Δημήτρης Τσιάπρας MD FESC ΩΝΑΣΕΙΟ ΚΑΡΔΙΟΧΕΙΡΟΥΡΓΙΚΟ ΚΕΝΤΡΟ

Δημήτρης Τσιάπρας MD FESC ΩΝΑΣΕΙΟ ΚΑΡΔΙΟΧΕΙΡΟΥΡΓΙΚΟ ΚΕΝΤΡΟ

Αποφυγή ισομετρικής άσκησης Χημειοπροφύλαξη Β-αναστολέα επί αισθήματος παλμών Συζήτηση περί πιθανότητας χειρουργικής επιδιόρθωσης στον κατάλληλο χρόνο

Classic Non Classic BILEAFLET Posterior ML

The most common valve disease (2% to 3% in the general population). MVP may be familial or sporadic Complications: Mitral Regurgitation, Atrial Fibrillation, Congestive Heart Failure, Endocarditis, Stroke Risk factors for cardiac morbidity Age 50 years, Left atrial enlargement, Mitral Regurgitation, Flail Leaflet, Atrial Fibrillation Delling FN. Circulation. 2014;129:2158 2170, Marks AR. N Engl J Med. 1989;320:1031 1036, Avierinos JF. Circulation. 2002;106:1355 1361, Kligfield P. Am Heart J. 1987;113:1298 1307 Vohra J. PACE 1993;16:387 393.

Rate of Sudden Cardiac Death in MVP ranges from 0.2% -0.4%/y in prospective follow-up studies Left ventricular dysfunction resulting from severe MR identifies a patient subgroup at high risk of SCD. However, life-threatening ventricular arrhythmias also occur in patients with MVP with trivial or absent mitral regurgitation. Delling FN. Circulation. 2014;129:2158 2170, Marks AR. N Engl J Med. 1989;320:1031 1036, Avierinos JF. Circulation. 2002;106:1355 1361, Kligfield P. Am Heart J. 1987;113:1298 1307 Vohra J. PACE 1993;16:387 393.

47 individuals (1.3%) had classic and 37 (1.1%) had nonclassic MVP, estimated overall prevalence of 2.4%. With respect to sex, MVP was equally distributed between men and women. MVP was described as a benign entity : 1 patient (1.2%) had atrial fibrillation, 1 patient (1.2%) had cerebrovascular disease 3 patients (3.6%) had syncope Young (<50 years of age), medically treated patients presenting with normal left ventricular function and no symptoms have excellent survival, even with severe MR. Delling F. Circulation 2014;129:2158-70, Freed LA. N Engl J Med 1999;341:1-7, Avierinos JF. Circulation 2002;106:1355-1361

24 with idiopathic out-of-hospital cardiac arrest. Bileaflet MVP was found in 10 (42%). Over a median 1.8 years from ICD placement, 13 of 24 patients (54%) received appropriate ventricular fibrillation terminating ICD shocks. Only bileaflet MVP was associated with VF recurrences requiring ICD therapy on followup. Sriram C. J Am Coll Cardiol 2013;62:222 30

Sriram C. J Am Coll Cardiol 2013;62:222 30

Small subset of patients, recently described as bileaflet MVP syndrome predominantly women, with bileaflet prolapse, complex ventricular ectopy, and abnormal T waves Basso C. Circulation. 2015;132:556 566 Sriram CS. J Am Coll Cardiol. 2013;62:222 230, Faisal F. Syed. Circ Arrhythm Electrophysiol. 2016;9:e004005

1982-2013, all the hearts of SCD victims 40 years of age studied. 43/650 (7%) SCD cases were selected in whom MVP caused by myxomatous valve disease was the only cardiac abnormality found at autopsy. 30 consecutive patients referred to the Cardiology Clinic with complex ventricular arrhythmias (1/2010 12/2013) Cristina Basso et al. Circulation. 2015;132:556-566

The 12-lead basal ECG at the time of admission to the emergency department for palpitations. Single and coupled ventricular premature beats with RBBB morphology are present; note the negative T wave on the inferior leads. Nonsustained VT also recorded on the 24-hour Holter ECG. Myxomatous degeneration of both leaflets of the mitral valve with elongated chordae is visible on gross examination. Histology shows severe myxoid thickening of the posterior mitral valve leaflet and myocardial fibrosis of the left ventricular inferobasal wall and papillary muscle. Cristina Basso et al. Circulation. 2015;132:556-566

Myocardial scarring is visible at the level of the inferobasal LV free wall under the posterior mitral valve leaflet Myocardial scarring at the papillary muscles plus adjacent free wall. Close-up of the scarring areas showing patchy replacement-type fibrosis with interspersed cardiomyocytes. Cristina Basso et al. Circulation. 2015;132:556-566

Cristina Basso et al. Circulation. 2015;132:556-566

A and B, A 30-year-old woman with mitral valve prolapse and complex ventricular arrhythmias. LGE of the papillary muscle is visible on mid shortaxis view. Nonsustained ventricular tachycardia with RBBB morphology originating from the posterior papillary muscle (superior axis). C and D, A 33-year-old woman with mitral valve prolapse and complex ventricular arrhythmias. LGE of the left ventricular inferobasal region under the posterior valve leaflet with endocardial-midmural extension. Nonsustained ventricular tachycardia with RBBB morphology originating from the left ventricular inferobasal wall near the mitral annulus(inferior axis). E and F, A 38-year-old man with mitral valve prolapse and aborted sudden cardiac death. CMR performed 6 months before cardiac arrest shows LGE in the LV inferobasal region. ECG recording of polymorphic supraventricular tachycardia degenerating into ventricular fibrillation. Cristina Basso et al. Circulation. 2015;132:556-566

All patients had at least 1 left ventricular papillary or fascicular VE focus. Purkinje origin Ventricular Ectopy was identified as the VF trigger in 6 of 6 cardiac arrest patients (4 from papillary muscle). Faisal F. Syed. CircArrhythm Electrophysiol. 2016;9:e004005

Ventricular Late Potentials higher in MVP patients Raised catecholamine levels and receptor upregulation Khan MA. Professional Med J 2015; 22:227-234, Hu X.irc J 2014;78:1486-93 Bobkowski CW.Cardiol Young 2002;12:333

Chenni S. Sriram et al. J Am Coll Cardiol 2013;62:222 30 Bileaflet mitral valve thickening and mitral valve prolapse T-wave changes in the inferior leads Frequent ventricular ectopic activity with PVBs of outflow tract origin alternating with papillary muscle or fascicular origin.

TRIPLEX DISEASE : Ασθένεια με κακή πρόγνωση!!! ΠΡΟΠΤΩΣΗ ΜΙΤΡΟΕΙΔΟΥΣ Η ανίχνευση μέσα σε μεγάλο πληθυσμό καλοήθους πρόγνωσης των ασθενών με κακή πρόγνωση και η κατάληλη αντιμετώπισή τους αποτελεί κλινική πρόκληση!! 12 lead ECG High Resolution ECG Holter monitoring CMR. Αποτελούν πολύτιμα κλινικά εργαλεία.