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Flail Leaflet

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  • 1. Flail Mitral Leaflet
  • 2. Mitral Valve Prolapse - General Considerations The n e w e ng l a n d j o u r na l of m e dic i n e• A Epidemiology Aortic valve • Likely occurs in around 2 to 5 percent of the Left coronary population artery Right fibrous trigone• Terminology Left fibrous Bundle of His • Normally the mitral valve billows slightly into trigone the LA - if this is exaggerated it is called a AC Anterior leaflet PC billowing mitral valve Circumflex • artery Floppy valve is an extreme form of billowing Posterior leaflet Coronary Annulus • sinus Flail leaflet occurs with a ruptured chordae B C• Annulus MVP is characterized by myxomatous degeneration Leaflet • In younger patient’s it manifests as excess leaflet tissue with diffuse, generalized thickening AC PC Secondary cord A3 of the valve -> Barlow’s Syndrome A1 Primary cord A2 P3 • P1 In older patients, the prolapsing mitral valve P2 tends to have thickening to an isolated area -> Papillary muscle fibroelastic dysplasia• Increasing severity of MR imposes volume load on Figure 1. The Mitral Valve. the LV resulting ventricular dilation, hypertrophy, The mitral valve has anterior and posterior leaflets, which are separated by the anterior commissure (AC) and the and heart failure Braunwald et al. p1565-7 posterior commissure (PC) (Panel A). The leaflets are inserted on the circumference of the mitral annulus, which is in continuity with the aortic annulus and the left and right fibrous trigones. The circumflex coronary artery, coronary Foster, E NEJM 2010 sinus, aortic valve, and bundle of His are all close to the mitral valve. Panel B shows the mitral-valve leaflets, each of which usually consists of three discrete segments or scallops. These are designated A1, A2, and A3 for the anterior leaflet and P1, P2, and P3 for the posterior leaflet. The valve leaflets each receive chordae tendineae from the anter-
  • 3. The Ruptured Chordae• In Western countries, flail mitral leaflet is The n e w e ng l a n d j o u r na l of m e dic i n e the most common cause of mitral regurgitation requiring surgical correction• Abnormalities of the chordae are important causes of MR • May be congenitally abnormal • Causes of rupture • Spontaneous (primary) • Infective endocarditis • Trauma • Osteogenesis imperfecta • Relapsing polychondritis • Acute LV dilation • No identifiable case is apparent other than increased mechanical Figure 2. Echocardiographic Evidence of Rupture of the Elongated Chordae. strain Panel A, in the parasternal long-axis view, shows severe displacement of the posterior leaflet (arrow). Panel B, • a Doppler color-flow echocardiogram, shows severe mitral regurgitation. Panel C shows the M mode of the mitral Chordae in the posterior leaflet valve with color flow superimposed, indicating holosystolic mitral regurgitation (arrow). Panel D, a transesophageal echocardiogram, shows a flail posterior mitral leaflet (ML, arrow). LA denotes left atrium, and LV left ventricle. rupture more frequently than the Foster, E NEJM 2010 anterior leaflet Baxley, Wi Circulation 1973 veloped countries who have native-valve endo- the onset of atrial fibrillation may have an abrupt 11
  • 4. Death from cardiac cause 31 21 4 33 7 4.3 25 9 32 11 0.10 Outcome in patients with flail leaflet Congestive heart failure 55 30 4 63 8 8.2 53 10 82 14 0.015 Chronic atrial fibrillation† 13 8 3 30 12 2.2 30 16 29 17 0.13 CLINICAL OUTCOME OF MITRAL REGURGITATION DUE TO FL AIL LEAFLET Thromboembolism 13 12 3 12 3 1.9 10 3 19 9 0.36 Hemorrhage 3 1 1 3 2 0.4 2 2 8 8 0.62 Endocarditis 10 5 2 8 3 1.5 9 4 0 0.45• Study Design Mitral-valve surgery Mitral-valve surgery or death 143 188 57 69 3 82 4 90 2. 20.0 26.3 71 7 93 6 0.001 3 TABLE 3 OUTCOME AT 80AND 10 YEARS WITH MEDICAL TREATMENT OF MITRAL 5 5 96 4 0.001 • 229 patients with flail leaflet diagnosed btw Janin1980 and Dec Outcome subgroups of patients REGURGITATION DUE TO FLAIL LEAFLET.* 1989 at Mayo Clinic had clinical follow-upDeath obtained data 10-YEAR RATE ACCORDING through 1994-5 NYHA class III or IV NYHA class I or II‡ 86 EVENT — 18 4 9 33 9 34.0 4.1 OVERALL POPULATION TO NYHA CLASS LINEAR- • Ejection fraction 60% 24 4 39 8 5.3 Exclusion criteria - papillary muscle rupture, previous valve IZED Ejection fraction 60%§ 47 11 60 12 11.3 NO. OF 5-YEAR 10-YEAR YEARLY P Congestive heart failure I II surgery, associated aortic or congenital heart disease Left atrial diameter 18 5 59 12 5.4 EVENTS RATE RATE RATE CLASS CLASS VALUE 30 mm/m2 ‡ percent• Results Left atrial diameter 30 mm/m2 47 9 75 10 Death from any cause Death from cardiac cause 14.5 45 31 28 4 21 4 43 7 33 7 6.3 4.3 32 25 9 9 34 32 11 11 0.26 0.10 • Baseline Characteristics *Plus–minus values are means SE. NYHACongestive heart failure denotes New York Heart Association. 