Pseudocoarctation of the Aorta: A Variant or an Entity?

Pseudocoarctation of the Aorta: A Variant or an Entity?

Pseudocoarctation of the Aorta: A Variant or an Entity? T. Z. Laio!)..\I.D.. F.C.C.P.;O C. v. .\ Ieckstroth...\I.D.. F.C.C.P.;oo K. P. Klasseu ..\I.D...

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Pseudocoarctation of the Aorta: A Variant or an Entity?

T. Z. Laio!)..\I.D.. F.C.C.P.;O C. v. .\ Ieckstroth...\I.D.. F.C.C.P.;oo K. P. Klasseu ..\I.D.. F.C.C.P.;t ami x.]. Shermau ..\I.D.t

Four patients with pseudocoarctation of the aorta are presented. Two patients had congestive heart failure due to associated aortic stenosis and insufficienc~·. Cardiac catheterization and angiograph)' confirmed the diagnosis and depicted other associated congenital anomalies. One patient had an aortic vah'e replacement but died of complications arising from pseudocoarctation. The pseudocoarctation was resected in the second patient on partial cardiopulmonary' bypass and no further aortic "alvular surge!")' was necessary. Two patients were asymptomatic. had retrograde aortography and required no further treatment. Based on our clinical and pathologic material. pseudocoarctation seems to be a variant of coarctation. The incidence of associated congenital defects is frequent and ~imilar to coarctation of the aorta.

elinieal eases of pseudoeoarctation of the aorta ali(I the relation of pseudocoarctation to coarctation as supported hy our own pathologic material ali(I laboratory studies.

pseudocoarctation of the aorta was first descrihed hy Dotter and Steinlwrg I and Souders and coworkt'rs:! in IH.~l. wlwn t}wy diagnosed an up}Jt'r nlt'diastinal mass callsed hy dongation of the aortic arch and "kinking" of the aorta at the ductus le\"('1. The laek of a pn'ssure gradient across the "huck led" or "kinked" segnwnt and ahsence of coIlalt'ral circulation hecame the essential criteria to diagnose pseudocoarctation of the aorta. This anomaly is descril)('d only in ahout 80 cases in the literature. DiscO\'ery of frequently associated congenital ahnormalities:: and even coarctation of the aorta -t added to the misunderstanding of this ddeet. Suhelinical coaretation.;; atypieal eoaretation'i and "mild" coarctation j without collatt·ral circulation or with minimal gradit'nt \\"ere lesions primarily l11('ntiOlH'd as variants of coarctation. Heports somehow fail to outline the distinct relationship hetween pseudocoarctation and "classical" coarctation. -t. s It is tlH' purpose of this paper to diseuss four

\I.-\TEHIAL

Our first two patients ht'calllt' symptomatic IweallS(' of tht· ;L'is(K."iated cOIIl,!t'llital aortic \Oaklliar d,·ft'd.

C."SE 1

.\ 27-year-old mall W;L'i admitted to our hospital in \ ~J()4. with tilt' chid complaint of dw.. t pain. .\ Illllrlllllr was nokd at ,1I.!l' 14. First symptoms of pseudocoarctation occurred at al,!p 21. with the onst't of pre<:ordial chest pain with radiation down the Idt anll, t'pisodt's of \,prtlgO and shortnt'''s of hreath Oil walkinl,! ont' f1il,!ht of stairs. Dil,!italizatioll prolllptt-ti an illllllt'(liatt- remis .. ioll of sylllptollls and a \.'5-pollnd wt'il.!ht loss. His fathN dit'(l at al.!t' 41. after ha\Oilll.! hatl four myocardial infardiom and his fip,t co""in had \larfan's "Ylltlronlt'° Physical t'\aminatioll rt'\"('alt'(l a yOlml,! man in mod"ratt' re.. piratory di ..trt·ss. Then' WNt· no stil,!mata of \larfan's .. yndronl{'. TIlt' blood prt'ssnrl' was 130/S0 mill Hl,!. in hoth arms and til{' pillS(' rat<· was 72/mill. TIlt' Illnl,!s WNt' dt'ar. TIIt'J"t· was markt'tl Idt \"('ntricular hea\"(' anti systolic thrill O\'N tilt· entire pn'ctmliulII and at tilt' sllprasternal notch . .\ I.!radt, \'/\'1 t'jection mllrmllr was transmittt'(l into tht' nt'ck and a blowinJ,! dia..tolic l.!ratlt· III !TI llI11rnlllr was Iwartl alone th.. lower left sternal hordt·r. The resllit-. of ht·mol.!ralll and llrinalysio" wen' normal. TIll' t'1t'(."trocardiol.!ram ..howt'(1 in-

