Cardiac hypertrophy of unknown etiology in young adults

CARI)TAC

‘HYl’ERTROPHY OF l!NK.VOWN TN YO I:N( ; ADUI;TS

I~TIOTJOGY

N THK course of t.wo and a htllE ;VCIM, there have been observed three picture. ClinicJly, tha outstanding feature was tl conridcrable drgrcc of cardiac enlargement, fat which no cause was apparent. All died within it year after the symptomn bccamc well cstctablished. Tho lcdons in the! three heartn, a~ studied 81t nutopq-, though exhibiting many points of nimilr~ritg, wcrc not idc!nticrL The etiology remztincbd obscurv. I young men who prcsentcd un unfamiliar

(LSS L-Unit it’o. 69WO. S. R., whitc male, aged twenty-nine years, automobile mocbanic*, married, was fir& admitted to tlw hospital on ,%ptcmbcr 17, complaining of palpitation which had hccn prctwnt continuously for two and one-half monthr. His father died at thus :qc of fifty-six, of heart &war. Ho had brrn u~~usually healthy. Tonaillcrtomy wax p(~rformed at the ugc of clcv~n. He had gonorrhea nt twenty, but denied ayphilia. At the age of twcmty-four, IIC ww in Soutlt Amerieu for r short pried, while in t.lw NHVJ. 11~ 11ad becu married for aix yearn. Jliu wife nnd two ehildrcn were livieg and ltc*althy ; there had been no mieearringcs. His eonrumption of alcaohol and tobacco WIIR very moderrrtcx. For twelve yearn, be bad hewn nn automobilo mecht&*. His usuttl weight rnnged from l!lO to 210 pound8. 11~: fir& was conscious of irregularity of the heart fin years prodonely while working in South Am&ca. After a day’s labor, during which he was much axposed to sun and fumIca, IN would lie down to rvst. It wns then that kc beeaiui~ awart’ of the irregular rind rapid beating of his heart, whkh occ*urrcd in paroxysmn lasting from five to fifteen minutcr. The attacks inrrc*asrd in frequcnc*y until hc had acvcrtil cit& day. After 11icr return to NW York they IBOCIIIIIClc!ss frequent, rcu:urriuy at irregular intcrv:llw. For two and one-half months, however, tachyeardie ltad pclrt&tad trlmorrt continuourdy and he had felt weak. lie consulted several physirians without relief. He was unablcs to obtabt tbc* prnpcr amount of deep hcaamw of tbo pounding of hi8 henrt and throbbing of hia arteries. Examination showed n large, well-devclopcd man, who nppeared to be quite ill. There wne no dyqmea or cyanosis. Tllc tousila had beon woll romowd. The thyroid was not palpable. The lungs wem ehnar. The beart wan considerably oularged, dullness extonding 13 em. to the left in the fifth spnee, and 4 cm. to the right in tho fourth space. The rate WIIS variublc, at times 2”O to 160, with regular rhythm, then a few irregular beats, followed Iy :I auddcln drop to 66. The sounds *From the Medical yE;;no and Pathology

Clinic of the Presbrterirn Hosuit:&& rind the Departmcntx of of the Collc~c of Ph;vsiclans ant1 Surgeona Columbia l?ni-

Reail before the Association

1983.

of American 178

Physicians,

Washlntion,

1). C.. Mar

9.

Administration of quinidinc, 11s mouth, in modcratalp Jnrgc dcscs, did not affect either the rate or the rhythm. The USC of digitalis, iirst in full doses, am1 then in maintenance ration, slowed the rate to UI, with wcasiumrl short l~arcxysms of tachvcardia tc 1%. ‘I’hc l~lood prcssurc row to 130/70. On dischnrgc, after four iniJu7~vcd. and was hstm~~tctl to weeks in the hospital, the patient felt glcntly continue with small daily doses of digitalis. lost

He did not return to the out-patient departmc~nt fur further sight of until June 1 of the fcllowing year (right months

0JJservation later), when

and was hc came

to the clinic despcratcly ill. Lie stated that, after Icaring the hospital hc again a-cnt to South America on a job, but had tc lcncc after liw ww,ks, becnusc uf recurrence of the parovgsms of tachycardia. Hc returned to this country two weeks ago, and since that time had great difficulty in getting his breath. Hc had taken digitalis in Jictuid form, without. bcnetit. On admission, he was cold and a clammy sweat covered the skin. The rcspiraThe pulse \ras barely palpable. The Imart rate was tions were rapid and shallow. The temperature was 99.4” F. ahout 180, and the rhythm apparently regular. The blood \Vasscrmnun rcwtion was again ncgaThe blood l>ressure vvas 134/90.

