J. clin. Path., 1970, 23, 623-626

Rheumatoid heart disease with complete heartblock

M. HARRIS1From the Department ofPathology, University of Bristol

SYNOPSIS A case of florid rheumatoid heart disease with complete heart block is described,and the histopathology of the atrioventricular conducting tissue is described in detail.

Although the association of heart block withrheumatoid arthritis has been noted by a numberofauthors (Cathcart and Spodick, 1962; Lebowitz,1963), examples where a detailed pathologicalstudy of the heart has been recorded are extremelyrare and it is for this reason that the followingpatient is described.

pacemaker was inserted and the heart was pacedat 70 beats per minute, but, although there wasslight improvement she remained confused andin heart failure, and died on 14 September.On the day of her death the plasma electrolytes

were: urea 104 mg%, sodium 140 m-equiv/1,potassium more than 8 m-equiv/1, chloride 104m-equiv/1, and bicarbonate 12 m-equiv/1. Thecause of the marked hyperkalaemia is unknown.

Case Report

The patient was a woman aged 69 years who hadsuffered from severe rheumatoid arthritis forseven years. The joints affected were the shoulders,wrists, fingers, neck, and knees. In June 1967she was admitted to hospital for treatment of aurinary infection; her blood pressure was 160/80mm Hg, the pulse rate was 76 beats per minute,and no abnormality of the heart was found onclinical examination.

In August 1967 a course of treatment withACTH, 40 units per day, was started but wasstopped after five days because the patientdeveloped tachycardia and oedema of the legs.On 11 September 1967 she was admitted to

hospital in congestive heart failure. A loudsystolic murmur was heard in the aortic area.The pulse rate was 40 beats per minute and com-plete heart block was diagnosed. On 13 Septem-ber she had repeated episodes of cardiac stand-still and unconsciousness; an intravenous

Received for publication 24 October 1969.'Present address: Department of Pathology, University of theWest Indies, Mona, Kingston 7, Jamaica.

Necropsy Findings

There was moderate oedema of both ankles.The joints of both hands showed typical

rheumatoid deformities, but other joints appearednormal externally.The heart weighed 345 g and there was dilata-

tion of all its chambers, notably of the leftventricle which was slightly hypertrophied. Thetip of the cardiac pacemaker was in perfectposition in the right ventricle. The aortic valvebore a rounded thrombotic vegetation (0-6 cmdiameter) on the superior aspect of the leftcoronary cusp, the cusp itself being slightlythickened. There was no significant growthfrom a culture of a fragment of the vegetation.Only the basal third of the right coronary cuspwas present and was strikingly thickened; theremainder of the cusp had been destroyed. Thenon-coronary cusp appeared normal. Apart fromthe presence of a small white nodule (0'3 cmdiameter) in the base of the anterior cusp, themitral valve was macroscopically normal. Thepulmonary and tricuspid valves were normal.

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M. Harris

Fig. 1 Left coronary cusp of aortic valve. Stripsof necrosis, appearing dark in the picture, areapparent in the cusp beneath the thromboticvegetation. Haematoxylin and eosin x 17.

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Fig. 2 Higher-power photomicrograph of the leftcoronary cusp. The typical structure of a rheumatoidgranuloma is now apparent wtih a central necroticzone bordered by a palisade offibroblasts andhistiocytes. Haematoxylin and eosin x 105.

The coronary arteries were almost free fromatheroma and no occlusions were found. Themyocardium appeared normal. There was mildaortic atheroma but no evidence of aortitis.No other abnormality was found.

Histology

METACARPO-PHALANGEAL JOINTThere is complete destruction of the articularcartilage which is replaced by vascular fibroustissue infiltrated by chronic inflammatory cells.The synovium shows papillary hyperplasia andthere is osteoporosis of the adjacent bone.

AORTIC VALVEThe vegetation on the left coronary cusp (Fig. 1)has the structure of recently formed thrombus;there is no organization of its base and no bacteriaare seen in a Gram-stained section. Within thecusp, beneath the vegetation, there is a strip offibrinoid necrosis bordered by fibroblasts andhistiocytes arranged in palisade fashion, appear-ances typical of a rheumatoid granuloma (Figs.1 and 2). Around the granuloma there is fibrosisand infiltration by numerous lymphocytes andplasma cells. Similar changes, including granu-lomata, are present in the valve ring and althoughchronic inflammatory cell infiltration extends intothe adventitia at the root of the aorta there isno inflammation or fibrosis of the aortic media.The remnant of the right coronary cusp shows

severe fibrosis and contains several rheumatoidgranulomata.

