Characteristics and outcomes of double outlet left ventricle.

Background: Double outlet left ventricle (DOLV) is a rare cardiac malformation in which both great arteries originate from the morphological left ventricle. There are limited data regarding the anatomical spectrum, characteristics and outcomes of DOLV.

Methods: All patients with DOLV between 1960 and 2008 were identified.

Assessment of myocardial deformation in children using Digital Imaging and Communications in Medicine (DICOM) data and vendor independent speckle tracking software.

Background: Analysis of myocardial deformation from data stored in Digital Imaging and Communications in Medicine format using vendor-independent software may be useful for clinical and research purposes but has not been evaluated in children.

Methods: Grayscale images were prospectively acquired on Vivid 7 (GE Healthcare) and iE33 (Philips Medical Systems) ultrasound systems in 49 children. Digital Imaging and Communications in Medicine and raw data were analyzed using vendor-independent software (Cardiac Performance Analysis, Tomtec Imaging Systems) and vendor-specific software (EchoPAC and QLAB) and results compared.

Randomized, controlled trial of individualized heparin and protamine management in infants undergoing cardiac surgery with cardiopulmonary bypass.

Objectives: We sought to determine whether infants (younger than 1 year old) had similar clinical benefits with individualized anticoagulation management as older children and adult undergoing cardiopulmonary bypass (CPB).

Background: Individualized heparin and protamine management in older children and adults undergoing CPB has been associated with improved clinical outcomes.

Methods: Ninety infants younger than 1 year of age undergoing CPB were enrolled in a randomized, controlled trial comparing weight-based anticoagulation management using activated clotting time (ACT) to individualized management with Hemostasis Management System Plus.

Hyperglycemia after pediatric cardiac surgery: impact of age and residual lesions.

Objective: We evaluated the effect of patient age and significant residual cardiac lesions on the association between hyperglycemia and adverse outcomes in children after cardiac surgery. The incidence, severity, and duration of hyperglycemia in this patient population and perioperative factors predisposing to hyperglycemia were also delineated.

Design: Retrospective, observational cohort study.

Variability in tacrolimus blood levels increases the risk of late rejection and graft loss after solid organ transplantation in older children.

  Late graft rejection impairs the long-term function of organ transplants in children. Previous studies suggest patients with wide variation in tacrolimus levels may have higher rates of late kidney and liver graft rejection. The reproducibility of this finding and impact on graft and recipient survival have not been reported.

Population trends toward increasing cardiovascular risk factors in Canadian adolescents.

Objective: To determine prevalence and cross-sectional trends over time for cardiovascular risk factors in Canadian adolescents.

Study Design: Cross-sectional trends in cardiovascular risk and lifestyle factors were gathered annually in 14- to 15-year-old students in the Niagara region, Ontario, Canada.

Results: A total of 20 719 adolescents were screened between 2002 and 2008.

Impact of prenatal diagnosis and anatomical subtype on outcome in double outlet right ventricle.

Background: We sought to investigate the influence of prenatal diagnosis and risk factors for adverse outcomes in double outlet right ventricle (DORV) not associated with heterotaxy.

Methods: Patients with a pre or postnatal diagnosis of DORV from 2000 to 2007 were identified and classified into 3 subgroups: subaortic ventricular septal defect (VSD) and normal great artery (GA) arrangement (=VSD type), tetralogy of Fallot type, and transposition of the GA type (=TGA type). Patients with heterotaxy, atrioventricular septal defect, valve atresia, and ventricular hypoplasia were excluded.

Corticosteroid administration for patients with coronary artery aneurysms after Kawasaki disease may be associated with impaired regression.

Introduction: Corticosteroid administration in Kawasaki disease (KD) is controversial but accepted as treatment for patients who do not respond to initial treatment. The impact of corticosteroids on evolving coronary artery aneurysms (CAA) and future vascular remodelling is unknown.

Methods And Results: The clinical history of 80 patients (73% male; median age at diagnosis 2.

Living at an altitude adversely affects exercise capacity in Fontan patients.

Background: Data assessing the effect of altitude on Fontan haemodynamics are limited to experimental models and case reports. Both suggest a detrimental impact. This study describes exercise performance in patients with Fontan circulation and matched controls at a low altitude versus at sea level.

