Annotations 571

6. Zinsser, H. F.: Pericardiectomy for recurrent idiopathic pericarditis in the absence of signifi- cant cardiac constriction, Tr. Am. Clin. & Ciimatol. A. 69:40, 19.57.

7. Zinsser, H. F., Johnson, J., and Blackemore,

W. S.: The concept of pericardiectomy as a treatment for recurrent idiopathic pericarditis in the absence of cardiac constriction, Am. J. M. SC. 283:464, 1959,

Corticosteroids in Stokes-Adams syndrome

The nature of the Stokes-Adams syndrome was first recognized in 1776,’ and it was particularly well defined in 1927, when Parkinson2 and his group classified these attacks in the following categories: (a) ventricular standstill; (b) ventricular tachycar- dia and ventricular standstill; (c) ventricular tachy- cardia or fibrillation, or both; and (d) extreme brady- cardia due to heart block. Since that time, other cardiac conditions have also been recognized as capa- ble of producing this syndrome, and DeBoer3 has defined it as “every disturbance of the action of the heart that begins and ends abruptly and causes such interruption of the circulation that more or less complete cerebral &hernia results.” Clinically, there may be all grades of disturbances of consciousness, from a mild lightheadedness or dizziness to complete unconsciousness with convulsions.

Ordinarily, the basic pathologic abnormality is arteriosclerosis with fibrosis which causes interfer- ence of A-V conduction or, in some instances, an irritable focus with the production of arrhythmias. As a result of the poor supply of blood, there may be ischemia or actual myocardial necrosis. In other cases the defect may be due to a myocarditis of rheumatic or, very rarely, of diphtheritic etiology. Interference with conduction and the production of arrhythmias may also result pharmacologically from the use of certain drugs, notably quinidine and digitalis.

Treatment of Stokes-Adams attacks has been attempted with many drugs, including epinephrine, ephedrine, isoproterenol, atropine, and molar sodium lactate. Ouinidine and procaine amide have also been useb-, although in some instances they may actually precipitate episodes of ventricular *tach$- cardia or fibrillation.4 Recentlv. electrical nace-

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makers have also come into use.6 The drug or pro- cedure of choice is controversial. Robbin and asso- ciates,& and Schumacher and Schmock7 believe that the drug to use is isoproterenol, but ZoW and his group favor ephedrine or epinephrine for standstill or for a slow idioventricular rhythm.

At times none of these agents is effective. Re- cently, there have been several reports of the use of corticosteroids for the treatment of this syndrome. Prinzmetal and Kennamers reported that, on two separate occasions, the use of ACTH terminated attacks of ventricular asystole and syncope in a patient who had a posterior myocardial infarction. Phelps and Lindsay9 brought about abolition of complete A-V block through the use of cortisone intramuscularly in a patient who had anterior in-

farction. In another instancelo a case of complete ii-V block reverted to partial (3:l) ,4-V block, and, at the same time, episodes of syncope and convul- sions due to ventricular tachycardia and standstill were abolished during the administration of oral cortisone. In this case there was reversion to com- plete A-V block, and death, when this medication was stopped. At autopsy there was fibrosis and calci- fication in the region of the A-V node and bundle of His, and there were also vascular scars which were thought to be due most probably to a previous rheu- matic myocarditis.

The beneficial effect of the corticosteroids may be due to several possible mechanisms. There is good evidence that these agents have an anti-inflammatory effect. Massell”~‘2 and his group found that the corti- costeroid-hormone treatment of 66 patients with rheumatic fever caused the prolonged P-R interval to revert to normal in 54 of them. There may also be a “biologic cooperation” between C-ll-oxysteroids and the sympathetic nervous system, as postulated by Lown and co-workers,‘3 who found a correlation between the urinary excretion of 17-ketosteroid and the A-V conduction time. They studied a group of patients with Addison’s disease and also another group with Gushing’s syndrome, and found that those with Addison’s disease tended to have long P-R intervals, and that, in some instances, there was first-degree .4-V block, whereas many of those with Cushing’s syndrome had definitely short P-R in- tervals. Gerisch and associates,‘* in a study of the effect of cortisone treatment in experimentally pro- duced myocardial infarction in dogs, found that there was increased vascularity of the myocardium due to dilatation of the collateral arteries. The result- ing areas of fibrosis in the infarction were smaller than those in the control animals. Also, no throm- boses were seen in the smaller ressels of the treated animals, in contrast to the findings in the control animals. Gerisrh and co-workers also treated a group of patients with myocardial infarction with cortisone and found that this group derived very definite benefit. Another factor which may be of importance in steroid treatment is the production of alkalosis. The presence of alkalosis has been postulated’s,‘6 as the factor which makes oossible the beneficial effect of molar sodium lactate. Houle17 and his group found a diminished cardiac and pressor response to epine- phrine in the presence of acidosis. Levels of serum potassium may also be important in relationship to the effect of the steroid hormones.1* However, ad- ministration of potassium has had a beneficial effect

572 Annotations

in abolishing A-V block in some patients although increasing the degree of block in others.