MITRAL VALVEThe nodule in the base of the anterior cusp isdue to the presence of rheumatoid granulomatawith accompanying fibrosis and chronic inflam-matory cell infiltration.Rheumatoid granulomata are also found in

the upper part of the muscular interventricularseptum and in the interatrial septum.

It is of interest at this stage to note an addi-tional feature which is present in several of theotherwise typical rheumatoid nodules. This isthe presence of polymorph infiltration, sometimesmarked, at the periphery of the necrotic zones ofthe nodules. Often the polymorphs show con-siderable pyknosis and karyorrhexis and wherethis occurs the necrotic material acquires avariable degree of basophilia. Within suchbasophilic zones it is still possible to detectfibrinoid material and there is no doubt thatthe basophilic zones constitute part of therheumatoid nodules since they can be seen tobe continuous with the typical, brightly eosino-philic, necrotic areas elsewhere in the granulomata.

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Rheumatoid heart disease with complete heart block

The atrioventricular conducting tissue wasexamined by the technique of Hudson (1963).In all sections from proximal to distal ends of thesystem the conducting tissue is abnormal. Themildest changes, seen in the most proximal partsof the system, are diffuse infiltration by lympho-cytes, plasma cells, and histiocytes, with slightinterstitial fibrosis and some vacuolation of themuscle fibres. The inflammatory cell infiltrationin this and all other regions involves adjacentadipose tissue, fibrous tissue, and atrial muscleand is continuous with the inflammatory changesat the base of the aortic valve.

In addition to the above features, which arenot specific, there are zones of necrosis in allregions of the system. In any one section up tohalf of the conducting tissue may be necrotic.For the most part the necrotic zones are baso-philic and infiltrated by many polymorphs but ina few small areas the basophilia is seen to mergewith zones of fibrinoid necrosis bordered byhistiocytes, fibroblasts, and a few multinucleatedgiant cells forming an indistinct palisade pat-tern (Fig. 3). It is considered, because of thesefeatures, and by analogy with the essentiallysimilar findings in the valvular lesions, that thenecrotic lesions in the conducting tissue arerheumatoid granulomata.

There is no arteritis of the vessels within theconducting tissue nor in any other tissues andneither is there any vascular thrombosis.No other histological abnormality is found.

Discussion

The histological lesions of rheumatoid heartdisease may be specific or non-specific. The speci-fic lesions are granulomata identical in appear-ance to subcutaneous rheumatoid nodules(Cruickshank, 1958) and may occur in the peri-cardium, myocardium, or endocardium. Thevalvular lesions in the case reported here areundoubtedly of the specific granulomatousvariety and, together with the long history ofrheumatoid arthritis, firmly establish the diag-nosis of rheumatoid disease of the heart withunusually severe damage to the aortic valve.For reasons given in the histological report the

necrotic lesions in the atrioventricular conductingtissue are also considered to be rheumatoidgranulomata.

Clinical studies show that rheumatoid diseaseof the heart may present in a variety of ways,including heart block. Cathcart and Spodick(1962) studied 254 patients with rheumatoidarthritis and found 88 with evidence of organicheart disease. Of these, 29 were classified as'indeterminate heart disease' on the basis ofunusual cardiac findings and were probablyexamples of rheumatoid disease of the heart;nine of them had some form of heart block.

Fig. 3 Necrotic zone in atrioventricular bundlehaving the structure of a rheumatoid granuloma.Haematoxylin and eosin x 105.

Thus, although heart block is probably a fairlyfrequent manifestation of rheumatoid heartdisease, it is, in absolute figures. an uncommoncomplication of rheumatoid arthritis. Not sur-prisingly, there are very few cases on recordwhere a full histopathological study of the hearthas been made and even fewer in which theatrioventricular conducting tissue has beenexamined. Those found in the literature aresummarized in the Table.Nine cases of heart block, two of which were

complete, from a paper by Lebowitz (1963) havebeen excluded from the Table because the patho-logical details recorded are scanty.Examples of heart block associated with anky-

losing spondylitis have also been excluded fromthe Table because it is felt that it is more helpfulto regard this condition as being distinct fromrheumatoid arthritis, although related to it.Nevertheless, it is of interest that there appearsto be a definite association between ankylosingspondylitis and heart block, the latter beingfound in 8-5% of 519 patients with spondylitisby Graham and Smythe (1958). In all but fourof these patients with heart block, none of whomwere necropsied, there was also aortic incompe-

