J. ELECTROCARDIOLOGY 12 (3), 1979, 325-329
Treatment of Heart Block Due to Sarcoid Heart Disease BY RAY LASH, M.D., JAMES COKER, M.D. AND BERT Y. S. WONG, M.D.
SUMMARY The role of glucocorticosteroid therapy for myocardial sarcoidosis is not well defined. This report shows the effect of prednisone t h e r a p y on atrioventricular (AV) conduction in a patient with myocardial sarcoidosis and AV block. On three separate occasions AV block was documented prior to prednisone therapy. On the first two occasions the patient had first and second degree AV block which by His bundle electrogram initially was shown to be in the AV node. On the third occasion the patient developed complete heart block. On each occasion t r e a t m e n t with prednisone resulted in improved AV conduction. The results indicate t h a t prednisone therapy can be beneficial in the t r e a t m e n t of AV block due to myocardial sarcoidosis. minute, blood pressure of 108/70 mm Hg, posterior a u r i c u l a r l y m p h a d e n o p a t h y , and bilateral basilar dry rales in her lung fields. On cardiac examination the point of maximal impulse was in the sixth intercostal space in the midclavicular line; the first and second heart sounds were normal, a Grade II/VI systolic ejection m u r m u r was best heard at the lower left sternal border and a prominent apical third h e a r t sound was present. Her hematological and routine serum chemical analyses were normal. Her PA chest roentgenogram showed a widened superior mediast i n u m and a cardiothoracic ratio of 13.5/26. Her electrocardiogram showed a sinus tachycardia with a prolonged P-R interval of 0.28 seconds and nonspecific ST-T segment changes. I n t e r m i t t e n t Mobitz-type I AV block occurred during 24-hour continuous ambulatory cardiac monitoring (Fig. 1A). His bundle electrograms (Fig. 2) obtained by the method of Scherlag 3 showed a prolonged atrial-His (A-H) i n t e r v a l (180 msec) w i t h n o r m a l P wave-atrial (P-A) interval (30 msec), normal His ventricular (H-V) interval (40 msec) and a normal sinus node recovery time after right atrial pacing at 120 beats per minute for two minutes (820 msec). After intravenous administration of atropine (1 mg), the A-H interval decreased slightly to 170 msec but the P-A and H-V intervals remained the same. The echocardiogram was normal. Her pulmonary function studies at this time showed a r e s t r i c t i v e p a t t e r n w i t h a n o r m a l single breath transport factor (Dco). Intradermal s k i n tests for h i s t o p l a s m o s i s , coccidi0sis, trichophyton and tuberculosis were all nonreactive. A posterior auricular lymph node biopsy showed chronic noncaseating granulomatous changes typical of sarcoidosis with no acid-fast or fungal organisms. Because of her pulmonary involvement the
Cardiac sarcoidosis was first noted in 1929 by Bernstein. 1 As early as 1959 Gold 2 rep o r t e d t h e t r e a t m e n t of m y o c a r d i a l sarcoidosis with glucocorticosteroids, but as yet t h e role of s t e r o i d s in t h e t r e a t m e n t of myocardial sarcoidosis has been difficult to ascertain because the number of patients with clinically apparent myocardial sarcoidosis is small, spontaneous remission may occur, and t r e a t m e n t with glucocorticoids has often been combined with other drugs. We report this case because it documents the beneficial effect of prednisone t h e r a p y on a t r i o v e n t r i c u l a r (AV) conduction in a patient with myocardial sarcoidosis and recurrent AV block who was treated with prednisone on three separate occasions.
