mustard's operation for patients with ventriculoarterial...

Br Heart j 1985; 53: 443-50

Mustard's operation for patients with ventriculoarterialconcordanceReport of two cases and a cautionary tale

P ARCIPRETE, F J MACARTNEY, M DE LEVAL, J STARK

From the Thoracic Unit, The Hospital for Sick Children, London

suMMARY Three patients with ventriculoarterial concordance and an abnormal atrioventricular con-

nexion were investigated. One patient had total anomalous pulmonary venous connexion to a leftvertical vein. In 1972 this was repaired and a ventricular septal defect was closed, but the presence ofatrioventricular discordance was missed and the patient died. In the other two patients, Mustard'soperation resulted in survival. One patient had situs solitus and atrioventricular discordance with an

associated ventricular septal defect, which was closed. The other had left isomerism (quasi solitus)with an ambiguous atrioventricular connexion (quasi discordant). A previously noted ventricularseptal defect had spontaneously closed.

Intra-atrial baffle procedures (venous switches) suchas Mustard or Senning operations are normally usedto compensate congenitally determined singleswitches in the circulation. By far the commonest ofthese is complete transposition, in which the patienthas a single switch (ventriculoarterial discordancewith atrioventricular concordance). A much rarerindication for venous switching occurs when thesingle switch is at atrioventricular level and there is noswitch at ventriculoarterial level-that is, there is ven-triculoarterial concordance. In fact, only eight suchoperations have been hitherto described. 1-6 The pur-pose of this-report is to document two further exam-ples and to illustrate the danger of an incorrectpreoperative diagnosis in a third.

Patients and methods

CASE 1A 3Y2 year old girl was referred from Germany with adiagnosis of total anomalous pulmonary venous con-nexion. The angiocardiograms on which this diag-nosis was based were not of high quality, but onreviewing them it was clear that there was situs solituswith atrioventricular discordance and ventriculoarter-

Requests for reprints to Mr i Stark FRCS, Thoracic Unit, The Hos-pital for Sick Children, Great Ormond Street, London WC1N 3JH.

Accepted for publication 14 November 1984

ial concordance, a ventricular septal defect, and totalanomalous pulmonary venous connexion to a left ver-tical vein. The ventricles were normally related foratrioventricular discordance, but the great arterieswere side by side with the aorta to the right.On physical examination, she had moderate

cyanosis and finger clubbing. There was clinicalhypertrophy of both ventricles and a systolic thrill atthe lower left sternal border. On auscultation, the firstheart sound was normal, the second heart sound wassplit, and there was a pansystolic murmur at the lowerleft sternal border. There was no diastolic murmurand no sign of heart failure. Her chest x ray filmshowed a large heart with increased pulmonary vascu-lar markings and a wide mediastinum, and her elec-trocardiogram showed sinus rhythm and right atrialhypertrophy with a PR interval of 0-16 s, a QRSinterval of 0-0 s, and a somewhat atypical right bun-dle branch block pattern.

DiagnosisShe underwent repeat cardiac catheterisation in June1972. The vertical common pulmonary vein wasentered and contained blood with an oxygen satura-tion of 92%. There was a 10 mm Hg mean pressuregradient between the vertical vein and the superiorvena cava. Both ventricular systolic pressures wereequal at 90 mm Hg and the pulmonary artery pressurewas 75/40 mm Hg. The pulmonary and systemicartery oxygen saturations were equal (80%). Repeatcineangiograms were performed with injection into

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444the common pulmonary vein, pulmonary trunk, andleft sided ventricle, but a fault in processing destroyedthe frontal plane film. She was therefore referred forsurgery with a diagnosis of situs solitus with normalcardiac connexions, total anomalous pulmonary ven-ous connexion to a vertical vein, and a ventricularseptal defect.

OperationAt operation a ventricular septal defect 2 cm in diame-ter was closed through the right atrioventricularvalve. An anastomosis was constructed between thepulmonary venous confluence and the left atrium, anda patent foramen ovale was closed. The common pul-monary vein was ligated. She could not be weanedfrom cardiopulmonary bypass despite an attempt toreopen the ventricular septal defect, and she died onthe operating table.

