anatomically malposition great arteries report with atrial · junction (van praagh and van praagb,...

British Heart Journal, 1974, 36, 207-2I5.

Anatomically corrected malposition of the greatarteriesReport of 2 cases, one with congenital asplenia;frequent association with juxtaposition of atrialappendages

Robert M. Freedom and Donald P. HarringtonFrom the Department of Pediatric Cardiology, The Children's Hospital Medical Center, and Department ofPediatrics, Harvard Medical School, Boston, Massachusetts; and the Division of Pediatric Cardiology, theDepartment of Pediatrics, the Johns Hopkins Hospital, and the Cardiovascular Division, Department ofRadiology, theJohns Hopkins Hospital, Baltimore, Maryland, U.S.A.

Anatomically corrected malpositions are uncommon cardiac anomalies in which the pulmonary artery orig-inates above the morphological right ventricle, and the aorta above the morphological left ventricle. However,because of interposition of abnormal subaortic conal myocardium, there is absence of aortic valve-mitralvalve fibrous continuity. The relation between the pulmonary artery and aorta is also abnormal because of theabnormal conal morphology.Two cases of anatomically corrected malposition of the great arteries are presented, one of which is the first

reported in a patient with the congenital asplenia syndrome; in the other, there was left juxtaposition of theatrial appendages. Review of all known cases of anatomically corrected malposition reveals a significantassociation with juxtaposition of the atrial appendages and hence with hypoplasia of the right ventricle, sub-pulmonary and subaortic obstruction. Possible surgical implications of this association are discussed.

Although Harris and Farber first suggested theconcept of anatomically corrected transposition ofthe great arteries in I939, recognition and docu-mentation of this unusual entity was first providedby Van Praagh and Van Praagh in I967.

In anatomically corrected transposition of thegreat arteries, the aorta arises above the morpho-logical left ventricle but with aortic-mitral dis-continuity because of abnormal subaortic conus,and the pulmonary artery originates above themorphological right ventricle. Because of theabnormal conal morphology, abnormal relationbetween the aorta and atrioventricular valve, andabnormal relation between the great arteries, thisentity was initially thought to be a form of trans-position (Van Praagh and Van Praagh, I967).However, because transposition of the great arteriesis not literally present (the aorta does not originateabove the morphological right ventricle and thepulmonary artery does not arise above the leftReceived I7 September 1973.

ventricle), more correctly, then, a positional anomalyof the great arteries, i.e. a malposition, is present;thus anatomically corrected transposition of thegreat arteries is more properly designated anatomi-cally corrected malposition of the great arteries(Van Praagh et al., I97I).

It is the purpose of this communication to presenttwo further cases of anatomically corrected mal-position of the great arteries (one documented atnecropsy and the other by angiocardiography) andthe first case documented in a patient with the con-genital asplenia syndrome, to review other knowncases, and to comment on the frequent associationwith juxtaposition of the atrial appendages.

Case reportsCase IThis male infant was admitted to the Children's HospitalMedical Center, Boston, Massachusetts, at 7 weeks ofage for evaluation of cyanotic congenital heart diseaseand bloody diarrhoea.

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F I G. I Case i. Admission PA chest radiographshows dextrocardia, slightly diminished pulmonaryvascularity, and abnormally symmetrical hepaticshadow.

Physical examination revealed a cyanosed, obtunded,dehydrated infant with abdominal distension. Dextro-cardia was diagnosed; a soft systolic murmur and singlesecond sound were heard. Bowel sounds were absent.Admission chest radiograph (Fig. i) showed dextro-

cardia with an unusual cardiac silhouette and dim-inished pulmonary vascularity. A symmetrical hepaticsilhouette and visceral heterotaxia were evident. A flatplate of the abdomen suggested intestinal obstruction,but a barium enema was unremarkable.An electrocardiogram revealed a frontal QRS and P

axis of +go9 and +I20°, respectively, with a negativeP wave in lead I, right atrial enlargement, and rightventricular hypertrophy. T wave inversion was presentin the limb and praecordial leads.

