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n the Footsteps of Senning: Lessons Learned Fromtrial Repair of Transposition of the Great Arteries

li Dodge-Khatami, MD, PhD, Alexander Kadner, MD, Felix Berger, MD,itendu Dave, MD, Marko I. Turina, MD, and Rene Pretre, MD

ivisions of Cardiovascular Surgery and Cardiology, Center for Congenital Heart Diseases, Children’s Hospital, University ofurich, Zurich, Switzerland

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he Senning operation has evolved from being the initialurgical correction that allowed survival in completeransposition of the great arteries to an integral part ofhe anatomic repair of congenitally corrected transposi-ion. In patients with complete transposition, the Sen-ing operation has given satisfactory initial and long-erm surgical results, but the potential for right
entricular failure and atrial arrhythmias have drastically
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Strasse 75, CH-8032 Zurich, Switzerland; e-mail: [email protected].

2005 by The Society of Thoracic Surgeonsublished by Elsevier Inc

educed its indications in the current era. The long-termollow-up and pertinent postoperative issues of the Sen-ing operation will be reviewed, along with its new-ound role in the anatomic repair of congenitally cor-ected transposition.

(Ann Thorac Surg 2005;79:1433–44)
© 2005 by The Society of Thoracic Surgeons
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n 1958 in Stockholm, Sweden, Ake Senning performedthe first procedure that would later bear his name. It

as initially conceived to be the complete and definitiveurgical correction for transposition of the great arteriesTGA) [1]. In 1961 Senning moved to Zurich, Switzerlandnd performed all atrial switches at Children’s Hospitalf Zurich from 1962 to 1978, after which Marko I. Turinaerformed the rest of our series until 2003, for a total of45 patients.Without this operation, the natural history of patientsith all variants of TGA was dismal, with 55%, 85%, and

0% mortality rates at 1 month, 6 months, and 1 year,espectively [2]. This ingenious procedure, also known ashe atrial or venous switch, involves rerouting the pul-

onary veins through the tricuspid valve to the systemicight ventricle (RV) by means of an atrial flap (fashionedrom the free wall of the right atrium) plus the redirectionf systemic venous blood from both vena cavae, throughhe mitral valve to the pulmonary left ventricle by usinghe intraatrial septum.

The initial results of this procedure were disappointing3], as may be seen by the 7 hospital deaths from a seriesf 11 patients (63.7% mortality) that was reported byirklin and colleagues [4] in 1961. The high mortality andifficulty in reproducing Senning’s own better experi-nce [5] motivated others to modify the procedure. Thisltimately lead to the Mustard operation in 1964 [6], inhich a pericardial baffle was inserted. Quaegebeur and

olleagues revived the Senning operation through tech-ical modifications, resulting in considerable improve-ent of in-hospital survival [7].Until the late 1970s, the atrial baffle operations were the

ddress reprint requests to Dr Dodge-Khatami, Division of Cardio-ascular Surgery, Children’s Hospital, University of Zurich, Steinwies-

nly established procedures for the repair of completeransposition, and with increased experience, surgical

ortality steadily decreased to low levels (1% to 9%) [8].owever, intermediate- to long-term survivors were be-

ng recognized with RV failure, systemic and pulmonaryenous pathway leaks and obstructions, varying degreesf tricuspid valve insufficiency, atrial arrhythmias, andnexpected late sudden deaths.Jatene successfully performed the first arterial switch

peration (ASO) in 1975, which increasingly gained pop-larity, was reproducible with an acceptable learningurve, and resulted in lower mortality rates than theenning operation. More important, it represented annatomic and physiologic repair of transposition, placinghe left ventricle (LV) in the systemic position, thusvoiding potential long-term RV failure that complicatedhe atrial baffle operations. This fact, and mortality thateaches zero in many centers that perform the ASO [9],ave made the Senning operation a palliative procedure.s a result, the Senning operation has become nearlybsolete in the surgical management of neonates withGA. It is important to note that the ASO transfers theulmonary valve to the systemic position, with the po-

ential for late neo-aortic valve incompetence, the long-erm significance of which is still unknown.

Renewed interest in the Senning operation hasmerged since the 1990s, as it is an essential part of thenatomic repair in patients with congenitally correctedransposition of the great arteries (CCTGA). Althoughhis relatively new strategy achieves anatomic and phys-ologic repair of CCTGA, the number of large series isimited and the follow-up is short. Theoretically, theong-term complications that have been witnessed after aenning operation for TGA could be anticipated afternatomic repair of CCTGA [10].In this review we present technical details of the

enning operation, summarize the results of larger re-

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1434 REVIEW DODGE-KHATAMI ET AL Ann Thorac SurgLONG-TERM ISSUES AND REVIVAL OF THE SENNING OPERATION 2005;79:1433–44

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ent series, and address its potential long-term iatrogenicnd physiologic implications. In a failing post-Senningeart, the indications for taking down an atrial baffle andetraining the left ventricle (LV) en route to an ASO wille discussed. It is hoped that the lessons learned from

he atrial repair of TGA will serve us to better treat thoseatients with CCTGA who need an anatomic repair, inhich the Senning operation has its newfound role.

