(‘ONGENITAL COMPLETE HEART-BLOCKM

AN &COUNT OF EIGHT CASES

MarmIcx f’AMPBELr,, M.D., F.R.C’.P. AND

s. s. SUZRIAN, M.R.C.P. LONDON, ENGLAND

C ONGENITAL complete heart,-block is such an uncommon condition that an account of the following 8 cases and of one specimen from

the Guy’s Hospital Museum is of interest.. In a recent review by Yate9 only 30 of the published cases were accepted as congenital heart-block. He rejected 31 others for lack of sufficient evidence, either owing to the absence of graphic records or because there was a history of some infec- tion which might possibly have been the cause of the block. Probably many of these were genuine cases, but even accepting them all, the num- ber reported has not been very great.

We believe the condition to be more common than would appear from t.hese figures! for we have seen 8 cases in the last six years. It is not difficult for some of them to be missed because the symptoms are gener- ally slight, and the heart rate is faster than in other types of complete block, and the signs of morbus caeruleus arc generally absent. If a slow pulse is found in a young man who is an athlete, it is generally sinus bradycardia. But if a slow pulse is found in a child, the possibility of complete heart-block must he remembered, and if it is present and Ijersists, it is likely to be congenital, for infection plays a less important r6le in its production than is sometimes supposed.

Diphtheria is one of the commonest causes of temporary heart-block, which, as a rule, does not last more than a short time: when diphtheria produces a sufficiently severe myocarditis with heart-block, the symptoms are so serious that they are not likely to bc missed in determining the cause of heart-block some years later. In a series of 100 cases all followed for more than five years by Jones and WhiteI” no example of heart-block was found to persist, and in another series of 100 nlstead3 mentions only one, described fully elsewhere by himself and Chamber- lain,0 where permanent heart-block result.ed. Wilkinsonz6 has not seen persistent heart-block in the diphtheritic cases he has studied at Birming- ham, and Place’s experience is the same.?’

Alstead comments on the curious fact that though tempora,ry complete heart-block is not uncommon in diphtheria, a.ny prolongation of the P-R interval above 0.2 sec. is rarely found without actual A-V dissociation.

*From the Cardiographic Department of Guy’s Hospital and the National Hospital for Diseases of the Heart. London.

304

TABL

E 1

e

SUM

MAR

Y O

F O

UR

EIG

HT

ChSE

.S

P E

I I

AGE

IN

YEAR

S

CASE

NU

MBE

R SE

X

, Av

erag

e --

WHE

N SL

OlV

PT

JI,S

E FI

RS1

/ O

BSER

VED

-/ / 0.

G

I 1

i ‘,

~ :

j it 13

___.

. ..-

.- 3.

75

VHEN

FI

RS?

SEEN

W

ITH

EK

G.

_i_j

-

1’7

12

12

27

2%

13

__I_

~~

12

.6

IIEAR

T R

AT

AT

RES

T 1

13

; GO

-68

lG

33

I 13

6 10

4-11

2 6

' 88

-10s

21

: 84

-100

17

!

75-86

~

---

___

15.7

107

VENT

RICL

E

BLO

OD

PRES

STJR

E

WAS

SERM

AKK

SYST

OLI

C

DIA

STO

LIC

R

EAC

TIO

N

IIEI\f

ARKS

n

CAMPBELL AND YUZMAN : CONGEKITAL CO~~I’IXTE lIEART-BLOCK 307

oldest thirteen years, but he was the only one who passed his fifth year without attention being drawn to his heart.

The average age at which they first came under our observation and had electrocardiograms taken was 12.6 years. One was eighteen months, one was twent,p-seven years, but, most of lhem were about twelve years old.

811 the patients are still in good health except one ((‘ase 6), who diet1

when six years old. Two patients (Cases -I and 5) live too far away to return to the hospital but have replied fully to our enquiries, and all the others have been reexamined this year. Their ages are now thirty-

three, twenty-one, nineteen, seventeen, sixteen, thirteen, and three years. (?ase 2 WRS I’llll~ esnminccl \\~llt?ll tW\;FIVP ~CCIIX Old ilIId again \VhtXll

nineteen.

