Jurding Transposition Aorta

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* From the Depar tm ents of D ia gnostic Rad io log y and Surgery , Schoo l of M ed ic ine, U n ivers ity of A labam a in B irm ingham , B irm ing-ham , A la bam a.t R es ea rc h A ss oc ia te in Rad io log y . P re sen tly at L H# { 244 } p it a lS t e . J us ti ne , M on tr ea l, Q ue be c, C an ad a.

VOL . 110, N o. 4

74 7

TRANSPO SIT ION OF THE GREAT ARTER IES*DETERM INA T IO N O F TH E PO S IT IO N O F TH E GREA T ARTER IES

IN CONVENT IONAL CHEST RO EN TGENOGRAM SBy RONALD GUER IN , M .D .,t B EN IGNO SO TO , M .D ., R OBERT B . KARP , M .D .,

JOHN W. KIRKLIN, M.D., an d ALBERTO BARCIA, M.D.B IRM INGHAM , ALABAMA

T HE hea rt o f pa tien ts w ith tran sp os i-tion o f th e g reat a rteries is c la ss ic a lly

d esc ribed a s h av in g a n arrow vascu larped ic le p la ced in fro n t o f th e ve rteb ra lco lum n as seen in co nven tion al p os te ro -an te rio r che s t roen tg en og ram s. T h e m e-d ias tin um is n arrow b ecau se the ao rta andpu lm on ary arte ry a re sup erim posed in thean tero po sterio r p lane .4

It w as o u r ex pe rience in a serie s o f 11 4cases p rev iou sly stud ied a t the M ayo C lin icth a t th e g reat a rteries are no t a lw ay s lo -c a ted in the sam e an tero po s ten io r p lan e.W ith th e pu rpo se o f an alyz ing th e d iffe ren tp oss ib le po s itio ns o f th e g rea t a rte ries ina ll typ es o f transp os itio ns , w e co rre la tedthe con ven tion al ro en tgen ogram s and an -g iocan d iog raph ic fin d ing s from 70 patientsstu d ied a t the U n ive rsity o f A labam aM ed ical C en te r.

METHOD OF ST UDY AND DE FIN IT IO NS

The co nv en tio na l ro en tgen ogram s w e retaken in po stero an terio r and la te ra l p ro -je c tio ns us in g a h ig h k ilo vo ltage tech n iq ueof io k v . an d 3 ph ase x -ray gene rato rs .The ang iocard io g rap h ic stud ies w ere d onew ith b ip lan e la rge ro l l f ilm techn iqu eby in jectin g the con tra st m ed ia se lectiv elyin to bo th v en tricu la r cham bers . T he roen t-geno gram s w e re tak en a t a ra te o f 12 p ersecon d in each p lane d u rin g th e first 2seco nds o f the se r ies . P a tien ts stu d ied on lyby c ineang io g rap hy w ere exc lud ed due tola ck o f d eta il an d th e im po ssib ility o f aqu an titativ e an a lysis . T he size o f the pu l-m on ary a rte ry an d aorta w as m easu redimm edia te ly abo ve th e sinuses o f V a lsa lvain the an te rop os ten io r and la tera l p ro je c-

tio ns . T he po s itio ns o f the p u lm ona rya rtery and the ao rta on th e p os te roan ten io rche s t roen tg en og ram w ere rep rod uced ontran spa ren t pape r p rio r to com parison w iththe ang io g rap h ic fin d ing s.