° .\s.. i..tallt Proft·..sor of SlIn.!t·ry. The State Cni\'ersity of ~ew York at BlIffalo alld the Buffalo Ct'nl'ral Hospital. oOA ..... ot'iak Proft·....or of Suref'r\'. Ohio Statt- l'nh"'p,it\', ColulIlbllS. . . tProft·....or of SUr!.!er\,. Ohio Stat.· l'nin·rsit\'. Colulllhus. : Rt,..id(·nt of SIIr!.!t'r~·, Ohio Statt' l'lIin·rsit~·. Colllllllm..,

571

572

LAJOS ET AL

COII,pl..t<' Idl IIIII"II,·-hralld, hlod;, f"''1"''111 "'lra"',I"I,·, aile! Idl "'lIlriclllar "yp.. rtrop"~, CI,,',I ,-ra~' pidllf<' "-"e-al"e! Idl "'lItriclllar e!Olllillalll"" ane! "lorlll",ily" aile! dilalalioll "f Ih.. Ht·tnll!.radc· allrtll~raphy dC'lIloll,tralt·d a "Illild" coan:tatioll

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I.o/en traeill/! ,I"m", Ihl' 1'",lolll'r"li,'" hral'hial ar!l'ry; 1/0/1)0 Illln I"I~, and arlt',,": I:>O ..'SO Illlll Hg. Tht'fl' is ,till a lIl'n il~ across the "kink",

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On tl ... fourllt alld final adlllission on .-\n!!u,1 17. 1')6.5. a

St.,rr-I :d"';lnl.. \ ah"" was in,(·rtc·d with i!oc){1 coronary arh'ry pcrfll"ilill durill!.! tilt' thrlT-holir hYJla'~ throll.1!h tlU' ric:ht

FU:l'UF I. Shuw, thl' ori1!in of tilt, rn;tin \ t·s,.·I ... fnuu thl' aortic.: ardl. tilt' torhlflll'" pftl\illlal aorta and tht' narrowed 'l'':!IIl~nt

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ft'lI10rai arlt'ry, O\'t'r tilt' 11l"t fom Itour,. followin!! hypass. ,,"'<'f" rigl,l l"alf pain d",',·lop,-d willo ,w,·lIin~ and (Iilllini,hl'd pul,ations of the dorsali, IlI'di, and Ihl' pn,lerior tihial arll'ri",. Thi, Wa' f"lt 10 reprl',,,nl an "anlerior tihiali, COIllI'artn nl ,ynd1'll,n"." possihly ou II", hasi, of i,dwlllia dmin,.,: ll(:rftl ioll . .-\11 alltt:"rior and po'tc.'rinr fasl'iohHl1y \\'as I)(-"r(mlll,'(!. III tl ... IlIlStnpl"ratin' l"ours" I'Ylll,tl'lhion den·loped with Ill'IlHH"dnhiul1ria. jauw.lic.:l'. Jlro~rl'~~h·l' azotl'1I1ia and It,ft '"l'nIricIILtr failml". Tht' pali,'nl di,·d on tl,l' fifth po,t"pl'ralinday. in ~Jlitt· uf \·i"1!()roll~ n'~piratory ~IIPJlo.. t (respirator) and l'anliololli,' 1Ill'dication, (digitali,. i,oproll'rl'nol and '"a'oprc'S~fJr~ ). :\lItOJ1'~" ,',amillali"n ,I"',,",'d a h"art ,,"l'i,ghin~ l.O.'jO ~m. '1'1,,· It~ )ll'rtropl,it'd left "'lIlrit'lIlar wall n1l'asmt'd 2,'1 t'1I1. '1'1 ... a....tic rill!! and mitral allnlllu, ,I",w,·d l'akifkatioll. Thl' ,"ai" art,'ri,', of tht' 'IOrtit' arch aro", fnllll tht· ri~I,1 10 It,ft as f"lI"ws: ri~ht com mOil t·arotid. 1..£1 common carolid. left ,"hcla"iall arlc-rit',. Thl" ridll ," ltd a , ian arlc-ry I"ok "II la,1 alld ''''''I,d I hind II ... ('sopl,a!!II' 10 tl,l' rid.1 Ihor;,l'it· "" tId. Distal 10 tl ori~ill of IIIl' ri~hl ,"hda,ia" artt'ry. II ... aortic an'" Wa' t,lon!!at..d and hll('kll'd al II,.. ,ilc- of the alll)\'" l:ou... tri('tiu)) and