Fig. l.-Electrocardiogram. Case 1. Complete heart-block : ventricle 75, Lead I ; 65, Lead III: auricle, 143. Time rna~k~r in this am? all other electrocardiograms one- flfth SIX.

The first criterion of a slow pulse at an early age was therefore fairly

well satisfied, though curiously enough this was never noted durjng the first few weeks of life. Bs regards the second, there were physical signs in all 8 cases to suggest congenital heart disease, generally a defect of the interventricular septum. None had morbus caeruleus and 0111,~ one was diagnosed patent ductus arteriosus and pulmonary stenosis. The third criterion of the absence of infect,ions which might have caused tho heart-block was generally observed, and none gave a history of previous diphtheria or rheumatism. Case 6 had acute rheumatism, but this was two years after the heart-block had been diagnosed, and there was noth- ing to suggest an earlier rheumatic carditis. Stokes-Ada.ms attacks oc- curred in Case 4 only. The full notes of our 8 cases follow, and certain details are summarized in Tahlc I.

Fig. 3.-Orthodiapram. Cast 1. M;lxhnum tl,ansversr diameter 9.7 cn~ (3.3 plus Ii.41 in R chest of 15 cm. 8cale rc(luced to one-yuartcr.

Fig. 3.-Electrocardio~ralll. Care 2, on 5. iii, :!G. Ilvart-block probably e<~mplctc : auricle 136 ; ventricle 66. or 44 with pulsus bigeminus. A~nother electrocardiogram of the same date showed no cxtraa?;stoles and definite cornglttc heart-block: aurirlt, 116, ventricle 64.

The confinement had hrcn normal. There was no history of any infectious dis-

ease. The child appeared normal cscqrt for IJcing r:ttlnr undersized. There was

no cyanosis and no clubbing of the lingers. ‘l’ln~ lrr:~rt rate after resting was 52;

there was eomplcte heart-lllovk. (Pig. 1.1 ‘1’11~ llwrt was enlarged to the left,

with a forcible al~ex beat. There was :I rougl~ systolic n~urmur and a thrill, loudest

over the pulmonary area. S-ray tilnrs contirnlc~rl tills I’nl:ll.gelllcnt to the left nntl

showed some increase to the right also.

CASE: 3.--(‘ongcXuit:l 1 compMe hcnrt~lllo~k and Iutcnt interventricular septum.

At the age of one year eongenital 1re:rrt disvasc with a slow pulse was diagnosed at a welfare clinic. When twelve yl’ara old the patient was first seen at the Heart Hospital. There was. no history of rheumatjam or auy other infections. She looked well and the only symptulu cum[)laincd of evils slight breathlessness on exertion,

hat she had nercr been allowed to do mnc~h iu thaw way of exercise. The apex

pcdic~lr (maximum transverse diameter 12.7 cm. in zi chrst of 42.8 cm. j (Fig. 5). Th(x conus and pulmonary artery and the aorta were promintnt and showed vigorous

pulsation. Blood pressure was lW/Sll. Thr~ pulse aft,cr exercise row to 19 in the

first quarter minutr, dropping to 48 prr minute after one minute, thus illustrnting the

rapid drop to normal.

Seven years after her first clectrooardiogram at the hospital and eighteen years

after the first obscrvatiou of her slow pulse, this girl was in good health and almost

without symptoms. At sight she would haw been takw for a wry fit, nornlxl girl.

CASE R.--Congenit:tl complrte heart-block with slight patent interventricular

septum. This patient’s heart was noticed to bc slow at the age of two, when the doctor attended him for bronchitis, aud again at the age of three when he had

measles. These hnd been his only illnesses. At six years old, at a routine school

examination, 2 heart murniur ~2s nlso rwordd Hc KU srnt to the Heart Hospital

when twelve years old by Dr. J. 1%. B. 11~~; hitherto hr had not been allowed to play games at school, but had led a nornml life otherwise.

He was a healthy looking boy with no signs of morbus caeruleus. The heart rate

x-aried between 44 and 48 and occasionally 52: it was regular csccpt for an occa-

sional cxtrasyslolc. The eicctrocardiogram (Fig. 1;) confirmed complete heart-block.

