Cardiology: Calcific Aortic Stenosis Quiz
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Questions and Answers

What is the primary feature of calcific aortic stenosis morphology?

  • Increased valve mobility
  • Inflammation in the valve tissue
  • Mounded calcified masses on the outflow surfaces of the cusps (correct)
  • Fused commissures

    • Statins can effectively prevent valvular calcific degeneration.

    False

    Which layer of semilunar valves is primarily affected by calcific aortic stenosis?

    Fibrosa

    Calcific nodules can be found at the base of the anterior mitral ________.

    <p>leaflet</p> Signup and view all the answers

    Match the following types of aortic stenosis with their characteristics:

    <p>Calcific aortic stenosis = No commissural fusion Rheumatic aortic stenosis = Fused commissures Congenital bicuspid valve stenosis = Bicuspid valve present Mitral annular calcification = Calcific nodules at the mitral leaflets</p> Signup and view all the answers

    What is a result of a valve failing to open completely?

    <p>Stenosis</p> Signup and view all the answers

    Mitral insufficiency can result from intrinsic disease of the valve leaflets.

    <p>True</p> Signup and view all the answers

    What is the most common cause of aortic stenosis?

    <p>Calcification and sclerosis of anatomically normal or congenitally bicuspid aortic valves</p> Signup and view all the answers

    Chronic injury to the valves is similar to factors implicated in __________.

    <p>atherosclerosis</p> Signup and view all the answers

    Match the following valvular lesions with their associated conditions:

    <p>Aortic stenosis = Calcification Aortic insufficiency = Dilation of the ascending aorta Mitral stenosis = Rheumatic heart disease Mitral insufficiency = Myxomatous degeneration or dilation</p> Signup and view all the answers

    What is a potential acute cause of valvular insufficiency?

    <p>Chordal rupture</p> Signup and view all the answers

    Calcific aortic stenosis is the most common valvular abnormality.

    <p>True</p> Signup and view all the answers

    At what age range does calcific aortic stenosis commonly occur?

    <p>7th to 9th decades of life</p> Signup and view all the answers

    What is a common complication associated with calcific aortic stenosis?

    <p>Angina pectoris</p> Signup and view all the answers

    Mitral annular calcification usually affects the valvular function significantly.

    <p>False</p> Signup and view all the answers

    What condition is associated with loss-of-function mutations in NOTCH1?

    <p>Congenitally bicuspid aortic valve</p> Signup and view all the answers

    Calcific nodules in mitral annular calcification may lead to an increased risk of __________.

    <p>embolic stroke</p> Signup and view all the answers

    Match the following conditions with their described features:

    <p>Calcific aortic stenosis = Obstruction to left ventricular outflow Mitral valve prolapse = Protrusion of leaflets into the left atrium during systole Mitral annular calcification = Ulcerated nodules at the base of the leaflets Congenitally bicuspid aortic valve = 50% of cases of aortic stenosis in adults</p> Signup and view all the answers

    Which of the following symptoms may occur in patients with calcific aortic stenosis?

    <p>Syncope</p> Signup and view all the answers

    Calcium deposits in mitral annular calcification can lead to arrhythmias.

    <p>True</p> Signup and view all the answers

    Name two complications that can arise from mitral valve prolapse.

    <p>Regurgitation and infective endocarditis</p> Signup and view all the answers

    What is a common complication of mitral valve prolapse?

    <p>Infective endocarditis</p> Signup and view all the answers

    Most individuals diagnosed with mitral valve prolapse (MVP) are symptomatic and have notable clinical features.

    <p>False</p> Signup and view all the answers

    What percentage of adults in the US are affected by Mitral Valve Prolapse?

    <p>2% to 3%</p> Signup and view all the answers

    What is the only recognized cause of mitral stenosis?

    <p>Rheumatic heart disease</p> Signup and view all the answers

    The characteristic delay in symptom onset of acute rheumatic fever is due to the time needed to generate an ________ response.

    <p>immune</p> Signup and view all the answers

    Mitral Valve Prolapse is a primarily serious condition that leads to severe complications in the majority of cases.

    <p>False</p> Signup and view all the answers

    What is the key histologic change in the tissue of Mitral Valve Prolapse?

    <p>Marked myxomatous degeneration of the spongiosa layer</p> Signup and view all the answers

    What is the most common age range for initial attacks of rheumatic fever (RF)?

