Publications by authors named "Arshid Azarine"

44 Publications

Interventricular septum mass presenting as a late acute coronary syndrome with ST-segment elevation: a case report.

Eur Heart J Case Rep 2021 May 12;5(5):ytab164. Epub 2021 May 12.

Radiology Department, Hopital Saint-Joseph, 185 Rue Raymond Losserand, 75014 Paris, France.

Background: Intracardiac masses are relatively rare but the diagnosis can be challenging for the cardiologist and the clinical presentation can be misleading. While most of the cardiac masses are benign, malignant masses are mostly metastatic tumours.

Case Summary: An 81-year-old man was admitted to the cardiology department for congestive heart failure with the complaint of recent dyspnoea. The initial electrocardiogram was suggestive of a late presentation of an anterior myocardial infarction. Blood test showed mild and stable elevation of troponin and brain natriuretic peptide. Doppler-echocardiography revealed an interventricular septal thickening. Contrast echocardiography revealed a mass with a possibly necrotic centre and peripheral hypervascularization. Cardiac computed tomography (CT) confirmed the existence of a cardiac tumour with a hypodense centre and also revealed the presence of a large tumour of the lung's left lower lobe with multiple enlarged lymph nodes associated with possible left adrenal gland metastasis. Computed tomography-guided percutaneous biopsy of the pulmonary mass demonstrated a squamous cell lung cancer which was likely the primary cancer. The patient was discharged home waiting for chemotherapy to start but died a few days later at home of an unknown cause.

Discussion: Diagnosis of intracardiac mass is difficult, often requiring multiple imaging modalities. Contrast-enhanced echocardiography may help early diagnosis and can be easily implemented with other imaging modalities such as cardiac magnetic resonance imaging or CT.
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http://dx.doi.org/10.1093/ehjcr/ytab164DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8189305PMC
May 2021

Male Sex Is Associated With Cervical Artery Dissection in Patients With Fibromuscular Dysplasia.

J Am Heart Assoc 2021 Jun 17;10(11):e018311. Epub 2021 May 17.

Department of Neurology Inserm U1237 Normandie UniversitéUniversité Caen NormandieCHU Caen Normandie Caen France.

Background Cervical artery dissection (CeAD) is a frequent manifestation of fibromuscular dysplasia (FMD). However, risk factors for CeAD are unknown. We investigated factors associated with CeAD in the ARCADIA (Assessment of Renal and Cervical Artery Dysplasia) registry. Methods and Results The ARCADIA registry includes women or men aged ≥18 years, with a diagnosis of renal, cervical, or intracranial artery FMD, who were prospectively recruited at 16 university hospitals in France and Belgium. Diagnosis of acute or past CeAD at inclusion was established on imaging according to standard diagnostic criteria. Associations between potential determinants and CeAD were assessed by logistic regression analyses. Among 469 patients (75 men) with FMD, 65 (13.9%) had CeAD. Patients with CeAD were younger, more likely to be men, have a history of migraine, and less likely to have a history of hypertension than patients without CeAD. In the multivariable analysis, male sex (odds ratio [OR], 2.66; 95% CI, 1.34-5.25), history of migraine (OR, 1.90; 95% CI, 1.06-3.39), age ≥50 years (OR, 0.41; 95% CI, 0.23-0.73), history of hypertension (OR, 0.35; 95% CI, 0.20-0.64), and involvement of ≥3 vascular beds (OR, 2.49; 95% CI, 1.15-5.40) were significantly associated with CeAD. To validate the association between CeAD and sex, we performed a systematic review. We collected additional data on sex from 2 published studies and unpublished data from the US Registry for Fibromuscular Dysplasia and the European/International FMD Registry. In the pooled analysis (289 CeAD, 1933 patients), male sex was significantly associated with CeAD (OR, 2.04; 95% CI, 1.41-2.95; =0%). Conclusions In patients with FMD, male sex and multisite involvement are associated with CeAD, in addition to other previously known risk factors. Registration URL: https://www.clinicaltrials.gov; Unique identifier: NCT02884141.
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http://dx.doi.org/10.1161/JAHA.120.018311DOI Listing
June 2021

Renal Outcome and New-Onset Renal and Extrarenal Dissections in Patients With Nontrauma Renal Artery Dissection Associated With Renal Infarction.

Hypertension 2021 Jul 10;78(1):51-61. Epub 2021 May 10.

Hypertension Unit (A.-L.F., G.B., A.L., M.A., L.A.), AP-HP, Hôpital Européen Georges Pompidou, Paris, France.

[Figure: see text].
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http://dx.doi.org/10.1161/HYPERTENSIONAHA.120.16540DOI Listing
July 2021

Rare loss-of-function mutations of PTGIR are enriched in fibromuscular dysplasia.

Cardiovasc Res 2021 Mar;117(4):1154-1165

Department of Radiology, Assistance-publique-hôpitaux de Paris, Hopital Européen Georges Pompidou, F-75015 Paris, France.

