Darrell Crase

Update: Mitral Valve Prolapse Syndrome

ABSTRACT A contemporary “in” diagnosis in the field of

cardiology is the mitral valve prolapse syndrome. This abnormality is evident in populations ranging from very young children to older adults. It is being recognized with increasing frequency among college and university students. In many cases, the syndrome is of limited con- sequence, while in others, it may be life threatening. This paper (1) defines what it is, (2) reports on its occur- rence, (3) depicts some relationships with related health entities and (4) discusses certain implications for health professionals and heart health education.

Mitral valve prolapse (MVP), also known as the click- murmur syndrome, Barlow’s syndrome, billowing mitral leaflet syndrome and floppy valve syndrome,’.* has been referred to as the “in” diagnosis in the cardiology field during the past ten years.3 Indeed, a cursory examination of contemporary medical literature adds strong support to this contention. There is a plethora of scientific papers now being published that focus on such dimensions of MVP as etiology, path- ology, incidence and therapy, among others. The purpose of this brief article is to update the health pro- fessional about the nature of the valvular syndrome and to draw certain implications germane to health educa- tion.

WHAT IS IT? Mitral valve prolapse may result from the

myxomatous degeneration of the leaflets of the mitral valve and chordae tendineae.‘ The tendineae, or thin fibrous strands, do not open or close the valve but act to limit the valve’s movements and prevent it from being everted. Other known and unknown causes of mitral valve prolapse may not be associated with the generative process.

The normal function of the mitral valve is to permit blood flow from the lungs into the left atrium to flow unimpeded into the left ventricle. During contraction of the heart, the valve closes and does not allow blood to regurgitate backwards to the left atrium. Persons with prolapse of the mitral valve have a portion of the valve or its structure pushed back too far (prolapsed) into the left atrium during ventricular contraction.’ A sound emanating in the corditendineae or from blood that is permitted to flow backwards through the billowing leaf- let(s) of the mitral valve may be heard through the steth- oscope as a mid-to-late systolic click or as a systolic murmur. Either of these may be heard alone, however.

Etiology of MVP in many cases is unknown. How- ever, it has been associated with a variety of congenital heart disorders,’ with children with Duchenne’s muscular dystrophy,6 among individuals with Marfan’s Syndrome, Turner’s Syndrome and a host of other dis- orders including a history of rheumatic fever.2 MVP has

354 THE JOURNAL OF SCHOOL HEALTH AUGUST 1982

also been observed to have a familial i n ~ i d e n c e 2 ~ ~ ~ ~ and occurs more among young women than among young men.3.4.9

In some cases, MVP may not be associated with any particular symptom. In other instances, it may be associated with rhythm disturbances referred to by some physicians as dysrhythmias (skipped heart beats, extra ventricular contractions or ventricular ectopy), fatigue, shortness of breath and dizziness. Many individuals with MVP are asymptomatic, however, with a diagnosis being made as a result of a clicking sound or valvular murmur detected during a routine physical examina- a t i ~ n . ~

Diagnosis of mitral valve prolapse is usually made through physical examinations. Echocardiography (valve scanning procedure) is performed in many cases as a confirmatory procedure to determine the etiology for dysrhythmias. Some other noninvasive techniques available to the physician include electrocardiography, cineangiography and exercise stress testing. A portable Holter monitor which can record the EKG over a 24-hour period is also helpful in identifying premature ventricular contractions, tachycardia-bradycardia (in- creased and decreased heart rate patterns) and atrial flutter.

INCIDENCE OF M V P Mitral valve prolapse has come under increasing

scrutiny in recent years by a number of investigators following published works by Barlow and associates. I ’

The syndrome is now observed among diverse groups. While many aspects clearly are not understood or re- quire clarification, authorities agree that MVP occurs in both sexes and at all ages. It has been found in “normal” populations ranging from 4 to 6.3%I2-l4 to nearly 18070.~~ As with any health problem, it should be noted that a number of MVP abnormalities go undiag- nosed while others identified through echocardio- graphic procedures may simply represent a variant of normal.’4 One source also indicates that for every patient identified with symptomatic MVP, there are hundreds of asymptomatic persons. l 6

Mitral valve damage is a condition considerably more common among college-age and older adults than among very young children. However, some investiga- tors have reported the syndrome in populations of very young children. For example, Pickoff and associates” identified six children with echocardiographic evidence of MVP out of 51 who had presented with ventricular dysrhythmias over a four-year period. A higher incidence of MVP in children has been reported by Brown. Mitral value prolapse was generally associated with chest pain and exercise tolerance and not so much

with premature ventricular contractions, excessive sweating, psychological difficulties or dizziness. Brown suggests that the rate of MVP is higher among children than heretofore suspected and calls for a greater aware- ness of the diverse nature of MVP among pediatricians in order to better diagnose and manage specific problems.