    <p>5-15 years</p> Signup and view all the answers

    Match the following mitral valve complications with their descriptions:

    <p>Infective endocarditis = A serious infection of the heart lining. Mitral insufficiency = Failure of the mitral valve to close properly. Stroke = Cerebral infarct due to embolism. Arrhythmias = Irregular heartbeats that can be both ventricular and atrial.</p> Signup and view all the answers

    Which of the following symptoms might some patients with mitral valve prolapse experience?

    <p>Dyspnea</p> Signup and view all the answers

    Arthritis is more common in children than in adults with rheumatic fever.

    <p>False</p> Signup and view all the answers

    The characteristic anatomic change in Mitral Valve Prolapse is __________ of the mitral leaflets.

    <p>ballooning</p> Signup and view all the answers

    Match the secondary changes in the morphology of Mitral Valve Prolapse with their descriptions:

    <p>Fibrous thickening of valve leaflets = Thickening occurs where leaflets rub together Linear fibrous thickening of LV endocardial surface = Caused by abnormally long cords snapping Thrombi on atrial surfaces = Blood clots form at sites of contact Focal calcifications = Deposits at the base of the posterior mitral leaflet</p> Signup and view all the answers

    Acute rheumatic fever is a chronic condition that occurs several weeks after a respiratory infection.

    <p>False</p> Signup and view all the answers

    Name one of the classic clinical features associated with acute carditis in rheumatic fever.

    <p>pericardial friction rubs</p> Signup and view all the answers

    What is primarily responsible for the damage to heart tissue in acute rheumatic fever?

    <p>Antibody- and T cell-mediated reactions</p> Signup and view all the answers

    Which disorder is frequently associated with Mitral Valve Prolapse?

    <p>Marfan syndrome</p> Signup and view all the answers

    The _____ are composed of thrombotic debris and organisms in infective endocarditis.

    <p>vegetations</p> Signup and view all the answers

    Which of the following complications can arise from rheumatic heart disease (RHD)?

    <p>Arrhythmias</p> Signup and view all the answers

    The annulus of the mitral valve may be dilated in cases of Mitral Valve Prolapse.

    <p>True</p> Signup and view all the answers

    What anatomical change occurs to the mitral leaflets in Mitral Valve Prolapse?

    <p>The mitral leaflets become enlarged, redundant, thick, and rubbery.</p> Signup and view all the answers

    Match the following terms with their correct definitions:

    <p>Vegetations = Formation of thrombotic debris and organisms on heart valves Myocarditis = Inflammation of the heart muscle Rheumatic fever = An inflammatory disease triggered by streptococcal infection Infective endocarditis = Microbial infection of the heart valves or endocardium</p> Signup and view all the answers

    What is an important consideration in the prognosis of rheumatic heart disease?

    <p>Surgical repair or replacement of valves</p> Signup and view all the answers

    What types of infective endocarditis are classified based on severity?

    <p>Acute and subacute</p> Signup and view all the answers

    Study Notes

    Valvular Heart Diseases

    • Valvular disease can be stenosis, insufficiency, or both.
    • Sudden valvular disease can be caused by infection.
    • Chronic rheumatic mitral stenosis develops over years.
    • The failure of a valve to fully open obstructs blood flow.
    • Acquired valvular stenosis is chronic.
    • Valvular insufficiency occurs when the valve doesn't fully close, leading to backflow.
    • Valvular insufficiency can be caused by intrinsic valve leaflet disease or disruption of supporting structures.
    • Insufficiency can appear abruptly or insidiously
    • The most common valvular abnormality is calcific aortic stenosis.
    • Aortic stenosis is the calcification and sclerosis of normal or congenitally bicuspid aortic valves.
    • Aortic insufficiency is often due to ascending aorta dilation, related to hypertension and aging.
    • Rheumatic heart disease (RHD) is a cause of mitral stenosis.
    • Myxomatous degeneration can cause mitral insufficiency.
    • Left ventricular dilation can be a cause of mitral insufficiency.
    • Ischemic or nonischemic heart failure can result in mitral insufficiency.

