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

An in-depth analysis of valvular abnormalities in various connective tissue diseases, including systemic lupus erythematosus, antiphospholipid antibody syndrome, ankylosing spondylitis, rheumatoid arthritis, scleroderma, polymyositis, and mixed connective tissue disease. It covers the prevalence, echo findings, differential diagnosis, and therapy for each disease.

Typology: Slides

2011/2012

Uploaded on 10/19/2012

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Role of Echo in
Connective Tissue Diseases
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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