The presence of MVP syndrome has been reported in studies of school-age children,15J9-*l college students,‘ young ~ o m e n , ~ . ’ ~ young men,13.14 and has been associated with certain disease ~ t a t e s . ~ . ~ As director of a university health center, Bergy4 identified MVP as the most common heart disease evidenced in college and university health services today. During 1978 and 1979, 33 consecutive patients seen by the physician/director had been diagnosed with MVP with echocardiographic confirmation. These individuals, 24 women and nine men, ages 19-49, presented with 42% being asymptomatic and 50% with associated symptoms.

IS IT A SERIOUS HEALTH CONCERN? Mitral valve prolapse syndrome has been referred to

as a “medical infant” in terms of understanding its nature and long-term effect^.^ It is generally viewed as a nonlife-threatening entity and most individuals diag- nosed with it should expect to live a normal life.’ How- ever, there are some potentially complicating factors and many questions that remain to be answered by authorities in clinical and research settings.

Some of the dangerous factors associated with MVP syndrome are those of infective endocarditis, spontan- eous rupture of the chordae tendineae, serious dys- rhythmias, progressive mitral regurgitation and sudden death. According to berg^,^ there is considerable disagreement among authorities on these issues. It is recommended, however, for persons diagnosed with abnormal mitral valves to be protected against bacterial infection with prophylactic antibiotics prior to having certain procedures (eg, dental and gynecological) performed. The American Heart Association has recently published guidelines governing prophylaxis of endocarditis.22 Bacterial infection has been reported by various investigators though the percentage of cases among those with MVP is not high.23

Rhythm abnormalities (dysrhythmias) may be associ- ated with prolapsed valves. One of the more serious rhythmic disturbances, ventricular fibrillation, can lead to sudden death. Bergy reported data covering a two- year period. Nineteen (57%) of the university students with MVP presented with symptoms of dysrhythmias.‘ Following a review of pertinent literature, however, he indicates that a relatively small number of MVP patients experience life-threatening ventricular dysrhythmias.

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Regarding serious mitral regurgitation and rupture of the structures, it should be pointed out to persons with MVP that while these conditions are possible they happen infrequently. Bergy indicates that physicians need more time to accumulate data, but in the meantime

. . . it is prudent to tell patients that accurate data is not available, that progression to serious mitral regurgitation probably happens only to one or two persons out of every hundred, and that surgical valve replacement is a well- established procedure which can help those who d o have this outcome.4(~l38)

Less threatening dysrhythmias and premature ventricular contractions may be exacerbated through excessive use of such substances as coffee and cigarettes. Various forms of stress and/or excitement can also produce stimulation of the sympathetic nervous system and in turn lead to rhythmic disturbances. Many of these abnormalities can be controlled through modification of lifestyles. Drug therapy is also effective in the management of dysrhythmias.

Sudden death associated with MVP and related complications does occur and has been documented in some st~dies.~~-26 The percentage of deaths, however, is relatively small among those diagnosed with mitral valve prolapse syndrome. With the accumulation of additional data over longer periods, physicians will be better able to make conclusions relative to this issue.

EXERCISE RESTRICTIONS Some rhythm disturbances such as premature heart

beats usually do not threaten the individual nor require any modifications in exercise regimens. However, physical exercise and sports may be contraindicated among some persons with complications associated with mitral valve prolapse. l o The likelihood of ventricular tachycardia/fibrillation, for example, is one of the major threats among runners with MVP.I0 Wei and associates have demonstrated that high-grade ventricular ectopy is occasionally seen in association with MVP.27 Sudden death has also been observed among those whose lifestyles have included active par- ticipation in strenuous exercise

Overall, the existing literature suggests that cardiolo- gists do not recommend a significant limitation in exer- cise in the absence of potentially complicating factors. Each individual case necessitates management on its own merit again depending upon the diagnosis. Limit- ing exercise without justifying and supporting evidence might lead to further fears and misapprehensions. Until more research has been conducted and the literature reflects some degree of unanimity on the subject of exer- cise, physicians and health professionals must continue to individualize exercise regimens that range from no

restrictions at all, to confinement to a carefully super- vised, cardiac rehabilitation exercise program, to total avoidance of most exercise forms. In either case, the decision is clearly the responsibility of a physician.

IMPLICATIONS FOR HEALTH PROFESSIONALS

Mitral valve prolapse syndrome is now being diagnosed across the lifespan. According to one physi- cian, MVP “is the most common heart disease seen in college and university health se~vices.”~ In light of this, and since certain complications may attend serious forms of MVP, health professionals would be advised to become more cognizant of its nature. Mild MVP is usually a benign disorder that is associated with a normal lifespan. The major modification of lifestyle, in- cluding limitation of regular periods of exercise, is rarely needed and should not be required without a physician’s recommendation. The item most necessary to emphasize is the use of antibiotics for bacterial endocarditis propholaxis. Health professionals should also know something of the potential seriousness of MVP and understand that mental stress and certain stimulants may aggravate an existing abnormality.

