British HeartJournal, I974, 36, 220-223.

Heart block resulting from myocardial sarcoidosis

F. J. Fawcett and M. J. GoldbergFrom the Department of Pathology, Peterborough District Hospital, Peterborough, and Leicester RegionalCardio-thoracic Unit, Groby Road Hospital, Leicester

The clinical and pathologicalfindings in a 6o-year-old man with complete heart block are described. He diedas a result of pacemaker failure, and necropsy showed massive myocardial involvement by sarcoidosis whichhad destroyed the atrioventricular node and upper part of the bundle of His. There was no clinical evidence ofsarcoidosis.

Myocardial sarcoidosis is a relatively uncommon

cause of heart block. In their review of the pub-lished reports in I968, Bashour et al. could onlycollect 45 cases of proven sarcoidosis in whomdeath was due to this pathology. Of these cases, 35had electrocardiographic studies before death, andcomplete heart block was shown in 14 of them. Onthe basis of necropsy findings, the incidence ofmyocardial involvement in cases of sarcoidosisranges from I3 to 20 per cent (Longcope andFreiman, I952; Mayock et al., I963; Branson andPark, I954). However, H. A. Fleming (I972, personalcommunication) from his own personal experienceand having collected the data of cases from severalcentres in the British Isles, and having written toa large number of cardiac and thoracic centres, hasshown that the condition is probably more frequentthan generally thought.

This report describes a case with extensive myo-

cardial sarcoidosis but little involvement of otherorgans.

Case report

A 6o-year-old European farmer, who had previouslybeen healthy, was diagnosed as having complete heartblock in June I97I after the onset of recurrent bouts offaintness and syncope, and of severe breathlessness on

effort. He was initially treated with long-acting oralisoprenaline tablets (Saventrine) which abolished thebouts of syncope. However, he developed urinary re-

tention from an enlarged prostate gland, and as it was

considered that a prostatectomy was necessary, he was

referred for the insertion of a pacemaker in September197I.He was found to be in complete heart block with a

heart rate of 40 per minute. There were no signs ofcardiac failure, the heart was not clinically enlarged, andthere were no cardiac murmurs. Blood pressure was

140/70 mmHg. The liver, spleen, and lymph nodes were

not enlarged, and there were no rashes. Except fordiffuse enlargement of the prostate, the rest of theexamination was normal.The electrocardiogram showed complete heart block

with a ventricular rate of 40 a minute, and a QRS patternof left anterior hemiblock and right bundle-branch block.The chest x-ray showed slight cardiac enlargement,but the lung fields were normal and mediastinal lymphnode enlargement could not be seen. The full bloodcount, erythrocyte sedimentation rate, blood electrolytes,and blood urea were normal. A trace of albumin waspresent in the urine, but urine microscopy and cultureshowed no abnormality.A pacemaker was inserted, pacing being carried out by

the endocardial route with a St. George's catheter elec-trode, and a fixed rate Devices Thin Film pacemaker(type 382I). In October I97I a prostatectomy wasuneventfully carried out.

After his prostatectomy he was asymptomatic, andpacing tests on the 28 October 1971 showed satisfactorypacing and pacemaker function. However, on the 22December 197I, he died suddenly while sitting at homeafter a day's shooting. The pacemaker was removed atnecropsy and was found to have failed.

NecropsyThe body was that of a well-nourished elderly man andthere were no significant external findings. The pace-maker coil was implanted in the anterior abdominalwall; the catheter ran up to the right jugular veinand down into the apex of the right ventricle, andwas intact throughout its length. The lungs showedsmall old calcified tuberculous lesions at the apex ofboth lungs but no other abnormalities. The lymphnodes around both main bronchi were enlarged (largest2 X 2 X IP5 cm) and had a fleshy texture. The heart,490 g, showed numerous white fleshy nodules involvingthe walls of all the cavities, but the left ventricular walland interventricular septum were most severely in-volved, with nodules up to 3-5 X 3 X I-5 cm (Fig. I).The upper part of the interventricular septum was infil-