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CHEST, VOL. 58, NO.6. DECEMBER 1970

PSEUDOCOARCTATIO 11 d

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CH S , VOL. 58,

O. 6, DECEM BER 1970

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574

LAJOS ET AL

TI", n""I" 01 ph,si,'al,'\a,,,ination and tI,l' 1a1",r,lton' data W"n' within norn,al lillli", clll"t \-LlY pil'hlfl' dl'll"lI,,'tral,'d a rua.. . s ill tbl' It·ft '1l1wrilJr lJH'dia~titlllln and a torhlllll'. aorta.

Thefe wa~ til) rib not<:hillC:. On \lar"h :21. I ')(j(i. a rdrol:rad" aorto~ralll showed dilalation of tl,,' aorti" ard. and po,"tt-nllti" dilatatilll1 of tIlt' aorta hdllw th,' d"d'" 1<-",'1. TI",n' wa' a d"finitl' "kinkill!!" of the "ortk an,l, witl, all alll'I1I'\'"nal tak,'otr of thl' left ,"I",la,';an 'Irtery, TIlt' """.I "ollakral t'ir,,"latilll ",,"<:iat..d with d,,"ical l'll1H!('lIital ('naretation wa' COIJlpll·h·ly ah't"nt. Tilt' aortk ,'ak.. had tl,r,'" ""I"pdl'nt I<-aflds, Th,'re was no I!radi.'nt aert'" tl,,' "killk ..· Th., pati""t W,I' ,'o"si,I.,rt,d to han' pselldocoardation of thl' aorta, witll ,",,'m~ ""al dilatation "f tIlt' 1..1t sllhcla"ial1 arte.~~·. SllTl!t'r~ wa, lIot ilJdll'ah·d and he was Jischarl.!('d to

IIf' f"lIl1w.,c! "" all ""tpati"lIt h,tsi, 'Illd was last s<,ell a half ~'(-'ar a~().

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This ,; l-y"ar-old whi'" nlall wa, rt'kr",d to the hospital for dia!!nll,til' stlldi,'s of a Idt "Pl't'f IIll'diastinal mass fllllnd 011 rollti.ll' "I""t \-1',,,', TI", n""lts Ilf admi"inn. clinil'al and lal)oratl)ry ,hulit·... \\"('n' 1I0IlColltrihlltllf". Thl' pati.'lIt was "atl","'ri/..d awl ,,,'If lie ,mdo!!raphy d"Illolistratf'cI a (,'Olllllt'tt'llt lJic.:II"pid aortic \"... h"l'. Then' was a ... lIlall narro\\"illl! dill' til ··hlll"kliIH.:·· of tht' dt·sl·t'l)(lint! thoracic "orta I,,',on
Dls/:t'ss[o:" Pseudocoarctatioll is all uncommon. congenital allomaly of the aortic arch \\'hich elllhrnllcwicalh' allll anatolllicall~' s('\'ms to he closely 'rela::'d t~ coarctation, :\ortic lIarro,,'illg dol'S not o('cllr. hnt a dilated descending aorta. poststenotic dilatation ma~' he' Sl'ell. I'; This certainly suggests that turhu1\'lIc\' amI a small gradil'lIt acTOSS the "kink" \\'hich l11a\' ('n'n increase ,,'ith exercise is 1I0t UIIUSU,tl. \\'hile thl' main ahnormality in pseudocoarctation of till' aorta is till' elongatioll of the distal aortic arch cauSl'd hy ahnormal growth of the predudal aorta. ('oardation similarly is thl' [('suit of thl' maldew'lopnll'lIt of the dudus area. tllTlT to nine intercostal Tabl.. )-P.p,"lo#:oarr'at;tU/ oj ,hp "'orta Catl""pr;:a';oll I)t/ta (.:1......