O ur serie s inc lud es ca se s o f c lass ic co rn -p le te transp os i tio n , co rrec ted transp os itio n ,and doub le ou tle t r igh t ven tr ic le o f thety pe in w h ich the ao rta is transp osed inre la tion to the pu lm on ary a rte ry . T h egene raliza tio ns co ncern ing lo ca liz a tion o fth e p os it ion s o f th e g rea t a rte ries a re ap -p lic ab le to bo th transpo s itio ns an d doub leo u tle t righ t ven tric le , an d on co nv en tion a lro en tgen ogram s the tw o cann o t be d is -tingu ished . A c la ssic com p lete transp osi-tio n is p re sen t w hen th e ao rta ar ise s fromthe m orp ho log ic righ t ven tric le an d th epu lm on ary a rte ry from the m orph o log icle ft v en tric le an d th e a tria and ven tric le sare concordan t, th a t is , bo th in situ s so h itu so r in ve rsus. M ost frequ en tly , com ple tetran spo sition o ccurs w ith s itu s so litu s o f thea tria , s itu s so litu s o f the ven tr ic les (d ex tro -ven tricu lar loo p) , rig h t-s ided sub ao rticco nus and the ao rta to th e r igh t o f thep u lm ona ry arte ry at the sem iluna r v alv elev el . A co rre c ted transp os itio n is p re sen tw h en the location o f th e ven tric les re la tiv eto th e a tria is d is co rd an t. M ost frequ en tly ,co rre cted transpo s itio n occu rs w ith si tu sso litu s o f th e a tn ia , inve rsion of th e v en -tric le s , le ft-s ided subaon tic co nus , an d aortato the le ft o f the pu lm ona ry a rtery a t thesem ilu na r va lve leve l. V an P raagh s6 8 loo pru le s ta te s th at the p os it ion o f th e v en -tric le s can be iden tified by the locat ion o fth e ao rta . A n ao rtic va lve loca ted to th erig h t o f th e p u lm ona ry va lve ind ica te s a


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TYPESOF INTERRE LATIONETW EENHEGRE ATARTE R IESAT THE SEM I LUN ARVA LVELEV EL.

II

S ID E B Y S ID E

AORTATO R IG HT ,5 % AORTATO LEFT ,10 %

OBL IQUEA OR TAT O R IG HT ,30 % A OR TAT O L EF T,22 %AORTA ANTERO AORTAR IGHT PO STER IOR LEFT35% 33% 32%

74 8 G uen in , So to , K a rp , K irk lin an d B a rcia DECEM BER, 1970

IANTERO POSTER IORAORTAANTER IOR ,3 %

F IG . s . Summary of th e ty pes o f i nt er re la ti on be -tw een the g reat a r ter ie s , an d the ir re la tiv e in -c id en ce in th is se rie s of 70 pat ien ts .

r igh t-s ided m o rp ho lo g ic rig h t ven tr ic le (d -v en tricu la r loo p ). A n aortic va lve to th elef t ind ica te s a lef t-s ided m o rp ho lo g ic rig h tv en tric le (1 -ven tricu lar loo p ).8

RE 5 U LT SW e found th at the g rea t a rte rie s a t th e

sem iluna r va lve leve l m ay b e in 3 mainpositio ns in pa tien ts w ith transpositio nand re la ted m alfo rm ations : (i) an te ropos -t eno r ; (2 ) side -by -side ; and (3 ) ob l ique .W e had prev io us ly d esc ribed th ese p os i-tio ns in d oub le o u tle t rig h t ven tric le .2 Inth e firs t g rou p th e ao rta w as alw ay s lo -ca ted an terio r to th e p u lm ona ry a rte ry .In the seco nd gro up , th e ao rta w as to therigh t o r to the le ft o f the p u lm on ary a rte ry .W hen the g rea t arterie s w e re in an o b liq ue

po si tion the ao rta w as alm ost a lw ay s an -teno r to the rig h t o r an te rio r to the le ft o fth e pu lm ona ry a rte ry . In o n ly i case w asth e ao rta pos te rio r to the pu lm ona ry ar-tery .

