Background Extensive myocardial calcification is a rare phenomenon typically associated with severe systemic illness. It may develop rapidly in critically ill patients following myocardial injury, particularly in the context of sepsis, vasoplegia, and profound metabolic derangements.Case Summary A 39-year-old man with poorly controlled type 1 diabetes mellitus presented in a comatose state due to severe diabetic ketoacidosis and hypothermia. Laboratory investigations revealed acute kidney injury, hypophosphataemia, and an elevated inflammatory profile. Initial imaging, including non-contrast CT, was unremarkable. During his ICU stay, he developed multi-organ failure with vasoplegia requiring high-dose vasopressors and transient cardiac dysfunction. On Day 14, he experienced an in-hospital cardiac arrest. Post-resuscitation CT unexpectedly revealed extensive calcification of the left ventricular myocardium, which had not been present on earlier imaging. Despite temporary haemodynamic stability, he suffered irreversible neurological injury and died on Day 19. Autopsy confirmed diffuse dystrophic myocardial calcifications without evidence of infarction or fibrosis.Discussion This case illustrates rapid-onset dystrophic myocardial calcification occurring in the setting of severe metabolic and inflammatory stress. Prolonged vasopressor exposure, acidosis, and systemic inflammation likely contributed to myocardial injury and subsequent calcium deposition. Awareness of this rare complication may improve diagnostic vigilance and support early prognostic discussions in similar patients.

AIMS:Left bundle branch block (LBBB) septal strain patterns are associated with left ventricular (LV) reverse remodelling after cardiac resynchronization therapy (CRT). However, their prognostic value and impact in patients undergoing CRT, as well as in CRT-eligible patients receiving conservative treatment, remains underexplored. This study aimed to validate the prognostic significance of LBBB strain patterns and elucidate the mechanisms underlying CRT response by evaluating their interaction with myocardial scar and clinical outcomes. METHODS AND RESULTS:In this multicentre study, 267 CRT patients underwent pre-implantation speckle-tracking strain analysis, with 155 also undergoing cardiac magnetic resonance imaging. CRT-treated patients were compared with 116 CRT-eligible conservatively treated patients. LBBB septal strain curves were categorized into five stages (LBBB-0 to LBBB-4). Endpoints included all-cause mortality, heart transplantation, and volumetric response in CRT recipients.CRT outcomes showed a stepwise improvement across LBBB stages in volumetric response (P < 0.001) and survival (log-rank P = 0.002). Myocardial scar, present in 52% of CRT patients, inversely correlated with LBBB stages (P = 0.003). After multivariable adjustment, LBBB stages independently predicted volumetric response (OR 2.30, P < 0.001) and survival (HR 0.64, P = 0.038), while scar burden did not. Survival benefits were greater in CRT-treated patients than in conservatively treated patients, ranging from HR 1.42 (P = 0.436) in LBBB-0 to HR 16.49 (P < 0.001) in LBBB-4. CONCLUSION:LBBB strain stages independently predict CRT outcomes, with lower stages associated with attenuated benefits, possibly due to higher scar burden. This classification provides a framework for understanding LBBB pathophysiology and CRT response.

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 first choice of treatment is a conservative treatment, consisting of exercises, skin care, lymph drainage and compression. Reconstructive lymphatic surgery is also often performed, that is, lymphovenous anastomoses, lymph node transfer or a combination. However, robust evidence on the effectiveness of reconstructive lymphatic surgery is missing. Therefore, the objective of this trial is to investigate the added value of reconstructive lymphatic surgery to the conservative treatment in patients with lymphoedema.Methods and analysis A multicentre randomised controlled and pragmatic trial was started in March 2022 in three Belgian university hospitals. 90 patients with arm lymphoedema and 90 patients with leg lymphoedema will be included. All patients are randomised between conservative treatment alone (control group) or conservative treatment with reconstructive lymphatic surgery (intervention group). Assessments are performed at baseline and at 1, 3, 6, 12, 18, 24 and 36 months. The primary outcome is lymphoedema-specific quality of life at 18 months. Key secondary outcomes are limb volume and duration of wearing the compression garment at 18 months. The approach of reconstructive lymphatic surgery is based on presurgical investigations including clinical examination, lymphofluoroscopy, lymphoscintigraphy, lymph MRI or CT angiography (if needed). All patients receive conservative treatment during 36 months, which is applied by the patient’s own physical therapist and by the patient self. From months 7 to 12, the hours a day of wearing the compression garment are gradually decreased.Ethics and dissemination The study has been approved by the ethical committees of University Hospitals Leuven, Ghent University Hospital and CHU UCL Namur. Results will be disseminated via peer-reviewed journals and presentations.Trial registration number NCT05064176

