[Sinus node dysfunction associated with cardiac sarcoidosis: A case report]. / Dysfonction sinusale au cours de la sarcoïdose cardiaque: à propos d'un cas.

INTRODUCTION: Sarcoidosis is a multisystemic granulomatous disease of unknown cause occurring in young adults. Cardiac sarcoidosis patients are at increased risk for atrioventricular blocks and ventricular arrhythmias. Sinus node dysfunction is scarcely reported. OBSERVATION: We report a case of cardiac sarcoidosis revealed by a sinus node dysfunction and focus on cardiac and thoracic imaging to guide diagnosis. CONCLUSION: Sinus node dysfunction may be the first manifestation of cardiac sarcoidosis. In unexplained sinus node dysfunction in young patients, advanced cardiac imaging is a key to cardiac sarcoidosis diagnostic. Early recognition of cardiac sarcoidosis enables to start immunosuppressive treatment and discuss implantable cardioverter defibrillator implantation.

4D flow MRI: impact of region of interest size, angulation and spatial resolution on aortic flow assessment.

Objectives.In cardiovascular magnetic resonance, the 3D time-resolved phase-contrast technique, also known as 4D flow, is gaining increasing attention due to applications that exploit three-directional velocity encoding throughout the cardiac cycle. Blood flow volume assessment usually requires an expert to draw regions of interest (ROI) around the vessel cross section, whereas the errors involved in this estimation have not been thoroughly investigated. Our objective is to quantify the influence of ROI sizing, angulation and spatial resolution of the reconstructed plane employed in blood flow measurements using 4D flow.Approach.Three circular ROIs were drawn around the ascending, arch and descending aorta of healthy volunteers (n= 27) and patients with a dilated ascending aorta or bicuspid valve (n= 37). We applied systematic changes of ROI diameter (up to ±10%), tilt angle (up to ±25°) and spatial resolution (from 0.25 to 2 mm) of the reconstructed oblique planes, calculating the effects on net, forward and backward blood flow volumes.Main results.Patients had a larger ascending aorta than healthy volunteers with similar ages and male sex proportion (60 ± 15 y.o. vs 58 ± 16 y.o. and 84% vs 70%, respectively). Higher forward and backward flow volumes were observed in the ascending aorta and the aortic arch of the patients with respect to controls (p< 0.001), whereas net volumes were similar: 74.0 ± 20.8 ml versus 75.7 ± 21.8 ml (p= 0.37), respectively. The ascending aorta was the most sensitive to ROI modifications. Changes of ±10% in the ROI diameter and ±25° in tilt angles produced flow volume differences of up to 9 ml (10%) and 18 ml (15%) in controls and patients, respectively. Modifying the reconstructed planes spatial resolution produced flow volume changes below 2 ml.Significance.Since the setting of the ROI size and plane angle could produce errors that represent up to 20% of the forward and/or backward aortic flow volume, a good standardization for vessel segmentation and plane positioning is desirable.

Cardiopulmonary assessment of patients with systemic sclerosis for hematopoietic stem cell transplantation: recommendations from the European Society for Blood and Marrow Transplantation Autoimmune Diseases Working Party and collaborating partners.

Systemic sclerosis (SSc) is a rare disabling autoimmune disease with a similar mortality to many cancers. Two randomized controlled trials of autologous hematopoietic stem cell transplantation (AHSCT) for SSc have shown significant improvement in organ function, quality of life and long-term survival compared to standard therapy. However, transplant-related mortality (TRM) ranged from 3-10% in patients undergoing HSCT. In SSc, the main cause of non-transplant and TRM is cardiac related. We therefore updated the previously published guidelines for cardiac evaluation, which should be performed in dedicated centers with expertize in HSCT for SSc. The current recommendations are based on pre-transplant cardiopulmonary evaluations combining pulmonary function tests, echocardiography, cardiac magnetic resonance imaging and invasive hemodynamic testing, initiated at Northwestern University (Chicago) and subsequently discussed and endorsed within the EBMT ADWP in 2016.

