Aortic Dilatation in Children and Young People With ADPKD.

Introduction: Aortic root dilatation is a reported cardiovascular sequela seen in children and young people (CYP) with chronic kidney disease (CKD) but has yet to be described in those with autosomal dominant polycystic kidney disease (ADPKD).

Methods: Single center, cross-sectional study in a dedicated ADPKD clinic. Echocardiograms were evaluated for the presence of dilatation (defined by a z-score ≥2 [≥99th percentile] SDs from the mean) at 4 standardized locations, namely the aortic valve annulus, sinuses of Valsalva (SoV), sinotubular junction (STJ), and the ascending aorta.

Prevalence of cardiac valvar abnormalities in children and young people with autosomal dominant polycystic kidney disease.

Background: Valvar abnormalities in children and adults with autosomal dominant polycystic kidney disease (ADPKD) have previously been reported as a frequent occurrence. Mitral valve prolapse (MVP), in particular, has been reported in almost one-third of adult patients and nearly 12% of children with ADPKD. Our objective in this study was to establish the prevalence of valvar abnormalities in a large, contemporary series of children and young people (CYP) with ADPKD.

Transcatheter creation of a pulmonary artery to left atrial fenestration in a failing Fontan circulation using the Atrial Flow Regulator (AFR).

Transcatheter creation of an interatrial communication using the Occlutech Atrial Flow Regulator Device for pulmonary hypertension or heart failure is well described. We report a case of an 8-year-old boy with a failing Fontan circulation, in whom the Atrial Flow Regulator was used to successfully create a fenestration between the pulmonary artery and left atrium, improving his clinical condition.

Silent and dangerous: catheter-associated right atrial thrombus (CRAT) in children on chronic haemodialysis.

Background: Catheter-associated right atrial thrombus (CRAT) is a recognised complication of central venous catheter (CVC) use for haemodialysis (HD) patients.

Methods: This was a single-centre retrospective longitudinal observational study of consecutive children aged 6 months-18 years over a 7-year period receiving in-centre chronic HD. Echocardiograms as per routine cardiac surveillance were performed 6 months or earlier given clinical concerns.

Central blood pressure and measures of early vascular disease in children with ADPKD.

Background: There is growing recognition of hypertension in a significant proportion of children with ADPKD. In this study, we assessed blood pressure and cardiovascular status in children with ADPKD.

Methods: A prospective two-centre observational study of children (< 18 years) with ADPKD was compared against age- and BMI-matched healthy controls.

Echocardiographic approach to catheter closure of atrial septal defects: patient selection, procedural guidance and post-procedural checks.

Echocardiography is the imaging modality of choice to diagnose different types of atrial septal defect and to determine which defects are suitable for catheter occlusion. In addition to assessment of defect size and rims, transoesophageal echocardiography may be used to guide the procedure itself including device placement, procedural complications and post-procedural checks. This review covers a practical approach to this subject and is accompanied by online videos illustrating the technique.

Electrocardiography is a poor screening test to detect left ventricular hypertrophy in children.

Background: Detection of left ventricular hypertrophy (LVH) is clinically important because it can be predictive of adverse clinical outcome. However, the best method for detecting LVH in clinical practice is unclear. The aim of this study was to evaluate electrocardiography (ECG) compared with echocardiography (ECHO) as a screening test to detect LVH in a high risk population.

Incidence of left ventricular hypertrophy in children with kidney disease: impact of method of indexation of left ventricular mass.

Aims: Patients with chronic kidney disease are at high risk of cardiovascular morbidity and mortality. Increased left ventricular mass (LVM) has been shown to be an adverse prognostic factor. LVM may be indexed for body size by different methods related to height, weight, or body surface area (BSA).

An echocardiographic study of tetralogy of Fallot in the fetus and infant.

Objective: To document the echocardiographic features of tetralogy of Fallot during fetal and postnatal life. Correlation of echocardiographic findings with the requirement for early intervention prior to definitive repair.

Design: Retrospective observational study.