The apex beat was forcible zmd in the nipplt line. An x-ray examination confirmed

slight enlargement of the heart to the left (maximum transverse diameter 10.9 cm.

in a chest of 20.4 cm.), with a blunt tipped left. ventricle: pulsation was well

markrd in the aorta and in the pulmonar,v artery, rind this was 8 little fuller than

usual. (Fig. 7.) There was a fairly harsh systolic murmur, hr~ard rather widelp

;111(1 br%st ol-er the prccordium. The pulmonary second sound was accentuatrd and

at times rcduplicatcd. Ko thrill \ws felt. Iilood pressure was lSO./tj5. He was

SW~ again four months later when his condition was unchanged. Hc had been

very liheral in interpreting his permission to t,nke more exercise and had been

I)la?-ing football at school with pleasure and without ill result. It was felt wiser,

however, to forbid this until he had been seen for a longer time and his exercise

in~~rc~+sed more gradually. After nnothcr four months hc ~53s getting on well and

still anxious to be allowed to plar all g?tnes at schcml.

C’lSE -I.-Congenital complete heart-block with some patency of the intcrrentricular

septum. He was the only patient in this series with Stukes-Adams attacks. At the :~gc of two years he was plzcying on the Sandy when he had a fit in which hr went

Fig. 7.-Ortltodiagr;im, Case 3. Maximurt~ transverse diameter 10.9 cm. (2.7 plw 8.2) in n. chest of 20.4 cm. SPaIt rPclucrc1 to one-qllarter.

stiff and blue. For tlvo years he had these attacks rather frequently, sometimes as many as three a week, but otherwise remained quite well. Unfortunately the doctor who attended him then is dead, but the patient’s mother remembers his saying

it was due to his heart which was always slow: i.e., in between t,hes attacks when he

appeared quite well.

During the nest eight years he was much better and had only four attacks. His mother described the last One as follows: After a sudden groan he went uncon- scious; he was stiff and blue with no movements of any sort; she thougkt that he

n-as unconscious for twenty minutes; afterward he apparently recovered very

quickly. Except for these attacks he had no complaints. There was no history of

rheumatism or of any infectious diseases. He was sent to Guy’s Hospital by Dr. C. A. Hicks, when twelve years old.

He appeared to be a healthy boy. His heart rate at rest was generally about 42, with at times fairly frequent extrasystoles; and an clcctrocardiogram (Fig. 8)

confirmed complete heart-block. The heart was slightly, if at all, enlarged (no x-ray examination was made). There was a systolic murmur best heard in the fourth, fifth, and sixth spaces to the left of the stcruum. No thrill was rerorded.

Blood pressure was 120/45.

cardiogram confirmed the diaguo*is of complete heart~hlork (Fig. 11). With extr

cise the heart quickened, being at the rntc of 6U iI1 the lirst quarter minute after~

ward.

The apex beat was fcrciblc and just outGdc the nipple line. From time to times

it was specially forrible and at the same time the carotid pulsation mas increased.

S-rer films confirmed slight enlargrmcnt to the left (maximum transverse diameter

11.7 cm. in a chest of 23.2 cm.; Fig. ISi. Thcl ~onns ~vas prominent, and this and

I . _. _ . - -. .- -..

Fig. Il.-Elcctrocardiogrnm, C:ase 7. Complctc hwrt-blwk: ventricle 46. auricle 100.

Fig. 12.-Orthocliagram, Case 7. Maximum transverse diameter 11.7 cm. (2.9 ~1~s 8.8) in a chest 23.2 cm. Scale reduced to one-quarter.

the aortic knob and the llcart it&elf showed vary vigorous pulsation. There was a

systolic murmur best heard inside the apex, and a systolic thrill in the same site.

Auricular sounds were frequently heard. Xlood premne was lSO/SO.

The attacks of faintness and giddiness were regnrdrd as ordinary faints and not as Stokes-Adams attacks, and this was confirmed lty thc4r disappearance with treat-

ment-an iron tonic and l.~RSsUl.illl(l~-31l~l l);v th<lir o,.rurrence under emotional stress. On two occasions when a Iwriod of faintness was observed, the pulse rate

rose to 60 and was of poor volume; she was not unconscious, and as she recovered,

the volume of the pulse improved and the rate fell to 44.