CASE REPORT This twenty-eight year old black woman first presented to the University of Kansas Medical Center in October of 1975 with dizziness, weight loss, anorexia, and dyspnea on exertion. Her p e r t i n e n t p h y s i c a l findings included a r e g u l a r pulse of 104 beats per
From the Cardiovascular Department, University of Kansas Medical Center, College of Health Sciences and Hospital, Kansas City, Kansas. The costs of publication of this article were defrayed in part by the payment of page charges. This article must therefore be hereby marked "advertisement" in accordance with 18 U.S.C. w 1734 solelyto indicate this fact. Reprint requests to: Bert Wong, M.D., Cardiovascular Department, University of Kansas Medical Center, College of Health Sciences and Hospital, 39th and Rainbow Boulevard, Kansas City, Kansas 66103. 325
326
LASH ET AL
p
p i! 84
B
Fig. 1. Rhythm strips obtained before (A) and after 8 months of the first period of treatment with prednisone (B). Before treatment there is a sinus rhythm with occasional Mobitz-type I AV block. Following treatment the P-R interval returned to normal.
patient was treated with prednisone (40 mg daily) and isoniazid (300 mg daily). After eight months of treatment, the P-R interval on her resting ECG had decreased from 0.28 seconds to 0.18 seconds (Fig. 1B). During this time the patient gained weight and developed hypertension which was treated with hydrochlorothiazide. Because of her weight gain the p a t i e n t d i s c o n t i n u e d the p r e d n i s o n e and isoniazid on her own. Within a two-month period after stopping the drugs her resting ECG showed a prolonged P-R interval >~ 0.38 sec with Mobitz-type I AV block (Fig. 3A). Prednisone therapy was reinstituted over the next four months. The degree of AV block progressively decreased until the P-R interval was 0.16 second (Figures 3B, 3C, 3D). Then, because her serial pulmonary function tests had shown no improvement, the prednisone was again stopped. Subsequently the patient developed complete heart block, initially with a slow junctional rhythm followed by a slow idioventricular r h y t h m (Fig. 4A). At this time a His bundle potential could not be identified with intracardiac electrocardiographic studies and administration of intravenous atropine (1 rag) and isoproterenol did not improve AV conduction. Cardiac catheterization at this time showed normal right and left h e a r t p r e s s u r e s , m i l d - m o d e r a t e (2+) mitral regurgitation, and normal coronary arteriograms. Prior to this time prednisone had been given primarily for treatment of the patient's pulmonary involvement. Although the concomitant improvement of AV conduction with prednisone therapy was noted, we could not be sure that this was not a coincidental occurrence. Thus the patient's complete heart
block was treated with placement of a permanent t r a n s v e n o u s pacemaker. The p a t i e n t was then restarted on t r e a t m e n t with prednisone (60 mg every other day) for the third time to determine if AV conduction could be restored again. After two months of t r e a t m e n t the patient's r e s t i n g E C G showed a paced v e n t r i c u l a r rhythm with occasional sinus capture beats, and intravenous atropine (1 mg) produced a transient sinus rhythm with Mobitz-type I AV block (Fig. 4B). After six months, sinus rhythm with a prolonged P-R interval was restored (Fig. 4C). During this time, because of s y m p t o m s of d y s p n e a on exertion, she w a s s t a r t e d on t r e a t m e n t w i t h Digoxin, furosemide and isoniazid. H e r serum electrolytes and calcium obtained periodically throughout this time remained within normal limits. DISCUSSION This p a t i e n t p r e s e n t e d w i t h the typical findings of pulmonary sarcoidosis with cardiac involvement. A l t h o u g h biopsy of the myocardium was not done, the occurrence of progressive heart block and mitral regurgitation in a patient with sarcoidosis in the absence of any other specific cause is highly suggestive of sarcoid heart disease. 4,5 She developed AV block on three separate occasions. On the first two occasions the site of block occurred in the AV node. On the third occasion the site of block probably involved both the AV node and the His Purkinje system. The lack of response to intravenous atropine and isoproterenol indicated that the AV block on each occasion was due to organic disease and not to enhanced vagal tone. J. ELECTROCARDIOLOGY, VOL 12, NO. 3, 1979
HEART BLOCK DUE TO SARCOID HEART DISEASE
327
A
II
,
A.-
_
V l ~
,
I
AEmm~,qr
-
P-A = 30 MSEC
A-H = 180 MSEC
,
'
I
B
i
_
I
i
A
i
'
H-V = 40 MSEC
H
i
~
I
i
I
I
Vl
AE
A
A H
'
H 170
I
P-A = 30 MSEC
A-H = 170 MSEC
N-V - ~0 ~SEC
Fig. 2. His bundle electrogram with bipolar atrial electrogram and surface electrocardiographic leads I, II, VI during sinus rhythm before (A) and after (B) administration of intravenous atropine (1 mg).
