Valvular heart disease
Connective tissue diseases

Author: R. Amita, M.D. (see Authors page)

Revised: 19 March 2018, last major update September 2014

Copyright: (c) 2014-2018, PathologyOutlines.com, Inc.

PubMed Search: Connective tissue diseases [title] heart

Cite this page: Amita, R. Connective tissue diseases. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/heartconnectivetissue.html. Accessed March 20th, 2018.
Definition / general
  • Systemic autoimmune inflammatory conditions frequently involving the blood vessels and heart
  • Echocardiographic and autopsy studies show valvular abnormalities in up to 70% of patients with systemic autoimmune disease, although far fewer patients have symptoms
  • In seronegative spondyloarthropathies, aortic root or left sided valvular insufficiency is noted in up to 100% of cases in autopsy series
  • Mitral valve is most frequently affected, followed by the aortic, tricuspid, and pulmonic valves, in descending order of involvement
Diagrams / tables

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Rheumatic diseases and sites of involvement

  • The mechanisms of antiphospholipid antibody associated valvular dysfunction are unknown, but several have been suggested:
    1. Immune complexes may injure the valvular endothelium
    2. Disruption of phospholipid interaction between endothelial cells and platelets
    3. Capillaries within the valvular endothelium may be damaged
  • Any of these processes may lead to subsequent thrombotic and fibrotic changes of the valve
Clinical features
  • Valvular disease associated with autoimmune diseases is most often clinically quiescent but a few patients develop rapid, progressive valvular incompetence
  • Cardiac involvement can be the first presentation of systemic autoimmune disease
  • Neither serologic nor acute phase reactant monitoring or sequential imaging is completely reliable to detect recurrent or ongoing inflammation
  • If elevation of acute phase reactants is associated with aortitis preoperatively, this may be useful to follow, with the caveat that normal levels of acute phase reactants do not exclude active progressive vascular inflammation
Radiology description
  • Serial imaging by magnetic resonance angiography with edema weighted images may be helpful in detecting wall thickening or "edema," but these findings do not always indicate active inflammation
  • Conversely, inflammation can exist or progress without visible changes on imaging
Case reports
  • Bacterial endocarditis prophylaxis should be considered for these patients with valvular heart disease following American Heart Association guidelines (AHA: Prevention of infective (bacterial) endocarditis (2017))
  • Patients with SLE who have persistent antiphospholipid antibodies (measured in the same laboratory at least twice, at least 6 weeks apart) without the clinical syndrome do not require treatment, but often are given low dose aspirin (81 mg / day)
Clinical images

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Transthoracic echocardiogram

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Transesophageal echocardiogram

Gross description
  • Rheumatoid arthritis:
    • Fibrosis, thickening and calcific changes detected most commonly at the base of the valve and in the valve ring
  • SLE:
    • Most common is nonspecific thickening of the mitral and aortic valves
    • Owing to this thickening, the valve cusps may not close properly
    • The most characteristic valvular abnormality is Libman-Sacks endocarditis: noninfectious verrucous valvular vegetations, most commonly on the mitral valve but often on multiple valves
    • The verrucae are most often found in the recess between the ventricle wall and posterior valve leaflet but can involve either surface of the valve, the commissures and the rings and, less commonly, the chordae, papillary muscles and endocardium
    • Healing of these verrucous lesions results in scarring and shortening of leaflets with valvular insufficiency
  • Antiphospholipid Antibody Syndrome (APLA syndrome):
    • Both primary and secondary APLA syndrome are associated with an increased prevalence of cardiovascular abnormalities, e.g. nonspecific thickening of valve leaflets, thrombotic valvular vegetations, free floating thrombi and valvular insufficiency
  • Ankylosing spondylitis:
    • Aortic root dilation, valvular fibrosis with retraction of the cusp bases and inward rolling of the cusp margins
    • Additionally, mitral insufficiency may occur as a result of subaortic fibrosis of the anterior valve leaflet
Gross images

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Stenotic trileaflet aortic valve

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Myxomatous mitral valve disease

Microscopic (histologic) description
  • SLE: microscopic appearance may vary with hematoxylin bodies, immunoglobulin deposits, fibrin and granular material
Microscopic (histologic) images

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Various images