hemodynamic residual defects and no restrictions to participate in physical activity are in the process of being enrolled by invitation after informed consent . Physical fitness is measured as the maximal oxygen uptake ( Vo(2 ) ) at baseline and after 12 months by an assessor blinded to the r and omization group . After baseline testing , the patients are 1:1 r and omized to an intervention group or a control group . Individually fully automated tailored e-Health encouragements -- SMS , Internet , and mobile applications--  aim ed at increasing physical activity  are delivered to the participants in the intervention group once a week . The B and ura 's Social Cognitive Theory inspires the behavioral theoretical background . The e-Health intervention and the Godfrey cycle ergometer protocol have been feasibility tested and seem applicable to adolescents with CHD . The trial is expected to contribute with new knowledge regarding how physical activity in adolescents with CHD can be increased and , possibly , comorbidity be reduced BACKGROUND The objective of this prospect i ve study was to assess the efficacy of exercise training as add-on to medical therapy in patients with congenital heart disease associated pulmonary arterial hypertension ( CHD-APAH ) . METHODS Patients with invasively confirmed CHD-APAH received in-hospital exercise training for 3 weeks and continued at home . Efficacy parameters were evaluated at baseline , after 3 and 15 weeks . Medical treatment remained unchanged . Worsening events and survival rate were assessed in a follow-up period of 21 ± 14 months . RESULTS Twenty consecutive CHD-APAH patients ( 16 female , 4 male , mean pulmonary arterial pressure 60 ± 23 mm Hg ) were included . Patients significantly improved the mean distance walked in 6 min compared to baseline by 63 ± 47 m after 3 weeks ( p<0.001 ) and by 67 ± 59 m after 15 weeks ( p=0.001 ) . Quality of life-score ( p=0.05 ) , peak oxygen consumption ( p=0.002 ) and maximal workload ( p=0.003 ) improved significantly by exercise training after 15 weeks . The 1- and 2-year survival rates were 100 % , the transplantation-free survival rate was 100 % after 1 year and 93 % after 2 years . CONCLUSION Exercise training as add-on to medical therapy may be effective in patients with CHD-APAH and improved work capacity , quality of life and further prognostic relevant parameters . It was associated with an excellent long-term survival . Further r and omized controlled studies are needed to confirm these results BACKGROUND In acquired heart disease , brain natriuretic peptide ( BNP ) and N-Terminal pro-brain natriuretic peptide ( NT-proBNP ) are increasingly used as diagnostic and prognostic markers . In adult congenital heart disease , the abnormal anatomy and physiology complicate assessment of cardiac function . We studied the clinical correlates of measurement of natriuretic peptides ( NP ) in adults with a right ventricle in the systemic position or with Fontan-type physiology . METHODS A prospect i ve longitudinal study ( follow up time 23+/-13 months , mean+/-S.D. ) was conducted in a specialised centre on 61 patients ( age 26+/-8 years ; NYHA class 1.5+/-0.6 ) including Senning/Mustard corrected transposition , congenitally corrected transposition and Fontan/total cavopulmonary connection .  Plasma NP concentration was compared with NYHA class , exercise capacity and echocardiographically determined systemic systolic ventricular function . RESULTS Neurohormone concentrations were generally elevated ( mean=290 % of upper reference limit ) and related to NYHA class ( P<0.001 , NYHA I vs. II-IV ) . No clinical ly significant relationship to ventricular function or exercise capacity was found however . An NP measurement could not predict the future course of the disease in terms of functional status or ventricular function . CONCLUSION In contrast to patients with acquired heart disease , measurement of NP seems to have low clinical value in adults with a right ventricle in the systemic position or with Fontan-type physiology OBJECTIVE The objective of this study was to evaluate whether remote ischemic preconditioning can protect kidney function in children undergoing operation for complex congenital heart disease . METHODS Children ( n = 113 ) aged 0 to 15 years admitted for complex congenital heart disease were r and omly allocated according to age to remote ischemic preconditioning and