Elie Mousseaux

Fibromuscular Dysplasia of Cervical and Intracranial Arteries

Fibromuscular dysplasia is an uncommon, segmental, nonatherosclerotic arterial disease of unknown aetiology. The disease primarily affects women and involves intermediate-sized arteries in many areas of the body, including cervical and intracranial arteries. Although often asymptomatic, fibromuscular dysplasia can also be associated with spontaneous dissection, severe stenosis that compromises the distal circulation, or intracranial aneurysm, and is therefore responsible for cerebral ischaemia or subarachnoid haemorrhage. Fibro-muscular dysplasia affects middle and distal portions of the internal carotid and vertebral arteries, and occasionally, intracranial arteries. Several pathological and angiographic patterns exist. The most frequent pathological type is medial fibromuscular dysplasia, which is associated with the ‘string of beads angiographic pattern. Unifocal lesions are less common and can be associated with several pathological subtypes. The pathophysiology of the disease is widely unknown. Fibromuscular dysplasia may in fact result from various causes and reflect a non-specific response to different insults. The poor knowledge of the natural history and the lack of randomised trials that compared the different treatment options do not allow any satisfactory judgement to be made regarding the need for or the efficacy of any treatment.

Diagnosis and management of fibromuscular dysplasia: an expert consensus

Alexandre Persu, Emmanuel Touzé, Elie Mousseaux, Xavier Barral, Francis Joffre and Pierre-François Plouin Division of Cardiology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium, Department of Neurology, Université Paris Descartes, INSERM UMR S894, Pôle neuroscience, Hôpital Sainte-Anne, Paris, France, Department of Radiology, Université Paris Descartes, Hôpital Européen Georges Pompidou, Paris, France, Department of Vascular Surgery, CHU Hôpital Nord, Saint-Etienne, France, Department of Radiology, Rangueil University Hospital, Toulouse, France, Université Paris-Descartes; Assistance Publique-Hôpitaux de Paris; Hôpital Européen Georges Pompidou, Hypertension Unit, Paris, France

Non-surgical septal myocardial reduction by coil embolization for hypertrophic obstructive cardiomyopathy: early and 6 months follow-up

AIMSnTo evaluate the feasibility and the incidence of complete heart block (CHB) after non-surgical septal myocardial reduction by coil embolization in hypertrophic obstructive cardiomyopathy (HOCM).nnnMETHODS AND RESULTSnTwenty patients with HOCM and drug-refractory symptoms underwent non-surgical myocardial septal reduction by coil embolization with detachable coils. Occlusion of septal perforator branches was successfully performed in all patients. We detected neither ventricular tachycardia nor CHB. One patient presented an interventricular septal defect after the procedure, and died 19 days later. Cardiac magnetic resonance imaging showed, in all patients, an increase in areas of hyperenhancement in the interventricular septum (IVS) compared with baseline. At 6-month follow-up, NYHA functional class and peak oxygen consumption were significantly improved compared with baseline (14.8 +/- 4.5 vs. 18.5 +/- 4.5 mL/kg/min; P = 0.001, respectively). Echocardiography showed a significant reduction of the IVS thickness and left ventricular outflow tract gradient (21 +/- 3 vs. 17 +/- 4 mm, P < 0.0001; 80 +/- 29 to 35 +/- 29 mmHg, P < 0.0001, respectively).nnnCONCLUSIONnThe results of this pilot non-randomized study suggest that non-surgical septal myocardial reduction by coil embolization in HOCM is feasible and does not induce CHB. Larger studies, ideally with a randomized comparison between coil embolization and alcohol septal ablation, are warranted.

A polydioxanone electrospun valved patch to replace the right ventricular outflow tract in a growing lamb model.

A major issue in congenital heart surgery is the lack of viable right ventricular outflow tract (RVOT) replacement materials. Several biomaterials have been used, with different scaffolds and cells, but they have failed to restore a tri-layered RVOT, and reoperations are often required. We investigated the function, histological changes and potential of growth and tissue regeneration of polydioxanone (PDO) electrospun bioabsorbable valved patches seeded with mesenchymal stem cells (MSCs) in the RVOT of growing lambs. Autologous blood-derived MSCs were labeled with quantum dots and seeded on PDO electrospun valved patches. Those were implanted into the RVOT of 6 growing lambs followed up until 8 months. Results were assessed by echocardiography, magnetic resonance imaging (MRI), histology, immunohistochemistry and biochemical assays. Tissue-engineered RVOT were neither stenotic nor aneurismal and displayed a growth potential, with less fibrosis, less calcifications and no thrombus compared with control polytetrafluoroethylene (PTFE)-pericardial patches. The PDO scaffold was completely degraded and replaced by a viable, three-layered, endothelialized tissue and an extracellular matrix with elastic fibers similar to that of native tissue. Detection of quantum dots at 1 month suggested that at least some of the cells were-derived from the grafted cells. A polydioxanone electrospun tissue-engineered valved transannular patch seems to be a promising device in restoring a living RVOT and could ultimately lead to applications in the treatment of congenital RVOT diseases.