Aims: Fibromuscular dysplasia (FMD) and spontaneous coronary artery dissection (SCAD) are related, non-atherosclerotic arterial diseases mainly affecting middle-aged women. Little is known about their physiopathological mechanisms. We aimed to identify rare genetic causes to elucidate molecular mechanisms implicated in FMD and SCAD.

Methods And Results: We analysed 29 exomes that included familial and sporadic FMD. We identified one rare loss-of-function variant (LoF) (frequencygnomAD = 0.000075) shared by two FMD sisters in the prostaglandin I2 receptor gene (PTGIR), a key player in vascular remodelling. Follow-up was conducted by targeted or Sanger sequencing (1071 FMD and 363 SCAD patients) or lookups in exome (264 FMD) or genome sequences (480 SCAD), all independent and unrelated. It revealed four additional LoF allele carriers, in addition to several rare missense variants, among FMD patients, and two LoF allele carriers among SCAD patients, including one carrying a rare splicing mutation (c.768 + 1C>G). We used burden test to test for enrichment in patients compared to gnomAD controls, which detected a putative enrichment in FMD (PTRAPD = 8 × 10-4), but not a significant enrichment (PTRAPD = 0.12) in SCAD. The biological effects of variants on human prostaclycin receptor (hIP) signalling and protein expression were characterized using transient overexpression in human cells. We confirmed the LoFs (Q163X and P17RfsX6) and one missense (L67P), identified in one FMD and one SCAD patient, to severely impair hIP function in vitro.

Conclusions: Our study shows that rare genetic mutations in PTGIR are enriched among FMD patients and found in SCAD patients, suggesting a role for prostacyclin signalling in non-atherosclerotic stenosis and dissection.
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http://dx.doi.org/10.1093/cvr/cvaa161DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7983006PMC
March 2021

Four-dimensional Flow MRI: Principles and Cardiovascular Applications.

Radiographics 2019 May-Jun;39(3):632-648. Epub 2019 Mar 22.

From the Departments of Medical Imaging (A.A., N.C., G.A., S.S., V.M., M.Z.), Cardiology (P.G.), and Vascular Medicine (A.S.), Saint Joseph Hospital, 185 rue Raymond Losserand, 75014 Paris, France; and Department of Pediatric Cardiology, Necker Enfants Malades Hospital, Paris, France (D.S.).

In-plane phase-contrast (PC) imaging is now a routine component of MRI of regional blood flow in the heart and great vessels. In-plane PC MRI provides a volumetric, isotropic, time-resolved cine sequence that enables three-directional velocity encoding, a technique known as four-dimensional (4D) flow MRI. Recent advances in 4D flow MRI have shortened imaging times, while progress in big-data processing has improved dataset pre- and postprocessing, thereby increasing the feasibility of 4D flow MRI in clinical practice. Important technical issues include selection of the optimal velocity-encoding sensitivity before acquisition and preprocessing of the raw data for phase-offset corrections. Four-dimensional flow MRI provides unprecedented capabilities for comprehensive analysis of complex blood flow patterns using new visualization tools such as streamlines and velocity vectors. Retrospective multiplanar navigation enables flexible retrospective flow quantification through any plane across the volume with good accuracy. Current flow parameters include forward flow, reverse flow, regurgitation fraction, and peak velocity. Four-dimensional flow MRI also supplies advanced flow parameters of use for research, such as wall shear stress. The vigorous burgeoning of new applications indicates that 4D flow MRI is becoming an important imaging modality for cardiovascular disorders. This article reviews the main technical issues of 4D flow MRI and the different parameters provided by it and describes the main applications in cardiovascular diseases, including congenital heart disease, cardiac valvular disease, aortic disease, and pulmonary hypertension. RSNA, 2019 See discussion on this article by Ordovas .
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http://dx.doi.org/10.1148/rg.2019180091DOI Listing
March 2020

Presumption of pericardial endometriosis using MRI: Case report and review of the literature.

J Gynecol Obstet Hum Reprod 2019 Jan 4;48(1):71-73. Epub 2018 Jul 4.

Department of Radiology, Fondation Hôpital St Joseph, 185, rue Raymond-Losserand, 75014 Paris, France.

Endometriosis is a condition where hormonal-responsive endometrial tissue grows outside the uterus usually within the pelvic cavity. Extra-pelvic endometriosis is rare and may involve the pericardium. We report the first case of pericardial endometriosis suspected using cardiac magnetic resonance imaging.
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http://dx.doi.org/10.1016/j.jogoh.2018.06.009DOI Listing
January 2019

Intra-aortic chord: A new entity?

J Thorac Cardiovasc Surg 2018 07 6;156(1):355-356. Epub 2018 Apr 6.

René Descartes University, Paris, France; Department of Cardiovascular Surgery, Georges Pompidou European Hospital, Paris, France.

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http://dx.doi.org/10.1016/j.jtcvs.2018.02.003DOI Listing
July 2018

Cause of renal infarction: a retrospective analysis of 186 consecutive cases.