control groups . After exclusion of 8 patients , we conducted the analysis on 105 patients ( remote ischemic preconditioning group , n = 54 ; control group , n = 51 ) . Before surgery , remote ischemic preconditioning was performed as 4 cycles of 5 minutes of ischemia by inflating a cuff around a leg to 40 mm Hg above the systolic pressure . End points were development of acute kidney injury , initiation of dialysis , plasma creatinine , estimated glomerular filtration rate , plasma cystatin C , plasma and urinary neutrophil gelatinase-associated lipocalin , and urinary output . Secondary end points included postoperative blood pressure , inotropic score , and mortality , as well as morbidity reflected by reoperation and stays in the intensive care unit and hospital . RESULTS Overall , 57 of the children ( 54 % ) had acute kidney injury develop , with 27 ( 50 % ) in the remote ischemic preconditioning group and 30 ( 59 % ) in the control group ( P > .2 ) . Remote ischemic preconditioning was not associated with improvement in either any of the renal biomarkers or any of the secondary end points . CONCLUSIONS We found no evidence that remote ischemic preconditioning provided protection of kidney function in children undergoing operation for complex congenital heart disease BACKGROUND Reduced hemostatic capacity is common following  congenital heart surgery using cardiopulmonary bypass ( CPB ) . The etiology is multifactorial with dilutional coagulopathy , as well as platelet adhesion and activation in the CPB circuit and oxygenator . The purpose of the present study was to evaluate platelet count and function in children following CPB .  METHODS In a prospect i ve , observational study comprising 40 children , platelet count and function ( Multiplate Analyzer ( ® ) ) were measured before surgery , immediately after bypass , and on the first postoperative day . Furthermore , conventional coagulation analysis and thromboelastometry ( ROTEM ( ® ) ) were carried out . RESULTS A significant decrease in platelet count was observed immediately after coming of bypass ( P < 0.001 ) and persisted to the first postoperative day ( P = 0.002 ) . Platelet function was reduced immediately after bypass after induction with ADP ( P < 0.001 ) or TRAP ( P = 0.03 ) . The duration of CPB correlated significantly with the decrease in platelet count ( r = -0.62 , P = 0.0001 ) and reduction in platelet function ( r = -0.42 - 0.63 ; P < 0.01 ) . Moderate to deep hypothermia during CPB was associated with a decreased platelet function ( P = 0.01 - 0.12 ) , whereas cyanosis or previous heart surgery caused no further changes in platelet function following CPB . CONCLUSION Both platelet count and platelet function were significantly reduced after CPB in children undertaken correctional heart surgery . Duration of CPB and hypothermia was associated with significant changes in platelet function OBJECTIVES To evaluate the safety , tolerance , and efficacy of palivizumab in children with hemodynamically significant congenital heart disease ( CHD ) . STUDY DESIGN A r and omized , double-blind , placebo-controlled trial included 1287 children with CHD r and omly assigned 1:1 to receive 5 monthly intramuscular injections of 15 mg/kg palivizumab or placebo . Children were followed for 150 days . The primary efficacy end point was antigen-confirmed respiratory syncytial virus ( RSV ) hospitalization . RESULTS Palivizumab recipients had a 45 % relative reduction in RSV hospitalizations ( P=.003 ) , a 56 % reduction in total days of RSV hospitalization per 100 children ( P=.003 ) , and a 73 % reduction in total RSV hospital days with increased supplemental oxygen per 100 children ( P=.014 ) . Adverse events were similar in the treatment groups ; no child had drug discontinued for a related adverse event . Serious adverse events occurred in 55.4 % of palivizumab recipients and 63.1 % of placebo recipients ( P<.005 ) ; none were related to palivizumab . Twenty-one children ( 3.3 % ) in the palivizumab group and 27 ( 4.2 % ) in the placebo group died ; no deaths were attributed to palivizumab . The rates of cardiac surgeries performed earlier than planned were similar in the treatment groups . CONCLUSIONS Monthly palivizumab ( 15 mg/kg IM ) was safe , well-tolerated , and effective for prophylaxis of serious RSV disease in young children with hemodynamically significant CHD Background : Vitamin D is recognized as