The pe rcen tag e d is trib u tio n o f th e 3typ es o f in te rre latio n be tw een the g rea ta rte r ies is show n in F igu re i . The freq uencyo f th ese 3 typ eS in com p lete tran spo sit ion ,co rrec ted tran spos itio n and doub le ou tletr igh t ven tric le is de ta iled in F igu re 2. Inc lass ic com p le te transpo s itio n , the m ostc ommo n i nter rela tio nsh ip s w ere th e ob liqu ew ith th e ao rta to the righ t (F ig . 3, C an dD ; and 4 , A and B ), and th e an te ro -pos te rio r (F ig . 3, ii an d B ). In 5 ca ses o fcom ple te tran spos it ion , the ao rta w aslo ca ted an terio rly and to th e left in re la tionto the p u lm ona ry arte ry . T he se excep tio nsto V an P raag h s lo op ru le w e re du e in eachca se to the m a rked righ t-to -le ft ro ta tion o fthe hea rt p rodu ced by rig h t ven tricu la rhype rtroph y an d dex trocon vex dorsal sco li-o sis . In co rrec ted transpositio n th e m ostcomm on in te rre la tio nsh ip w as eithe r th eside -b y -side (F ig . 5 , z l -D) or o b liqu e (F ig .6, 1 an d B ), in bo th in s tances w ith theao rta to the le ft. T he an te rop os ten io r re -la t ion w as less frequ en t than in com ple tetran spo sition (F ig . # {2 31} , a nd D ). In doub leou tle t righ t ven tric le the re a lso frequ en tlyw as a side -by -s id e (F ig . 3, ii an d B ) orob liq ue re la tionsh ip . A s sh ow n in F igu re 2,th e ao rta w as to th e le ft in 6 (i + 5) casesand to the righ t in 5 (3+2) . O f th e 3 pa -tien ts w ith an te ropo s terio r re la tion , 2 ha dpu lm on a ry atre s ia .

F igu re 7 sh ow s th e p os itio n o f th e g rea ta rte r ies a t th e sem ilu na r v a lve leve l re -la ted to th e v isc ero atria l situ s , the ven tr icu -la r lo op , the s iz e o f th e g reat arterie s an dthe h ea rt ax is . T h e v isce roa tn ia l s itu s w asso litu s in 90 p er cen t o f the p atien ts an din ve rsu s in 10 p er cen t. N o pa tien ts w ithin de term in ate v isce ro atr ia l s itu s w erestu d ied in o ur se r ies . Th e ven tr icu la r lo opand the p osition of the a scend ing ao rtaw as con co rdan t in a ll c ase s o f com p letetranspo s itio n ex cep t in p atien ts w ith d -lo op , in w h ich the ao rta to the left o f the


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VOL . 110, No. T ransp os itio n o f th e G rea t A rte ries 74 9

TYPESOF INTERRELAT IONBETMEENTHE GREATARTER IESIN TRANSPOS IT IONSND DOUBLEOUTLETR IGHTVENTR ICLE .

1 0 C AS ESI

ANTERO POSTER IOR

IIS ID E B Y S ID E

A D TAR IG HT LE FT G

II IOBL IQUE

R IG HT A OR TA L EFT

COMPLETETRANSPOS IT ION 38 0

0 19 5

CORRECTEDTRANSPOS IT ION18 5 0 6

DOU BLEOUTLE TR IGHTVENTR ICLE 3 3 1 2 514

FIG . 2 . Deta i l of the frequency o f the 3 ty pes o f com ple te tran spo sition , co rrectedtransp ositio n , and doub le ou tle t r igh t ven tr icle .

B S

pu lm on ary arte ry w as an ex cep tion to th eloo p ru le .

T h e p os ition o f the g rea t arterie s an dth eir in terre la t ion sh ip co u ld be p red ic tedaccu ra te ly in the m a jo rity o f th e case sfrom ch es t roen tg en og ram s don e w ith h ighk ilo vo ltage techn iqu e. D ifficu ltie s aro sew hen th e re w as a m ark ed d isc rep an cy in thes ize o f th e ve sse ls , p articu larly w h en theao rta w as sm a ll an d the p u lm on ary a rte rylarge .

DISCUSS IONThe h ig h k ilo vo ltag e ch es t roen tgeno -

g rap h ic te ch n iq ue al low s be tte r v isu al iza -t ion o f th e h ea rt an d m ed ia s tinum by in -c re as in g the con tra st be tw een air an d so ftt is su e an d decrea sin g the con tras t be tw eenso ft tis sue and bone s .5 W ith th is te ch n iq ueit is po ssib le to o u tline the a scend ing ao rta(F ig . 3-6 ) in the m a jo rity o f the ca se seven w hen the a rte ry is no t b o rd er-fo rm -ing .

