regurgitation systemic lupus erythematosus requiring ... · Aortic regurgitation in systemic lupus...
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British Heart Journal, I974, 36, 4I3-4i6. Aortic regurgitation in systemic lupus erythematosus requiring aortic valve replacement W. M. C. Oh, R. T. Taylor,' and E. G. J. Olsen From the National Heart Hospital and Westminster Hospital, London A woman with proven systemic lupus erythematosus is described, in whom the aortic valve was also involved. She was treated with corticosteroids. The effect of the aort&c valve involvement progressed and necessitated replacement by Starr-Edwards prosthesis. Pathological features of the operation specimen are detailed. Endocarditis is well recognized in systemic lupus erythematosus since the original description by Libman and Sacks in I924. In the series reported by Brigden et al. (i960) it was present in half the cases coming to necropsy, the mitral valve being more frequently affected than the aortic valve. It was clinically apparent in only 20 per cent of all their cases, as it was in the series of Dubois and Tuffan- elli (i964). But severe valvar dysfunction is exceed- ingly rare. Shulman and Christian (i969) first re- ported a case of Starr valve replacement for severe aortic regurgitation due to systemic lupus erythema- tosus endocarditis. We describe here a further case. 1 Present address: Kidderminster General Hospital, Kidder- minster, Worcestershire. Case report A 38-year-old business woman first presented in I965 with joint pains in her hands and wrists of 4 years' dura- tion. She was normotensive. Her ESR was 30 mm/hr (Westergren) and tests for rheumatoid factor were nega- tive. In I968 a soft early diastolic murmur was first heard; electrocardiogram and chest x-ray were normal. There was no history of rheumatic fever and the Wassermann reaction was negative. In I969 she had an episode of left- sided pleurisy. In February I97I she was admitted to Westminster Hospital because of a low grade fever, night sweats, and dyspnoea. Her blood pressure was 240/ 30 mmHg; the heart murmur was louder. Chest x-ray showed some cardiac enlargement and there was electro- cardiographic evidence of pericarditis. The ESR was 83 mm/hr (Westergren), blood cultures were negative, FIG. I Surgical specimen of aortic valve. Note the vegetations arranged in bead-like chains over the closure margin and the free edge of the valve leaflets. (Reconstructed after tissue for histological investigation had been selected.) on October 29, 2020 by guest. Protected by copyright. http://heart.bmj.com/ Br Heart J: first published as 10.1136/hrt.36.4.413 on 1 April 1974. Downloaded from
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British Heart Journal, I974, 36, 4I3-4i6.
Aortic regurgitation in systemic lupuserythematosus requiring aortic valve replacement
W. M. C. Oh, R. T. Taylor,' and E. G. J. OlsenFrom the National Heart Hospital and Westminster Hospital, London
A woman with proven systemic lupus erythematosus is described, in whom the aortic valve was alsoinvolved. She was treated with corticosteroids. The effect of the aort&c valve involvement progressed andnecessitated replacement by Starr-Edwards prosthesis. Pathological features of the operation specimen are
detailed.
Endocarditis is well recognized in systemic lupuserythematosus since the original description byLibman and Sacks in I924. In the series reported byBrigden et al. (i960) it was present in half the casescoming to necropsy, the mitral valve being morefrequently affected than the aortic valve. It wasclinically apparent in only 20 per cent of all theircases, as it was in the series of Dubois and Tuffan-elli (i964). But severe valvar dysfunction is exceed-ingly rare. Shulman and Christian (i969) first re-ported a case of Starr valve replacement for severeaortic regurgitation due to systemic lupus erythema-tosus endocarditis. We describe here a further case.1 Present address: Kidderminster General Hospital, Kidder-minster, Worcestershire.
Case reportA 38-year-old business woman first presented in I965with joint pains in her hands and wrists of 4 years' dura-tion. She was normotensive. Her ESR was 30 mm/hr(Westergren) and tests for rheumatoid factor were nega-tive. In I968 a soft early diastolic murmur was first heard;electrocardiogram and chest x-ray were normal. Therewas no history of rheumatic fever and the Wassermannreaction was negative. In I969 she had an episode of left-sided pleurisy. In February I97I she was admitted toWestminster Hospital because of a low grade fever, nightsweats, and dyspnoea. Her blood pressure was 240/ 30mmHg; the heart murmur was louder. Chest x-rayshowed some cardiac enlargement and there was electro-cardiographic evidence of pericarditis. The ESR was83 mm/hr (Westergren), blood cultures were negative,
FIG. I Surgical specimen of aortic valve. Note the vegetations arranged in bead-like chainsover the closure margin and the free edge of the valve leaflets. (Reconstructed after tissue forhistological investigation had been selected.)