a pleiotropic hormone important for the functioning of organ systems , including those central to critical illness pathophysiology . Recent studies have reported associations between vitamin D status and outcome among critically ill adults and children . Preoperative vitamin D status , impact of operative techniques , and relationship between immediate postoperative vitamin D levels and clinical course have not been described in the pediatric congenital heart disease ( CHD ) population . The objective of this study was to describe the impact of CHD surgery on vitamin D status and relationship between postoperative levels and clinical course . Methods : A prospect i ve cohort study was conducted from 2009 to 2011 at a single tertiary care pediatric hospital . A total of 58 children with CHD were enrolled and  blood collected preoperatively , intraoperatively , and postoperatively .  Serum 25-hydroxyvitamin D ( 25OHD ) was measured using liquid chromatography – mass spectrometry . Results : The mean preoperative 25OHD was 58.0 nM ( SD , 22.4 ) , with 42 % being deficient ( < 50 nM ) . Postoperatively , we identified a 40 % decline in 25OHD to 34.2 nM ( SD , 14.5 ) with 86 % being deficient . Intraoperative measurements determined that initiation of cardiopulmonary bypass coincided with abrupt decline . CHD patients requiring catecholamines had lower postoperative 25OHD ( 38.2 vs. 26.5 nM , P = 0.007 ) , findings confirmed through multivariate logistic regression . Lower postoperative 25OHD was associated with increased fluid requirements and intubation duration . Conclusions : Most CHD patients are vitamin-D deficient postoperatively due to low preoperative levels and a significant intraoperative decline . Interventional studies will be required to determine whether prevention of postoperative vitamin D deficiency improves outcome Significant benefits can be derived by heart failure patients from exercise training . This paper provides an evidence -based assessment of expected clinical benefits of exercise training for heart failure patients . Meta-analyses and r and omized , controlled trials of exercise training in heart failure patients were review ed from a search of PubMed , Cochrane Controlled Trial Registry ( CCTR ) , CINAHL , and EMBASE . Exercise training improves functional capacity , quality of life , hospitalization , and systolic and diastolic function in heart failure patients . Heart failure patients with preserved systolic function ( HFnEF ) participating in exercise training studies are more likely to be women and are 5–7 years older than their systolic heart failure ( CHF ) counterparts . All patients exhibit low functional capacities , although in HFnEF patients this may be age related , therefore subtle differences in exercise prescriptions are required . Published works report that exercise training is beneficial for heart failure patients with and without systolic dysfunction OBJECTIVE The purpose of this study was to describe social-emotional outcomes and the relationship with neurodevelopmental outcomes in a cohort of 2-year-old children who underwent surgery for congenital heart disease ( CHD ) in infancy , and explore the relationship between the outcomes and parental and surgical factors . DESIGN A two-center prospect i ve cross-sectional cohort study . PATIENTS A cohort of 105 2-year-olds who underwent surgery in infancy for severe CHD MEASURES : Social-emotional and neurodevelopment was evaluated with the  Infant and Toddler Social and Emotional Assessment tool ( ITSEA ) , and the Bayley Scales of Infant Toddler Development , Third Edition . RESULTS Neurodevelopment was delayed in the CHD cohort with significantly worse results compared to published Australian-based norms in all domains ( P < .001 ) and in the Cognitive ( P < .001 ) and Language ( P < .001 ) domains with respect to the reported American norms . Social-emotional outcome was similar to Australian norms in all domains but better than the American based norms in the Internalizing domain ( P < .05 ) . Higher maternal education was associated to better neurodevelopmental outcome in all domains and better scores in the internalizing and externalizing domains of the ITSEA . There was a moderate correlation ( r = 0.43 , P < .001 ) between Language and social-emotional competence . Motor development was influenced by the need for a significant cardiac reoperation . CONCLUSIONS The influences of social factors may be underestimated in the outcome of children with CHD .  