1. Valvular Heart Disease
Jay L. Rubenstone, D.O., F.A.C.C

2. Normal Structure Mitral Valve
Cross sectional Area 4-6cm²
Anterior and Posterior Leaflets
Chordae Tendineae  Papillary Muscles

3. Mitral Stenosis Etiology & Pathology
Rheumatic Fever- 99%
Other
Congenital
Carcinoid
Lupus
Amyloid
Infective Endocarditis
Mucopolysaccharide Disease

4. Stenotic Pathology Etiology & Pathology
Commissural 30%
Cuspal 15%
Chordal 10%
Mixed Remaining
Valve becomes funnel shaped or “fish mouthed”
Thickened immobile leaflets or chordal structures

5. Stenotic Pathology Debate: Smoldering rheumatic process or
Constant blood flow trauma leading to valve fibrosis and thickening

6. 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)

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

8. History Exertional Dyspnea Cough/Wheezing Orthopnea/PND/CHF
Hemoptysis-Rupture of Pulmonary Vein-Bronchial Vein Shunts

9. 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

10. Physical Exam Auscultation Decreased B/P if C.O. decreased
O.S.
Diastolic Rumble
Assoc Murmur of MR
Loud S1-thickened leaflets
Increased P2-pulmonary hypertension
Decreased B/P if C.O. decreased
Prominent a wave if sinus rhythm present

11. Physical Exam
Mitral Facies-pink, purple facial patches due to decrease CO and systemic vasoconstriction
Hepatomegally
Edema
Ascites
Hydrothorax With Right Heart Failure

12. Diagnosis ECG Left Atrial Abnormality RVH- right axis deviation
P wave becomes bifid and greater than 0.12 sec in duration in V1 and Lead II
RVH- right axis deviation
R wave > S wave in V1

13. Diagnosis Chest X-ray Echo- Cornerstone of Diagnosis
Dilated LA, RA, RV
Elevated Left Main stem Bronchus
Interstitial Edema
Echo- Cornerstone of Diagnosis
Thickened Calcified Leaflets
Doming of Leaflets on Opening
Bernoulli equation

14. Diagnosis
Cardiac Catherization
Gorlin Equation

16. Natural History Asymptomatic for 15-20yrs following Rheumatic Fever
Additional 5-10 yrs for progression from mild to severe stenosis
Stenosis progression approximately .09 cm²/yr

17. Natural History Presurgical Survival Rates NYHA Class II 80%-10yrs
Class III 38%-10yrs, 62% 5yrs
Class IV 15%-5yrs

18. Management-Medical Endocarditis Prophylaxis Activity Limitation
Diuretics- Decrease Na Intake
Heart Rate Control for A-fib or Sinus Rhythm
Anticoagulation

19. Percutaneous Balloon Angioplasty
Moderate-Severe MS
Mild MS- if Pulmonary Artery Pressures or Wedge Pressure Elevate with Exercise

20. Valve Replacement Indications Mortality
Combined MS/MR
<1.5 cm²-NYHA III or IV
<1 cm²
Class II if Pulmonary Artery Pressure >70mmHg
Mortality
3-8%
Valve Type-Prosthetic or Bioprosthetic

21. 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

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

23. 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

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

25. 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.

26. History Shortness of Breath Exertional Dyspnea
Congestive Heart Failure
Right Heart Failure
Significant symptoms in chronic MR usually do not develop until LV decompensation occurs.

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

28. Diagnosis Physical Exam ECG Chest X-ray Holosystolic Murmur
Increase Carotid Impulse
ECG
LA abnormality
LVH
RVH
Chest X-ray
Increase LA, LV, RV, Interstitial Edema

29. Diagnosis Echo Transesophageal superior to transthoracic
Evaluation of Chamber Sizes, Regurgitant Jet, Leaflets

30. Management of Acute MR Medical
After load Reduction (Nitropresside & Intra aortic balloon pump)
Decrease impedance to LV ejection
Decrease regurgitant volume into left atrium
Inotropic Support (Dobutamine)-if LV function reduced

31. Management of Acute MR Surgical Intervention
Progressive LV Failure or Hemodynamic Deterioration
CHF
Hypertension
Valve Disruption

32. Management of Chronic MR
Medical
Digoxin
Diuretics*
After load Reduction
Anticoagulation in A-fib
Endocarditis Prophylaxis

33. Management of Chronic MR
Surgical
Indications
Asymptomatic Class I
EF < 60% or LV Systolic Diameter >45mm
Severe MR Class II, III, or IV
generally considered for surgery unless EF <30%
Valve Repair vs. Replacement

34. Mitral Valve Prolapse Systolic Click-Murmur Syndrome Barlow’s Syndrome
Billowing Mitral Valve Syndrome
Floppy Valve Syndrome
Myxomatous Valve Syndrome
Parachute Valve