Hospital course Shortly after admission the infantsustained a cardiorespiratory arrest requiring intubationand artificial ventilation. After resuscitation, a leftpneumothorax was present and was drained with needlethoracentesis. Abdominal distension became moreprominent, and mesenteric artery thrombosis secondaryto polycythaemia and dehydration was suspected; subse-quently, he underwent an exploratory laparotomy wherethe mid-ileum was found to be oedematous and cyanotic,and the distal ileum was obviously necrotic. The distalileum was resected and a Mikulicz ileostomy was per-formed, but the infant died I0 hours after operationbefore cardiac catheterization could be performed.

Salient findings at necropsy included: dextrocardia;asplenia, abdominal heterotaxy but with basic situssolitus of atria; right superior vena cava and right in-ferior vena cava draining into the morphological rightatrium, right-sided; the left hepatic veins drainedseparately into the morphologically left atrium, left-sided; left superior vena cava to left atrium, left-sided;

FIG. 2 Case i. The diminutive right ventricle(R V) is seen. There is a slit-like, subcristal ventricularseptal defect (VSD). The subpulmonary conal myo-cardium (C) is well seen. The pulmonary artery (PA)originates above this diminutive right ventricularoutlet chamber.

small primum atrial septal defect; normal pulmonaryvenous return to morphologically left atrium, left-sided;a common atrioventricular valve emptying wholly intothe morphologically left ventricle, left-sided; a rudi-mentary outflow chamber, and almost complete absenceof the right ventricular sinus, right-sided (D-loop)(Fig. 2); subcristal ventricular septal defect, slit-like,obstructively small (Fig. 2); crista supraventricularislargely above the morphologically left ventricle (Fig. 3),but also above the very hypoplastic right ventricularsinus; a bilateral conus (subpulmonary and subaortic)preventing fibrous continuity between either semilunarvalve and atrioventricular valve; mild infundibular andvalvar pulmonary stenosis with a bicuspid pulmonaryvalve and a defective right septal-left septal commissure,but with severe pulmonary outflow obstruction causedby the small obstructive ventricular septal defect; L-malposition of the aorta with the aorta to the left andslightly anterior (almost side by side) to the pulmonaryartery, and originating entirely above the morpho-logically left ventricle (Fig. 4); a right aortic arch;probe-patent right ductus arteriosus; an abnormallysymmetrical liver; pulmonary isomerism with bilateraleparterial bronchial anatomy; left-sided stomach andright-sided gall bladder; abnormal small bowel mesen-tery; semi-annular pancreas and horseshoe adrenalgland; multiple peripheral emboli with focal renal in-farction; and thrombosis of the superior mesenteric



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Anatomically corrected malposition of the great arteries 209

FIG. 3 Case I. The posterior ventricle, the morphological left ventricle (LV) is seen. Thisventricle is moderately hypertrophied. The aorta (AO) is seen to originate above this ventricle.A distinct muscular band, the subaortic conus (C), prevents aortic-mitral fibrous continuity.

FIG. 4 Case i. The apex of the heart is rotatedslightly superiorly to show the relation between thegreat arteries. The aorta (AO) is to the left of thepulmonary artery (PA). This great vessel relationsuggests the 'corrected' transposition relation.

artery with infarction of long segments of the distaljejunum, ileum, and proximal ascending colon.

This infant with the classic features of the aspleniasyndrome had a right-sided heart with basically situssolitus of the atria, with a morphologically right atrium,right-sided, receiving the right superior and inferiorvena cava. There was atrioventricular concordance assitus solitus of the atria is concordant with a D-loop. Theventricular morphology is typically characteristic of asingle left ventricle with a rudimentary outflow chamberand pronounced hypoplasia of the right ventricularsinus. Hence, despite the presence of bilateral conalmyocardium, the great vessels were above the morpho-logically appropriate ventricle. Though the circulationwas potentially normal, this was impaired by the commonmixing of systemic venous and pulmonary venous bloodat the ventricular level.

Case 2

This I4-year-old boy has been followed since infancyfor cyanotic congenital heart disease.

