British Heart Journal, 1979, 41, 730-733

Mitral valve replacement for mitral stenosis causedby Libman-Sacks endocarditisK. C. VAUGHTON, D. R. WALKER, AND M. F. STURRIDGE

From the Department of Cardiothoracic Surgery, The Middlesex Hospital, Mortimer Street, London

SUMMARY A woman who developed mitral stenosis from Libman-Sacks endocarditis is described. Themitral valve was replaced by a Starr-Edwards prosthesis. One year later, despite her being maintainedon steroids and azathioprine, the verrucous endocarditis progressed to cause sudden, severe dysfunctionof the prosthetic valve.

A non-bacterial verrucous endocarditis which waslater shown to be associated with systemic lupuserythematosus was described by Libman and Sacksin 1924. Since then endocardial involvement foundat necropsy in patients with systemic lupus erythe-matosus has been reported in 13 to 63 per cent ofcases (Kong et al., 1962; Heijtmancik et al., 1964;Bulkley and Roberts, 1975a). Valvular involvementrarely produces significant haemodynamic dys-function (Kong et al., 1962; Heijtmancik et al.,1964), and only 4 instances of valve replacementhave been reported. Shulman and Christian (1969)described 3 patients with aortic regurgitation, 1 ofwhom required valve replacement and a furthercase was added by Oh et al. (1974). Mitral valvereplacement has been described by Murray et al.(1975) and 1 has been reported from the NationalInstitute of Health, Bethesda, Maryland (Myero-witz et al., 1974; Paget et al., 1975). We report apatient with systemic lupus erythematosus whopresented with obstruction of the mitral valve andafter mitral valve replacement proceeded to obstruc-tion of the prosthesis within one year of operation.As far as we know, no case has been previouslydescribed in which active verrucous endocarditishas progressed to cause serious dysfunction of aprosthetic valve.

Case report

A 36-year-old housewife who had no past historyof rheumatic fever was admitted in January 1975.For 5 months she had suffered from a febrile illnessassociated with myalgia and painful swollen kneeand ankle joints. One month before admission sheexperienced 3 episodes of pleuritic chest pain. Twoweeks later she noted shortness of breath on oneflight of stairs and developed a dry cough, trouble-

some at night. On examination she was normo-tensive and in sinus rhythm. There were no signs ofcongestive cardiac failure. The pulmonary com-ponent of her second heart sound was loud and ashort soft diastolic murmur was heard at the apex.The rest of her physical examination was normal.Investigations showed her haemoglobin to be 12-1g/dl, white cell count 11 000/mm3 with a normaldifferential, ESR 55 mm/h, blood urea 6 mmol/l.Antinuclear antibody was negative but lupuserythematosus cells were present. Blood cultureswere consistently negative. Electrocardiogramshowed sinus rhythm and left atrial hypertrophy.Echocardiography indicated mitral valve diseasewith thickening and rigidity of the valve cusps. Theintracardiac pressures found at cardiac catheterisa-tion were as follows: right atrium: mean 9 mmHg;right ventricle: 50/0-10 mmHg; pulmonary artery:50/24 mmHg; pulmonary wedge pressure: mean31 mmHg; left ventricle: 125/0-13 mmHg; aorta:125/80 (mean 95) mmHg. A left ventricular angio-gram showed a filling defect arising from the inter-ventricular septum. This was thought to be either athickened papillary muscle or vegetations associatedwith the mitral valve (Fig. 1).A diagnosis of systemic lupus erythematosus was

made and it was thought the patient had endo-cardial involvement affecting the mitral valve. Thepatient was treated with frusemide, prednisone 30mg daily, and azathioprine 100 mg daily and hercondition improved.

In March 1976 she was readmitted with in-creasing shortness of breath and ankle oedema. Shewas apyrexial. Her jugular venous pressure wasraised 2 cm and she had fine crepitations at bothbases. The apical diastolic murmur was stillpresent. Electrocardiogram showed sinus rhythmand there was pulmonary venous congestion on the

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Mitral valve replacement for mitral stenosis caused by Libman-Sacks endocarditis

Fig. 1 Left ventriculogram showing a filling defect whichwas thought to be vegetations associated with the mitralvalve.

chest x-ray film. Her haemoglobin was 10-3 g/dl,white cell count 12 000/mm3, with a normaldifferential and ESR 74 mm/hour.

In view of her deteriorating clinical condition andthe presence of mitral stenosis it was decided toexplore her mitral valve. At operation the orifice ofthe mitral valve was almost completely obscured bya plaque of red granulation tissue resembling clot.A tiny aperture at the anterolateral commissurewas the only communication to the left ventricle.The tissue extended onto the left atrial wall andmeasured 5 cm in diameter by 0 5 cm in thickness.It was so adherent to the valve cusp that it could notbe separated. The valve was, therefore, excised withit and the affected left atrial wall was scraped clean.The valve was replaced by a No. 3M Model 6120Starr-Edwards prosthesis without complication.There was no evidence of disease in the left ven-tricle.