T h e ascen d in g ao rta m ay be d ire c tlylocalized o n co nv en tio na l ch es t roen tgeno -g ram s m ade in th is w ay w hen it is iden tif iedo n the rig h t (F ig . 3 , z1 -D ; an d 4 , 1 an d B )

or le ft (F ig . 5, A -D; an d 6 , A and B )m edia s tina l b o rd er. W hen the ascen d in gao rta is n o t ou tlin ed , w e try to id en tifyth e m a in p u lm on ary a rtery . T h e m a inp u lm ona ry artery d irec tly v isu a lized onthe le ft m ed ia st ina l bo rde r (F ig . A an dB ) ind ic ate s a rig h t-s id ed ao rta . T o locatethe m a in pu lm on ary a rtery w hen it is n o td irec tly ou tlin ed in hearts w ith a w id eva scu la r p ed ic le , on e iden tifie s the rig h tand left pu lm on ary b ran ch e s. If the p u l-m on ary b ranche s a re a t the sam e lev e l an do f th e sam e len g th , th e m a in pu lm on arya rtery w as m ed ia lly p laced ind ica tin g aleft-s id ed ao rta (F ig . z l-D) . W hen therig h t p u lm ona ry a rtery is h ig he r than theleft (F ig . 6 , A an d B ), the ascen d ing ao rtaw as lik ew ise loca ted in th e left up pe rm ed ia s tin um . A ll ex cep t o f th e p at ien tsw ith th ese arrang em en ts h ad co rre c tedtranspos i t ion.

W hen the re is a n arrow v ascu lar ped ic leand one canno t id en tify the a scen d in gao rta o r the pu lm ona ry a rte ry , th e re la tiv epo si tion o f the le ft an d righ t m ain b ranche sm ay he lp to locate th e m ain p u lm on arya rte ry . If the left pu lm ona ry b ran ch is


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75 0 G uerin , S o to , K arp , K irk lin and B arc ia DEC EM BER , 1 970

11G . 3 . (In th is and subseq uen t F igu res, (A ) an d (C ) a re fro n ta l conv en tiona l chest roen tg en ogram s, andB an d D are schem atic d iag ram s o f correspon d ing ang io card io gram s.) (4 an d B) D oub le o u tle t r igh tven tricle w ith bo th su baortic and subpu lm onary co nus and ven tricu la r sep ta l de fec t. T he gas tric a ir b ubb leto th e le ft in d icates tha t th e v isce ro atria l situ s is so litu s . T he vascu la r ped ic le is w ide as the grea t a rte riesare s id e-by-s id e . T he ascend in g aorta to th e righ t o f the p rom inen t m ain pu lm onary arte ry iden tifie s then orm al (so litu s) pos itio n of th e righ t v en tr ic le , acco rd ing to the ioo p ru le . (C an d D ) C om ple te tran spos i-tio n o f the g reat ar te rie s w ith v en tricu la r s ep tal de fec t an d ob lique in te rre la tion be tw een the g rea t a r ter ie s .The w ide v ascu la r ped ic le is due to prom in en ce of the ascend in g ao rta on the righ t m ed ias tin al bord er andto the m ain pu lm on ary arte ry on the le ft. T here is s itu s so litu s o f v iscera, a tria and ven tricles . A O = aorta ;PA = pu lm ona ry a rtery ; RA = r ig ht a tr iu m; R V = r ig ht v en tric le ; LV = l ef t v en tr ic le .


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0POST .

75 1

na an des. T heed iast i -he Vas-


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hG . (A an d B) Corrected transpos ition w ith ven tricu la r sep ta l de fect and side -by -side in te rre lation b e-tw een the grea t a rte ries. T he d ila ted aorta is p rom inen t on the le ft m ed ias tina i b order. T h e m ain pu l-m onary arte ry is m ed ia lly p laced w ith r igh t and le f t b ranches at th e sam e leve l. T h e le f t-s id ed locatio n o fthe ao rta ind ica tes a le ft-s ided righ t ven tric le . T he v isce ra an d a tria a re in s itus so litu s . T he a tria andventr icle s a re in d iscord an t pos itio n . (C an d D ) Comm on ven tric le w ith und erdeve loped righ t ven tricu la rsinu s and large righ t-s ided m orp ho log ic le ft ven tric le. T he grea t a rteries a re transposed and s ide-by-s idew ith the ascend ing aorta to the lef t of the pu lm ona ry a rte ry , forming a w ide vascu la r p ed ic le. LA = le fta trium ; I = in fund ibu ium.