Necropsy findingsAt necropsy, the reason for the operative death.became clear. The bronchi and atria were in situssolitus. Pulmonary venous return had been success-fully directed to the left atrium and a patent foramenovale closed, but these two procedures had been disas-trous in the presence of atrioventricular discordancewith ventriculoarterial concordance. The right sidedmitral valve had two sets of papillary muscles and noinsertion into the septum.There was a large perimembranous ventricular sep-

tal defect excavating both inlet and outlet septa, suchthat posteriorly it was roofed by the two atrioventricu-lar valves in continuity, and anteriorly it. was roofedby the outlet septum. Into the underside of this wasinserted the anteroseptal commissure of the tricuspidvalve (the remainder of which was normal). There wasmild malalignment of the trabecular and outlet septasuch that the aortic valve overrode the septumslightly, originating 85% above the left ventricle.There was a bilateral infundibulum, with 7 mm of

muscle separating the mitral and aortic valves and 5mm of muscle separating the tricuspid and pulmonaryvalves. The great arteries lay side by side with theaorta to the right-that is, with a slight clockwisespiral as they ascended. The right ventricle was a littlesmall, being only two thirds the length of the leftventricle, and lay to its left, the septal orientationbeing as is usually found in congenitally correctedtransposition. The coronary artery pattern wasinverted.

CASE 2A 4 month old baby boy, weighing 5-2 kg, was admit-ted to our unit in congestive heart failure. Poor feed-ing and moderate cyanosis at rest had been notedsince birth. On examination the child was dyspnoeic

Arciprete, Macartney, de Leval, Stark

and severely cyanosed on crying. His pulse rate was145 beats/minute and his blood pressure 100/60 mmHg. On auscultation no cardiac murmurs weredetected. His chest was clear. There was nohepatomegaly, and all peripheral pulses were palp-able. Laboratory investigations showed a haemoglo-bin concentration of 17.7 g/dl and a packed cell vol-ume of 51%. His chest radiography showed pulmo-nary plethora with mild cardiomegaly, and his elec-trocardiogram recorded sinus rhythm, right axisdeviation, and a deep Q wave in leads II, III, aVF andV4R. There was a dominant R wave in V4R with anupright T wave.

DiagnosisCardiac catheterisation and angiocardiographyshowed situs solitus, atrioventricular discordance,ventriculoarterial concordance, ventricular septaldefect, and patent foramen ovale. The great arterieswere normally related, spiralling clockwise as theyascended. There was a very short muscular subaorticinfundibulum and a longer subpulmonary infun-dibulum. The intracardiac pressures were: mean rightatrium 3 mm Hg, mean left atrium 14 mm Hg, rightventricle 70/0 mm Hg, left ventricle 70/0 mm Hg,pulmonary artery 70/30 (mean 42) mm Hg, and sys-temic artery 70/50 (mean 60) mm Hg. The aorticoxygen saturation was 54% and improved to 66% afterballoon septostomy. The pulmonary artery oxygensaturation before septostomy was 92%. In view of thesevere degree of arterial desaturation and failure tothrive, it was decided to proceed to surgical correc-tion.

OperationAfter the pericardium had been opened the anatomyof the heart was assessed. The heart was enlarged.The pulmonary artery was anterior and to the left ofthe aorta, which was posterior and to the right. Bothvenae cavae were normally connected with the rightatrium, which was on the right. The right atrium wasconnected to the morphologically left ventricle. Thepulmonary veins emptied into the left atrium, whichdrained to the somewhat superior and leftward mor-phologically right ventricle. There was only one largecoronary artery arising from the aorta anteriorly, giv-ing off the left and the right coronary arteries whichwere distributed on the anterior aspect of the heart.After cannulation of the aorta and both venae cavae,cardiopulmonary bypass was established. The patientwas cooled to 20°C nasopharyngeal temperature, theaorta was cross clamped, and cardioplegic solutionwas infused. The right atrium was opened and it waspossible to confirm the angiographic diagnosis. Theventricular septal defect was located in the inlet sep-tum and was closed through a short right ven-



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Mustard's operation for patients with ventriculoarterial concordance

Fig. 1 Case 3: left ventricular angiocardiograms (frontalplane). (a) Shows the catheter passing round an azygos

continuation (Az) into the nght sided atnum and thence to the leftventicle (LV), from which the aorta (Ao) opacifies via a

subaortic infundibulum (In). An aneurysm (An) of themembranous septum is seen, through which two tiny ventricularseptal defects (small black arrows) opacify the right ventricle.Note the tvo long morphologically left bronchi (white arrows).(b) Shows the catheter, having crossed a patentforamen ovale,entering the right ventricle (RV), from which the pulmonarytrunk (PA) opacifies.

triculotomy using a Dacron patch. Anteriorly thestitches were inserted on the morphological right sideof the ventricular septum, according to the techniquepreviously described to reduce the risk of injuring theHis bundle.7 Mustard's operation was then performedusing a pericardial patch. The new pulmonary venousatrium was enlarged with a woven Dacron patch.When perfusion was terminated the patient was insinus rhythm. Postoperative systolic pressures were:

aorta 100 mm Hg and pulmonary artery 30 mm Hg.The postoperative course was uneventful, and thepatient was discharged two weeks later in an excellentcondition.

