Br Heart J 1981; 45: 20-8

Rastelli procedure for transposition of the greatarteries, ventricular septal defect, and left ventricularoutflow tract obstruction*Early and late results in 41 patients (1971 to 1978)A L MOULTON,t M R DE LEVAL, F J MACARTNEY, J F N TAYLOR,J STARK

From the Thoracic Unit, The Hospital for Sick Children, Great Ormond Street, London

SUMMARY Forty-one children with transposition of the great arteries, ventricular septal defect, and leftventricular outflow tract obstruction underwent a Rastelli operation between 1971 and 1978. Ahomograft valve preserved in an antibiotic solution and extended with a Dacron tube was theconduit of choice. Alternatively, conduits with porcine heterografts or valves constructed from calfpericardium were used. They were positioned to the left of the aorta whenever possible. Theintraventricular tunnel from the left ventricle to the aorta was constructed from Dacron velour. Therewere four early and seven late deaths. The last 13 consecutive patients have survived. Early deathswere related to unfavourable anatomy, conduit compression, and sepsis. Residual ventricular septaldefects and postoperative infection were the main factors contributing to the late deaths.

The combination oftransposition ofthe great arteries,ventricular septal defect, and left ventricular outflowtract obstruction is uncommon. It occurred in 97(0-67%) of the 15 104 patients with congenitalheart disease reviewed by Keith and colleagues';but significant left ventricular outflow tract ob-struction occurred in 28 to 31 per cent of patientswith transposition of the great arteries plus ventri-cular septal defect.2 3 Early attempts to resect theleft ventricular outflow tract obstruction in com-bination with a Mustard procedure and closure ofthe ventricular septal defect usually failed to relieveobstruction completely.4-6

After the first clinical use of an unvalved peri-cardial tube as an extracardiac conduit,7 theexperimental work of Arai and colleagues,8 and thefirst clinical application of an aortic homograft asa conduit,9 Rastelli'0 introduced a new procedurefor the treatment of patients with the combinationof transposition of the great arteries, ventricularseptal defect, and left ventricular outflow tract

* This work was supported in part by a British Heart Foundationgrant.t Present address: Division of Thoracic and Cardiovascular Surgery,University of Maryland Hospital, 22 S. Greene Street, Baltimore,Maryland 21201, USA.

obstruction. He suggested diverting left ventricularoutput to the aorta by occluding the proximalpulmonary artery and creating a conduit withinthe right ventricle which would carry blood ejectedthrough the ventricular septal defect into the aorta.Right ventricular flow would be carried to thedistal pulmonary trunk by means of an externalaortic homograft conduit. This achieved completebypass of left ventricular outflow tract obstructionand an anatomical as well as physiological correction(Fig. la-c).The purpose of this paper is to review the

complete series of patients who have undergonethis type of operation for these three anomaliesat The Hospital for Sick Children, Great OrmondStreet, London.

Subjects

Between 1971 and 1978, 41 children underwentRastelli's operation for transposition of the greatarteries, ventricular septal defect, and left ventricularoutflow tract obstruction (some of these childrenwere subjects of previous reports" 12). There were30 male and 11 female subjects in the group, rangingin age from 2 years 3 months to 14 years 10 months

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Ao PA Ao PA PA

CONDUIT

aI

Fig. la, b, c Principle of Rastelli's operation for transposition of the great arteries, associated with ventricular septaldefect and left ventricular outflow tract obstruction. Ao, aorta; PA, pulmonary artery; RV, right ventricle; LV, leftventricle; VSD, ventricular septal defect; LVOTO, left ventricular outflow tract obstruction.

(median 7 years 9 months). At the time of operationthe children weighed from 12-5 kg to 50 kg (median20 3 kg). All patients were cyanotic at the time ofoperation. Aortic oxygen saturations were 55 to 88per cent (median 78%) and haemoglobin levels11 4 to 24 8 g/100 ml (median 19 69 g/lOO ml).