Glucocorticosterolds have been used prev i o u s l y for the t r e a t m e n t of AV block in p a t i e n t s w i t h a n d w i t h o u t c a r d i a c sarcoidosis. ~'4'~'7 Because of the variable course of AV block, during which spontaneous remission m a y occur, and the inconsistent response to t r e a t m e n t with glucocorticosteroids, results have been difficult to evaluate, s,9,1~This patient's clinical course is unique because AV block responded to treatment with prednisone on three separate occasions. The fact that this patient's AV conduction improved concomitJ. ELECTROCARDIOLOGY, VOL 12, NO. 3, 1979
antly with prednisone therapy on three sepa r a t e occasions m a k e s t h e p o s s i b i l i t y of spontaneous remission unlikely. Although the patient did take several other medications, only p r e d n i s o n e t h e r a p y correlated with improvement of AV conduction. Thus, it seems reasonable to conclude t h a t steroid therapy improved AV conduction in this patient with myocardial sarcoidosis. The clinical manifestations of myocardial sarcoidosis are varied and multiple. They include sudden death, congestive heart failure,
328
LASH ET AL
Y
A
P
P
P
P
P
. IPFI.~0.38
y B 1:R:0,24
C
D
Fig. 3. Rhythm strips obtained before (A) and after I month (B), 3 months (C) and 4 months (D) of the second period of treatment with prednisone. Prior to therapy Mobitz-type I AV block has recurred. Following treatment there is a sinus rhythm with a gradual return of the P-R interval to normal.
r e c u r r e n t p e r i c a r d i a l effusions, p a p i l l a r y muscle dysfunction, ventricular aneurysms, tachyarrhythmias, and AV conduction disturbances, s'9,1~-19 The pathological features of cardiac sarcoidosis consist primarily of infilt r a t i o n of t h e m y o c a r d i u m w i t h sarcoid granulomas, myocardial fibrosis, ventricular aneurysm, and focal fibromuscular dysplasia of the small coronary arteries. 7,11 The exact cause of AV block in cardiac sarcoidosis is not known. Sarcoid granulomas have a predilection for the interventricular septum and potentially can physically encroach upon and disrupt the conduction system. 7,1~,~2,'3 Since prednisone therapy may decrease the size of granulomas, 7 this theoretically could result in improved AV conduction. The long period of time required for AV conduction to improve following prednisone t h e r a p y suggests t h a t the immediate hormonal and cellular effects of glucocorticosteroids were not the m e c h a n i s m w h e r e b y AV c o n d u c t i o n improved. Ischemia of the AV node due to fibromuscular dysplasia involving the AV node artery has also been previously described as a possible cause of heart block in sarcoid heart disease. 11 The coronary arteriograms in this patient were completely normal. Although this rules out large vessel coronary artery
disease, it does not exclude the possibility of small vessel involvement. This report documents the improvement of AV c o n d u c t i o n w i t h g l u c o c o r t i c o s t e r o i d therapy in a patient with cardiac sarcoidosis. The r e s u l t s s u g g e s t t h a t t r e a t m e n t w i t h glucocorticosteroids can be beneficial to patients with cardiac sarcoidosis manifested by AV block. W h e t h e r g l u c o c o r t i c o s t e r o i d therapy is beneficial in the t r e a t m e n t of other manifestations of cardiac sarcoidosis remains to be determined.
1.
2. 3.
4.