Physical examination showed a small, cyanosed boy inno distress. Moderate clubbing was evident. Cardiacexamination revealed a prominent left ventricularimpulse. The first sound was of normal intensity. Thesecond sound was loud and single at the left uppersternal border. A harsh systolic ejection murmur wasbest appreciated along the left sternal border. All pulseswere full and equal. No hepatomegaly was evident.The chest radiograph (Fig. 5) showed a peculiar



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2IO Freedom and Harrington

F I G. 5 Case 2. Standard PA chest radiographshows abdominal situs solitus. The heart has a flatright heart border and a peculiar silhouette. The heartsize is normal, and the pulmonary vascularity appearsnormal.

cardiac silhouette, with a flat right heart border and dim-inished pulmonary artery segment. The pulmonaryvascularity is slightly diminished. The abdominal situsappears normal. The aortic arch is left sided.The electrocardiogram reveals left axis deviation of

- 750, right atrial enlargement, and left ventricularenlargement.A peripheral venous angiogram performed by injec-

tion of contrast into the right antecubital vein showedopacification of the superior vena cava, right atrium,hepatic veins, and also left juxtaposition of the atrialappendages, and the right ventricular window of tri-cuspid atresia (Fig. 6). With the catheter positioned inthe left ventricle through the atrial septal defect, a leftventricular angiogram was performed. This showed adilated and hypertrophied morphological left ventricle,left sided, and a hypoplastic right ventricle (Fig. 7 and8a and b). Hence the ventricles are normally related andare concordant to the atria. The aorta originates solelyabove the morphological left ventricle, but is abnormallyrelated to its atrioventricular valve. Mitral-aortic fibrouscontinuity is not present because of interposition ofabnormal subaortic conus. The pulmonary valve arisesabove the hypoplastic right ventricular outflow chamber,and subpulmonary conus results in significant sub-valvular pulmonary obstruction. Hence bilateral conus,subaortic and subpulmonary, is present. The aortic valveis abnormally related to the pulmonary valve, the aorticvalve being more leftward and anterior than normal.The left pulmonary artery is either absent or severelyhypoplastic.

FIG. 6 Case 2. A peripheral venous angiogramfrom the right median basilic vein shows opacificationof the superior vena cava, right atrium, inferior venacava, and hepatic veins. The right atrial appendage(RAA) is clearly juxtaposed to the left and is superiorto the left atrial appendage (LAA). The left ventricle(LV) is clearly opacified. The right ventricularwindow (W) is outlined.

In summary, this patient had left juxtaposition of theatrial appendages, tricuspid atresia, D-ventricular loopwith a hypoplastic right ventricle, subaortic conus pre-venting mitral-aortic continuity but with the aortaoriginating solely above the morphological left ventricleand abnormally leftward (L-malposition), subpulmonaryconus with significant infundibular and valvar obstruc-tion, ventricular septal defect, atrial septal defect, leftaortic arch, and probable absence of the left pulmonaryartery.

DiscussionAnatomically corrected transposition of the greatarteries is a most uncommon condition and, iftransposition is defined literally, then transpositionis not present, but a malposition is. The term'anatomically corrected malposition of the greatarteries' has been recently proposed for this entity(Van Praagh et al., I97I).



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FIG. 7 Case 2. Frame from selective left ventricular cine angiocardiogram in the antero-posterior projection. The venous catheter has been advancedfrom the saphenous vein, across theatrial communication into the left ventricle (LV). Contrast material opacifies a large ventricle,clearly a morophological left ventricle, and a diminutive right ventricular outlet chamber (RV).The right pulmonary artery (RPA) is clearly seen. The aorta (AO) is clearly to the left of thepulmonary artery. Bilateral conal myocardium (C) is evident.

In this malposition, the great arteries and ven-tricles appear to have twisted in opposite directions,resulting in a severe conoventricular malalignment,with a ventricular defect at the conoventricularjunction (Van Praagh and Van Praagb, I967).Bilateral conal myocardium (subaortic and sub-pulmonary) is usually, but not invariably, present.The abnormal subaortic conal myocardium isalways present and separates the aortic and mitralvalve. In addition, the subpulmonary conus is fre-quently present, separating the pulmonary fromthe mitral valve. Recently, however, Van Praaghhas cited an example of anatomically correctedL-malposition of the great arteries with pulmonary-mitral continuity, i.e. only the subaortic conalmyocardium is present (Van Praagh, I973a).

