An unusual instance of mitral valve prolapse in endomyocardial fibrosisJJ Dalal1, AS Vengsarkar1, AM Mondkar1, Suman G Kinare2, KG Nair3
1 Department of Cardiology, Seth G.S. Medical College and K.E.M. Hospital, Parel, Bombay 400012, India
2 Department of Pathology, Seth G.S. Medical College and K.E.M. Hospital, Parel, Bombay 400012, India
3 Department of Medicine, Seth G.S. Medical College and K.E.M. Hospital, Parel, Bombay 400012, India
Correspondence Address: Source of Support: None, Conflict of Interest: None PMID: 745154
Source of Support: None, Conflict of Interest: None
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.
Considerable knowledge of endomyocardial fibrosis (EMF) has accumulated , since its recognition by Davies and his associates.  EMF is not an uncommon entity in India, particularly in the Southern areas of the country. ,
The syndrome of mitral valve prolapse with its multiple etiological factors is now well established.  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.
Mr. V. G... a 21 year old student presented with a history of progressively increasing dysponea 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 o (degrees) with biventricular hypertrophy.
The X-ray showed an enlarged heart (C.T, ratio 0.7) with a prominent main pulmonary artery and pulmonary venous congestion. The E.S.R. and the W.B.C. count were normal.
The echocardiogram was performed using a 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 191 A Camera.
The echocardiogram showed presence of left ventricular volume overload. The mitral valve excursions were brisk and a mid-late systolic prolapse of both the leaflets was present See [Figure 1] on page 236B.
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 236B showed pros. mitral regurgitation and an irregular left ventricular cavity strongly suggestive of an EMF Right ventricular angiography was not under taken as there was no clinical indication for it.
The patient received a Bjork Shiley prosthetic valve and was discharged following an uneventful post-operative period. One month later he was readmitted with severe breathlessness thought to be due to a paravalvular leak and died before a surgical re-exploration 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.
Mitral valve involvement is not unusual in left sided EMF  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  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.Y. can exist with EMF has been shown unequivocally.
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.
[Figure 1], [Figure 2]