SurLym trial: study protocol for a multicentre pragmatic randomised controlled trial on the added value of reconstructive lymphatic surgery to decongestive lymphatic therapy for the treatment of lymphoedema.

Introduction: Lymphoedema is a chronic condition caused by lymphatic insufficiency. It leads to swelling of the limb/midline region and an increased risk of infection. Lymphoedema is often associated with mental and physical problems limiting quality of life.

The Hammock Sign in Computed Tomography as a Detection Aid for Bicuspid Aortic Valves.

Introduction: Bicuspid aortic valve is difficult to detect on standard transverse images.

Purpose: We aimed to investigate the usefulness of the hammock sign for detection of bicuspid aortic valve.

Methods: We retrospectively investigated the usefulness of a newly proposed 'hammock sign' in a population of 45 contrast enhanced computer tomographic studies to discern tricuspid (22) from anatomical bicuspid aortic (23) valves.

Serum Calcification Propensity T50 Associates with Disease Severity in Patients with Pseudoxanthoma Elasticum.

Pseudoxanthoma elasticum (PXE) is a currently intractable genetic disorder characterized by progressive ectopic calcification in the skin, eyes and arteries. Therapeutic trials in PXE are severely hampered by the lack of reliable biomarkers. Serum calcification propensity T50 is a blood test measuring the functional anticalcifying buffer capacity of serum.

A case report: cardiac dysphagia-a ghost of the past?

Background: Rheumatic heart disease has become rare in developed countries and physicians have grown unfamiliar with the disease and its clinical course. The mitral valve is most commonly affected leading to mitral regurgitation and/or stenosis. The chronic volume and/or pressure overload leads to atrial remodelling and enlargement, driving the development of atrial fibrillation and thrombo-embolic events.

Abnormal Aortic Wall Properties in Women with Turner Syndrome.

Background:  Turner syndrome (TS) is associated with aortic dilatation and dissection, but the underlying process is unclear. The aim of this study was to investigate the elastic properties and composition of the aortic wall in women with TS.

Methods:  In this cross-sectional study, 52 women with TS aged 35 ± 13 years (50% monosomy, 12 with bicuspid aortic valve [BAV] and 4 with coarctation) were investigated using carotid-femoral pulse wave velocity (CF-PWV) by echocardiography and ascending aortic distensibility (AAD) and aortic arch pulse wave velocity (AA-PWV) by magnetic resonance imaging (MRI).

Myocardial disease and ventricular arrhythmia in Marfan syndrome: a prospective study.

Background: Aortic root dilatation and-dissection and mitral valve prolapse are established cardiovascular manifestations in Marfan syndrome (MFS). Heart failure and arrhythmic sudden cardiac death have emerged as additional causes of morbidity and mortality.

Methods: To characterize myocardial dysfunction and arrhythmia in MFS we conducted a prospective longitudinal case-control study including 86 patients with MFS (55.

Left atrial appendage segmentation from 3D CCTA images for occluder placement procedure.

Background: Percutaneous left atrial appendage (LAA) closure (placement of an occluder to close the appendage) is a novel procedure for stroke prevention in patients suffering from atrial fibrillation. The closure procedure planning requires accurate LAA measurements which can be obtained from computed tomography (CT) images.

Method: We propose a novel semi-automatic LAA segmentation method from 3D coronary CT angiography (CCTA) images.

An unexpected cause of liver cirrhosis and cardiomyopathy in a young man.

Introduction Juvenile hemochromatosis is a rare but severe form of hereditary hemochromatosis that typically presents early in life and can be fatal if left untreated. Case presentation We present the case of a 30-year-old man with a clear symptomatology of juvenile hemochromatosis, but in whom the diagnosis was initially mistaken for alcoholic liver disease because of known excessive use of alcohol, with the consequence that an adequate treatment was postponed. Discussion In this report, we discuss the diagnosis and treatment of juvenile hemochromatosis, focusing on the interaction between hemochromatosis and alcohol induced liver disease and how to differentiate both.

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.

Efficacy of losartan as add-on therapy to prevent aortic growth and ventricular dysfunction in patients with Marfan syndrome: a randomized, double-blind clinical trial.

