1. Valvular Heart Disease
Ni Chao, M.D.
Division of Cardiology

2. Objectives To understand the pathophysiology of the major VHDs
To learn how to examine the patient
To understand the principles of laboratory diagnosis
To learn the fundamentals for treatment of cardiac valve abnormalities

3. Mitral Stenosis

4. Etiology Almost always the result of rheumatic fever
Less common causes
Congenital MS
Systemic lupus erythematosus
Rheumatoid arthritis
Atrial myxoma
Bacterial endocarditis.

5. Epidemiology Rare in industrialized countries in patients <40
Very common in developing countries, esp. South Asia with severe disease often at early age (<20)
2/3 of all patients with MS are female.
The onset of symptoms is usu. between the 3rd and 4th decades.

6. Pathology The mitral valve area (MVA) is normally 4-6 cm2 in adult
Acute rheumatic fever causes immune-medi-ated inflammation of the mitral and other valves
The leaflets thickened and the commissures fused along with thickening and shortening of the chordae tendineae  narrowing of the mitral valve orifice

8. Pathophysiology
MVA reduced to 2 cm2: increased left atrial pressure (LAP) is necessary for normal trans-mitral flow
MVA reduced to 1cm2: a LAP of 25 mm Hg is required  a rise in:
Pulmonary venous preesure (PVP)
Pulmonary capillary wedge pressure (PCWP)
exertional dyspnea

9. Pathophysiology
Progressive dilation of the LA predisposes mural thrombi and atrial fibrillation
Chronic elevation of LAP  pulmonary hypertension, tricuspid and pulmonary re-gurgitation  right heart failure

10. Pathophysiology Patients at high risk are of mural thrombi
over 35 years old
atrial fibrillation with a low cardiac output (CO)
having a large left atrial appendage.
Atrial fibrillation
in up to 40% of patients
decreases CO by 20%.

12. Clinical Manifestations
Histories of rheumatic fever, murmur
Dyspnea
Palpitations
Chest pain
Hemoptysis
Edema
Thromboembolism

13. Physical Examination Low-pitched diastolic rumble Opening snap
S1, atrial fibrillation, P2 
RV heave
Elevated neck veins, hepatomegaly, ascites, pedal edema
Coexistent murmurs
Thromboembolic events

14. Chest X-Ray Left atrial enlargement Pulmonary edema
Prominence of pulmonary arteries
Enlargement of right ventricle

16. EKG Left, right atrial abnormalities atrial fibrillation
Right Venticular hypertrophy

17. Echocardiogram Enlarged LA
Markedly thickened, often calcified MV with very narrow, "fish-mouth" shaped orifice
Delayed LV filling with transmitral gradient in diastole

18. Echocardiogram RV hypertrophy, RV hypokinesis
TV thickness, stenosis or regurgitation
Pulmonary hypertension
LV function usually preserved

21. Severity of Mitral Stenosis
Mild
Moderate
Severe
MVA
(cm2)
<1.0
Mean PG (mmHg)
<10
10-20
>20

22. Natural History
In industrialized countries, year latent period between episodes of rheumatic fever and clinical signs of MS
Once mild symptoms develop, progression to complete disability is very rapid (5 years) without intervention
Time course more fulminant in developing countries

23. Management—Medical Salt reduction Diuretics
Control of heart rate with digoxin
Anti-arrhythmic drugs
Prevention of thromboemboli with adequate anti-coagulants

24. Management—Surgical
Interventional therapy indicated for mitral valve area of <1.0 cm2
Mitral commisurotomy or mitral valve replace-ment are common surgical approaches
For selected patients (primarily young with pure MS), mitral balloon valvuloplasty is a successful option

25. Mitral Regurgitation

26. Etiologies—Acute
Endocarditis (most often caused by Staphylo-coccus aureus)
Papillary muscle rupture (from infarction) or dysfunction (from ischemia)
Chordal rupture (from myxomatous valvular disease)

27. Etiologies —Chronic Rheumatic fever Mitral valvular prolapse
Marfan syndrome
Cardiomyopathy

28. Pathophysiology—Acute
Abrupt elevation of LA pressure in setting of LA with normal size and compliance
Backflow into pulmonary circulation with elevated PVP and PCWP and pulmonary edema
Decreased forward flow of CO, hypotension and shock occur often

29. Pathophysiology —Chronic
Gradual elevation of LA pressure with dilat-ation of LA and LV
Increased preload and eccentric LV hyper-trophy, LV function falls
Elevated pulmonary vascular filling  Pul-monary hypertension

