1. JAY L. RUBENSTONE, D.O., F.A.C.C VALVULAR HEART DISEASE Fall 2012

2. NORMAL STRUCTURE MITRAL VALVE Cross sectional Area 4-6cm2 Anterior and Posterior Leaflets Chordae Tendineae  Papillary Muscles 2

3. MITRAL STENOSIS ETIOLOGY & PATHOLOGY Rheumatic Fever- 99% Other Congenital Carcinoid Lupus Amyloid Infective Endocarditis Mucopolysaccharide Disease 3

4. PATHOPHYSIOLOGY Mild MS- orifice <2 cm2 Critical MS- <1 cm2 A-V pressure gradient >20mmHg Increased LA Pressure Increase Pulmonary Venous + Capillary Pressures Increase Pulmonary Artery Systolic Pressure Decrease RV Function (when PAS>30-60mmHg) 4

5. PATHOPHYSIOLOGY Pulmonary HTN Passive Backward Transmission Of Incr. LA pressure  Pulmonary Arteriolar Constriction  Organic Obliterative Changes in Pulmonary Vascular Bed  RV Failure 5

6. HISTORY Exertional Dyspnea Cough/Wheezing Orthopnea/PND/CHF Hemoptysis-Rupture of Pulm Vein-Brochial Vein Shunts 6

7. HISTORY Chest Pain-Increase RV Pressures or Unknown Etiology Systemic Emboli (LA clots) Increased LA size, Decreased C.O., Atrial Fib, IE Significantly decreased w/anticoagulation 7

8. DIAGNOSIS Cardiac Catherization Gorlin Equation 8

9. NATURAL HISTORY Asymptomatic for 15-20yrs following Rheumatic Fever Additional 5-10 yrs for progression from mild to severe stenosis Stenosis progression approx..09 cm2/yr 9

10. NATURAL HISTORY Presurgical Survival Rates NYHA Class II 80%-10yrs Class III 38%-10yrs, 62% 5yrs Class IV 15%-5yrs 10

11. MANAGEMENT-MEDICAL Endocarditis Prophylaxis Activity Limitation Diruetics- Decrease Na Intake Heart Rate Control for A-fib or Sinus Rhythm Anticoagulation 11

12. PERCUTANEOUS BALLOON ANGIOPLASTY Moderate-Severe MS Mild MS- if Pulmonary Artery Pressures or Wedge Pressure Elevate with Exercise 12

13. VALVE REPLACEMENT Indications Combined MS/MR <1.5 cm2-NYHA III or IV <1 cm2 Class II if Pulm Artery Pressure >70mmHg Mortality 3-8% Valve Type-Prosthetic or Bioprosthetic, TAVR 13

14. MITRAL REGURGITATION Etiology Rheumatic Heart Disease Infective Endocarditis Collagen Vascular Disease Cardiomyopathy Ischemic Heart Disease Mitral Valve Prolapse- most common cause for valve surgery in US 14

15. PATHOPHYSIOLOGY Decreased Impedance to Ventricular Emptying Determinants of Regurgitant Flow Instantaneous Size of MV Orifice Dependent on Preload, Afterload, LV Contractility, LV Size LA-LV Pressure Gradient dependent on Systemic Vascular Resistance, LV Pressure, & LV Size 15

16. PATHOPHYSIOLOGY LV Compensation Increased End Diastolic Volume Increased Wall Tension Increased Preload Increased LV Emptying Normal Ejection Fraction should be Super Normal >65% to maintain forward cardiac output and B/P 16

17. PATHOPHYSIOLOGY LV Decompensation Increase End Systolic Volume Increased End Diastolic Volume Leads to Annulus Dilatation (MR begets MR) Decreased Ejection Fraction and Stroke Volume 17

18. PATHOPHYSIOLOGY Ejection Fraction in Mitral Regurgitation >65% normal in compensated MR 50-65% mild impairment 40-50% moderate-severe impairment <35% advanced impairment As ejection fraction decreases operative risk increases. 18

19. HISTORY Shortness of Breath Exertional Dyspnea Congestive Heart Failure RHF Significant symptoms in chronic MR usually do not develop until LV decompensation occurs. 19

20. HISTORY Medical Treatment Survival 80% 5yr 60% 10yr 30-45% 5yr if MR severe 20

21. MANAGEMENT OF CHRONIC MR Medical Digoxin Diruetics* Afterload Reduction Anticoagulation in A-fib Endocarditis Prophylaxis 21

22. MANAGEMENT OF CHRONIC MR Surgical Indications Asymptomatic Class I EF 45mm Severe MR Class II, III, or IV generally considered for surgery unless EF <30% Valve Repair vs. Replacement 22

23. MITRAL VALVE PROLAPSE Systolic Click-Murmur Syndrome Barlow’s Syndrome Billowing Mitral Valve Syndrome Floppy Valve Syndrome Myxomatous Valve Syndrome Parachute Valve 23

24. MITRAL VALVE PROLAPSE Over diagnosed 2.4% of population Females>Males 2:1 Severe MR- Elderly Male>Young Female 24

25. MVP ETIOLOGY Primary Valvular most frequent Connective Tissue Diseases Hyperthyroidism Myotonic Dystrophy Periarteritis Nodosa Von Willebrands 25

26. MVP PATHOLOGY Myxomatous Proliferation and Degeneration of Valve Leaflets Increased Quantity of Acid Mucopolysaccharide in Middle Layer of Valve Tissue 26

27. MVP HISTORY Most are asymptomatic throughout life Chest pain, fatigue, anxiety Orthostasis-questionable autonomic dysfunction Arrhythmia-SVT, PACs, PVCs Symptoms of MR if present 27