Descending aorta subject-specific one-dimensional model validated against in vivo data.

The aorta plays a major role in the cardiovascular system and its function and structure are primarily affected by aging, eating habits, life style and other cardiovascular risk factors, inducing increased stiffness which is associated with cardiovascular and cerebral morbi-mortality. Our objective was to develop and validate a robust subject-specific one-dimensional wave propagation numerical model of the descending aorta. This model with a cross-sectional area, velocity and pressure formulation is built using geometric and hemodynamic data measured on a specific person and is validated against in vivo data acquired on the same subject at three distinct anatomical locations along the thoracic aorta. We studied seven healthy volunteers, who underwent carotid applanation tonometry and aortic cardiovascular magnetic resonance (CMR). Responses of our model in terms of changes in central pressure waveform with arterial alterations were consistent with previously described physiological knowledge. Quantitative validation averaged over the three descending aortic locations and the seven subjects provided low rms errors (given in percentage of the maximal clinical value) between simulated and CMR data, i.e. area: 10±6%, velocity: 11±3%, flow rate: 9±3%. Finally, we also found low rms (5±2%) when comparing simulated pressure in the proximal aortic location against tonometric carotid pressure curves. In conclusion, this simple model performs similar to more complex models of the entire systemic arterial tree at a fraction of the cost, and could be of major usefulness in the non-invasive and local estimation of proximal biomechanical and hemodynamic indices.

Accuracy of multi-detector computed tomographic angiography assisted by post-processing software for diagnosis atheromatous renal artery stenosis.

PURPOSE: To compare the diagnostic performance of MDCTA versus renal angiography in the detection of>50% renal artery stenosis in patients suspected of reno-vascular hypertension. MATERIALS AND METHODS: Between January 2005 and January 2010, 92 MDCTA and renal arteriographies were retrospectively analysed. Renal angiographies were read by one interventional radiologist. Three blinded independent readers (two senior radiologists and one technician) scored MDCTA images using three different approaches. Reader 1 scored stenosis using only MPR and MIP. Reader 2 (technician) used only proprietary automatic arterial segmentation software. Reader 3 used the cited software, using manual diameter measurements. RESULTS: A total of 92 patients, (235 renal arteries) were assessed in which 48 significant stenosis were found by arteriography. Sensitivity, specificity, of MDCTA compared to renal arteriography were respectively per patient for reader 1: (88%; 80%); for reader 2: (58%; 80%); for reader 3: (96%; 90%) (P<.02). CONCLUSION: When using automated vessel analysis software edited by a radiologist, MDCTA studies had a Sensibility/Specificity of 96%/90% to detect>50% renal artery stenosis.

Three-dimensional evaluation of thoracic aorta enlargement and unfolding in hypertensive men using non-contrast computed tomography.

Aging produces a simultaneous thoracic aorta (TA) enlargement and unfolding. We sought to analyze the impact of hypertension on these geometric changes. Non-contrast computed tomography images were obtained from coronary artery calcium scans, including the entire aortic arch, in 200 normotensive and 200 hypertensive asymptomatic men. An automated algorithm reconstructed the vessel in three-dimensions, estimating orthogonal aortic sections along the whole TA pathway, and calculated several geometric descriptors to assess TA morphology. Hypertensive patients were older with respect to normotensive (P<0.001). Diameter and volume of TA ascending, arch and descending segments were higher in hypertensive patients with respect to normotensive (P<0.001) and differences persisted after adjustment for age. Hypertension produced an accelerated unfolding effect on TA shape. We found increments in aortic arch width (P<0.001), radius of curvature (P<0.001) and area under the arch curve (P<0.01) with a concomitant tortuosity decrease (P<0.05) and no significant change in aortic arch height. Overall, hypertension produced an equivalent effect of 2-7-years of aging. In multivariate analysis adjusted for age and hypertension treatment, diastolic pressure was more associated to TA size and shape changes than systolic pressure. These data suggest that hypertension accelerates TA enlargement and unfolding deformation with respect to the aging effect.

