La Dysplasie Fibromusculaire Une cause sous-estime dhypertension - PowerPoint PPT Presentation

La Dysplasie Fibromusculaire Une cause sous-estimée d’hypertension secondaire Alexandre Persu, M.D.-PhD Service de Cardiologie Cliniques Universitaires Saint-Luc Université Catholique de Louvain Brussels, Belgium

HTA secondaires: Pourquoi les rechercher? Rares mais… - Causes curables - Traiter maladie sous-jacente - Suivi imagerie Chez qui? - HTA sévère - HTA résistante - Enfants et adolescents non-obèses - Signes cliniques/biol. évocateurs (exemple: hypokaliémie)

Prévalence des causes secondaires d’hypertension en cas d’HTA résistante Pedrosa R P et al. Hypertension 2011;58:811-817

Etiologie des sténoses artérielles rénales (1) Athéromatose (SAAR) – Cause fréquente (90% des cas) – Âge: le plus souvent après 45-50 ans – Atteinte proximale (ostiale, post-ostiale) – Facteurs de risque cardiovasculaire présents – Autres lits vasculaires souvent atteints par l’athérosclérose – Thrombose artérielle possible – Atrophie rénale possible Courtesy of J-P. Lengelé

Quand rechercher une sténose athéromateuse des artères rénales? • HTA + asymétrie rénale (imagerie) • HTA d’apparition brutale, résistante ou maligne • Aggravation inexpliquée d’une HTA pré-existante surtout si • Insuffisance rénale inexpliquée ou post-IEC/sartan • Athéromatose d’autres lits vasculaires / facteurs de risque • Episodes répétés d’œdème pulmonaire aigu • Jeune femme (= dysplasie fibromusculaire) Plus rare: souffle abdominal, hypokaliémie

Mast and Beutler; J Hypertens 2009; 27:1333-40

HTA rénovasculaire Mise au point simplifiée 1. Écho-Doppler des artères rénales 2. Angio-IRM ou angio-CT • selon fonction rénale et expertise locale 1 er choix si obésité morbide • 3. Artériographie rénale

Meta-analysis of RCTs testing PTA ± stent vs. medical R/alone Caielli et al., NDT 2015; 30: 541-553

Meta-analysis of RCTs testing PTA ± stent vs. medical R/alone Caielli et al., NDT 2015; 30: 541-553

Meta-analysis of RCTs testing PTA ± stent vs. medical R/alone Caielli et al., NDT 2015; 30: 541-553

Consultation (02 12 2005) 51 ans Antécédents • Polyarthrite + diabète sous corticothérapie • Obésité androïde – HTA • Carrefour aorto-bifémoral 06/03 Histoire clinique • 09/05: USI à Marche pour carbonarcose • Mise en évidence IRA (Créat 6.3 mg/dl) • Sédiment pauvre; absence de toxique • 10/05: IRT (Créat 8 mg/dl) : Prise en dialyse

IRM: sténose artérielle rénale serrée bilatérale 24 11 2005

Arguments pour ou contre une angioplastie rénale Pour Contre HTA réfractaire ou maligne Patient âgé, mauvais pronostic global Risque majeur / antécédent de maladie HTA + dégradation significative de la FR sous des emboles de cholestérol ou IEC/Sartans néphropathie induite par le contraste HTA légère et/ou bien contrôlée, SAR à caractère progressif FR peu altérée ; absence d’œdème aigu pulmonaire SAR bilatérale serrée Néphropathie diabétique SAR serrée sur rein unique fonctionnel Protéinurie abondante IRC modérée à sévère / progressive Atrophie rénale < 7.5 cm Œdème aigu du poumon (« flash » OAP) Indices de résistance > 80 % HTA et DFM SAR modérée asymptomatique Pr. J-F De Plaen adapté par A. Persu

Etiologie des sténoses artérielles rénales (2) Dysplasie fibromusculaire (DFM) – Cause moins fréquente ( ∼ 10% des cas) – Affecte le plus souvent les femmes (80%) de 15 à 50 ans – Atteinte distale de l’artère principale ou branches intra-rénales – Autres territoire atteints (30%): carotido-vertébral, intracérébral, … – Formes familiales (≤ 10%) – Thrombose artérielle et atrophie rénale rares « Collier de Focale Tubulaire perles » Multifocale Unifocale Plouin PF. et al. Orphanet J Rare Dis . 2007; 2: 28. Safian RD.,Textor SC., NEJM 2001; 344: 431-442. Trinquart L. et al. Hypertension. 2010; 56: 525-532.