28. PHYSICAL EXAMINATION Ascultation Murmur- mid to late crescendo progressing to holosystolic if MR becomes severe Click and murmur move closer to S1 during strain phase of valsalva, sudden standing, and Amyl Nitrate 28

29. NATURAL HISTORY Progressive MR in 15% over 10-15 yrs Infective Endocarditis Cerebral Emboli-tearing of endothelial covering of myxomatous valve with platelet activation Sudden Cardiac Death-V fib, increased Q-T interval (not well established) 29

30. MVP MANAGEMENT Endocarditis prophylaxis if MR present Holter monitor-beta blocker for ectopy? Aspirin if focal neurological events present MR-treat like any other MR, valves usually amenable to repair *MVP is usually a benign disease* 30

31. AORTIC VALVE NORMAL STRUCTURE Valve sits at the base of Aortic Root Three Leaflets (cusps)-non coronary, right coronary, left coronary Cusps give rise to ostea of right coronary artery and left main coronary artery Normal cross-sectional area 3-4cm2 31

32. AORTIC STENOSIS ETIOLOGY AND PATHOLOGY Valvular Supravalvular Subvalvular Hyperthrophic Cardiomyopathy 32

33. CONGENITAL AORTIC STENOSIS Unicuspid Presents less than one year of age Bicuspid Adult Presentation Chronic turbulent flow Leads to fibrosis, rigidity, calcification Tricuspid Leaflets of unequal size 33

34. ACQUIRED AORTIC STENOSIS Rheumatic Rare Usually mitral valve also involved Degenerative or Senile Most common cause of adult AS Most common cause of valve replacement Years of normal mechanical stress leads to calcium deposits on leaflets Inflammatory or Infectious component?? >age 65 2% frank AS, 30% Aortic Sclerosis 34

35. HEMODYNAMICS Severe AS Mean systolic pressure gradient ≥ 40mmHg in the presence of normal cardiac output Valve area ≤ 1.0cm 2 Moderate AS 1-1.5cm 2 Mild AS 1.5-2cm 2 Aortic Sclerosis 35

36. HISTORY Long latent period of increasing obstruction Symptoms usually begin in 5 th or 6 th decade Angina in 2/3 of patients Hyperthrophied myocardium Increased ventricular systolic pressure All of which increase myocardial oxygen consumption Oxygen supply-demand imbalance leads to subendocardial ischemia 36

37. HISTORY Syncopy Reduced cerebral perfusion Vasodilation in the presence of fixed cardiac output leads to hypotension Baroreceptor-vasodepression due to high LV systolic pressure Dyspnea (CHF) Particularly with exertion due to fixed cardiac output Pulmonary Venous HTN can lead to CHF 37

38. NATURAL HISTORY Asymptomatic latent period With moderate-severe AS valve area can decrease on average 0.12cm2 per year *Angina, synocopy or CHF Average 1-3 year survival 50% Sudden cardiac death rare 38

39. SURGERY (VALVE REPLACEMENT) Indications Symptomatic Patients -valve area ≤ 1.0cm 2 Asymptomatic Patients-progressive LV disfunction (EF <35%) or hypotensive response to mild exercise Delaying surgery in asymptomatic patients with good exercise tolerance is controversial Valve type Prosthetic, Bioprosthetic or TAVR 39

40. KEEPSURGERY (VALVE REPLACEMENT) Results Effective prosthetic valve area not normal Surgery replaces Critical AS with Non-critical AS Symptoms can persist if valve-patient mismatch occurs 10 year survival –85% 40

41. AORTIC REGURGITATION ETIOLOGY AND PATHOLOGY Valvular Rheumatic-Fibrotic Retraction of Leaflets Ankylosing Spondylitis, Behcets, Psoratic Arthritis, Giant Cell Arteritis Degenerative AS-75% w/AR Infective Endocarditis-Leaflet Distruction Trauma-ascending aortic tear Bicuspid aortic valve-prolapse or incomplete closure Myxomatous Degeneration-like MVP Appetite suppressant drugs-serotonin related valve deposits 41

42. ETIOLOGY AND PATHOLOGY Aortic Root Disease-More common than primary valvular. Root Dilatation leads to non-coaptation of leaflets. Degenerative-Hypertensive Aortic Dilatation Cystic Medial Necrosis-Classic Marfans Syndrome Aortic Dissection Syphilitic Aortitis Rheumatic Disease-same as valvular 42

43. HISTORY Acute AR LV cannot accommodate acute regurgitant volume can lead to cardiovascular collapse Chronic AR Gradual LV enlargement-eccentric hypertrophy Exertional dyspnea, orthopnea, PND, CHF Presents 4 th or 5 th Decade 43

44. NATURAL HISTORY Acute AR Cardiovascular collapse Inotrophic agents and vasodilators Prompt surgical intervention Chronic AR 75% Five Year Survival 50% Ten Year Survival Progressive downhill course of CHF, Episodic Pulmonary Edema, Sudden Cardiac Death 44

45. MEDICAL TREATMENT Acute AR As above Chronic AR Asymptomatic Mild-Moderate Follow by Echo Yearly Endocarditis Prophalaxis for all AR May not require medical treatment 45

46. MEDICAL TREATMENT Symptomatic Moderate-Severe AR Limit exertional activity Aggressively treat B/P Diuretics Salt Restriction Digoxin Vasodilators (Nifedipine?) 46

47. SURGICAL TREATMENT Indications Defer surgery for chronic severe AR if good exercise tolerance, EF greater than 50%, end systolic diameter < 50 mm, and end diastolic diameter < 70 mm Be aware that progressive decline in LV function or size increases surgical morbidity and mortality 47

48. SURGICAL TREATMENT Mortality 3-8% perioperative 5-10% late mortality with significant preop LV dysfunction 48