Thirty-three patients had undergone previouspalliative procedures (Table 1). Some patientshad one or more operations. One 25-year-old childunderwent a Mustard operation with closure ofventricular septal defect and attempted relief ofleft ventricular outflow tract obstruction. Becauseof severe postoperative heart failure he requiredreoperation and a Rastelli procedure was success-fully performed.

OPERATIVE TECHNIQUEThe principle of the technique described inRastelli's original paper has been followed. Aftera median sternotomy, any functioning shunts, theductus arteriosus (if patent), and proximal pul-monary arteries are dissected. The conduit isprepared before establishing cardiopulmonary by-pass and a Dacron portion preclotted. At present, weprefer a fresh aortic homograft preserved in anantibiotic solution. The homograft is obtained assoon as possible after death. It is placed in a valve-

Table 1 Previous operations

None 8Pulmonary artery banding 2Balloon atrial septostomy 13Blalock-Hanlon septectomy 10Blalock-Taussig shunt 24Other aortopulmonary shunts 5

preserving nutrient antibiotic solution, containing:medium 199 with no sodium bicarbonate (10 ml),preheated "Cals" serum No. 1 (8 ml), 4A4 per centsodium bicarbonate (5 ml), sterile distilled water(77 ml), nystatin (250 000 units in 10 ml), methi-cillin (1000 mg 1-5 ml), erythromycin 600 mg in12 ml, gentamicin 400 mg in 10 ml, and strepto-mycin 20 000 units.The coronary ostia of the homograft are over-

sewn, the mitral valve and adjacent ventricularmuscle trimmed, and a Dacron tube of matchingdiameter is then anastomosed to the "ventricular"end of the homograft. If a suitable homograft isnot available, we use the Hancock conduit (Dacrontube with a gluteraldehyde-preserved porcinevalve). Other alternatives are pericardial or dura-mater valves inserted into the Dacron tube.The aorta is cannulated close to the origin of

the innominate artery with a right-angled metalcannula. Right angled Rygg cannulae are introducedthrough the right atrial appendage and the rightatrium just above the inferior vena cava. Pre-existingshunts are occluded. A Waterston shunt is eithercompressed or the pulmonary artery is snaredon either side of the shunt; a Potts anastomosis isoccluded by digital pressure through the leftpulmonary artery; Blalock-Taussig shunts orpersistent ductus arteriosus are ligated beforecardiopulmonary bypass is established. Cardio-pulmonary bypass with a flow of 2-4 1/min per m2is used and the perfusate cooled to 20 to 250C. Theleft ventricle is vented through the left atrium orthrough the left ventricular apex. The aorta iscross-clamped and cold (4°C) cardioplegic solutioncontaining St. Thomas's solution13 is infused intothe aortic root until the myocardial temperature is

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lowered to 8 to 12°C. The right atrium is opened AOand cardioplegic solution partly aspirated with adiscard sucker. The pericardial cavity is alsofilled with the cold solution. When the nasopharyn-geal temperature falls to 22 to 25°C, perfusion ;flow is reduced to 1-8 to 1-2 1/min per M2. Short _periods with an even lower flow or circulatoryarrest have been used.The intraventricular anatomy is assessed through

the right atrium and tricuspid valve to evaluatethe feasibility of performing the Rastelli procedure.The ventricular septal defect must be adequate indiameter to allow a completely unobstructed outflowfrom the left ventricle; this may involve enlarge-ment. The position of the aorta and the tricuspidvalve with its subvalvar apparatus must permitplacement of the patch to redirect the left ventri-cular outflow into the aortic root.