Arterial Stiffness as an Imaging Biomarker Are All Pathways Equal

See related article, pp 161–167nnThe measurement of arterial stiffness is increasingly popular among physicians and researchers mainly because its predictive value for cardiovascular (CV) events has been well demonstrated. The largest amount of evidence has been given for aortic stiffness, measured through carotid-femoral pulse wave velocity (cfPWV). This has been initially reported in the late 1990s or early 2000s.1 Currently, as many as 19 studies consistently showed the predictive value of aortic stiffness for fatal and nonfatal CV events in various populations having different levels of CV risk: general population, patients with hypertension, elderly subjects, patients with type 2 diabetes mellitus, and patients with end-stage renal disease. In a recent meta-analysis,2 17 longitudinal studies totalizing 15 877 subjects with a mean follow-up of 7.7 years showed, for 1 SD increase in PWV, a risk ratio of 1.47 (1.31–1.64) for total mortality, 1.47 (1.29–1.66) for CV mortality, and 1.42 (1.29–1.58) for all-cause mortality.nnTo be considered as a novel risk marker, arterial stiffness should add predictive information to established, standard risk markers, particularly the Framingham Risk Score or the European SCORE. This has been demonstrated with cfPWV in ≥3 studies: in patients with hypertension,3 in elderly subjects from a general population,4 and in middle-aged subjects from a general population.5 Another important requirement is that the measurement of the novel risk marker changes predicted risk sufficiently to change recommended therapy. This is indeed the case because several studies showed that a substantial amount of patients at intermediate risk could be reclassified into a higher or a lower CV risk, when arterial stiffness was measured.4–6 Aortic stiffness, measured through cfPWV, can thus be considered as a novel imaging biomarker for predicting CV events, although its value as a true surrogate end point requires a large intervention …

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.

Cardiac Structure and Function in Cushing's Syndrome: A Cardiac Magnetic Resonance Imaging Study

Background: Patients with Cushings syndrome have left ventricular (LV) hypertrophy and dysfunction on echocardiography, but echo-based measurements may have limited accuracy in obese patients. No data are available on right ventricular (RV) and left atrial (LA) size and function in these patients. Objectives: The objective of the study was to evaluate LV, RV, and LA structure and function in patients with Cushings syndrome by means of cardiac magnetic resonance, currently the reference modality in assessment of cardiac geometry and function. Methods: Eighteen patients with active Cushings syndrome and 18 volunteers matched for age, sex, and body mass index were studied by cardiac magnetic resonance. The imaging was repeated in the patients 6 months (range 2–12 mo) after the treatment of hypercortisolism. Results: Compared with controls, patients with Cushings syndrome had lower LV, RV, and LA ejection fractions (P < .001 for all) and increased end-diastolic LV segmental thickness (P < .001). Treatment of hypercortisolism was associated with an improvement in ventricular and atrial systolic performance, as reflected by a 15% increase in the LV ejection fraction (P = .029), a 45% increase in the LA ejection fraction (P < .001), and an 11% increase in the RV ejection fraction (P = NS). After treatment, the LV mass index and end-diastolic LV mass to volume ratio decreased by 17% (P < .001) and 10% (P = .002), respectively. None of the patients had late gadolinium myocardial enhancement. Conclusion: Cushings syndrome is associated with subclinical biventricular and LA systolic dysfunctions that are reversible after treatment. Despite skeletal muscle atrophy, Cushings syndrome patients have an increased LV mass, reversible upon correction of hypercortisolism.

Anomalous Origin of the Left Coronary Artery From the Right Coronary Sinus Diagnosed by Electron Beam Computerized Tomography

This picture was obtained in a 61-year-old female patient referred for myocardial infarction of the left anterior ventricular wall. A coronary angiogram demonstrated nonsignificant coronary artery disease, but catheterization of the left coronary artery was difficult and raised the suspicion of anomalous aortic origin of the left …

Optimal follow-up in adult patients with congenital heart disease and chronic pulmonary regurgitation: towards tailored use of cardiac magnetic resonance imaging.

BACKGROUNDnPulmonary regurgitation (PR) is a common complication of right ventricular outflow tract (RVOT) reconstruction and leads to right ventricular (RV) dilatation and dysfunction. Although cardiac magnetic resonance (CMR) is the gold standard for evaluating PR and RV dysfunction, cost and limited availability are problems in many centres.nnnAIMSnTo determine clinical, electrocardiographic and echocardiographic predictors of these complications and optimize patient selection for their short-term follow-up by CMR.nnnMETHODSnNinety-four patients with a history of RVOT repair were prospectively included. All patients had a clinical examination, electrocardiography, echocardiography and CMR.nnnRESULTSnQRS duration, indexed end-diastolic RV (EDRV) diameter and area on echocardiography were significantly associated with RV dilatation on CMR (P<0.001). The distal localization of Doppler PR flow was the strongest echocardiographic criterion associated with severe PR (P<0.001). Arrhythmia history and high Tei index were significantly associated with low RV ejection fraction (P<0.001 and P=0.017, respectively). In multivariable analysis, grade of PR, QRS duration, arrhythmia and valvulotomy were strongly associated with severe PR and RV dilatation or systolic RV dysfunction. From these results, an approach based on a scaled scoring system for selecting patients who need short-term CMR evaluation and close follow-up was evaluated. This method should avoid 31% of CMR examinations, with a sensitivity of 97.7%.nnnCONCLUSIONnClinical, electrocardiographic and echocardiographic criteria can be used to accurately evaluate patients with RVOT repair. The combination of such features facilitates identification of patients who do or do not require close CMR evaluation.

Correspondence Between Delayed Enhancement Patterns in Multislice Computed Tomography and Magnetic Resonance Imaging in a Case of Acute Myocarditis

A 42-year-old man who was an active smoker with no significant medical history presented with severe substernal pain 5 days after acute enteritis. The examination showed mild fever, blood pressure of 113/75 mm Hg, and heart rate of 85 beats per minute.nnThe ECG showed sinus rhythm with incomplete left bundle-branch block. Laboratory tests showed elevated levels of troponin I (23 μg/L) and C-reactive protein (104 mg/L). …