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Vasospastic (Prinzmetal) Angina

Cardiology > Mitral Valve Prolapse

Mitral Valve Prolapse

1. Knuuti J, Wijns W, Saraste A, Capodanno D, Barbato E, Funck-Brentano C, et al. 2019 ESC Guidelines for the diagnosis and management of chronic coronary syndromes. Eur Heart J. 2020;41(3):407-477.
PMID: 31504439
DOI: https://doi.org/10.1093/eurheartj/ehz425


2. Fihn SD, Gardin JM, Abrams J, Berra K, Blankenship JC, Dallas AP, et al. 2012 ACCF/AHA/ACP/AATS/PCNA/SCAI/STS guideline for the diagnosis and management of patients with stable ischemic heart disease. J Am Coll Cardiol. 2012;60(24):e44-e164.
PMID: 23182125
DOI: https://doi.org/10.1016/j.jacc.2012.07.013


3. Khan MA, Hashim MJ, Mustafa H, Baniyas MY, Al Suwaidi SKBM, AlKatheeri R, et al. Global epidemiology of ischemic heart disease: Results from the Global Burden of Disease Study. Cureus. 2020;12(7):e9349.
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4. Ibanez B, James S, Agewall S, Antunes MJ, Bucciarelli-Ducci C, Bueno H, et al. 2017 ESC Guidelines for the management of acute myocardial infarction in patients presenting with ST-segment elevation. Eur Heart J. 2018;39(2):119-177.
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5. Amsterdam EA, Wenger NK, Brindis RG, Casey DE Jr, Ganiats TG, Holmes DR Jr, et al. 2014 AHA/ACC guideline for the management of patients with non–ST-elevation acute coronary syndromes. J Am Coll Cardiol. 2014;64(24):e139-e228.
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DOI: https://doi.org/10.1016/j.jacc.2014.09.017

   Background 

Mitral valve prolapse (MVP) is a valvular abnormality characterized by the systolic displacement of one or both mitral valve leaflets into the left atrium due to myxomatous degeneration or connective tissue abnormalities. This can lead to mitral regurgitation (MR) if leaflet coaptation is impaired. While often benign, MVP can be associated with arrhythmias, MR, and rarely sudden cardiac death. 

II) Classification or Types 

By Etiology: 

    • Primary (Sporadic or Genetic): 
      Associated with myxomatous degeneration; can be familial (autosomal dominant with variable expression). 
    • Secondary (Functional/Structural): 
      Occurs due to left ventricular remodeling (e.g., ischemic heart disease, hypertrophic cardiomyopathy). 

By Leaflet Morphology (on echocardiography): 

    • Classic MVP: Leaflet thickness ≥5 mm with redundancy 
    • Non-classic MVP: Leaflet thickness <5 mm 

By MR Severity: 

    • MVP with no MR 
    • MVP with mild, moderate, or severe MR 

III) Epidemiology 

    • Sex: More common in women 

    • Age: Typically diagnosed in young to middle-aged adults 

    • Prevalence: ~2–3% of the general population 

    • Comorbidities: May coexist with connective tissue disorders (e.g., Marfan syndrome, Ehlers-Danlos), scoliosis, or chest wall deformities 

  Etiology

I) What Causes It 

    • Myxomatous degeneration of mitral valve leaflets and chordae 

    • Connective tissue disorders (e.g., Marfan syndrome, Ehlers-Danlos syndrome) 

    • Papillary muscle or chordal dysfunction 

    • Idiopathic degeneration 

    • Secondary to ischemic cardiomyopathy or hypertrophic cardiomyopathy 

II) Risk Factors 

    • Family history of MVP 

    • Connective tissue disorders 

    • Female sex 

    • Low BMI or thin habitus 

    • History of chest trauma (rare) 

    • Mitral annular disjunction (associated with arrhythmic MVP) 

  Clinical Presentation

I) History (Symptoms) 

    • Often asymptomatic 

    • Atypical chest pain (non-exertional, sharp or stabbing) 

    • Palpitations or skipped beats 

    • Fatigue or reduced exercise capacity 

    • Dyspnea, especially if MR develops 

    • Anxiety or panic attacks (possibly autonomic in origin) 