J Hypertens 2018 03;36(3):634-640

Hypertension Unit.

Background: Renal infarction can cause abrupt and severe hypertension and less frequently renal failure. Renal infarction results from disruption of renal blood flow in the main ipsilateral renal artery or in a segmental branch. Underlying mechanism is either general, 'embolic' or 'thrombophilic', or local related to primary 'renal artery lesion'. It depends on various causes. In absence of an identified cause, renal infarction is classified as 'idiopathic'. Previous studies report a significant number of 'idiopathic' renal infarction.

Objective: The aim of this study was to analyze various renal infarction causes.

Methods: Between July 2000 and June 2015, 259 consecutive patients with renal infarction were admitted to our hospital center and retrospectively identified from weekly multidisciplinary round. Main clinical and biological characteristics were extracted from clinical data warehouse. Renal imaging was reviewed by two readers unaware of the diagnosis.

Results: Of 259 initially identified patients, 30 were excluded owing to a lack of imaging or clinical data and 43 because iatrogenic renal infarction. In the 186 studied patients, dissection was observed in 76 patients (40.8%) and occlusion in 75 (40.3%). Renal infarction mechanisms were 'renal artery lesion' (n = 151; 81.2%), 'embolic' (n = 17; 9.1%), 'thrombophilic' (n = 11; 5.9%) and 'idiopathic' (n = 7; 3.8%). Predominant renal artery lesions were atherosclerosis disease (n = 52; 34.4%) followed by dissecting hematoma (n = 35; 23.2%) and fibromuscular dysplasia (n = 29; 19.2%). Right and left kidneys were equally involved.

Conclusion: Renal artery lesion is the most frequent cause of renal infarction. This result underlines the need for extensive arterial exploration to identify the renal infarction mechanism and, in case of renal artery lesion, the underlying vascular disease.
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http://dx.doi.org/10.1097/HJH.0000000000001588DOI Listing
March 2018

Abdominal Aortic Calcifications Influences the Systemic and Renal Hemodynamic Response to Renal Denervation in the DENERHTN (Renal Denervation for Hypertension) Trial.

J Am Heart Assoc 2017 Oct 10;6(10). Epub 2017 Oct 10.

INSERM CIC1418 Assistance Publique-Hôpitaux de Paris Hôpital Européen Georges Pompidou, Paris, France

Background: The DENERHTN (Renal Denervation for Hypertension) trial confirmed the efficacy of renal denervation (RDN) in lowering daytime ambulatory systolic blood pressure when added to standardized stepped-care antihypertensive treatment (SSAHT) for resistant hypertension at 6 months.

Methods And Results: This post hoc exploratory analysis assessed the impact of abdominal aortic calcifications (AAC) on the hemodynamic and renal response to RDN at 6 months. In total, 106 patients with resistant hypertension were randomly assigned to RDN plus SSAHT or to the same SSAHT alone (control group). Total AAC volume was measured, with semiautomatic software and blind to randomization, from the aortic hiatus to the iliac bifurcation using the prerandomization noncontrast abdominal computed tomography scans of 90 patients. Measurements were expressed as tertiles. The baseline-adjusted difference in the change in daytime ambulatory systolic blood pressure from baseline to 6 months between the RDN and control groups was -10.1 mm Hg (=0.0462) in the lowest tertile and -2.5 mm Hg (=0.4987) in the 2 highest tertiles of AAC volume. Estimated glomerular filtration rate remained stable at 6 months for the patients in the lowest tertile of AAC volume who underwent RDN (+2.5 mL/min per 1.73 m) but decreased in the control group (-8.0 mL/min per 1.73 m, =0.0148). In the 2 highest tertiles of AAC volume, estimated glomerular filtration rate decreased similarly in the RDN and control groups (=0.2640).

Conclusions: RDN plus SSAHT resulted in a larger decrease in daytime ambulatory systolic blood pressure than SSAHT alone in patients with a lower AAC burden than in those with a higher AAC burden. This larger decrease in daytime ambulatory systolic blood pressure was not associated with a decrease in estimated glomerular filtration rate.

Clinical Trial Registration: URL: http://www.clinicaltrials.gov. Unique identifier: NCT01570777.
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http://dx.doi.org/10.1161/JAHA.117.007062DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5721886PMC
October 2017

Systemic Artery to Pulmonary Vein Fistula After Right Upper Lobectomy Demonstrated by 4-Dimensional Flow Magnetic Resonance Imaging.