    Etiology of Acquired Heart Valve Disease

    • Mitral Valve Disease:
      • Mitral Stenosis: postinflammatory scarring, typically from rheumatic heart disease.
      • Mitral Insufficiency: abnormalities of leaflets, commissures, postinflammatory scarring, infective endocarditis, mitral valve prolapse, and "Fen-phen".
    • Aortic Valve Disease:
      • Aortic Stenosis: postinflammatory scarring (rheumatic heart disease), senile calcific aortic stenosis, calcification.
      • Aortic insufficiency: intrinsic valvular disease, postinflammatory scarring, infective endocarditis, Aortic disease, degenerative aortic dilation, syphilitic aortitis, ankylosing spondylitis, rheumatoid arthritis, Marfan syndrome.

    Calcific Aortic Stenosis

    • The most common valvular abnormality, with prevalence of 2%.
    • Common in the 7th to 9th decades
    • In bicuspid valves, stenosis can appear much earlier (1-20 decades earlier).
    • Bicuspid valves have more mechanical stress than normal valves.
    • A common finding associated with bicuspid valves is dilation of the proximal ascending aorta.
    • Age related wear and tear/ recurrent chronic injury (atherosclerosis, hyperlipidemia, and hypertension
    • The abnormal valve cells resemble osteoblasts (synthesize bone matrix proteins) that promote calcium salt deposition.
    • Statins or other measures do not prevent calcific degeneration of the valves.
    • Calcified masses are on the outflow surfaces of the cusps.
    • The layered valve architecture is preserved.
    • Inflammation is variable, but metaplastic bone is often present.

    Semilunar Valves

    • Semilunar valves (aortic and pulmonary valves) have three layers: fibrosa, spongiosa, and ventricularis.

    Calcific Aortic Stenosis, Morphology

    • Calcium masses accumulate within the sinuses of Valsalva.
    • Commissures aren't fused, unlike in post-rheumatic aortic stenosis.

    Mitral Annular Calcification

    • Calcification forms in the fibrous annulus.
    • The calcification appears as irregular, hard nodules.
    • The nodules are typically at the base of the leaflets.

    Mitral Annular Calcification, Secondary Changes

    • Fibrous thickening of leaflets, particularly where they rub against one another.
    • Linear thickening on the LV endocardium, where abnormally long cords rub against the endocardium.
    • Thickening of the LV mural endocardium or LA as a result of friction injury from prolapsing leaflets.
    • Thrombi on atrial surfaces of leaflets or atrial walls.
    • Focal calcification at the posterior mitral leaflet base.

    Myxomatous Degeneration of the Mitral Valve

    • Mitral valve leaflets are "floppy" and protrude into the atrium during systole.
    • The afflicted leaflets are frequently enlarged, redundant, thick, and rubbery.
    • The tendinous cords might be elongated, thinned, or ruptured.
    • The annulus might be dilated.

    Mitral Valve Prolapse, Pathogenesis

    • Typically unknown but may be associated with heritable connective tissue disorders and Marfan syndrome

    Mitral Valve Prolapse, Morphology

    • The characteristic change in MVP is hooding of the mitral leaflets.
    • The affected leaflets tend to be enlarged, redundant, thick, and rubbery
    • The related chordae tendineae may be elongated, thinned, or ruptured.
    • The annulus may be dilated.

    Mitral Valve Prolapse, Histology

    • The key histologic change is marked myxomatous degeneration of the spongiosa layer.
    • Collagenous fibrosa layer attenuates, affecting structural integrity

    Mitral Valve Prolapse, Secondary changes

    • Fibrous thickening of the valve leaflets as they rub against one another.
    • Linear fibrous thickening along the LV endocardium where abnormally long cords rub against it.
    • Thickening of the LV or LA mural endocardium, caused by friction-induced injury from prolapsing leaflets.
    • Thrombi on the atrial surfaces of the leaflets or walls.
    • Focal calcification at the base of the posterior mitral leaflet.

    Rheumatic Fever and Rheumatic Heart Disease

    • Rheumatic fever is an acute, immunologically mediated, multisystem inflammatory disease that typically follows group A streptococcal pharyngitis.
    • Active Rheumatic fever is often accompanied by a few weeks of group A streptococcal infections in other sites , like the skin
    • Acute rheumatic carditis manifests prominently, frequently progressing into chronic rheumatic heart disease (RHD)

    Rheumatic Heart Disease (RHD)

    • Characterized by deforming fibrotic valvular disease, especially the mitral valve.
    • The only known cause of mitral stenosis
    • A significant public health problem in low-income countries.