Visualisation of the papillary muscles andchordae of the tricuspid valve helps to select theoptimal ventriculotomy site. The ventriculotomyis an oblique or vertical incision directed towardsthe main pulmonary artery. Care must be takento avoid damage to major branches of the coronaryarteries. In the first patients in this series, Rastelli's

Fig. 3 Left ventricular flow is diverted to the aortathrough the ventricular septal defect using a large patch.Ao, aorta.

original description was followed, that is with aconduit lying to the right of the aorta. Since 1973we have directed the ventriculotomy incisionand the conduit to the left of the aorta wheneverthe main pulmonary artery is to the left of orbehind the aorta. In this position, the conduitdoes not cross the midline, and compression by thesternum is avoided. We do not resect a button ofmyocardium, though very hypertrophied right ven-tricular musculature at the edge of the ventriculo-tomy is often resected.The ventricular septal defect is again inspected

via the ventriculotomy. If its diameter is smallerthan the aortic valve annulus, it is enlarged by

-_

incision or resection of the septum towards thelateral-superior aspect of the defect (Fig. 2). Ifaccessible, the leaflets of the pulmonary valve areoversewn through the ventricular septal defect,though this may also be done later via the pulmonaryartery. A generous patch of Dacron velour is thenfashioned to direct blood from the left ventriclethrough the ventricular septal defect into the aorta(Fig. 3). Interrupted mattress sutures with Teflon

Fig. 2 The ventricular septal defect viewed through the pledgets are usually used. If running Prolene isright ventriculotomy; the area available for ventricular used, it is reinforced with several pledgetedseptal defect enlargement is cross-hatched. mattress sutures. The suture line starts at the lower

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corner of the ventricular septal defect, near thetricuspid valve and runs along the border of theventricular septal defect (with the usual care toavoid the conduction system) to the anterior rightventricular wall, to provide an adequate leftventricular outflow channel. In five patients, thetricuspid chordae and papillary muscle apparatuscrossed the ventricular septal defect and precludednormal placement of the ventricular septal defectpatch. In one patient the tricuspid valve wasreplaced; in three others a papillary muscle was

detached and reattached. In one patient, a 26 mmDacron tube was successfully inserted as an

intraventricular conduit between the ventricularseptal defect and aorta, coursing among the tri-cuspid chordae.The proximal pulmonary artery is now ligated.

We prefer this to transection because bleedingfrom the relatively inaccessible ventricular end ofthe pulmonary artery has been bothersome. The

s&

Fig. 4 The conduit is attached distally to the pulmonaryartery. The proximal anastomosis to the right ventricle isstarted. The initial stitches are anchored to the upperborder of the ventricular septal defect patch. Insert showsoblique trim to the proximal end of the conduit.

Table 2 Operative technique in 41 patients

ConduitComposite homograft 19Porcine heterograft 22

VSD enlarged 36Resuspension tricuspid papillary 3Replacement tricuspid valve 1Intraventricular Dacron tube 1

Pulmonary arteryDivided and oversewn 14Ligated only 16Ligated and pulmonary valve oversewn 9Pulmonary valve oversewn only 2

Relation conduit to aortaLeft 23Right (1-TGA in six patients) 18

VSD, ventricular septal defect; TGA, transposition of the greatarteries.

pulmonary trunk distal to the ligature is openedwidely, usually from the left to the right pulmonaryartery. The conduit is trimmed so that the valveis as close to the pulmonary artery as possible. Thevalve is thus protected by the large aorta, whichin our view diminishes the possibility of valvecompression or distortion. The conduit is stitchedto the pulmonary artery with a running sutureof Prolene. When the distal anastomosis is com-pleted, the cross-clamp is released and the aircarefully evacuated from the aortic root and leftventricular vent. The conduit is fashioned andsutured to the margins of the ventriculotomy,incorporating the upper edge of the ventricularseptal defect patch (Fig. 4). The proximal anasto-mosis is performed on a beating heart while thepatient is being rewarmed, with a second sumpplaced in the right ventricle via the tricuspidvalve. A patent foramen ovale or atrial septaldefect is closed. The right atriotomy is thensutured and cardiopulmonary bypass terminatedin the usual manner when the body temperaturehas returned to normal. In none of the patientsin this series did we have to delay sternal closure,or excise any of the sternum to accommodate theconduit. Details of the operative technique usedare summarised in Table 2.