Fig. 14.-Teleradiogram, Case S. Heart appears rather rounded and prominent to the right and in the pulmonary arc. Maximum transverse diameter 13.9 cm.

1’1 IF. I1 14:.\1<7’ IC.\‘I’k: .\‘I‘ lilqS’l

The rate at YCS~ g;encr;lll>- \‘i\riecl l)<?w(~<~ll -1-C ;lnd 5,Ci and the ~VCIX~P

was 50. It may be because of this relalivcly I’ast rate that the diagnosis is not made more frequently. It. was unnsnal to fiiltl a rate snider 40,

though Fig. 6 shows a rate of 38. Table II sho~vs the slowest I--cntricular ratr observed in our 8 and in 3X of t,hc 3-S collcct4 c;tscs ; in t,he remnin-

ing 7 the b1oc.k was not. complete. In additioll to the 30 cxws e.ol1ecte.d

by YiiteP and our own 8: single cases have brrn Iqorted by Anderson” Koenen,‘* Brandenberg.” Godl’r~y ant1 I’al~nrr,~~ TXYX~~ I5 Nicholson,l” 7 Mar,“” Sprague and Wliit:e,“4 iIll< WOOtl Zltld ltO~l?W.2s Rccentl>

Aitken’ report,rd two wsw 01’ hrr own a11tl ;\dded OllC rcp’wlrd 1,).

(‘alandlr~ mid two rcl)ortcd by Ayln;a~l.~ \vliet*c a11 (~Ie~trocwrtlio~ram has since been obtained. This with the sl)cvimen 01’ htoxonl” makes a tot,al of 58 citses t171d ~~CSP it?‘P the OllW IY’I’CIWY~ to S~ll)$C((llClltl~ ;IS CollWtd

cases.

TABLE 11

SLOWEST VE,NTRIC~:IAR JLYTE IN COS(:KNITAL ((ON I’I,~:‘~Ic HEAKT-BLOCK --__~-- ~

Ventricular rate, -__.. -

1 20-09 j ysl 1 “I-~‘jY;” 1 5$54 j :( ( __--

4 55.ri!, 60 and river Number of cases 2 I _._ _-__ ~.~_..~._~~

The rate may be much faster th;~n this; most of olltr patients at times had rates of over 50 when at rest. A ventricular rntcl of 75 was recorded

in an infant (Lead I Fig. 1) ; and a rate o[ 61 WIS recorded in Case 2 when the patient was twelve years old. Fnstrr rates were noted after exercise. Although this is such a contrast. to the rates found in elderly patients with degenerative chanp~ and coml)lctc heart-block, it is not

confined to the congenital group, for sometimes rates much faster than these have hecbn observed ill complete heart-block during diphtheria.

The aurirular rate was also rather I’M, averaging 107 : in four patients it was never noted under 100: but possibly it would have been if they

hat1 rested longer before the electrocardioXrnms were taken.

7%~ Blood Pw.ssrr~c.-111 the three elder pat.i,nts this was higher than

mipht be espcctrd-l(iO/iiO~ 1 W/90. ant1 1 SO/!)0 at ages nineteen, t.wenty- ,swen, and twent>--one, rcsl)ectivclj- (( ‘nsc~s 2? ,5. and 7‘1. In the re- mainder the figures wrre normal (Table I). There was no renal disease

and no cridence of any arterial change. alld I~rohitbl>~ the rise is a IV- action to maintain a normal pressure during the prolonged diastolic period.

THE EFFECT OF EXERCISE OS THE HEART RATE

The ventricular rate generally returns to its normal rather quickly iLfteY exercise. Tt is therefore ?ilSy to miss the maximum rise, which maJ- be quite high iI the rate is taken for a. short periodl such as fifteen seconds. The results in four patients arc shown in Table III. Except, in Case 2 the fall was so rapid that the rise for a whole minute was not much, though the increase during the first fifteen seconds was con- siderable. all rising to 60 or over. Aitkenl mentions rates of 57 and 58 in her two cases, and Fleming and StexTenson11 also n&cd a rise from 49 to 68 after cscrcise.