Cor triatriatum sinistrum in childhood. A single institution's experience.

Introduction: Cor triatriatum sinistrum (CTS) is a rare congenital cardiac defect that can present with a wide range of symptoms and may be associated with other structural cardiac defects. Very limited data are available for pediatric patients.

Objective: To analyze the experience with CTS at a single pediatric centre, highlighting symptoms on presentation, mode of diagnosis and outcome.

Determinants of short- and long-term outcomes following triple valve surgery.

Background And Aim Of The Study: Triple valve surgery (3VS) is required in some cardiac disorders that simultaneously involve the aortic valve (AV), mitral valve (MV), and tricuspid valve (TCV). Herein are reported details of the authors' experience with 3VS, and their attempts to identify those factors associated with a poor clinical outcome.

Methods: The medical records of patients who underwent 3VS between 1985 and 2005 were reviewed.

Pretreatment with the nitric oxide donor SNAP or nerve transection blocks humoral preconditioning by remote limb ischemia or intra-arterial adenosine.

We have previously shown that remote ischemic preconditioning (rIPC) by transient limb ischemia leads to the release of a circulating factor(s) that induces potent myocardial protection. Intra-arterial injection of adenosine into a limb also leads to cardioprotection, but the mechanism of its signal transduction is poorly understood. Eleven groups of rabbits received saline control or rIPC or adenosine administration with additional pretreatment with the nitric oxide (NO) synthase blocker N(G)-nitro-l-arginine methyl ester, the NO donor S-nitroso-N-acetylpenicillamine, its non-NO-donating derivative N-acetylpenicillamine, or femoral nerve section.

Myocardial tissue Doppler velocity imaging in children: comparative study between two ultrasound systems.

Background: The investigators compared pulsed-wave tissue Doppler (PWTD) and color tissue Doppler (CTD) ventricular tissue velocities obtained using Vivid 7 (GE) and iE33 (Philips) ultrasound systems within a 30-minute time frame and examined interobserver and intraobserver variability.

Methods: Longitudinal PWTD and CTD images were prospectively acquired in 49 children from the base of the right and left ventricular free wall and septum and stored for offline analysis.

Results: Intraobserver and interobserver coefficients of variation for PWTD velocities ranged from 4% to 6% and 5% to 10% for GE and from 1% to 10% and 2% to 14% for Philips, respectively.

Anthropometric growth and utilization of enteral feeding support in pediatric heart transplant recipients.

We sought to outline trends in anthropometric growth before and after cardiac transplantation and to document our experience with the use of EFS in this population. A total of 130 patients (59% male) were enrolled and followed for a median of 4.4 yr after transplantation.

Comparison between different speckle tracking and color tissue Doppler techniques to measure global and regional myocardial deformation in children.

Background: Myocardial deformation parameters obtained by speckle-tracking echocardiography (STE) and color Doppler tissue imaging (CDTI) using two different ultrasound systems and three different software packages were compared.

Methods: Apical four-chamber, short-axis grayscale, and color Doppler tissue images were prospectively acquired using Vivid 7 and iE33 ultrasound systems in 34 children and then analyzed using EchoPAC and QLAB (STE) and SPEQLE (CDTI).

Results: Measurement of myocardial deformation was feasible for all three modalities.

Usefulness of the right ventricular systolic to diastolic duration ratio to predict functional capacity and survival in children with pulmonary arterial hypertension.

The objective of this study was to investigate the systolic to diastolic duration ratio (S:D ratio) in children with pulmonary arterial hypertension (PAH) and its association with right ventricular (RV) performance, hemodynamics, 6-minute walk test, clinical outcomes, and survival. We reviewed 503 serial echocardiograms in 47 children with PAH (mean pulmonary artery pressure >or=25 mm Hg) and compared the S:D ratio, assessed from Doppler flow of tricuspid valve regurgitation, to that in 47 age-matched controls. We reviewed echocardiograms, catheterization data, 6-minute walk tests, clinical data, lung transplantation, and death and used univariate linear regression models with a maximum likelihood algorithm for parameter estimation to investigate associations between S:D ratio and RV function, hemodynamics, functional capacity, and clinical outcomes.

Parental anxiety associated with Kawasaki disease in previously healthy children.