Language development in those with CHD may be improved with intervention targeting social-emotional competence ; further research is needed in this area Children with hemodynamically significant congenital heart disease ( CHD ) are at risk for serious respiratory syncytial virus ( RSV ) disease . This study was design ed to assess the safety and tolerability of motavizumab versus palivizumab in children with CHD and was not powered for efficacy . Patients ( n = 1236 ) aged ≤24 mo were r and omized to receive five monthly doses ( 15 mg/kg ) of motavizumab or palivizumab during the RSV season . Adverse events ( AEs ) and serious AEs ( SAEs ) were recorded through 30 d after the last dose . RSV hospitalizations and RSV outpatient medically attended lower respiratory tract infections ( MALRI ; season 2 ) were summarized . Approximately 93 and 50 % of patients reported an AE or SAE , respectively . Skin events occurred in 19.3 % of motavizumab recipients and 16.2 % of palivizumab recipients . Rates of hospitalizations and RSV MALRI were similar between treatment groups [ relative risk ( RR ) : 0.75 ; 95 % CI , 0.34–1.59 and RR : 0.49 ; 95 % CI , 0.10–1.99 , respectively ; both p > 0.05 ] . Motavizumab and palivizumab had similar safety profiles in children with hemodynamically significantly CHD ; with the exception of skin events which were increased in motavizumab recipients . Safety and efficacy were consistent with another study comparing motavizumab with palivizumab in premature infants without CHD BACKGROUND Previous research on developmental outcomes of infants with congenital heart disease ( CHD ) has shown delays in both cognitive and motor skills . AIMS To describe outcomes on the Bayley Scales of Infant and Toddler Development , 3rd edition ( Bayley-III ) for infants with CHD and to compare those findings to published results for similar sample s of infants assessed on the 2nd edition of the Bayley Scales ( BSID-II ) . STUDY DESIGN Prospect i ve cohort . PARTICIPANTS AND OUTCOME MEASURES Of 50 infants with CHD who participated in this longitudinal study ( 2006 - 2008 ) at the Royal Children 's Hospital in Melbourne , Australia , 47 were assessed on the Bayley-III ( median age=24.5 months ) ,  administered by a psychologist or neonatologist . Although neither assessor was blind to the CHD diagnosis , they were unaware of results of previous developmental assessment s conducted in this longitudinal study . RESULTS For the Bayley-III cognitive composite score , 17.0 % of infants showed mild delays ( 1 - 2 SD below the mean ) , 2.1 % had moderate delays ( 2 - 3 SD below the mean ) , and none had severe delays ( greater than 3 SD below the mean ) . Motor composite scores showed mild delays in 10.9 % of infants and moderate delays in 2.2 % ; none had severe motor delays . These findings differ from study results using the BSID-II in similar infants . CONCLUSIONS The Bayley-III may underestimate developmental delay in 2-year-old children with CHD when compared to results of similar children tested at 12 - 36 months of age on the BSID-II Background — We report neuropsychological and structural brain imaging assessment s in children 16 years of age with d-transposition of the great arteries who underwent the arterial switch operation as infants . Children were r and omly assigned to a vital organ support method , deep hypothermia with either total circulatory arrest or continuous low-flow cardiopulmonary bypass . Methods and Results — Of 159 eligible adolescents , 139 ( 87 % ) participated .  Academic achievement , memory , executive functions , visual-spatial skills , attention , and social cognition were assessed . Few significant treatment group differences were found . The occurrence of seizures in the postoperative period was the medical variable most consistently related to worse outcomes . The scores of both treatment groups tended to be lower than those of the test normative population s , with substantial proportions scoring ≥1 SDs below the expected mean . Although the test scores of most adolescents in this trial cohort are in the average range , a substantial proportion have received remedial academic or behavioral services ( 65 % ) . Magnetic resonance imaging abnormalities were more frequent in the d-transposition of the great arteries group ( 33 % ) than in a referent group ( 4 % ) . Conclusions — Adolescents with d-transposition of the great arteries who have undergone the arterial switch operation are at increased neurodevelopmental risk . These data suggest that children with congenital heart disease may benefit from ongoing surveillance to identify emerging difficulties . Clinical Trial Registration — URL : http://www . clinical trials.gov . Unique identifier : NCT00000470 Background The objective of the study was to evaluate the implication s of different classifications of rheumatic heart disease on estimated prevalence , and to systematic ally assess the importance of incidental findings from echocardiographic screening among schoolchildren in Peru . Methods We performed a cluster r and omized observational survey using portable echocardiography among schoolchildren aged 5 to 16 years from r and omly selected public and private schools in Arequipa , Peru . Rheumatic heart disease was defined according to the modified World Health Organization ( WHO ) criteria and the World Heart Federation ( WHF ) criteria . Findings Among 1395 eligible students from 40 classes and 20 schools , 1023 ( 73 % ) participated in the present survey . The median age of the children was 11 years ( interquartile range [ IQR ] 8–13 years ) and 50 % were girls . Prevalence of possible , probable and definite rheumatic heart disease according to the modified WHO criteria amounted to 19.7/1000 children and ranged from 10.2/1000 among children 5 to 8 years of age to 39.8/1000 among children 13 to 16 years of age ; the prevalence of borderline/definite rheumatic heart disease according to the WHF criteria was 3.9/1000 children . 21 children ( 2.1 % ) were found to have congenital heart disease , 8 of which were referred for percutaneous or surgical intervention . Conclusions Prevalence of RHD in Peru was considerably lower compared to endemic regions in sub-Saharan Africa , southeast Asia , and Oceania ; and paralleled by a comparable number of undetected congenital heart disease . Strategies to address collateral findings from echocardiographic screening are necessary in the setup of active surveillance programs for RHD . Trial Registration Clinical Trials.gov identifier : BACKGROUND AND OBJECTIVES Steroid-resistant nephrotic syndrome is a rare kidney disease involving either immune-mediated or genetic alterations of podocyte structure and function . The rare nature , heterogeneity , and slow evolution of the disorder are major obstacles to systematic genotype-phenotype , intervention , and outcome studies , hampering the development of evidence -based diagnostic and therapeutic concepts . To overcome these limitations , the PodoNet Consortium has created an international registry for congenital nephrotic syndrome and childhood-onset steroid-resistant nephrotic syndrome . DESIGN , SETTING , PARTICIPANTS , & MEASUREMENTS Since August of 2009 , clinical , biochemical , genetic , and histopathologic information was collected both retrospectively and prospect ively from 1655 patients with childhood-onset steroid-resistant nephrotic syndrome , congenital nephrotic syndrome , or persistent subnephrotic proteinuria of likely genetic origin at 67 centers in 21 countries through an online portal . RESULTS Steroid-resistant nephrotic syndrome manifested in the first 5 years of life in 64 % of the patients . Congenital nephrotic syndrome accounted for 6 % of all patients . Extrarenal abnormalities were reported in 17 % of patients . The most common histopathologic diagnoses were FSGS ( 56 % ) , minimal change nephropathy ( 21 % ) , and mesangioproliferative GN ( 12 % ) . Mutation screening was performed in 1174 patients , and a genetic disease cause was identified in 23.6 % of the screened patients . Among 14 genes with reported mutations , abnormalities in NPHS2 ( n=138 ) , WT1 ( n=48 ) , and NPHS1 ( n=41 ) were most commonly identified . The proportion of patients with a genetic disease cause decreased with increasing manifestation age : from 66 % in congenital nephrotic syndrome to 15%-16 % in schoolchildren and adolescents . Among various intensified immunosuppressive therapy protocol s , calcineurin inhibitors and rituximab yielded consistently high response rates , with 40%-45 % of patients achieving complete remission . Confirmation of a genetic diagnosis but not the histopathologic disease type was strongly predictive of intensified immunosuppressive therapy