Bilateral or combined conal myocardium is mostfrequently found in double outlet right ventricle,transposition of the great arteries with subpul-monary stenosis or atresia, the asplenia syndrome,the juxtaposition of atrial appendages syndrome,and usually in anatomically corrected malpositions(Van Praagh, 1973a, c). Goor, Dische, and Lillehei(I972) have recently shown that subaortic conalabsorption accounts for aortic-mitral fibrous con-

tinuity. One might speculate, then, that persistenceof bilateral conal myocardium in the human repre-sents in part a developmental arrest at 20 to 30days ovulation age (Horizon I5).Of I3 adequately documented cases of anatomi-

cally corrected transposition (malposition) of thegreat arteries, 7 had left juxtaposition of the atrialappendages and an additional patient had rightjuxtaposition of the atrial appendages (Table).Melhuish and Van Praagh (I968) had previouslybriefly mentioned this association. It is of interestthat left juxtaposition of the atrial appendages isnormal to about 27 days ovulation age (HorizonsI2-I3) (Van Praagh, 1973b). Hence, left juxtaposi-tion of the atrial appendages may be interpreted asrepresenting the abnormal persistence of a normalembryonic condition. Significant subvalvar pul-monary obstruction (9/I3), tricuspid atresia orhypoplasia (6/I3), and hypoplasia of the rightventricular sinus (8/13) are common in anatomicallycorrected malpositions (Table) and in patients withjuxtaposition of the atrial appendages.

Anatomically corrected malpositions had pre-viously been documented in patients with visceralsitus solitus (Van Praagh and Van Praagh, I967;



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TABLE Comparative anatomical findings in patients with anatomically corrected malposition of the great arteries

Case No., Heart Viscero- Ventricular Great vessels Atrial Atrial A V valves Morphological.sex, and age at position atrial loop septum appendages rt. ventricledeath situs

I, M, 7 wk Dextrocardia Asplenia D L-malposition ASD I Normal Common Hypoplasia ofsinus

2, M, I4 yr* Laevocardia Solitus D L-malposition ASD II Left juxtaposition Tricuspid Hypoplasia ofof atrial atresia sinusappendages 7

3, F, 2 yr Dextrocardia Solitus D L-malposition ASD II, Left juxtaposition Tricuspid Hypoplasia oflarge of atrial atresia sinus

appendages4, F, 4 wk Laevocardia Solitus D L-malposition ASD II, Left juxtaposition Hypoplasia of Hypoplasia of

large of atrial tricuspid sinusappendages valve

5, M, 4 wk Laevocardia Solitus L D-malposition Intact Normal Normal Hypoplasia ofsinus

6, not known, Dextrocardia Not D L-malposition PFO Left juxtaposition Normal Normal3 mth known of atrial

appendages7, F, 7 mth Dextrocardia Solitus D L-malposition ASD II Left juxtaposition Tricuspid Hypoplasia of

of atrial atresia sinusappendages

8, F, 13 mth Laevocardia Solitus D L-malposition ASD II Left juxtaposition Tricuspid Hypoplasia ofof atrial atresia sinusappendages -

9, F, i dy Mesocardia Solitus Malrotated D-malposition ASD II Right juxtaposition Hypoplasia of Hypoplasia ofD of atrial tricuspid sinus

appendages valve

io, F, 4 yr Laevocardia Solitus D L-malposition PFO Normal Normal Normal

II, M, 3 mth Dextrocardia Inversus D L-malposition Intact Normal Normal Not known

I2, M, 3I yrt Dextrocardia Solitus D L-malposition Intact Normal Normal Normal t

I3, F, 8 yrt Dextrocardia Solitus D L-malposition Intact Left juxtaposition Normal Normalof atrialappendages

Note: Cases i and 2, present cases; Cases 3, 4, and 5, Cases I, 2, and 3, respectively, of Van Praagh and Van Praagh (I967); Case 6 RaghibI966); Case 7, Case i6 ofMelhuish and Van Praagh, I968; Case 8 (Stewart and Wynn-Williams, 1956); Case 9, Case 6 of Wagner et al.,Case IO (Sunada et al., I967); Case ii (Anderson et al. I972), Cases I2 and I3, Cases i and 2 of Kirklin et al. (I973).* Alive. Diagnosed at cardiac catheterization.t Alive. Diagnosed at cardiac catheterization and confirmed at operation.Abbreviations: 0, absent; D-loop, non-inverted ventricles with morphologically right ventricle, right-sided; L-loop, inverted ventricles; iposition, malpositioned aortic valve is to the right of pulmonary valve; L-malposition, malpositioned aortic valve is to the left of pulmonary vASD I, ostium primum atrial septal defect; ASD II, secundum type of atrial septal defect; VSD, ventricular septal defect.