Follow upAfter almost six years the patient was still asymptoma-tic and in sinus rhythm. Recatheterisation in January1984 showed normal intracardiac pressures with no

residual shunts and no evidence of caval pathway obs-truction. A cross sectional echocardiogram showed

typical atrioventricular discordance, with reversedoffsetting of the atrioventricular valves. Precordialshort axis views showed the typical "circle and saus-age" appearance of normally related great arteries, theaorta being the circle and the right ventricular outflowtract and pulmonary trunk being the sausage.

CASE 3A male child had had cyanosis and dyspnoea since theage of 2 weeks. Cyanosis and clubbing increased pro-gressively, while exercise tolerance graduallydecreased. At the age of 12 years he was investigatedabroad. The diagnosis of transposition of the greatarteries with a small ventricular septal defect andinterruption of the inferior vena cava with rightazygos continuation was made. Pressures at the timeof cardiac catheterisation were equal in the two atria(mean 7 mm Hg). The pressure in the subpulmonaryventricle was 24/0-5 mm Hg and in the pulmonaryartery 22/7 mm Hg. The systolic pressure in the sub-aortic ventricle and aorta was 110 mm Hg. The pul-monary artery oxygen saturation was 86%, whereasthat in the frontal artery was 72%.The patient was lost to follow up. At 16 years of age

he was referred to us for further evaluation and treat-ment. On examination, he was physically under-developed, with severe cyanosis and clubbing and wasdyspnoeic at rest. His blood pressure was 120/60 mmHg. There was an ejection systolic murmur at the leftsternal border and a split second heart sound. Hischest was clear on auscultation. His liver was enlargedto 4 cm below the costal margin. The spleen was notpalpable. All peripheral pulses were palpable andequal. Laboratory investigations showed a haemoglo-bin concentration of 22-8 g/dl and packed cell volumeof 72%. Coagulation tests and serum electrolyte andblood urea concentrations were all within normallimits. His chest radiograph showed mild cardiomeg-aly with a cardiothoracic ratio of 0 54 and increasedpulmonary vascular markings. His electrocardiogramrecorded a superior P wave axis with right axis devia-tion and right bundle branch block.

DiagnosisA review of the angiocardiograms (Fig. 1) establisheda diagnosis of left atrial isomerism (quasi solitus),ambiguous (quasi discordant) atrioventricular connex-ion, ventriculoarterial concordance, left hand patternof ventricular morphology, small ventricular septaldefect in the aneurysm of the ventricular septum,aberrant right subclavian artery, atrial septal defect atthe fossa ovalis, and interruption of the inferior venacava with azygos continuation to the right superiorvena cava. The pulmonary valve lay anterior to theright of the aorta, and the great arteries spiralledcounterclockwise as they ascended.

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"'evTFig. 2 Case 3 (after repair): cross sectional echocardiograms. (a) The apical four chamber cut, angledfonvard to show leftventricular outflow tract, shows the fight venticle (RV) identifed as such by the moderator band (MB). The pulmonary venous atrium(PVA) is now connected to the left ventricle (LV), which has a normal outflow tract roofed by the aortic valve (AV). (b) Theparasternal long axis cut looks remarkably normal. Note the mitral valve (MV) and right ventricular ou'low tract (RVOT). Otherabbreviations as above. (c) The subcostal paracoronal cut shows the right ventricle (RV) above the left ventricle (LV). The mitralvalve (MV) separates theLVfrom what used to be the right sided morphologically left atrium. (d) The apicalfour chamber cut showspronounced reversed offsetting of the mitral (MV) and tricuspid (TV) valves. Other abbreviations as above. A, anterior; L, left; R,right; S superior.

