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Clinical Update: Common Valvular Heart Disease Chowdhury H Ahsan, MRCP, MD, Ph.D. Assistant Professor of Medicine University of California Medical Center Mitral Stenosis Rheumatic (nearly all adult MS) Degenerative (calcification) Congenital (parachute MV) Others: post-inflammatory, metabolic syndromes etc., Etiology Mitral Stenosis Clinical Presentations Asymptomatic Dyspnea, PND, Orthopnea Hemoptysis Atrial fibrillation Systemic embolization Mitral Stenosis Diagnosis Clinical - Loud S1 and P2 (pulmonary hypertension) - mid diastolic rumble - OS indicating pliable leaflets - short OS-S2 interval indicates severe MS - other auscultatory signs as per co-existing disease ECG - P mitrale: broad, notched P wave in II and biphasic in V1 - RVH and rightward axis if significant PHT Mitral Stenosis Diagnosis CXR - LAA and LA enlargement - increased upper lobe vascularity - Kerley B and A lines - dilated PA - MV calcification Mitral Stenosis Management Principles Asymptomatic - no specific therapy - endocarditis prophylaxis - if appropriate, rheumatic fever prophylaxis Mild and Mod MS ( MVA 1.5 sq cm and 1.0 to 1.4 sq cm) - Normal physical activity - No specific therapy, restoration of NSR in case of AFib - restoration of NSR and anticoagulation in case of Afib - intervention if PASP 60 mm of Hg or exertional symptoms Mitral Stenosis : 53 yr old female from East Europe presented with increasing SOB and Fatigue Has Rheumatic Mitral stenosis Exercise Echo followed by Cath was done Mitral Valve: hockey stick appearance indicating Rheumatic Valve Disease Valve area varied between 1.4 to 1.6 Exercise Echo was done Exercise or DSE for evaluation of MS Lower MVA at rest showing more pronounced change in mean diastolic gradient with stress Hecker S et al, AJC 1997 and Cheriex EC et al, Int J Cardiol 1994 Exercise did not change the gradient across MV Not severe MS to account for patients symptoms Simultaneous LV and LA pressure tracing Kaplan-Meier product-moment event rates in patients with mitral stenosis. The cut- off point (18 mm Hg) was derived from the obtained performance of the test according to the presence or absence of clinical events Reis et al, JACC 2004 36 year old male with HIV, HTN, 36 year old male with HIV, HTN, Hepatitis B +C and a history of Hepatitis B +C and a history of cocaine and alcohol abuse is found cocaine and alcohol abuse is found to have a heavily calcified Mitral to have a heavily calcified Mitral valve and Mitral annulus with valve and Mitral annulus with severe Mitral Stenosissevere Mitral Stenosis 2-D Echo showing heavily calcified Mitral valve leaflets and Mitral stenosis 3-D Echo of Mitral Stenosis3-D Echo of Mitral Stenosis LA viewLA viewLV viewLV view 3-D measurement of Mitral valve area3-D measurement of Mitral valve area MVA= 0.914 cmMVA= 0.914 cm 2 2 LA viewLA viewLV viewLV view Real Time TTE of the Mitral Valve LA ABC DEF G Real Time TTE of MS Real Time TTE of MVA in MS Zamarano et al., JACC 2004; 43: Pages 2091 Bland-Altman graphs displaying differences against average values between traditional and real-time three-dimensional (RT3D)-determined mitral valvular area. The thick line represents mean difference, and the thin lines represent the limits of agreement (all measurements in cm2). Real Time TTE of MVA in MS Zamarano et al., JACC 2004; 43: Pages 2091 3-D TEE ALL gated acquistion Superior image resolution Temporal artifacts Intra-operative guidance Surgeons want it in the OR Mitral Stenosis Management Principles Severe MS - is usually symptomatic - Percutaneous mitral commissurotomy (PMC) is the treatment modality of choice in the vast majority - PMC in optimal anatomy has acturial survival rate of 95% after 7 years - PMC in skilled centers has a mortality of 50 at rest or 60 with exercise) in the absence of LA thrombus or moderate to severe MR Patients with NYHA III-IV symptoms, moderate or severe MS and a nonpliable calcified valve who are at high risk for surgery in the absence of LA thrombus or mod/sev MR Class IIb Asymptomatic patients, moderate or severe MS and valve morphology favorable for percutaneous balloon valvotomy who have new onset of AF in the absence of left atrial thrombus or mod/sev MR. Patients in NYHA III-IV, mod or sev MS, and a nonpliable calcified valve who are low-risk candidates for surgery Relative