75 2 G uerin , S o to , K arp , K irk lin and B arc ia DEC EM BER , 1 970


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VOL . no , N o. T ransp ositio n of the G rea t A rter ie s

F IG . 6. (A an d B) Correc ted transpo sition w ith o b liqu e in te rre la tion be tw een th e g reat ar te rie s . T he ascen d-ing ao rta is lo cated on the lef t m ed ia stina l bo rd er . T he m a in p u lm ona ry a rte ry is m ed ia lly p laced and th er igh t pu lmona ry b ran ch is h igher than th e le f t. (C an d D ) C orrected transpos itio n with anteroposteriorin te rre la tion b e tw een the g rea t a rte r ies . T h e vascu la r ped ic le is na rrow w ith bo th g reat ar te rie s m ed ia llyp laced . The right and le f t p u lm ona ry a rte rie s a re a t the sam e leve l. T he re is situ s so litu s of t he v isce ra andatr ia w ith d isco rdan t po sition o f th e v en tric le s .


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POS IT IONOF GREATARTER IESN RELAT IONO V ISCERO#{ 149 }ATR IALITUS,VEN TRICULAROO P,GR EATAR TER YSIZE,A ND HE ARTAXIS .1 0 C A SE S

I II IIIANTERO -POSTER IOR

23

S I DEBY S IDEAORTAR IGHT LEFT

OB LIQU EAO RTAR IGHT LEFT

& 21 16 I V ISCERO SOL ITUS 631ATR IAL II S I TUSL INVERSUS iJV EN TR IC ULA RfD . LO OP 46

LOOP L1 .OOP 24GREAT AO>PA 30

ARTERY [AO=PA 1SIZE I AO


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VOL. iso, No. T ran sp os ition o f the G reat A rterie s 755arterie s w ith the ao rta an te rio r an d to theleft . T he o the r excep tio n w as a case o fd oub le o u tle t righ t ven tric le , si tu s so litu so f a tria an d v en tric les , an d left-s id ed ao rta .

T h u s it is o f p rim e im po rtance to locatethe ascen d in g ao rta o n the con ven tion alche s t ro en tgen ogram s to e stab lish th e in -te rre la tio n be tw een th e g rea t a rte ries andthe pos it ion o f th e ven tric le s . T he fo llow inga re the fa c to rs a ffec tin g the iden tifica tio no f the ascen d in g ao rta in the co nv en tio na lc he st ro en tg en og ram s: (i) the p os itio n o fthe ao rta in re la tio n to the m ain pu lm on aryartery; (2 ) th e re la tive s iz e o f the g rea tarteries ; (, the p os itio n o f th e h ea rt ax is ;an d ( the ro ta tion o f th e h ea rt an d d e -g ree o f rig h t v en tricu la r hyp e rtrop hy .

W hen an an terop oste rio r in te rrela tion -sh ip o f th e g reat a rte ries o ccu rs , the re la t ivesize o f the arterie s is th e m ost im portan tfa c to r in th e v isu a liza tio n o f the ao rta . Ineve ry ca se o f p u lm ona ry a tres ia and inm o st ca ses w ith seve re p u lm on a ry s ten os is ,the ao rta w as d ire ct ly iden tified on thech es t ro en tgen ogram s. W hen the g rea tarte rie s a re s id e-b y -s id e , the p os ition o f th eve ssels is m o re im portan t than th eir re la-tive size in the iden tifica tio n o f the ao rta .In ob liqu e in te rre la t ion sh ip the ao rta ism ore ea si ly seen a s a bo rde r-fo rm ing s truc -tu re w hen it is on th e le ft. T h is is a co ns-equ en ce o f the d eg ree o f righ t ven tricu larh yp ertro ph y an d th e c lo ckw ise ro ta tio n o fth e h ea rt. T h us the ao rta is p rom inen teven w hen it is no t en larg ed an d sm a lle rth an th e pu lm on ary a rtery .