OperationAt operation a midline sternotomy was performed andthe pericardium resected for a patch. The outside

anatomy of the heart was as expected from angiocar-diography. Both atria were of left morphology. Theheart was moderately enlarged. The pulmonary artery

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Fig. 3 Case 3: cross sectional echocardiograms in theparasternal short axis cuts showing (a) the systemic venous atrium(SVA), formerly the left sided left atrium, draining via thetricuspid valve (TV) to the right ventricle (RV), which windsaround the left ventricular outflow tract (LVOT); and (b) at aslightly higher level, the right ventricular outlow tract (RVOT)leading out to the pulmonary trunk and left pulmonary artery(LPA), which partly encircle the aorta (Ao). Note that theseshort axis views are orientated more or less as the mirror image ofnormal, indicating counterclockwise spiralling great arteries. A,anterior; L, left.

ascended to the right of the aorta, having arisen fromthe left sided morphologically right ventricle. Theaorta was posterior and to the left, arising from theinferior and right sided morphologically left ventricle.The superior vena cava and hepatic veins entered theright sided morphologically left atrium, which was

connected to the morphologically left ventricle, whilethe left sided morphologically left atrium received thepulmonary veins and emptied into the morphologi-cally right ventricle.

In view of the rather small volume of the right sidedleft atrium, it was decided to perform the Mustardrather than the currently favoured Senning operation.After cannulation of the aorta, superior vena cava,and common hepatic vein, cardiopulmonary bypass

was started and the patient cooled to 20°Cnasopharyngeal temperature. The aorta was crossclamped and cardioplegia solution was infused to theroot of the aorta. The right sided atrium was openedand the anatomy visualised. The right sided atriumopened into the morphological left ventricle through avalve which had all the characteristics of a mitralvalve. The remnant of the atrial septum was resectedand then the left atrioventricular valve visualised. Itwas a tricuspid valve. A ventricular septal defect wasnot found after careful inspection of the ventricularseptum. It was therefore assumed that it had closed inthe four years between cardiac catheterisation andoperation. Mustard's operation with the modificationsas usually practised in our unit8 was then performed.When completed, the cross clamp was removed, airevacuated from the heart, the patient rewarmed, andperfusion discontinued. At the end of the operation,the patient was in sinus rhythm with the followingpressures: right ventricular systolic 40 mm Hg, pul-monary artery systolic 35 mm Hg, left ventricular sys-tolic 80 mm Hg, aortic systolic 80 mm Hg, superiorvena caval mean 12 mm Hg, and pulmonary venousatrial mean 10 mm Hg.

His postoperative progress was entirely uneventfuland he was discharged two weeks postoperatively toreturn home. Before discharge, a cross sectionalechocardiogram showed typical features of ventricularinversion, except that there was ventriculoarterialconcordance. Parasternal short axis cuts appeared as amirror image of the normal appearance, because ofthe counterclockwise spiralling of the great arteries(Figs. 2 and 3).

Discussion

These patients illustrate clearly the two ways in whichthe pulmonary and systemic circulations can beswitched at atrioventricular level. The first twopatients had atrioventricular discordance in that theright atrium was connected to the left ventricle andthe left atrium to the right ventricle. The third, whohad two atria, both of which were morphologicallyleft, can only be described as having an ambiguousatrioventricular connexion. In planning surgicalrepair, however, it was helpful to note that the pul-monary veins drained to the left sided atrium and thesystemic veins to the right sided atrium and to treatthe patient as if he had situs solitus with atrioventricu-lar discordance. Strictly speaking, therefore, the seg-mental anatomy would be described as left isomerism(quasi solitus) with an ambiguous atrioventricularconnexion (quasi discordant) and ventriculoarterialconcordance.9Both conditions described here are extremely rare,

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Table Details ofpatients in the present study and in previous reports with ventniculoarterial concordance and atrioventricular discordance orquasi concordance