contraindications Left atrial thrombus TEE frequently performed prior to the procedure to rule out thrombus According to Palacios and Vahanian, no consensus regarding thrombus localized in L atrial appendage. Limit to patients with contraindications to surgery or those with urgent need for intervetion Significant MR (3+ to 4+) Determination of echocardiographic score (Wilkins Score) Braunwald: Heart Disease: A Textbook of Cardiovascular Medicine, 6th ed Immediate outcomes of percutaneous valvuloplasty of MV The immediate results of percutaneous mitral valvotomy are similar to those of surgical mitral commissurotomy Mean MVA doubles (from 1.0 cm2 to 2.0 cm2) 50% to 60% reduction in transmitral gradient. Overall, 80% to 95% procedural success (MVA 1.5 cm2 and a decrease in LA pressure to 1.5:1 L-R shunt) in 8 8 to 12 12 P 701959 Poor correlation between Echo score and higher age and outcome Shaw TRD et al, Heart 2003 Aortic Stenosis Most common surgical valve disease in the developed world Degenerative/calcification - most common cause in the industrialized world - under 70 years of age 70 % bicuspid and 15 % tricuspid - over 70 years of age, 50 % tricuspid and 25 % bicuspid Rheumatic - most common cause in the developing world - almost always associated with MV disease Other - associated with other congenital cardiac abnormalities (Co-arctation, VSD, Hypoplastic left heart, etc.,) Etiology Aortic Stenosis Diagnosis Clinical - pulsus parvus et tardus (absent in hypertensives and elderly) - systolic thrill and typical heaving apical impulse - S4 and late peaking ejection systolic murmur - paradoxical split of 2nd HS in severe AS - other auscultatory signs modified by co-existing disease ECG - LVH with strain CXR - dilated ascending aorta (post-stenotic dilatation) - Valve calcification Aortic Stenosis Diagnosis Echo (primary diagnostic modality) - AV anatomy (tricuspid, bicuspid, calcification) - Mild Vs. Moderate Vs. Severe AS - AVA and gradients can be calculated - progression of disease can be monitored - assessment of LV function and coexisting lesions Cath - ususally done to assess coronaries prior to valve surgery - helpful to assess severity in complex situations Aortic Stenosis Management Principles Asymptomatic - no specific therapy - endocarditis prophylaxis - if appropriate, rheumatic fever prophylaxis Mild and Mod AS ( AVA 1.5 sq cm and 1.0 to 1.4 sq cm) - Normal physical activity - No specific therapy, restoration of NSR in case of AFib - approx. progression is a decrease by 0.1 sq cm per year - annual echo follow-up Aortic Stenosis Management Principles Severe AS - usually symptomatic within 2 years - asymptomatic severe AS : no surgery - asymptomatic severe AS: exercise symptoms=?surgery - symptomatic severe AS: surgery - symptomatic severe AS if not operated has an average life expectancy of 2 to 3 years - severe AS with HF has mortality of nearly 100% in 1to 2 years if not operated Nonsurgical (Balloon vavuloplasty) - only a palliative treatment - high risk elderly patients or as an emergent procedure Aortic Stenosis Management Principles AS: Case History 66 yr. Male, s/p CABG 10 yrs back Increasing dyspnea Inferolateral fixed defect, no ischemia Echo: dilated LV, global HK, EF 20% AV- thickened, markedly restricted 3.2 m/sec Severe AS with LV Dysfunction Two Important questions to ask: a. Is there a non-severe aortic stenosis with a small calculated AVA due to or reflecting a low cardiac output in the presence of LV dysfunction unrelated to valve disorder b. Is there sufficient contractile reserve to indicate a potential benefit and a reasonable peri- operative risk that would justify surgery AORTIC STENOSIS WITH POOR LV DYDFUNCTION 67 yr. Male no prior H/O of CAD presented with class III/IV symptoms of CHF and had AS with severe LV systolic dysfunction 67 yr. Male, SOB, h/o CAD No significant change in gradient on dobutamine infusion No evidence of sufficient contractile reserve LVOT velocity on rest (left) LVOT velocity on peak dose dobutamine (right) DSE in AS with LV systolic dysfunction DSE directed AVR showed benefit Schwammenthal E etal, Chest, June, 01 Dobutamine Echo in severe AS With increase in flow, significant increase in AVA in non- severe AS and a fall in valve resistance CRITICAL AS: LOW CARDIAC OUTPUT VELOCITY 4 In-between Dobutamine AVA, Resistance Ribichini, Anotorini-Canterin, Heart, 1999 AV