35. Mitral Valve Prolapse Over diagnosed 2.4% of population
Females>Males 2:1
Severe MR- Elderly Male>Young Female

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

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

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

39. Physical Examination Body type Auscultation
Asthenic, low weight body habitus, straight back syndrome
Auscultation
Systolic click- multiple, non-ejection (after carotid upstroke) due to tensing of elongated chordae and prolapsing valve

40. Physical Examination Auscultation
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

41. Diagnosis ECG and Chest X-ray Echo Normally unremarkable
Billowing of one or both leaflets into the left atrium during systole at least 2mm
Parasternal long axis view for diagnosis
Associated MR
Leaflet thickness

42. 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)

43. 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*

44. 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

45. Aortic Stenosis Etiology and Pathology
Valvular
Supravalvular
Subvalvular
Hyperthrophic Cardiomyopathy

46. 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

47. 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

48. Is this atherosclerotic disease?
Degenerative A.S. accelerated in diabetes and hyperlipidemia.
Associated with tobacco use and HTN.
Potentially treated with HMGcoA agents?

50. Hemodynamics Critical (Surgical) AS Moderate AS Mild AS
Peak systolic pressure gradient > 50mmHg in the presence of normal cardiac output
Valve area < cm²
Moderate AS
1-1.5cm²
Mild AS
1.5-2cm²
Aortic Sclerosis

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

52. History Syncope Dyspnea (CHF) Reduced cerebral perfusion
Vasodilatation 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

53. Diagnosis Physical Examination Systolic Murmur Pulses Parvus
Diamond-Shaped, harsh, left sternal boarder to right intercostal spaces, neck and apex
Late peak, obliteration of S2, consistent with bedside Dx of Critical AS
Pulses Parvus
Delayed and Prolonged Carotid Impulse

54. Diagnosis ECG Chest X-ray Echo Classic LVH Concentric LVH
Calcification of Aortic Valve
Echo
Bernoulli (continuity) equation-calculation of LV-Aortic pressure gradient and valve area

55. Diagnosis
Cardiac Catherization
Gorlin Equation

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

57. Medical Management Endocarditis Prophylaxis Limit Physical Activity
Watch Beta Blockers and Diuretics
*Treatment of Critical AS in viable candidates is surgery

58. Surgery (Valve Replacement)
Indications
Symptomatic Patients -valve area cm² or less
Asymptomatic Patients-progressive LV dysfunction (EF <35%) or hypotensive response to mild exercise
Delaying surgery in asymptomatic patients with good exercise tolerance is controversial

59. Surgery (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%

60. Aortic Regurgitation Etiology and Pathology
Valvular
Rheumatic-Fibrotic Retraction of Leaflets
Ankylosing Spondylitis, Behcets, Psoriatic Arthritis, Giant Cell Arteritis
Degenerative AS-75% w/AR
Infective Endocarditis-Leaflet Destruction
Trauma-ascending aortic tear
Bicuspid aortic valve-prolapse or incomplete closure
Myxomatous Degeneration-like MVP
Appetite suppressant drugs-serotonin related valve deposits

61. 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

62. History Acute AR Chronic 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 4th or 5th Decade

64. Physical Examination Diastolic Murmur Left sternal boarder
Decrescendo, high pitched
Best heard Sitting Up, End Expiration
Longer murmur equals worse AR

65. Physical Examination de Mussett’s Sign (head bobbing)
Corrigan’s Pulse “water hammer”
Abrupt Distention with Quick Collapse
Bisferiens-pulse
2 peaks
Traube’s Sign
Pistol shot sounds over femoral pulse
Duroziez’s Sign
Murmur over femoral pulse with compression

66. Physical Examination Quinckes Sign Muller’s Sign Hill’s Sign
Capillary pulsations
Muller’s Sign
Systolic pulsations of uvula
Hill’s Sign
Popliteal pulse exceed brachial pulse by > 60mmHg

67. Physical Examination Korotkoff Sounds Can persist to 0mmHg
Wide Pulse pressure

68. Diagnosis ECG Chest X-ray Echo Cardiac Catheterization LVH
Cardiomegaly predominantly inferior and leftward
Echo
Can aid in detecting etiology, quantifying degree of regurgitation, and assessing LV size and function
Cardiac Catheterization

69. Natural History Acute AR Chronic 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

70. Medical Treatment Acute AR Chronic AR As above
Asymptomatic Mild-Moderate
Follow by Echo Yearly
Endocarditis Prophylaxis for all AR
May not require medical treatment

71. Medical Treatment Symptomatic Moderate-Severe AR
Limit exertional activity
Aggressively treat B/P
Diuretics
Salt Restriction
Digoxin
Vasodilators (Nifedipine?)

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

73. Surgical Treatment Mortality 3-8% perioperative
5-10% late mortality with significant preop LV dysfunction