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Common Congenital Heart Lesions - page 22 / 126





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22 of 126

Mitral Valve Prolapse (MVP)

  • -

    aka “floppy” mitral valve, myxomatous mitral valve, or Barlow’s syndrome Clinical Presentation:

    • Usually asymptomatic

    • Affected individuals may describe chest pains or palpitations because of associated arrhythmias

    • Clinical course of MVP is often benign

  • Most common complication: development of gradually progressive mitral regurgitation

  • Rare complications:

    • o

      rupture of myxomatous chordae to cause sudden severe regurgitation and pulmonary edema

      • o

        infective endocarditis

      • o

        peripheral emboli due to microthrombus formation behind the billowy valve tissue

      • o

        atrial or ventricular arrhythmias

Physical Exam:

  • Typical findings:

    • o

      midsystolic “click”

      • o

        late systolic murmur heard best at the cardiac apex

      • o

        click and murmur are both altered with sudden squatting (click and murmur come later in systole due to increased volume in the LV) and with sudden standing (click and murmur occur earlier in systole due to decreased volume in the LV)


  • condition may be inherited as a primary autosomal dominant disorder

  • may occur as a part of other connective tissue diseases such as the Marfan or Ehlers-Danlos syndromes

  • MVP occurs in about 2.4% of the population and is more common among women, especially those with a lean,

thin body type


  • Typical path features of MVP are:

    • o

      Enlarged valve leaflets, especially the posterior leaflet

      • o

        Instead of dense collage and elastin matrix, the valvular fibrosa is fragmented and replaced with loose, “myxomatous” connective tissue

      • o

        In severe MVP, may find: elongated or ruptured chordae, annular enlargement, and/or thickened leaflets


  • The midsystolic click likely corresponds to sudden tensing of the involved mitral leaflet or chordae tendinae as the

leaflet is forced back toward the LA.

  • Late systolic murmur corresponds to regurgitant flow through the incompetent valve.

Diagnostic imaging/testing:

  • Echocardiography:

oUsed to confirm diagnosis by demonstrating posterior displacement of one or both mitral leaflets into the LA during systole.

  • Electrocardiogram & Chest X-Ray:

oUsually normal (unless chronic MR has led to LA and LV enlargement).


  • Reassure the patient about the usually good prognosis for MVP.

  • Antibiotic prophylaxis for endocarditis, but only if substantial valve thickening or MR are present.

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