Congenital Aneurysmal Defect of the Mem-

branous Portion of the Ventricular SeptumAssociated with Heart Block, Ventricular Flutter,

Adams-Stokes Syndrome and DeathBy RICHARD J. CLARK, M.D., AND PAUL D. WHITE, M.D.

A case of congenital anieurysmal defect of the ventricular septumi is reported which appears to beunique in that this lesion was the only significant finding at autopsy. The patient had evidence ofauriculoventricular block for a period of 26 years, at first partial and then complete. She succumbedat the age of 47 to a series of Adams-Stokes attacks which were demonstrated to be set off by parox-

ysmal ventricular flutter followed byr ventricular tachy (caridia and ventricular standstill. Pertinentliterature is briefly reviewed.

T HE FOLLOWING case is conisideredworthy of recording since it appearsto be the only one which we can find

on record where an aneurysmal defect of theventricular septum was the sole significantanatomic lesion found at autopsy. It demon-strates that ventricular flutter, unsuspectedwithout cardiographic evidence, may precipi-tate the Adams-Stokes type of syncope.

PREVIOUS LITERATURE

Aneurysm of the Ventricular Septum. Becauseof its presumed lack of clinical significance,little has been written on the subject of anetu-rysm of the ventricular septum. Abbott, inOsler's Modern Medicine,1 described the path-ologic condition briefly avid reviewed the origi-nal studies of Mall.2 In, 1938 Lev and Saphir'published two cases and reviewed 70 caseswhich had been reported in the literature.Since this review wve find only one further casereported, that of Castoldi4 in 1942.

Adams-Stokes Syndrome. The Morgagni-Adams-Stokes syndrome as originally de-scribed referred to the association of syncope,epileptiform (convulsions and marked slowingof the heart action. Parkinson and co-workers`in 1941 reviewe(d all reported cases of theAdams-Stokes syndrome with electrocardio-

From the Cardiac Department, Winchester Hospi-tal and the Cardiac Clinics and Laboratory of theNIsschsetts ('ener.al llospital, Boston.

725

graphic tracings and found that only 55 percent of reported attacks were associated withventricular arrhythmias. Parkinson defined theAdams-Stokes disease as the "name applica-ble to patients with heart block who sufferfrom recurrent attacks of loss of consciousnessdue to ventricular standstill, ventricular tachy-cardia, ventricular fibrillation or a combinationof these." Schnur,6 in 1948 reiterated theseviews and presented a case of ventricularfibrillation, tachycardia and asystole imposedupon complete heart block. Pastor and Wor-rilow7 have recently reviewed the electrocardio-graphic patterns in the Adams-Stokes syndromeand found 20 cases on record in which bothventricular arrhythmias and ventricular stand-still had been showni to occur during theAdams-Stokes syncope; inl eight of these easesthe arrhythmias were inl association with com-plete A-V block.

(Congenital Heart Block. Heart block of ( on-genital origin as reported in the literature hasbeen reviewed by Yater and associates," witha study of 44 acceptable cases of which aventricular septal defect was found to be pres-enit in 26. Faessler9 reported eight cases of theAdams-Stokes syndrome associated with (on-genfital heart disease, of which six were diag-nosed as having ventricular septal defects. Es-senftially all types of congenital defects havebeeni reported as associated with heart block.10

I entriceida Flatter ilersuis Fibrillation. Ini 1925Circulation, Iolume V, May, 19.52

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CONG76,NITAL ANEURYSMAI, DEFECT OF VENTRICULAR SEPTUM

Sir Thomas Lewis'1 stated that the nature ofthe disturbances inicluded under the termventricular fibrillation was undefined. Hebelieved that varying grades of ventriculararrhythmias occurred, as iln the auricles, rangingfrom flutter, to impure flutter and grosslyimpure flutter or fibrillation. Fastier andSmirk'2 described the condition of ventricularflutter, observed in experimental cats and dogs,as showing characteristic regular undulatorywaves on the electrocardiogram which arequite in contrast with the disorganized move-ment, usually of smaller amplitude, seen illventricular fibrillation.