30 4 63 8 55 Chronicat risk fibrillation† atrial fibrillation,8and all 30 12 †The 175 patients presenting in sinus rhythm were atrial for chronic 13 3 229 8.2 2.2 53 30 10 16 82 29 14 17 0.015 0.13 • 82 % of patients had a history of murmur or cardiac Thromboembolism patients were at risk for all the other end points. ‡P 0.001 as compared with the higher category.Hemorrhage 13 3 12 3 1 1 12 3 3 2 1.9 0.4 10 2 3 2 19 8 9 8 0.36 0.62 symptoms > 3 months Endocarditis 10 5 2 8 3 1.5 9 4 0 0.45 §P 0.034 as compared with the higher category. • Mitral-valve surgery 143 57 3 82 4 20.0 71 7 93 6 0.001 77% of patients did not have an identifiable cause Mitral-valve surgery or death 188 69 3 90 3 26.3 80 5 96 4 0.001 Outcome in subgroups of • 82% had posterior leaflet involvement Death patients • NYHA class III or IV 86 9 — 34.0 87% of patient had grade 3 or 4 MR In the multivariate analysis (Table 3), the base-line NYHA class I 100 or II‡ 18 4 33 9 4.1 variables that were independently predictive of sur- Ejection fraction 60%§ Ejection fraction 60% 24 4 39 8 5.3 • Overall Outcomes vival were age, NYHA class, and ejection fraction. Congestive heart failure80 47 11 60 12 11.3 Expected Survival (%) Figures 2 and 3 show the Kaplan–Meier survival Left atrial diameter 18 5 59 12 5.4 30 mm/m260 • Long term survival with medical treatment was shorder curves according to NYHA class and ejection frac- Left atrial diameter tion, respectively. 30 mm/m240 ‡ 47 9 75 10 14.5 Observed than expected survival The incidence of congestive heart failure was *Plus–minus values are means SE. NYHA denotes New York Heart Association. • 20 The rate of death 30 4 cardiac causes was 21+4 percent at 10 †The 175 patients presenting in sinus rhythm were at risk for chronic P 0.016 from percent at 5 years and 63 8 percent atrial fibrillation, and all 229 years (Fig. 4). 10 years at 5 years and 33+7 percent at Multivariate predictors of the devel- patients were at risk for all the other end points. 0 opment of congestive heart failure were age, ejection ‡P 0.001 as compared with the higher3 4 5 6 7 8 9 10 0 1 2 category. • Multivariate analysis showed that age, NYHA class, and for body- fraction, and left atrial diameter adjusted surface area (Table 3). Of the 55 patients with a first §P 0.034 as compared with the higher category. Years after Diagnosis EF were independent predictors of survival the diagnosis of mitral episode of heart failure after NO. AT RISK 229 133 115 103 84 70 52 34 21 12 7 • Surgery regurgitation, 27 (49 percent) underwent surgery. Most of the remaining patients had symptomatic Figure 1. Long-Term Survival with Medical Treatment, as Com- pared with Expected Survival, in 229 Patients with Mitral Re- In the multivariate analysis (Table 3),to Flail Leaflet. gurgitation Due the base-line 100 • Occurred in 62% of patients with a medicalof 23+32 butthat were independently predictive of sur- improvement with mean treatment, variables these pa- tients nevertheless had a higher mortality rate than 80 months vival were age, NYHA class, and ejection fraction. urvival (%) those without an episode of heart failure (adjusted Figures 2 and 3 show the Kaplan–Meier survival 60 • hazard ratio, 16.53; 95 percent confidence interval, NYHA class and ejection frac- 5 yr survival was 79% and31.36; P 0.001). 66% that according to 8.72 to 10 yr survival was curves Ling, LH NEJM 1996 was not different than expected survival were tion, respectively. at Of the 175 patients who in The incidence of congestive heart failure was sinus rhythm 40
