Journal of Postgraduate Medicine
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CASE REPORT
 
 
Year : 1979  |  Volume : 25  |  Issue : 4  |  Page : 230-232  

An unusual instance of mitral valve prolapse in endomyo­cardial fibrosis

JJ Dalal1, AS Vengsarkar1, AM Mondkar1, SG Kinare2, KG Nair3,  
1 Department of Cardiology, Seth G. S. Medical College and K.E.M. Hospital, Parel, Bombay-400 012, India
2 Department of Pathology, Seth G. S. Medical College and K.E.M. Hospital, Parel, Bombay-400 012, India
3 Department of Medicine, Seth G. S. Medical College and K.E.M. Hospital, Parel, Bombay-400 012, India

Correspondence Address:
J J Dalal
Department of Cardiology, Seth G. S. Medical College and K.E.M. Hospital, Parel, Bombay-400 012
India

Abstract

This is a report of an unusual case showing a mitral valve pro­lapse in the presence of a biventricular endomyocardial fibrosis (EMF). The EMF was strongly suspected on left ventricular angio­graphy and later proved at autopsy. The prolapse of the mitral valve was detected at echocardiography. To the best of our knowledge this is the first report of a case of EMF associated with mitral valve prolapse.



How to cite this article:
Dalal J J, Vengsarkar A S, Mondkar A M, Kinare S G, Nair K G. An unusual instance of mitral valve prolapse in endomyo­cardial fibrosis.J Postgrad Med 1979;25:230-232


How to cite this URL:
Dalal J J, Vengsarkar A S, Mondkar A M, Kinare S G, Nair K G. An unusual instance of mitral valve prolapse in endomyo­cardial fibrosis. J Postgrad Med [serial online] 1979 [cited 2022 May 29 ];25:230-232
Available from: https://www.jpgmonline.com/text.asp?1979/25/4/230/42224


Full Text

 Introduction



Considerable knowledge of endomyo­cardial fibrosis (EMF) has accumulatedl. [1],[4] since its recognition by Davies and his associates. [3] EMF is not an uncommon en­tity in India, particularly in the Southern areas of the country. [6],[7]

The syndrome of mitral valve prolapse with its multiple etiologic al factors is now well established. [2] The essence of the pa­thology of EMF is ventricular fibrosis in­volving the papillary muscles which would prevent a prolapse of the mitral valve into the left atrium. The presence of both these features in this case is un­doubted, but the explanation is difficult. It is postulated that patchy involvement results in an uneven distribution of ten­sion in the mitral apparatus resulting in a prolapse.

 Case report



Mr. V.G. a 21 year old student presented with a history of progressively increasing dysp­noea over a period of three years along with exertional palpitations. He had suffered two episodes of moderate haemoptysis. There was no history of systemic embolization or conges­tive heart failure. He gave no history of rheumatic fever.

General examination revealed an averagely built man with a regular pulse rate of 90/min. and a blood pressure of 130/80 mm Hg. Promi­nent 'a' and 'v' waves were present in the jugular pulse. There was no cyanosis or club­bing. Precordial examination revealed an apex in the 6th left intercostal space in the anterior axillary line along with a marked right ventri­cular lift. On auscultation a grade 4/6 pansy­stolic murmur was present at the apex and conducted towards the left axilla. There was no click at the apex. There was evidence of severe pulmonary hypertension.

The electrocardiogram showed a QRS axis of +120 degrees with biventricular hypertrophy.

The X-ray showed an enlarged heart (C.T ratio 07) with a prominent main pulmonar, artery and pulmonary venous congestion. The E.S.R. and W.B.C. count were normal.

The Echocardiogram;

The echocardiogram was performed using Unirad Diagnostic Ultrasound Unit Model 902 The transducer used was a 2,25 mHz, non focus type with a repetition rate of 1000/sec. The recording was made on a photographic film roll 400 ASA, 120 size using a Hewlett Packard 197 A Camera.

The echocardiogram showed presence of left ventricular volume overload. The mitra valve excursions were brisk and a mid-lat( systolic prolapse of both the leaflets was pre­sent (See [Figure 1] on page 232 A).

Catheterization and angiography

The catheterization data is depicted in [Table 1]. A left ventricular angiogram in the R.A.O, position (See [Figure 2] on page 232A) showed glen mitral regurgitation and an irregular left ven­tricular cavity strongly suggestive of an EMF. Right ventricular angiography was not under­taken as there was no clinical indication for it.

The patient received a Bjork Shiley prosthe­tic valve and was discharged following an uneventful postoperative period. One month later he was readmitted with severe breath­lessness thought to be due to a paravalvular leak and died before a surgical reexploration could be attempted.

The autopsy showed presence of EMF in­volving both the ventricles. The mitral valve leaflets were not thickened and did not reveal any myxomatous degenerative change.

 Discussion



Mitral valve involvement is not un­usual in left sided EMF [5] and is usually based on the distortion of the mitral ap­paratus due to fibrosis of the endocardium and papillary muscles. In contrast to the usual feature of tethering of the mitral leaflets to the left ventricle, this case had prolapse of the valve into the left atrium. Though the literature on mitral valve pro­lapse is voluminous, [2] and the etiological factors multiple-and diverse, EMF has to our knowledge, not yet been associated with a prolapse of the mitral valve.

Though the autopsy has confirmed the diagnosis of EMF, it has not been of help in explaining the prolapse. The possibil­ity of unequal fibrosis and uneven distri­bution of tension as a cause of the pro­lapse remains unproven, but the fact that M.V.P. can exist with EMF has been shown unequivocally.

 Acknowledgements



We wish to thank the Dean, K.E.M. Hospital and Seth G.S. Medical College, Bombay for permission to publish this data. Thanks are also due to Mr. Sorab for technical assistance.

References

1Chew, C. Y. C., Ziady, G. M., Raphael M. J., Nellen, M. and Oakley, C. M.; Primary restrictive cardiomyopathy. Non­tropical endomyocardial fibrosis and hyper-eosinophilic heart disease. Brit, Heart J., 39: 399-413, 1977.
2Criley, J. M. and Kissel, G. L.: Prolapse of the mitral valve-The click and late systolic murmur syndrome. In, "Progress in Cardiology". (Editors-Yu, P. N. and Goodwin, J. F.), Vol. IV, Lea and Febiger, Philadelphia, 1975, pp. 23-36.
3Davies, J. N. P.: Endocardial fibrosis in Africans. East African Med. J, 25: 10-14, 1948 as Quoted by Falase et a1 [4]
4Falase, A. 0., Kolawole, T. M. and Lagundoye, S. B.: Endomyocardial fibro­sis: Problems in differential diagnosis. Brit. Heart J., 38: 369-374, 1976.
5Fowler, J. M. and Somers, K.: Left ven­tricular endomyocardial fibrosis and mitral incompetence: A new syndrome. Lancet, 1: 227-228, 1968.
6Nair, D. V.: Endomyocardial fibrosis in Kerala. Ind. Heart J., 23: 182-190.
7Reddy, D. J., Omer, S., Prabhakar, V., Shyamsunder, P. and Rao, K. S.: Endo­myocardial fibrosis. J. Ind. Med. Assoc., 45: 440-445, 1965.

 
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