The leucocyte count was 13,000, tire. A blood culture showed no growth. per cent polymorpllonuclcar~. There was no anemia. The blood urea was 41 100 c.e. Electrocardiograms shcvvcd at times aurirular tnchyenrdia, with 170 and incomplete bundle-branch block ; and at other tirncs A-V nodal with rate prrmaturc and T was 1~wvionsly

with

42

mg. per rate of rhythm

of $0 to 811, with complete hundlc-branch l,lock, and numerous ventricular bents. When the rate was slow, QRS mcnsured 0.17 swond, R was notched inverted in all Irnds. The records vvcro wry different in form from those obtained. (Fig. C, f3.)

J~vspnca persisted and was more marked during the paroxysms of tachycardia. Administration of large doses of digitalis was without allparent benefit. I-Ie began The trmperaturc spitting “11 blood, cGdcntlp as a result of pulmonary infarction. row to ~Lfl3.4~ F., and the leucoryte count to 31,0110, with 70 per cent polymorphoand hr died on June 10, six unclenrs. Orthopnen and prostration lsccame estrrmc. days after entering the hospital. So. 1001;.-The ernminntion shn~c~l edema of the crurnl

-4wtopsy

tcrnal pitting

autopsy was performed slight cyanosis of the regions and feet.

11y Dr. face and

12. M. Rousselot. nail-beds. There

Eswas

Il’lmrncTic C“cmity: The pericardial sac contained 30 C.C. of clear, straw-colored fluid. I7a siiz, the heart nplxarrd to be enormously mlarged, measuring 19 cm. in its maxunu~n trnnsrcr~c diainetcr. The right pleural cnritg contained lo0 C.C. of thin. blood-tinged fluid.

27uvf: The hrart wrighcd 740 grams. There were numerous pcricardial hemorrhagcs. A few rpicnrdinl hemorrhngrs were seen on t,hr posterior surface of the right auricle, and also on its lntcml surface ncnr the base of the superior vena cava. The right nurielt w:Is iorereil with numerous ICne, fil)rous tabs. A small tendinous ldnrlurJ mcnsuring 1 cm. in dinmetcr, was found near the base and posterior surface of thcb right ventriclr. All the c~hnmlxrs of the heart vverc great,ly dilated. The mnculi pwtinnti of the right nurirulnr appendngc were hypertrophicd ; no thrombi were prrsrnt. Thr 1caIlets of thr tricuspid valve wxc enlarged, but the cusps were not thickcncd and the chordnc tcndineae wcrc thin and dclicatp. The columnae earneac and papillary muscles in thr right rcutricle were markedly hypertrophird. l’htrc vv:rs 110 scarring of tfic wall on the right side. The pulmonary leaflets did not lwwent the same~ rchatirc incrcqse in size as did those of the triouspid valve. The wall of the lrft auricle was modcrntely hyprrtrophicd. The mitral cusps were cnlargrd and thickened. There was edema of the free margins, and near the line of ~lvsnrr of t11r :Irwtil* IlYlfict tllc~rr~ WV1’,1 :I nrm~l~rr of fims granul:ir (~xcrescenws, rowring nn 3rc:a I)..? cm. in ~liametc~r. A small :rrcLa of atllc~roma was found at the fxse of this same lwllet. Thea pnpilkrry muwlrs, wlnmnar rarn~:ac, and myocardinl wall of the left rcntriclf wre hyl~~~rtrnl~hied. The chordnc wcrc thin and delicate. Thrre was gross warring of th: lmsterior papillary muscles of the aortic leaflet of tht. niitwl valve. Thr (xavity of thr IrIft rcntricle, in addition to being dilated, rsteudcd ~~11 down to Ihe n1les, and hcrc the wall of the myoc~nrdium was quite thin, m(2suring (I..? rnr. This was in contrast to the rcmaindcr of the IX-all of the rcntril.lr, whic~h mensurwl 2.1 ~ni. in thickness. Several small thrombi filled in the recesses 1ictwrcn the coli~mn:tcr c:~PII~:I~~. Ikleath 111~ aortic1 ring, there was slight thick&ng of the se1ltal surfncc of the cndocnrdium, which at this place measured L’ 1,111,. ‘1’11~~aortic leatlcts wcl'r increased in size, but not thickened. ‘f’he f)ranch

curoa:ir~

there

There nerc right kidney. pancreas, and

were

arteries many

multiple There kidneys.