  • 5. AF Per AF present 2.40 0.97–5.95 0.059 *There were 347 patients in the model. The risk of sudden death AF atrial fibrillation; CI confidence interval; EF ejection fraction; NYHA New York Heart Association. II and 7.8% in class III or IV (p 0.0001) (Fig. 2). fibrillation at diagnosis (p 0.0004) (Fig. 4). Among the 25 However, of the 25 patients with SUD, 10 were in func- patients with SUD, 16 (64%) were in sinus rhythm at• Study Designclass I at was in class wereAmong the 5 were in class baseline and 13patients who until SUD. SUD (20%) pre- tional III and only 1 diagnosis, 9 IV. in class II, 19 patients in class I or II at baseline, 10 (five in each class) had worsening Overall, five remained so experienced • sented no evidence at any time during their follow-up of Pts first diagnosedSUD. These patients did not undergo atrial fibrillation,anddysfunction or severe symptoms,not undergo of symptoms before with flail mitral leaflet btw Jan 1980 LV Dec 1994 who did until surgical correction within one rapidly withof diagnosis surgery because the symptoms improved monthof treat- SUD. ment and were not considered disabling. The rate SUD Associated treatment—SUD. No significant differences • was higher in functional class II than in class I (p 0.01). Exclusion criteria - papillary muscle rupture, previous valve surgery, associated during or However, this difference was confined to patients with were detected comparing the medical treatment aortic follow-up of group I versus group II or III with respect to congenital heart disease and EF (p 60%0.002).consid- angiotensin-converting enzyme inhibitors, calcium channel either atrial fibrillation or EF 60% only patients in sinus rhythm were When blockers, beta-blockers, digoxin, hydralazine, class I antiar-• ered, yearly rates of SUD in patients in functional class II Results and I were not different (0.5% and 0.9%, respectively; p 0.60; average 0.8%). The linearized rate of SUD in patients rhythmic agents, diuretics or nitrates (all p 0.10). How- ever, when compared with survivors, group I patients were • Duringfunctional class I or II, in sinus rhythm, with EF 60% more+often months, 27% 0.001), calcium died and 7% of in a mean medical follow-up of 48 months and with no history of CAD was also 0.8% per year. 41 taking digoxinp (68%of patient channel diuretics (80% vs. 28%; vs. 43%; p 0.008), patient suffered fromrates of SUDdeath with a blockers (28% vs. 11%; p 0.001) and nitrates (16% vs. 5%; The yearly linearized sudden in patients baseline EF 50% were 12.7%, 0.9% for an EF 50% to 59% p 0.03) and were less often taking beta-blockers (4% vs. • By multivariate 60% (p 0.0001) (Fig. 3).predictors of sudden death were functional class, EF, and 1.5% for an EF analysis baseline Among 17%; p 0.09). development SUD,2EF at diagnosis was infibrillation In patients who underwent the operation, SUD occurred the 25 patients with (68%), 50% to 59% in of CHF, and atrial 60% in 17 postoperatively in seven, leading to a total number of 32 (8%) and 50% 6 (24%). • Echocardiography, repeated in five patients within six SUDs in the cohort. In a multivariate proportional hazards Occurs SUD, showed a decrease of EF under 60% in year and accounts for one-fourthof SUD, deaths under months of in a linearized rate of 1.8% per analysis that included the significant predictors of all conservativepatient.EF was confirmed in functional surgery performed at any time (time-dependent variable) and 50% in one management only one patient, whereas Of the 19 patients 60% in three independently and favorably influenced the incidence of • class I or II who had SUD, 15 had an EF 60% (eight in class I and seven in class risk The yearly linearized rates of SUD in patients in sinus unexpected death (adjusted hazard ratio 0.29 [95% CI 0.11 In the absence ofII). factors there remains a 0.8% riskThis effect persistedsudden entire to 0.72], p 0.007). per year of when the death cohort (n 468) was analyzed (p 0.0001) and when the • Surgery was associated with a reduction in the rate of sudden death (p 0.007) rhythm was 1.3%, whereas it was 4.9% in patients with atrial Grigioni et al. JACC 1999 Figure 2. Relation between New York Heart Association Figure 3. Relation between left ventricular ejection fraction
  • 6. Surgery and Flail LeafletEarly Surgery in Patients with Mitral Regurgitation Due to Flail Leaflet, Circulation 1997• Study Design • 221 patients (mean age 65+13 years, 71% males) with flail leaflets diagnosed from 1980-9 at Mayo Clinic • Patients divided in 2 grps • Grp 1 63 patients who had early surgery • Grp II 158 patients initially treated conservatively (80 of which were operated on later Outcomes in Mitral Regurgitation Due to Flail Leaflets, JACC 2008 • Study Design • 394 patients enrolled from 4 European centers (mean age 64 + 11 years, 67% men) • Study enrolled patients between 1988 and 2004 with a median follow up of 3.9 years • Eligibility criteria • Presence of echo diagnosed flail leaflet • Exclusion of ischemic MR (incliding pap Outcome in all 394 Outcomes in 102 muscle rupture, AV disease, congenital asymptomatic patients disease or mitral stenosis patients with EF>60%