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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.We report a case of cardiac sarcoidosis revealed by a sinus node dysfunction and focus on cardiac and thoracic imaging to guide diagnosis.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.

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 10.07- 22). Actuarial survival was 82% at 20 years. Tricuspid valve insufficiency was I 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.

3D luminal vessel geometry description and visualization are important for the diagnosis and the prognosis of heart attack and stroke. A general mathematical framework is proposed for 3D reconstruction of vessel sections from a few angiograms. Regularization is introduced by modeling the vessel boundary slices by smooth contours to get the reconstruction problem well posed. A dynamic contour approach is applied to optimize the shape of the contour according to the recorded angiograms and the internal smoothness constraints. The solution is achieved following the minimization of a nonconvex energy function assigned to the contour with a simulated annealing algorithm. Preliminary testing on noisy and truncated synthetic images produces promising results. Evaluation and validation of the method on hardware phantoms are also presented.

L’augmentation considerable du nombre de publications concernant le ventricule gauche non compacte (VGNC) temoigne de l’interet grandissant de la communaute internationale pour cette entite particuliere du myocarde. Ce VGNC est caracterise par la presence de trabeculations proeminentes et de recessus intratrabeculaires profonds [1]. La paroi myocardique est dans cette entite constituee d’une couche fine de muscle compacte associee a une couche epaisse de muscle sous-endocardique trabecule. Il existe cependant toujours des discussions pour savoir si cette entite est une reelle cardiomyopathie bien individualisee ou s’il s’agit d’une expression phenotypique partagee entre plusieurs cardiomyopathies distinctes. Cette anomalie peut etre effectivement presente de facon isolee ou associee a diverses cardiopathies (anomalies congenitales simples telle que la maladie d’Ebstein ou complexes cardiopathies hypertrophiques et/ou dilatees).

Aortic root dilatation is mainly observed in children with various connective tissue disorders. The most frequent cause of aortic root dilatation is infantile Marfan syndrome.1 Aortic root dilatation has also been recognized as being associated with bicuspid aortic valves, with or without coarctation.2 The isolated forms are rare.3 This dilatation may be worrisome, because it can lead to aortic insufficiency or dissection. Here we report a series of 14 children who underwent ascending aorta replacement.

Le scanner permet aujourd’hui la detection du calcium qui se depose dans la plaque d’atherome, sur les valves ou dans la paroi aortique. Il ressort ainsi que des scores de calcifications sont utiles dans la determination du risque cardiovasculaire, scores qui permettent essentiellement de reclasser le risque des sujets a risque intermediaires (5 a 20 % sur 10 ans), meme apres la prise en compte des facteurs de risques conventionnels. De nombreux travaux sont en cours avec cette methode afin d’ameliorer la comprehension de la physiopathologie de ces calcifications chez l’homme et de tester de nouvelles approches therapeutiques.

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.

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, p0.04. 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? 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.

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.

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.

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.

The advent of helical multidetector CT has significantly modified several traditional clinical approaches to cardiovascular diagnosis. The current availability of rapid image acquisition has provided the basis for investigating direct imaging in real time of cardiac structures using CT. Application of thin-section submillimetric image acquisitions to three-dimensional (3D) reconstruction algorithms produces 3D data sets from which images of the coronary arteries may be obtained in any anatomic plane. The advantage of the submillimetric isotropic spatial resolution is partly offset by reduced temporal resolution as well as reduced contrast resolution compared to MRI. This lack in contrast sensitivity prevents accurate perfusion imaging and restricts the clinical use to coronary artery imaging. Moreover, the large amount of iodinated contrast medium injected has potential nephrotoxic effects, which can be deleterious if coronary artery angiography must to be performed. On the other hand, MRI has less spatial resolution, and acquisitions must be performed in the plane of each coronary artery because of reduced volume coverage. Both techniques play a role in the non-invasive assessment of coronary artery disease, by providing complementary information already useful in a growing number of clinical situations.