Case No Reports Age Apex Situs AV connexion Great arteries

Spiral Ao-PA1 Lev,2' van Praagh" 4 wk L Solitus Discordant Clockwise R2 Lev," 12 yr L Solitus Discordant Parallel AR3 van Praagh2' 4 mnth L Solitus Discordant Clockwise PR4 Espino-Velal3 19 yr L Inversus Discordant Clockwise PR5 Clarkson' 2 yr R Inversus Discordant Clockwise PL6 Anderson26 3 mnth R Inversus Discordant ?Parallel AL7 Squarcia27 1 yr R Inversus Discordant Clockwise PR8 Quero-Jimtnez28 7 mnth L Solitus Discordant Clockwise PR9 Quero-Jimenez28 5 mnth L Solitus Discordant Clockwise PR10 Fox2 R Inversus Discordant11 Fox2 L Solitus Discordant12 Zakheim29 4 wk L Solitus Discordant Parallel AR13 Hazan3 9 yr R Quasi inversus Quasi discordant Clockwise PL14 Attie20 L Solitus Discordant ?Counterclockwise PL15 Attie20 L Solitus Discordant ?Counterclockwise PL16 Attie20 L Solitus Discordant ?Clockwise PR17 Attie'8 4 mnth L Solitus Discordant Clockwise PR18 Leijala6 4 mnth R Inversust Discordantt Clockwise PR19 Calabrb'° 2 mnth R Solitus Discordant Clockwise PR20 Ostermeyer4 10 yr R Solitus4 Discordant* ? PL21 Ostermeyer4 10 mnth L Solitus Discordant Clockwise PR22 Snider' 3 wk L Solitus Discordant Clockwise PR23 Present report 3Y2 yr L Solitus Discordant Clockwise R24 Present report 4 mnth L Solitus Discordant Clockwise PR25 Present report 16 yr L Quasi solitus Quasi discordant Counterclockwise PL

AL, anterior and leftward; AR, anterior and rightward; ASD; atrial septal defect; AV, atrioventricular; AVSD, atrioventricular septal defect; Acc,accessory; CAVO, common atrioventricular orifice; Coarc, coarctation of the aorta; Cont, continuation; IVC, inferior vena cava; L, left; LAW, leftatrioventricular valve; LJAA, left juxtaposition of the atrial appendage; MS, mitral stenosis; MV, mitral valve; PA, pulmonary artery; PAPVC, partialanomalous pulmonary venous drainage; PDA, persistent ductus arteriosus; PL, posterior and leftward; PR, posterior and rightward; PS, pulmonarystenosis; R, right; RA, right atrium; RAA, right aortic arch; RJAA, right juxtaposition of the atrial appendage; RV, right ventricle; SAS, subaorticstenosis; TAHVC, total anomalous hepatic venous connexion; TAPVC, total anomalous pulmonary venous drainage; TV, tricuspid valve; VSD,ventricular septal defect.*Architectural pattern.tPossibly quasi inversus and quasi discordant.tPossibly quasi solitus and quasi discordant.

there being only 22 reports so far published in theEnglish language (Table). Since both are associatedwith a single switch in the circulation both may beconfused clinically with complete transposition of thegreat arteries. The irony of our first case is thatbecause total anomalous pulmonary venous returnresulted in equalisation of the systemic and pulmon-ary arterial oxygen saturations, and angiocardiogra-phy was suboptimal, neither complete transpositionnor isolated atrioventricular discordance'0 was sus-pected preoperatively. Careful evaluation of theangiocardiograms with particular attention to the ven-tricular morphology should have established the cor-rect diagnosis. Though cross sectional echocardiogra-phy was undertaken only postoperatively and only intwo patients, the features were sufficiently striking tosuggest that the diagnosis could have been reachedbefore cardiac catheterisation, as reported by Sniderand colleagues.5The Table lists those patients in English language

reports with a similar sequential chamber organisa-tion. Three cases previously designated as having"isolated ventricular inversion"" or "ventricularinversion without transposition'2 13 have not been

included since they definitely or almost certainly hadan absent atrioventricular connexion.The first step in the diagnosis of atrioventricular

discordance is to establish atrial situs. 14 This is not aseasy as it seems. In Clarkson's case there was situssolitus of the abdominal viscera and situs inversus ofthe atria and bronchi.' In our third case, the bronchialpattern'5 16 and atrial appendage morphology as seenat operation indicated left atrial isomerism. This leadsus to suspect that the case of Hazan and colleagues3had left isomerism rather than situs inversus. Therewas an azygos continuation of the inferior vena cavaon the left, a common atrium, and a completeatrioventricular septal defect, all commonly associatedwith left isomerism.9 In the lateral angiocardiogram,both pulmonary arteries looped posterior to thetrachea, a pattern described by Soto and colleagues ascharacteristic of left isomerism.'7 Similar, but not soclear cut, considerations apply to the heart describedby Leijala and colleagues6 and the first case of Oster-meyer and colleagues.4Thus some diversity exists at the atrioventricular

junction in these patients, with even a crisscross rela-tion having been recorded.'8 There is even more

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Ventricular Mitral-aortic Assoctated defects Treatment Resultmorphology* continuity

