Annals of the Rheumatic Diseases, 1985, 44, 425-428

Case report

Complete heart block and bowel infarctionsecondary to rheumatoid diseaseMARGARET WILSHER,1 W M IAN SMEETON,2 TIMOTHY D KOELMEYER,2AND ANTONY H G ROCHE'From the 'Department of Cardiology, Green Lane Hospital, and the 2Department of Pathology, School ofMedicine, Auckland, New Zealand

SUMMARY A case of complete heart block secondary to rheumatoid involvement of theconduction system is presented. The same patient died after infarction of the colon as aconsequence of arterial spasm superimposed upon pre-existing rheumatoid changes in themesenteric arteries. The coexistence of rheumatoid involvement of the heart and of the bowelvasculature has not previously been reported.

Key words: arthritis, arrhythmia, mesenteric artery, rheumatoid nodule, vasculitis.

Cardiac arrhythmias complicating rheumatoidarthritis are uncommon, but well reported. 1-12However, in only a few instances have detailedstudies of the conduction tissues been performed.611 Ischaemic changes in the alimentary tractsecondary to involvement of mesenteric arteries byrheumatoid disease are rare. We present a case of apatient with complete heart block due to rheuma-toid disease, who died after infarction of the colonas a consequence of arterial spasm superimposedupon pre-existing rheumatoid changes in the mesen-teric arteries.

Case report

The patient, a 59-year-old Caucasian male, had a15-year history of seropositive erosive arthritis,involving predominantly hands, feet, elbows, andshoulders. Although deformity was minimal, painand stiffness, particularly in the upper limb joints,restricted him to a sedentary lifestyle. Extra-articular manifestations included rheumatoidnodules, and during the year before admission tothis hospital he had developed hepatosplenomegaly,

Accepted for publication 8 January 1985.Correspondence to Dr M Wilsher, Chest Medical Unit, GreenLane Hospital, Auckland 3, New Zcaland.

lymphadenopathy, cutaneous vasculitis,. and aperipheral sensorimotor neuropathy. He had beentreated with a variety of non-steroidal anti-inflammatory drugs but had never received gold,penicillamine, or steroids.Four months before admission he developed rapid

atrial fibrillation and heart failure, and subsequentlyatrioventricular block appeared. He was admittedelectively for pacemaker insertion.On examination the patient was pale and cach-

exic. Pertinent cardiac findings included a bradycar-dia of 40 beats/min, supine blood pressure of 120/80mmHg, raised jugular venous pressure, mild car-diomegaly, and a soft ejection systolic murmur atthe left sternal edge. There was no peripheraloedema, and the lung bases were clear.The electrocardiogram (ECG) showed atrial fib-

rillation with complete atrioventricular block and anidioventricular rhythm of 40 beats/min. Radio-graphy of the chest disclosed mild cardiomegaly.The erythrocyte sedimentation rate (ESR) wasraised to 116 mm/h, and there was an anaemia(haemoglobin 9-8 g/dl) in the presence of normaliron stores. There was no abnormality of theleucocyte or platelet count. Renal function wasnormal, but there was a mild derangement of theliver enzymes. The rheumatoid factor was positiveto a titre of 1:1024 (Rose-Waaler).

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426 Wilsher, Smeeton, Koelmeyer, Roche

Before the pacemaker could be inserted the Apatient collapsed in asystolic arrest. He was resusci-tated, and a temporary transvenous pacemaker wasinserted. Blood pressure was restored, though therewas clinical and radiological evidence of severe leftventricular failure. Five hours later the patientdeveloped severe central abdominal pain and waterydiarrhoea.Laparotomy was performed based on a clinical

diagnosis of bowel ischaemia secondary to mesen-teric artery embolus. The entire small and largebowel was found to be ischaemic but not gangren-ous. There were no pulsations in the mesentericarteries. The extent of ischaemia could be explainedon the basis of a single embolus. A Fogarty catheterpassed down the main right colic and major ileocolicarteries to the abdominal aorta without meeting an _obstruction. On withdrawal of the catheter bloodpulsated from each vessel, only to slow and finallystop within a minute. Marked arterial spasm waspresumed and an infusion of nitroprusside was

Fig. 2 Photograph of the heart opened down the obtusemargin showing the necrotic nodule (arrows) within the leftatrial wall. MV=mitral valve; LCA =left circumflex artery;LV=left ventricular wall.

commenced at a rate of 33 gxg/min. Almost im-mediately there was an improvement in the colour

J ~~~~~~~ofthe bowel, with restoration of arterial pulsationsand return of peristaltic activity. The operativeprocedure was concluded, and the patient returned

--to the intensive care unit on a nitroprusside infusion,-but circulatory collapse ensued and the patient died

12 hours after surgery.The autopsy findings were as follows:

-~~- (1) Bowel. The colon was gangrenous throughout its- ~ , ~' entire length. The small bowel, though congested,

ko~~ ~ ~ ~ ~~- was not infarcted and appeared viable. The major- -, ~~~mesenteric vessels were free of thrombotic or

embolic material. Histology of mesenteric arteries- ~~~taken from both superior and inferior mesenteric

Fig. 1 A histological section ofa distal mesenteric artery territories close to the bowel wall showed markeddemonstrating the marked intimal hyperplasia. intimal proliferation but no evidence of active(Haemotoxylin and eosin, x 80). vasculitis (Fig. 1).