Examination ofthe valve showed it to be thickenedand fibrotic with increased vascularity. A largefibrinous vegetation was attached to its uppersurface. Areas offibrinoid degeneration were presentwithin the valve and the histological appearanceswere considered to be consistent with the diagnosisof Libman-Sacks endocarditis. No infective agentwas found (Fig. 2).The patient made an uncomplicated recovery and

was discharged on prednisone 10 mg daily, azathio-

prine 100 mg daily, warfarin, and frusemide.She remained well on the above regimen until

January 1977 when she developed more discomfortand swelling in the knees and ankles and her ESRwas found to have risen from 42 to 62 mm/hour.Prednisone was increased to 15 mg daily. InFebruary 1977 she was readmitted as an emergencywith a 3-day history of increasing shortness ofbreath, haemoptysis, and paroxysmal nocturnaldyspnoea. On examination she was cold and clammy.Her pulse rate was 100 per minute, blood pressure90/60 mmHg, and there were signs of severe pul-monary oedema. A loud pansystolic murmur washeard at the apex and radiated to the axilla. It wasthought she had developed acute mitral regurgita-tion with resultant cardiac failure. Her conditiondeteriorated rapidly after admission and she had acardiac arrest. She was immediately resuscitated,taken to theatre and put on cardiopulmonary by-pass. At operation the ball of the mitral prosthesiswas found to be fixed in the open position. Three-quarters of the orifice below the valve seating wasobstructed by friable, acellular tissue leaving asmall fixed opening which was both obstructive andregurgitant. When the prosthesis was removed theleft ventricular cavity was found to contain a largemass of similar tissue attached to the posterior wall.This was removed with the prosthesis and a newNo. 4M Model 6120 Starr-Edwards valve wasinserted.

Postoperatively the patient remained critically ill.She was unconscious, unresponsive, and developedepileptic fits. She became anuric and requiredperitoneal dialysis. In spite of all supportivemeasures her condition deteriorated and she died 4days later.

Histological examination of the tissue removedfrom around the prosthetic valve showed it to con-sist predominately of collagen, areas of which werenecrotic. It was relatively acellular but occasionalplasma cells were present. On the surface were bothorganised and unorganised thrombus. Necropsyshowed severe bilateral pulmonary oedema andhepatic venous congestion. There was a right-sidedcerebral infarction. Both kidneys showed the grossappearances of acute tubular necrosis and on histo-logical examination the characteristic lesions ofsystemic lupus erythematosus were seen.

Discussion

The initial diagnosis of systemic lupus erythe-matosus in this patient was made on the history ofarthritis without deformity. the evidence of pleuralinvolvement, a raised sedimentation rate, and thepresence of lupus erythematosus cells. When a

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K. C. Vaughton, D. R. Walker, and M. F. Sturridge

Fig. 2 Excised mitralvalve with adherentvegetations.

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hII1I i I I I

diastolic murmur at the apex was heard the possi-bility of Libman-Sacks endocarditis was raised. Amurmur is the commonest initial cardiovascularmanifestation (Kong et al., 1962). There is, however,a poor correlation between the appearance of amurmur and the presence of Libman-Sacks valvu-litis at necropsy (Shearn, 1959; Kong et al., 1962).Mitral valve involvement was demonstrated in thiscase by echocardiography and cardiac catheterisa-tion.The left ventricular filling defect seen at cardiac

catheterisation (Fig. 1) was thought to be a mass ofverrucous material associated with the mitral valve.A similar appearance has been reported by Seningenet al. (1974). At the initial operation, however, nomass was seen and the left ventricle was grossly freeof disease. At the second operation a large mass oftissue was found attached to the posterior wall of theleft ventricle and this was the cause of the prostheticvalve dysfunction.The pathology of the endocarditis has been well

described (Gross, 1940; Klemperer et al., 1941).Histological examination of the tissue removed fromthis patient did not show all the classical featuresbut the absence of lupus erythematosus cells andhaematoxyphil bodies has been reported in othercases (Oh et al., 1974) and was attributed to the

effect of steroid therapy. Recently BuLkley andRoberts (1975a) have also drawn attention to thealteration to pathology induced by corticosteroidtherapy. They postulate that 'healing' of Libman-Sacks endocarditis may occur by fibrosis and calcifi-cation. This was rare before the advent of steroidsbut now, either as a direct effect of the drug or as aresult of increased longevity of the patients, this'healing' process is seen more commonly. Oneconsequence of this could be to increase the inci-dence of valve dysfunction by scarring (Bulkley andRoberts, 1975a).The distribution of the endocardial lesions also