Objective: The objective of this study was to explore the lived experience of parents of children diagnosed with Kawasaki disease (KD) and to identify factors associated with increased levels of parental anxiety.

Study Design: Three focus groups were conducted including 25 parents of 17 patients with KD, seven (41%) of whom had coronary artery complications. A conceptual model was developed to depict parental experiences and illustrate the key issues related to heightened anxiety.

Mitral valve replacement with the Quattro stentless pericardial bioprosthesis: mid-term clinical and echocardiographic follow up.

Background And Aim Of The Study: Preservation of the subvalvar apparatus during mitral valve replacement (MVR) is associated with improved ventricular function and patient outcome. The Quattro valve is a chordally supported stentless mitral valve bioprosthesis that undergoes anticalcification treatment and is sutured to both annulus and papillary muscles heads, thus preserving annuloventricular continuity. The study aim was to assess the mid-term hemodynamic and functional results following MVR using the Quattro valve.

Superior results following the Ross procedure in patients with congenital heart disease.

Background And Aim Of The Study: The Ross procedure is a versatile operation that can be applied for aortic valve replacement (AVR) in patients with congenital heart disease (CHD), including small infants and those with complex left ventricular outflow tract (LVOT) obstruction. Herein, the clinical outcome is reported following the Ross procedure in patients with CHD at the authors' institution.

Methods: The medical records of patients who underwent the Ross procedure for CHD between 1991 and 2007 were reviewed.

Intravenous immunoglobulin preparation type: association with outcomes for patients with acute Kawasaki disease.

To determine whether two different intravenous immunoglobulin (IVIG) preparations were equally efficacious in the treatment of Kawasaki disease (KD). Single centre retrospective review of all patients treated with IVIG for KD between January 1990 and April 2007. Comparison of IVIG (dose 2 g/kg) from two commercial preparations; Iveegam stabilized with sugar (lyophilized, 5 g/ml glucose, pH 6.

Outcomes with ventricular assist device versus extracorporeal membrane oxygenation as a bridge to pediatric heart transplantation.

Extracorporeal membrane oxygenation (ECMO) has long been the sole means of mechanical support for pediatric patients with end-stage cardiac failure, but has a high waitlist mortality and a reported survival to hospital discharge of less than 50%. The purpose of this study was to compare waitlist mortality and survival for ECMO versus ventricular assist device (VAD) support. A review was conducted of all patients listed for heart transplantation (HTx) since 2002 and requiring mechanical support.

Macrophage activation syndrome in the acute phase of Kawasaki disease.

Rare cases of macrophage activation syndrome (MAS) occurring during the acute phase of Kawasaki disease (KD) have been reported. We sought to characterize, review treatment, and outcomes of KD patients with clinical features of MAS. Medical histories of patients treated for KD and MAS between January 2001 and March 2008 were reviewed.

Management and monitoring of anticoagulation for children undergoing cardiopulmonary bypass in cardiac surgery.

Cardiopulmonary bypass (CPB) creates a pro-coagulant state by causing platelet activation and inflammation leading to thrombin generation and platelet dysfunction. It is associated with severe derangements in normal homeostasis resulting in both thrombotic and hemorrhagic complications. This derangement is greater in children with congenital heart disease than in adults because of the immaturity of the coagulation system, hemodilution of coagulation factors, hyperreactive platelets, and in some patients, physiologic changes associated with cyanosis.

Long-term anticoagulation in Kawasaki disease: Initial use of low molecular weight heparin is a viable option for patients with severe coronary artery abnormalities.

Patients with severe coronary artery involvement after Kawasaki disease (KD) require long-term systemic anticoagulation. We sought to compare our experience with thrombotic coronary artery occlusions, safety profile, and degree of coronary artery aneurysm regression in KD patients treated with low molecular weight heparin (LMWH) versus warfarin. Medical records of all KD patients diagnosed between January 1990 and April 2007 were reviewed.

The Ross procedure in children: preoperative haemodynamic manifestation has significant effect on late autograft re-operation.

Objectives: The Ross procedure is the aortic valve-replacement procedure of choice in children. Nonetheless, late autograft re-operation for dilatation and/or valve regurgitation is of concern. We examined whether preoperative haemodynamic manifestation (e.