Methodology for jointly assessing myocardial infarct extent and regional contraction in 3-D CMRI.

Automated extraction of quantitative parameters from cardiac magnetic resonance images is crucial for the management of patients with myocardial infarct. This paper proposes a postprocessing procedure to jointly analyze Cine and delayed-enhanced (DE) acquisitions, in order to provide an automatic quantification of myocardial contraction and enhancement parameters and a study of their relationship. For that purpose, the following processes are performed: 1) DE/Cine temporal synchronization and 3-D scan alignment, 2) 3-D DE/Cine rigid registration in a region about the heart, 3) myocardium segmentation on Cine-MRI and superimposition of the epicardial and endocardial contours on the DE images, 4) quantification of the myocardial infarct extent (MIE), 5) study of the regional contractile function using a new index, the amplitude to time ratio (ATR). The whole procedure was applied to ten patients with clinically proven myocardial infarction. The comparison between the MIE and the visually assessed regional function scores demonstrated that the MIE is highly related to the severity of the wall motion abnormality. In addition, it was shown that the newly developed regional myocardial contraction parameter (ATR) decreases significantly in delayed enhanced regions. This largely automated approach enables the combined study of regional MIE and left ventricular function.

An automated quantification of the transmural myocardial infarct extent using cardiac DE-MR images.

Evaluating myocardial viability is an important prognostic factor in the follow-up of infarctions. Delayed Enhancement magnetic resonance (DE-MR) imaging allows precise delineation of the infarct transmural extent. Visual interpretation is the most commonly used method to assess the myocardial infarction (MI) transmural extent. This study proposes to automate the segmentation of the (DE) images prior to the estimation of the extent of infarcted tissue. Indeed the segmentation of the myocardium was performed using cine contraction images which present a high contrast between cavity and myocardium. After the segmentation, the segmental transmurality is estimated on a conventional five point scale. A head to head comparison was performed between visual and quantitative analysis of infarct transmurality on DE-MR imaging. Results on 921 sub-segments (9 patients) showed an absolute agreement of 80% and a relative agreement (with one point difference) of 97%.

Subclinical atherosclerosis modeling: Integration of coronary artery calcium score to Framingham equation.

Medical prevention consists to identify as soon as possible apparently healthy individuals who develop a disease and to engage them for active preventive treatment. Several cross-sectional studies of general populations or high cardiovascular risk have shown that coronary calcium score (coronary artery calcium, CAC) was positively associated with traditional risk factors (hypertension, dyslipidemia, diabetes, and smoking) and some new risk factors (fibrinogen). In this work, we first calculated, among 618 men, the risk of 10-years cardiovascular heart disease (CHD) according to the Framingham risk model, and then we calculated the probability that the CAC score of an individual falls in all four CAC categories (0, 1-100, 101-400 and > 400). We obtained risk factors adjusted relative risk (RR) estimates from a meta-analysis comparing the risk of coronary heart disease in individuals with CAC scores of 1-100 (RR = 1.7), 101 - 400 (RR = 3.0) and > 400 (RR = 4.3) with the risk of a person with a CAC score zero. The new model for the risk of CHD for each CAC score category were then calculated assuming an average 1-year risk of CHD and risk assessment of the four CAC score categories, weighted by the probability that scores fall into each category. The combination of modeling the CCA with the modeling of conventional risk factors allows obtaining a remarkable predictive value that can improve the assessment of overall risk Framingham through the reclassification of the risk of CHD to an extent which may be clinically important.

An automated myocardial segmentation in cardiac MRI.

