Makindo Medical Notes

Related Subjects: | Mitral Regurgitation (Incompetence) | Mitral Stenosis | Mitral Stenosis vs Regurgitation | Mitral Valve Prolapse | Atrial Fibrillation (AF) | Cardiac Valve Replacement | Prosthetic Valves

Mitral Valve Prolapse (MVP), also known as floppy mitral valve, is generally benign but can have significant associations. While most patients experience a benign clinical course, there is a potential risk of stroke and sudden cardiac death, emphasizing the need for appropriate monitoring and management.

About: Characteristics

• Also known as floppy mitral valve with multiple associations.
• Unclear whether associations are coincidental or causative.
• Most patients with MVP have a benign clinical course.
• There is an increased risk of stroke and sudden cardiac death in some cases.

Aetiology

• Possible autosomal dominant genetic component.
• Myxomatous degeneration of the valve, such as in Marfan's syndrome, involving defects in the fibrillin gene.
• Elevated urine and plasma catecholamine levels observed in patients with MVP.

Clinical Presentation

• Classically presents with a mid-systolic click followed by a mid to late systolic murmur, known as the "click-murmur" complex.
• Associated with atypical chest pain and palpitations due to arrhythmias.

Complications

• Endocarditis: Requires antibiotic prophylaxis.
• Sudden cardiac death: Very rare.
• Stroke: Also very rare.
• Development of mitral regurgitation.
• Atrial fibrillation (AF) and other arrhythmias: Rare occurrences.

Associations with MVP

• Genetic Syndromes:
  • Marfan's syndrome
  • Ehlers-Danlos syndrome
  • Pseudoxanthoma elasticum
  • Osteogenesis imperfecta
  • Pectus excavatum
• Other Conditions:
  • False positive exercise stress tests in females
  • Acute rheumatic fever
  • Ischaemic heart disease
  • Hypertrophic cardiomyopathy
  • Wolff-Parkinson-White syndrome
  • Von Willebrand's disease
  • Atrial septal defect (ASD) and Ebstein anomaly

Investigations

• Blood Tests:
  • Full Blood Count (FBC)
  • Urea & Electrolytes (U&E)
  • Liver Function Tests (LFTs)
  • C-reactive Protein (CRP) if needed
• Imaging:
  • Chest X-Ray (CXR): May show cardiomegaly in advanced severe cases.
  • Electrocardiogram (ECG) and 24-hour Holter monitor to detect arrhythmias.
  • Echocardiography: Essential for evaluating mitral valve anatomy and function. Early echocardiograms may over-diagnose MVP due to limited visualization.

Management

• Antibiotic Prophylaxis: Consider in cases with significant mitral regurgitation or when there is a history of endocarditis. The necessity of prophylaxis is debated and should be based on individual risk factors.
• Medications:
  • Beta-blockers: Indicated for palpitations and arrhythmias.
  • Aspirin or Warfarin: Considered if there is evidence of embolic phenomena.
  • Ace Inhibitors (ACEIs) and Angiotensin Receptor Blockers (ARBs): Avoid until postpartum due to potential fetal anomalies.
  • Hydralazine and Nitrates: Alternative medications during pregnancy.
• Non-Pharmacological:
  • Physiotherapy and regular exercise to maintain cardiovascular health.
  • Orthopedic surgery: In severe cases, spinal fusion may be necessary for related conditions.
• Special Considerations:
  • High risk of recurrence with future pregnancies. Discuss sterilization or effective contraception.
  • Access to neonatal Intensive Care Unit (NICU) if needed.

General Medical Advice for Pregnant Patients with MVP

• Prefer vaginal delivery over Caesarean section to reduce risks of pulmonary embolism (PE) and endometritis.
• Exclude preeclampsia as a differential diagnosis.
• Use IV Heparin instead of Low-Molecular-Weight Heparin (LMWH) for better control during pregnancy.
• Avoid ACEIs and ARBs to prevent fetal anomalies. Use hydralazine and nitrates instead, along with beta-blockers.
• Ensure access to neonatal Intensive Care Unit (NICU) if required.

References

• Ankylosing Spondylitis: A Review by Farrouq Mahmood and Philip Helliwell