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Heart block resulting from myocardial sarcoidosis 22I

FIG. i Left ventricle showing extensive infiltrationof wall and interventricular septum by sarcoidosis.

trated throughout the majority of its breadth and depth,as was the adjacent interatrial septum including theatrioventricular node and the upper part of the bundle ofHis.The papillary muscles were also involved, more in the

right than in the left ventricle. Many of the nodules ex-

tended from the pericardium to the endocardium andthere were also plaques primarily involving the endo-cardium and adjacent myocardium. There was an area

of dense fibrosis, 3 mm in diameter, of the papillarymuscles at the apex of the right ventricle aroundthe tip of the catheter. The mitral valve showedthickening of the cusps but no adhesions of the com-

missures; the other heart valves were normal. The cor-

onary arteries showed minimal atheroma with only a

minor degree of stenosis. The only other abnormal find-ings were similar fleshy masses in the kidneys mainly inthe subcapsular area (maximal diameter I-5 cm) whichwere otherwise normal (total weight 300 g). The eyes andparotid glands were not examined in detail at the time ofnecropsy but their external appearance was normal.

HistologyThe fleshy nodules in the heart, mediastinal lymphnodes,and kidneys showed non-caseating granulomatous lesionswith many epithelioid cells and multinucleate giant cellssurrounded by a cuff of lymphocytes (Fig. 2). There

were asteroids present in some of the giant cells in allaffected organs but no Schaumann bodies were seen.No organisms could be demonstrated in the lesions,neither was any organism grown on culture of the freshmyocardial lesions. In the heart, many of the lesionswere surrounding small arteries but the wall was not in-filtrated and the lumen was not stenosed. There was avariable amount of hyaline fibrous tissue around thelesions. There were granulomatous lesions at the base ofthe mitral and aortic valve cusps but only fibrousthickening of the cusps with a few lymphocytes in themitral valve cusps and no cells in the aortic cusps. Thesuperior vena cava was encased by the granulomatousreaction for approximately i cm above the right atrium,but the other great vessels were not involved. Sectionsfrom the other organs which appeared normal macro-scopically were free from granulomas.

DiscussionMyocardial sarcoidosis is a relatively rare condition,the first case being recorded in I929 (Bernstein,Konzlemann, and Sidlick, I929). The condition hasrecently been reviewed by Ghosh et al. (1972) andGozo et al. (I971).

It may present clinically in the following ways.a) Disturbances of rhythm are the most common

presenting features, and complete heart blockis the most frequent and important dysrhythmia.This is explained by the frequent involvementof the ventricular septum. Of the 29 cases ofmyocardial sarcoidosis reviewed by Peacock,Lippshutz, and Lukas (1957), I5 cases sufferedAdams-Stokes attacks orhad completeheart block.Ventricular tachycardia, atrial fibrillation, andatrial tachycardia are less frequent manifestations.

b) Sudden death is common in myocardial sarcoi-dosis and is frequently the first manifestation ofthe condition. However, it is possibly a mani-festation of severe disturbances of the conductivesystem.

c) Congestive cardiac failure, which may be a mani-festation of severe rhythm disturbances or ofimpairment of cardiac contractility, caused byventricular infiltration.

d) Miscellaneous: valve involvement (Raftery,Oakley, and Goodwin, I966; Simkins, I95i;Laroche et al., I955) and recurrent pericardialeffusions (Shiff, Blatt, and Colp, 1969) have beenreported but are rare.The present case presented with complete heart

block without any other manifestations of sarcoid-osis to suggest an underlying myocardial pathology.There was no radiological evidence of hilar lymphnode enlargement. It is of interest that he respondedwell initially to oral isoprenaline, and that he waswell controlled by his pacemaker. There is oneprevious report of control of complete heart block

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222 Fawcett and Goldberg

FIG. 2 Sarcoid granuloma in myocardium with epithelioid cells, multinucleate giant cells, andlymphocytes. (x 350.)

with a pacemaker (Johansen, I966). The patient wasdiagnosed clinically as having sarcoidosis because oftypical chest x-ray appearances 8 years before the de-velopment of complete heart block. The heart blockwas successfully treated with an internal pacemakerfor 3I months, but he suddenly died from pacemakerfailure. Sarcoid granulomas were demonstrated inthe ventricular septum.