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626 M. Harris

Reference Age Sex Duration Subcutaneous Type of Pathology of Heart Pathology of Atrio-of Arthritis Nodules Heart Block ventricular System

Handforth & 68 F 3 years - Complete Rheumatoid nodule at Not recordedWoodbury upper end of inter-(1959) ventricular septum;Case 2 valves not diseasedGowans 59 M 49 years - Complete Rheumatoid nodules in Not recorded(1960) epicardium, endocardium,

right atrial appendage, andinterventricular septum;valves not diseased

Carpenter 65 F 15 years LBBB' Acute and healed Proximal portion of leftet al and pericarditis; rheumatoid bundle branch completely(1967) first degree nodules in all valves, interrupted by a

epicardium, and rheumatoid granulomamyocardium

Kirk & Cosh 69 F 7 years First Pericardial adhesions; Rheumatoid granulomata(1969) degree rheumatoid nodules in in atrioventricular node andCase 10 epicardium, b_tween roots bundle of His

of both great vessels;fibrinoid change in someintramural arteries

This case 69 F 7 years ? Complete Rheumatoid granulomata Rheumatoidof aortic and mitral granulomata in all partsvalves; aortic vegetation; of atrioventricular systemdestruction of one aorticcusp

Table Summary of reported cases'LBBB = left bundle branch block.

tence suggesting that there is usually an accom-panying aortitis. This correlates with the casesof spondylitis recorded by Weed, Kulander,Mazzarella, and Decker (1966) and by Sobinand Hagstrom (1962) in which there was non-specific chronic inflammatory destruction of theatrioventricular conducting tissue in continuitywith aortic inflammation. It seems possible thatthe proximity of the aortic valve to the atrio-ventricular conducting tissue, combined with thesusceptibility of the aortic valve to inflammationin ankylosing spondylitis (Clark, Kulka, andBauer, 1957), determines the apparent risk ofheart block in this disease, rather than there beinga specific susceptibility of the conducting tissueto the disease process. A similar argument cannot,however, be applied to heart block in rheuma-toid arthritis because, although in the caserecorded here there was aortic valve damage incontinuity with the lesions of the conductingtissue, there was no aortic valve disease in threeof the other four cases summarized in the Table.

I should like to thank Dr D. W. Barritt forpermission to publish the clinical details andDr J. A. Cosh for giving me details of case 10from his publication.Thanks are also due to Mr C. Jeal for the

photomicrographs and to Mrs A. Daniel andMrs V. Horler for the typing.

References

Carpenter, D. F., Golden, A., and Roberts, W. C. (1967). Quad-rivalvular rheumatoid heart disease associated with leftbundle branch block. Amer. J. Med., 43, 922-929.

Cathcart, E. S., and Spodick, D. H. (1962). Rheumatoid heartdisease; a study of the incidence and nature of cardiaclesions in rheumatoid arthritis. New Engl. J. Med., 266,959-964.

Clark, W. S., Kulka, J. P., and Bauer, W. (1957). Rheumatoidaortitis with aortic regurgitation: an unusual manifesta-tion of rheumatoid arthritis (including spondylitis). Amer.J. Med., 22, 580-592.

Cruickshank, B. (1958). Heart lesions in rheumatoid disease. J.Path. Bact., 76, 223-240.

Gowans, J. D. C. (1960). Complete heart block with Stokes-Adams syndrome due to rheumatoid heart disease: reportof a case with autopsy findings. New EngI. J. Med., 262,1012-1014.

Graham, D. C., and Smythe, H. A. (1958). The carditis andaortitis of ankylosing spondylitis. Bull. rheum. Dis., 9,171-174.

Handforth, C. P., and Woodbury, J. F. L. (1959). Cardiovascularmanifestations of rheumatoid arthritis. Canad. nmed. Ass.J., 80, 86-90.

Hudson, R. E. B. (1963). The human conducting system and itsexamination. J. clin. Path., 16, 492-498.

Kirk, J., and Cosh, J. (1969). The pericarditis of rheumatoidarthritis. Quart. J. Med., 38, 397-423.

Lebowitz, W. B. (1963). The heart in rheumatoid arthritis(rheumatoid disease). Ann. intern. Med., 58, 102-123.

Sobin, L. H., and Hagstrom, J W. C. (1962). Lesions of cardiacconduction tissue in rheumatoid aortitis. J. Amer. med.Ass., 180, 1-5.

Weed, C. L., Kulander, B. G., Mazzarella, J. A., and Decker, J. L.(1966). Heart block in ankylosing spondylitis. Arch.intern. Med., 117, 800-806.

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