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\'L'ssl'ls and the fourth aortic arch, The emhryologic association of pseudocoarctation to coarctation is supported by the high incidence of similar. assoc'iall'd. congenital anomalies s, I II, I!' such as hicuspid aortic \'al\-e. 1 7 aneurysm of the sinus of Yalsah'a,:!lI l'ndol'ardial fihroelastosis. I';,:! I :!,I PIc. Our clinical material also suggests that pscudocoardation of tIll' aorta is prohably a \'ariant of coarctation. since ('mhryologically they Sl'elll to ha\'e a close relationship. Two of our four p,ltients had the association of pSt'udol'oardation aJl(I memhranous coarelation with Jack of l'ollatl'ral circulation. They displayed the wl'lI-knO\\'l1 abnormalities of the suhl'ladan arterit,s in l1>arelation.:! the associated atrl'sia in caSl' L of Il'ft subclad,lII artery. and the anomalous origin of thl' right subda\'ian artery originating proximal to thl' coarelation as a last hranch of the aortil' arch (case I I, Both of these patients had a congenital hicuspid \'al\'/;' as \\'ell as did the patient in caSl' .t, Thl' other t,,'o patients were complPlely aS~'lllptolllatic. had no gradient or collaterals. Therefore. surgical interkrence "'as contraindicated \ Tahlt' I l, Thl' absl'n('e of collateral circulation. in fact. l'realt's an ahnormal hemodynamic situation in spite of "nonsigllificant" narro\\'ing or kinking of pseudocoarctation, This may also predispose to the de\'L'Iopment of the arch deformity. to kinking or to poststl'notic dilatation and to its potentially lethal complications:! l - TIIpture of the proximal or distal aortic sl'gnwnt. dissecting aneUT\'sm of the distal thoral'ic aorta. aneurysm of the ao'rtic arch. de.:!:! The abs('nc(' of aJl('ur~'smal dilatation of the

CHEST, VOL. 58, NO.6, DECEMBER 1970

575

PSEUDOCOARCTATION OF THE AORTA

intercostal arteries beyond the kink supports the view that the development of these is rather related to the increased collateral circulation. than to the poststenotic pathology. 2:~ The ductus area seems to be involved in the development of pseudocoarctation. possibly in the development of coarctation. This is supported by clinical and experimental observations. Clinically, a funnel-shaped deformity may persist at the aortic side following surgical division and suture of the patent ductus arteriosus. Experimentally, pseudocoarctation of the aorta was produced in six-week-old puppies by ligation. division of the ductus and fixation of the "aortic" stump to the vertebral hody. Blood flow measurements and retrograde angiography demonstrated the development of a kink of the aortic arch and associated turhu.lence without a gradient (Fig.5 ).:!;' The development of pseudocoarctation is likely caused by combination of turbulent flow, fixation and rotation of this area:!;' and elongation of the aortic arch. In clinical cases exact delineation of the pseudocoarctation is desirahle with angiographic studies. Gradients must he measured across the aortic valve and the area of aortic narrowing which may develop on exercise. There are borderline cases when a pseudocoarctation can hardly be diHerentiated from a mild coarctation. namely. when a narrowing is minimal and the gradient is small. Our first case demonstrates the problems which can arise preoperati\'ely, during and after aortic valve replacement; in this case a 40 mm Hg gradient across the coarctation hecame detrimental to the patient (case 1). In retrospect, we believe this ohstruction should have heen repaired prior o~ simultaneouslv with valvular surgery. . Perhaps experience with children in the treatment of "true" coarctation and aortic stenosis is our best guide to the treatment of aortic stenosis with pseudocoarctation in the adult. In a recent survey at the Children's Hospital. Columbus. Ohio. four such cases were revealed. All had the coarctation resected first; two had subsequent aortic valvotomy. two months and three \·e.us later. . . Pseudocoarctation requires no surgical treatment if no or a small gradient exists across the kink and the patient is asymptomatic. \Vhen an associated congenital aortic valvular lesion exists. however. it is this lesion which usually becomes symptomatic. in later life. and requires surgical repair. Left ventricular failure ensues aggravated hy the narrO\ving of the arch at the descending aorta which represents an increased resistance. Thus. the vicious cycle CHEST, VOL. 58, NO.6, DECEMBER 1970