Background: Marfan syndrome (MFS) is a multisystemic hereditary connective tissue disease. Aortic root aneurysms and dissections are the most common and life-threatening cardiovascular disorders affecting these patients. Other cardiac manifestations include mitral valve prolapse, ventricular dysfunction and arrhythmias.

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.

Grading of mitral regurgitation based on intensity analysis of the continuous wave Doppler signal.

Objectives: Echocardiographic methods are used to quantify mitral regurgitation (MR) severity; however, their applicability, accuracy and reproducibility have been debated. We aimed to develop and validate a novel custom-made transthoracic echocardiographic method for grading MR severity based on average pixel intensity (API) analysis of the continuous wave (CW) Doppler envelope.

Methods: MR was assessed in 290 patients using API, colour Doppler imaging, vena contracta width (VCW) and proximal iso-velocity surface area (PISA) method.

Increased aortic stiffness in prepubertal girls with Turner syndrome.

Background: Aortic dilation and dissection contribute highly to the increased mortality of Turner syndrome (TS) but the exact pathophysiology is not completely understood.

Design: Prospective case - control study.

Methods: 15 prepubertal TS girls (median age 10.

Quadriscupid Aortic Valve with Concurrent Aortic Stenosis and Insufficiency.

We present the case of a 22-year-old man with a congenital mixed aortic valve dysfunction who underwent cardiac Magnetic Resonance Imaging (MRI) for the assessment of aortic valve morphology and function prior to valve replacement. Cardiac MRI showed a four-leaf-clover aortic valve morphology, the typical presentation of a quadricuspid aortic valve. The patient underwent a successful Bentall procedure to replace the aortic valve, aortic root and ascending aorta.

Organ Doses and Radiation Risk of Computed Tomographic Coronary Angiography in a Clinical Patient Population: How Do Low-Dose Acquisition Modes Compare?

Objective: To compare the organ doses and lifetime-attributable risk of cancer for electrocardiogram-triggered sequential and high-pitch helical scanning in a clinical patient population.

Methods: Phantom thermoluminiscence dosimeter measurements were used as a model for the organ dose assessment of 314 individual patients who underwent coronary computed tomographic angiography. Patient-specific lifetime-attributable cancer risks were calculated.

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.

MR pulse wave velocity increases with age faster in the thoracic aorta than in the abdominal aorta.

Purpose: To assess the difference between thoracic and abdominal aortic pulse wave velocity (PWV) in apparently healthy subjects including young adults to elderly subjects.

Materials And Methods: We performed PWV and distensibility measurements and analysis of thoracic and abdominal aortic segments in 96 apparently normal subjects aged 20-80 years with magnetic resonance (MR). Both unadjusted correlation and General Linear Model (GLM) analysis of log-transformed PWV (thoracic and abdominal aorta) and distensibility (four aortic cross-sections) were performed.

Robust segmentation methods with an application to aortic pulse wave velocity calculation.

Aortic stiffness has proven to be an important diagnostic and prognostic factor of many cardiovascular diseases, as well as an estimate of overall cardiovascular health. Pulse wave velocity (PWV) represents a good measure of the aortic stiffness, while the aortic distensibility is used as an aortic elasticity index. Obtaining the PWV and the aortic distensibility from magnetic resonance imaging (MRI) data requires diverse segmentation tasks, namely the extraction of the aortic center line and the segmentation of aortic regions, combined with signal processing methods for the analysis of the pulse wave.

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.

Noninvasive assessment of carotid-femoral pulse wave velocity: the influence of body side and body contours.

Background: Recently, an expert group advised to measure carotid-femoral (cf) pulse wave velocity (PWV) on the right side of the body, and to use a sliding caliper when tape measure distance cannot be obtained in a straight line. The present study investigates the evidence for this advice by comparing the real travelled cf path lengths (RTPLs) at both body sides and comparing the straight distance (as can be obtained with a sliding caliper) with the tape measure distance.

Methods: RTPLs were measured with MRI in 98 individuals (49 men, age 21-76 years).