T h e p os ition o f th e hea rt ax is (lev o -card ia o r dex trocard ia) w as n o t an im po r-tan t fac to r in the id en tific a tion o f theao rta . In d ex troca rd ia s w ith lev ov en tricu -la r lo op , the ao rta w as m o re o ften d ire ctlyiden tified to the le ft in 10 o f 12 ca s e ss tud ied , becau se th e side -by -s id e an d ex -trem e ob liq ue po sit ion s w ere m o re freq uen t.

S UMMARYTo d eterm ine th e re la tiv e po s itio n o f th e

ao rta and pu lm ona ry artery in co nv en -tio na l che s t roen tg en og ram s, a co rre la tiv estudy w ith la rge ro ll f ilm ang io ca rd io g ram s

w as m ade in 70 cases o f transpos itio n of th eg rea t arte rie s , inc lu d in g 14 ca ses o f doub leo u tle t rig h t ven tric le o f th e transp os itio ntype .

W e fo und 3 m ain typ es o f in terre la tionbe tw een the g rea t a rte r ies : an te ropo ster io r ,ob liq ue and s id e-b y -s id e .

F rom th e in te rp re ta tion o f the co nv en -t ion al h igh k ilo vo ltage ro en tgeno gram itw as poss ib le to de te rm in e th e po s ition o fthe g rea t a rteries in the m ajo rity o f th ecases w ith levo ven tr icu la r loo p and le ft-s id ed aorta. In pa tien ts w ith d ex trov en tri-cu la r lo op an d an tero po sterio r re la tio nsh ipbe tw een th e g rea t a rteries , it w as m ored ifficu lt to iden tify the po sition of th earter ies .

S ix ex cep tion s to the loo p ru le o ccu rredin 70 cases .A lberto Ba rcia , M.D .D ep artm en t o f D iagno stic Radio l o g yU nive rsity o f A lab am a M edica l C en te rB irm ingh am , A labam a 35233

REFERENCESI. BARCIA , A ., K INCAID , 0 . W ., DAVIS , G . D .,

KIRKL IN , J. W ., an d ONGLEY , P . A . T ran s-p osition of g rea t a r ter ie s: ang iocard io g rap h icstudy . AM . J. ROENTG ENOL ., RAD . TH ERA PY &NUCLEAR M ED ., 1967 , ZOO , 249-283.

2. H IGHTOWER , B . M ., BARCIA , A ., BARGERON ,L . M ., JR. , and K IR KL IN , J. W . D o ub le -o ut le tr igh t ven tricle w ith tran spo sed great a rte rie sand su bpu lm on ary ven tr icu la r sep ta l de fec t:T au ss ig-B in g m a lform a tion . Circu la t ion , 1969 ,39 , Supp l . I, 2 07 -2 53 .

3. KE ITH , J. D ., ROWE , R . D ., and VLAD , P.H eart D isease in In fan cy and Childhood .S econd ed ition . The M acm illan Company ,N ew Y ork , 1967, p. 704.

4. KJELLBERG , S . R ., MANNHE IMER , E ., RUDHE ,U ., and JoNssoN , B . D iag nosis o f C ongen ita lH eart D isea se. A clin ica l and tech n ical s tudyby the card io lo g ic team o f the P ed iatr icClin ic, K arolin sk a S ju khuse t, S to ck holm .S econd ed itio n . Y ea r Book P ub lish ers , In c.,Chicago, 1959 , p . 776 . MAGUIRE , G. H., BE IQUE , R. A., an d ROTEN -B E RG , A . D . S e lectiv e f iltra tion : p rac tical ap -p ro ach to h ig h -k ilov o ltage rad iog raphy . Ra -d io l ogy , 5965 , 8 5, 3 43 -3 5 5.

6. PAUL , M. H., VAN PRAAGH , S., and VAN PRAAGH ,R. T ran sp os it ion o f th e g rea t a rte ries . In :Pediatric Cardiology. Edited by H. Watson.C. V. Mosby Company, St. Louis, 1968, p. 576 .


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G uerin , S o to , K a rp , K irk l in an d B arc ia5 6 DECEMBER, 19707. PAUL , M. H., MUSTER , A. J. , S I NHA , S . N ., COLE ,

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