In this paper we present an automatic approach to segment Cardiac Magnetic Resonance (CMR) images. A preprocessing step that consists in filtering the image using connected operators (area opening and closing filters) is applied in order to homogenize the cavity and solve the problems due to the papillary muscles. Thereby the GVF snake algorithm is applied with one point clicked in the cavity as initialization and an optimized tuning of parameters for the endocardial contour extraction. The epicardial border is then obtained using the endocardium as initialization. The performance of the proposed method was assessed by experimentation on thirty-nine CMR images. A high agreement between manual and automatic contours was obtained with correlation scores of 0.96 for the endocardium and 0.90 for the epicardium. Overlapping percentage, mean and maximum distances between the two contours show a good performance of the method.

[A pitfall of transseptal catheterisation for percutaneous mitral commissurotomy: interruption of the inferior vena cava with azygos continuation]. / Piège du cathétérisme transseptal pour commissurotomie mitrale percutanée: l'interruption de la veine cave inférieure avec continuation azygos. A propos d'un cas et revue de la littérature.

Transseptal catheterisation is a widely used technique in interventional cardiology. The authors report the case of a 37 year old woman admitted for percutaneous mitral commissurotomy of a symptomatic rheumatic mitral stenosis in whom transseptal catheterisation was impossible because of a rare congenital anomaly: interruption of the inferior vena cava with azygos vein continuation.

Stroke after surgical treatment of sinus venosus type atrial septal defect: percutaneous treatment.

BACKGROUND AND PURPOSE: Sinus venosus-type atrial septal defect can be associated with anomalous drainage of the upper right pulmonary vein into the superior vena cava. Surgical correction provides reconstruction of superior vena cava and rerouting of the pulmonary vein into the left atrium. SUMMARY OF CASE: We report the case of a young woman who experienced 2 strokes 11 years after surgical repair of sinus venosus-type atrial septal defect. CONCLUSIONS: Interventional treatment resolved the superior vena cava stenosis, rerouted the pulmonary vein into the left atrium and abolished the right to left shunt.

[Diagnostic value of a two-dimensional echocardiographic score for left ventricular hypertrophy validated by the Imatron CT scan in familial hypertrophic cardiomyopathy]. / Valeur diagnostique d'un score echocardiographique bidimensionnel d'hypertrophie du ventricule gauche validé par scanner Imatron dans la cardiomyopathie hypertrophique familiale.

The aim of this study was to validate a two-dimensional echocardiographic score for left ventricular hypertrophy in familial hypertrophic cardiomyopathy (HCM) by fast CT scan and to study the diagnostic value by an indexed threshold value in affected and genotyped families in comparison with the classical diagnostic method of maximal wall thickness (E max). The study was performed successively in two patient groups with HCM. The echo/CT scan population comprised 26 patients. They underwent echocardiography and Imatron CT scanning. The E max and 2D echo score (sum of the thickness of 4 segments) were measured by echocardiography and compared to the left ventricular mass obtained by the CT method. The 2D echo score was closely correlated to the CT left ventricular mass (r = 0.85) with a higher correlation coefficient than the E max (r = 0.78). The echo/generic population comprised 109 genotyped adults with an identified mutation. The E max and 2D echo score were measured. The genotype was the reference for diagnosis. A theoretical value of the 2D echo score was determined in healthy individuals by a multiple linear regression model of ages, sex and body surface area. A threshold value for abnormality was established after analysis of the ROC. The sensitivity and specificity were 63% and 100% respectively for E max and 73% and 96% respectively for the indexed 2D echo score. The improvement in sensitivity was marked in young adults (< 50 years) with 69% for the indexed 2D echo score versus 54% for E max, p < 0.04. The authors conclude that the indexed 2D score has been validated as an index of hypertrophy by the Imatron CT and has a better diagnostic value than E max, especially in young adults. This echocardiographic criterion could be proposed as an alternative diagnostic sign for screening families.

[Does hypertension need treatment following correction of coarctation in childhood?]. / Faut-il traiter l'hypertension artérielle après cure de coarctation dans l'enfance?