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blood urea 54 mg/Ioo ml, urine protein 200 mg/24 hr,and the total haemolytic complement level was low. Thediagnosis of systemic lupus erythematosus was con-firmed by positive tests for LE cells and antinuclear
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FIG. 2 Section of a valve leaflet showing slightthickening of part of the cusp due to an increase inpredominantly elastic tissue, while the top part showsdestruction of normal architecture. Demarcation ofvalve cusp and fibrous core of the vegetation is in-distinct. (Weigert's elastic van Gieson. x 8.)
factor. Treatment was started with prednisone 40 mg/dayand bethanidine, and her general condition improved. InJune I971 she developed cerebellar ataxia and was notedto be severely cushingoid in appearance. A gradual re-duction in corticosteroid dosage was begun. Purpuraassociated with a low platelet count (44,ooo/mm3)appeared in July I97i and the cerebellar signs wereworse. Azathioprine I25 mg/day was started. Then shewas readmitted to hospital with acute left ventricularfailure. There was pronounced cardiac enlargement withsevere left ventricular hypertrophy. She improved withdiuretic therapy but remained restricted by generalweakness and some exertional dyspnoea. Six monthslater, in February I972, she was readmitted in severeleft ventricular failure. The cerebellar signs were lessobvious, platelets 84,000/mm3, urea 76 mg/ioo ml, urineprotein I-2g/24 hr. Though the antinuclear factor testwas still positive, no LE cells were found: complement(C,) was normal and DN binding not increased.
She was transferred to the National Heart Hospitalwhere physical examination revealed blood pressure190/95 mmHg, collapsing pulse, pronounced left ven-tricular hypertrophy, a loud long diastolic murmur, andan Austin Flint murmur. Electrocardiogram showedsevere left ventricular hypertrophy and strain. An aorto-gram indicated severe aortic regurgitation with slightaortic dilatation and a left ventricular angiogram showedan enlarged left ventricular cavity with normal con-tractility and no mitral regurgitation. There was nogradient across the mitral or aortic valve. The leftventricular end-diastolic pressure was 20 mmHg and themean indirect left atrial pressure was 22 mmHg.
In March I972 her diseased aortic valve was replacedby a Starr-Edwards prosthesis. Dense pericardial adhe-sions were noted at operation. Her postoperative coursewas uneventful. Her heart diminished in size and theradiographic signs of pulmonary venous congestion dis-appeared. There has been no demonstrable deteriora-tion in any of the other clinical or laboratory features ofher disease since the operation.
PathologyMacroscopical appearancesThe aortic valve had been opened and measured go mmin length (upper limit of normal 80 mm). The valveleaflets were slightly thickened, particularly along thefree margin and the line of closure. On the ventricularsurface (deformed face) in line with the corpora arantii Fand along the line of closure, there were a few small dis-crete vegetations about 3 mm in diameter. More con-spicuous, however, were confluent vegetations, reddish-yellow in colour, arranged in bead-like chains (Fig. i).
Histological appearancesThe valve cusps were slightly thickened throughout theentire length. The thickening was predominantly due toan increase in the elastic tissue, but the other major com-ponent (collagen tissue) of normal valve structure couldbe recognized. Along the line of closure and the freemargin there was a moderately severe increase in col-lagen tissue, with destruction of normal architecture.
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Aortic regurgitation in systemic lupus erythematosus requiring aortic valve replacement 415
FIG. 3 Photomicrograph of the vegetation showing a fibrous core with superimposition ofrecent fibrin. The cellular infiltrate is sparse. (Haematoxylin and eosin. x 85.)
The vegetations consisted of a core of cellular fibroustissue but demarcations between valve component andthose of the vegetation were impossible to define (Fig. 2).Superimposed on this fibrous core was recent fibrin(Fig. 3).
Using Lendrum et al. (I962) stain, recent 'fibrinoid'could be seen in a few small areas within the fibrous core.Cellular elements were scanty and consisted of somemononuclear cells. A very occasional Anitschkow cellwas also found. Haematoxyphil bodies and LE cellswere not found. Vascularity of the valve cusps was notincreased.
