Role of Echo in Connective Tissue Diseases 고신 의대 내과 주 승 재

Role of Echo inConnective Tissue Diseases

고신 의대 내과

주 승 재

Connective Tissue Diseases

• Systemic lupus erythematosus

• Antiphospholipid antibody syndrome

• Ankylosing spondylitis

• Rheumatoid arthritis

• Scleroderma

• Polymyositis and dermatomyositis

• Mixed connective tissue disease

Cardiac Diseases in Pts with SLE• Pericardial disease

Pericarditis

• Valvular disease Fibrin deposits (Libman-Sacks) Fibrous thickening of leaflets and chordae Valvular regurgitation and/ or stenosis Infective endocarditis

• Coronary artery diseases

• Myocardial diseae

• Hypertrophy

• 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%)

Prevalence of Pericarditis in Pts with SLE

• Valve masses or

Libman-Sacks vegetation

• Leaflet thickening

• Valvular regurgitation

• Valvular stenosis

Valvular Disease Associated with SLE

Libman-Sacks Vegetation

• 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

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

(Cardiol Clin 1998;16;531)

Libman-Sacks Vegetation and MR

• Valve thickness > 3 mm for MV and TV> 2 mm for AV

• PrevalenceTTE; 30%, TEE; 50%

• Mitral and aortic valves• Generally diffuse but predominant on the mid and t

ip portions• Commonly associated with valve regurgitation or v

alve masses or both• Valve stenosis is rare (<3%).• Leaflet calcification is uncommon.• Involvement of the annular and subvalvular appara

tus is rare (1%).

Abnormal Leaflet Thickening in SLE

• 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.

Valvular Regurgitation in SLE

Differential Diagnosis Libman-Sacks vegetation

• Infective endocarditis (IE) Vibratory or rotatory motion independent of t

he leaflet motion

• Pseudoinfective endocarditis A clinical syndrome of active SLE mimics IE. Low WBC count

Elevation of antiphospholipid Ab

Negative or low positive CRP

• 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

Differential Diagnosis Abnormal Leaflet Thickening in SLE

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

• 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%

• Mortality 20% at 5 yrs Causes of death

Refractory HF, infective endocarditis, complicated postoperative course and CVA

• 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 SLE-associated valvular disease

• Antibiotic prophylaxis for dental or nonsterile procedures

• Antiplatelet therapy

Therapy of Pts with SLE and Valvular Disease

Antiphospholipid Antibody Syndrome• Venous or arterial thrombosis, recurrent fetal los

s, 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 i

n SLE pts with aPLs

• Therapy Long-term, high intensity oral anticoagulation (INR 3)

Aortic Root Disease and Valve Disease Associated with Ankylosing Spondylitis

• Pathology The inflammatory process predominantly of the adventiti

a and intima of the aortic root results in a fibroblastic reparative response and vascularized fibrous tissue thickening

• Aortic valvulitis Cusp thickening and retraction Thickening of the aorto-mitral junction or subaortic bump Proximal aortitis leading to aortic root thickening and dila

tion Aortic and mitral regurgitation

• 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 wa

ll and membraneous interventricular septum Mild-to-moderate aortic regurgitation


TEE findings 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)


(Roldan et al. JACC 1998;32:1397)

• 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 sev

ere aortitis and valvular dysfunction Prophylactic antithrombotic therapy Antibiotic prophylaxis for infective endocarditis in p

ts with moderate valvular regurgitation


Cardiac Disease Associated with Rheumatoid Arthritis

• Pericarditis Autopsy studies; 40% Echo studies; 50% Clinically significant pericardial effusion; < 3%

• Valvular heart disease

• Coronary arteritis

• Myocarditis

• Conduction disturbance

• Valvular diseases Valvular thickening Valvular regurgitation Valvular granulomas

• Prevalence Autopsy studies; 23 to 75% Echo studies

TTE; 30%

TEE; Thickening 66%

Valvular nodules 50%

Valvular regurgitation 13%

Valvular Heart Disease Associated with Rheumatoid Arthritis

• Unique to RA

• Small (<0.5 cm2)

• Oval in shape

• Well-defined border

• Homogenous reflectance

• Not calcified

• Usually single

Valvular Nodules Associated with Rheumatoid Arthritis


• Correlation with clinical features A higher prevalence of valvular disease in pts with er

osive 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 val

vulitis with the use of steroids or cytotoxic therapy Mitral and aortic valve replacement in severe regurgi

tation

Valvular Heart Disease Associated with Rheumatoid Arthritis

• 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

Cardiac Disease Associated with Scleroderma

• 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

Valvular Heart Disease Associated with Scleroderma


• Myocarditis

• Pericarditis

• Mitral valve prolapse; up to 50%

Cardiac Disease Associated with Polymyositis and Dermatomyositis

• Pericarditis

• Pulmonary hypertension and

cor pulmonale

• Mitral valve prolapse; up to 32%

• Verrucous thickening of the mitral valve and mitral regurgitation

Cardiac Disease Associated with Mixed Connective Tissue Disease

Summary• Valvular abnormalities unique to a specific disease

Libman-Sacks vegetation; SLE Valve nodules; RA Subaortic bump; AKS

• Differential diagnosis Infective endocarditis Rheumatic valvular disease Degenerative valvular disease

• Echocardiography, especially TEE, has the potential to redefine the prevalence rates and to characterize better the cardiac abnormalities associated with connective tissue diseases.

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Role of Echo in Connective Tissue Diseases 고신 의대 내과 주 승 재