+ Mitral Valve Prolapse A Surgeon’s Perspective Charles Anderson, M.D. Saint Joseph’s Hospital of Atlanta

+ Mitral Valve Prolapse The most common cause of mitral regurgitation is MVP Most commonly associated with myxomatous degeneration (50% of primary MR in the US) Myxomatous degeneration and MVP is more common in women, and is more common in advancing age, but men over the age of 50 are most likely to present with complications of MVP Interestingly, according to a study by the Mayo Clinic, women had less posterior prolapse, less flail segments, more leaflet thickening and less frequent severe MR Women with severe MR were less likely to have surgical repair despite having the same survival advantage with repair as men Survival without repair in severe MR was worse for women than men

+ Mitral Valve Prolapse Myxomatous degeneration is a connective tissue disorder resulting in an accumulation of dermatan sulfate within the connective tissue matrix of the valve Results in thickening and stretching of the leaflets of the valve and the chordae tendineae Prevents the leaflets from fully coapting when the valve is closed, causing the valve to prolapse into the left atrium and can lead to mitral regurgitation

+ Mitral Valve Prolapse – previously over diagnosed True MVP probably occurs in less than 3% of the population Most people have no symptoms and don't need treatment Most are able to lead normal, active lives

+ Mitral Valve Prolapse Carpentier classification = Type II (excess leaflet motion) The most common diseases that cause degenerative mitral valve disease are Barlow’s disease and FED (etiology) The lesions in degenerative valve disease that result in the Type II dysfunction are usually chordal elongation or rupture Annular dilatation is almost always present in the myxomatous form Modified from Carpentier A, Adams DH, Filsoufi F. Carpentier’s Reconstructive Valve Surgery. From Valve Analysis to Valve Reconstruction Saunders Elsevier.

+ Degenerative Valve Disease MVP patients fall into the spectrum of degenerative valve disease Degenerative valve disease includes excess leaflet motion (type II) Barlow’s Myxomatous degeneration (multisegmental disease) Fibroelastic deficiency (usually with single or few chordal ruptures) Pure annular dilatation (type I) Modified from Carpentier A, Adams DH, Filsoufi F. Carpentier’s Reconstructive Valve Surgery. From Valve Analysis to Valve Reconstruction Saunders Elsevier.

+ Barlow’s Disease Typical MVP patients usually have a Barlow’s valve with myxomatous degeneration Lesions resulting from myxoid degeneration include; Leaflet thickening Large redundant leaflets Chordal elongation or rupture Annular dilatation As the degenerative process often affects the entire valve, patients with Barlow’s disease generally have complex valve pathology and dysfunction, which is most often multisegmental

+ Mitral Valve Prolapse - Regurgitation Very few people who have MVP will need surgery to repair their mitral valves (~5%) Only those who develop MV regurgitation will fall into this group Regurgitation can be from a severely prolapsing leaflet or a flail leaflet due to ruptured chordae or papillary muscle

+ But when regurgitation is present… Based on existing data, it appears that if surgery is undertaken before onset of symptoms and where LV function is preserved, the life expectancy should be similar to that of the general population When significant symptoms of heart failure have developed (NYHA III – IV) before surgery is undertaken, the long term survival is significantly reduced, regardless of the LV function Similarly, patients with an impaired EF at time of surgery have a reduced long-term survival

+ Mitral Valve Regurgitation – Repair early if MR Risk of postop LV dysfunction along with the feasibility of repair, has led to the recommendation that surgical correction be performed early in the course of MR when valve repair can predictably be performed

+ Which MVP pts develop MR? ECHO characteristics

+ MVP that is most likely to develop regurgitation Prolapsed mitral valves are classified into several subtypes, based on leaflet thickness, concavity, and type of connection to the mitral annulus Subtypes can be described as classic, nonclassic, symmetric, asymmetric, flail, or non-flail Thickening of the mitral leaflets >5 mm and leaflet displacement >2 mm indicates classic mitral valve prolapse

+ MVP that is most likely to develop regurgitation Patients with asymmetric prolapse are susceptible to severe deterioration of the mitral valve, with the possible rupture of the chordae tendineae and the development of a flail leaflet

+ Natural History of MR with flail leaflet The outcome of MR due to flail leaflet treated medically shows excess mortality with a high incidence of cardiovascular complications and an increased risk of late death among older patients, those with advanced symptoms, and those with a reduced EF at diagnosis Death or the need for surgery is almost unavoidable within 10 years after the diagnosis Surgical correction of MR improves long-term survival (N Engl J Med 1996;335: )