becomes accelerated with fast deterioration of the patient's condition. COXCLUSIO~S

On the basis of our cases, experimental data and literature. the following conclusions are drawn: 1. Pseudocoarctation is a congenital variant of coarctation; therefore, there is a high incidence of associated congenital lesions similar to coarctation. 2. Elongated aortic arch and "kinking" of the ductal area were additional abnormalities.. :3. :\1 istaken roentgenologic diagnosis of a "mediastinal mass" rna" lead to unnecessarv thoracotomv. 4. Detailed an'giographic studies of the conditi~n is mandatory hecause of the high incidence of associated congenital abnormalities and complications, on long-term follow-up. .5. Aortic valve slugery can be fatal in the presence of a "nonsignificant" coarctation; therefore. the "kink" should he preferably dealt with at the first stage. REFEREXCES

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Dotter CT, Steinherj.! I: An1!;iocardioj.!raphy, :\'t'W York. Paul B. Hoeher, Inc., H).5!. p IHI Souders CR, Pearson C\1. .-\dams HD: An aortic deformity silllulatinj.! nwdiastinal tumor: a suhclinical form of coarctation. Dis Chest 20::n, H).51 Steinherg I, Engle \IA, Holswade CR. et al: Pseudocoarctation of the aorta associated with conj.!enital heart disease: Report of tt'n cases. :\nlt'r J Rocntj.!cn 106: 1. IH69 :\cen·do RD, Thilenius OC. \Ioulder P", et al: Kinkinj.! of the aorta ( pseudocoarctation) with coarchltiou, AJllt'r J Cardiol 21 :442. 196H Edmunds LH Jr. \IcClenathan JE, Hufnaj.!e1 C:\: Suhclinical coarctation of the aorta. Ann Surg 1.Sf): IHO, H)62 KOZllka T. :\'osaki T, Sato K. et al: Roentj.!enoloj.!ic diaj.!nosis of atypical coarctation of the aorta. Acta Hadiol 4:¥)7. lU66 Kjdll><.'rj.! SR, \ lannlll'inlt'r E, Hudllt' IT. et al: Diagnosis of conj.!l'nital heart uisease. Chicaj.!o, The Year Book Puhlisllt'rs Inc. H).58 BrtJwt'r :\J, Burchell HB: Kinkin1!; of aortic arch (pst'udocoarctation, suhclinical coarctation) . .IA\IA 162: 144.5. 1~J,56

H Jones T\\'. \'etto HR. Wintercllt'id LC. et al: ArtNial cOlllplications indirt'ct to cannulation in open Iwart surgery. Ann Surg 152:H69, 1960 10 \Volfgang 1, Chand pathak ~: Bt'richt tiher :360 oppt'rit'rte aortenisth-musstt'noscn und die Bt'j.!leitft·hler. Friih lind Sp_itkomplicationt'n. sowit' ZWt'itorx>rationen. Erj.!cbnisse (h'r Chimrgie und Orthopadie. 46: 167. 19f)4 1I Stallworth .1\1, \\·einherj.! \1, Jeffords JD: Surj.!ical corrt'ction of coarctation of tht' aorta comhined with aortic valve regurj.!itatioll. Sur1!;ery 40:.57.5, H).56 12 Rivera C, Carlhoys H. Glover RP: Simultaneous surj.!ical treahnent of co-existt'nt coarctation of tllP aorta and aortic valvular stenosis. Anll'r J Cardiol.5:.5.51. W60 1.3 Jacobson C, Coshy HS. Griffiths CC. t't al: \"alntlar