Early results

The postoperative course is summarised in Table3. Inotropic agents were liberally administered,usually before a low cardiac output syndrome haddeveloped. One of the two patients in whom anintra-aortic balloon was inserted survived, thoughhe needed embolectomy and has a residual peronealpalsy with foot drop. Ventilatory support for several

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Table 3 Postoperative course in 37 early survivors

Inotropic support 19Intra-aortic balloon pump 1Renal failure, dialysis 3Tracheostomy 2Early reoperations 9Mediastinal debridement 2Laparotomy (bleeding caused by dialysis catheter) 1

days postoperatively was common, two patientsrequired tracheostomy for prolonged mechanicalventilation. All patients were noted to be in sinusrhythm with right bundle-branch block at thetime of discharge. Only one patient (a long-termsurvivor) had associated left anterior hemiblock.Four of the 41 patients undergoing the Rastelli

procedure (9.9%) died in the hospital (Fig. 5)but there have been no deaths in the 13 patientsoperated on since January 1977. One patient died onthe table. Because of the long distance between theaorta and the ventricular septal defect (which wascrossed by an anomalous muscle band), there wasa 40 mmHg gradient between the left ventricleand aorta at completion of the repair. After revisionof the ventricular septal defect patch, the patientstill could not be weaned from bypass.Three patients died in the immediate post-

operative period with a low cardiac output. One hadrequired tricuspid valve replacement as part of therepair and died two days later despite treatmentwith the balloon pump. One patient died fourdays postoperatively and was suspected of havingconduit compression as the conduit had been placedacross the midline to the right of the aorta. Thethird patient died 15 days after operation withcontinued low cardiac output, peritoneal dialysisfor renal failure, and proven sepsis.

After early reoperation for mediastinal infection,he developed a residual ventricular septal defectand tricuspid regurgitation and died with aorticdissection at attempted reoperation 10 weekslater. Necropsy showed that the ventricular septaldefect patch had torn away, creating holes in theseptal leaflet of the tricuspid valve though thereattached papillary muscle was intact. The otherpatient, who had undergone early reoperation formediastinal infection, died suddenly five monthsafter operation with massive haemoptysis. Necropsywas not performed. The fifth child had an earlypostoperative fever and leukocytosis but positiveblood cultures were not obtained until 15 monthslater, just before his death. The sixth late deathresulted from damage to the left coronary artery,which originated high on the right side of the aorta.This origin was obscured by adhesions and thedamage was caused during reoperation for residualventricular septal defect nine months after theoriginal Rastelli procedure. The seventh childunderwent uneventful repair of a residual ventri-cular septal defect 14 months after operation,but died nine days after hospital discharge frompulmonary embolism.

RASTELLI PROCEDURE - 41 PATIENTSHOSPITAL DEATHS

In theatre Unsuitdbleanatomy

2 days Balloon pump

4 days Low cardiac Conduitoutput compresslon

15 days Renal failure,septicaemia

Fig. 5 Rastelli procedure in 41 patients. The illustrationshows the hospital deaths.

Late results

Table 4 lists the causes of the late deaths from the37 early survivors. Two patients died suddenlythree months and three years postoperatively. One ofthese patients had an abnormal tricuspid papillarymuscle detached and resuspended on the ventricularseptal defect patch during the Rastelli procedure.

Table 4 Late deaths in 37 early survivors

Sudden 3 mth, 3 y 2Aortic rupture at reoperation 10 wk IHaemoptysis 5 mth 1Sepsis 15 mth 1Damage to the coronary artery at

reoperation 9 mth 1Pulmonary embolus 14 mth I

Two other patients developed right ventricularaneurysms associated with residual ventricularseptal defect and are doing well after reoperationwith conduit replacement.14 These were the onlytwo cases in our series requiring conduit replace-ment.

