valvular heart disease arthur c. aufderheide, m.d. med 6655. cardiovascular system
TRANSCRIPT
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VALVULAR HEART DISEASE
Arthur C. Aufderheide, M.D.
Med 6655. Cardiovascular System
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RHEUMATIC HEART DISEASE (RHD): ACUTE (1)
• Acute rheumatic fever
• Group A beta-hemolytic strep (pharyngitis). In only 3% of these, rheumatic fever develops 2-6 weeks later
• Strep and endocardium = shared antigen (M protein: M 18 ?) responsible for
virulence (molecular mimicry) capsular glycoprotein
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RHEUMATIC HEART DISEASE (RHD): ACUTE (2)
• Clinical: brain (Sydenham’s chorea [St. Vitus’ dance]) –
purposeless, erratic motor movements joints, skin, heart, pancarditis
• Incidence: declining 1946 = 343 / 100,000 1986 = 0.3 / 100,000 But still in Third World
U.S.A. Why?
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RHEUMATIC HEART DISEASE: ACUTE
• Pancarditis:
Myocarditis (Aschoff bodies)
collagen focal necrosis with granuloma
(histiocytic cells — multinucleate and
Anitschkow)
Endocarditis
Pericarditis (fibrinoid)
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Myocardial Aschoff body
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Ashoff body
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Lymphs,macs,multinucleates
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Anitschkow cells
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ACUTE RHD: ENDOCARDITIS
• Valves: Mitral and aortic (tricuspid rare; pulmonary,
"never") Collagen necrosis:
surface thrombus ("verrucae") murmur
• Active inflammation may continue for years after other lesions heal
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Endocardial Aschoff body
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pericarditis
fibrinous
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ACUTE RHD: LAB TESTS
• ASO (Anti-Streptolysin-O Titer — Todd test)
Others: anti-DNase B, antistreptokinase
• CRP (C-Reactive Protein)
• ECG ( P-R interval)
• None of above are specific for rheumatic fever
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Minithroimbus on mitral cusp (Aschoff )
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RHEUMATIC VEGETATIONS
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RHEUMATIC VEGETATIONS
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CHRONIC RHD: MITRAL STENOSIS (1)
• Most common. Mostly females. Post-rheumatic
fever.
• Thick, fibrous, calcified valve
• Functional defect: stenosis > regurgitation.
Lt. atrium fibrillation (big problem) stasis
thrombi emboli:
lung
systemic
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MITRAL STENOSIS
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Huge left atrium
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Short chordae
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CHRONIC RHD: MITRAL STENOSIS (2)
• Lt. atrium fails chronic passive congestion lung:
lung hemosiderosis + fibrosis pulmonary hypertension acute pulmonary arteritis (>60 mm)
• Rx: medical; balloon valvuloplasty; surgical repair
preferred; prosthesis, antibiotics
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Fishmouth mitral
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Mitral stenosis
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Normal LV hypertrophy Mitral stenosis
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Mitral stenosis
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Atrial thrombus
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Embolus infarct,kidney
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Embolus infarct,spleen
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Embolus infarct,brain
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Calcified thrombi on mitral valve
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Calcified embolus to
coronary artery
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CHRONIC RHD: MITRAL STENOSIS (2)
• Lt. atrium fails chronic passive congestion lung:
lung hemosiderosis + fibrosis pulmonary hypertension acute pulmonary arteritis (>60 mm)
• Rx: medical; balloon valvuloplasty; surgical repair
preferred; prosthesis, antibiotics
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Mitral stenosis
RVH
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PULMONARY CONGESTION
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HEMOSIDERIN-FILLED MACROPHAGES
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PULMONARY FIBROSIS
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Pulmonary arteritis
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CHRONIC RHD: MITRAL STENOSIS (2)
• Lt. atrium fails chronic passive congestion lung:
lung hemosiderosis + fibrosis pulmonary hypertension acute pulmonary arteritis (>60 mm)
• Rx: medical; balloon valvuloplasty; surgical repair
preferred; prosthesis, antibiotics
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Chordae fused, gone
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CHRONIC RHD: MITRAL REGURGITATION
• Rarely “pure” if due to rheumatic fever
• Left heart failure occurs later than in mitral stenosis
• Other causes: ruptured papillary muscle after infarction mitral valve prolapse
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AORTIC STENOSIS: CAUSES
• Congenital:
Stenotic at birth
Bicuspid; later calcification
• Rheumatic heart disease
• Atherosclerosis. Rare.
