Clinical Care for Cardiovascular Disease in Patients With Williams-Beuren Syndrome.

Williams-Beuren syndrome (WBS) is a congenital multisystem disorder affecting the cardiovascular, central nervous, and musculoskeletal systems. Cardiovascular abnormalities, which consist principally of vascular stenoses, occur in approximately 80% of people with WBS and are the predominant cause of early morbidity and mortality. Supravalvar aortic stenosis and peripheral pulmonary artery stenosis are the most common stenotic lesions in WBS, though other stenoses often occur, including stenoses of the coronary arteries.

[Fetal ECG and arrhythmias].

The normal fetal heart rate ranges between 110 und 180 beats per minute (bpm). Intrauterine arrhythmias are not an uncommon finding. Fetal echocardiography (ECG) allows for correct diagnosis of the arrhythmia, which is prerequisite for decision making and treatment.

Insufficiency of a Damus-Kaye-Stansel anastomosis in a Fontan patient: Transfemoral implantation of an Edwards Sapien 3 valve.

We present a 22-year-old patient with a univentricular heart who had already undergone five open heart surgeries including a Damus-Kaye-Stansel procedure, Fontan completion and tricuspid valve replacement. In addition, epimyocardial pacemaker implantation and repeated revisions had been necessary. He developed symptomatic free regurgitation of the pulmonary portion of his DKS anastomosis.

Quantification of pulmonary regurgitation and prediction of pulmonary valve replacement by echocardiography in patients with congenital heart defects in comparison to cardiac magnetic resonance imaging.

Pulmonary regurgitation (PR) is common in patients with congenital heart defects (CHD) and contributes to morbidity and mortality in the long-term. We investigated in this retrospective analysis whether readily accessible echocardiographic parameters are useful for quantification of PR and for predicting pulmonary valve replacement (PVR) in comparison to the gold-standard phase contrast (PC) flow measurements from cardiovascular magnetic resonance (CMR). Continuous wave (CW) Doppler and colour flow images in echocardiograms from 53 patients with CHD were analysed.

Midterm results after arterial switch operation for transposition of the great arteries: a single centre experience.

Background: The arterial switch operation (ASO) has become the surgical approach of choice for d-transposition of the great arteries (d-TGA). There is, however an increased incidence of midterm and longterm adverse sequelae in some survivors. In order to evaluate operative risk and midterm outcome in this population, we reviewed patients who underwent ASO for TGA at our centre.

Catheter ablation of idiopathic left and right ventricular tachycardias in the pediatric population using noncontact mapping.

Background: Idiopathic ventricular tachycardia (VT) in children with a structurally normal heart can cause significant morbidity, and although rare, mortality. Conventional activation and pace mapping may be limited by nonsustained tachycardia or unstable hemodynamics.

Objective: The aim of this study was to assess feasibility of catheter ablation of idiopathic VT in the pediatric population guided by noncontact mapping.

Incidence of coronary artery injury immediately after catheter ablation for supraventricular tachycardias in infants and children.

Background: Several case reports have documented coronary artery stenosis or even occlusion after radiofrequency energy ablation (RFA) in children and adults. Coronary involvement early and late after RFA has also been described in animal models.

Objective: The purpose of this study was to assess prospectively the incidence of coronary artery injury early after catheter ablation for supraventricular tachycardias (SVT) in children.

Management of emergencies in adults with congenital cardiac disease.

The aim of the study was to assess the quantity and nature of emergencies affecting adults with congenital cardiac disease (CCD) and evaluate infrastructural requirements for adequate management. There is an increasing number of adults with CCD requiring specialized complex care. This multicenter study evaluated all emergency admissions to 1 of 5 centers for adults with CCD within 1 year.

Normality of cardiopulmonary capacity in children operated on to correct congenital heart defects.

Background: One of the main goals of corrective surgery of congenital heart defects in children is the improvement of quality of life, which in young children is predominantly determined by exercise capacity. It is not known whether this goal can be achieved in school-aged children who have undergone cardiac surgery in infancy.

Objectives: To determine if primary school-aged children who underwent surgery to correct congenital heart defects in infancy are physically as fit as their peers.

Prognostic value of lymphatic and blood vessel invasion in neuroendocrine tumors of the lung.

Few data on the influence of vessel invasion on the progression of neuroendocrine lung tumors are available. Because of the lack of specific markers, previous studies could not reliably discriminate lymphatic and blood vessels. By immunostaining for podoplanin, specific for lymphatic endothelium, and CD34 antigen, we assessed lymphatic and blood vessel invasion in 120 tissue specimens of patients with neuroendocrine lung tumors.

Risk of sudden death in the Williams-Beuren syndrome.

Williams-Beuren syndrome (WBS) is a genetic disorder characterized by a distinctive facial gestalt, mental retardation, mild growth deficiency, and cardiovascular disease. The occurrence of sudden death in the WBS is known from several case reports, but information about the risk of sudden death as derived from the data of a large cohort of patients is lacking. We analyzed the data of 293 WBS patients who had been treated for 43 years at the same two institutions.