Πρόπτωση μιτροειδούς βαλβίδας και αρρυθμιογένεση: μια ιστορικά (και κλινικά;) ενδιαφέρουσα ανασκόπηση

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1 Πρόπτωση μιτροειδούς βαλβίδας και αρρυθμιογένεση: μια ιστορικά (και κλινικά;) ενδιαφέρουσα ανασκόπηση Γ. ΚΟΥΡΓΙΑΝΝΙΔΗΣ 251 ΓΕΝΙΚΟ ΝΟΣΟΚΟΜΕΙΟ ΑΕΡΟΠΟΡΙΑΣ

2 Ουδεμία σύγκρουση συμφερόντων

3 TIMELINE 1887 midsystolic click Cuffer and Barbillon 1963 Barlow click-murmur syndrome Criley MVP MVP Endemic (5-15 % συχνότητα) 1987 Levine Saddle shape of MV MVP Syndrome Current knowledge 2-3% general population 1% >> Delling et al Circulation. 2015;131: % Turker et al. EurRevMedPharmacolSci 2015;19:

4 Atrial premature beats 35 to 90 % Paroxysmal supraventricular tachycardia 3 to 32 % Ventricular premature beats 58 to 89 % Complex ventricular ectopy 30 to 56 % Atrial Fibrillation -1 to 25 % Sudden Cardiac Death (0,2-0,4%)? Kligfield et al Am Heart J 1987; 113:1298. Schaal et al Cardiovasc Clin 1992; 22:307. Kligfield et al Cardiovasc Clin 1990; 21:143. Babuty et al Pacing Clin Electrophysiol 1994; 17:1090. Zuppiroli et al Am Heart J 1994; 128:919 Berbarie Am J Cardiol 2006; 97:1039.

5 Selection Bias Μη στατιστ. Συσχέτιση/αναφορά της MR Υπερδιάγνωση λόγω διαγν. κριτηρίων τεχνολογίας MVP Syndrome

6 Arch Intern Med;1984:2360 Σύγκριση συμπτωματικών ασθενών με MVP με συμπτωματικούς μάρτυρες Undefined cause Pt Awareness Young women Unrelated to MR Bountoulas et al Hellenic Journal of Cardiology (2016) 57, 73e85

7 Framingham Heart Study 84 pts MVP, vs 3403 controls subjects without MVP. MR trace atrial ectopy (3.2 versus 1.6 percent), atrial fibrillation (1.2 versus 1.7 percent), ventricular ectopy (2.6 versus 1.4) Freed et al. Prevalence and clinical outcome of mitral-valve prolapse N Engl J Med 1999;341:1-7

8 Kligfield et al. Am J Cardiol 1965;55: ) Kligfield et al AHJ May 1987;113(5) 1298 Turker et al Predictors of atrial arrhythmias in patients with mitral valve prolapse. Acta Cardiol Dec;64(6): Turker et al. Predictors of ventricular arrhythmias in patients with mitral valve prolapse. Int J Cardiovasc Imaging 2010; 26:139.

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10 Ινωση και Τάση στους ΘΜ DAEDs trigger reentry VT Wilde et al. Mitral valve prolapse and ventricular arrhythmias: observations in a patient with a 20-year history J Cardiovasc Electrophysiol Mar;8(3):

11 Παρα την καλοηθη πορεια αναφορες για ΑΘ ακομα και με μικρή MR Boudoulas et al.hellenic Journal of Cardiology (2016) 57, 73e85 Vohra et al. Malignant Ventricular Arrhythmias in Patients with MVP and MR (PACE, Vol. 16, March, Part )

12 0,2-0,4% (x2 gp) AUTOPSY STUDIES SCD (0-24%) Basso et al 7% MVP 5% δείγματα (99 από 2007 δείγματα) MVP αιτία θανάτου 17 ασθ (0.8% των περιπτώσεων) F. WALLER et al Cardiac Pathology in 2007 Consecutive Forensic Autopsies Clin. Cardiol. 15, (1992)

13 Narayanan et al Mitral Valve Prolapse and Sudden Cardiac Arrest in the Community Heart Rhythm Feb 2016, Volume 13, Issue 2, Pages SCA 12-year period MVP was observed in 17 (2.3%) prior to the SCA event MR 14 SCA patients with MVP (82.3%) and was moderate or severe in 10 (58.8%).