+ Data on Repair A small sampling

+ Comparison of Early Surgery Versus Conventional Treatment in Asymptomatic Severe Mitral Regurgitation Duk-Hyun Kang, MD, PhD et al. (Circulation. 2009;119: ) MV repair can correct the pathology thereby preserving continuity of the MV apparatus and LV systolic function As prediction of MV repair has become clinically feasible, and MV repair has shown excellent long-term results, there is a need to compare clinical outcomes of early surgery vs. the conventional treatment strategy of watchful waiting

+ Methods , 447 consecutive asymptomatic pts (50 +/- 15 yrs) with severe degenerative MR (prolapse or flail) and preserved LVF were evaluated prospectively End point was defined as the composite of operative mortality, cardiac death, repeat mitral valve surgery, and urgent admission due to CHF during follow-up Early surgery was performed on 161 pts, and the conventional treatment strategy was used for 286 pts There were no significant differences between the 2 groups in terms of age, gender, euro SCORE or EF

+ Results During a median follow-up of 5.4 yrs; 2 repeat surgeries and no cardiac deaths or operative mortality in the operated group 12 cardiac deaths, and 22 admissions for congestive heart failure in the conventional treatment group Estimated actuarial 7-year cardiac mortality rate was 0% in the operated group and 52% in the conventional treatment group (P 0.008)

+ Conclusions Compared with conservative management, early surgery was associated with an improved long-term event rate by decreasing cardiac mortality and congestive heart failure hospitalization in patients with severe MR Early surgery may therefore further improve clinical outcomes in asymptomatic severe MR with preserved left ventricular systolic function and a high likelihood of mitral valve repair

+ Long-term survival in asymptomatic patients with severe degenerative mitral regurgitation: a propensity score-based comparison between an early surgical strategy and a conservative treatment approach Montant P, et al. J Thorac Cardiovasc Surg Dec;138(6): Study to assess the long-term survival, incidence of cardiac complications, factors that predict outcome, and effect of mitral surgery on the long-term prognosis of patients with asymptomatic severe MR amenable to valve repair

+ Methods 192 asymptomatic pts (mean age 63 +/- 13 yrs) with severe degenerative MR diagnosed between were prospectively followed for a median of 8.5 yrs

+ Results Cardiovascular, and event-free survival was evaluated in 2 groups of patients: "conservative approach” (n = 67) and an "early surgery” (n = 125) 10-year overall survival was significantly lower with the conservative approach than early surgery (50% vs 86%, log-rank < )

+ Conclusion Outcome of asymptomatic patients with severe degenerative mitral regurgitation is better with an early surgical approach rather than a more conservative treatment strategy

+ How Durable is Repair? Will it last long enough if we send them early or should we wait till symptoms force us to act?

+ Durability of repair In studies looking at the long-term durability of mitral repair, over 90% of degenerative valve repairs are still effective 20 years later if the prolapse involved one leaflet If the prolapse involves both leaflets then this number drops to just over 80% The main reason that repairs fail over time is that degenerative disease can affect the remaining valve tissue

+ Durability of mitral valve repair for degenerative disease AM Gillinov et al. J Thorac Cardiovasc Surg 1998;116: Mitral valve repair is applicable in the majority of degenerative valve patients and has become the procedure of choice Study to asses durability of repair for degenerative disease , 1072 pts underwent isolated mitral valve repair for MR caused by degenerative disease Durability was assessed by multivariable risk factor analysis of reoperation

+ Results At 10 years, freedom from reoperation was 93% Among 30 patients who required reoperation, the repair had failed in 16 (53%) as a result of progressive degenerative disease Durability was adversely affected by pathology other than posterior leaflet prolapse, use of chordal shortening, annuloplasty alone, and posterior leaflet resection without annuloplasty

+ Conclusions Repair durability is greatest in patients with isolated posterior leaflet prolapse who have posterior leaflet resection and annuloplasty Chordal shortening, annuloplasty alone, and leaflet resection without annuloplasty jeopardize late results

+ Repair > Replacement Numerous studies have compared long term-survival of pts undergoing mitral valve repair or replacement A survival benefit is consistently shown with mitral valve repair The ‘repair rate’ is thus an important variable Overall replacement rate in degenerative disease may be as high as 50% In a review of United States practice in 1999 and 2000, only 42.4% of patients having isolated mitral valve surgery for regurgitation had a valve repair (all etiologies of mitral disease) Savage EB, et al. Use of mitral valve repair: analysis of contemporary United States experience reported to the Society of Thoracic Surgeons National Cardiac Database. Ann Thorac Surg 2003 March;75(3):820-5.

+ Thank You Questions?