Evaluation of a nurse-led multi-component transition program for adolescents with congenital heart disease.

Objective: To evaluate the effectiveness of the transition program for adolescents with congenital heart disease (CHD) 'Transition With a Heart' (TWAH) on disease-related knowledge, quality of life (QoL), transition experiences, and gaps in follow-up.

Methods: A study with pre-posttest and control group (post-test) using consecutive sampling, including adolescents with moderate to severely complex CHD, without intellectual disability, aged≥ 12 y, and parents. After weighting, t-tests were performed.

Long-term follow-up of atrioventricular valve function in Fontan patients: effect of atrioventricular valve surgery.

Objectives: The aim of this study was to evaluate the relationship between atrioventricular valve and ventricular function in Fontan survivors, including the effect of atrioventricular valve surgery.

Methods: Analysis focused on transplant-free survival and the need for atrioventricular valve surgery in single ventricle patients after Fontan completion. Longitudinal echocardiographic examination of long-term valve and ventricular function was performed.

Autonomic symptoms and associated factors in patients with chronic heart failure.

Objective: Autonomic disorders are common in chronic illness, and their symptoms may restrict the daily functioning of patients. However, in chronic heart failure, extensive knowledge about autonomic symptoms is still lacking. This study aims to explore self-perceived autonomic symptoms, associated factors, and their relationship with health-related quality of life in chronic heart failure.

QRS Duration During Follow-Up of Tetralogy of Fallot: How Valuable is it? Analysis of ECG Changes in Relation to Pulmonary Valve Implantation.

Long-term results after tetralogy of Fallot (TOF) repair are determined by the extent of right ventricular remodeling to chronic pulmonary regurgitation entailing progressive RV dysfunction and a risk of developing ventricular arrhythmia. Pulmonary valve replacement (PVR) can alleviate this burden. As a predictor of ventricular arrhythmia, QRS duration remains a strong parameter in this decision.

ACARPA, a rare case of ALCAPA.

An anomalous circumflex coronary artery arising from the right pulmonary artery is very rare. Clinical presentation varies from absence of symptoms to arrhythmia and sudden cardiac death. Since prevalence is very low, no definite therapy has been delineated yet.

A new dimension in patent foramen ovale size estimation.

Background: Detailed multidimensional assessment of patent foramen ovale (PFO) size with transesophageal echocardiography (TOE) may help to determine PFO pathogenicity in cryptogenic stroke patients. We explored the potential additive value of Live xPlane and three-dimensional (3D) TOE anatomical PFO sizing techniques.

Methods: Imaging data of 45 patients who underwent a 3D TOE-assisted percutaneous PFO closure were studied.

Development of a transition program for adolescents with congenital heart disease.

Thanks to advances in care, most children with congenital heart disease nowadays survive into adulthood. The majority of patients remain at high risk for future complications. Hence, life-long follow-up is mandatory.

Disproportion and dysmorphism in an adult Belgian population with Turner syndrome: risk factors for chronic diseases?

Background: Turner syndrome (TS) is characterized by dysmorphism and body disproportion. TS women are also susceptible to a range of chronic disorders including arterial hypertension (AHT), osteoporosis, sensorineural hearing loss (SNHL), type 2 diabetes mellitus (DM2) and thyroid disease. The association between dysmorphism/body disproportion and chronic disease has never been studied in TS women.

Body mass index in adults with congenital heart disease.

Objective: To investigate the status of body mass index (BMI) in adult people with congenital heart disease (ACHD).

Methods: Five hundred thirty-nine adults with CHD (53.8% men) were seen in the outpatient clinic from 2013 to 2015 and compared to a reference population (n = 1737).

Partial anomalous pulmonary venous return in Turner syndrome.

Purpose: The aim of this study is to describe the prevalence, anatomy, associations and clinical impact of partial anomalous pulmonary venous return in patients with Turner syndrome.

Methods And Results: All Turner patients who presented at our Turner clinic, between January 2007 and October 2015 were included in this study and underwent ECG, echocardiography and advanced imaging such as cardiac magnetic resonance or computed tomography as part of their regular clinical workup. All imaging was re-evaluated and detailed anatomy was described.

How to Size ASDs for Percutaneous Closure.

Percutaneous closure is the treatment of choice for secundum-type atrial septal defects (ASD). Balloon sizing (BS) has been the method of choice for deciding on device size. Improved 2D- and 3D-transesophageal echocardiographic (TEE) imaging challenged the necessity of BS.

Proximal aortic stiffening in Turner patients may be present before dilation can be detected: a segmental functional MRI study.

Background: To study segmental structural and functional aortic properties in Turner syndrome (TS) patients. Aortic abnormalities contribute to increased morbidity and mortality of women with Turner syndrome. Cardiovascular magnetic resonance (CMR) allows segmental study of aortic elastic properties.

Adverse outcome of coarctation stenting in patients with Turner syndrome.

Objectives: This study examines the outcome and procedural outcomes of percutaneous stent angioplasty for aortic coarctation in patients with Turner syndrome (TS).

Background: TS occurs in 1 in 2,500 live-born females and is associated with aortic coarctation.

Methods: In this multicenter, retrospective cohort study, all patients with TS and a coarctation of the aorta, treated with percutaneous stent implantation were included.

Aggressive extensive cardiac mass in an HIV-1-infected patient: should we go for comfort therapy?

Cardiac masses are rare, the differential diagnosis includes infections with vegetations or abscesses, neoplasms, thrombi, and structural abnormalities. A pathology specimen is essential in therapeutic strategy planning for a cardiac mass, also if the primary imaging findings look dramatic at the start. Even in an inoperable setting, a life-saving therapy might be available.

Arterial hypertension in Turner syndrome: a review of the literature and a practical approach for diagnosis and treatment.

Turner syndrome is a rare chromosomal disorder with complete or partial absence of one X chromosome that only occurs in women. Clinical presentation is variable, but congenital and acquired cardiovascular diseases are frequently associated diseases that add significantly to the increased morbidity and mortality in Turner syndrome patients. Arterial hypertension is reported in 13-58% of adult Turner syndrome patients and confers an increased risk for stroke and aortic dissection.

Reference values for echocardiographic assessment of the diameter of the aortic root and ascending aorta spanning all age categories.

Thoracic aortic dilatation requires accurate and timely detection to prevent progression to thoracic aortic aneurysm and aortic dissection. The detection of thoracic aortic dilatation necessitates the availability of cut-off values for normal aortic diameters. Tools to evaluate aortic dimension above the root are scarce and inconsistent regarding age groups.

Abnormal aortic arch morphology in Turner syndrome patients is a risk factor for hypertension.

Hypertension in Turner syndrome (TS) is a multifactorial, highly prevalent and significant problem that warrants timely diagnosis and rigorous treatment. The objective of this study was to investigate the association between abnormal aortic arch morphology and hypertension in adult TS patients. This was a single centre retrospective study in 74 adult TS patients (age 29.

Functional analysis of the anatomical right ventricular components: should assessment of right ventricular function after repair of tetralogy of Fallot be refined?

Objective: Follow-up after tetralogy of Fallot (ToF) repair is directed to detect timely right ventricular (RV) dysfunction by following pulmonary regurgitation and global RV size, with little attention for the effective contribution of regional RV dysfunction. This study investigates the contribution of regional RV dysfunction on exercise capacity after ToF repair.

Methods: Forty-two patients were investigated with cardiac magnetic resonance imaging for regional RV dysfunction in relation to global RV function by functional quantification of the sinus and outflow part of the RV.