Figure 1 CT scan of the thorax showing the free mediastinal air (Panel A and B) as well as the air in the left atrium (Panel C and D).The arrows show the pathological collection of air.

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. Here, we evaluated T50 in PXE patients aiming to investigate its determinants and suitability as a potential biomarker for disease severity. Fifty-seven PXE patients were included in this cross-sectional study, and demographic, clinical, imaging and biochemical data were collected from medical health records. PXE severity was assessed using Phenodex scores. T50 was measured using a validated, nephelometry-based assay. Multivariate models were then created to investigate T50 determinants and associations with disease severity. In short, the mean age of patients was 45.2 years, 68.4% was female and mean serum T50 was 347 min. Multivariate regression analysis identified serum fetuin-A (p < 0.001), phosphorus (p = 0.007) and magnesium levels (p = 0.034) as significant determinants of T50, while no correlations were identified with serum calcium, eGFR, plasma PPi levels or the ABCC6 genotype. After correction for covariates, T50 was found to be an independent determinant of ocular (p = 0.013), vascular (p = 0.013) and overall disease severity (p = 0.016) in PXE. To conclude, shorter serum T50—indicative of a higher calcification propensity—was associated with a more severe phenotype in PXE patients. This study indicates, for the first time, that serum T50 might be a clinically relevant biomarker in PXE and may thus be of importance to future therapeutic trials.

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. The gold standard of aortic morphology was the definite diagnosis in the patient’s medical file, established by computed tomography, magnetic resonance, or surgery. Results: Computer tomographic (CT) studies of each aortic morphology were randomly evaluated for the presence of the hammock sign on coronal and sagittal images, by two readers blinded to the diagnosis. Sensitivity for detecting an anatomic bicuspid valve was 86%, and specificity was 100%. Conclusion: The hammock sign allows for a quick and easy diagnosis of an anatomical bicuspid aortic valve, merely by scrolling through the standard coronal reconstructions of any type of contrast-enhanced thoracic CT study, and regardless of any other findings associated with bicuspid aortic valve. Functional bicuspid aortic valves were not the scope of this study.

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. Case summary A 87-year-old patient with a history of rheumatic mitral stenosis and mitral valve replacement was admitted to the neurology department for vertigo. A stroke was suspected, and she underwent a transoesophageal echocardiogram (TOE) which was complicated by dysphagia. Oesophageal manometry and computed tomography revealed oesophagogastric junction outflow obstruction due to extrinsic compression by a giant left atrium (GLA). Discussion Dysphagia due to a GLA is rare. Various diagnostic criteria exist and the prevalence thus depends on which criterium is used. It is mostly encountered in rheumatic mitral disease, although there are reports of non-rheumatic aetiology. When the left atrium assumes giant proportions it can compress adjacent intrathoracic structures. Compression of the oesophagus can lead to dysphagia, as in our case. A TOE in these cases is relatively contraindicated and should only be performed if there is considerable reason to believe that it may change patient management.