has been modified by steroid therapy. Previously allfour valves were commonly involved but now thereis a preponderance of left-sided lesions affectingparticularly the mitral valve (Heijtmancik et al.,1964; Buildey and Roberts, 1975b). In the two pre-viously reported cases of mitral valve replacementin this condition the lesions had produced mitralregurgitation. It is of interest that in this patient thevegetations produced predominant mitral stenosis.Our patient had an initially successful mitral

valve replacement but over the course of a year theendocarditis progressed to produce malfunctionof the prosthesis, which was ultimately fatal. Duringthis period therapy with prednisone and azathio-

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Mitral valve replacement for mitral stenosis caused by Libman-Sacks endocarditis

prine was maintained and though her sedimentationrate had remained moderately raised at about 42mm/hour she had been asymptomatic. One monthbefore her final admission she developed a recur-rence of joint pains and the dose of prednisone wasincreased. Perhaps, in spite of being asymptomatic,the therapy was inadequate and she should havebeen on higher doses. However, Heijtmancik et al.(1964) question the value of steroids in preventingLibman-Sacks endocarditis and the history of thispatient may provide further evidence to supportthis view.We, therefore, offer a warning that the endo-

cardial manifestations of the disease can progressdespite therapy, and relatively silently, to causesudden and severe dysfunction of a prosthetic valve.In addition, we feel that the disease behaves like amalignant growth with local extensions, and perhapsthe affected portion of left atrial wall, found at theinitial operation, should have been excised.

We thank Dr F. Scadding for referring this patient.

References

Bulkley, B. H., and Roberts, W. C. (1975a). The heart insystemic lupus erythematosus and the changes induced in itby corticosteroid therapy. American Journal of Medicine, 58,243-264.

Bulkley, B. H., and Roberts, W. C. (1975b). Systemic lupuserythematosus as a cause of severe mitral regurgitation.American Journal of Cardiology, 35, 305-308.

Gross, L. (1940). The cardiac lesions in Libman-Sacksdisease. American Journal of Pathology, 16, 375-408.

Heijtmancik, M. R., Wright, J. C., Quint, R., and Jennings,F. L. (1964). The cardiovascular manifestations of systemiclupus erythematosus. American Heart Journal, 68, 119-130.

Klemperer, P., Pollack, A. D., and Baehr, G. (1941). Pathologyof disseminated lupus erythematosis. Archives of Pathology,32, 569-631.

Kong, T. Q., Kellum, R. E., and Haserick, J. R. (1962).Clinical diagnosis of cardiac involvement in systemic lupuserythematosus. Circulation, 26, 7-11.

Libman, E., and Sacks, B. (1924). A hitherto undescribed formof valvular and mural endocarditis. Archives of InternalMedicine, 33, 701-737.

Murray, F. T., Fuleihan, D. S., Cornwall, C. S., and Pinals,R. S. (1975). Acute mitral regurgitation from rupturedchordae tendineae in systemic lupus erythematosus. 'ournalof Rheumatology, 2, 454-459.

Myerowitz, P. D., Lawrence, M. L., and McIntosh, C. L.(1974). Mitral valve replacement for mitral regurgitationdue to Libman-Sacks endocarditis. Journal of Thoracicand Cardiovascular Surgery, 67, 869-874.

Oh, W. M. C., Taylor, R. T., and Olsen, E. G. J. (1974).Aortic regurgitation in systemic lupus erythematosusrequiring aortic valve replacement. British Heart Jrournal,36, 413-416.

Paget, S. A., Bulkley, B. H., Grauer, L. E., and Seningen, R.(1975). Mitral valve disease of systemic lupus erythema-tosus. A cause of severe cardiac failure reversed by mitralvalve replacement. American J7ournal of Medicine, 59, 134-139.

Seningen, R. P., Borer, J. S., Redwood, D. R., Bulkley, B. H.,and Paget, S. A. (1974). Libman-Sacks endocardoma:diagnosis during life with radiographic, fluoroscopic, andangiocardiographic findings. Radiology, 113, 597-598.

Shearn, M. A. (1959). The heart in systemic lupus erythema-tosus. American Heart Journal, 58, 452-466.

Shulman, H. J., and Christian, C. L. (1969). Aortic insuffi-ciency in systemic lupus erythematosus. Arthritis andRheumatism, 12, 138-146.

Requests for reprints to M. F. Sturridge, Esq.,F.R.C.S., The Department of CardiothoracicSurgery, The Middlesex Hospital, MortimerStreet, London WlN 8AA.

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