Does hypertension need treatment following correction of coarctation in childhood? The results of the surgical repair of aortic coarctation (CoA) are excellent. Prenatal diagnosis of this defect is made in 40% of the cases and this allowed a reduction of preoperative mortality. Beside these successes, patients who underwent a CoA repair in infancy remain at high risk for resting hypertension (HT) later in life. Indeed, half of the adolescents are hypertensive and 2/3 of the patients around 30 years. This HT is responsible for an increased mortality mostly related to cardiovascular events. Screening for HT and its risk factors is the main objective of the follow-up. Some residual anomalies such as recoarctation or hypoplasia of the horizontal aorta may be treated either by surgery or by interventional catheterisation. Recently, new contributors to hypertension have been identified such as abnormal geometry of the aortic arch or alteration of the mechanical properties of the arterial wall. In a given patient, the co-existence of vascular dysfunction and abnormal geometry of the aortic arch confers a high risk for HT. The indications to treat exercise HT or the type of antihypertensive drug to be given remain unsolved questions. Prevention may rely on substantial modifications of the surgical techniques to optimise the aortic arch geometry. The indications to correct abnormal geometries at high risk without recoarctation are not yet defined. The long-term benefit of either preventive or curative strategies might be difficult to evidence and will probably need the analysis of intermediate markers such as vascular function and left ventricular hypertrophy.

[The potential of magnetic resonance imaging for the surveillance of septal alcohol ablation in hypertrophic obstructive cardiomyopathy. A case report]. / Potentialité de l'imagerie par résonance magnétique pour la surveillance du traitement par alcoolisation septale de la cardiopathie hypertrophique obstructive. A propos d'un cas.

We describe here the MRI surveillance of septal alcohol ablation in a case of symptomatic obstructive cardiomyopathy. MRI examinations were performed before, 15 days and 2 months after alcohol ablation using an identical protocol to study the function, regional perfusion and the equilibrium perfusion in order to directly visualize the infarction. MRI seems to be an excellent investigation in the surveillance of such patients, allowing precise quantification of the infarcted zone. The various stages of cellular necrosis in the induced infarct were demonstrated, and the role of remodelling in increasing the functional area of the systolic ejection pathway.

[Aortic arch shape and flow dynamics]. / Architecture de la crosse aortique et circulation des fluides.

Post-operative deformation of the aortic arch architecture is associated with an increased risk of hypertension following correction of coarctation. In addition to morphological analysis, MRI allows a functional analysis of the thoracic aorta. We report three examples which illustrate the direct relationship between aortic arch morphology and blood flow in the thoracic aorta.

[Relationship between aortic arch shape and blood pressure response after coarctation repair]. / Relation entre l'architecture de la crosse aortique et la pression artérielle après cure de coarctation.

The mechanisms of secondary hypertension after repair of coarctation of the aorta are not well understood. Abnormalities of the architecture of the aortic arch and their consequences on blood pressure have not been studied. In order to study the relationship between abnormalities or aortic arch architecture and resting blood pressure ninety-four patients without re-coarctation were followed up prospectively from 1997 to 2004 (mean age 16.9 +/- 8.1 years; mean weight 57.5 +/- 18.3 Kg; interval since surgery 16.3 +/- 5.4 years). All underwent MRI angiography of the thoracic aorta which enabled the abnormalities to be classified in 3 groups: gothic arch, crenellated arch and roman arch. Twenty-four patients (25.5%) were hypertensive and 70 (74.4%) normotensive. There were 40 gothic arches (42.5%). 14 crenellated arches (15%) and 40 roman arches (42.5%). Gothic arches were more commonly observed in the hypertensive patients (18/40, [45%, 95% CI 31-62]) than the crenellated arches (4/14, [28.5%, 95% CI 7-48]) or the roman arches (2/40, [5%, 95% CI 2-12]). Only the gothic arch was independently correlated with hypertension on multivariate analysis. The authors conclude that gothic deformation of the aortic arch is an independent predictive factor of hypertension in patients operated for coarctation with an excellent result on the isthmic region. Patients with a gothic appearance of their aortic arch should be followed up closely.