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MKROCOPY ■UOUITION TBT CHART 
 
 (ANSI and ISO TEST CHART No. 3) 
 
 1.0 ^*^ 1^ 
 
 .s TIPPLED IM/1GE 
 
ffif-- yr,£. 
 
 STATISTICS OF CONGENITAL CARDIAC 
 DISEASE 
 
 % 
 
 1400 OASES ANALYZED) 
 
 MAUDE E AHUOTT, H.A„ MI). 
 
 Tub Jouknal of Mbuical Hi 
 
 IbBAHCK, VOLUMB XIX,, No. 1 
 
 (N«w Serioa, VM. XIV., No, i ). pp, 7:-Si. Julv, igo» 
 
 ■'I' 
 
 I 
 
 I 
 
 BOSTON 
 
 MASSACHUSETTS 
 
 U,S.A. 
 
 
 I 
 
 '5 
 
 » 
 
 '•*'*'»~*»«»«MPM«»»«XH' «f 
 
ob 
 
Ill iiiNIIEMTAr I\R11TM li|>t;,\SK. 
 
 Thf subject of congenital cardiac disease is one that lends 
 iUelf well to statistical study, for (he conditions, being often 
 complex and of recognized rarity, are usually rep irted in 
 nnicli detail. Moreover, the cases are so infrequent in any 
 one person's e.vpcrience tliat some micIi method as this, of 
 makini; use of the availahle literature, must be ,id<i|ited in 
 order to arrive at any generalizations. 
 
 Tor another purpose. 1 have had occasion to make a detailed 
 statistical study of some four hundred and twelve cardiac 
 defects, A few of these are drawn from persou.il experience, 
 the remainder from the literature. ( Inly well-authenticated 
 cases with post-mortem report attaclied have been included. 
 The only exception to this statement is formed by three cases 
 included In the series of patent ductus arteriosus diagnosed 
 by characteristic physical signs and by the X-Rays and not 
 confirmed by post-mortem. The results of the analysis of 
 four hundred of these cases are shown in the accompanying 
 chart. This chart is presented here merely as a demonstra- 
 tion of the manner in which these defects were studied, and 
 without any intention of entering at length into the ligures. 
 It represents a chart which was originally printed for the 
 analysis of the individual defect, .i.id is here modified in a few 
 particulars to admit of the presentation of the total results 
 obtained. 
 
 The chart presents four main divisions. The First Division 
 includes the Classification of the defect. Number of cases 
 analyzed, Age. and Sex. In the classification a simple 
 anatomical order h.is been followed, based also on the 
 
 ■ Fresenicl l.efore ihc Assod-ii'nn oJ i'Lithologlsts and Iticlerioiagisis ai Ann .\rl>or 
 Micli., April 18. i.»8. Ke,-,.iv<-.l for publk-.-iiion May I. igo8. 
 
jA \nii'tn 
 
 pri liplc", of the develnpmcn* of :he heart, io f-.r as thcfc are 
 known Thus, defects of the cardiac an-' aortic septa are 
 followed by transpusition of the arterial trunks, due (accord- 
 ing to Kukitaii^ky) to a deviation of t'..e aortic septum ; and 
 this a^iain by pulmonary and aortic stenosis or atresia (some 
 cases of which arc probably likewise due t > a deviation of the 
 aortic septum). The cahos of pulmonary and aortic stenosis 
 or atresia arc sub-classified (foUowinii Rauchfuss) according 
 to the presence or absence of defects of the intcrauricular and 
 interventricular septa, and this affords a clinical grouping of 
 much value, hv coarctatiMU of the aorta the distinction 
 drawn by Hountt ' is observed between the infanliL- form, a 
 simple persistence of the isthmus aortii-, and t'lC typical 
 '• a«tult t;je" of coarctation in which the aorta is obstructed 
 or even oblitir.»ted by a sharp constriction at or above the 
 insertion of tlif tluctus. 
 
