Tissue Doppler imaging detects impaired biventricular performance shortly after congenital heart defect surgery.

Cardiac surgery with cardiopulmonary bypass is associated with the development of a systemic inflammatory response, which can lead to myocardial damage. However, knowledge concerning the time course of ventricular performance deterioration and restoration after correction of a congenital heart defect (CHD) in pediatric patients is sparse. Therefore, the authors perioperatively quantified left ventricular (LV) and right ventricular (RV) performance using echocardiography.

Mild residual pulmonary stenosis in tetralogy of fallot reduces risk of pulmonary valve replacement.

Background: Current surgical strategies that aim at preventing pulmonary regurgitation in patients with corrected tetralogy of Fallot (cToF) may result in a certain grade of residual pulmonary stenosis (PS). The clinical implications of a postoperative residual PS in cToF patients remain unclear. Pulmonary valve replacement (PVR) is frequently needed during follow-up of cToF patients.

Impaired cardiac reserve in asymptomatic patients with moderate pulmonary restenosis late after relief of severe pulmonary stenosis: evidence for diastolic dysfunction.

Background: Patients with moderate pulmonary valve restenosis late after relief of severe pulmonary stenosis (PS) may show decreased exercise tolerance. To elucidate the mechanism of decreased exercise tolerance, we evaluated cardiac response to physical and pharmacological stress in these patients and compared results with those of patients with native moderate PS.

Methods: Twenty asymptomatic patients with moderate PS were divided into 2 groups: Group I (late after relief of severe PS, n=9), and Group II (no previous intervention, n=11).

The diagnostic and therapeutic aspects of loss-of-function cardiac sodium channelopathies in children.

Background: Loss-of-function sodium channelopathies manifest as a spectrum of diseases including Brugada syndrome (BrS) and cardiac conduction disease.

Objective: To analyze the diagnostic and therapeutic aspects of these disorders in children.

Methods: Patients aged ≤ 16 years with genetically confirmed loss-of-function sodium channelopathies (SCN5A mutation), presenting with cardiac symptoms, positive family history, and/or abnormal electrocardiogram (ECG), were included.

Is cardiac CT a reproducible alternative for cardiac MR in adult patients with a systemic right ventricle?

Objective: 20 % of patients with a systemic RV are pacemaker dependent, and unsuitable to undergo cardiac magnetic resonance (CMR). Multidetector row computed tomography (MDCT) could provide a reproducible alternative to CMR in these patients. The aim of this study was to compare variability of MDCT with CMR.

Normal and abnormal development of the cardiac conduction system; implications for conduction and rhythm disorders in the child and adult.

The cardiac conduction system is a specialized network that initiates and closely coordinates the heart beat. Cardiac conduction system development is intricately related to the development and maturation of the embryonic heart towards its four-chambered form, as is indicated by the fact that disturbed development of cardiac structures is often accompanied by a disturbed formation of the CCS. Electrophysiological studies have shown that selected conduction disturbances and cardiac arrhythmias do not take place randomly in the heart but rather at anatomical predilection sites.

Relation of prolonged tissue Doppler imaging-derived atrial conduction time to atrial arrhythmia in adult patients with congenital heart disease.

Atrial arrhythmia (AA) is common in adult patients with congenital heart disease (CHD). To enable the prevention of AA or its complications, timely identification of adult patients with CHD at risk of AA is crucial. Long total atrial activation times have been related to AA.

Mid-term results of bidirectional cavopulmonary anastomosis and hemi-Mustard procedure in anatomical correction of congenitally corrected transposition of the great arteries.

Objectives: The Senning or Mustard procedure combined with the arterial switch operation (ASO) (± VSD and no left ventricular (LV) outflow tract obstruction) or the Rastelli operation (VSD and LV outflow tract obstruction) has become the preferred strategy over conventional repair as it is thought to prevent long-term dysfunction of the right ventricle (RV). More recently, hemi-Mustard rerouting of blood from the inferior vena cava to the RV in combination with bidirectional cavopulmonary anastomosis (BCPA) has been adopted by some centres for potential benefits over the classic atrial switch procedure. The aim of this study was to analyse our experience with hemi-Mustard and BCPA as part of an anatomical repair of congenitally corrected transposition of the great arteries (CCTGA) in selected patients.

Psychological functioning and disease-related quality of life in pediatric patients with an implantable cardioverter defibrillator.

The objective of this multicenter study was to evaluate psychological functioning and disease-related quality of life (DRQoL) in pediatric patients with an implantable cardioverter defibrillator (ICD) in The Netherlands. Thirty patients were investigated; the mean age was 16.3 years, and the mean duration of implantation was 3.

Effect of age on exercise capacity and cardiac reserve in patients with pulmonary atresia with intact ventricular septum after biventricular repair.

Objectives: In patients with pulmonary atresia with intact ventricular septum (PAIVS), biventricular repair is considered to be the optimal treatment option in the absence of significant right ventricular (RV) hypoplasia. However, long-term clinical outcome studies are limited. We evaluated exercise capacity and cardiac function during pharmacological stress in children and young adults with PAIVS after biventricular repair.

Wide distribution of closely related, antibiotic-producing Arthrobacter strains throughout the Arctic Ocean.

We isolated 16 antibiotic-producing bacterial strains throughout the central Arctic Ocean, including seven Arthrobacter spp. with almost identical 16S rRNA gene sequences. These strains were numerically rare, as revealed using 454 pyrosequencing libraries.

Tissue-velocity magnetic resonance imaging and tissue Doppler imaging to assess regional myocardial diastolic velocities at the right ventricle in corrected pediatric Tetralogy of Fallot patients.