In Lease 3 this was cvnfirmed b- taking an electrocardiogram as soon ;IS 1)ossible, and then, though a rather longer interval elapsed while the l)atiellt was I yin: down and the instrument, being adjusted, a ventricular ?'illC of Iiti nils rec~rtled, where it had been 16 just before. On this occ&oll the ;ll~ric~~la7* rate could also 1~ measured, and it was 97 instead of his usual 60 t(J 68 ; i.cu.. the auricular rate had increased just as much ;IS (Ilie would expect after esercise in a normal person, and the ventricu- lar rate had increased, thorlzh not lo the same extent. It. seems most likely that. t.he chemical chanp~ in the blood. especially the increased C-‘i), COlltCllt, iI1'E respoiisil)lc l’Ol’ tllc illPW;lSic~ of rate.

318 THE AMERICAN HEART .JC)UR~..\I.

In ordinary cases of heart-block the rise in ventricular rate with exercise is less than this, but, occasionally- it; is even more. In one of UUI patients in whom the rate at rest was gt~nerally 42 to 50, it rose to 70 after exercise.

Lil jestrand and Zander’” have investigated one case very thoroughly, and their work is useful in understanding the circulation in heart-block. He was a young man of twent.y who was able to take part in strenuous

games. and to swim “for as long as he liked.” When three years old hc had pneumonia and was found to have an irregular pulse. After that, he was liable to Fainting att,acks, and when five years old, he was sent to the hospital where he was found to have a vent,ricular rate of 45 to 50

and complete heart-block. He had steadily improved from then on and hi1d lost any tendency to i’ainting attacks. It is possible that the block was congenital and not due to his pneumonia. llis vent,riculwr rate increased up to over 100 with strenuous cscrcisc.

The minute rolume 01’ his circulation was about normal, but on one occasion when the rate \vils 37 the out,put per beat was 128 C.C. (i.e., minute volume A.5 litersj compared with a normal when the rate was ‘70 with an output per beat ol’ 70 C.C. ( i.e., minutts volume 4.9 lit,ers). With violent exercise the minute volume was iilcreasc(l to 16.4 liters (ventricu- lar rat,c 94 with an output per beat ol’ 174 c.c.) : a control taking the satnc exercise increased his minute volume to 19.8 liters (ventricular rate 140 wit,11 an output per beat of 140 c.c.). Lundsgaardl~ also found the output, per beat raised t,o ;Ibout 150 V.C. in complete heart-blork. This increased output per beat is associated with the forcible beat of the rcntricle to be seen when many of these l)at,ients are s-rayed. Tt es-

plains why they have no undue dysl,nea with moderate exertion, in spite of their slow heart rate.

THE EFFECT OP .~TROI’INJ~: ON THE HEART RATE

Tn two of our cases observations were made after injection of atropinc. In Case 3 l/l50 gr. did not, increase the aurieular rate above 70 (t,hirty five minut,es) or the ventricular above 49 (forty minutes), 60 and 45 being the rates at rest that, morning for the auricle and ventricle, re- spectively. In Case 7 l/150 gr. raised the auricular rate to 120 from 77 and the ventricular rate t.o 56 from 14 after forty minutes.

Aitkenl has collected 12 cases in which the effect of atropine was observed. Auriculoventricular dissociation was never restored, and she rightly adduces this as showing that, the vagus is not the cause of the block. Both auricular and ventricular rates were considerably increased, in four, but in two the auricle wa.s faster and the ventricle was unchanged. Atropine was without effect on either, in the remaining six; so possi- bly the dose was insufficient. In Leech’s easel” the ventricle increased from 47 to 79 in twenty minutes, but generally the greatest effect was

CAMPBELL AND SUZMAN : CONGENITAl, COMPLETE HEART-BLOCK 319

noted in about thirty-five to forty minutes-94 from 62, and 83 from 55 in Aitken’s two cases, and 78 from 56 in one of Fleming’s cases.ll

TIIE EFFECT OF FAINTNESS ON THE HEART RATE

The main complaint for which one patient (Case 7) came to the hospital was faintness, and we were fortunate in observing this twice. We had already formed the opinion that the faints were of vagal origin

Fig. 15.-ElectrocarJioera~n. Case 7, just after the onset of faintness, forty min- utes after atropine had been given. I,ead I: ventricle 53, nuricle 107 : Lead II: ven- tricle 52, auricle 92; Lend III: ventrirlr 50, mn+le 99.