CASE HISTORY

S. B., aged 47. This patient was said to have beenin normal health, except for diphtheria in childhood,until 1921, when, at the age of 21, she was deliveredof her first and only child. A review of her hospitalrecord indicated that following a normal labor anddeliverv she had had five fainting spells followed b-a convulsion, associated with irregularity of thepulse which at times was not palpable. BY the pa-tient's own statement, her pulse subsequent to thiswas usually slow, about 40 per minute, but she wasgenerally well and led a normal life. In 1936 a carefulexamination showed a pulse rate of 44, a blood pres-sure of 136/80, with heart sounds of good quality.An electrocardiogram showed first degree A-V blockwith a ventricular rate of 42 and a P-R interval of0.24 of a second; the tracing was otherwise notremarkable.

In 'March 1947, at the age of 47, the patient wasseen in consultation by one of us (R. J. C.). For theprevious four or five years she had complained of"lizzy sensations," distinct increase in dyspneaand brief bouts of low substernal pressure withradiation to the left arm brought on by excitementor exercise. Examination showed a pulse rate of 48,regular and full. The blood pressure was 165/100.The heart showed a left border of dullness 2 cm.outsi(le the midelavicular line. The heart soundswere forceful in character with an accentuatedpulmonary second sound which was greater thanthe aortic second sound. There was a grade 2 systolicmurmur, without a thrill, heard best between theapex an(l the left sternal border. The lungs wereclear and there was no evidence of congestive failure.Fluoroscopic examination showed a slight increasein the transverse diameter of the heart with theappearance of slight left ventricular hypertrophy.An electrocardiogram showed a regular ventricularrate at 42 with the P-R interval increased to 0.26of a second, but was otherwise not remarkable. A

urinal-sis was normal. A Hinton test w-as negative.Photoelectric determination of hemoglobiin was 13.5Gm. The presumptive diagnosis at that time wascongenital heart disease with a ventricular septaldefect and associated heart block wdith superimposedcoronary artery disease.On the morning of June 15, 1947, the patient was

admitted as an emergency case to the WinchesterHospital because of an attack of generalized con-vulsions and unconsciousness of 10 minutes duration.Directly after admission a recurrent attack wasobserved. The pulse could not be obtained and noheart sounds were heard. One-half cc. of 1: 1000adrenaline solution wvas given intramuscullarly.Another attack occurred after a half hour, duringwhich an electrocardiogram was obtained. Thisshowed ventricular flutter at a rate of 220 in theportion taken during the acute attack. This wasfollowed by complete A-V block wvith an auricularrate of 130 and a ventricular rate of 42, also variatbleectopic ventricular beats. The basic pattern hadnot otherwise changed significantly from that foundin 1March.When it became apparent that the true situation

w-as one of Adams-Stokes episodes associated withventricular flutter, adrenaline was omitted and thepatient was started on quinidine sulfate by mouthand atropine sulfate, 0.6 mg. by hypodermic injec-tion. During the afternoon she continued to haveconvulsions at approximately hourly intervals; fre-quently clusters of two or three attacks covered aperiod of 15 to 20 minutes. On several occasions,between attacks, the patient complained of severesubsternal oppression with radiation to the left armwherefore morphine sulfate 10.6 mg. by hypodermicwas given. She was maintained in an oxygen tent.The patient was seen in consultation by one of us(P.D.W.) that afternoon. Quinidine sulfate wasstepped up in dosage from 0.2 Gm. orally to 0.4 Gm.of lactate every two hours intramuscularly becauseof vomiting. During the night the patient's attacksdecreased slightly in frequency but the followingday at 12:30 P.M. there was a more severe seizurein which she expired. During her period of hospitali-zation the patient had some 30 convulsive seiz-ures. In the course of 24 hours she received 3.5 Gm.of quinidine.Numerous electrocardiographic observations were

made which showed periods of ventricular flutterlasting up to three minutes, frequently followed byperiods of ventricular tachycardia lasting two orthree seconds, followed by periods of ventricularstandstill with P waves decreasing in frequency.The longest period of measured standstill was 75seconds. Following the ventricular standstill thepatient resumed complete heart block with theventricular rate speeded to about 60 at first thendropping into the 40's. Figure 1 shows a recording ofone of the more severe episodes.

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RICHARD J. CLARK AND PAUL D. WHITE7

The clinical (liagnosis prior to autol)sy wtas acuteimvocarlidal infarctioIn iml)osedl upon coronairartery (lisease, with lpossil)le congenital heart block.