The teaching of cardiovascular health education is an important function within health education for all learners including very young children.28 Students of all ages should know the caweleffect of such disorders as hypertension, coronary artery disease and vascular problems and be knowledgeable of primary risk factorsz9 in order to make prudent decisions that may lead to more positive lifestyles. So too, should heart health education include information relative to rhythmic disturbances and potential association with what is now being referred to as the mitral valve pro- lapse syndrome.

School health professionals can assist medical author- ities by helping to allay fears that may be associated with a positive diagnosis of MVP especially among college and university students who now range in age from 17 to 70. They can also stress that MVP syndrome (1) is fairly prevalent among college-age students, (2) re- quires antibiotic protection against possible infective endocarditis, (3) is usually benign and associated with a normal lifespan but may have complications, ( 5 ) occurs more frequently among female populations and has some family incidence, (6 ) is in its infancy in terms of completely understanding its long-term effects and (7) is a subject of some controversy among authorities.

356 THE JOURNAL OF SCHOOL HEALTH AUGUST 1982

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5. Rippe JM, Sloss LJ, Angoff G , et al: Mitral valve prolapse in adults with congenital heart disease. A m Heart J 97:561-573, 1979.

6. Sanyal SK, Leung RK, Tierney RC, et al: Mitral valve prolapse syndrome in children with Duchenne’s progressive muscular dystrophy. Pediatr 63:116-123, 1979.

7. Barlow JB, Pocock WA: Mitral valve prolapse, the specific billowing mitral leaflet syndrome, or an insignificant non-ejection systolic click. Am Heart J 97:277-285,1979.

8. Cooper MJ, Abinader EG: Family history in assessing the risk for progression of mitral valve prolapse. Am J Dis Child 135:647-649, July 1981.

9. Markiewicz W, Stoner J , London E, et al: Mitral valve prolapse in one hundred presumably healthy young females. Circulation 53:464, 1976.

10. Cantwell JD: Rhythms of runners. Phys and Sportsmed

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12. Procacci PM, Savran SV, Schreiter SL, et al: Prevalence of clinical mitral valve prolapse in 1169 young women. N Engl J Med

13. Darsee JR, Mikolich JR, Nicoloff NB, et al: Prevalence of mitral valve prolapse in presumably healthy young men. Circulation

14. Sbarbaro JA, Mehlman DJ, Wu L, et al: A prospective study of mitral valvular prolapse in young men. Chest 75:555-559, 1979.

15. Cohen M, Pocock WA, Lakier JB, et al: Four years follow-up of black school children with non-ejection systolic clicks and mitral systolic murmurs. Am Heart J 95:697-701, 1978.

16. Motulsky AG: Biased ascertainment and the natural history of diseases. N Engl J Med 298:1196-1201, 1978.

17. Pickoff AS, Gelband H , Ferrer P , et al: Premature ventricular contractions as the representing feature of mitral valve prolapse in childhood. J ofPediafr 94:615-617, 1979.

18. Brown LM: Mitral valve prolapse in children. In Barness LA (ed): Advances in Pediafrics, Vol. 25, Chicago: Year Book Medical Publishers, 1978, pp. 327-348.

19. Barlow JB, Pocock WA: The problem of nonejection systolic clicks and associated mitral leaflet syndrome. Am Heart J 90:636, 1975.

20. Engle MA: Heart sounds and murmurs in diagnosis of heart disease. Pediatr Annals 10:18-31, 1981.

21. Jennings RB Jr , Johnson DH, Moore AA: Myocardial infarction in early adolescence: A possible manifestation of the prolapsing mitral valve syndrome. J Pediafr 93:806-808, 1978.

22. American Heart Association Committee Report: Prevention of bacterial endocarditis. Circulufion 56: 139A-l43A, 1977.

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25. Shappell SD, Marshall CE, Brown RE, et al: Sudden death in the familial occurrence of the mid-systolic click, late systolic murmur syndrome. Circulation 48:1128-1134, 1973.

26. Jeresaty RM: Sudden death in the mitral valve prolapse: Click syndrome. Am J Cardiol37:317-318, 1976.

27. Wei JY, Bulkley BH, Schaeffer AH, et al: Mitral valve prolapse syndrome and recurrent ventricular tachyarrhythmias. Arch Intern Med 89:6-9, 1978.

28. Tevis B: The American Heart Association and heart health education in the young. J Sch Health 49:196-197,1979.

29. Crittenden IH: Risk factors in coronary heart disease: A child- hood concern. J Sch Health 49:210-212. 1979.

Darrell Crase, PhD, is with the Department of Health, Physical Education and Recreation, Memphis State University, Memphis, Tennessee 38152.

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