32. Clinical manifestations
Dyspnea on exertion
Paroxysmal nocturnal dyspnea
Orthopnea
Palpitations
Edema

33. Physical Examination Laterally displaced PMI, RV heave
Holosystolic murmur
S3 gallop
Atrial fibrillation
Loud, palpable P2
Signs of endocarditis
Thromboembolic events

34. Chest X-ray Dilated LV (chronic)
Pulmonary vascular redistribution (chronic) pulmonary edema (acute and chronic)

35. EKG Left ventricular hypertrophy Left or/and right atrial enlargement
Atrial fibrillation
Nonspecific ST-T wave changes
Ischemic ST-T wave changes
Myocardial infarction

36. Echocardiogram Dilated LA, LV, RA, RV Decreased LV function
Mitral regurgitation
Segmental wall motion abnormalities (if in-farction)
Vegetations (if endocarditis)

37. Severity of Mitral RegurgitationⅠ Ⅱ Ⅲ Ⅳ
Length
(cm)
1.5
≥1.5
≥3.0
≥4.5
Area
(cm2)
IA/LAA
(% )
<20
20-40
≥40

38. Exercise Radionuclide Ventriculography
Fall in LV ejection fraction (>5% decline in ejection fraction) indicating incipient LV dysfunction
Dilated LV, LA, RV, RA

39. Cardiac Catheterization
Elevated LVEDP, PVP, PCWP and PAP
Coronary artery occlusions (if infarction)

40. Natural History Time course is variable for chronic form
Extent of LV cavity dilatation is inversely related to survival
5 year survival of patients treated medically is 45-80% depending on exercise limitation
Acute form is associated with much higher mortality

41. Management —Medical Salt reduction Diuretics Digoxin
Vasodilators (ACEIs, nitrates, hydralazine)
Anticoagulation
Anti-arrhythmics
Intra-aortic balloon pump (IABP)

42. Management —Surgical
Mitral valve repair or replacement: must be performed before LV function too seriously compromised

43. Aortic Stenosis

44. Etiologies Bicuspid aortic valve (most common con-genital anomaly)
Calcification of tri-leaflet aortic valve
Congenital unileaflet valve
Rheumatic fever (<1% of patients with isolated aortic valve disease - usually also involves mitral valve)

45. Epidemiology
Largest group is y/o with disease on tri-leaflet valves
Next largest group is y/o with disease on bicuspid aortic valves
There is also a small group of patients with congenital AS

48. Pathophysiology Obstruction to LV outflowthe LV hyper-trophies
Aadequate cardiac output at rest preserved for years with increasing trans-aortic gradient

49. Pathophysiology
When the AVA<0.7 cm2, CO may be normal at rest but fails to rise with exertion due to obstruction to LV emptying from stenotic valve
The LV dilates with longstanding AS

50. Pathophysiology
Progressive LV dysfunctiona fall in SV, CO and the LV-aortic gradient while the LAP, PCP, PAP, LVDP, RVDP increase
The dilatation of mitral valve annulus MR
the LV mass increases, diminished LV com-pliance, increased diastolic stiffness

51. Clinical Manifestations
Syncope
Angina
Heart failure
Emboli
Endocarditis

52. Physical Exam Loud systolic murmur, radiates to the neck
Sustained point of maximal impulse
Diminished and delayed carotid upstroke (parvus et tardus)
Precordial thrill
Single, soft S2 valve, S4
Aortic ejection sounds (heard after S1)

53. Chest X-ray Calcific aortic valve Tortuous aorta LA enlargement
LV enlargement (late)

54. EKG
LV hypertrophy
LA abnormality
Interventricular conduction delay

55. Echocardiogram LV hypertrophy Thickened, immobile aortic valve
Dilated aortic root (post-stenotic dilatation)
LV-aortic gradient
LA enlargement
MR, LV dilatation (late)

56. Severity of Aortic Stenosis
Mild
Moderate
Severe
MAV (cm2)
1-1.5
<0.7
Mean PG (mmHg) 25
25-50
>50

57. Cardiac Catheterization
LV- aortic gradient
Coexistent coronary artery disease may be present but is unrelated to development of AS

58. Natural History
May be asymptomatic for years despite severe obstruction
Development of certain symptoms portends a bad prognosis
Aangina (average survival 3 years)
Syncope (average survival 2 years)
Heart failure average survival 1.5 years)

59. Management —Medical Endocarditis prophylaxis Anti-arrhythmics
No specific role for medical therapy

60. Management —Surgical
Aortic valve replacement: improved survival and LV function even in those with pre-operative LV dysfunction as well as in octogenarians
Aortic balloon valvuloplasty: purely palliative, survival or adequate long-term benefits improved not improved
Reserved for pt who cannot tolerate surgery.

