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Role of Echo in
Connective Tissue Diseases
고신 의대 내과
주 승 재
Connective Tissue Diseases
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Systemic lupus erythematosus
Antiphospholipid antibody syndrome
Ankylosing spondylitis
Rheumatoid arthritis
Scleroderma
Polymyositis and dermatomyositis
Mixed connective tissue disease
Cardiac Diseases in Pts with SLE
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Pericardial disease
 Pericarditis
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Valvular disease
 Fibrin deposits (Libman-Sacks)
 Fibrous thickening of leaflets and chordae
 Valvular regurgitation and/ or stenosis
 Infective endocarditis
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Coronary artery diseases
Myocardial diseae
Hypertrophy
Prevalence of Pericarditis in Pts with SLE
• Meta-analysis of 26 studies
(Mayo Clin Proc 1999;74:255)
 Clinical or echo prevalence
595/2,147 (28%)
 Autopsy prevelence
188/291 (65%)
 Cardiac tamponade
16/2,147 (0.7%)
• Echo prevalence
 22 – 54% (Control; 0 – 10%)
Valvular Disease Associated with SLE
• Valve masses or
Libman-Sacks vegetation
• Leaflet thickening
• Valvular regurgitation
• Valvular stenosis
Libman-Sacks Vegetation
Cauliflower-like or flat, red multiple spreading
masses of 2 – 4 mm in diameter present on the
free margins or line of closure of the heart valve
Echo findings
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(Cardiol Clin 1998;16;531)
Prevalence
TTE; 10%, TEE; 30%
Mitral and aortic valves
< 1 cm2 in size
Irregular borders
Heterogenous echo density
No independent motion
Associated with thickening or
regurgitation
Libman-Sacks Vegetation and MR
Abnormal Leaflet Thickening in SLE
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Valve thickness
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Prevalence
TTE; 30%,
TEE; 50%
Mitral and aortic valves
Generally diffuse but predominant on the mid and
tip portions
Commonly associated with valve regurgitation or
valve masses or both
Valve stenosis is rare (<3%).
Leaflet calcification is uncommon.
Involvement of the annular and subvalvular
apparatus is rare (1%).
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> 3 mm for MV and TV
> 2 mm for AV
Valvular Regurgitation in SLE
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The most frequent abnormality (up to 79%)
Moderate-to-severe regurgitation in 7% to 41%
MV>TV>AV>PV
Moderate or severe regurgitation is almost
always accompanied by leaflet thickening.
Differential Diagnosis
Libman-Sacks vegetation
• Infective endocarditis (IE)
 Vibratory or rotatory motion independent of
the leaflet motion
• Pseudoinfective endocarditis
 A clinical syndrome of active SLE mimics IE.
 Low WBC count
Elevation of antiphospholipid Ab
Negative or low positive CRP
Differential Diagnosis
Abnormal Leaflet Thickening in SLE
• Rheumatic valvular disease
 Leaflet thickening localized to the leaflet tips
 Chordal thickening, fusion, tethered motion
and calcification
• Age-related valvular disease
 Valve sclerosis is marked in the valve annulus.
 Frequently associated with calcification
Evolution of Valvular Disease in SLE
Resolve (24%), appear de novo (12%), or
persist but change over time (40%)
Initial
2 mon later
20 mon later
(NEJM 1996;335:1424)
Clinical Course of Pts with SLE
and Valvular DIsease
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Neither the presence nor the changes over time in valvular
disease were temporarily associated with pt’s age or with
the duration, activity, severity, or therapy of SLE
21% incidence of valve-related complications with a 5-yr F/U
 Symptomatic severe valvular regurgitation
 Infective endocarditis
 Ischemic stroke
Vegetation, valvulitis, and LA thrombus in 70 to 90%
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Mortality
 20% at 5 yrs
 Causes of death
Refractory HF, infective endocarditis, complicated postoperative
course and CVA
Therapy of Pts with SLE and
Valvular Disease
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Prosthetic valve replacement or valve repair
Higher morbidity and mortality of valve
replacement
Steroid or cytotoxic therapy has no effect on
the presence or the evolution of SLEassociated valvular disease
Antibiotic prophylaxis for dental or nonsterile
procedures
Antiplatelet therapy
Antiphospholipid Antibody Syndrome
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Venous or arterial thrombosis, recurrent fetal
loss, or thrombocytopenia accompanied by an
increased levels of antiphospholipid Ab (aPLs)
Primary or secondary (SLE)
Valvular lesions
 Vegetation, thickening, or regurgitation
 Prevalence
 32% to 38% in primary APS
 A significantly higher prevalence of valvular defects
in SLE pts with aPLs
Therapy
Long-term, high intensity oral anticoagulation (INR 3)
Aortic Root Disease and Valve Disease
Associated with Ankylosing Spondylitis
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Pathology
The inflammatory process predominantly of the