Ann Thorac Surg 2017 Aug;104(2):e169-e171

Department of Thoracic Surgery, Paris Descartes University, Georges Pompidou European Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Sorbonne Paris Cité University, Paris Descartes University, Paris, France. Electronic address:

Postoperative systemic artery to pulmonary vein fistula is very rare. In this report, we describe an exceptional condition of both intrapulmonary arteriovenous fistula and systemic artery to pulmonary vein fistula, involving all right hemithoracic systemic arteries, inducing left-to-left shunt. This condition was responsible for heart failure, 24 years after a right upper lobectomy for inflammatory tumor. Investigations included computed tomographic angiography, arteriography, and four-dimensional flow magnetic resonance imaging. Differential diagnosis and management are discussed.
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http://dx.doi.org/10.1016/j.athoracsur.2017.02.061DOI Listing
August 2017

High Prevalence of Multiple Arterial Bed Lesions in Patients With Fibromuscular Dysplasia: The ARCADIA Registry (Assessment of Renal and Cervical Artery Dysplasia).

Hypertension 2017 09 17;70(3):652-658. Epub 2017 Jul 17.

Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Hypertension Unit (P.-F.P., M.A., L.A.); Department of Radiology (A.A., E.M.), Department of Genetics (X.J.), and Department of Clinical Epidemiology (G.C.); INSERM CIC1418, Paris (M.A., G.C.); Department of Neurology (C.O.) and INSERM U894, Hôpital Sainte-Anne (C.O.), Paris; Faculté de Médecine, Université Paris-Descartes (P.-F.P., M.A., L.A., A.A., E.M., X.J., G.C., C.O.); Department of Cardiology, CHU de Grenoble (J.-P.B., O.O.), Department of Radiology (F.T.), and INSERM U1039 (J.-P.B.), France; Hypertension Unit, CHU Timone, Marseille, France (F.S.); Department of Radiology, CHU Gabriel-Montpied, Clermont-Ferrand, France (L.B.); Hypertension Unit, CHU Rangueil, Toulouse, France (B.B.); Hypertension Unit, Hôpital Cardiologique, Lille, France (C.M.-V.); Normandie Université, University of Caen Normandy, INSERM U919, CHU de Caen, Unité Neurovasculaire, France (E.T.); Department of Vascular Medicine (C.L.H.), Caen, France; Hôpital Saint André, Bordeaux, France (P.G.); Pole of Cardiovascular Research, Institut de Recherche Expérimentale et Clinique (A.P.); Division of Cardiology (A.P.) and Division of Radiology (F.H.), Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium.

Fibromuscular dysplasia (FMD) commonly affects the renal and cervical arteries but has been described to affect other vascular beds as well. The prevalence of and clinical characteristics associated with multisite FMD (string-of-beds or focal stenoses affecting at least 2 vascular beds) are not known. In the prospective ARCADIA registry (Assessment of Renal and Cervical Artery Dysplasia), symptomatic patients with renal artery (RA) FMD underwent tomographic- or magnetic resonance-angiography from the aortic arch to the intracranial arteries and those with cervical FMD from the diaphragm to the pelvis. Of 469 patients (84.0% women), 225 (48.0%) had multisite FMD. In addition, 86 of 244 patients with single-site disease had dissections or aneurisms affecting other vascular beds, totaling 311 patients (66.3%) with lesions in >1 vascular bed. Among patients with a cerebrovascular presentation, the prevalence of RA lesions was higher in patients with than in those without hypertension (odds ratio, 3.4; 95% confidence interval, 1.99-6.15). Among patients with a renal presentation, the prevalence of cervical lesions was higher in patients with bilateral than in those with unilateral RA lesions (odds ratio, 1.9; 95% confidence interval, 0.99-3.57). In conclusion, FMD is a systemic arterial disease. At least 2 vascular beds were affected by dysplastic stenoses in 48.0% of cases and by dysplastic stenoses, aneurysms, and dissections in 66.1% of cases. RA imaging should be proposed to hypertensive patients with a cerebrovascular presentation. Cervical artery imaging should be considered in patients with a renal presentation and bilateral RA lesions.

Clinical Trial Registration: URL: www.Clinicaltrials.gov. Unique identifier: NCT02884141.
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http://dx.doi.org/10.1161/HYPERTENSIONAHA.117.09539DOI Listing
September 2017

Asymptomatic aortic coarctation diagnosed because of large abdominal arterial collateral.

Vasc Med 2017 08 19;22(4):347-348. Epub 2017 Mar 19.

4 Groupe hospitalier Paris Saint-Joseph, Paris, Île-de-France, France.

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http://dx.doi.org/10.1177/1358863X17692423DOI Listing
August 2017

Head-to-head comparison of the diagnostic performance of coronary computed tomography angiography and dobutamine-stress echocardiography in the evaluation of acute chest pain with normal ECG findings and negative troponin tests: A prospective multicenter study.

Int J Cardiol 2017 Aug 2;241:463-469. Epub 2017 Mar 2.

University Paris Descartes, Assistance Publique-Hôpitaux de Paris, European Georges Pompidou Hospital, Department of Radiology, and INSERM 970 PARCC, Paris, France. Electronic address:

Objective: To perform a head-to-head comparison of coronary CT angiography (CCTA) and dobutamine-stress echocardiography (DSE) in patients presenting recent chest pain when troponin and ECG are negative.