-- __“_.L>_ * . . _ 1 .-. .-- _______ .

-_.-.._ .._.. -- . .-.. ._. . ..---L-f-.--L--L.

Fig. 16.-Electrocardiogram. Case 7, taken three minutes after Fig. 15, during an attack of faintness. Lead II only: ventricle 56 to 51, auricle 112 to 109.

and not Stokes-Adams attacks, from the general description, from their recent onset, and from the effect of emotion. This view was confirmed by their disappearance with treatment and by her return to work.

On one occasion atropine had been injected forty minutes before, and no doubt she was tired with repeated esaminat.ions and eleetrocardio- grams. dust before Fig. 15 was taken, she complained of “a funny feeling in the stomach. ” Three minutes later while still sitting in the chair she “ fainted. ” She was pale and there was no cyanosis. She was

Iiot uncons(3ious. HC~I’ ~)~LSC IXte \Yi\S (i0. illId 110 I)eriod of il slow pulse was noted, al though she was undc~* almost tout inuous obwr~a- tion. This is in contrast to the oydinaq- faint whcr~ the weak ral)icl pulse comes later, and W~CJY at the lxginnin~ the pulse is slow am1 sometimes not quite regular. Fig. 16 \vas taken (luriiig thr ;ittiipk which lasted about five minutes. Ill ten JJlilllliCT Sllf? I'CII \vCll ;\lld \V;lS ill)lC’ IO walk home. The other ol~scrvetl attack W:IY v(lr!- similar.

111 most of the reported casC3 there IlilS lKYl1 SOllJf? dC?gJYX Of cardiac enlargement, but generally it has bccl~ slight 01’ moderate. as was true in our S cases. It is ofteu easy to sa!- that, ;I patient has congenital heart disease but more difficult to tlwidc esnvtlyi what the coilgenital abnormality is. None of our 8 uses had obvious cyanosis or clubbed fingers or the complete pictuw of mo~*lms c~;~c~~ulcus. .I11 Ilad signs which suggested that there was a l)atcnt inte~~\r~lt~i~ul~~~~ septtnn. init one patient, probably had some degree of’ pulmona~~~ stenosis.

This agrees with most other reports 01’ collgwital heart-block. Of OLIV

8 and the 45 collected cases F (15 per cciit ) ha11 obvious q-anosis at rest. generally with clubbed fingers ; in thcsc thrhrc ~21s gross congenital heart disea,se, most often pulmonary stenosis with other iWSo?iated lesions.

Apart from this 15 per- cent, anothw 35 (67 [NV rent) had signs c)C a patent interventricular seljtum. 111 tllc remainder the sigus serf too slight to make a detinitc diagiiosis, hut thcw \VCIT 1m1~- thrw or I'OUY in

which the authors Pound no signs to suggest structural c.ougenital tliseasc 01’ slight, degree. Among this group with l~tcnt illt~~~vc~~tl~icnl~~r sr~ptum there were 13 (35 per cent) with sonic clcgree of cyanosis, though often it was only slight or only present on cswtion. There mere also the 8 with more obvious cyanosis at, rest. hut this still leaves more than half (60 per ceut) in whom no cyanosis Jvils observed.

We have found published rel)orts of crirly thrcc~ P;IS:CS aud add a fourth of a specimen, so far as WC know not ln~blish~tl 1)reviously.

I. MOXOIL’S case (Guy’s Hoslutal museum. Spxirnen 23 L7.J .-A boy, aged two years, with extreme cyanosis and dyspnea, was admitted uiider 1~1~. Mason in 1879. The second somr11 was greatly accentuated in the pulmonary area and the pulse rate was 25 a minute. He was appareutlp well until he was a year old, when? a Ctcr haviug whooping cough, hc began to bc cyanosctl. This became much worse seven months hefori: his admission. He diecl suddenly after he had been in the hospital a month.