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Complete heart block and bowel infarction in RA 427

(2) Heart. There were loose pericardial adhesions.The heart weighed 430 g. The coronary arteriesshowed minimal atherosclerosis. The atria weremildly dilated, and the atrial walls were diffuselythickened and rigid in texture. The posterior leftatrial wall contained a necrotic nodule 1-5 cm indiameter (Fig. 2). The ventricular chambers werenormal in size, and the ventricular myocardium wasfree of scarring or necrosis. The heart valves werenormal.

Histologically, much of the atrial muscle includingthe sinus node was replaced by loose fibrous tissuecontaining a sparse focal chronic inflammatoryinfiltrate. Occasional small foci of fibrinoid necrosiswith surrounding pallisading of histiocytes werepresent. In the nodule shown in Fig. 2 there was

EgogtA...1

Fig. 3 Histological section through the membranousseptum (MS) down into muscular interventricular septum.The region of the branching bundle (BB) has beendestroyed by inflammatory tissue which extends down intothe proximal left bundle branch (LB). TV=tricuspid valve.(Haematoxylin and eosin, x 10).

tissue necrosis, with the inflammatory infiltratecontaining a prominent neutrophilic component.The atrioventricular conduction tissues were

serially sectioned at 10 [im with every 20th sectionstained. Dense chronic inflammation with centralcavitation replaced the atrioventricular node. Theinflammatory involvement followed the penetratingbundle (His bundle) as it passed through the centralfibrous body to reach the crest of the interventricu-lar septum (Fig. 3) to become the branching bundleand then further involved the proximal portion ofthe left bundle branch. Elsewhere the ventricularmyocardium contained only a few sparse occasionallymphocytic aggregates. The coronary arteries werenot inflamed, though occasional intramyocardialarteries displayed marked intimal thickening, afeature particularly prominent in the atrioventricu-lar node artery.

Discussion

Rheumatoid heart disease may take several forms.Although specific for the condition, cardiac rheuma-toid nodules are found in only 1-3% of patients withrheumatoid arthritis who come to autopsy. Thesegranulomas have been reported in the pericardium,epicardium, myocardium, endocardium, all valvecusps and rings (predominantly mitral and aortic),and chordae tendinae. Other non-specific types ofcardiac lesions, however, have been observed atnecropsy in these patients, including pericarditis,myocarditis, coronary arteritis, and patchy valvularfibrosis.'3 14 Conduction disturbances have beendescribed in association with rheumatoid percarditisand valvular heart disease.7 12There are a number of mechanisms by which the

atrioventricular conduction system can be involvedin rheumatoid arthritis. These include direct in-volvement by granulomas or by extension of thegranulomatous inflammation from the base of theaortic or mitral valve. In one case no activegranulomas were present in the conduction system,but fibrotic patches were present in the areas wherepresumably active granulomas had been present. i'There is a range of vascular lesions occurring in

rheumatoid arthritis and these may be encounteredsingly or in combination. These changes includeintimal proliferation (most often involving the dig-ital arteries), necrotising arteritis of both small andmedium sized arteries (indistinguishable frompolyarteritis nodosa), a subacute arteritis (where theinvolved vessel is infiltrated with mononuclearcells), and a leucoclastic vasculitis of the venules andcapillaries in the skin. 14 As a result of these vascularchanges a variety of clinical features have beendescribed, which include skin rashes, cutaneous

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428 Wilsher, Smeeton, Koelmeyer, Roche

ulceration, peripheral gangrene, peripheral neuro-pathy, and rarely, visceral lesions.

Involvement of small arteries can cause multipleischaemic ulcers of the intestine, resulting inhaemorrhage or perforation. 15 Involvement of largerarteries may cause segmental (or more extensive)bowel gangrene.16 17 Perisplenitis and splenic in-farcts are a well known cause of left hypochondrialpain in rheumatoid patients, and acute arteritis ofhepatic, cystic, and pancreatic and renal vessels hasbeen reported.'5A remarkable feature of our case was the intense

spasm of the mesenteric arteries seen at laparotomy,possibly precipitated by the previous arrest. Also,rather than the acute arteritis described in otherreported cases, the lesion in the mesenteric arteriesseen here was intimal proliferation. To our know-ledge this has not been described before. Patientswho develop conduction disturbances generallyhave severe rheumatoid disease, often with extra-articular manifestations or high rheumatoid factortitres. 11 Our patient probably had systemic vasculitisas shown by cutaneous vasculitis and peripheralneuropathy, and it is possible that mesentericarteritis preceded the development of intimal pro-liferation. We are not aware of any reports in theliterature of the coexistence of rheumatoid involve-ment of the heart and of the bowel vasculature.

We are grateful for the assistance of Dr A C Hayton, Physician,Taranaki Base Hospital and Dr W Tangi, Surgical Registrar,Auckland Hospital.

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14 McAllister H A. Collagen vascular disease and the cardiovascu-lar system in cardiovascular pathology. In: Silver M D, ed.Cardiovascular pathology. New York: Churchill Livingstone,1983; 2: 1005-33.

15 Lindsay M K, Tovadia H B, Whyte A S, Lee P, Webb J. Acuteabdomen in rheumatoid arthritis due to necrotizing arteritis andintestinal infarction in rheumatoid disease. Br Med J 1973; ii:592-3.

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