Methods: Two hundred seventeen patients with recent chest pain, normal ECG findings, and negative troponin were prospectively included in this multicenter study and were scheduled for CCTA and DSE. Invasive coronary angiography (ICA), was performed in patients when either DSE or CCTA was considered positive or when both were non-contributive or in case of recurrent chest pain during 6month follow-up. The presence of coronary artery stenosis was defined as a luminal obstruction >50% diameter in any coronary segment at ICA.

Results: ICA was performed in 75 (34.6%) patients. Coronary artery stenosis was identified in 37 (17%) patients. For CCTA, the sensitivity was 96.9% (95% CI 83.4-99.9), specificity 48.3% (29.4-67.5), positive likelihood ratio 2.06 (95% CI 1.36-3.11), and negative likelihood ratio 0.07 (95% CI 0.01-0.52). The sensitivity of DSE was 51.6% (95% CI 33.1-69.9), specificity 46.7% (28.3-65.7), positive likelihood ratio 1.03 (95% CI 0.62-1.72), and negative likelihood ratio 1.10 (95% CI 0.63-1.93). The CCTA: DSE ratio of true-positive and false-positive rates was 1.70 (95% CI 1.65-1.75) and 1.00 (95% CI 0.91-1.09), respectively, when non-contributive CCTA and DSE were both considered positive. Only one missed acute coronary syndrome was observed at six months.

Conclusions: CCTA has higher diagnostic performance than DSE in the evaluation of patients with recent chest pain, normal ECG findings, and negative troponine to exclude coronary artery disease.
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http://dx.doi.org/10.1016/j.ijcard.2017.02.129DOI Listing
August 2017

A neuropathological study of cerebrovascular abnormalities in a signal transducer and activator of transcription 3-deficient patient.

J Allergy Clin Immunol 2015 Nov 30;136(5):1418-21.e1-5. Epub 2015 Jun 30.

Sorbonne Paris Cité, Paris Descartes University, Imagine Institute, Paris, France; Centre de référence des déficits immunitaires héréditaires (CEREDIH), Necker Children's Hospital, Assistance Publique-Hôpitaux de Paris (APHP), Paris, France; Study Centre for Primary Immunodeficiencies, Necker Children's Hospital, Assistance Publique-Hôpitaux de Paris (APHP), Paris, France; Laboratory of Human Genetics of Infectious Diseases, Necker Branch, INSERM UMR 1163, IMAGINE Institute, Paris, France.

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http://dx.doi.org/10.1016/j.jaci.2015.05.021DOI Listing
November 2015

Age-specific changes in left ventricular diastolic function: a velocity-encoded magnetic resonance imaging study.

Eur Radiol 2015 Apr 28;25(4):1077-86. Epub 2014 Nov 28.

Sorbonne Universités, UPMC Univ Paris 06, UMR 7371, UMR_S 1146, Laboratoire d'Imagerie Biomédicale, 75013, Paris, France.

Objectives: Our objectives were to assess the ability of phasecontrast MRI (PC-MRI) to detect sub-clinical age-related variations of left ventricular (LV) diastolic parameters and thus to provide age-related reference ranges currently available for echocardiography but not for MRI-PC, and to identify independent associates of such variations.

Methods: We studied 100 healthy volunteers (age = 42 ± 15years, 50 females) who had MRI with simultaneous blood pressure measurements. LV mass and volumes were assessed. Semiautomated analysis of PC-MRI data provided: 1) early transmitral (Ef) and atrial (Af) peak filling flow-rates (ml/s) and filling volume (FV), 2) deceleration time (DT), isovolumic relaxation time (IVRT), and 3) early myocardial longitudinal (E') peak velocity.

Results: MRI-PC diastolic parameters were reproducible as reflected by low coefficients of variations (ranged between 0.31 to 6.26 %). Peak myocardial velocity E' (r = -0.63, p < 0.0001) and flow-rate parameters were strongly and independently associated to age (Ef/Af:r = -0.63, DT:r = 0.46, IVRT:r = 0.44, Ef/FV:r = -0.55, Af/FV:r = 0.56, p < 0.0001). Furthermore, LV relaxation parameters (E', DT, IVRT), were independently associated to LV remodelling (LV mass/end-diastolic volume) and myocardial wall thickness (p < 0.01).

Conclusions: PC-MRI age-related reference ranges of diastolic parameters are provided. Such parameters might be useful for a fast, reproducible and reliable characterization of diastolic function in patients referred for clinical MRI exam

Key Points: • MRI age-related reference values of left ventricular diastolic parameters are provided. • MRI diastolic parameters can characterise sub-clinical age-related variations in healthy individuals. • Diastolic function would complement cardiac MRI exam with currently neglected data. • Diastolic function would enhance MRI diagnostic value in cardiomyopathy and heartfailure.
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http://dx.doi.org/10.1007/s00330-014-3488-zDOI Listing
April 2015

Cardiac structure and function in Cushing's syndrome: a cardiac magnetic resonance imaging study.

J Clin Endocrinol Metab 2014 Nov 5;99(11):E2144-53. Epub 2014 Aug 5.