CommentIn this patient it was felt that her prognosis as aresult of severe aortic valve disease was extremelypoor. Because of the improvement in cerebellarsigns and platelet count, the normal DNA bindingand serum complement, and the absence of LEcells, the systemic lupus erythematosus was thoughtto be under control.
Recent studies indicate a io-year survival ratein systemic lupus erythematosus of nearly 6oper cent (Estes and Christian, 1971; Siegel et al.,I969). The clinical features consistently associatedwith a poor prognosis are severe renal and neuro-logical disease. In our patient renal biopsy was un-successful but judging from the clinical features itwas unlikely that diffuse proliferative or mem-branous glomerulonephritis was present. Severerenal involvement in systemic lupus erythematosusis associated particularly with a poor prognosis(Baldwin et al., I970; Estes and Christian, I97I;Pollak and Pirani, I969). Cerebellar ataxia is recog-nized as an uncommon feature of the disease(Johnson and Richardson, I968). As this wasimproving it was felt that operation should be
undertaken, though its effect on our patient's ulti-mate prognosis is unknown.The pathology was detailed, among others, by
Gross in I940, and Klemperer, Pollack, andBaehr in I94I. Both sides of the heart were equallyaffected in the series published by Baggenstoss(I952). This author found 'non-bacterial verrucousendocarditis' in 40 per cent of cases. Brigden et al.in I960 reported predominantly left-sided heartinvolvement. The aortic valve is usually spared butthese authors found aortic valve involvement in 4of their cases.Most of the pathological descriptions were de-
tailed in the published reports before the advent ofsteroid therapy. Though the patient described heredid not show all the classical features (haema-toxyphil bodies and LE cells), the appearances arethose of 'non-bacterial verrucous endocarditis'. Theabsence of these features can be explained as beingdue to the steroid therapy this patient had pre-viously received.
We wish to thank Dr. F. Dudley Hart, Dr. AubreyLeatham, and Mr. Donald Ross for their permission toreport this case, and Dr. E. J. Holborow in whose lab-oratory at Taplow the measurements of DNA bindingwere made.
ReferencesBaggenstoss, A. H. (I952). Visceral lesions in disseminated
lupus erythematosus. Proceedings of the Staff Meetings ofthe Mayo Clinic, 27, 4I2.
Baldwin, D. S., Lowenstein, J., Rothfield, N. F., Galo, G.,and McCluskey, R. T. (I970). The clinical course of theproliferative and membranous forms of lupus nephritis.Annals of Internal Medicine, 73, 929.
Brigden, W., Bywaters, E. G. L., Lessof, M. H., and Ross,I. P. (I960). The heart in systemic lupus erythematosus.British Heart Journal, 22, I.
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Dubois, E. L., and Tuffanelli, D. L. (I964). Clinical mani-festations of systemic lupus erythematosus; computeranalysis of 520 cases. Journal of the American MedicalAssociation, I90, I04.
Estes, D., and Christian, C. L. (I97I). Natural history ofsystemic lupus erythematosus by prospective analysis.Medicine, 50, 85.
Gross, L. (I940). The cardiac lesions in Libman-Sacksdisease. American Journal of Pathology, x6, 375.
Johnson, R. T., and Richardson, E. P. (I968). Neurologicalmanifestations of systemic lupus erythematosus - Clinicalpathological study of 24 cases and review of the literature.Medicine, 47, 337.
Klemperer, P., Pollack, A. D., and Baehr, G. (1941). Pathologyof disseminated lupus erythematosus. Archives of Patho-logy, 32, 569.
Lendrum, A. C., Fraser, D. S., Slidders, W., and Henderson,R. (I962). Studies on the character and staining of fibrin.Journal of Clinical Pathology, 15, 401.
Libman, E., and Sacks, B. (I924). Hitherto undescribed formof valvular and mural endocarditis. Archives of InternalMedicine, 33, 701.
Pollak, V. E., and Pirani, C. L. (I969). Renal histologicfindings in systemic lupus erythematosus. Mayo ClinicProceedings 44, 630.
Shulman, H. J., and Christian, C. L. (I969). Aortic insuffi-ciency in systemic lupus erythematosus. Arthritis andRheumatism, 12, I38.
Siegel, M., Gwon, N., Lee, S. L., Rivero, I., and Wong, W.(I969). Survivorship in systemic lupus erythematosus:relationship to race and pregnancy. Arthritis and Rheu-matism, I2, I17.
Requests for reprints to Dr. E. G. J. Olsen, Departmentof Histopathology, National Heart Hospital, Westmore-land Street, London WiM 8BA.
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