Incidence

Equally distributed between men and women; more common in young women, but higher incidence of complications in men.

Prevalence

• Most commonly diagnosed cardiac valvular abnormality.
• 2–3% of the general population based on current criteria.
• MVP in Marfan syndrome is reported to be between 40 and 80%

• Most patients are asymptomatic.
• MVP syndrome includes atypical chest pain, dyspnea, palpitations, syncope, and anxiety.
• Serious complications, such as endocarditis, cerebrovascular events, progressive mitral regurgitation (MR), arrhythmias, and sudden cardiac death, are uncommon but related to higher morbidity.
• Moderate to severe MR and depressed left ventricular (LV) function are major risk factors for complications.
• Mild MR, flail leaflet, left atrial enlargement, atrial fibrillation, and age older than 50 are minor risk factors for complications.

• Usually has a benign course and excellent prognosis.
• Survival rate is similar to that in an age-matched and sex-matched population without MVP.

History

• Age of diagnosis
• Complications from MVP, such as endocarditis, cerebrovascular events, MR, and arrhythmias
• Associated disorders
• Exercise tolerance

Signs/Physical Exam

• Mid-systolic click, often accompanied by a late systolic murmur in mild MVP.
• Holosystolic murmur in severe MR; click may also disappear.
• S3 and rales seen in CHF.
• Other signs of CHF, such as pulmonary edema, JVD, and lower extremity edema.
• Look for signs of connective tissue disorders.

Labs/Studies

• Echocardiography for diagnosis, as well as assessment of LV function and severity of MR.
• EKG if there is concern for arrhythmias.
• CXR may show pulmonary congestion if the patient is in heart failure.
• Other studies dictated by medications and other comorbidities.
• Send blood cultures if there is concern for endocarditis.

• Ensure sufficient anxiolysis to avoid tachycardia, which may reduce LV size and possibly worsen prolapse and regurgitation.
• Give premedication judiciously in patients with hemodynamically significant MR and depressed LV function.
• Bacterial endocarditis prophylaxis should be considered in patients with mitral valve "click" and regurgitation or high-risk features including:
  • Prosthetic cardiac valve
  • Previous endocarditis
  • Cyanotic congenital heart disease that is unrepaired (including those with palliative shunts and conduits).
  • Completely repaired congenital heart disease with prosthetic material or device for the first 6 months post-procedure (either surgical or catheter intervention).
  • Repaired congenital heart disease with residual defects at the site or adjacent to the site of a prosthetic patch or prosthetic device.
  • Cardiac transplantation recipients with cardiac valvular disease.

Parental consent if a minor.

• Dependent on procedure, provider preference, and patient preference.
• Functional MVP without significant MR or LV dysfunction will tolerate all forms of anesthesia. However, those with significant LV dysfunction should be considered for local or peripheral nerve blocks when possible or appropriate.

• Dependent on the extent of procedure.
• Patients with functional MVP usually do not require invasive monitoring.
• Patients with significant MR and LV dysfunction may benefit from an arterial line for continuous blood pressure monitoring and a central line for vasopressor support. Consider a pulmonary artery catheter and/or transesophageal echocardiography (TEE) for cases involving large volume shifts.

• In patients with functional MVP, avoid hemodynamics that will enhance MVP such as decreases in LV end-diastolic volume, decreases in SVR, increases in contractility, or tachycardia.
• In patients with significant MR, hemodynamic goals should promote forward flow such as mildly increased heart rate, increased preload, and decreased SVR while avoiding myocardial depression.

• Minimize sympathetic stimulation and undesirable decreases in LV emptying that may worsen prolapse, as above.
• Patients with significant MR will benefit from hemodynamic goals that improve forward flow, as above.
• Intra-operative arrhythmias may occur especially in head up positions (presumably due to decrease in preload and accentuation of MVP). Consider beta blockers or lidocaine.
• Fluids: Maintenance of a positive fluid balance will help with decreases in preload that may accentuate prolapse or MR.

Avoid excessive sympathetic stimulation which may worsen prolapse or MR.