Heart & vascular pathology

Valvular disease

Degenerative valve disease



Last author update: 13 April 2023
Last staff update: 13 April 2023

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PubMed Search: Degenerative valve disease

Rohail Jameel, M.D.
Aliya N. Husain, M.D.
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Cite this page: Jameel R, Li H, Husain AN. Degenerative valve disease. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/heartdegenerative.html. Accessed February 23rd, 2024.
Definition / general
  • Degenerative valve disease is a term used to describe deterioration of the valve over time, which leads to either stenosis or regurgitation from the valve (J Am Coll Cardiol 2006;47:1707)
Essential features
  • Degenerative mitral valve disease is more common than aortic valve disease
  • Degeneration happens over time due to multiple pathophysiologies
  • Diagnosis is made clinically
  • Aortic valve often needs replacement whereas mitral valve can be repaired
Terminology
  • Calcific aortic stenosis
  • Mitral annular calcification, mitral degenerative disease
  • Mitral valve prolapse (Barlow syndrome), floppy valve disease, balloon mitral valve
ICD coding
  • ICD-10: I35.9 - nonrheumatic aortic valve disorder, unspecified
Epidemiology
  • Calcific aortic stenosis is seen in ~9 million people worldwide
  • Calcific aortic stenosis is highly age related; an increasing trend is noted in developed countries (Nat Rev Cardiol 2021;18:853)
  • Degenerative mitral valve disease is seen in ~24 million people (Nat Rev Cardiol 2021;18:853)
Sites
  • Aortic and mitral valves
Pathophysiology
  • In calcific aortic stenosis, prior injury due to hyperlipidemia, hypertension, inflammation and other factors precede calcification of the valve
    • This leads to the release of VEGF, leading to angiogenesis and collagen X which leads to calcification of the valve
    • These valves contain abnormal cells which resemble osteoblasts, which synthesize bone matrix and deposit calcium (J Cardiol 2018;71:215)
  • In degenerative mitral stenosis, calcific deposits develop in the peripheral fibrous ring (annulus), which are stony hard, irregular ulcerated nodules which appear behind the leaflets
  • In degenerative mitral valve prolapse, there are portions of the leaflets that become thick, redundant and rubbery
  • Over time, the chordae tendineae become thinner and rupture, leaving the annulus dilated; this rupture allows the valves to become floppy and prolapse into the atrium during systole
  • Reference: Kumar: Robbins & Cotran Pathologic Basis of Disease, 10th Edition, 2020
Etiology
  • Old age is the most common cause of degenerative valve disease
  • Early occurrence of degeneration can occur if the patient has a prior valve disease (Int J Stroke 2018;13:612)
Diagrams / tables

Images hosted on other servers:
Pathophysiology of degeneration of valves Pathophysiology of degeneration of valves

