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Key Features

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Essentials of Diagnosis
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  • Midsystolic click

  • Late systolic "whooping" or "honking" murmur

  • Typical symptoms include chest pain, palpitations, and dizziness.

  • Often overdiagnosed on routine cardiac ultrasound

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General Considerations
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  • Occurs in about 2%of thin female adolescents, a minority of whom have concomitant mitral insufficiency

  • Although mitral valve prolapse is usually an isolated lesion, it can occur in association with connective tissue disorders such as

    • Marfan syndrome

    • Loeys-Dietz syndrome

    • Ehlers-Danlos syndrome

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Clinical Findings

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  • Most patients are asymptomatic

  • A midsystolic click (with or without a systolic murmur) is the hallmark of this entity.

  • Chest pain

  • Palpitations

  • Dizziness

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Diagnosis

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Imaging
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  • Chest radiography

    • Not usually indicated

    • Left atrium may be enlarged in the rare case of significant mitral valve insufficiency

  • Echocardiography

    • Assesses the degree of myxomatous change of the mitral valve and the degree of mitral insufficiency

    • Significant posterior systolic movement of the mitral valve leaflets to the atrial side of the mitral annulus is diagnostic

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Diagnostic Procedures
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  • Electrocardiography

    • Usually normal

    • Diffuse flattening or inversion of T waves may occur in the precordial leads

    • U waves are sometimes prominent

  • Other testing

    • Invasive procedures are rarely indicated

    • Holter monitoring or event recorders may be useful in establishing the presence of ventricular dysrhythmias in patients with palpitations

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Treatment

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  • Propranolol may be effective in treatment of coexisting arrhythmias

  • Prophylaxis for infectious endocarditis is no longer indicated

  • Surgery for mitral insufficiency is rarely needed

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Outcome

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Prognosis
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  • Twenty years of observation indicate that isolated mitral valve prolapse in childhood is usually a benign entity

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Reference

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Delling  FN, Vasan  RS: Epidemiology and pathophysiology of mitral valve prolapse: new insights into disease progression, genetics, and molecular basis. Circulation 2014 May 27;129(21):2158–2170
[PubMed: 24867995] .
CrossRef

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