responsiveness . Post-transplant disease recurrence was noted in 25.8 % of patients without compared with 4.5 % ( n=4 ) of patients with a genetic diagnosis . CONCLUSIONS The PodoNet cohort may serve as a source of reference for future clinical and genetic research in this rare but significant kidney disease OBJECTIVES To determine neonatal global and regional brain volumes in infants with congenital heart disease ( CHD ) in comparison with healthy controls and to determine brain growth . STUDY DESIGN Prospect i ve cohort study in infants undergoing open-heart surgery for complex CHD . Global and regional volumetric measurements on preoperative cerebral magnetic resonance imaging were manually segmented in children without overt brain lesions . RESULTS Preoperative brain volumetry of 19 patients demonstrates reduction in total and regional brain volumes , without any specific regional predilection compared with 19 healthy control infants ( total brain volume reduction : 21 % , regional brain volume reduction 8%-28 % , all P < .001 ) . CONCLUSIONS Infants with CHD undergoing bypass surgery  have smaller brain volumes prior to surgery without a specific regional predilection . This suggests a fetal origin of reduced brain growth Abstract Background Pneumonia is the major cause of death under 5 years . With high CAP numbers in China and growing access to PICUs , factors associated with severe CAP need to be determined to optimize care . Objective To prospect ively determine PICU CAP admission features and outcomes . Methods A 4 year prospect i ve study of CAP aged 1 month to < 14 years admitted to PICU , Children 's Hospital Affiliated to Soochow University , China . All were managed in a st and ard manner . Clinical , laboratory , and imaging findings were collected systematic ally . All received antibiotics . Results Eight hundred ten ( 7 % ) of 10,836 CAP hospital admissions needed PICU . Seven hundred seven ( 87 % ) were enrolled . PICU CAP children were young ( 76 % ≤ 12 months ) and 33 % had co‐morbid conditions ; 21 % congenital heart disease.21 % required mechanical ventilation . The average length of PICU stay was 5 days ( range , 3–27 ) . The case fatality rate was 5.8 % . Viruses were detected in 38 % , RSV 24 % ; bacteria in 23 % , Streptococcus pneumoniae 7 % , Haemophilus influenza b 4 % , Mycoplasma 11 % . On single factor analysis , PICU admission respiratory rate > 70/min , grunting/groaning , head nodding , cyanosis , and anemia were associated with respiratory failure and with fatality . On multivariate analysis only presence of congenital heart disease , Trisomy 21 and immunodeficiency correlated with fatality ; not microbe nor PICU findings . Conclusions Young age and underlying congenital heart disease were associated factors for PICU support in CAP in China . Early referral if altered sensorium , high respiratory rate , head nodding , grunting and anemia , and universal access to conjugated vaccines may decrease morbidity and mortality . Pediatr Pulmonol . 2013 ; 48:390–397 . © 2012 Wiley Periodicals , BACKGROUND Pulmonary dysfunction is one of the most common manifestations of inflammatory response after cardiopulmonary bypass ( CPB ) . OBJECTIVE This prospect i ve r and omized study was conducted to evaluate the effect of a modified ultrafiltration ( MUF ) technique on pulmonary function after CPB in children . METHODS Forty patients weighing from 5 to 10 kg with congenital heart disease who required CPB for primary biventricular operative repair were prospect ively r and omized into two groups . The control group received conventional ultrafiltration ( CUF ) during CPB , and the study group received CUF and MUF .  Pulmonary compliance ( static and dynamic ) and gas exchange capacity of the lung expressed as oxygen index , respiratory index , ventilation index , and alveolar-arterial oxygen pressure difference were measured after intubation ( baseline ) , at the termination of CPB , at the end of MUF , on admission to the ICU , and 6 h postoperatively . RESULTS There was no significant difference in lung compliance and gas exchange between the two groups before CPB . CPB produced a significant decrease in static and dynamic lung compliance in both groups . In the control group , static and dynamic lung compliance decreased from 1.0 + /- 0.3 to 0.90 + /- 0.3 mL/cm/kg and 0.87 + /- 0.2 to 0.71 + /- 0.1 mL/cm/kg ( + /- SE ) [ p = 0.0002 and p = 0.002 , respectively ] . In the study group , static and dynamic lung compliance decreased from 1.0 + /- 0.2 to 0.89 + /- 0.03 mL/cm/kg and 0.94 + /- 0.2 to 0.77 + /- 0.1 mL/cm/kg ( p = 0.002 and p = 0.002 , respectively ) . There was no significant difference in the decrease in static ( p = 0.9 ) or dynamic lung compliance ( p = 0.3 ) between the two groups . MUF produced a significant immediate improvement in both static lung compliance ( 0.89 + /- 0.2 to 0.98 + /- 0.2 mL/cm/kg , p = 0.03 ) and dynamic lung compliance ( 0.77 + /- 0.1 to 0.93 + /- 0.2 mL/cm/kg , p = 0.007 ) . The same was observed regarding the gas exchange capacity . CPB produced a significant decrease in lung gas exchange capacity , and MUF produced a significant immediate improvement in lung gas exchange capacity . The effect of MUF on lung compliance and gas exchange capacity was not sustained after admission to the ICU nor 6 h later postoperatively . There was no significant difference in the time of extubation between the two groups ( 12 + /- 3 h and 13 + /- 2 h , p = 0.4 ) , the length of ICU stay , or the total hospital stay postoperatively . CONCLUSIONS The use of MUF after CPB can produce an immediate improvement in lung compliance and gas exchange capacity , which may effectively minimize pulmonary dysfunction postbiventricular repair of congenital heart disease . However , these improvements are not sustained for the first 6 h postoperatively and do not reduce the duration of postoperative intubation , ICU stay , or total hospital stay Background With emerging interest in exercise and lifestyle interventions for children and adolescents with spina bifida , there is a need for appropriate measurements in exercise testing . Objective The purpose of this study was to assess both reliability and agreement of maximal and submaximal exercise measures in “ normal ambulatory ” and “ community ambulatory ” children and adolescents with spina bifida . Design This was a reproducibility study . Methods Twenty-three children and adolescents with spina bifida ( 10 normal ambulatory and 13 community ambulatory ) participated in the study . Maximal exercise outcomes were measured using a grade d treadmill test .  Peak measures ( peak oxygen uptake [ V̇o2peak ] , peak heart rate [ HRpeak ] , heart rate response [ HRR ] , and oxygen pulse ) were recorded . For submaximal measures , heart rate ( HR ) and oxygen uptake ( V̇o2 ) at the ventilatory threshold and oxygen uptake efficiency slope ( OUES ) were derived from the maximal measures . Functional performance was measured as the 6-minute walking distance and the maximal speed during the treadmill test . After checking for normality and heteroscedasticity , paired t tests , intraclass correlation coefficients ( ICCs ) , and the smallest detectable difference ( SDD ) or the coefficient of variation ( CV ) were calculated . Results Performance measures showed good reliability and agreement . For maximal measures , acceptable ICCs were found for all measures . For submaximal measures , only HR at the ventilatory threshold showed an ICC of less than .80 . Agreement showed a CV of less than 10 % for all measures , except for V̇o2 at the ventilatory threshold , HRR , and OUES . Limitations Limitations of the study include missing data due to equipment failure . Furthermore , the outcomes were limited to normal ambulatory and community ambulatory children and adolescents with spina bifida . Conclusions Both maximal and submaximal measures of exercise testing can be used for discriminative purpose s in ambulatory children and adolescents with spina bifida . For evaluative purpose s , HR measures are superior to V̇o2 measures , while taking into account the individual variation of 5 % to 8 % . The SDD was 0.5 km/h for peak speed and 36.3 m for 6-minute walking distance . Heart rate response , oxygen pulse , and OUES are not recommended in the evaluation of exercise testing in this population BACKGROUND A cardiac rehabilitation and prevention program ( CRPP ) is a recognized nonpharmacological modality in the management of coronary heart disease ( CHD ) . However , the effect of a CRPP on systolic function of the heart is controversial , and no data exists on diastolic function in CHD . A r and omized , controlled study was conducted to address these issues . METHODS Patients ( n = 269 ) with recent acute myocardial infa rct ion ( n = 193 ) or after percutaneous coronary intervention ( PCI ) ( n = 76 ) were r and omized to either CRPP ( 2-hour twice-weekly exercise program for 8 weeks ) or conventional therapy ( control group ) . Serial treadmill exercise testing and at-rest echocardiography were performed during phases 1 ( baseline ) , 2 ( post-exercise training ) , and 3 ( 8-month follow up ) . RESULTS The prevalence of left ventricular ( LV ) abnormal relaxation pattern ( ARP ) of diastolic dysfunction was increased in the control group only in phase 3 ( 65 % vs 88 % , chi2 = 7.6 , P < .01 ) . Significant improvement of individual LV diastolic parameters towards less severe delayed relaxation was also observed in the CRPP group , especially in those with recent acute myocardial infa rct ion or ARP . The gain in exercise capacity was faster and more substantial in the CRPP than the control group ( P < .001 for phase 2 , P < .05 for phase 3 ) , and was significantly correlated with LV diastolic indices in those with ARP . Exercise training had neutral effects on LV systolic function and rate-pressure product . CONCLUSIONS In patients with CHD , CRPP prevented the progression of resting LV diastolic dysfunction , without affecting systolic function . In those with ARP , the improvement of diastolic function predicted the gain in exercise capacity OBJECTIVE To test the validity of the Framingham , Systematic Coronary Risk Evaluation ( SCORE ) , and UK Prospect i ve Diabetes Study ( UKPDS ) risk function in the prediction of risk of coronary heart disease ( CHD ) in population s with normal glucose tolerance ( NGT ) , intermediate hyperglycemia , and type 2 diabetes . RESEARCH DESIGN AND METHODS Calibration and discrimination of the three prediction models were tested using prospect i ve data for 1,482 Caucasian men and women , 50–75 years of age , who participated in the Hoorn Study . All analyses were stratified by glucose status . RESULTS During 10 years of follow-up , a total of 197 CHD events , of which 43 were fatal , were observed in this population , with the highest percentage of first CHD events in the diabetic group . The Framingham and UKPDS prediction models overestimated the risk of first CHD event in all glucose tolerance groups . Overall , the prediction models had a low to moderate discriminatory capacity . The SCORE risk function was the best predictor of fatal CHD events in the group with NGT ( area under the receiver operating characteristic curve 0.79 [ 95 % CI 0.70–0.87 ] ) , whereas the UKPDS performed better in the intermediate hyperglycemia group ( 0.84 [ 0.74–0.94 ] ) in the estimation of fatal CHD risk . After exclusion of known diabetic patients , all prediction models had a higher discriminatory ability in the group with diabetes . CONCLUSIONS The use of the Framingham function for prediction of the first CHD event is likely to overestimate an individual 's absolute CHD risk . In CHD prevention , application of the SCORE and UKPDS functions might be useful in the absence of a more valid tool OBJECTIVE The purpose of this study was to assess the ( 1 ) feasibility of real-time three-dimensional echocardiography ( RT-3DE ) data acquisition and ( 2 ) volumes and function of the abnormal left ventricle ( LV ) in adult patients with congenital heart disease ( CHD ) , compared with magnetic resonance imaging ( MRI ) data . METHODS Thirty-two patients ( 59 % were male ) with CHD were evaluated on the same day by MRI and RT-3DE . Acquisition of RT-3DE data sets was feasible in 29 of the 32 patients ( 91 % ) . The time of 3D data acquisition was 4 + /- 2 minutes , and LV analysis was 17 + /- 5 minutes per patient for manual border tracing . RESULTS A good correlation was observed between RT-3DE with manual border detection and MRI for LV end-diastolic volume ( r = 0.97 ) , LV end-systolic volume ( r = 0.98 ) , and LV ejection fraction ( r = 0.94 ) . CONCLUSION RT-3DE is feasible for volumetric analysis of the abnormal LV allowing accurate determination of LV volume and ejection fraction compared with MRI in adult patients with CHD
Children and adolescents with CHD have lower VO2max and HR compared to controls
BACKGROUND Children and adolescents with congenital heart disease often have alterations in their exercise capacity that can be evaluated by various functional testing .
OBJECTIVE To evaluate the functional capacity of children and adolescents with congenital heart disease ( CHD ) with systematic review and meta-analyses .