In summary, although the rationale for the use of corticosteroid hormones in selected cases of Stokes- Adams attacks is as yet unclear, there are several different possible beneficial mechanisms; these in- clude: (a) the improvement of collateral circulation, (b) a specitic antiinflammatory effect, (c) the produc- tion of alkalosis, and (d) the “snecific cooperative . effect” in conjunction with the sympathetic nervous system. The relationship and relative importance of these various factors is not yet well defined, and others may be still unrecognized. However, in se- lected cases of heart block and Stokes-Adams at- tacks, when conventional treatment methods have been ineffective, the use of corticosteroids seems to be indicated and may be benelicial.

Edward L. Perry, M.D. Gundersex Clinic and Foundation

La Crosse, Wis.

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REFERENCES

Major, R. H.: Classic description of disease, Springfield, Ill., 1939, Charles C Thomas, Pub- lisher. Parkinson, J., Papp, C., and Evans, W.: The electrocardiogram of the Stokes-Adams attack, Brit. Heart J. 3:17, 1941. DeBoer, S.: On the origin and essence of the Morgagni-Adams-Stokes syndrome, Ann. Int. - - Med. 37:48, 1952. Schwartz. S. P.. Margolies. M. P.. and Firenze. A.: Transient ventr&ular’ fibrillation. V. The effects of the oral administration of quinidine sulfate on patients with transient ventricular fibrillation during established A-V dissociation, AM. HEART J. 45:404, 1953. Zoll, I’. W., Linenthal, A. J., Norman, L. R., Paul, M. H., and Gibson, W.: External electric stimulation of the heart in cardiac arrest, A.M.A. Arch. Int. Med. 96:639, 19.55. Robbin, S. R., Goldfein, S., Schwartz, M. J., and Dach, S.: The treatment of ventricular asystole, ventricular tachycardia and ventriru- la; fibrillation associated* with complete heart block, Am. J. Med. 18:577, 1955. Schumacher, E. E., Jr., and Schmock, C. L.:

8.

9.

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11.

12.

13.

14.

15.

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17.

18.

The control of certain cardiac arrhythmias by. isopropyl norepinephrine, AM. HEART J. 48:933. 1954. Prinzmetal, M., and Kennamer, M. D.: Emer- gencq treatment of cardiac arrhythmias, J.A.M.A. 154:1049, 1951. Phefps, M. B., and Lindsay, J. I)., Jr.: Cortisone in Stokes-Adams disease secondary to riiyo- cardial infarction. Report of :I case, New- Eng- land J. Med. 256:204, 1957. Perry, E. I,., and Jaeck, J. L.: I!se of cortic,o- steroids in Stokes-.qdams syndrome, Ann. Int. Med. 53:589, 1960. Massell, B. F.: ACTH and cortisone therapy of rheumatic fever and rheumatic rarditis, New England J. Med. 251:183, 1954. Massell, B. F. : ACTH and cortisone therapy of rheumatic fever and rheumatic carditis, New England J. Med. 251:221, 1954. Lawn, I?., Arons, W. L., Ganong, W’. F., \‘aeif- dar, J. I’., and Levine, S. A.: Adrenal steroids and auriculoventricular conduction, A&c. HEART J. 50:760, 1955. Gerisch, Ii. A., Johnson, A. S.. Xnderson, \V. L., Moehlig, R. C., and Compeau, L.: The treat- ment of acute myocardial infarction in man with cortisone. In exhibit and report at the Annual

Meeting of the American IMedical Association, New York, 1961. Bellet. S.. Wasserman. F.. and Brodv. I. I.: Molar sodium lactate.’ Its effect in complete auric~uloventricular heart block and cardiac arrest occurring during Stokes-Adams seizures and in the terminal stage, New England j. Med. 253:891, 1955. Bellet, S., Wasserman, F., and Brody, J. I.: Effect of molar sodium lactate in increasing cardiac rhythmicity, J.A.M.A. 160:1293, 1956. Houie. D. B. Quoted by Vandam, L. I)., and McLemore, G. .A.: Circulatory arrest in patients with complete heart block during anesthesia and surgery, Ann. Int. Med. 47~518, 1957. Bettinger. J. C., Surawicz, B., Bryfogle, J. \2’., Anderson, B. W., Jr., and Bellet, S.: Effect of intravenous administration of potassium chlo- ride on ectopic rhythms, ectopic beats and dis- turbances in A-V conduction, Am. J. Med. 21:521, 19.56.