 Amunv; the defeat-* enumeratcti in this classification those 
 of clinical importance are: 
 
 Defects of the interaurlcular septum, 2S cases; defects of 
 t:.e interventricular septum, 40 cases; cuniplete defects of 
 the cardiac septa (biloculate heart, etc.), \2 cases; defects 
 of the aortic septum, 14 cases; transposition of the arterial 
 trunks, 44 cases; pulmonary stenosis, 75 cases; pulmonary 
 atresia. 23 cases; tricuspid stenosis, 2 cases; tricuspid 
 atresia, <j cases; patent ductus arteriosus, 2^ cases ; coarcta- 
 tion of the aorta, }} cases; hypoplasia of the aorta. 2 cases. 
 iJndcr -Nge " are three columns in which the maximum, 
 minimum, and mean a^jes of the cases in each grcup are cal- 
 culated. 
 
 The Seconj Divisii.n the chart includes tht-"" Post- 
 
 mortem Findings of especial importance in cardiac defects. 
 Here are noted the condition of the fetal passages, whether 
 closed or patent, the pre-ence of hypoplasia or dilatation of 
 the pulmonary artery or the aorta, the existence of a col- 
 lateral ci^-culation (important in coarctation of 'he aorta), 
 the incid^'nce of arterial disease, of acute endocarditis, and of 
 
 • Honiift. R.-vm: dc Mi'^ilicini-, 1903. 
 
slAIIMh 
 
 i>t 
 
 'MJKMIAI < \Uhl\' hl^l.A-i . 
 
 79 
 
 chronic valvu'.ir ilisc.isc, tlu- prcseiut' of hyp -rtropliy aiu! 
 (lil.ilation of Uu' •litfcrciu cli.inihcrt nt t)u- hort, aiiil \d%i\y 
 the cxistcnci; uf n-fsucialctl anoiitahcs in tlie heart, vesscN, or 
 cKcwhcri.', 
 
 The Ihinl 1 visi in potc; points of cUnic.i! interest, Mich as 
 the prt'sciicc of "(iiuntions having; .in <-tiol";;ical luMriny on 
 ihc f.ituily iiistor)'. iml in the p'Ttuinl liistttry tlie iiici- 
 lience of ihcum.itism, puhiiunary tiilierciilosis. or congenita! 
 syphilis, am! tlie pmportion of l '.cs recu/cnnt; from the 
 acute infectious fevers (which cyan >tic patients are sail! to 
 pass throut;h *el!), L'culer special symptoms arc cr)lunins 
 for cyanosis in its ditVerciit (let,'rees, clubbing of the finj^ern, 
 tlyspnea, djspncic attacks and delayed development. I'hysical 
 sitjns may be vascular or cardiac, .iml aniun^,' the tatter the 
 occurrence ))f visible [luK.ition. precordial bulj^iiij;, llirill. 
 increased dulness, accentuation ol the heart sounds, ,inJ 
 the existence of murmurs, presystolic, systolic, diastolic. 
 Continuous, double (i.e., systolic and disastcdic in rhythm), 
 or indefinitely stated, arc noted. I-'inally. under causes of 
 death we find the defect itself provinj* fatal sudtlenly or by 
 failing com|)ensation, or a termination by bronchn-pncumonia, 
 cerebral complications or the acute infectious fevers. 
 
 The Fourth Division of the chart, that of Relative Fre- 
 i|ucncy, 3 of the j;reati\st importance. Cardiac anomalies 
 are so of:en complicated that the number of times a {^iven 
 defect occurs alone or as the primary condition by no means 
 represents its total incidcur-; in the four hundrei! cases. In 
 this division there are, therefore, ihree columns. In the first 
 of these stands " the number of cases classified as the pri- 
 mary lesion," the figures of wh'ch are identical wiih those at 
 the beyinninti of the chart showinj^ '■ the number of cases 
 analyzed" in each group. The sum of the figures in this 
 column is the four hundred cases analyzed. In the next 
 column stands the number of cases in each group in which 
 the defer occurs complicating; other conditions, and this 
 with the number of cases classified as the primary lesion 
 
So 
 
 l[ivi-<t the tuul inchUnci- of the itcrcct 
 ill the liiit column of the chart. 
 