Metastases are the most frequent tumors of the heart even though they seldom are recognized. Most primary cardiac tumors are benign. The main role of imaging is to differentiate a cardiac tumor from thrombus and rare pseudotumors: tuberculoma, hydatid cyst. Echocardiography is the fist line imaging technique to detect cardiac tumors, but CT and MRI are useful for further characterization and differential diagnosis. Myxoma of the left atrium is the most frequent benign cardiac tumor. It usually is pedunculated and sometimes calcified. Sarcoma is the most frequent primary malignant tumor and usually presents as a sessile infiltrative tumor. Lymphoma and metastases are usually recognized by the presence of known tumor elsewhere of by characteristic direct contiguous involvement. Diagnosing primary and secondary pericardial tumors often is difficult. Imaging is valuable for diagnosis, characterization, presurgical evaluation and follow-up.

An increasing number of patients with the acquired immunodeficiency syndrome (AIDS) and cardiac lymphoma have been documented. Antemortem diagnosis of cardiac non-Hodgkin lymphoma in AIDS is difficult because of the non-specificity of the clinical findings. Rapid progression of cardiac dysfunction is common after symptoms appear. We report the case of a patient with AIDS and cardiac lymphoma revealed by an atrial flutter.

In the past, the diagnosis and course of Takayasu arteritis were monitored by following angiographic findings, in addition to clinical symptoms and biological tests. More recently, cross-sectional imaging techniques especially Computed Tomography (CT) depicted mural changes in aorta and main vessels in this disease. Within the same acquisition, spiral mode also allows to show luminal changes, similarly to conventional angiography. In addition to its diagnostic value especially in the early phase of the disease, CT seems accurate in the follow-up of treated patients and may be proposed as a therapeutic guide. CT features are presented, according to the stage of the disease and the vessels involved.

The results of transcatheter balloon angioplasty in teenagers and adults with aortic recoarctation are uncertain. Therefore, there is a current trend to prefer a more complex procedure including the implantation of a stent. This study deals with 8 patients aged 7 to 25.3 years (median: 15 years), weighing 20 to 68 kg. (median: 57) and having undergone resection of an aortic coarctation during infancy (24 days to 4 years). All had their lower limb pulses diminished or abolished, elevated blood pressure at rest (and at exercise in the 5 tested patients), and left ventricular hypertrophy. MRI documented the lesion and helped to select seven patients whose stenosis was short and remote enough from the origin of the main aortic collateral. In one case, the decision to stent was taken as an emergent measure to treat an aortic dissection which appeared shortly after balloon dilatation. The effectiveness of the procedure was immediate in all patients with a 50% increase in diameter of the dilated area, total relief of the gradient, drop to normal values of the blood pressure. These good results persisted at follow-up (3-24 months) in 6 patients, with moderate hypertensive rebounds in the last 2. There were 2 technical problems (premature burst of the balloon, asymmetrical inflation of the stent like an "Eiffel Tower") that could finally be overcome and should no longer occur with the new specially designed so-called "BIB" balloons. Would long term follow-up confirm these early results, one should conclude that this method offers an attractive, safe and effective option to surgery for adolescents and adults with late recoarctation of the aorta.

Objectif Une limite importante en chirurgie cardiaque pediatrique est le manque de materiaux de remplacement de la voie de sortie ventriculaire droite (RVOT), doues de croissance et evitant les reoperations. Objectif : restaurer une RVOT fonctionnelle autologue a l’aide d’un patch en polydioxanone (PDO) biodegradable valve, ensemence de cellules souches mesenchymateuses (CSMs) autologues, dans un modele ovin en croissance. Methodes Des patchs en PDO biodegradables valves ensemences de CSMs autologues marquees par quantum dots ont ete implantes en position transannulaire sur la RVOT de 6 agneaux (suivi : 8 mois). Des patchs non ensemences ( n = 2) ou en pericarde autologue ( n = 2) constituaient les groupes controle. Resultats Echocardiographie et IRM demontrent l’absence de stenose et d’anevrysme de la neo-RVOT et son potentiel de croissance. Histologie et immunohistochimie objectivent moins de fibrose et de calcifications (0,08 % ± 0,03 % Ca2+), une degradation complete du polymere, l’apparition d’un neo-tissu organise et endothelialise avec matrice extracellulaire de type natif. Conclusion Un patch en PDO valve transannulaire ensemence de CSMs autologues restaure une RVOT vivante et fonctionnelle, et pourrait ameliorer le pronostic des cardiopathies droites congenitales.

