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mitral valve prolapse (MVP)

Etiology: 1) primary, idiopathic - familial 2) in association with disorders: a) Marfan's syndrome b) Ehlers Danlos syndrome c) adult polycystic kidney disease d) osteogenesis imperfecta e) pseudoxanthoma elasticum f) 15% of patients with Ebstein's anomaly 3) secondary to a) papillary muscle dysfunction due to myocardial ischemia b) dilated mitral annulus due to dilated cardiomyopathy c) small left ventricular cavity - hypertrophic cardiomyopathy - hypovolemia - atrial septal defect (septum secundum_ Epidemiology: 1) common condition - most common cause of clinically significant mitral regurgitation - most patients have minimal to no mitral regurgitation [1] 2) occurs in 3-5% of population > 15 years of age Pathology: 1) thickening of mitral valve leaflets 2) thinning, stretching & sometimes rupture of chordae tendinae 3) dilation of mitral annulus Genetics: 1) autosomal dominant inheritance, incomplete penetrance 2) 3 loci identified, but genes are unknown (see OMIM) a) MMVP1 (chromosome 16p) b) MMVP2 (chromosome 11p15) c) MMVP3 (chromosome 13p31) Clinical manifestations: 1) chest pain, palpitations, dizziness, dyspnea, syncope 2) panic attacks may occur 3) high-pitched mid-systolic click followed by late systolic murmur - heard best at apex of heart (lower left sternal border) - not right second intercostal space - click not described as ejection click - click related to tensing of the chordae tendinae or valve leaflets [1] 4) systolic murmur of mitral regurgitation intensified by: a) isometric handgrip b) Valsalva maneuver c) squatting to standing d) also causes click to occur earlier [1] Special laboratory: 1) electrocardiogram 2) 24 hour HOLTER for arrhythmias 3) echocardiogram a) mitral regurgitation b) indicators of MVP severity 1] enlarged left atrium 2] enlarged left ventricle 3] thickened anterior mitral valve leaflet Complications: 1) arrhythmias a) PAC, paroxysmal SVT b) ventricular arrhythmias, PVC, ventricular ectopy b) sudden death 2) syncope 3) MVP increases risk of stroke in patients with a) ischemic heart disease b) congestive heart failure 4) endocarditis a) risk higher than general population b) absolute risk is low c) emboli 5) sudden cardiac death, absolute risk is low Management: 1) endocarditis prophylaxis a) for patients with systolic murmurs, but not systolic clicks [1] b) no longer indicated [4,5] 2) avoidance of dehydration or excessive caffeine 3) beta blockers to treat chest pain & arrhythmias, anxiety, palpitations or fatigue [1] 4) aspirin for TIA if sinus rhythm & not atrial thrombi 5) anticoagulation with warfarin a) recurrent TIA or stroke b) atrial fibrillation & > 65 years of age 6) surgery a) significant mitral regurgitation b) flail leaflet caused by ruptured chordae tendineae c) marked elongation of chordae tendineae [1] 7) patient education - avoidance of competitive athletics (or vigorous physical activity if: 1] history of syncope 2] family history of sudden death from MVP 3] sustained or non-sustained ventricular arrhythmias

Related

heart murmur mitral regurgitation (MR)

General

valvular heart disease

Database Correlations

OMIM correlations

References

  1. Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 17, 18. American College of Physicians, Philadelphia 1998, 2006, 2015, 2018. - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  2. National Heart, Lung, and Blood Institute (NHLBI) Mitral Valve Prolapse https://www.nhlbi.nih.gov/health-topics/mitral-valve-prolapse
  3. UpToDate 14.1 http://www.utdol.com
  4. Wilson W et al, Prevention of infective endocardititis: guidelines from the American Heart Association Rheumatic Fever, Endocarditis and Kawasaki Disease Committe, Council on Cardiovascular Disease in the Young, and Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. J AM Dent Assoc 2008, 139:3S
  5. Choosing Wisely. Feb 23, 2015 Infectious Diseases Society of America Five Things Physicians and Patients Should Question http://www.choosingwisely.org/doctor-patient-lists/infectious-diseases-society-of-america/