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 (55.8% women, mean age 36.3 yr—range 13–70 yr–) and 40 age—and sex-matched healthy controls. Cardiac ultrasound, resting and ambulatory ECG (AECG) and NT-proBNP measurements were performed in all subjects at baseline. Additionally, patients with MFS underwent 2 extra evaluations during 30 ± 7 months follow-up. To study primary versus secondary myocardial involvement, patients with MFS were divided in 2 groups: without previous surgery and normal/mild valvular function (MFS-1; N = 55) and with previous surgery or valvular dysfunction (MFS-2; N = 31). Results Compared to controls, patients in MFS-1 showed mild myocardial disease reflected in a larger left ventricular end-diastolic diameter (LVEDD), lower TAPSE and higher amount of (supra) ventricular extrasystoles [(S)VES]. Patients in MFS-2 were more severely affected. Seven patients (five in MFS-2) presented decreased LV ejection fraction. Twenty patients (twelve in MFS-2) had non-sustained ventricular tachycardia (NSVT) in at least one AECG. Larger LVEDD and higher amount of VES were independently associated with NSVT. Conclusion Our study shows mild but significant myocardial involvement in patients with MFS. Patients with previous surgery or valvular dysfunction are more severely affected. Evaluation of myocardial function with echocardiography and AECG should be considered in all patients with MFS, especially in those with valvular disease and a history of cardiac surgery.

Aortic stiffness is an important diagnostic and prognostic parameter for many diseases, and is estimated by measuring the Pulse Wave Velocity (PWV) from Cardiac Magnetic Resonance (CMR) images. However, this process requires combinations of multiple sequences, which makes the acquisition long and processing tedious. We propose a method for aorta segmentation and centerline extraction from para-sagittal Phase-Contrast (PC) CMR images. The method uses the order of appearance of the blood flow in PC images to track the aortic centerline from the seed start position to the seed end position of the aorta. The only required user interaction involves selection of 2 input seed points for the start and end position of the aorta. We validate our results against the ground truth manually extracted centerlines from para-sagittal PC images and anatomical MR images. The resulting measurement values of both centerline length and PWV show high accuracy and low variability, which allows for use in clinical setting. The main advantage of our method is that it requires only velocity encoded PC image, while being able to process images encoded only in one direction.

Abstract 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). As control group, 13 women with BAV without TS and 48 healthy patients were included. Results Women with TS showed a higher AA-PWV (β = 1.08, confidence interval [CI]: 0.54–1.62) after correcting for age and comorbidities compared with controls. We found no significant difference in AAD and CF-PWV. In women with TS, the presence of BAV, coarctation of the aorta, or monosomy (45, X) was not associated with aortic stiffness. In addition, aortic tissue samples were investigated with routine and immunohistochemical stains in five additional women with TS who were operated. The tissue showed more compact smooth muscle cell layers with abnormal deposition and structure of elastin and diminished or absent expression of contractile proteins desmin, actin, and caldesmon, as well as the progesterone receptor. Conclusion Both aortic arch stiffness measurements on MRI and histomorphological changes point toward an inherent abnormal thoracic aortic wall in women with TS.

Van Schandevyl, Steven; Waterschoot, Robbe; De Vos, Nicolas; Devos, Daniel; Goethals, Ingeborg Author Information

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. The method segments the LAA, proposes the location for the occluder placement (a delineation plane between the left atrium and LAA) and calculates measurements needed for closure procedure planning. The method requires only two inputs from the user: a threshold value and a single seed point inside the LAA. Proposed location of the delineation plane can be intuitively corrected if necessary. Measurements are calculated from the segmented LAA according to the final delineation plane. RESULTS:Performance of the proposed method is validated on 17 CCTA images, manually segmented by two medical doctors. We achieve the average dice coefficient overlap of 92.52% and 91.63% against the ground truth segmentations. The average dice coefficient overlap between the two ground truth segmentations is 92.66%. Our proposed LAA orifice localization is evaluated against the desired location of the LAA orifice determined by the expert. The average distance between our proposed location and the desired location is 2.51 mm. CONCLUSION:Segmentation results show high correspondence to the ground truth segmentations. The occluder placement method shows high accuracy which indicates potential in clinical procedure planning.