REFERENCES BERNSTEIN, M, KONZLEMANN, F W AND SIDLICK, D M: Boeck's sarcoid; report of a case with visceral involvement. Arch Intern Med 44:721, 1929 GOLD,J A AND CANTOR,P J: Sarcoid heart disease. Arch Intern Med 104:101, 1959 SCHERLAG,B J, LAU,S H, HELFANT,R H, STEIN E, BERKOWITZ,W D AND DAMATO, A N: Catheter techniques for recording His bundle activity in man. Circulation 39:13, 1969 STRAUSS,G S, LAWTON,B R, WENZEL, F J AND RAY, J F I I I : Detection of covert myocardial sarcoidosis by scalene node biopsy. Chest 69:790, 1976
J. ELECTROCARDIOLOGY, VOL 12, NO. 3, 1979
HEART BLOCK DUE TO SARCOID HEART DISEASE
329
A
B
Fig. 4. Rhythm strips after prednisone was stopped before insertion of transvenous pacemaker and during third period of treatment with prednisone after insertion of permanent transvenous pacemaker. Prior to therapy there is complete AV block with a junctional escape rhythm competing with a ventricular escape rhythm (A). After 6 weeks of prednisone therapy, transient sinus rhythm with Mobitz-type I AV block could be produced by intravenous atropine (B) though the basic resting rhythm was a paced rhythm. After 6 months of prednisone treatment (C) sinus rhythm with a prolonged P-R interval is present. The transvenous permanent pacemaker has been activated transiently with a magnet.
5. STEIN, E, STIMMEL, B AND SILTZBACH, L E: Clinical course of cardiac sarcoidosis. Ann N Y Acad Sci 278:470, 1976 6. PERRY, E L: Corticosteroids in Stokes-Adams syndrome. Am Heart J 64:571, 1962 7. ROBERTS, W C, MCALLISTER, H A AND FERRANS, V J: Sarcoidosis of the heart. A clinicopathologic study of 35 necropsy patients (Group I) and review of 78 previously described necropsy patients (Group II). Am J Med 63:86, 1977 8. FLEMING,H A: Sarcoid heart disease. Br Heart J 36:54, 1974 9. DUVERNOY,W F C AND GARCIA,R: Sarcoidosis of the h e a r t p r e s e n t i n g with v e n t r i c u l a r tachycardia and atrioventricular block. Am J Cardiol 28:348, 1971 10. FRIEDBERG, C K: Disturbances in conduction: Heart block and bundle branch block. In Diseases of the Heart, W.B. Saunders, Philadelphia, 1966, p 605 11. JAMES,T N: Sarcoid heart disease. Circulation 56:320, 1977 12. PHINNEY,A O: Sarcoid of the myocardial sep-
J. ELECTROCARDIOLOGY, VOL 12, NO. 3, 1979
13. 14. 15. 16. 17.
18. 19.
tum with complete heart block; Report of two cases. Am Heart J 62:270, 1961 FAWCETT,F J AND GOLDBERG,M J: Heart block resulting from myocardial sarcoidosis. Br Heart J 36:220, 1974 BASHOUR, F A, McCONNELL, T, SKINNER, W AND HANSON, M: Myocardial sarcoidosis. Diseases of the Chest 53:413, 1968 ZONERAICH, S, GUPTA, M P, MEHTA, A, ZONERAICH, O AND WESSELY, Z: Chest 66:452, 1974 CHUN, S K, ANDY, J J, JILLY, P AND CURRY, L: Ventricular aneurysm in sarcoidosis. Chest 68:392, 1975 AHMED, S S, ROZEFORT, R, TACLOB, L T AND BANCATO, R W: Development of ventricular aneurysm in cardiac sarcoidosis. Angiology 28:323, 1977 GHOSH, P, FLEMING, H A, GRESHAM, G A AND STOVIN, P G: Myocardial sarcoidosis. Br Heart J 34:769, 1972 MILLER,A, JACKLER, I AND CHUANG, M: Onset of sarcoidosis with left ventricular failure and multisystem involvement. Chest 70:302, 1976
Treatment of Heart Block Due to Sarcoid Heart Disease BY RAY LASH, M.D., JAMES COKER, M.D. AND BERT Y. S. WONG, M.D.