Resistance: Cath: (1.33 X P)/(CO/HR XSEP) Echo: (1.33 X4 Vmax2)/ (LVOT area X Velocity) LOW OUTPUT LOW GRADIENT SEVERE AS: DOES CONTRACTILE RESERVE MATTER? Monin J et al. Circulation 2003 CASE PRESENTATION 46 yrs. Female with SOB, Orthopnea, PND. Rt Heart Cath: PA 55/25 PCWP 30 with prominent V waves (upto 50 mmHg) Lt Heart Cath: EF 70% with minimal CAD TEE shows: Severe Mitral Regurgitation Repair vs. Replacement: Better LV function Improved Survival Survival of patients with severe MR and EF Survival of patients with severe MR and NYHA class Mitral Valve Prolapse Women 20 to 50 years Low BP, orthostatic hypotension, palpitations, chest pain Mid systolic click, maybe mid systolic murmur Echo: - thickened, redundant leaflets - leaflet excursion (prolapse) into LA in systole - redundant chordae tendinae, trivial or mild MR Little progression of MR, Abx prophylaxis Types Mitral Valve Prolapse Men 40 to 70 years Myxomatous and thickened MV Significant leaflelt prolapse Significant MR, progressive MR Complications: Chordal rupture, Afib Endocarditis prophylaxis Surgery for MR often required Types Classic or non-classic combined MVP equal in male and females. More complications in MEN Transthoracic echocardiographic image in parasternal long-axis view, showing posterior mitral leaflet bowing backward and prolapsing into left atrium during systole. LV=left ventricle. LA=left atrium. PML=posterior mitral valve leaflet. Transgastric short axis view of mitral valve with corresponding scallops. Hayek, E et al. Lancet, 2005 Chronic MR Key Points - Asymptomatic severe MR: ususally no surgery - Asymptomatic severe MR: ? surgery if MV repair possible - Symptomatic severe MR is an indication for surgery - LV contractility is abnormal in severe MR even if EF is “normal” - EF 45 mm is an indication to operate - Afterload reduction provides symptomatic relief but ? prevents progression (except in established LV dysfunction) Chronic AR Key Points - Asymptomatic severe AR: ususally no surgery - Symptomatic severe AR is an indication for surgery - EF 55 mm is an indication to operate - Afterload reduction (ACEI and Nifedipine) slow progression of AR - Valve repair is generally not possible unlike MR large-volume, collapsing pulse bounding peripheral pulses; also known as Watsons water hammer pulse low diastolic and increased pulse pressure Corrigans pulse (rapid upstroke and collapse of the carotid artery pulse) de Mussets sign (head nodding in time with the heart beat) Quinckes sign (pulsation of the capillary bed in the nail) Traubes sign (systolic and diastolic murmurs described as pistol shots heard over the femoral artery when it is gradually compressed) Duroziezs sign (a double sound heard over the femoral artery when it is compressed distally) Enriquez-Sarano, M. et al. N Engl J Med 2004;351:1539-1546 Example of a Jet of Aortic Regurgitation, as Shown by Color-Flow Imaging Enriquez-Sarano, M. et al. N Engl J Med 2004;351:1539-1546 Classification of the Severity of Aortic Regurgitation Enriquez-Sarano, M. et al. N Engl J Med 2004;351:1539-1546 Example of Quantitation of Aortic Regurgitation by the Convergence of the Proximal Flow Enriquez-Sarano, M. et al. N Engl J Med 2004;351:1539-1546 Guidelines for Indications for Surgery in Patients with Severe Aortic Regurgitation Infective Endocarditis In Intravenous Drug Abusers the incidence increases severalfold greater than that of patients with rheumatic heart disease or prosthetic valves. The incidence in IVD is believed to be 1.5-20 per 1000 addicts per year 65-80% of cases are men Average age ranges from 27-37 years of age Steps in the Pathogenesis Valvular endothelial damage Platelet-fibrin thrombus formation Adherence of bacteria to platelet-thrombus plaque Local bacterial proliferation with hematogenous seeding Right or Left? Left sided IE Well established in the literature that infective endocarditis in the general pop. occurs most frequently on the left side of the heart (MV and Aortic valves) The predilection of Left sided IE is related to 3 factors: higher pressures on the left side that produce more turbulent flow, predisposing the mv and av valves to damage higher oxygen content supporting bacterial growth more congenital and acquired lesions of the left valves than right Right sided IE Rights sided (TV) IE accounts for 5-10% of cases of IE Approximately 76% of cases of