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stenosis as a cause of death in surgi<:lllly tn'ated coarctation of the aorta. Allier Heart J I :HHH. uri:) Gross, HE: Coardation of tlw aorta, surgical treatment of 100 (:a:'l's. Circulation I :41, I H:iO Smith DE, \lathews \lB: Aortic \'ah-ular stenosis with coardation of tlU' aorta. Brit Hl'art J 1i: WH, 1955 Steinberg I, Hagstrom J\\'C: Congenital aortic valvular stenosis and PSl'udocoardation ("kinking, hl\(:kling") of the arch of the aorta. nl'port of four cases indudin~ an autopsy study of onl' case with parietal endocardial fibrosis and fihroda ,tosis. Circulation 2.5:.=>4.5, 1962 Lochaya S, Kaplan B, Shaffer, AB: PSt'udocoardation of the aorta with hi~'uspid aorti<: v,llw and kinked left subclavian artery: possihle cauSt::' of subclavian steal. Amer Heart J 73:36!J, 1967 Hamilton WF, \loore J\V, Kissman J\\', t't al: Shldies on circulation. IV. Further analysis of tht' injedion method and of changes in hemodynami(:s under physiolo~ical and pathological conditions. Amer J Physiol 99:534, 1932 Shapiro II., Candiolo B\I, Neal nw, et al: Pseudocoarctation of the aorta. Arch Intern \It-d 122:345, 1968 Steinberg I: Anomalies (pst'udocoarctation) of arch of

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aorta: report of eight new and rt·view of eight previously published cases. Amer J Roentgt'n H8:73, 1962 Gay \VA Jr, Young WG Jr: Pseudocoarctation of the aorta-a reappraisal. Circulation, suppl VI 3H:80, 1968 Daughterly HK, Sangt'r P\V, Hobicsek F, t·t al: Pseudocoarctation assodated with mwurysm of til(' aortic arch. Cardio-Pulmonary Dis 11-12:7.50, wee Edwards JE, Cart·y lS, Neuefdd HN, et al: Congenital Heart Disease, Correlation of PatholoJ.(ic Anatomy and Angiocardiography. Philadelphia, W. B. Saunders Publishing Co, 1965, Vol II, p 683 Xewcombe CP, Ongley PA, Edwards JE, et al: Clinical, pathologic and hemodynamic considemtions in coarctation of the aorta associated with ventricular septal defect. Circulation 24: 13.56, 1961 Lajos, 1£: Unpublished data Gyurko G, Szabo \I: Experimental investigation', of the role of hemodynamic factors in fonnation of intimal changes.Surge~·66:871, 1969

Reprint requests: Dr. Lajos, 100 High Street. Buffalo 14203.

Machiavelli: Triumph, Humiliation and Vindication Machiavelli (1469-1527), citizen of Florence. was twentv-five \lears old when Charles VI I I of France overr~n Itah:. ~fachiavelli was endowed bv nature with consummat~ political ability. ~Ierit won hi'm, at the age of twenty-nine, the post of Secretary of the Government. After fourteen Yl'ars of this experience he had become perhaps hetter qualified than any other living Italian for takin~ a hand ill the urgent task of helping Italy to work out her politk'al salvation. In 1.512 he was deprived of his Secretaryship of Statl' and in the following year he suffered imprisonmellt and torture. The price which he had to pay for his release from prison was a perpetual rustication on his fann. In these hours was conceived and written TIl(' Prince. The book failed to achieve it.. author's immediatl' aim but it is not to sa" that The PrinCt' was a failure. Through his writings \Iachiavelli

was able to return to the world on a more etherial plane, on which his effect on the world has been vastly greater than the highest possible achievement of a Florentine Secretary of State immersed in the details of practical politics. In those magic hours of Catlwrsis ~Iachiaveni succeeded in transmuting his practical energies into a series of mighty intellectual works-The Prince, The Discourses 011 LiI:!I, TIll' Art of "'ar and The History of Florl'IICl'-which have heen the seed of our modern \\'estem political philosophy. Toynl>ee, A J: A Study of History, (Abridgment of vol. I-VI by Somervell. DC). Oxford University Press. New York and London, (16-th printin~) 1962

CHEST, VOL. 58, NO.6, DECEMBER 1970