SUMMARY The role of glucocorticosteroid therapy for myocardial sarcoidosis is not well defined. This report shows the effect of prednisone t h e r a p y on atrioventricular (AV) conduction in a patient with myocardial sarcoidosis and AV block. On three separate occasions AV block was documented prior to prednisone therapy. On the first two occasions the patient had first and second degree AV block which by His bundle electrogram initially was shown to be in the AV node. On the third occasion the patient developed complete heart block. On each occasion t r e a t m e n t with prednisone resulted in improved AV conduction. The results indicate t h a t prednisone therapy can be beneficial in the t r e a t m e n t of AV block due to myocardial sarcoidosis. minute, blood pressure of 108/70 mm Hg, posterior a u r i c u l a r l y m p h a d e n o p a t h y , and bilateral basilar dry rales in her lung fields. On cardiac examination the point of maximal impulse was in the sixth intercostal space in the midclavicular line; the first and second heart sounds were normal, a Grade II/VI systolic ejection m u r m u r was best heard at the lower left sternal border and a prominent apical third h e a r t sound was present. Her hematological and routine serum chemical analyses were normal. Her PA chest roentgenogram showed a widened superior mediast i n u m and a cardiothoracic ratio of 13.5/26. Her electrocardiogram showed a sinus tachycardia with a prolonged P-R interval of 0.28 seconds and nonspecific ST-T segment changes. I n t e r m i t t e n t Mobitz-type I AV block occurred during 24-hour continuous ambulatory cardiac monitoring (Fig. 1A). His bundle electrograms (Fig. 2) obtained by the method of Scherlag 3 showed a prolonged atrial-His (A-H) i n t e r v a l (180 msec) w i t h n o r m a l P wave-atrial (P-A) interval (30 msec), normal His ventricular (H-V) interval (40 msec) and a normal sinus node recovery time after right atrial pacing at 120 beats per minute for two minutes (820 msec). After intravenous administration of atropine (1 mg), the A-H interval decreased slightly to 170 msec but the P-A and H-V intervals remained the same. The echocardiogram was normal. Her pulmonary function studies at this time showed a r e s t r i c t i v e p a t t e r n w i t h a n o r m a l single breath transport factor (Dco). Intradermal s k i n tests for h i s t o p l a s m o s i s , coccidi0sis, trichophyton and tuberculosis were all nonreactive. A posterior auricular lymph node biopsy showed chronic noncaseating granulomatous changes typical of sarcoidosis with no acid-fast or fungal organisms. Because of her pulmonary involvement the
Cardiac sarcoidosis was first noted in 1929 by Bernstein. 1 As early as 1959 Gold 2 rep o r t e d t h e t r e a t m e n t of m y o c a r d i a l sarcoidosis with glucocorticosteroids, but as yet t h e role of s t e r o i d s in t h e t r e a t m e n t of myocardial sarcoidosis has been difficult to ascertain because the number of patients with clinically apparent myocardial sarcoidosis is small, spontaneous remission may occur, and t r e a t m e n t with glucocorticoids has often been combined with other drugs. We report this case because it documents the beneficial effect of prednisone t h e r a p y on a t r i o v e n t r i c u l a r (AV) conduction in a patient with myocardial sarcoidosis and recurrent AV block who was treated with prednisone on three separate occasions.
CASE REPORT This twenty-eight year old black woman first presented to the University of Kansas Medical Center in October of 1975 with dizziness, weight loss, anorexia, and dyspnea on exertion. Her p e r t i n e n t p h y s i c a l findings included a r e g u l a r pulse of 104 beats per
From the Cardiovascular Department, University of Kansas Medical Center, College of Health Sciences and Hospital, Kansas City, Kansas. The costs of publication of this article were defrayed in part by the payment of page charges. This article must therefore be hereby marked "advertisement" in accordance with 18 U.S.C. w 1734 solelyto indicate this fact. Reprint requests to: Bert Wong, M.D., Cardiovascular Department, University of Kansas Medical Center, College of Health Sciences and Hospital, 39th and Rainbow Boulevard, Kansas City, Kansas 66103. 325
326
LASH ET AL
p
p i! 84
B
Fig. 1. Rhythm strips obtained before (A) and after 8 months of the first period of treatment with prednisone (B). Before treatment there is a sinus rhythm with occasional Mobitz-type I AV block. Following treatment the P-R interval returned to normal.