In one of Phinney's cases (I96I) pacemaking wasprobably of the external, transthoracic type, and socannot be quoted as a failure of pacing as has beendone in previous reviews.

In most reported cases, the left ventricle andinterventricular septum were predominantly in-volved, but the present case shows an unusual degreeof left atrial involvement. The lesions are most fre-quently seen around small blood vessels in the myo-cardium. Frequently the granulomas are atypicalin the myocardium, presenting as a nonspecificgranuloma or a patchy hyaline fibrosis with littlespecific epithelioid and giant cell granuloma forma-tion, though other organs contain better definedsarcoid tubercules (Scadding, i967). In the presentcase the granulomas were florid and of a typicalsarcoid appearance.

ReferencesBashour, F. A., McConnell, T., Skinner, W., and Hanson, M.

(1968). Myocardial sarcoidosis. Diseases of the Chest, 53,413.

Bernstein, M., Konzlemann, F. W., and Sidlick, D. M. (I929)Boeck's sarcoid; report of a case with visceral involvement.Archives of Internal Medicine, 44, 721.

Branson, J. H., and Park, J. H. (I954). Sarcoidosis - hepaticinvolvement: presentation of a case with fatal liver in-volvement, including autopsy findings and review of theevidence for sarcoid involvement of the liver as found inthe literature. Annals of Internal Medicine, 40, III.

Ghosh, P., Fleming, H. A., Gresham, G. A., and Stovin,P. G. I. (1972). Myocardial sarcoidosis. British HeartJournal, 34, 769.

Gozo, E. G., Cosnow, I., Cohen, H. C., and Okun, L. (I97I).The heart in sarcoidosis. Chest, 60, 379.

Johansen, A. (I966). Isolated myocarditis versus myocardialsarcoidosis. Acta Pathologica et Microbiologica Scandi-natvca, 67, 15.

Laroche, C., Gennes, J. L. De, Hazard, J., and Samarcq, P.(i955). Maladie de Besnier - Boeck - Schaumann avecmanifestations polyarticulaires et localizations endo-myo-p6ricardiques mortelles. Bulletins et Memoires de la SocidtiMedicak des hOpitaux de Paris, 71, 908.

Longcope, W. T., and Freiman, D. G. (I952). A study ofsarcoidosis based on a combined investigation of i6o casesincluding 30 autopsies from the Johns Hopkins Hospitaland Massachusetts General Hospital. Medicine, 31, I.

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Mayock, R. L., Bertrand, P., Morrison, C. E., and Scott,J. H. (I963). Manifestations of sarcoidosis. Analysis of I45patients with a review of 9 series selected from the liter-ature. American Journal of Medicine, 35, 67.

Peacock, R. A., Lippshutz, E. J., and Lukas, A. (I957).Myocardial sarcoidosis. Circulation, I6, 67.

Phinney, A. 0. (I96I). Sarcoid of the myocardial septum withcomplete heart-block - report of 2 cases. American HeartJ'ournal, 62, 270.

Raftery, E. B., Oakley, C. M., and Goodwin, J. F. (I966).Acute subvalvar mitral incompetence. Lancet, 2, 360.

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Shiff, A. D., Blatt, C. J., and Colp, C. (I969). Recurrent peri-cardial effusion secondary to sarcoidosis ofthe pericardium.New Englandjournal of Medicine, 28I, 14I.

Simkins, S. (I95i). Boeck's sarcoid with complete heart blockmimicking carotid sinus syncope. Journal of the AmericanMedical Association, 146, 794.

Requests for reprints to Dr. F. J. Fawcett, PathologyDepartment, Peterborough District Hospital, MidlandRoad, Peterborough PE3 6DA.

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