adventitia and intima of the aortic root results in a
fibroblastic reparative response and vascularized fibrous
tissue thickening
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Aortic valvulitis
 Cusp thickening and retraction
 Thickening of the aorto-mitral junction or subaortic bump
 Proximal aortitis leading to aortic root thickening and
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dilation
Aortic and mitral regurgitation
Aortic Root Disease and Valve Disease
Associated with Ankylosing Spondylitis
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Prevalence
 Autopsy studies; 24 to 100%
 Echo studies
TTE; 8 to 31%
TEE; 82% (control; 27%)
Echo findings
 Nonspecific thickening of aortic and mitral valves
 Increased echogenicity of the posterior aortic
wall and membraneous interventricular septum
 Mild-to-moderate aortic regurgitation
Aortic Root Disease and Valve Disease
Associated with Ankylosing Spondylitis
(Roldan et al. JACC 1998;32:1397)
TEE findings
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Aortic root
Thickening; 61%
Increased stiffness; 61%
Dilatation; 25%
Valve thickening
aortic valve 41%, mitral valve 34%
Nodularities of the aortic cusp and
basal thickening of the anterior
mitral valve leaflet (subaortic bump)
Valve regurgitation; 50% (moderate)
Aortic Root Disease and Valve Disease
Associated with Ankylosing Spondylitis
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No correlation with clinical features of AKS
Evolution (Roldan et al. JACC 1998;32:1397)
39-mon F/U of 25 patients
 New abnormalities; 6 (24%)
 Progression of valve regurgitation; 3 (12%)
 Resolved; 5 (20%)
Therapy
 Questionable role of corticosteroid
 Aortic and mitral valve replacement in pts with
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severe aortitis and valvular dysfunction
Prophylactic antithrombotic therapy
Antibiotic prophylaxis for infective endocarditis in
pts with moderate valvular regurgitation
Cardiac Disease Associated
with Rheumatoid Arthritis
• Pericarditis
 Autopsy studies; 40%
 Echo studies; 50%
 Clinically significant pericardial effusion;
< 3%
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Valvular heart disease
Coronary arteritis
Myocarditis
Conduction disturbance
Valvular Heart Disease Associated
with Rheumatoid Arthritis
• Valvular diseases
 Valvular thickening
 Valvular regurgitation
 Valvular granulomas
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Prevalence
 Autopsy studies; 23 to 75%
 Echo studies
TTE; 30%
TEE; Thickening
Valvular nodules
Valvular regurgitation
66%
50%
13%
Valvular Nodules Associated with
Rheumatoid Arthritis
• Unique to RA
• Small (<0.5 cm2)
• Oval in shape
• Well-defined border
• Homogenous reflectance
• Not calcified
• Usually single
(Cardiol Clin 1998;16;531)
Valvular Heart Disease Associated
with Rheumatoid Arthritis
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Correlation with clinical features
 A higher prevalence of valvular disease in pts with
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erosive polyarticular and nodular disease, systemic
vascularitis, and high titers of RA factor
No association with the pt’s age, duration of RA, or
peripheral nodular disease
Therapy
 A few cases of significant improvement of severe
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valvulitis with the use of steroids or cytotoxic therapy
Mitral and aortic valve replacement in severe
regurgitation
Cardiac Disease Associated with
Scleroderma
• Pericarditis
• Myocardial disease
 Myocardial fibrosis, myocarditis
 CHF; 5%
• Conduction disturbance
• Pulmonary hypertension
 One of the major causes of death
• Valvular heart disease
• Coronary artery disease
Valvular Heart Disease Associated
with Scleroderma
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Limited echocardiographic data
Nonspecific thickening of the
mitral or aortic valve
A high prevalence of MVP (67%)
Aortic valvulitis with a perforated
cusp and severe regurgitation
Noninfective mitral valve
vegetations similar to those of
SLE
(Cardiol Clin 1998;16;531)
Cardiac Disease Associated with
Polymyositis and Dermatomyositis
• Myocarditis
• Pericarditis
• Mitral valve prolapse; up to 50%
Cardiac Disease Associated with
Mixed Connective Tissue Disease
• Pericarditis
• Pulmonary hypertension and
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cor pulmonale
Mitral valve prolapse; up to 32%
Verrucous thickening of the mitral valve
and mitral regurgitation
Summary
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Valvular abnormalities unique to a specific disease
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Differential diagnosis
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Echocardiography, especially TEE, has the
potential to redefine the prevalence rates and to
characterize better the cardiac abnormalities
associated with connective tissue diseases.
 Libman-Sacks vegetation; SLE
 Valve nodules; RA
 Subaortic bump; AKS
 Infective endocarditis
 Rheumatic valvular disease
 Degenerative valvular disease