[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.

[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.

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.

[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.

[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.

[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.

Angiographic and electron-beam computed tomography studies of retrograde cardioplegia via the coronary sinus.

Retroperfusion of the coronary sinus does not provide homogeneous distribution of cardioplegic solution. The goal of this study was to analyze the distribution of flow during retrograde cardioplegic infusion in cadaveric human hearts with two different techniques of coronary sinus cannulation: (1) internal occlusion of the coronary sinus by balloon inflation and (2) external occlusion by tightening the orifice of the coronary sinus around a simple catheter. To evaluate differences between the two techniques, angiographic and electron-beam computed tomographic studies were performed. Computed digital angiography was performed on 14 hearts. Angiographic patterns varied according to type of coronary sinus cannulation. With the balloon inflation technique, the marginal vein and the anterior descending vein were perfused first; the posterior descending vein was not perfused. This vein was opacified secondarily through a venovenous anastomosis located at the apex of the heart. Backward flow into the right atrium (steal phenomenon) was demonstrated. At completion of retroperfusion, the inferior part of the septum remained poorly opacified. Conversely, angiographic findings after external occlusion of the coronary sinus revealed simultaneous injection of all venous channels. The entire septum was well opacified at completion of retroperfusion. Electron-beam computed tomographic study was performed on eight hearts with the external occlusion technique and nine with the internal occlusion technique. The computed tomographic findings confirmed the results of digital angiography. The peak myocardial enhancement and the peak rising rate of myocardial enhancement within the interventricular septum were significantly more important (p < 0.0001) when the external coronary sinus occlusion mode was used than when the internal coronary sinus occlusion mode was used. In all hearts except one, the right ventricular wall was not opacified, regardless of the type of cannulation and the type of radiologic analysis. This study demonstrates the importance of coronary sinus cannulation technique in optimizing the protection of the interventricular septum with retrograde cardioplegic infusion.

Evaluation by electron beam computed tomography of intracardiac masses suspected by transoesophageal echocardiography.

OBJECTIVES: To assess the suitability of electron beam computed tomography (EBT) in evaluating and subsequently managing cardiac masses suspected as a result of examination by transthoracic and transoesophageal cross sectional echocardiography. DESIGN AND PATIENTS: In 76 consecutive patients with suspected cardiac masses, the impact of the EBT examination was analysed by reviewing whether EBT examination altered management by confirming or excluding a mass, whether EBT imaging substantially contributed to decision-making, and lastly whether EBT influenced the decision to operate on the patient. RESULTS: EBT modified the diagnosis or confirmed results which had been questionable with cross sectional echocardiography in 53 cases (70%): these cases included those with anatomical features such as dense calcifications or fatty structures (n = 20), lipomatous lesions (n = 5), equivocal thrombi (n = 5), pericardial masses (n = 5), or hydatid cysts (n = 2). EBT gave information over and above cross sectional echocardiography in all patients with malignant tumours (n = 16) by providing a better delineation of the relation between intracavity masses and normal structures, including the mural site of tumour attachment and tumour extension to the great vessels and the adjacent extracardiac mediastinal structures. EBT provided no additional information over cross sectional echocardiography in the remaining patients (n = 23), particularly in those with myxomas (n = 11) or small valvar lesions (n = 6). EBT only confirmed that the lesion was well demarcated, limited in extent, or not associated with a mass. CONCLUSIONS: EBT has considerable potential for clinical management and surgical planning and may help clinicians to decide against surgery, particularly in patients with unresectable tumours.

Cardiac metastatic melanoma investigated by magnetic resonance imaging.