The pulmomrr?~ zrtrr~- \vas c-ornph~tc~ly occluclctl at its origin and was only a. small tube up to the entrance of the patent, ductus arteriosus, where it became of normal size. There was a large opening in the upper

I>ill’t ol’ the inte~rrllt~icula~ se~~tuln, illld the right ventricle was greatly

hpl)ertr~ophied, the \yilll being thiekrr than that of the left. Except for the condition of the heart thr other viscera were normal.

The slow pnlsc and the cyanosis were not noted until after his whoop- ing rough, but in view of the condition of the heart it is certain that. the

lesion ~-as congenital a~Jc1 therefore it is probable that the slow pulse was ;IIso congenital, the illness having made his condition much more noticrahle. Only one other VBSE has been recorded with such a slow pulse J*atc> ( D ‘Espine illld ( ‘ottin”’ 1. but it is difficult to think of anp

diagnosis but coml)letc heart -bloc~k. The (>ar*liest generally accepted case

is that of van den Hcllval ill 190X.”

II. JVilson and Grant ‘s ( 'ilSC."-A cliil(l of’ fourteen months wi-ith inconil~letc heart-block. Il:s;\millatioll showed complet,e stenosis of the pulmonary art cry with il Iayy l’atcnt tluctus arteriosus and a common ventricle with only the rudiment of an interventricular septum in the form 01’ a roundccl muscular prominence on the posterior wall. His- tologically, the a~~riculovcntr~~~~~l~~~~ node was well developed, and situ- atcd in the intcrauricular scl)tnm and the central fibrous body. Rut the fibers coming i’rom it were intrrrnl~trtl and broken up bp the fibrous tissue of the crlltl*ill fibrolls body. The fibers ultimately reunited, how- ever, and diritled noimally into right ant1 left branches.

IIT. Pcrotti’s WSC.“‘--r\ c4lil.d three clays old, in whom examination showed complete iIl)sen(Je oi’ the membranous portion 01’ the interven- t ricnlar septum. Histologic rsnniination was not made.

Il7. Yater ‘s cilsc.‘“--,\ child a few days old, in whom examination showed transposition 01’ all thr> \-isccra and great vessels, except the ventricles of thr Ileill’t. The ductns arteriosus was patent, and there W’ilS a pateJJt fOYillllC’J1 orale and a small patenc,v in the interventricular septum. The sine-auricular node was found in the wall of the auricle one the left side (structurally this ~orrcspondcd with the right auricle). The nnriculoventric.~ll~~l~ node lil>T against the right side of the central fibron~ botl>- in the auri~lr of the right side. The node was separated into t\vo l)arts 117 til)rons tissw fmnl the celltral fib~ons body, thus scl);lritti?tg the 11-T’ ~lodc from tljc hmrclle ol’ His.

As few of the patieilts tlescrihed have been over twenty years of age and as none scrms to have beeJ1 older than our patient (Case 5), it might seem thilt the outlook is good for a time but t,hat few survive long in adult life. Probably this is incorrect and when patients have heen followed for a longer time they may be found in good health at a more advanced age. They ~nay not have been reported at older ages because the possibility of the l~lodr being congenital has not heen con- sidered.

322 THE AMERICAN HEART JOURNAL,

In some cases, especially in patients who have died in the first few weeks of life, the associated malformation of the heart and not the

heart-block was the cause of death. Stokes-Adams attacks increase the risk of sudden death, but they are found in a small minority only and the risk is less than might be expected.

Nine of the 53 patients are known to be dead. Four died in early

infancy, probably because of the associated congenital malformations. Our patient (Case 6) died of infective endocarditis, an added complica- tion of his congenital heart! and the block was probably without sig- nificance. The remaining four died suddenly. The first, whose sister also

has congenital heart-block, had a patent interventricular septum and

occasional attacks of cyanosis ; at three months old the pulse became

irregular and she died in one of these attacks.” The second died sud-

denly at two years, having had gross cyanosis and dyspnea for some time.18 The third had extreme cyanosis and pulmonary stenosis and died suddenly at the age of nine years.z3 All these three had serious congenital heart disease as well as block anal are mentioned here onl! hecausc of the suddenness of their deaths. Were it not for the next case, one might feel that the prognosis \vas excellent as regards the heart-block and need be considered only as regards the associated mal- formation. The fourth had no other signs of heart disease and no symp- toms, and was accustomed to bathing and strenuous exercise, but she died suddenly when thirteen years old just as she was going into the water.2 In her, at any rate, a fatal Stokes-Adams attack seems the likely explanation, but curiously enough none of these four gave >I his- tory of syncopal attacks, though these were present in 8 other patients, all, so far as we know, still alive.