Wagner, Alday, and Vlad, 1970) visceral situsinversus (Anderson, Arnold, and Jones, 1972), andnow in the congenital asplenia syndrome with vis-ceral heterotaxia. A bilateral conus is most commonin the asplenia syndrome (Ivemark, 1955), yet in anecropsy review of 32 patients with congenitalasplenia, an anatomically corrected malpositionwas found in only one case (Case i) and juxta-position of the atrial appendages in none (Freedom,I97I; Freedom and Fellows, I973). This mightpossibly be explained by the observation that left

juxtaposition of the atrial appendages is normal to27 days ovulation age, and that the congenital -asplenia syndrome results from a slightly later de-velopmental arrest (i.e. after the normal leftward Xswing of the ventricular apex results in the rightatrial appendage passing behind, and ending to theright of the conotruncus).

Although gross pathological evidence of anatomi-cally corrected malposition is lacking in our secondcase, we feel that this diagnosis is overwhelminglysupported by the angiocardiographic findings. The u



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5hologically It. ventricle Ventricular Type of Semilunar Outflow tract obstruction Aortic Additional anatomical dataseptum conus valve arch

AV valvecontinuity

ertrophy and VSD Bilateral 0 Valvar and infundibular Right Double inlet left ventricle,largement pulm. stenosis right persistent ductus

arteriosusVSD Bilateral o Valvar and infundibular Left

pulm. stenosis

ertrophy and VSD Bilateral o Valvar and infundibular Right Probe patent ductuslargement pulm. stenosis arteriosus

ertrophy and VSD Bilateral o Infundibular and valvar Right Right persistent ductuslargement pulm. atresia arteriosus

ertrophy and VSD Bilateral o Subaortic stenosis, Left Hypoplasia of ascendinglargement moderately severe aorta, preductal

coarctationttrophy and Multiple Bilateral o Subaortic stenosis, mild Left Mild cor triatriatum, leftlargement VSDs superior vena cava to

coronary sinus.rtrophy and VSD Bilateral o Valvar and infundibular Right Absent left coronary ostiumlargement pulm. stenosis

trtrophy and VSD Bilateral o Infundibular and valvar Leftlargement pulm. atresia

ttrophy and VSD Bilateral o Infundib. pulm. stenosis; Left Hypoplasia of ascendinglargement subaortic stenosis aorta and arch, left

persistent ductusarteriosus

VSD Bilateral o None Left Persistent ductus arteriosus,left superior vena cava

nown VSD Bilateral o None Not Not knownknown

gal Multiple ?Deficient + Infundibular pulm. stenosis NotVSDs known

ial VSD Bilateral o Valvar and infundibular Left Overriding aortapulm. stenosis

aorta originates above the morphologically leftventricle, but there is interposition of the abnormalsubaortic conus separating the aortic from themitral valve; the semilunar valves are abnormallyrelated, the aortic valve being more anterior thannormal, and finally the great arteries are not nor-mally related, the aorta being abnormally leftward.This diagnosis has been made only rarely beforedeath, but the angiographic features of this entityseem quite specific (Kirklin et al., I973). Becausethe aorta is abnormally leftward and anterior in thismalposition, it has been confused angiographicallywith corrected transposition of the great arteries(Raghib, Anderson, and Edwards, I966) in whichthe aorta is abnormally anterior and to the left ofthe pulmonary artery. In corrected transposition,however, there is both ventricular inversion andtransposition, with the aorta originating above the

morphological right ventricle, left-sided (in situssolitus). A selective ventriculogram in the left-sided ventricle will show a morphological rightventricle and the transposed aorta in the patientwith corrected transposition of the great arteries.Also, the electrocardiogram and vectorcardiogramwill prove useful in this differentiation (Ruttenberget al., i966). Kirklin and his associates (I973) havesuggested that anatomically corrected malpositionof the great arteries might be confused with doubleoutlet left ventricle or double outlet right ventriclewith an L-malposed aorta. Selective right and leftventricular biplane ventriculograms will help todistinguish these anomalies from anatomicallycorrected malpositions.