There is a high rate of cardiac involvement in malignant melanoma (MM), but such cardiac metastases are usually diagnosed late. This report describes four cases with different clinical presentations of MM cardiac involvement that were investigated by magnetic resonance imaging (MRI). The MM cardiac involvement was asymptomatic in one case, detected because of a superior vena cava syndrome in a second, and because a tamponade in the remaining two cases. MRI permitted the diagnosis of cardiac metastases of MM, which was not made by echocardiography in one case. By precisely detecting the extent of the tumors, MRI was a great help in management, especially when an isolated cardiac metastasis was suitable for surgical ablation (two cases). Because both clinical signs and transthoracic echocardiogram are not very sensitive for diagnosing MM myocardial involvement, MRI can help provide such a diagnosis and ensure better treatment-monitored decisions.

Idiopathic dilatation of the pulmonary artery: report of four cases.

Idiopathic dilatation of the pulmonary artery (IDPA) is a rare congenital disease which is usually detected fortuitously on chest x-ray, thus radiologists must be aware of this clinical entity. This report describes four cases to which Magnetic Resonance Imaging (MRI) played a major role in diagnosing IDPA and in detecting the concomitant findings observed in this disease. MRI is a non-invasive procedure with many advantages for the accurate and reproducible measurement of artery structures, which makes it the preferred option for combined use with echocardiography in the diagnosis and follow-up of patients with IDPA.

Ebstein's anomaly: repair based on functional analysis.

OBJECTIVE: 'Classical' repair of Ebstein's anomaly is usually performed with transverse plication of the atrialized chamber. However, the anterior leaflet has restricted motion which is an important factor of the tricuspid valve insufficiency. We studied the long term results of mobilization of the anterior leaflet associated with longitudinal plication of the right ventricule. METHODS: From 1980 to July 2002, 191 patients (mean age 24.4+/-15 years (1-65)) were operated on. Anterior leaflet function was assessed on pre-op echocardiography and on surgical examination. Conservative surgery was possible in 187 patients (98%) and included mobilization of the anterior leaflet, longitudinal plication of the right ventricle and prosthetic annuloplasty in adults. Bidirectional cavo-pulmonary shunt was associated in 60 patients. Four patients had valve replacement. RESULTS: Hospital mortality occurred in 18 patients: 9% (95%CL: 6-15%) due to right ventricle (RV) failure in nine patients. Mean follow-up was 6.4 years (0.07-22). Actuarial survival was 82% at 20 years. Tricuspid valve insufficiency was 1 or 2+ in 80% of the cases. Reoperation occurred in 8% (16 patients). A successful second repair was obtained in ten patients. Electron beam computerized tomography (20 patients) demonstrated improved left ventricle ejection fraction 56-66% (P<0.05). Supraventricular tachycardia and pre-excitation syndromes were reduced from 23 to 5%. CONCLUSION: Conservative surgery is indicated for all symptomatic patients. The incidence of valve repair is high when leaflet mobilization is performed. Valve replacement can be avoided in most cases. Functional and hemodynamic results are excellent.

Automatic detection of left ventricular borders on electron beam CT sequential cardiac images using an adaptive algorithm.

Detection of myocardial borders on sequences of electron beam CT images is carried out using an adaptive segmentation algorithm developed to enhance dynamic analysis of cardiac function. Adaptivity is based on description of the myocardial borders from the mean and standard deviation of the grey level and gradient distributions on each image of the sequence. Comparison of segmentations from five experimentators with automatically determined borders on a set of 416 endocardial and epicardial contours indicated differences between automatic and manual tracing very close to differences due to inter-observer reproducibility.

[Diagnosis of cardiac tumor by percutaneous thromboaspiration. A case report]. / Diagnostic d'une tumeur cardiaque par thromboaspiration percutanée. A propos d'un cas.

Percutaneous endovascular thromboaspiration is a valuable tool as illustrated by the case of a patient suffering from a large intracardiac tumour. Histological and immuno-histochemical analysis of the tumour fragments provided the diagnosis of a cardiac angiosarcoma. The patient was a 44 year old man admitted for a large sero-sanguinous pericardial effusion which recurred after drainage. The case was complicated by a haemorrhagic cerebrovascular accident unrelated to a secondary deposit. Initially suspected after transthoracic echocardiography, the diagnosis of a tumour invading the right atrium was confirmed by transoesophageal echocardiography and cardiac CT scan. Surgery was declined and as the diagnosis of lymphoma could not be excluded, the patient underwent biopsy by an original method of percutaneous thromboaspiration. This minimally invasive, low cost technique would appear to be a valuable alternative to other endovascular biopsy techniques (saber, biotome) and to surgical biopsy, and could be proposed as the technique of first intent in an a priori non-operable intra-cardiac tumour or when lymphoma is suspected.