CONCLUSIONS

Congenital heart-block is probably more common than has been thought, and this possibility should be remembered when treating chil- dren with a slow pulse. The block is more often complete t,han partial.

The ventricular rate is usually between 42 and 56 and quickens to 60 or faster with exercise. Stokes-Adams attacks are not common but occur in about one-eight,h of the patients, generally in the first few years of life.

As a rule, there is some degree of patency of the interventricular septum, and the heart is a little enlarged, often to both sides, with marked pulsation and some prominence of the pulmonary arc. More serious abnormalities and typical morbus caeruleus are less common.

Provided that the associated malformation is not in itself serious, the outlook is good. Sudden death is a rare catastrophe. Usually, the subject is able to lead an active life with little or no dyspnea, and survives to adult life or much longer.

CAMPBELL AKD SrZ>IAN : (CONGENITAL COMPLETE HEART-BLOCK :j%

REFERENCES

1. Aitken. J. K.: Lancet 2: 1375. 193%. 2. Aldrich, C. A.: Tr. Chicago ‘Pediat. Rot. June 6, 1927. 3. Alstead, 8.: Quart. J. Med. (New Series) 1: 277 1932. -L. Anderson, G. H.: Northwest Mod. 28: 227, 19&J. 5. Aylward, R. D.: Brit M J 1’ 943 19”s d . 6. Brandenberg, K. : Med. klin. ‘38: i464, 1929. 7. Clalandre. Lyi Arch. cardiol. v hemat. 2: 225. 1921

’ 8. Campbell, M.: Lancet 2: 180, 1931. 9. Chamberlain, E. S., and Alstead, 8.: Lancet 1: 970, 1931.

IO. 1) ‘Espine, A., and Cottin, E.: Rev. med. de la Buisse Born. 35: 516, 1915. 11. Fleming, G. B., and Stevenson, M. M.: Arch. Dis. Child. 3: 221, 1928. 1% Godfrev, I-. W., and Palmer, B. S.: New England J. Med. 207: 575, 19RL’. 1:X. Jones, k. D., and White, E. D.: AM. HEART J. 3: 190, 1947. 14. Koenen, H. P. J.: Nederl. tijdschr. v. geneesk. 49: 61’16, 1930. 15. Ileech. C. B.: Am. J. Dis. Child. 39: 131. 1930. 16. Liljestrand, A., and Zander, E.: Aeta med. Bcandinar. 66: 501, 1927. Ii. Lundsgaard, I’.: Deutsch. Arch. f. klin. Med. 120: 481, 1916. 1% Moxon~ W.: Guy’s Hosp. Museum Cat., Specimen 2317, Autopsy 403, 1879. I!). Xieholson, G., Shuln~an, H. I., and Green, D. 11.: Am. .J. Dis. Child. 36: 580,

1929. 20. I’erotti, D.: Boll. d. Sot. med.-chir. di Pavia. 3: 1, 19t’R. (Quoted by Pater.) 11. Place, E. H.: IYew England J. Med. 207: 52:7, 193’. w -2. S&r, M.: ALI. HURT J. 6: 289, 1930. L’3. Shapiro. M. J.: Minnesota Med. 10: 566, 1927. 2-L Sprague, H. B., and White. P. D.: M. Clin. N. Amer. 10: 1235, 1927. 2:5. I’an den Htuval, G. (1. J.: Proefschr. aan de Ryhs Univ. 12: 142, 1908. (Quorrd

by Yater.) L’6. Wilkinson, 1~. 1). : Personal communication, 1933. ‘7. Wilson, V. A., and Grant, R. T.: Heart 12: 295 19’15-1926. 28. Wood, W. A., and Rogers, H.: California and W)est.Med. 36: X7, I 9 3 2 . 29. Yarer, W. M.: Am. J. Dis. Child. 38: 112 and 551, 1929.