Usefulness of exercise-induced hypertension as predictor of chronic hypertension in adults after operative therapy for aortic isthmic coarctation in childhood.

Chronic hypertension is a major concern in adults who have undergone resection of coarctation of the aorta (CoA) in childhood. In otherwise healthy subjects, exercise-induced hypertension is prognostic for chronic hypertension; however, the prognostic value in patients with CoA remains unknown. The aim of the present study was to evaluate the predictive value of exercise-induced hypertension for chronic hypertension in these patients.

The perspective of patients with congenital heart disease: does health care meet their needs?

Objective: A first step in the delivery of tailored care is answering the following question: does health care meet the needs of patients? Therefore patients' perspective on health care use and their needs was examined. The design used was cross-sectional questionnaire study.

Patients: A total of 1109 adult congenital heart defect (CHD) patients attending one of eight Dutch hospitals were randomly selected from a national database (10% of all registered patients).

Rationale and design of a trial on the role of bosentan in Fontan patients: improvement of exercise capacity?

Background: The Fontan circulation is a palliative procedure performed in patients with complex congenital heart disease (CHD), making transpulmonary blood flow dependent on the systemic venous pressure. In a Fontan circulation a low pulmonary vascular resistance (PVR) is crucial, as is epitomized by the observation that a high PVR is a strong predictor of mortality. Long-term follow-up has shown that PVR may rise many years after the Fontan procedure has been performed, possibly due to micro-emboli from a dilated right atrium or from the venous system.

Size and shape of excluded volume polymers confined between parallel plates.

A number of recent experiments have provided detailed observations of the configurations of long DNA strands under nano-to-micrometer-sized confinement. We therefore revisit the problem of an excluded volume polymer chain confined between two parallel plates with varying plate separation. We show that the nonmonotonic behavior of the overall size of the chain as a function of plate separation, seen in computer simulations and reproduced by earlier theories, can already be predicted on the basis of scaling arguments.

Bicuspid stenotic aortic valves: clinical characteristics and morphological assessment using MRI and echocardiography.

BACKGROUND: Bicuspid aortic valve (BAV) is one of the most common congenital heart defects with a population prevalence of 0.5% to 1.3%.

Treatment of congenital heart disease: risk-reducing measures in young adults.

Adults with congenital heart disease form a new and relatively young population, since surgical treatment of heart defects became available three to four decades ago. Owing to improved survival this population is steadily growing in number and age. Little is known regarding long-term survival; however, late complications occur frequently.

WITHDRAWN: Predictors of long-term survival in pulmonary hypertension treated with bosentan.

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A novel autosomal dominant condition consisting of congenital heart defects and low atrial rhythm maps to chromosome 9q.

Congenital heart defects (CHDs) occur mostly sporadic, but familial CHD cases have been reported. Mutations in several genes, including NKX2.5, GATA4 and NOTCH1, were identified in families and patients with CHD, but the mechanisms underlying CHD are largely unknown.

'The sedentary heart': physical inactivity is associated with cardiac atrophy in adults with an intellectual disability.

Background: The cardiac muscle is well regulated in response to changes in loading conditions. This cardiac plasticity has been studied intensively and is well known in trained athletes. Conversely, the mechanisms leading to the opposite response are less clear.

Critical appraisal of the revised Ghent criteria for diagnosis of Marfan syndrome.

Marfan syndrome (MFS) is a connective tissue disorder with major features in cardiovascular, ocular and skeletal systems. Recently, diagnostic criteria were revised where more weight was given to the aortic root dilatation. We applied the revised Marfan nosology in an established adult Marfan population to define practical repercussions of novel criteria for clinical practice and individual patients.

Pregnancy in women with corrected tetralogy of Fallot: occurrence and predictors of adverse events.

Background: In women with corrected tetralogy of Fallot (ToF), pregnancy is associated with maternal cardiac, obstetric, and offspring complications. Our aim is to investigate the magnitude and determinants of pregnancy outcome in women with corrected ToF.

Methods: In this retrospective international multicenter study using 2 congenital heart disease registries, 204 women with corrected ToF were identified.

Associations between cardiovascular parameters and uteroplacental Doppler (blood) flow patterns during pregnancy in women with congenital heart disease: Rationale and design of the Zwangerschap bij Aangeboren Hartafwijking (ZAHARA) II study.