[Mass and geometry of the left ventricle. Contribution of ultrafast computed x-ray tomography and magnetic resonance imaging]. / Masse et géométrie ventriculaire gauche. Apports de la tomodensitométrie ultrarapide et de l'imagerie par résonance magnétique.

Ultrafast computed tomography and magnetic resonance imaging are two new methods of cardiac imaging. Measurements of left ventricular volume (end-diastolic, end-systolic volume, stroke volume) and mass have been validated with both methods. The calculations are independent of the geometric shape of the ventricle. Although regional analysis is difficult because of the complex movement of the left ventricle in the tomographic cuts, these methods present a number of advantages: excellent temporospatial tomographic resolution, approximately the same in all dimensions, appreciation of endocardial movement from an epicardial centre, the potential to record their transform spatial data in 3 dimensions from initial planar acquisition. However, all potential regional measurements are still being validated as they are operator-dependent and require visual identification and manual tracing of the cardiac contours or local infrastructures which affect the results of these techniques which are still relatively little used in cardiac imaging. In the context of clinical evaluation, these relatively non-invasive methods will become extremely accurate in the appreciation of parameters of left ventricular geometry and function. They will become very useful in the determination of the myocardial effects of drugs, surgery or other interventional procedures in different models of cardiac disease.

[Value and limitations of methods for measuring left ventricular mass]. / Intérêts et limites des méthodes de mesure de la masse ventriculaire gauche.

M-mode echocardiographic measurement of the left ventricular mass is inaccurate when the hypertrophy is asymmetric and the ventricule very deformed. The routine calculation of the mass is based on several hypotheses, verified in normal and hypertensive subjects: the standard error (SEE) is 30-40 g, 10-15% (r > 0.9). The standard deviation of inter-examination differences (> 25 +/- 30 g with a variation coefficient c = 10-15%) makes it difficult to appreciate variations of mass in a given patient. Two-dimensional echocardiographic measurement of left ventricular mass requires the use of geometric formulae which have not been validated in the cardiomyopathies. The absence of a consensus on the models used has favorized the use of MRI and of ultrafast computed tomography. MRI measurement of mass has been validated in normal and ischaemic hearts (r > 0.97, SEE < 8 g, c = 15%) but the times of acquisition are long. Using ultrafast CT, not universally available, this measurement has been validated in vivo and in vitro, including in cardiomyopathy for which the SEE is low (6%) and reproducibility excellent (c = 4-8%), comparable with results in normal subjects.

[Left ventricular thrombus revealed by electron bean computed tomography, in a patient with antiphospholipid syndrome]. / Thrombus de la pointe du ventricule gauche révélé par le scanner à faisceau d'électrons, chez une patiente atteinte d'un syndrome des antiphospholipides.

The authors report a case of a 48 year old woman admitted to hospital because of digital ischemia, in a context of antiphospholipid syndrome. The electrocardiogram-triggered electron beam computed tomography revealed an apical thrombus associated with a thinning left ventricular wall, suggesting painless myocardial infarction. The diagnosis was secondary confirmed by coronarography.

[Early constrictive pericarditis after coronary revascularization. Apropos of a case]. / Péricardite constrictive précoce après revascularisation coronaire. A propos d'un cas.

The authors report a case of severe constrictive pericarditis occurring 6 weeks after coronary bypass surgery and in the absence of any other predisposing factor. The diagnosis was confirmed by CT scan and cardiac catheterisation which showed signs of a diastole. The patient died despite early surgical reoperation because of the severity of the pericardial fibrosis.