    • Syncope or presyncope (rare, suggests arrhythmic MVP)

       

II) Physical Exam (Signs) 

Vital Signs: 

    • Usually normal; may have orthostatic changes in autonomic dysfunction 

Cardiac Exam: 

    • Midsystolic click followed by a late systolic murmur best heard at the apex 
      • Click and murmur timing may vary with position: 
      • Earlier with standing or Valsalva (decreased preload) 
      • Later with squatting (increased preload) 
      • Murmur may radiate to the axilla or base 

Other findings: 

    • Pectus excavatum or scoliosis in connective tissue disorders 
    • Features of Marfan or Ehlers-Danlos in syndromic patients 

  Differential Diagnosis (DDx)

    • Mitral regurgitation (other causes) 

    • Aortic stenosis or regurgitation 

    • Hypertrophic cardiomyopathy 

    • Tricuspid valve prolapse (rare) 

    • Anxiety or panic disorder 

    • Arrhythmogenic right ventricular cardiomyopathy (ARVC) 

    • Pericarditis (if chest pain present) 

  Diagnostic Tests

Initial Tests: 

    • Transthoracic Echocardiogram (TTE): 

      • Confirms leaflet prolapse >2 mm above annular plane 

      • Assesses MR severity and leaflet thickness 

      • Evaluates LV and LA size, systolic function 

      • Transesophageal Echocardiography (TEE): 

      • Better resolution; used if TTE inconclusive or pre-surgery 

      • Electrocardiogram (ECG): 

      • Often normal 

      • May show nonspecific ST-T changes or arrhythmias 

      • Holter Monitor or Event Recorder: 

      • For palpitations or syncope 

      • Detects supraventricular or ventricular arrhythmias 

      • Cardiac MRI: 

      • Useful for assessing myocardial fibrosis, mitral annular disjunction

         

  Treatment

I) Medical Management 

Asymptomatic MVP without MR: 

    • No specific treatment 
    • Reassurance and periodic monitoring 

Symptomatic MVP (e.g., palpitations, chest pain): 

    • Beta-blockers for palpitations or chest discomfort 
    • Avoid stimulants (e.g., caffeine, decongestants) 
    • Consider SSRI/SNRI if autonomic symptoms are prominent 

MVP with MR: 

    • Follow mitral regurgitation guidelines 
    • Diuretics, afterload reduction if heart failure develops 
    • Anticoagulation for atrial fibrillation 

II) Interventional/Surgical 

Indications (aligned with MR): 

    • Severe symptomatic MR 
    • Asymptomatic severe MR with reduced EF (≤60%) or LVESD >40 mm 
    • Valve repair preferred over replacement 

Surgical Options: 

    • Mitral valve repair (preferred) 
    • Mitral valve replacement (if repair not feasible) 
    • Transcatheter options (e.g., MitraClip) in select cases 

  Patient Education, Screening, Vaccines

    • Reassurance in benign MVP 

    • Educate on warning symptoms: worsening dyspnea, palpitations, syncope 

    • Avoid stimulants and dehydration 

    • Good hydration to reduce autonomic symptoms 

    • Maintain regular exercise (unless symptomatic MR present) 

    • Vaccines: 

    • Annual influenza 

    • Pneumococcal 

    • COVID-19 vaccine 

Endocarditis prophylaxis: 

    • Not indicated for isolated MVP unless prior endocarditis or prosthetic valve 

  Consults/Referrals

    • Cardiology: 
      • All patients with moderate to severe MR, arrhythmias, or progressive symptoms 
    • Electrophysiology: 
      • If ventricular arrhythmias or syncope 
    • Cardiothoracic Surgery: 
      • For consideration of mitral valve repair/replacement 
    • Genetic counseling: 
      • If syndromic features or family history of connective tissue disease 

  Follow-Up

    • Echocardiogram: 

    • Every 3–5 years in mild MVP without MR 

    • Annually or every 6–12 months if MR or LV dilation present 

    • Holter monitor if symptoms suggest arrhythmia 

    • Monitor for development or progression of MR 

    • Lifestyle counseling and reinforcement of red flags 

    • Adjust surveillance based on presence of arrhythmias or LV dysfunction 

 

 

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