endocarditis among IDUs occur on the right side. Factors include: IDU-related valvular endothelial injury ie the TV faces the heaviest bombardment and thus most endothelial damage Physiologic effects of injected substances cause vasospasm, intimal damage, and thrombus formation, predisposing the valve to bacterial aggregation Specific organisms associated with IDUs ie S. aureus has more right-sided involvement vs enterococcal which has more left- sided involvement. Levine et al. Microorganisms S. aureus and Groups A, B, and G Step most common. S. aureus (coagulase production/clotting cascade) V. Strep (Dextran production) S. aureus/v. strep (platelet aggregation) S. pyogenes (increased adherence) Streptococcus Enterococcus found in older men with GU disease/surgery S. epidermidis common among IVD S. bovis is often associated with a GI malignancy Clinical Features And Complications Fever: Most common symptom and sign in pts Heart murmurs: Commonly not audible in pts with TV IE Spleenomegaly: 15-50% of pts with IE Peripheral manifestations: petechiae, splinter hemorrhages (dark red, linear flame-shaped streaks in te nail bed), oslers nodes, Janewayss lesions Musculoskeletal: common complain systemic emboli: splenic, renal, stroke, coronary artery, extremities, mesenteric arteries CHF: secondary to valve destruction or distortion RI: immune complex-mediated glomerulonephritis Prophylaxis The recommended ab prophylaxis regimens depends on the degree of risk associate with both the cardiac abnormality and the bacteremia causing procedure Cardiac conditions are classified into high, moderate, and negligible risk Negligible risk does not require ab High and moderate differ only for GI/GU procedures Risk of Endocarditis Negligible RiskHigh RiskModerate Risk MVP without murmur or regurgitation Physiologic murmurs Isolaged secundum ASD Surgically repaired ASD, VSD, or PDA Cardiac pacemakers/defibrillators Hx of rheumatic fever or Kawasaki without valvular dysfunction Previous CABG All Prosthetic heart valve Previous bacterial endocarditis Complex cyanotic congenital heart disease Surgical pulmonary shunts MVP with regurgitation and/or myxomatous leaflets HCM Acquired valvular heart disease (acquired valvular stenosis, regurgiation, and rheumatic heart disease Congenital malformations not mentioned above (Ostium primum ASD, VSD, PDA, Bicuspid aortic valvue, coarctation) Prophylactic Regimens GU/GI SituationAntibioticsRegimen High-Risk ptsAmpicillin + Gentamicin Ampicillin 2.0 gm im or iv plus gent 1.5 mg/kg within 30min of starting procedure; 6 h later, amp 1 gm im/iv or amox 1 g orally Allergic to amp/amox Vancomycin + gentamicin Vanc 1 g iv over 1-2 h plus gent 1.5 mg/kg iv/im; complete within 30 min of starting procedure Moderate-RiskAmoxicillin or ampicillin Amoxicillin 2.0 g orally 1 h before procedure, or amp 2.0 g im/iv within 30 min of starting procedure Allergic to amp/amox Vancomycin Van 1 g iv over 1-2 h complete infusion within 30 min of starting procedure Key Points: Infective Endocarditis Mortality high (can be 20% in-hospital) 80% Native valve endocarditis Embolic events, large size, abscess, S.Aureus, DM are risk factors for poor outcome ASA Heparin no benefit in embolic situation Left sided IE benefit with surgery especially if associated with CHF BLOOD CULTURES AND TEE ESSENTIAL 2-D images of Mitral valve endocarditis AV Endocarditis Transthoracic Echo MV Endocarditis TEE Prosthetic Valve Prosthetic Valve Thrombosis: Early PHV thrombosis 5mm higher events Late PHV thrombosis: 50% CHF In-hospital mortality 25% 80% eventually needed Valve surgery Thrombolytics an option AML AML P1 P3 P1 P3 PML AV LA LA 3-D TEE of MV 3-D TEE of MV Bioprosthesis 28yr old F with MVR and thrombosis Prosthetic Heart Valve: VP-PM Valve Prosthesis- Patient Mismatch 30 Day Mortality can be as high as 25% Moderate mismatch 6% vs. mild 3% CHF, Refractory CHF and HF Deaths high Prosthesis size 90% JACC (2004) 43:1082-7JACC (2002) 39:1664-1669 Percutaneous repair of mitral regurgitation Background 2 emerging techniques Edge-to-edge repair Annuloplasty 1.Annuloplasty Uses close proximity of coronary sinus to MV annulus to “cinch” or compress posterior portion of annulus 2.Edge-to-edge repair Based on repair technique by Alfieri Suture placed in center of anterior and posterior leaflets creat

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