• Idiopathic
“degenerative” calcific aortic stenosis:
most common today in USA
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Normal aortic valve
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Bicuspid aortic valve stenosis
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AORTIC STENOSIS: CAUSES
• Congenital:
Stenotic at birth
Bicuspid; later calcification
• Rheumatic heart disease
• Atherosclerosis.
• Idiopathic “degenerative” calcific aortic
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Normal aortic valve
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rheumatic aortic stenosis;fused cusps
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AORTIC STENOSIS: CAUSES
• Congenital:
Stenotic at birth
Bicuspid; later calcification
• Rheumatic heart disease
• Atherosclerosis. Rare.
• Idiopathic
“degenerative” calcific aortic stenosis:
most common today in USA
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Calcific aortic stenosis,etiology?
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CHRONIC RHD: AORTIC STENOSIS (1)
• Given amount commissural fusion has more functional impairment of aortic than mitral
• Left Ventricle (pressure and hypertrophy)
• Angina: Systolic coronary artery compression slows or
stops flow in myocardial capillaries. BP in Valsalva sinus (Venturi effect) Valve calcification extends to narrow coronary
artery orifice Coronary atherosclerosis
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CHRONIC RHD: AORTIC STENOSIS (2)
• Sudden death Stokes-Adams syncope (arrhythmia? exercise systemic resistance but output fixed by stenosis
• Left failure late but resists Rx. Hence, surgical repair before failure occurs.
• Failure: diastolic filling defect plus systolic contractility are decreased because afterload is increased
symptoms correlate with degree of stenosis
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Calcific aortic stenosis, x-ray
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AORTIC STENOSIS: Rx
• Medical
• Surgical: Congenital, infant: valvulotomy
Acquired: can’t repair native valve surgically, so use
prosthesis perform before symptoms severe Quantitative CT scan good to assess status
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Plastic ball cracked
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ball escaped cage aorta
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CLOT
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CHRONIC RHD: AORTIC REGURGITATION
• RHD infrequent cause (some due to bacterial
endocarditis or aortic dissection)
• Syphilis:
Commissure separation (stretched ring)
Affects ascending aorta most
Mesoaortitis weakens aorta wall aneurysm
Symptoms do not correlate well with degree of
insufficiency
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MITRAL VALVE PROLAPSE (1)
• Mitral leaflet bulging into atrium in systole
• Valve leaflets deformed by focal myxomatous
degeneration of valve connective tissue:
Marfan syndrome (fibrillin gene defect)
Chemical change: myxomatous degeneration
in Down syndrome
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MITRAL VALVE PROLAPSE
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MITRAL VALVE PROLAPSE
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MITRAL VALVE PROLAPSE (2)
• Sx: systolic click. May be asymptomatic.
• Dx: Cardiac imaging
• Complications:
Left failure due to mitral regurgitation
Infectious endocarditis
Arrhythmias with sudden death
Embolism from valve thrombi
• Rx: Surgical repair, prosthesis
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CHEMICAL CAUSES OF VALVULAR DISEASE
• Fenfluramine, phentermine (fen-phen): Fibrous thickening of all valves Produces pulmonary hypertension
• Carcinoid tumor (serotonin)
• Ergot alkaloids (ergotamine; methysergide) Also pergolide mesylate (used to Rx Parkinson
disease)
• Mechanism of all of above: deposit of fibrous tissue plaque on valve with normal architecture