1
Valvular Heart Disease
Jay L. Rubenstone, D.O., F.A.C.C
2
Normal StructureMitral Valve
Cross sectional Area 4-6cm² Anterior and Posterior Leaflets Chordae Tendineae Papillary Muscles
3
Mitral StenosisEtiology & 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 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
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– 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 CatherizationGorlin Equation
15
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– 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– 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– 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– Normally unremarkable
Echo– 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 ValveNormal 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?
49
50
Hemodynamics
Critical (Surgical) AS– Peak systolic pressure gradient > 50mmHg in the
presence of normal cardiac output– Valve area <0.7-0.8cm²
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– 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
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– Classic LVH
Chest X-ray– 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 0.7-0.8cm² 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 RegurgitationEtiology 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– 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
63
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– 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– LVH
Chest X-ray– 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– 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– As above
Chronic AR– 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