Another three patients had ventricular septaldefects documented on postoperative cardiaccatheterisation; one had spontaneously closed ina later study, one had pulmonary vascular diseasebut was functioning well despite tricuspid re-gurgitation and moderate right ventricular failure,while the third is doing well on no medications.Five patients have clinically suspected ventricularseptal defects but are asymptomatic on maintenancedigoxin. The actuarial survival curve for the

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present group of patients is given in Fig. 6. Com-parative results of other series of Rastelli's procedurefor transposition of great arteries plus ventricularseptal defect plus left ventricular outflow tractobstruction are listed in Table 5.15 16

Discussion

The combination of these three anomalies isuncommon and the causes of the left ventricularoutflow tract obstruction are multiple. Isolatedvalvular stenosis is rare, though it may occur inassociation with other lesions."7 Subvalvular stenosismay be caused by a fibrous shelf, a fibromusculartunnel, herniation of accessory tricuspid tissuethrough the ventricular septal defect, abnormalattachment of the mitral valve, aneurysm of themembranous ventricular septum, or septal hyper-trophy.18-21

Direct attempts to resect this stenotic area incombination with a Mustard operation and ventri-cular septal defect closure may not alleviate thegradient-because of the arrangement of themitral valve, ventricular septum, and coronaryarteries.22 More recently, good results with therelief of left ventricular outflow tract obstructioncombined with the Mustard operation have beenachieved.2' 24 However, the Rastelli procedureoffers several advantages in this group of patientsby providing adequate relief of the left ventricularoutflow tract obstruction while transferring theleft ventricle and mitral valve to the systemic arterialcirculation. It should, therefore, avoid any concernabout late right ventricular function and the tri-cuspid regurgitation sometimes seen after the

Table 5 Results of Rastelli operation

Institution Period No. of Early and Mortalitypatients late deaths (%)

Mayo Clinic* 1968-75 59 16 27Great Ormond St. 1971-78 41 11 27Boston Children'st 1972-76 7 1 14

*Marcelletti et al.'5tNorwood et al."6

Mustard procedure.'5 26 Obstructions to pulmonaryand systemic venous return and post-Mustardarrhythmias will be avoided.The long-term success of the Rastelli procedure

will also depend upon continuing satisfactoryfunction of the conduit as well as on myocardialperformance. Early repairs used aortic homograftsto restore continuity between the right ventricleand the pulmonary artery. Reports of calcification,the development of high gradients, and valvularregurgitation in the aortic homograft,21-29 as wellas limited availability, have led to the introductionof a commercially available gluteraldehyde-preserved porcine heterograft valve in a Dacrontube.30Our experience and that of others3"-33 has shown

that relatively fresh antibiotic sterilised homograftswill function well over more than 10 years. Theyare easier to handle and better haemostasis isachieved. We, therefore, prefer to use a freshaortic homograft preserved in an antibiotic solution,when available.Some of the gradients between the right ventricle

and pulmonary artery are related to technical

0-8001

060 Fig. 6 Actuarial survival curvefor patients undergoing Rastelliprocedure for transposition ofgreat arteries, ventricular septaldefect, and left ventricular outflowtract obstruction.

04001-

02002c

2 3 4 5 6 7 8Y-erYears

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factors rather than the type of conduit used.34 35Completely asymptomatic patients may havesignificant gradients and some urge routine cardiaccatheterisation after the Rastelli procedure.'6 Re-operation for conduit stenosis was necessary in twopatients out of 37 early survivors (5°h) in this series,in contrast to eight patients (17%) of early survivorsin the Mayo Clinic series.15 If the ventricular septaldefect is restrictive, it must be enlarged in thefirst instance. In the Mayo Clinic series1' the ventri-cular septal defect was enlarged in 35 per cent ofthose patients undergoing the Rastelli procedure;early mortality in this group was only 10 per cent,as opposed to a mortality of 24 per cent in thosepatients whose ventricular septal defects werejudged "adequate" without enlargement. Theventricular septal defect was enlarged in 80 percent of our patients. The enlargement did notaffect early or late mortality.