Pathologic Exam inationAn autopsy was performed by D)octors 1)onald

McKay and 1)onald A. Nickerson. Significant find-ings xwere limited to the heart, which weighed 350

ness. The membranous lportion of the interventricu-lar septum was paper thin and herniated into theright ventricle, forming a p)ouch with its mouth onthe left ventricular side (fig. 2). The mouth of thepouch measured 1.8 cm. in diameter. The membranewas fused with the medial cusp of the tricuspidvalve. The p)ouch, when l)alloone(I out with thefinger, almost (omp)letely filled the orifice of the

Fin(. 1. Continuous electrocardiographic tracing, lead II, taken during a severe convulsive epi-sode. A. Onset with ventricular "flutter" at rate of 220. B. Widening of the beam caused by severeconvulsing and necessity of holding the patient. C. Shift to ventricular tachycardia at, slower rate.again followed by a brief reversion to "flutter." D. Appearance of ventricular stan(lstill with only1' waves in evidence, with progressive slowing of auricular activit. E. Resuml)tion of ventricularactivity (escape) with sinus bradycardia followed by high grade auriculoventricular dissociation.Note the disappearance of wi(le l)eam shadow ais convulsive movements cease. Time intervals equal0.04 second.

(Glm. The l)ericardiuml was smooth and glistening.The el)ieirldium was smooth. The coroiary arterieswere thin walled and patent throughout. The rightauricle appeare(l dilated. The mvocardium of theleft ventricle was firm and red brown1, showing noevidence of fibrosis or infarction; it measured 1.5Cm. in thickness. The right ventricular my-ocardiumwas slightly softer but otherwise not remarkable; thew-all of the right ventricle measured 0.4 cm. in thick-

tricusp}i(l xvalve. The remainder of the valves showedthin delicate leaflets with no evidence of inflamma-tion. The chordae tendineae were normal. Apartfrom the interventricular aneurvsmal defect therewere no congenital malformations of the heart orgreat vessels. The aorta was thin walled, elastic andlined by a smooth velvety intima. Microscopicsections taken from the right and left ventricularmusculature showed no recognizable abnormality of

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CONGE(INITAIL ANll'URYSMAIL DIFIICT OF VENTRICULAR SPEPTUM

muscle fibers or blood vessels. There was a moderatedegree of passive congestion in the luings, liver,kidneys and spleen. No specific cause of death wasfound anatomically.

Subsequently more detailed study of the heartwas carried out in the Pathological Laboratory ofthe Massachusetts General Hospital by DoctorsDavid Freiman and Robert Scullv. Block sections

A

B

Fic;. 2A. Aneurysiiial defect, as viewed from theleft ventricle, located in the membranous septumdirectly below the aortic valves. B. Aneurysmalpouch as viewed protruding into the right ventricle,showing adhesions to the tricuspid valve.

were Imadle of the entire aneurysm. These sectionsfailed to show evidence of anything which lookedconvincingly like the Bundle of His. The pouch was

counl)osedl of relatively acellular collagenous tissue.No evilence was found of mural endocarditis.

DIscuSSION'T'lhe diagnosis of aneurysmal defect of the

vent inular septum (cannot be made clinically.

In this case historical evidence pointed stronglyto the existence of heart block since at leastthe age of 21 years probably with Adams-Stokes syncope occurring following childbirth.A respite of 26 years without further occur-rence is remarkable. The "dizzy spells" whichthis woman had experienced for about fouryears were doubtless a result of her heart block.The history pointed strongly toward coronaryinsufficiency with mild angina pectoris in awoman with early hypertension, yet no coro-nary disease was found at autopsy. One mightspeculate as to whether her heart block couldhave produced a relative coronary insufficiencygiving rise to dyspnea and substernal distress.She had diphtheria in childhood. It has beendemonstrated that residual heart block fromdiphtheria occurs in only very rare individuals."3Barring such an assumption, the most likelycause of block at this age would be a congenitallesion, either of the septum or of the conductionfibers themselves. The heart murmur in thispatient was not typically that of a ventricularseptal defect either in location or intensity.No other congenital lesion was suggested. Atentative diagnosis of a ventricular septal defectwith an atypical murmur was made, but suchis doubtless an improbable assumption becauseof the absolute rarity of this condition.