In addition to the obvious embryological andpathological importance, clinical recognition ofanatomically corrected malpositions may have



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214 Freedom and Harrington

a bFIG. 8a and b. Case 2. Selected frames from the left ventricular biplane angiogram in thelateral projection. The semilunar valves are at approximately the same level in this projection.The pulmonary artery (PA) originates above the right ventricular outlet chamber. The aorticvalve is clearly separated from the mitral valve (MV) by subaortic conal myocardium (C). Thissubaortic conal myocardium is quite dynamic. The RV infundibulum (INF) is very narrow andbecomes even narrower with systole. Bilateral conal myocardium is evident in this projection.

important surgical implications. Fontan's ingeniousoperation for the correction of tricuspid atresia(Fontan and Baudet, I97I) uses the right atrialappendage as the proximal end of a valved conduit tothe proximal right pulmonary artery. As pointed outearlier, however, and as illustrated by our secondcase, tricuspid atresia and left juxtaposition of theatrial appendages occur frequently with anatomicallycorrected malpositions (Table). Hence, correctionof tricuspid atresia in the condition using a valvedconduit might prove more difficult. It also becameapparent that subaortic stenosis occurred not in-frequently in this malposition (Table), resultingfrom the poorly expanded subaortic conal myo-cardium. If unrecognized, and unrelieved, thiscondition would seriously jeopardize Fontan'soperation, and might possibly be considered acontraindication to it. It is of interest that subaorticobstruction (caused by an obstructively small

ventricular defect) was suggested as the cause ofdeath in a 7-year-old girl with tricuspid atresia,transposition of the great arteries, and severe pul-monary valve and infundibular atresia who under-went Fontan's operation (Ross and Somerville,I973).

It should also be emphasized that anatomicallycorrected malpositions may occasionally have anormal tricuspid valve and normal right ventricularsize (Raghib et al., I966; Sunada et al., I967) andthat the intracardiac defects (atrial and ventriculardefects) may be amenable to standard intracardiacrepair (Sunada et al., I967). Recently completecardiac repair with survival was reported in twopatients with anatomically corrected malposition ofthe great arteries (Kirklin et al., 1973). Their firstpatient, a 3i-year-old man, also had subpulmonaryobstruction and multiple ventricular septal defects.Left juxtaposition of the atrial appendages, valvar



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and subvalvar pulmonary obstruction, ventricularseptal defect, and overriding aorta was repaired intheir second patient, an 8-year-old girl.

Also, while juxtaposition of the atrial appendagesis usually associated with severe congenital cardiacdisease, this is not invariably so. Becker and Becker(I970) described right juxtaposition of the atrialappendages in an anencephalic female stillborn;in this infant the great arteries were normallyrelated, and the only intracardiac malformationswere an atrial septal defect and a bicuspid pul-monary valve.

Recently bifid right atrial appendage with partialjuxtaposition has been described (Charuzi et al.,I973). This pattern was found in 9 of I5 specimenswith left-sided juxtaposition and was associatedwith the same spectrum of complex cardiac mal-formations commonly associated with complete

' left-sided juxtaposition.In summary, anatomically corrected malpositions

are rare conotruncal-ventricular disturbances, char-acterized anatomically by bilateral conus but with-out transposition of the great arteries. This mal-position frequently occurs with juxtaposition of theatrial appendages, tricuspid atresia or hypoplasia,

s and hypoplasia of the right ventricle. Antemortemrecognition of this anomaly is possible because ofdistinct angiocardiographic features. Awareness ofthis entity has significant embryological and sur-gical implications.

ReferencesAnderson, R. H., Arnold, R., and Jones, R. S. (I972). D-

bulboventricular loop with L-transposition in situs in-versus. Circulation, 46, I73.

Becker, A. E., and Becker, M. J. (1970). Juxtaposition ofatrial appendages associated with normally orientedventricles and great arteries. Circulation, 41, 685.

Charuzi, Y., Spanos, P. K., Amplatz, K., and Edwards, J. E.(1973). Juxtaposition of the atrial appendages. Circulation,47, 62o.

Fontan, F., and Baudet, E. (1971). Surgical repair of tricuspidatresia. Thorax, 26, 240.