were fine

not owluded at any point. In the anterior areas of athcrowlcrosis without qalciheation.

descending

infarcts in the lungs, and a single infarct was present was chronic> passive congestion of the lungs, liver,

in the spleen,

182

THE

AMER1C.U

HEART

.JOliRS:\l,

V

LEVY

AND

ROUSSELOT

:

C.\RDI.iC

HYPERTROPHB

18’5

Autopsy No. ZZOlb5.-The autopsy, performed by Dr. TV. C. ron Glahn, was limited to examination of the heart and lungs. External inspection showed no &?mn of the feet or legs. There KX intcnsc vynnosis of the nail-beds and moderate qanosis of the lips. ~hOYUc!iO CtWify : The lwriwrdinl sac vontnincvl ouly about I.? c.c. of bloOdtinged fluid. The apcr of the hcnrt was against the lntcral chest wall in the left midnxillary line. The transi-rrse diameter of thct heart, in situ, was 1G.5 cm. Oror the base of the heart, in the region of the grcxt wsscls, wrr filnous adhesions. Heart: The heart xas greatly cnlnrgcd aud weighed 7.50 grams. The apes was formrd about crlually by the right and left vcntriclrs. The capillaries in the epicardium were ~cry prominent. On the posterior surface of the left ventricle, about 1.6 cm. below the :luricu!or~lltrit~ul:lr groove aud covering t.his groove, was a small, tendinous placluc. .4 few hc~uiorrlmgcs wcro fouud beneath the epicardium of the right auricle. The right auricle was greatly The heart mtwlc K:,S firul. enlargcxl ; its v-ails were enormously hypcrtrol~l~ied, meusuring 11 mm. in the thickest llortion. The endocardium of the: auricle was thickt,nccl in 11lnces to such an extent The tricusllid that it was no longer possilllc to sw the nuderlying nlyocardium. valve leatlets wvcre thin mid delivate. Tlw l~npilh~ry muscles and columnae carnca~ of the right rcntriclc were enururously l~yl~crtrol~l~icd, as were also the museuli pectinnti of t,lie nurivlr. The cnducnrdium of this vciitrirle was thin and transparent, rwrpt for a frw small areas nwr the alxs, wlrcw it was grcy and somewhat opa~pu’. At the apex of tlic right vcntri/lc wcrc four slightly irregular, greyish masses. surrounded by post-mortelll clot and not attach4 to the ~~11 of the ventric~lr at any point. The largest of these x-ns not more than 1 cm. in its greatrst dimcnsiou. They wcrr soft and lay bctwecn the hylwrtrophied ~olumnae carncw. It was im possible to say from the gross npprarnncc whether these wre tlirombi or rc:preThe sentcd post-morteni clot. The couus of the wntrivlc was grcntly dilated. pulmonary leaflets appeared norn~al. The left auricle vns slightly dilatccl; its wall mensurcd 4 mm. at the thickest lwrtion. In contrast to the right :iuricnlar :ippcrid:~ge, the left auricular appendage was small and the musculi prctinnti wcrc not hypwtrophicd. The mitral valve was normal in appearanw, exqt for slight marginal edrum of the posterior leaflet. Thv rhordae trndincac wc’rc thin am1 delicate. The papillary niusclcs and ~olumnae c:wneae of the left wntrivlc vcw l~ypwtropl~icd. The endoenrdium was opaq~~c and grey in color. In the lower half of t,he septum the endocardium was greatly thickened, appearing as a yellowish grey layer. The myocardium in this area vas not softened. The aortic lenflcts were thin and delicate. In the musculature of the right auricle w?rc numerous slightly opaquf, yrllowish areas, the largest of which mrasurcd 3 by 5 mm. Tim myoc:wdinu~ was of coarse texture. In the left ventricle, there was an occasional smull gwy urca: pvt ereryvhere throughout the heart the knife, as it cut, enconnterccl unusual resistnncc, making an audible creaking noise. In the interventricular sel~tum, beneath the area where the endocardium was thickened, the muscle had bccu rcplaccd 11y dcnsc connective tissue, and nrar the margins of this connwtivc tissue the muscle alqxared slightly sunken and dark red, in contrast to the mow grcyish red muwlc clsewhcre. The alteration in the interrcntricwlar septum was wry cstcnsive aud in one of the prominent r~oluninae c:rrneae, close to the postPiior m:wgin of tl,e septum, wre cneouritertd ureas which were opaque, ycllo\c, and homogeneous. These, wrc 1)ordered by a narrow zone of hemorrhage. The scarring in the septum was quite marked, and large nrcas of fibrosis were seen, which obriously had follow4 previous patches of necrosis. The coronary point of origin, and its lumcu