Objectives: In patients with corrected Tetralogy of Fallot (cToF), pulmonary regurgitation and subsequent increased right ventricular (RV) end-diastolic volume are diastolic parameters related to adverse outcome. In addition, abnormalities of the RV outflow tract (RVOT) independently promote RV dilatation in cToF patients. Tissue Doppler imaging (TDI) and tissue-velocity magnetic resonance imaging (TV-MRI) enable quantitative assessment of regional diastolic performance by measuring myocardial velocities.

Persistent fetal sinus bradycardia associated with maternal anti-SSA/Ro and anti-SSB/La antibodies.

Objective: To study the clinical course and outcome of fetal sinus bradycardia (SB) due to maternal antibody-induced sinus node dysfunction.

Methods: We reviewed the maternal, prenatal, and postnatal findings of fetuses with SB associated with elevated maternal anti-SSA/Ro and anti-SSB/La antibodies.

Results: Of the 6 cases diagnosed prenatally, 3 had isolated SB persisting after birth and had a good prognosis.

Tissue Doppler imaging in the left ventricle and right ventricle in healthy children: normal age-related peak systolic velocities, timings, and time differences.

Aims: Tissue Doppler imaging (TDI) enables assessment of velocities and timings within the left (LV) and the right (RV) ventricle with high temporal resolution. Knowledge on normal age-related values of peak systolic velocities and timings in healthy children may optimize the benefit of device-based therapies in paediatric patients with heart failure.

Methods And Results: A total of 123 healthy children (from 1 month to 18 years old) underwent TDI evaluation of the RV and LV.

Percutaneous treatment of native aortic coarctation in adults.

Aortic coarctation is a common congenital cardiac defect, which can be diagnosed over a wide range of ages and with varying degrees of severity. We present two cases of patients diagnosed with aortic coarctation in adulthood. Both patients were treated by an endovascular approach.

Comparison of transplacental treatment of fetal supraventricular tachyarrhythmias with digoxin, flecainide, and sotalol: results of a nonrandomized multicenter study.

Background: Fetal tachyarrhythmia may result in low cardiac output and death. Consequently, antiarrhythmic treatment is offered in most affected pregnancies. We compared 3 drugs commonly used to control supraventricular tachycardia (SVT) and atrial flutter (AF).

Exercise capacity and cardiac reserve in children and adolescents with corrected pulmonary atresia with intact ventricular septum after univentricular palliation and biventricular repair.

Objective: Management of pulmonary atresia with intact ventricular septum is challenging and depends on the severity of right ventricular hypoplasia. Clinical outcomes of biventricular repair seem favorable to univentricular palliation, but data on superiority of biventricular repair regarding exercise capacity are conflicting. We investigated the response to physical and pharmacologic stress in patients with surgically corrected pulmonary atresia with intact ventricular septum.

Real-time three-dimensional echocardiography: segmental analysis of the right ventricle in patients with repaired tetralogy of fallot.

Background: The accurate assessment of right ventricular (RV) function and dimensions has important prognostic implications in patients with repaired tetralogy of Fallot (ToF). Three-dimensional imaging is the preferred methodology to evaluate RV function. Novel postprocessing software applications to evaluate three-dimensional data have provided insight into RV function and dimensions by analyzing the various RV components (inlet, apical trabecular, outlet).

Thirty-year experience with the arterial switch operation.

Background: We evaluated the results of the arterial switch operation (ASO) being performed at our institution for more than 30 years and identified risk factors for mortality and reoperation.

Methods: Clinical outcome of 332 consecutive patients with transposition of the great arteries undergoing ASO was retrospectively analyzed, using surgical reports, medical charts, and latest follow-up echocardiography. Statistical analysis was performed using the Kaplan-Meier method and univariable and multivariable binary logistic and Cox regression analyses.

Dilatation of the great arteries in an infant with marfan syndrome and ventricular septal defect.

We describe an infant presenting with contractures of the fingers, a large ventricular septal defect (VSD), and severe pulmonary artery dilatation. He had clinical and echocardiographic features of both neonatal or infantile Marfan syndrome (MFS) and congenital contractural arachnodactyly. After surgical VSD closure, the aortic root developed progressive dilatation while the size of pulmonary artery returned to normal limits.

Microbial community structure of Arctic multiyear sea ice and surface seawater by 454 sequencing of the 16S RNA gene.

Dramatic decreases in the extent of Arctic multiyear ice (MYI) suggest this environment may disappear as early as 2100, replaced by ecologically different first-year ice. To better understand the implications of this loss on microbial biodiversity, we undertook a detailed census of the microbial community in MYI at two sites near the geographic North Pole using parallel tag sequencing of the 16S rRNA gene. Although the composition of the MYI microbial community has been characterized by previous studies, microbial community structure has not been.

Neo-coarctation after the arterial switch operation.

Neo-coarctation following arterial switch operation (ASO) for transposition of the great arteries (TGA) is a complication that is not regularly described, but may occur. We describe five patients who developed a neo-coarctation after operation. They were diagnosed with TGA, either with or without ventricular septal defect without signs or symptoms of a coarctation.

Corrected tetralogy of Fallot: comparison of tissue doppler imaging and velocity-encoded MR for assessment of performance and temporal activation of right ventricle.

Purpose: To compare velocity-encoded (VE) magnetic resonance (MR) imaging with tissue Doppler imaging to assess right ventricular (RV) peak systolic velocities and timing of velocities in patients with corrected tetralogy of Fallot and healthy subjects.

Materials And Methods: Local institutional review board approval was obtained; patients or their parents gave informed consent. Thirty-three patients (20 male, 13 female; median age, 12 years; interquartile range [IQR], 11-15 years; age range, 8-18 years) and 19 control subjects (12 male, seven female; median age, 14 years; IQR, 12-16 years; age range, 8-18 years) underwent VE MR imaging and tissue Doppler imaging.