patient was treated with prednisone (40 mg daily) and isoniazid (300 mg daily). After eight months of treatment, the P-R interval on her resting ECG had decreased from 0.28 seconds to 0.18 seconds (Fig. 1B). During this time the patient gained weight and developed hypertension which was treated with hydrochlorothiazide. Because of her weight gain the p a t i e n t d i s c o n t i n u e d the p r e d n i s o n e and isoniazid on her own. Within a two-month period after stopping the drugs her resting ECG showed a prolonged P-R interval >~ 0.38 sec with Mobitz-type I AV block (Fig. 3A). Prednisone therapy was reinstituted over the next four months. The degree of AV block progressively decreased until the P-R interval was 0.16 second (Figures 3B, 3C, 3D). Then, because her serial pulmonary function tests had shown no improvement, the prednisone was again stopped. Subsequently the patient developed complete heart block, initially with a slow junctional rhythm followed by a slow idioventricular r h y t h m (Fig. 4A). At this time a His bundle potential could not be identified with intracardiac electrocardiographic studies and administration of intravenous atropine (1 rag) and isoproterenol did not improve AV conduction. Cardiac catheterization at this time showed normal right and left h e a r t p r e s s u r e s , m i l d - m o d e r a t e (2+) mitral regurgitation, and normal coronary arteriograms. Prior to this time prednisone had been given primarily for treatment of the patient's pulmonary involvement. Although the concomitant improvement of AV conduction with prednisone therapy was noted, we could not be sure that this was not a coincidental occurrence. Thus the patient's complete heart
block was treated with placement of a permanent t r a n s v e n o u s pacemaker. The p a t i e n t was then restarted on t r e a t m e n t with prednisone (60 mg every other day) for the third time to determine if AV conduction could be restored again. After two months of t r e a t m e n t the patient's r e s t i n g E C G showed a paced v e n t r i c u l a r rhythm with occasional sinus capture beats, and intravenous atropine (1 mg) produced a transient sinus rhythm with Mobitz-type I AV block (Fig. 4B). After six months, sinus rhythm with a prolonged P-R interval was restored (Fig. 4C). During this time, because of s y m p t o m s of d y s p n e a on exertion, she w a s s t a r t e d on t r e a t m e n t w i t h Digoxin, furosemide and isoniazid. H e r serum electrolytes and calcium obtained periodically throughout this time remained within normal limits. DISCUSSION This p a t i e n t p r e s e n t e d w i t h the typical findings of pulmonary sarcoidosis with cardiac involvement. A l t h o u g h biopsy of the myocardium was not done, the occurrence of progressive heart block and mitral regurgitation in a patient with sarcoidosis in the absence of any other specific cause is highly suggestive of sarcoid heart disease. 4,5 She developed AV block on three separate occasions. On the first two occasions the site of block occurred in the AV node. On the third occasion the site of block probably involved both the AV node and the His Purkinje system. The lack of response to intravenous atropine and isoproterenol indicated that the AV block on each occasion was due to organic disease and not to enhanced vagal tone. J. ELECTROCARDIOLOGY, VOL 12, NO. 3, 1979
HEART BLOCK DUE TO SARCOID HEART DISEASE
327
A
II
,
A.-
_
V l ~
,
I
AEmm~,qr
-
P-A = 30 MSEC
A-H = 180 MSEC
,
'
I
B
i
_
I
i
A
i
'
H-V = 40 MSEC
H
i
~
I
i
I
I
Vl
AE
A
A H
'
H 170
I
P-A = 30 MSEC
A-H = 170 MSEC
N-V - ~0 ~SEC
Fig. 2. His bundle electrogram with bipolar atrial electrogram and surface electrocardiographic leads I, II, VI during sinus rhythm before (A) and after (B) administration of intravenous atropine (1 mg).