Prevalence of renal FMD Symptomatic FMD (Plouin et al., Orphanet JRD 2007, 2:28) Estimated to ~0.02-0.08% (based on the prevalence of HTN in middle-aged subjects, the prevalence of renovascular HTN in hypertensive patients and the proportion of renal artery stenosis due to FMD). Silent FMD (Hendricks et al., Vascular Medicine 2014;19: 363-367) Meta-analysis of data from kidney donors: ~4% CORAL database: 5.8%

Prevalence of renal FMD Critically depends from: • The characteristics of the population (age, gender ratio, population sample vs. kidney donors vs. hypertensive patients) • The first and second line screening tools (renal Duplex vs. CTA/MRA) • The awareness of the radiologist/caring physician

Provisional recommendations for screening • Recommended in hypertensive patients aged < 50 years, especially women and/or patients with severe/resistant hypertension • CTA (or if CI MRA) preferable to renal Duplex as first- line test, especially if high diagnostic probability/ low expected performance of renal Duplex • Increase awareness of radiologists and clinicians! Persu et al., FMD deserves to be revisited, submitted 2016

Differences according to the radiological classification CTA or MRA Angiographic Multifocal, 276 Focal, 61 p Men 47 (17%) 19 (31%) 0.02 Age at diagnosis, y 49 [42, 58] 30 [25, 39] <0.01 Clinical Bilateral stenoses 171 (62%) 13 (21%) <0.01 Small kidney 19 (10%) 16 (33%) <0.01 Interventions* 50 (35%) 28 (90%) <0.01 * Among patients with a FU ≥1 year Savard S et al, Circulation 2012;126:3062

Screening in a hypertensive patient Incidental finding

Screening in a hypertensive patient Incidental finding

Smoking cessation should be strongly encouraged in patients with FMD Savard S, … Plouin PF, Steichen O et al. Hypertension . 2013;61:1227-32.

FMD-related renal artery stenosis Indications of revascularisation • HTN of recent onset • Medical treatment failure • Renal function degradation (especially after administration of a RAS inhibitor) • Renal size reduction Persu et al. J Hypertens. 2014; 32:1367-78.

Renal FMD is not always a curable disease Meta-analysis: HTN cure rate following PTA Relationship with age Trinquart L. et al. Hypertension 2010; 56: 525-532.

FMD-related renal artery stenosis PTA vs. surgery • Renal PTA is the first-line revascularisation technique. • Stenting is usually not recommended (risk of kinking or stent fracture) • Surgery should be considered in the following cases: Stenosis associated with complex aneurysms Restenosis despite two attempts of PTA Complex lesions of arterial bifurcation or branches Persu et al. J Hypertens. 2014; 32:1367-78.

Screening in a hypertensive patient Incidental finding

Lüscher TF. et al. Nephron , 1986; 44 (suppl.1): 109-114 Varennes et al., Insights Imaging . 2015 ;6: 295-307.

FMD, a systemic disease? N ° vascular beds imaged N ° of patients N ° of patients 2 or more 357 35% FMD of 2 vb 3 or more 292 22% FMD of 3 vb 4 or more 232 9% FMD of 4 vb Renal Extracranial carotid/ arteries vertebral arteries 65% Olin JW, et al. Circulation. 2012; 125:3182-90

Screening for carotid FMD: echography is not enough CT- and MR- angiography are likely to perform better than Doppler in detecting lesions involving the medium and distal thirds of carotid and vertebral arteries. Sharma AM, Kline B. Tech Vasc Interv Radiol. 2014;17:258-63.

In case of hypertension In case of suggestive symptoms or if likely to alter management Screen for cerebral aneurysms if likely to modify management If suggestive symptoms Persu et al., FMD revisited, Hypertension 2016; 68:832-9.

Spontaneous coronary artery dissection Look for renal, iliac and cervical FMD Persu et al., FMD revisited, Hypertension 2016; 68:832-9.

Follow-up No revascularisation • Blood pressure: every 3 months • Creatinine and kidney length: yearly Revascularisation • Blood pressure and creatinine: at 1 month • Renal imaging: 6 months • Subsequent follow-up: see higher

Screening in a hypertensive patient Incidental finding

FMD, a familial disease? ~10% Pannier-Moreau I. et al. J. of Hypertens. 1997; 15: 1797-1801.

Different FMD angiographic subtypes in two sisters Multifocal : string of beads Unifocal : tubular Courtesy of X. Jeunemaitre

2016 Phosphatase and Actin Regulator 1 Associated with migraines and Cervical Artery Dissections Kiando SR,.....Jeunemaitre X, Bouatia-Naji N. .

Take-home messages on FMD • FMD is less rare than previously thought • FMD is not only a disease of young women • FMD is a systemic vascular disease • FMD patients may have a genetic predisposition • PTA does not always cure renal FMD • Stenting is not recommended • FMD is also a disease for cardiologists (SCAD) • FMD deserves to be revisited

BEL-FMD (A.Persu, P. Van der Niepen) H. Heuten W. Vinck T. De Backer P. Verhamme A. Persu P. Van der Niepen J.-C. Wautrecht J.-M. Krzesinski

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