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. We conclude that every young patient with suspected alcoholic liver disease and signs of iron overload should have a testing to rule out other iron overloading pathology, since early recognition and treatment with phlebotomy may prevent organ damage and improve life expectancy.

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. For the validation of the API method, a pulsatile in vitro cardiac phantom was used.RESULTS:Indices of MR severity, such as left ventricular and atrial dimension, pulmonary arterial pressure, significantly cosegregate with API severity (p≤0.002). The API method showed a linear correlation with colour Doppler (r=0.79), VCW (r=0.68), PISA-effective regurgitant orifice area (r=0.72) and PISA-regurgitant volume (r=0.67); p<0.001 for all. The API was significantly more applicable than VCW (95% vs 75% of all patients; p<0.001) and PISA-based methods (65%; p<0.001). Additionally, the API showed a stronger intraobserver and interobserver agreement compared with other methods. Finally, in the in vitro validation, API values showed a strong linear correlation with increasing regurgitant volumes (r=0.81; p<0.001).CONCLUSIONS:We showed the clinical feasibility and in vitro validation of a novel digital quantitative echocardiographic method to grade MR severity. This method is more applicable and has less interobserver and intraobserver variability compared with current quantitative methods.

A 16-year-old male patient was hit by a car during a bicycle ride. Because of extensive subcutaneous emphysema and haemodynamic instability at the emergency department, urgent thoracotomy was perfo...

Spontaneous coronary artery dissection or SCAD is a rare and challenging disease that is increasingly diagnosed. It is characterized by a non-traumatic, non-iatrogenic separation of the coronary artery wall and occurs predominantly in young and middle-aged women without traditional cardiovascular risk factors. SCAD is often associated with predisposing conditions such as the peripartum period, systemic inflammatory disease and heritable connective tissue disease. More recently, independent investigators have demonstrated an important association with fibromuscular dysplasia. Extreme emotional or physical stress as well as intense hormonal therapy or drug abuse have been pointed out as precipitating factors. The diagnosis of SCAD can be challenging and starts with clinical suspicion. Advanced imaging techniques such as intravascular ultrasound and optical coherence tomography are useful for the differentiation from atherosclerotic disease and are increasingly used for this indication. The proposed treatment in the acute setting is based on findings from single-centre retrospective series: in stable patients with a TIMI-flow ≥2 a conservative management is proposed because of the high risk of procedural failure and complications as well as a high probability of spontaneous healing. Long-term treatment is comparable to that in non-SCAD acute coronary syndromes (ACS) but dual antiplatelet therapy should only be started in case of stenting and should be kept as short as possible in patients with vascular Ehlers-Danlos syndrome. Prognosis seems to be better compared to non-SCAD ACS but there is a reasonable risk of recurrence. In this review, we discuss the current knowledge of SCAD and provide a clinical pathway for the diagnosis, management and work-up of SCAD patients.

This thesis recounts the work that has been done on aortic stiffness in our institution where cardiovascular radiology has played a central role. The papers comprised in this thesis represent different steps on the learning curve ultimately leading to the practical application of MR PVW in a specific population of Turner patients. We have scanned 100 normal individuals in a wide range of ages for normal values. We have used MRI as an anatomical reference to finetune the carotid-tofemoral PWV technique algorithm; we have developed a more integrated software solution for postprocessing; and we have investigated our Hospital’s Turner population with MRI PWV. The clinical part of this thesis aims to, both morphologically and functionally, study the entire aorta in a segmental way in Turner patients, and estimate the clinical impact of this measurement for Turner patients.

Supplemental Table S2 A, B and C. F and p values of PWV, aortic area and aortic distensibility in a multivariate GLM including age, height, weight, heart rate and blood pressure as confounders, performed seperately within each of three groups: TAV controls (S2A), TAV Turner patients (S2B) and BAV Turner patients (S2C). (XLSX 17 kb)