 which is ihi.i nhovvn 
 
 The result of thi-t .tiiiilvsi** brinn* out some rirmarkiihlc 
 f-ictn, ncvcriil of which art at v;ir..»nce with .iccL-pUil iiii .i-^. 
 The fnlluwinvi (»»tnt-t arc ..f rspccial inti-rt-Ht : 
 
 1 . The frei|iiLncy uf tk-ft-cts of thi- intcrvt utricular 
 septum. — While rL-lativcly rare altujc ( lliirty-twu difictn at 
 the base amoiiK the four huiulrcd), in combinati>>u witli 
 iithcr comlition'^ thjs is seen (o be the moil common of nil 
 cardiac anomalies {<tne hundrcil and fiTty nine annmt; the 
 four hundred cases) ; next in frc(iu<-ncy comes patent fora- 
 men ovale, under which arc includeil only cases of true 
 patency, nut simply a valvular or slit-like comlition, with one 
 hundred and thirty four cases, and then patent ductus arte- 
 riosus with one hundred and six. The frequency of trans- 
 position of the arterial trunks (forty-six cases) and of 
 pulmonary stenosis with defect of the interventricular sep- 
 tum (seventy-three cases) is noteworthy, while pulmonary 
 stenosis with closed interventricular septum is relatively 
 infrequent (seventeen cases). 
 
 2. The duration of life is seen to be relatively lont; in 
 uncomplicated defects of the interauricut.ir septum, patent 
 ductus arteriosus, coarctation of the aorta, and pulmonary 
 stenosis witli closed interventricular septum. In pulmonary 
 stenosis with defect of this septum the iluration of life is 
 seen to be much shorter. 
 
 3. I'atcncy of the ductus arteriosus is seen to be rare in 
 pulmonary stenosis, though very frequent in [lulmonary 
 atresia. 
 
 4. The riiiht chambers chief!)- are hypertrophied and 
 dilated in defects of the inierauricular septum, transposition 
 of the arterial trunks, pulmonary stenosis and atresia. Both 
 chambers, but chiefly the rijjht. arc enlar^jed in defects of 
 the interventricular septum and patent ductus arteriosus, the 
 left ventricle chiefly in coarctation of the aorta. 
 
 3. Acutt endocarditis is seen to be relatively common in 
 
if 
 
ClASSIKI CATION 
 
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^lATISTlCS ()!■ 
 
 lONCENlTM, CAKl.lAC IIISKASE. 
 
 8l 
 
 6. Cyanosis was absent i„ most of the defects of the 
 
 "'", A tnoderate degree of eyanosis was fairly common 
 ■n defects of the interventricular septum, a mar Jd ." 
 only three cases. Marked cyanosis was seen chieHy n'tran 
 pos,^„ o the arterial trunks, pulmonary stenosis Jit ; . 
 of he ,merve„tr,cular septum, pulmonary and tricuspid 
 atresia. Cyanosis w.-,s usually slight or ab.sent in patent 
 
 uc us artenosus and in coarctation of the aorta of the' it 
 t,|.e. I, sx cases of defect of the interauricular and in four 
 
 • terminal • ■"'"-"'-">- -P<-.. .1- cyanosis w 
 
 terminal appear.ni; only in the last few weeks of life 
 
 veninV , ""•' ^"■■''""" '■" "^'"'" ''••■'■"^'^ "f the inter- 
 
 ventricular septum at the base, and in pulmonary ste„o"s 
 w.. . closed ,nterve„tricular septum, or with defect of'^tl e i^e 
 ven tncular septum and patent foramen ovale. A I was 
 rela ,vely rare in pulmonary stenosis with defect of t , n er 
 ventr.cular septum and closed foramen ovale 
 
 8 In the great majority of cardiac defects the murmur 
 when present, was systolic in rhythm. 
 
 9. In some cases of pulmonary stenosis the pulmonary 
 second sound was accentuated. mionary 
 
 These are not all the conclusions to be drawn from a study 
 of .h,s analytical table; they are sufficient, however, to show 
 the value of a careful and detailed tabulation of the dati 
 Z[ by different observers in arriving at genera dedut 
 ..ons, such as could not legitimately be drawn fron, the facts 
 ■n the experience of any single worker.