Pathophysiology of degeneration of valves

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Potential pathways

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MVP subtypes

Diagnosis
Radiology description
  • Calcified aortic valve
    • Late in the disease, chest radiograph might reveal aortic calcifications and enlargement of the heart along with pulmonary congestion and alveolar edema
    • On echocardiogram, severity is determined by various factors
      • Mild aortic stenosis: jet velocity of 2.6 - 2.9 m/s and aortic valve area of > 1.5 cm
      • Moderate aortic stenosis: jet velocity of 3 - 4 m/s and aortic valve area of 1 - 1.5 cm
      • Severe aortic stenosis: jet velocity of > 4 m/s and aortic valve area of < 1 cm (Radiopaedia: Aortic Valve Stenosis [Accessed 19 January 2023])
  • Mitral valve prolapse
  • Mitral annular calcification
Case reports
  • 39 year old man with epilepsy, hypertension and a 1 month history of worsening shortness of breath was diagnosed with pulmonary edema (BMJ Case Rep 2019;12:e228414)
  • 50 year old man with a history of atrial fibrillation, hypertension, diverticulitis and asthma presented with fatigue and moderate exertional dyspnea (Autops Case Rep 2018;8:e2018058)
  • 55 year old man with a history of erosive, seropositive rheumatoid arthritis and interstitial lung disease presented with shortness of breath (Front Cardiovasc Med 2017;4:14)
Treatment
  • In severe aortic valve stenosis, transcatheter aortic valve replacement offers a better survival rate; percutaneous balloon valvotomy can also serve as a treatment option for poor surgical candidates
  • In mitral valve disease, transcatheter mitral valve repair is considered better than open heart surgery due to the low risk associated with it (Mayo Clin Proc 2010;85:483)
Gross description
  • Calcified aortic valve
    • Heaped up calcified cusps that prevent valve opening is hallmark
    • In the early stages, calcification begins in the valvular fibrosa at the point of maximal cusp flexion
    • Gradual narrowing of the orifice is noted
  • Mitral annular calcification
    • Calcific deposits are seen as irregular, stony hard and ulcerated nodules
  • Mitral valve prolapse
    • The leaflets appear enlarged, rubbery and thick with the connecting chordae tendineae appearing as thin, elongated or sometimes even ruptured
  • Reference: Kumar: Robbins & Cotran Pathologic Basis of Disease, 10th Edition, 2020
Gross images

Contributed by Aliya N. Husain, M.D.
Mitral valve

Mitral valve



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Minimally diseased
aortic valve (left) and
severely stenotic
aortic valve (right)

Microscopic (histologic) description
  • Calcified aortic valve
    • Large nodular calcific deposits obstructing the functional valve area
  • Mitral valve prolapse
    • Outer collagenous layer appears weakened, whereas the middle spongiosa layer appears thicker due to deposition of myxomatous material
    • Over time, there is more deposition of myxomatous material leading to rupture of the chordae tendineae
  • Reference: Kumar: Robbins & Cotran Pathologic Basis of Disease, 10th Edition, 2020
Microscopic (histologic) images

Contributed by Aliya N. Husain, M.D.
Mitral valve

Mitral valve

Aortic valve calcification

Aortic valve calcification

Mitral valve Mitral valve

Mitral valve

Sample pathology report
  • Mitral valve, repair:
    • Fibromyxoid valvular degeneration
Differential diagnosis
  • Bicuspid aortic valve:
    • Congenital disease
    • On gross appearance, aortic valve presents with 2 cusps compared to a normal valve of 3 cusps
  • Rheumatic heart valve:
    • Acute rheumatic fever
      • On gross examination, there are small vegetations on involved valves, typically mitral and aortic valves
      • On microscopic examination, Aschoff bodies (collections of lymphocytes, plasma cells and activated macrophages) can be seen in any part of the heart, including valves and myocardium
    • Chronic rheumatic fever
      • On gross examination, valves exhibit leaflet thickening, commissural fusion and shortening along with fusion of the chordae tendineae
      • On microscopic examination, the valves exhibit fibrosis and calcifications
  • Infectious endocarditis:
    • On gross appearance of both acute and subacute infectious endocarditis, friable and bulky vegetations are seen on the valve
    • On microscopic examination, the vegetations consist of fibrin, inflammatory cells and microorganisms
    • In subacute infectious endocarditis, granulation tissue may be present; fibrosis and calcification occurs over time
Board review style question #1
Which specific degenerative valve disease is the most common worldwide?

  1. Bicuspid aortic valve
  2. Calcific aortic stenosis
  3. Degenerative mitral valve
  4. Myxomatous mitral valve degeneration
Board review style answer #1
C. Degenerative mitral valve. Degenerative mitral valve disease is the most common, occurring in ~24 million people worldwide compared with 9 million with calcific aortic stenosis, which is the second most common.

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Board review style question #2
What test is used for definitive diagnosis of degenerative mitral valve?

  1. Chest MRI
  2. Chest ultrasonography
  3. Echocardiogram
  4. Physical examination
Board review style answer #2
C. Echocardiogram. Patients undergo auscultation at regular intervals to confirm the diagnosis, whereas a definitive diagnosis can be made with an echocardiogram once the diagnosis has been established.

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