In previously quoted cases6' 7 with a similarsequence of arrythmias, quinidine was quiteineffectual in therapy, as it proved to be inthis instance. The use of atropine in similarcases has been suggested to be of value.4' 1'5Lately procaine intravenously has been advo-cated in ventricular tachycardia and fibrilla-tion, both for prophylaxis and for emergencyuse on the operating table."6 Should anothercase similar to the one here reported be en-countered, a trial of Pronestyl (procaine amidehydrochloride) would seem worth while.'7

SUMMARY

A case history is presented of a woman Mitlheart block of at least 26 years duration, whodied at the age of 47 with Adams-Stokes at-

tacks precipitated by paroxysms of ventricui-lar flutter followed by ventricular tachycardia,ventricular standstill and complete auriculo--ventricular dissociation. At postmortem exami-

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RICHARD J. CLARK AND PAUL D. WHITE

nation the only significant abnormality was

the presence of a large aneurysmal defect ofthe ventricular septum.

ACKNOWLEDGMENTWe are indebted to Dr. Ernest MacDougall of

Wilmington, Massachusetts, who referred this pa-tient to us.

REFERENCESABBOTT, M. E.: Congenital heart disease. In

Osler's Modern Medicine, c l. 3. Philadelphia,Lea & Febiger, 1927. P. 699.

2 MALL, F. P.: Aneurysm of the membranousseptum. Anat. Rec. 6: 291, 1912.

3 LEV, M., AND SAPHIR, O.: Congenital aneurysm ofthe membranous septum. Arch. Path. 25: 819,1938.

kCASTOLDI, P.: Blocco di branca in portatore deaneurisma della pars membranacea septi. Clin.med. ital. 73: 161, 1942.

5 PARKINSON, J., PAPP, C., AND EVANS, W`.: Elec-trocardiogram of Stokes-Adams attack. Brit.Heart J. 3: 171, 1941.

6 SCHNUR, S.: Newer concept of Stokes-Adamssyndrome. Am. Heart J. 35: 298, 1948.

7 PASTOR, B. H., AND WORRILOW, S. H.: Electro-trocardiographic patterns in Stokes-Adamssyndrome. Ann. Int. M\ed. 34: 80, 1951.

S YATER, W. lM., LYON, J. A., AND MICNABB, P. E.:Congenital heart block: Review and report ofsecond case of complete heart block studied by

serial sections through conduction system.J. A. M. A. 100: 1831, 1933.

9 FAESSLER, B.: Das Adams-Stokessche Syndromin SAuglingsalter. Ann. padiat. 153: 327, 1939.

10 CLARK, R. J., AND FIRMINGER, H. I.: Coarctationof the aorta associated with Adams-Stokessyndrome, complete heart block and bicuspidcalcareous aortic valve. New England J. Med.240: 710, 1949.

LEWIS, T.: The Mechanism and Graphic Registra-tion of The Heart Beat, ed. 3. London, Shaw &Sons, 1925. P. 369.

12 FASTIER, F. N., AND SMIRK, F. H.: Some propertiesof amarin, with special reference to its use inconjunction with adrenalin for the productionof ideo-ventricular rhythms. J. Physiol. 107:318, 1948.

13 WHITE, P. D.: Heart Disease, ed. 3. New York,Macmillan, 1945. P. 393.

14 AA ILBUNE, M., SURTSHIN, A., RODBARD, S., ANDKATZ, L. N.: Inhibition of Paroxysmal Ventricu-lar Tachycardia by Atropine. Am. Heart .J. 34:860, 1947.

15 LEROy, G. V., FENN, G. K., AND GILBERT, N. C.:The influence of xanthine drugs and atropineon tile mortality rate after experimental oc-clusion of a coronary artery. Am. Heart J.23: 637, 1942.

16 BARBOUR, C. M., AND TOVELL, R. M.: Experienceswith procaine administered intravenously. Anes-thesiology 9: 514, 1948.

17 MILLER, H., NATHANSON, Ml. H., GRIFFITH, G. C.:The action of procaine amide in cardiac ar-rvtlhmias. J. A. WM. A. 146: 1004, 1951.

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RICHARD J. CLARK and PAUL D. WHITEAssociated with Heart Block, Ventricular Flutter, Adams-Stokes Syndrome and DeathCongenital Aneurysmal Defect of the Membranous Portion of the Ventricular Septum:

Print ISSN: 0009-7322. Online ISSN: 1524-4539 Copyright © 1952 American Heart Association, Inc. All rights reserved.

is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231Circulation doi: 10.1161/01.CIR.5.5.725

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