Valvular Abnormalities in Connective Tissue Diseases: A Comprehensive Review, Slides of Cardiology

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Role of Echo in

Connective Tissue Diseases

Connective Tissue Diseases

• Systemic lupus erythematosus
• Antiphospholipid antibody syndrome
• Ankylosing spondylitis
• Rheumatoid arthritis
• Scleroderma
• Polymyositis and dermatomyositis
• Mixed connective tissue disease

• Meta-analysis of 26 studies (Mayo Clin Proc 1999;74:255)  Clinical or echo prevalence

 Autopsy prevelence

 Cardiac tamponade

• 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 and MR

• Valve thickness > 3 mm for MV and TV

  2 mm for AV

• 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%).

Abnormal Leaflet Thickening in SLE

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

• 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

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

Aortic Root Disease and Valve Disease Associated with Ankylosing Spondylitis

• 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

• Aortic valvulitis

 Cusp thickening and retraction

 Thickening of the aorto-mitral junction or subaortic bump

 Proximal aortitis leading to aortic root thickening and

dilation

 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

wall and membraneous interventricular septum

 Mild-to-moderate aortic regurgitation

Aortic Root Disease and Valve Disease Associated with Ankylosing Spondylitis

• 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

severe aortitis and valvular dysfunction

 Prophylactic antithrombotic therapy

 Antibiotic prophylaxis for infective endocarditis in

pts with moderate valvular regurgitation

Aortic Root Disease and Valve Disease Associated with Ankylosing Spondylitis

Cardiac Disease Associated with Rheumatoid Arthritis

• Pericarditis

 Autopsy studies; 40%  Echo studies; 50%  Clinically significant pericardial effusion;

• Valvular heart disease
• Coronary arteritis
• Myocarditis
• Conduction disturbance