Faculté de Médecine (P.K., S.S., S.B., J.Y., P.C.), Univ Paris-Sud, F-94276 Le Kremlin Bicêtre, France; Faculté de Médecine (A.R., N.K.), Sorbonne Universités, Université Pierre et Marie Curie Univ Paris 06, F75006 Paris, France; Faculté de Médecine (E.M.), Université Paris Descartes, F75006 Paris, France; Service d'Endocrinologie et des Maladies de la Reproduction (P.K., S.S., J.Y., P.C.) and Service de Pharmacogénétique (S.B.), Biochimie Moléculaire et Hormonologie, Hôpital de Bicêtre, Assistance Publique-Hôpitaux de Paris, F-94275 Le Kremlin Bicêtre, France; Département d'Imagerie Cardiovasculaire (A.R.) and Service d'Endocrinologie (C.J.), Hôpital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, F75013 Paris, France; Service de Radiologie Cardiovasculaire (C.R., L.M., A.A., Z.R., E.M.), Hôpital Européen George Pompidou, Assistance Publique-Hôpitaux de Paris, F75015 Paris, France; Service d'Endocrinologie (L.G.), Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, F75006 Paris, France; U693 (P.K., J.Y., S.B., P.C.), Institut National de la Santé et de la Recherche Médicale (INSERM), F-94276 Le Kremlin Bicêtre, France; Unité Mixte de Recherche 7371 and Unité Mixte de Recherche en Santé 1146 (A.R., N.K.), Laboratoire d'Imagerie Biomédicale, ICAN Imaging Core Lab, INSERM, F-75013 Paris, France.

Background: Patients with Cushing's 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 Cushing's syndrome by means of cardiac magnetic resonance, currently the reference modality in assessment of cardiac geometry and function.

Methods: Eighteen patients with active Cushing's 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 Cushing's 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: Cushing's syndrome is associated with subclinical biventricular and LA systolic dysfunctions that are reversible after treatment. Despite skeletal muscle atrophy, Cushing's syndrome patients have an increased LV mass, reversible upon correction of hypercortisolism.
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http://dx.doi.org/10.1210/jc.2014-1783DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4223435PMC
November 2014

[Unusual tumor of the inferior vena cava].

Ann Pathol 2014 Jun 10;34(3):247-51. Epub 2014 May 10.

Service d'anatomie et de cytologie pathologiques, hôpital européen Georges-Pompidou, 20, rue Leblanc, 75015 Paris, France; Université Paris Descartes, 75005 Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.annpat.2014.03.005DOI Listing
June 2014

Rapid onset of peripheral artery disease in a chronic myeloid leukemia patient without prior arterial disorder: direct relationship with nilotinib exposure and clinical outcome.

Eur J Haematol 2015 Apr 23;94(4):363-7. Epub 2014 Aug 23.

Médecine Vasculaire, Hôpital Européen Georges-Pompidou, AP-HP, Paris, France; Sorbonne Paris Cité, Université Paris Descartes, Paris, France; INSERM U970, PARCC, Paris, France.

The second-generation tyrosine kinase inhibitor (TKI) of the BCR-ABL1 oncoprotein nilotinib used in patients with chronic myeloid leukemia is suspected to increase the risk of arterial occlusion, especially in patients with pre-existing cardiovascular risk factors or established cardiovascular diseases. Here, we describe a case of unexpected and rapid onset of symptomatic peripheral artery disease (PAD) associated with silent stenosis of digestive and renal arteries in a nilotinib-treated patient devoid of significant cardiovascular diseases (CVD) risk factor, prior atherosclerotic disease, or other cause of arterial damage. This is the first report to establish a direct relationship between nilotinib exposure and PAD and to reveal that arterial damage is irreversible despite rapid drug withdrawal. However, functional outcome was favorable upon rapid TKI replacement, specific cardiovascular disease management, and development of collateral arterial network.
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http://dx.doi.org/10.1111/ejh.12367DOI Listing
April 2015

Fulminant human herpesvirus 6 myocarditis in an immunocompetent adult: role of cardiac magnetic resonance in a multidisciplinary approach.

Circulation 2013 Dec;128(23):e445-7

Departments of Cardiovascular Imaging (G.A., L.M., A.A., E.M.,), Pathology (P.B.), and Cardiology (A.L., N.D.), Hôpital Européen George Pompidou, Université Paris Descartes, Paris, France; Medical and Molecular Virology Unit and EA-4684 (CardioVir), University Hospital and Medical School, Reims, France (L.A.); and the Department of Cardiovascular Imaging, Pitié Salpêtrière Hospital, Pierre et Marie Curie University, INSERM U678 and ICAN Imaging Core Lab, Paris, France (A.R.).

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http://dx.doi.org/10.1161/CIRCULATIONAHA.113.001801DOI Listing
December 2013

Recurrent tamponade and aortic dissection in syphilis.