    Acute Rheumatic Fever, Pathogenesis

    • Cause: host immune responses to group A streptococcal antigens that cross-react with host proteins.
    • A 2- to 3-week delay in symptoms is common, reflecting the time for an immune response.
    • Heart tissue damage is due to a combination of antibody- and T-cell-mediated reactions.
    • Streptococci are absent in the lesions.

    Acute Rheumatic Fever, Morphology

    • During acute RF, focal inflammation is seen in various tissues.
    • Distinctive lesions in the heart (Aschoff bodies) are comprised of T lymphocytes, occasional plasma cells, and plump activated macrophages (Anitschkow cells).
    • Macrophages exhibit abundant cytoplasm and central nuclei with chromatin condensed into a centrally located slender wavy ribbon. These are referred to as "caterpillar cells"
    • Inflammation is widespread throughout the heart layers.
    • Necrotic foci and small vegetations (verrucae) are present along lines of closure.
    • Subendocardial lesions may develop, often exacerbated by regurgitant jets.
    • Thickened areas are known as MacCallum plaques.

    RHD Morphology

    • Chronic RHD is characterized by thickened mitral leaflets, commissural fusion, and shortening.
    • The mitral valve is always involved in chronic RHD, sometimes in isolation in about two-thirds of cases.
    • Mitral valves are more commonly affected, often in conjunction with the aortic valve about 25% of the time.
    • Tricuspid valve involvement is less frequent.

    RHD, Micro

    • Valves show acute inflammatory organization, neovascularization and transmural fibrosis.
    • Aschoff bodies are seldom identified in tissue specimen from patients with chronic RHD, due to the interval between the initial insult and deformities.

    RHD, Morphology of acute mitral valvulitis

    • Acute rheumatic mitral valvulitis superimposed upon chronic rheumatic heart disease.
    • Small vegetations (verrucae) are visible along the line of closure of the mitral valve leaflet.

    Acute Rheumatic Carditis, Micro

    • Myocardium exhibits a circumscribed nodule of mixed mononuclear inflammatory cells with associated necrosis
    • Large activated macrophages have prominent nucleoli, and chromatin condensed into long, wavy ribbons (caterpillar cells).

    RHD, Morphology of mitral stenosis/thickning

    • Mitral stenosis shows diffuse thickening and distortion of the mitral valve leaflets and commissural fusion
    • Chordae tendineae thickening.

    Rheumatic Aortic Stenosis

    • Surgical specimens show thickening and distortion of the cusps, as well as commissural fusion.

    Rheumatic Fever, Clinical Features

    • Migratory polyarthritis of large joints
    • Subcutaneous nodules (typically on extensor surfaces of extremities)
    • Erythema marginatum (occurs in 5% of patients), the skin manifestation.
    • Sydenham chorea (neurologic disorder, characterized by involuntary movements)

    Rheumatic Fever, Diagnosis

    • Evidence of preceding group A streptococcal infection, and two or more of the following :
      • Two major manifestations or a combination of one major and two minor manifestations.
    • Criteria for minor manifestations are less specific and include fever, arthralgia, elevated acute-phase reactants.

    Rheumatic Fever, Diagnosis (Acute RF)

    • Acute RF typically appears 10 days-6 weeks after group A streptococcal infection about 3% of patients and predominantly affects 5-15 year olds.
    • Pharyngeal cultures are typically negative by the time the illness begins, but antibodies to streptococcal enzymes (e.g., streptolysin O, Dnase B).
    • Predominant clinical symptoms are often carditis and arthritis in both adults and children, though arthritis is more commonly found in adults.
    • Polyarthritis, accompanied by fever, is common; one joint after another becomes painful and swollen, and subsides spontaneously without residual disability.

    Rheumatic Fever, Diagnosis (Clinical Features of Acute Carditis)

    • Clinical features related to acute carditis include pericardial friction rubs, tachycardia, and arrhythmias.
    • Myocarditis can cause cardiac dilation, leading to functional mitral valve insufficiency.
    • 1% of those affected die from fulminant cardiac involvement.

    Rheumatic Fever, Diagnosis (Clinical Features)

    • Cardiac murmurs
    • Cardiac hypertrophy
    • Cardiac dilation,
    • Heart failure
    • Arrhythmias (particularly atrial fibrillation associated with mitral stenosis)
    • Thromboembolic complications

    RHD, Prognosis & Treatment

    • RHD outcomes are variable.
    • Surgical valve repair or replacement greatly improves survival outlook

    Infective Endocarditis (IE)

    • Microbial infection of the heart valves or mural endocardium, leading to vegetation formation.
    • Vegetations are comprised of thrombotic debris and microorganisms, often causing underlying cardiac tissue destruction.
    • Prompt diagnosis, pathogen identification, and effective treatment are critical.