Previous studies have shown that the multi-step approach of velocity or acceleration encoding is highly efficient in terms of the signal-to-noise ratio per unit time. This work describes a multidimensional extension of this method for simultaneously measuring multiple components of velocity and acceleration with a few encoding steps. N flow dimensions were encoded with an ND-matrix, obtained by combining the various flow-encoding gradients. The small matrix obtained with as few as two encoding steps can be extended by zero-filling in all N dimensions and using ND-Fourier transformation to obtain the maximum of the resulting peak in the ND-matrix, which gives simultaneously all the components of velocity and/or acceleration. The processing time was shortened by using a method of phase computation that gives the same precision as Fourier transformation, but is much faster. A rotating disk was used to show that the velocity-to-noise ratio increases with the number of dimensions acquired, demonstrating the efficiency of multidimensional flow measurements. The feasibility of the method is illustrated by 3D maps of the myocardium velocity, and 2D measurement of velocity and acceleration in the ascending aorta-both obtained by multidimensional phase encoding in volunteers.

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.

A method for estimating pressure gradients from MR images is demonstrated. Making the usual assumption that the flowing medium is a Newtonian fluid, and with appropriate boundary conditions, the inertial forces (or acceleration components of the flow) are proportional to the pressure gradients. The technique shown here is based on an evaluation of the inertial forces from Fourier acceleration encoding. This method provides a direct measurement of the total acceleration defined as the sum of the velocity derivative vs. time and the convective acceleration. The technique was experimentally validated by comparing MR and manometer pressure gradient measurements obtained in a pulsatile flow phantom. The results indicate that the MR determination of pressure gradients from an acceleration measurement is feasible with a good correlation with the true measurements (r = 0.97). The feasibility of the method is demonstrated in the aorta of a normal volunteer. Magn Reson Med 44:66-72, 2000.

We improved the three-point phase-contrast method by regularization of MR velocity data after acquisition of a low velocity-to-noise ratio (VNR) velocity image and a high VNR aliased velocity image. The phase unwrapping algorithm is based on the assumed correlation of the velocity of adjacent flow voxels on the low VNR and the unaliased high VNR images. We used Fourier encoding with eight velocity-encoding gradient steps to obtain reference velocity images of the aorta from five subjects (274 images) and compared them with the phase-contrast and three-point phase-contrast images with and without regularization. The VNR of the regularized velocity image was improved by 9.1 dB and the VNR of the three-point phase-contrast velocity image was improved by 0.7 dB with respect to the low first moment velocity image. Corresponding improvements of 9 dB and 3.7 dB were obtained for the estimations of instantaneous flow rate. Magn Reson Med 44:122-128, 2000.

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.

A technique dedicated to spatial regularization of magnetic resonance (MR) velocity data has been implemented to improve flow image quality. It is assumed that neighboring flow-velocity pixels are partially correlated, although large-velocity discontinuities remain possible. Increasing MR signal magnitude due to the in-flow effect also is used to enhance further reliability of the estimated velocity. By using an eight-step Fourier-encoding approach, 162 "reference" velocity images acquired in the ascending aorta from six healthy volunteers were compared with "raw" and "regularized" images that were computed from only two gradient steps. The mean square error decreased from 0.12 m(2) x s(-2) to 0.06 m(2) x s(-2) (P10-9) for velocity pixel values and from 1929 ml(2) x s(-2) to 1336 ml(2) x s(-2) (P0.01) for instantaneous flow rates. The regularization of two-step data sets provides the same velocity image quality as that found after using three-step data sets without regularization. The method can be applied to phase-velocity data sets of any MR technique to reduce velocity noise. J. Magn. Reson. Imaging 1999;10:851-860.