Ann Thorac Surg 2013 Nov;96(5):e111-3

Department of Vascular Medicine, European Hospital Georges Pompidou, Paris, France; PARCC IMSERM, University Paris Descartes, Paris, France. Electronic address:

Syphilitic cardiovascular disease has been described since the 19th century, mainly on autopsy series. Major clinical manifestations are aortic aneurysm, aortic insufficiency, and coronary ostial stenosis. The diagnosis of syphilitic cardiovascular disease is based mainly on positive serologic tests and overt clinical manifestations. We present here a rare and unusual clinical presentation of a tertiary syphilis with recurrent tamponade and type B aortic dissection, whose positive diagnosis was made by polymerase chain reaction on pericardial fluid analysis.
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http://dx.doi.org/10.1016/j.athoracsur.2013.05.096DOI Listing
November 2013

Partial anomalous pulmonary venous return in adults with prior curative congenital heart surgery detected by cross-sectional imaging techniques.

Int J Cardiol 2013 Oct 8;168(4):e109-10. Epub 2013 Sep 8.

Cardiovascular Imaging Department, AP-HP, Hôpital Européen Georges Pompidou, Université Paris Descartes, Paris, France; Adult Congenital Heart Disease Unit, AP-HP, Hôpital Européen Georges Pompidou, Université Paris Descartes, Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.ijcard.2013.07.098DOI Listing
October 2013

An egg-shaped cyst of the right interventricular septum.

Circulation 2013 Jun;127(23):2351-2

Interventional Cardiology Department, European Hospital George Pompidou, 20 Leblanc St, 75015 Paris, France.

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http://dx.doi.org/10.1161/CIRCULATIONAHA.113.001630DOI Listing
June 2013

Association of smoking with phenotype at diagnosis and vascular interventions in patients with renal artery fibromuscular dysplasia.

Hypertension 2013 Jun 22;61(6):1227-32. Epub 2013 Apr 22.

Hypertension Unit, Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Paris, France.

The pathogenesis of fibromuscular dysplasia (FMD) remains unclear, but tobacco use is thought to be involved. This retrospective cross-sectional study aimed to evaluate smoking first as a risk factor for renal artery FMD diagnosis and second as a modifier of the clinical and radiological phenotype of this disease. We retrieved 337 adult patients diagnosed with FMD in a referral center for hypertension management, who were first individually matched to controls with essential hypertension for sex, age, systolic blood pressure, number of antihypertensive drugs, and year of visit. Smoking status and other relevant data were collected at first visit. The proportion of current smokers was higher for patients with FMD than for the controls (30% and 18%, respectively, P<0.001; odds ratio, 2.5 [95% confidence interval, 1.6-3.9]). Second, characteristics of FMD were compared between current smokers and other patients. Among patients with multifocal FMD, current smokers experienced an earlier diagnosis of hypertension (36 versus 42 years, respectively; P<0.001) and FMD (43 versus 51 years; P<0.001) than other patients, and a greater likelihood of renal artery interventions (57% versus 31%; P<0.001) and of kidney asymmetry (21% versus 4%; P=0.001). In conclusion, current smoking is associated with a higher likelihood of renal artery FMD diagnosis. Rather than a higher incidence of FMD, this may reflect a more aggressive course in smokers, who have earlier hypertension leading to increased and earlier recognition of the disease. Smoking cessation should be strongly encouraged in patients with FMD.
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http://dx.doi.org/10.1161/HYPERTENSIONAHA.111.00838DOI Listing
June 2013

Association between 2 angiographic subtypes of renal artery fibromuscular dysplasia and clinical characteristics.

Circulation 2012 Dec 15;126(25):3062-9. Epub 2012 Nov 15.

Assistance Publique-Hoˆpitaux de Paris, Hoˆpital Europe´en Georges-Pompidou, Hypertension Unit, Paris, F-75015, France.

Background: Initially based on histology, the diagnosis of renal artery fibromuscular dysplasia (FMD) is now based mostly on angiographic appearance because arterial tissue samples are rarely available. This retrospective cross-sectional study aimed to assess the clinical relevance of a binary angiographic classification of FMD lesions (unifocal or multifocal) based on computed tomographic or magnetic resonance angiography.

Methods And Results: Adult patients diagnosed with FMD in a single tertiary care center for hypertension management were identified by screening of electronic files. FMD lesions were reviewed and classified according to computed tomography or magnetic resonance angiography as multifocal if there were at least 2 stenoses in the same arterial segment; otherwise, they were classified as unifocal. Of 337 patients with established renal artery FMD, 276 (82%) were classified as multifocal. Patients with unifocal and multifocal lesions differed significantly in median age at diagnosis of FMD (30 and 49 years) and hypertension (26 and 40 years), sex distribution (female:male ratio, 2:1 and 5:1), initial blood pressure (157/97 and 146/88 mm Hg), current smoking (50% and 26%), prevalence of unilateral renal artery lesions (79% and 38%), presence of kidney asymmetry (33% and 10%), renal revascularization procedures (90% and 35%), and hypertension cure rates in patients who underwent revascularization (54% and 26%).