; Freedom, R. M. (I97I). The heterotaxy syndrome (abstract).Circulation, 44, Suppl. 2, 15.

Freedom, R. M., and Fellows, K. E. (I973). Radiographicvisceral patterns in the asplenia syndrome. Radiology,107, 387.

Goor, D. A., Dische, R., and Lillehei, C. W. (I972). Theconotruncus. I. Its normal inversion and conus absorption.Circulation, 46, 375.

Harris, J. S., and Farber, S. (I939). Transposition of thegreat cardiac vessels with special reference to the phylo-genetic theory of Spitzer. Archives of Pathology, 28, 427.

Ivemark, B. I. (I955). Implication of agenesis of the spleenon the pathogenesis of cono-truncus anomalies in child-hood: an analvsis of the heart malformations in thesplenic agenesis syndrome, with fourteen new cases.Acta Paediatrica, 44, Suppl. 104, I.

Kirklin, J. W., Pacifico, A. D., Bargeron, L. M., and Soto, B.(I973). Cardiac repair in anatomically corrected malposi-tion of the great arteries. Circulation, 48, I53.

Melhuish, B. P. P., and Van Praagh, R. (I968). Juxtapositionof the atrial appendages. A sign of severe cyanotic con-genital heart disease. British Heart_Journal, 30, 269.

Raghib, G., Anderson, R. C., and Edwards, J. E. (I966).Isolated bulbar inversion in corrected transposition.American Journal of Cardiology, 17, 407.

Ross, D. N., and Somerville, J. (I973). Surgical correction oftricuspid atresia. Lancet, I, 845.

Ruttenberg, H. D., Elliott, L. P., Anderson, R. C., Adams, P.,and Tuna, N. (I966). Congenital corrected transpositionof the great vessels. Correlation of electrocardiograms andvectorcardiograms with associated cardiac malformationsand hemodynamic states. American Journal of Cardiology,17, 339.

Stewart, A. M., and Wynn-Williams, A. (I956). Combinedtricuspid and pulmonary atresia with juxtaposition of theauricles. BritishJournal of Radiology, 29, 326.

Sunada, T., Teramoto, S., Nakanishi, M., Fujiwara, T., andSenoo, Y. (I967). A case report of corrected transpositionof the great vessels (Cardell Bi type). Japanese Journal ofThoracic Surgery, 20, 389.

Van Praagh, R. (I973a). Conotruncal malformations. In HeartDisease in Infancy, Diagnosis and Surgical Treatment,P. 172. Ed. by B. G. Barratt-Boyes, J. M. Neutze, andE. A. Harris. Churchill Livingstone, London.

Van Praagh, R. (1973b). Conotruncal malformations. InHeart Disease in Infancy, Diagnosis and Surgical Treat-ment, p. I92. Ed. by B. G. Barratt-Boyes, J. M. Neutze,and E. A. Harris. Churchill Livingstone, London.

Van Praagh, R. (I973c). The segmental approach to diagnosisin congenital heart disease. In Fourth Conference on theClinical Delineation of Birth Defects (Birth Defects: OriginalArticle Series), p. I9. Ed. by V. A. McKusick. NationalFoundation - March of Dimes, White Plains, New York.

Van Praagh, R., Perez-Trevino, C., Lopez-Cuellar, M.,Baker, F. W., Zuberbuhler, J. R., Quero, M., Perez,V. M., Moreno, F., and Van Praagh, S. (I97i). Transposi-tion of the great arteries with posterior aorta, anteriorpulmonary artery, subpulmonary conus, and fibrous con-tinuity between aortic and atrioventricular valves. Ameri-can Journal of Cardiology, 28, 62I.

Van Praagh, R., and Van Praagh, S. (I967). Anatomicallycorrected transposition of the great arteries. British HeartJournal, 29, I12.

Wagner, H. R., Alday, L. E., and Vlad, P. (1970). Juxta-position of the atrial appendages. A report of six necropsiedcases. Circulation, 42, I57.

Requests for reprints to Dr. Robert M. Freedom, TheHelen B. Taussig Children's Cardiac Center, The Chil-dren's Medical and Surgical Center, The Johns HopkinsHospital, Baltimore, Maryland 2I205, U.S.A.



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