61. Aortic Regurgitation

62. Etiologies —Acute
Infective endocarditis
Trauma
Aortic dissection

63. Etiologies — Chronic Primary valvular
Rheumatic fever
Bicuspid valve
Marfan
Ehlers-Danlos
Ankylosing spondylitis
Lupus

64. Etiologies — Chronic Aortic root disease
Syphilis
Osteogenesis imperfecta
Aortic dissection
Behcet syndrome
Reiter syndrome
Hypertension

65. Pathophysiology —Chronic
The entire SV ejected into the high pressure aorta in aortic regurgitation (AR)
Part of the SV leaks back into the ventricle during diastole
An increase in LVEDV to maintain effective forward flow

66. Pathophysiology —Chronic
LV dilatation occurs as does a significant increase in LV stress
The LV mass dramatically increases, often greater than LV mass in AS
LVEDP remain relatively normal until late in disease
LVEF usually normal and may increase with exercise

67. Pathophysiology —Chronic
A fall in the LVEF with exercise portends the onset of intrinsic LV dysfunction
With longstanding AR, LV dysfunction en-sures with decreased LVEF, SV, CO and increased LVEDP, LAP, PVP and PCWP

68. Pathophysiology — Acute
The ventricle does not have time to dilate in response to the increased ventricular load
With part of the stroke volume leaking back into the ventricle in diastole, the effective CO falls
The rapid rise in LV pressure due to acute AR causes the mitral valve to close early

69. Clinical Manifestations
Dyspnea on exertion
Paroxysmal nocturnal dyspnea
Orthopnea
Palpitations
Angina
Cyanosis/shock (acute)

70. Physical Examination —Chronic
Diastolic decrescendo murmur
Dilated point of maximal impulse
Rapidly collapsing pulse (water-hammer or Corrigan pulse)
Head bobbing (deMusset sign)
Capillary pulsations (Quincke pulses) S3

71. Physical Examination —Chronic
Wide aortic pulse pressure with systolic hypertension
Pistol-shot sounds of the femoral arteries
Low pitched diastolic rumble due to narrowing of mitral valve orifice by AR jet (Austin-Flint)

72. Physical Examination — Acute
Normal pulse pressure
Soft or absent S1
Soft diastolic murmur
Cyanosis, shock, vasoconstriction

73. Chest X-ray
Enlarged LV
Tortuous, dilated aorta (mainly chronic)

74. EKG LV hypertrophy (chronic but not acute form)
Interventricular conduction delay
PR prolongtion

75. Echocardiogram
Chronic form: dilated LV; marked LV hyper-trophy, dilated aortic root, aortic regurgitation, enlarged LA, thickened aortic valve
Acute form: normal LV cavity size, aortic regurgitation, early closure of mitral valve

76. Severity of Aortic RegurgitationⅠ Ⅱ Ⅲ Ⅳ
IH/LVOH
(%)
1-24
25-66
47-64
>65
IA/LVOA
<4
4-24
25-59
>60

77. Radionuclide Ventriculography
Dilated LV
Normal LV ejection fraction at rest with fall in ejection fraction with exertion (chronic form)

78. Cardiac Catheterization
Chronic: markedly dilated LV; coronary arteries often normal despite angina; aortic root injection of contrast dye shows severity of AR.
LV end-diastolic pressure elevated only late in chronic form of disease}
Acute: normal LV cavity size; very high LV end-diastolic pressure

79. Natural History Chronic
Survival is 75% at 5 years
Once symptomatic, death usually occurs within 4 years of angina and 2 years of heart failure
Acute form has high mortality without surgical repair

80. Management —Chronic
Mild or moderate AR or severe AR in patients with good exercise tolerance and normal LV function can be managed with salt restriction, diuretics, vasodilators
Patients with severe AR and symptoms or with evidence of LV dysfunction should be considered for aortic valve replacement

81. Management — Acute
Medical emergency requiring urgent surgical replacement of damaged aortic valve
IABP contra-indicated (would increase AR)