    Infective Endocarditis, Classic Classification

    • Acute IE, caused by highly virulent organisms like S. aureus, rapidly produces destructive lesions and is often difficult to treat with antibiotics alone
    • Subacute IE, caused by less virulent organisms like viridans streptococci, is characterized by insidious infections of deformed valves with less destruction.

    Infective Endocarditis, Pathogenesis

    • RHD, MVP, degenerative calcific valvular stenosis, bicuspid aortic valves (or not), artificial heart valves and congenital cardiac defects increase risk of IE.
    • Common pathogens for IE include Streptococcus viridans (normal oral flora), Staphylococcus aureus (often associated with IV drug users), enterococci, HACEK group ((Haemophilus, Actinobacillus, Cardiobacterium, Eikenella, and Kingella).
    • In 10% of IE cases, no organism is isolated, often due to prior antibiotic therapy, difficulties isolating the agent, and organisms being deeply embedded in the vegetation.

    Infective Endocarditis, Pathogenesis (Source of bacteremia/fungemia).

    • Infections elsewhere.
    • Dental or surgical procedure.
    • Contaminated IV drug needles and usage
    • Breaks in epithelial barriers ( gut, oral cavity, skin).
    • Patients with valvular abnormalities or ongoing bacteremia may benefit from antibiotic prophylaxis.

    Infective Endocarditis, Morphology

    • Vegetations on heart valves are often friable, bulky, potentially destructive, and contain fibrin, inflammatory cells, and bacteria.
    • Aortic and mitral valves are most frequently involved, but valve involvement of the right side of the heart is particularly frequent in intravenous drug abusers.
    • Vegetations can be single or multiple, may involve more than 1 valve, and can erode into the underlying myocardium forming abscesses (abscess rings)
    • Vegetations can cause embolization, leading to septic infarcts or mycotic aneurysms.

    Infective Endocarditis, Morphology (Subacute IE)

    • Subacute IE vegetations have less valvular destruction compared to acute IE; this distinction is often subtle microscopically.
    • Granulation tissue is often present in the bases of the vegetations, indicative of healing.
    • Fibrosis, calcification, and a chronic inflammatory infiltrate may occur with time

    Infective Endocarditis. Clinical Features

    • Acute IE is rapid onset with fever, chills, weakness, and lassitude.
    • Fever can be mild or absent, while manifestations may include nonspecific fatigue, weight loss, and flu-like symptoms in older adults.
    • Many patients with left-sided IE present with a murmur.

    Modified Duke criteria for Infective Endocarditis

    • Diagnosis utilizing either clinical or pathological criteria. Clinically, 2 major criteria, 1 major + 3 minor or 5 minor criteria are needed for definitive IE diagnosis
    • Possible IE is defined as 1 major criterion + 1 minor criterion or 3 minor criteria

    Nonbacterial Thrombotic Endocarditis (NBTE)

    • Deposition of sterile thrombi on the leaflets of the cardiac valves.
    • Histologically, these thrombi are bland, nondestructive, and loosely attached to the valve; no inflammatory reaction is evident.
    • Thrombi can be a source for systemic embolization leading to infarction of the brain, heart, or other tissues.
    • NBTE is often found in debilitated patients with conditions like sepsis, cancer, hypercoagulability (chronic disseminated intravascular coagulation, hyperestrogenic states, and malignancy), or conditions like an indwelling catheter.

    Endocarditis of Systemic Lupus Erythematosus (Libman-Sacks Disease)

    • Small (1-4 mm) sterile vegetations, typically seen in the context of SLE & antiphospholipid syndrome.
    • Immune complex deposition that involves activation of complement and recruitment of Fc-receptor cells cause damage
    • These vegetations might occur anywhere on the valve leaflets, chordae or endocardium (atrial or ventricular)
    • Ultimately valvular scarring and leaflet fusion result from persistent injury.

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    Test your knowledge on calcific aortic stenosis and its morphology. This quiz covers the features, causes, and associated conditions related to aortic stenosis. Understand the implications of valve dysfunction and related complications in clinical practice.

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