Background: Previous research has shown that women with congenital heart disease (CHD) are more susceptible to cardiovascular, obstetric, and offspring events. The causative pathophysiologic mechanisms are incompletely understood. Inadequate uteroplacental circulation is an important denominator in adverse obstetric events and offspring outcome.

ALK2 mutation in a patient with Down's syndrome and a congenital heart defect.

Down's syndrome (DS), resulting from an additional copy of chromosome 21 (trisomy 21), is frequently associated with congenital heart defects (CHDs). Although the increased dosage of chromosome 21 sequences is likely to be part of the etiology of cardiac defects, only a proportion of DS patients exhibit a congenital heart defect (birth prevalence 40-60%). Through a large-candidate gene-sequencing screen in patients with atrioventricular septal defects, substitutions were identified in bone morphogenetic protein (BMP) type I receptor ALK2 and two other genes in a patient with DS and a primum-type atrial septal defect.

Turning 18 with congenital heart disease: prediction of infective endocarditis based on a large population.

Aims: The risk of infective endocarditis (IE) in adults with congenital heart disease is known to be increased, yet empirical risk estimates are lacking. We sought to predict the occurrence of IE in patients with congenital heart disease at the transition from childhood into adulthood.

Methods And Results: We identified patients from the CONCOR national registry for adults with congenital heart disease.

Mutations in the sarcomere gene MYH7 in Ebstein anomaly.

Background: Ebstein anomaly is a rare congenital heart malformation characterized by adherence of the septal and posterior leaflets of the tricuspid valve to the underlying myocardium. An association between Ebstein anomaly with left ventricular noncompaction (LVNC) and mutations in MYH7 encoding β-myosin heavy chain has been shown; in this report, we have screened for MYH7 mutations in a cohort of probands with Ebstein anomaly in a large population-based study.

Methods And Results: Mutational analysis in a cohort of 141 unrelated probands with Ebstein anomaly was performed by next-generation sequencing and direct DNA sequencing of MYH7.

Changing demographics of pulmonary arterial hypertension in congenital heart disease.

Pulmonary arterial hypertension (PAH) is a serious complication of congenital heart disease (CHD). Without early surgical repair, around one-third of paediatric CHD patients develop significant PAH. Recent data from the Netherlands suggest that >4% of adult CHD patients have PAH, with higher rates in those with septal defects.

Interventional and surgical treatment of cardiac arrhythmias in adults with congenital heart disease.

Arrhythmias are a major cause of morbidity, mortality and hospital admission in adults with congenital heart disease (CHD). The etiology of arrhythmias in this population is often multifactorial and includes electrical disturbances as part of the underlying defect, surgical intervention or hemodynamic abnormalities. Despite the numerous existing arrhythmia management tools including drug therapy, pacing and ablation, management of arrhythmias in adults with CHD remains difficult and challenging.

Ventricular response to stress predicts outcome in adult patients with a systemic right ventricle.

Background: Previous studies demonstrated that ventricular response to stress cardiovascular magnetic resonance (CMR) is frequently abnormal in patients with a systemic right ventricle (RV). However, the clinical implications of these findings remained unknown. We sought to evaluate whether abnormal response to stress CMR predicts adverse outcome in patients with a systemic RV.

Rationale and design of a trial on the effect of angiotensin II receptor blockers on the function of the systemic right ventricle.

Background: Angiotensin II receptor blockers have been proven to be beneficial in left ventricular failure. In patients with a morphologic right ventricle supporting the systemic circulation, its efficacy has not yet been established.

Methods: We designed a multicenter, prospective, randomized, double-blind, placebo-controlled trial studying the effect of valsartan in patients with a systemic right ventricle due to a congenitally or surgically corrected transposition of the great arteries.

The changing epidemiology of congenital heart disease.

Congenital heart disease is the most common congenital disorder in newborns. Advances in cardiovascular medicine and surgery have enabled most patients to reach adulthood. Unfortunately, prolonged survival has been achieved at a cost, as many patients suffer late complications, of which heart failure and arrhythmias are the most prominent.

Human immune responses that reduce the transmission of Plasmodium falciparum in African populations.

Malaria-infected individuals can develop antibodies which reduce the infectiousness of Plasmodium gametocytes to biting Anopheles mosquitoes. When ingested in a bloodmeal together with gametocytes, these antibodies reduce or prevent subsequent parasite maturation in the insect host. This transmission-blocking immunity is usually measured in human sera by testing its effect on the infectivity of gametocytes grown in vitro.