Glucocorticosterolds have been used prev i o u s l y for the t r e a t m e n t of AV block in p a t i e n t s w i t h a n d w i t h o u t c a r d i a c sarcoidosis. ~'4'~'7 Because of the variable course of AV block, during which spontaneous remission m a y occur, and the inconsistent response to t r e a t m e n t with glucocorticosteroids, results have been difficult to evaluate, s,9,1~This patient's clinical course is unique because AV block responded to treatment with prednisone on three separate occasions. The fact that this patient's AV conduction improved concomitJ. ELECTROCARDIOLOGY, VOL 12, NO. 3, 1979
antly with prednisone therapy on three sepa r a t e occasions m a k e s t h e p o s s i b i l i t y of spontaneous remission unlikely. Although the patient did take several other medications, only p r e d n i s o n e t h e r a p y correlated with improvement of AV conduction. Thus, it seems reasonable to conclude t h a t steroid therapy improved AV conduction in this patient with myocardial sarcoidosis. The clinical manifestations of myocardial sarcoidosis are varied and multiple. They include sudden death, congestive heart failure,
328
LASH ET AL
Y
A
P
P
P
P
P
. IPFI.~0.38
y B 1:R:0,24
C
D
Fig. 3. Rhythm strips obtained before (A) and after I month (B), 3 months (C) and 4 months (D) of the second period of treatment with prednisone. Prior to therapy Mobitz-type I AV block has recurred. Following treatment there is a sinus rhythm with a gradual return of the P-R interval to normal.
r e c u r r e n t p e r i c a r d i a l effusions, p a p i l l a r y muscle dysfunction, ventricular aneurysms, tachyarrhythmias, and AV conduction disturbances, s'9,1~-19 The pathological features of cardiac sarcoidosis consist primarily of infilt r a t i o n of t h e m y o c a r d i u m w i t h sarcoid granulomas, myocardial fibrosis, ventricular aneurysm, and focal fibromuscular dysplasia of the small coronary arteries. 7,11 The exact cause of AV block in cardiac sarcoidosis is not known. Sarcoid granulomas have a predilection for the interventricular septum and potentially can physically encroach upon and disrupt the conduction system. 7,1~,~2,'3 Since prednisone therapy may decrease the size of granulomas, 7 this theoretically could result in improved AV conduction. The long period of time required for AV conduction to improve following prednisone t h e r a p y suggests t h a t the immediate hormonal and cellular effects of glucocorticosteroids were not the m e c h a n i s m w h e r e b y AV c o n d u c t i o n improved. Ischemia of the AV node due to fibromuscular dysplasia involving the AV node artery has also been previously described as a possible cause of heart block in sarcoid heart disease. 11 The coronary arteriograms in this patient were completely normal. Although this rules out large vessel coronary artery
disease, it does not exclude the possibility of small vessel involvement. This report documents the improvement of AV c o n d u c t i o n w i t h g l u c o c o r t i c o s t e r o i d therapy in a patient with cardiac sarcoidosis. The r e s u l t s s u g g e s t t h a t t r e a t m e n t w i t h glucocorticosteroids can be beneficial to patients with cardiac sarcoidosis manifested by AV block. W h e t h e r g l u c o c o r t i c o s t e r o i d therapy is beneficial in the t r e a t m e n t of other manifestations of cardiac sarcoidosis remains to be determined.
1.
2. 3.
4.
REFERENCES BERNSTEIN, M, KONZLEMANN, F W AND SIDLICK, D M: Boeck's sarcoid; report of a case with visceral involvement. Arch Intern Med 44:721, 1929 GOLD,J A AND CANTOR,P J: Sarcoid heart disease. Arch Intern Med 104:101, 1959 SCHERLAG,B J, LAU,S H, HELFANT,R H, STEIN E, BERKOWITZ,W D AND DAMATO, A N: Catheter techniques for recording His bundle activity in man. Circulation 39:13, 1969 STRAUSS,G S, LAWTON,B R, WENZEL, F J AND RAY, J F I I I : Detection of covert myocardial sarcoidosis by scalene node biopsy. Chest 69:790, 1976
J. ELECTROCARDIOLOGY, VOL 12, NO. 3, 1979
HEART BLOCK DUE TO SARCOID HEART DISEASE
329
A
B
Fig. 4. Rhythm strips after prednisone was stopped before insertion of transvenous pacemaker and during third period of treatment with prednisone after insertion of permanent transvenous pacemaker. Prior to therapy there is complete AV block with a junctional escape rhythm competing with a ventricular escape rhythm (A). After 6 weeks of prednisone therapy, transient sinus rhythm with Mobitz-type I AV block could be produced by intravenous atropine (B) though the basic resting rhythm was a paced rhythm. After 6 months of prednisone treatment (C) sinus rhythm with a prolonged P-R interval is present. The transvenous permanent pacemaker has been activated transiently with a magnet.
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