~2s

artcries were rcmarknblg thin and &lieatc. the anterior cl.rsccuding branch of the left filled for n ~li-t;wcc of 1 ..m. by :L grcyish

About 1 cm. below its coronarymas distended, red mass, which slipped

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f .c.:

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790

THE

AiVIERICAN

tIIC.\RT

.JC)(XSII,

LEVY

ASD

ROUSSELO’T

:

C.\RDIhC

HTPERTROPHY

193

Iu the absence of such knowlr~lgc and of the etiology, it would serve no useful purpose at this time to attempt to clefhc diagnostic vritrriil. It is hoped that further observations will throw i1cltlcd light on this unusual form of cardiac c~rlln~~~~mc~~~. prOCC!SS.

Three cases have been described prcwnt ing an uiiCamiliar c4inical picI urt!. The main features wow avt2tt cardiac cwlargcmeiit, ink\-entricular thrombosis with embolisms to t,he viscera, and death within a year after the symptoms wrc well cstnblishcd, There wre no signs and the blood pressure was at of valvular disease or artcriosclcrosis, the 1lOrmd level or Sligh~l)h'l(Jw it. Microscopic examination of the myoeardium showed hypertro])h,v of muscle fibws in all, infarcts in varying stages of degeneration and repair in two, and ilt one of these, a curious hydropic degeneratio~l 01’ the heart muscles ~11s with vacuolization of the sarcoplasm. The. third case showc~l only a few hemorrhages bct,wvcen the muscle bundles. No inflamnintoqc~liangcs or signs of :Irteriolar disease were obserrecl. Though clinically the patients prtsentcd many essential features in cwmmon, the lesions at autopsy w’cw: not identical. It was not possible to state whether these cases reprtwntcd a siwle condition at different phast~ of its clerc~lolmieiit or kv(‘tx’ cwiiwly Iltiwlatcd in their l~athowncsis. The etiology rcmainrd obsc~ur~~. 7

Eppinger, II. : Die tosischc Myolyse des JIerzc~ns ljei Diphtheritis, Dcutsch. med. Wchnsvhr. 29: 10.77, l!lclS ; also, Ronmll, Diffuse Hydropic DeB. : Extensive generation of the RIyo~:!rtlium. Assocktcd 1Vith a Diphtheritic Infection, Bull. Buffalo Gen. Hosp. 7: -OY, 1939. 3. Howland, J. : Idiopathic Hypertrophy of the IIeart in Young Childrq Contributions to Med. and Biol. Research Dcdkatcd to Sir William O&r, 1919; 1: 582 (P. B. Hoeber). Stoloff, I?. G.: So-Called Idiopathic Enlnrgcment of the Heart in Infancy and in Childhood, Am. J. Dis. Child. 36: 1001, 1928. Kugel, M. A., and Stoloff, E. G.: Dilatation and Hypertrophy of the Heart in Infants and in Young Children, Am. J. Dis. Child. 45: 828, 1933. Steiner, M., and Bogin, M.: Idiopathic Cardiac Enlargement Associated With Status Thymicolymphaticus, Am. J. Dis. Child. 39: lZ5, 1930. Sprague, H. B., Bland, E. I?., and Whit?, P. I).: Congenital Idiopnt,hic Hypertrophy of the Heart: A Case With Unasunl l!lmnilg IIistory, Am. .I. Dis. Child. 41: 877, lY31. i. Lanhr~v, c., an11 \1~alsvr, J.: Sur 1111(‘a,; 11‘Insuflis:~nw (‘a1~lliaqov P’rilnitive: LIPS Nyorardies, Bull. et mem. tltx 1:~ SIX-. drs IQ. dts l’nris 49: 409, lYz5. k. \Talser, .J. : La JI,vllc,arrlie: Syn~iv.i,nl~~ II ‘Insuttis:in:.,~ (‘:tuli:lc(ne I’rirrlitiv(s, l!)L’.i, Paris, Librniric Octnre I)oin. !I. Wllittl~‘, C. II. : I‘ ldiollatlliv” !I,Y~)~‘rll~0l1lly of 11it~ IIt~:irl in :I Yc~ung Man, Lancet 216: 1351, 1929. 7.