14 ΑΙΦΝΙΔΙΟΣ ΚΑΡΔΙΑΚΟΣ ΘΑΝΑΤΟΣ vs ΑΡΡΥΘΜΙΚΟΣ ΘΑΝΑΤΟΣ ΣΥΝΥΠΑΡΧΟΥΣΕΣ ΠΑΘΗΣΕΙΣ Ηλεκτρολυτικές Διαταραχές Ρήξη τενόντιας χορδής Ινομυική Δυσπλασία Στεφανιαίων ΑΡΡΥΘΜΙΚΑ ΣΥΝΔΡΟΜΑ Πρώιμη επαναπόλωση Long Qt Γενετικά Σύνδρομα ΑΘ (πχ LAMIN A/C)

15 650 SCD ( )<40yo Autopsy Arm 43 pt (7%) MVP vs 15 controls Bileaflet 70% Fibrosis PM 100% INFBASAL 88% Living Arm (no MR) 30 MVP + Arrh vs 14 MVP Arrh - Bileaflet 70% LGE 93%vs 14% Basso et al.arrhythmic Mitral Valve Prolapse and Sudden Cardiac Death Circulation. 2015;132: Sheppard et al 3680 νεκροτομές 62 MVP (1,7%) 74% FIBROSIS

16 Patchy replacement fibrosis Subendocardial midmural Midapical Fibrous plaque

17 MITRAL ANNULUS DISJUNCTION (MAD) IN LGE MVP Marra et al. Circ Cardiovasc Imaging. 2016;9:e005030

18 Carmo et al. Cardiovascular Ultrasound 2010, 8:53 MAD is a common finding in myxomatous mitral valve disease patients, easily recognizable by transthoracic echocardiography. It is more prevalent in women and often associated with chest pain. MAD significantly disturbs mitral annular function and when severe predicts the occurrence of NSVT.

19 Lancelloti Circ Cardiovasc Imaging. 2016;9:e

20 Primum movens (Stretch) TRIGGER mechanically triggered PVCs SUBSTRATE stretch- and contact induced fibrotic changes Second Hit. MODIFIERS a just-right autonomic milieu, bileaflet and excessively redundant mitral valve tissue, and being young and female Noseworthy Circulation. 2015;132: ARRHYTHMIA/SCD

21 Sudden Cardiac Death, Mitral Valve Prolapse, and Long QT Syndrome The American Journal of Medicine, Vol 128, No 10, October yo fm SCA Long QT after azithromycin Flail posterior leaflet Severe MR EF 45% SCN5A c.3404 G>T DSP c.852 G>A

22 ΣΥΜΠΕΡΑΣΜΑΤΑ Η MVP χαρακτηρίζεται από καλοήθη πορεία Σημαντικότερος παράγοντας αρρυθμιών/αθ η MR Υπάρχουν μηχανισμοί αρρυθμιογένεσης/scd /(dormant) Ειτε με την αναπτυξη αιμοδυναμικής επιβάρυνσης (όψιμα/mr) Ειτε νωρίτερα με την πρώιμη ανάπτυξη ινωτικού υποστρώματος Η πρωτοπαθής πρόληψη του SCD δεν είναι δυνατή με την υπάρχουσα γνώση

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25 MR IS A STRONG PREDICTOR OF MVP ARRHYTHMIAS Grigioni et al. Atrial Fibrillation Complicating the Course of Degenerative Mitral Regurgitation JACC 2002; 40:84 92

26 1,200 patients 24 (16 women) SCAsurv (Bileaflet) MVP 10 (42%). Sriram J Am Coll Cardiol 2013;62: )90% Ꝗ 2)77.8% biphasic or inverted T waves 3) bigeminy ventricular tachycardia PVCs Only MVP was associated with ventricular fibrillation recurrences requiring ICD therapy on follow-up

27 Han et al. Cardiovascular Magnetic Resonance Characterization of Mitral Valve Prolapse (J Am Coll Cardiol Img 2008;1: Papillary muscles Fibrosis Diffuse Fibrosis Bui AH, et al. Diffuse myocardial fibrosis in patients with mitral valve prolapse and ventricular arrhythmia Heart 2017;103:

28 However, in the subgroup of patients with SCD, 40% of patients were categorised as NYHA class I (36). This highlights a subgroup of patients with SCD predominantly due to cardiac arrhythmia and not related to the severity of MR or LV failure in MVP. These patients were mostly young and asymptomatic. The subgroup of patients with NYHA class I had a yearly risk of SCD of 1% (36). This is equal to the overall mortality in hypertrophic obstructive cardiomyopathy (HCM, a pathology considered as one of the most common causes of SCD in young people (37). Consequently, it is logical to identify a highrisk group of MVP similar to the recommendation for primary prevention in HCM.

29 ANS