L No Coarc, VSD, PDA Medical DiedL VSD, PS Modified baffles DiedL Yes VSDs PA banding DiedR TAPVC (RA), ASD, VSD, PS, MS L Blalock shunt DiedR Yes ASD Mustard operation AliveR No VSD Medical DiedR VSDL Yes VSD, PDA, hypoplastic TV, supravalvar ring Blalock-Hanlon shunt, PA banding DiedL Yes VSD, RJAA, hypoplastic RV Medical DiedR VSD Mustard, VSD closed AliveL VSD, PAPVC Mustard, VSD closed DiedL No RAA, acc MV tissue, SAS, VSD, PDA PDA ligation, atrial septostomy DiedR Yes Common atrium, CAVO Mustard operation AliveLLL ? ?L Crisscross, LJAA, VSD, RV hypoplasia Medical DiedR Heniiazygos cont on left, TAHVC (RA) Mustard operation AliveL Yes Straddling TV, SAS, VSD, Coarc, PDA Medical DiedL Yes AVSD, partitioned orifices, PAPVC, Mustard, LAW repair Alive

TAHVC (right sided atrium)L Yes VSD Mustard, VSD closed AliveL Yes VSD Senning operation AliveL No VSD, TAPVC Repair of TAPVC DiedL No VSD, PFO Mustard, VSD closed AliveL Yes Aneurysm, membranous septum, interrupted Mustard operation Alive

IVC, azygos cont, right aberrant, rightsubclavian artery

diversity at the ventriculoarterial junction, despite thefact that all have ventriculoarterial concordance.Firstly, there is the question of mitral-aortic con-tinuity or discontinuity. This seems of relatively smallimportance, particularly in the light of the wide rangeof normality which exists in normal hearts. '9 Of con-siderably more importance, particularly to theechocardiographer, is the relation of the great arteries.If the aorta is posterior and the great arteries spiralclockwise, then the parasternal short axis cut or scanwill show the "sausage", corresponding to the rightventricular outflow tract and pulmonary trunk, in itsnormal position to the left of the "circle", the aorta.On the other hand, if (as in Fig. 3) they spiral coun-terclockwise the picture obtained will be the mirrorimage of this. Therefore it is vital to ensure that left-right orientation is correct when the echocardiogramis recorded.As the Table shows, whether or not there is situs

solitus or inversus the great arteries almost always infact spiral clockwise. In fact, our third case is the firstreported in which the arteries definitely spiralledcounterclockwise, though this was probably thearrangement in two cases described by Attie and col-leagues.20 Given that clockwise spiralling of the greatarteries is the norm, whatever the atrial situs, thefrailty of qualifying such terms as ventricular or atrialinversion or non-inversion, ventricular discordance,or atrioventricular discordance with the adjective"isolated"'I 1021 becomes immediately obvious. After

all, what should be regarded as the normal relation ofthe great arteries in, for example, situs solitus withatrioventricular discordance and a left hand pattern ofventricular architecture? Is it determined by the atrialsitus or the ventricular architecture? If the atrial situsis the determinant, then isolated ventricular inversionimplies clockwise spiralling, whereas if ventriculararchitecture is the determinant, isolated ventricularinversion implies parallel or counterclockwise greatarteries. The concept of chirality was emphasised byvan Praagh et al with relation to atrial and ventriculararchitecture, when they pointed out, for example,that a normal right ventricle (in a D-loop) cannot besuperimposed on an inverted right ventricle (in anL-loop), since one is a stereoisomer of the other.22Precisely the same argument applies to spiralling greatarteries. Clockwise spiralling great arteries cannot besuperimposed on counterclockwise ones. It is there-fore difficult to believe that two hearts, both with situssolitus and atrioventricular discordance with a lefthand pattern of ventricular architecture, are the resultof the same disturbance of morphogenesis if in oneheart the great arteries spiral clockwise and in theother counterclockwise. Though physiologically thesehearts are identical, anatomically they are quite dis-tinct.

Nevertheless, as far as surgical management ofthese conditions is concerned, spiralling of the greatarteries is unimportant compared with correcting forthe atrioventricular discordance or quasi discordance.

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Even if the fact that single unsuccessful surgical casereports are rarely published is borne in mind, it isimpressive that all patients save one who are reportedto have an interatrial baffle procedure survived. Sucha form of repair has the particular advantage that themorphological left ventricle remains supporting thesystemic circulation.

FJM is supported by the British Heart and VandervellFoundations. This work was supported in part by theChild Health Research Appeal Trust.

References

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