Residual ventricular septal defects are seen morefrequently after the Rastelli procedure than afterother operations for ventricular septal defect.35This could be related to the large patch necessaryto provide unobstructed outflow into the aorta.Anomalies of ventricular musculature and tricuspidchordae obscuring the margins of the ventricularseptal defect also increase the risk of a residualdefect. Five (14%) of the 37 early survivors in ourseries required reoperation for ventricular septaldefect (two with associated right ventricularaneurysm, one with concurrent tricuspid regurgita-tion) compared with five of 48 (1 1 %) in Marcelletti'sseries,"5 Three of our five patients died at or afterreoperation; therefore a residual ventricular septaldefect appeared to have an adverse effect onlong-term survival, though this impression couldnot be confirmed statistically.

Postoperative infectious complications have beena major problem in the series since they wereassociated with one early and three late deaths.We were unable to relate these infections to thetype of extracardiac conduit used, or to a particularantibiotic regimen. They were most probably aconsequence of the complexity of the surgicalprocedures involved, and the insertion of a largeamount of prosthetic material.

All patients in our series were discharged innormal sinus rhythm; only one patient-a long-term survivor-had associated left anterior hemi-block. Three patients had transient postoperativearrhythmias. Two late deaths were sudden andunexplained and could have been related toundiagnosed arrhythmias.The success of the Rastelli procedure depends

upon careful selection of patients as well as onmeticulous surgical techniques. The combination

of improved techniques of angiocardiography andsector echocardiography should enable accuratepreoperative diagnosis of most of the lesionsassociated with transposition, ventricular septaldefect, and left ventricular outflow tract obstructionwhich are likely to cause problems during operation.Tilted oblique projections of left ventricularangiocardiograms37 38 allow easy recognition ofthe small infundibular defect immediately underboth semilunar valves which may not be possibleto enlarge without resecting tissue inferiorly andthereby placing the penetrating bundle at risk.Such projections also readily identify the apicaltrabecular muscular defect which is too far fromthe aortic valve to permit insertion of an intra-ventricular conduit. In our experience, it is notalways easy to distinguish the inlet ventricularseptal defect, which is a long way from the aorticvalve, from the anterior trabecular defect whichis more appropriately sited, though it has beenclaimed that this is not difficult.'9 Identificationof a straddling tricuspid valve is possible by angio-cardiography,40 but is much more easily achievedwith sector scanning,4' which also permits recog-nition of malattachment of the tricuspid valveto the rim of the ventricular septal defect. Subaorticstenosis, when severe, is easily recognised by theresultant pressure gradient, but angiocardiographymay show infundibular subaortic stenosis which,though causing no obstruction preoperatively, maycontribute to left ventricular aortic gradientspostoperatively, unless it is relieved. Mitral valveanomalies must be assiduously sought for by thecombination of pressure measurements, angio-cardiography, and echocardiography. If the anatomyis unfavourable, other options for repair must beconsidered. These include a Mustard or Senningprocedure, ventricular septal defect closure andleft ventricular to pulmonary artery conduit,4' 43 orthe concept of biventricular conduits.43

We thank Mr K Ross, Mr J Munroe, Mr D NRoss, and Mr M Yacoub for supplying us with thehomograft valves, and Sister Siebert from thehomograft department, Southampton WesternHospital.

Note: Fig. 1 to 6 have been reproduced with kindpermission of the publishers from Stark J. TheRastelli operation. In: Operative surgery, cardio-thoracic surgery. London: Butterworths, 1978:130-5.

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Requests for reprints to Dr J Stark, ThoracicUnit, The Hospital for Sick Children, GreatOrmond Street, London WC1N 3JH.

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