Conclusions: A binary angiographic classification into unifocal or multifocal renal artery FMD is straightforward and discriminates 2 groups of patients with different clinical phenotypes.
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http://dx.doi.org/10.1161/CIRCULATIONAHA.112.117499DOI Listing
December 2012

Frequent and widespread vascular abnormalities in human signal transducer and activator of transcription 3 deficiency.

Circ Cardiovasc Genet 2012 Feb 14;5(1):25-34. Epub 2011 Nov 14.

Hematology Department, Necker Children's Hospital, Assistance Publique Hôpitaux de Paris, 149 rue de Sèvres, Paris, France.

Background: Signal transducer and activator of transcription 3 (STAT3) deficiency is responsible for autosomal dominant hyperimmunoglobulin E syndrome, characterized by recurrent bacterial and fungal infections, connective tissue abnormalities, hyperimmunoglobulin E, and Th17 lymphopenia. Although vascular abnormalities have been reported in some patients, the prevalence, characteristics, and etiology of these features have yet to be described.

Methods And Results: We prospectively screened 21 adult STAT3-deficient patients [corrected] (median age, 26 years; range, 17-44 years) [corrected] for vascular abnormalities. We explored the entire arterial vasculature with whole-body magnetic resonance imaging angiography, coronary multislice computed tomography, and echo-tracking-based imaging specifically for the [corrected] carotid arteries. We also assayed for serum biomarkers of inflammation and endothelial dysfunction. Finally, we studied murine models of aortic aneurysm in the presence and absence of inhibitors of STAT3-dependent signaling. Ninety-five percent of patients showed brain abnormalities (white matter hyperintensities, lacunar lesions suggestive of ischemic infarcts, and atrophy). We reported peripheral and brain artery abnormalities in 84% of the patients and detected coronary artery abnormalities in 50% of the patients. The most frequent vascular abnormalities were ectasia and aneurysm. The carotid intima-media thickness was markedly decreased, with a substantial increase in circumferential wall stress, indicating the occurrence of hypotrophic arterial remodeling in this STAT3-deficient population. Systemic inflammatory biomarker levels correlated poorly with the vascular phenotype. In vivo inhibition of STAT3 signaling or blockade of IL-17A resulted in a marked increase in aneurysm severity and fatal rupture in mouse models.

Conclusions: Vascular abnormalities are highly prevalent in patients with STAT3 deficiency. This feature is consistent with the greater susceptibility to vascular aneurysm observed after inhibition of STAT3-dependent signaling in mouse models.
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http://dx.doi.org/10.1161/CIRCGENETICS.111.961235DOI Listing
February 2012

Atrio-esophageal fistula complicating esophageal achalasia.

Interact Cardiovasc Thorac Surg 2011 Aug 12;13(2):211-3. Epub 2011 May 12.

Department of Cardiovascular Surgery, Assistance publique des Hôpitaux de Paris, Paris, France.

A 75-year-old male, known to have achalasia, was admitted to the intensive care unit with massive upper gastrointestinal bleeding and sepsis. He had a history of purulent pericarditis 18 months earlier. He also presented with atrial fibrillation associated with a cerebral transient ischemic accident two months earlier. A contrast computed tomography scan showed an atrio-esophageal fistula with active extravasation of contrast. He was operated on via a median sternotomy, and the defects in the atrial wall, inferior vena cava and diaphragm were closed using pericardial patches. An esophagectomy was to be performed 24 hours later, but the patient died from septic shock and multiple organ failure before his second procedure.
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http://dx.doi.org/10.1510/icvts.2011.267849DOI Listing
August 2011

[Renal artery fibromuscular dysplasia].

Presse Med 2011 Jul-Aug;40(7-8):720-5. Epub 2011 Apr 16.

Université Paris-Descartes, 75270 Paris cedex 06, France.

Fibromuscular dysplasia (FMD) encompasses a heterogeneous group of idiopathic, segmental, nonatherosclerotic diseases of the musculature of arterial walls, leading to the narrowing of small and medium-sized arteries. The most common locations of FMD are renal arteries and carotid arteries. The diagnosis of FMD is made on the "string of beads" appearance of the arteries. The French Health Authority recommends performing a CT scan or an MRA to assess the diagnosis of FMD. A recent meta-analysis showed the cure rates using current definitions of hypertension cure are only 36% and 54% after angioplasty and surgery, respectively.
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http://dx.doi.org/10.1016/j.lpm.2011.02.033DOI Listing
September 2011

Cardiac magnetic resonance demonstrating an isolated apical diverticulum of the left ventricle revealed by ventricular tachycardia.

Eur Heart J 2011 Apr 14;32(8):1039. Epub 2010 Dec 14.

Département de Radiologie, Hôpital Européen Georges Pompidou, 21 rue Leblanc, 75015 Paris, France.

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http://dx.doi.org/10.1093/eurheartj/ehq459DOI Listing
April 2011
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