aterial and Methods

e used the PubMed database (National Library ofedicine) to perform a computerized literature search by

nserting the key words “Senning,” “Mustard,” andatrial switch,” with no specific time frame.The data from our series and the ones presented in

able 1 and Table 2 were gathered from a retrospectivehart review of all consecutive patients who had a Sen-ing operation in each institution. Follow-up was per-

ormed by the respective cardiology teams in each hos-

Abbreviations and Acronyms

ASO � arterial switch operationCCTGA � congenitally corrected transposition of

the great arteriesCHSS � Congenital Heart Surgeons SocietyECMO � extracorporeal membrane oxygenationLV � left ventricle or left ventricularLVOTO � left ventricular outflow obstructionMRI � magnetic resonance imageryNR � not reportedNYHA � New York Heart AssociationPA � pulmonary arteryPHN � pulmonary hypertensionRV � right ventricle or right ventricularSVC � superior vena cavaTGA � transposition of the great arteriesTI � tricuspid insufficiencyVSD � ventricular septal defecty � years

able 1. General Results of Recent Series

enter PatientsOperativMortality

urich Current 345 14.3% (7% inthe last 4 y

HSS (Wells et al, 2000) [11] 173 14%ondon (Sarkar et al, 1999) [12] 141 6.4%elsinki (Kirjavainen et al, 1998) [8] 100 2%ashville (Bender et al, 1989) [13] 93 5.4%eiden (Helbing et al, 1994) [14] 68 8.8%oston (Marx et al, 1983) [15] 57 5%ortland (Reddy et al, 1996) [16] 54 9%ilwaukee (Litwin et al, 1987) [17] 40 0%

russels (Rubay et al, 1987) [18] 26 0%

Follow-up times in median years �/� standard deviation when availab

HSS � Congenital Heart Surgeons Society; NR � not reported; NYH

ital, by questionnaires sent to the patients residingutside of the country where the operation took place, oroth. The term operative mortality in Table 1 refers to anyeath that occurred within 30 days of surgery.

esults

urgical Techniquefter median sternotomy and pericardiotomy, the righttrium is marked with two stitches at the level of therista terminalis, with an equal distance between thearking stitches and the interatrial groove that is gener-

usly developed, indicating the site of the planned righttriotomy. This forms a square trap door that will bepened at the top and flipped downwards (Fig 1). Theechniques of cannulation and cardiopulmonary bypassre fairly standard and present no particularities.After cross-clamping, cardioplegia, and right atri-

tomy, an incision is made into the interatrial septumround the limits of the fossa ovalis (Fig 2). This creates aosteriorly based flap (Fig 3) that is lowered into the lefttrium and sutured above and around the orifices of theulmonary veins. This part of the procedure usually

nvolves the use of a small pericardial patch and aongitudinal incision in the coronary sinus that allows forn enlargement of the posterior portion of the pulmonaryenous atrium (Fig 4).The systemic venous tunnel is completed by suturing

he free edge of the right atriotomy around both cavalrifices and along the remaining cut rim of the atrialeptum (Fig 5). Pulmonary rerouting is accomplishedfter a horizontal incision is made into the left atrium,arallel to the interatrial groove. The free edge of theight atrium is brought down around the caval tunnelnd anastomosed to the opening in the interatrial groove.n Zurich, we have found it important at this stage to usegenerous in-situ pericardial flap that is left attached to

ts blood supply from the pericardiophrenic artery, thusssuring normal patch growth (Fig 5 insert). This modi-cation of Senning’s original technique has eliminated

LateMortality Follow-Up Intervala

NYHA Class Iat Follow-Up

8% 15.4 y range 0.7–33.3 y 73%

8% 10.0 y 59%9% 13.4 y range 0.32–17.9 y 92%8% 12.8 y range 6.2–18.4 y 85%1% 3.8 y range 0.8–9.4 y 97.5%

16.1% 11.0 y range 0.1–20 y 66%4% 1.1 �/� 0.7 y range 0.02–3 y NR0% 6.4 y range 0.5–12.1 y 94%5% range 0.5–5 y NR0% 4 y range 0.08–8 y 96%

llowed by range in years.

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A � New York Heart Association; y � years.

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1435Ann Thorac Surg REVIEW DODGE-KHATAMI ET AL2005;79:1433–44 LONG-TERM ISSUES AND REVIVAL OF THE SENNING OPERATION

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he incidence of pulmonary vein obstruction in oureries.

eview of Recent Surgical Serieshe results of recent larger series are summarized inable 1. Operative mortality of the Senning correctionanges from 0% to 15.7%; however, when the ratherisappointing results from the early years of our ownxperience are eliminated, mortality would be less than0%. In older series, higher early mortality was partiallyue to an initial learning curve, but more so to patient

able 2. Complications and Reoperations

enter Complications

urich Current 3 SVC stenosis, 2 severe TIHSS (Wells et al, 2000)[11]

Venous pathwaycomplications and RVfailure

ondon (Sarkar et al, 1999)[12]

1 SVC stenosis, 1 baffleleak, 2 LVOTO, 1 RVfailure

elsinki (Kirjavainen et al,1998) [8]

1 pulmonary vein occlusion1 severe TI

ashville (Bender et al,1989) [13]

1 baffle leak

eiden (Helbing et al,1994) [14]

9 TI

oston (Marx et al, 1983)[15]

6 pulmonary veinobstructions, 7 SVCstenosis

ortland (Reddy et al,1996) [16]

3 TI

ilwaukee (Litwin et al,1987) [17]

1 pulmonary vein stenosis,1 TI

russels (Rubay et al, 1987)[18]

2 mild SVC obstructions

VOTO � left ventricular outflow obstruction; nr � not reported;nsufficiency.

ig 1. Surgeon’s view and the proposed trap door right atrial inci-ion in dashed lines. The cannulas for cardiopulmonary bypass are
ot shown. (IVC � inferior vena cava; SVC � superior vena cava.) s
election [19], as illustrated by the number of youngatients undergoing the Senning operation with TGAlus ventricular septal defect (VSD) and pulmonary vas-ular obstructive disease. Late mortality is a troublesomeccurrence, seldom preceded by overt failure or activerrhythmias. Its incidence is reported from 0% to 16.1%n recent series, most often in the form of sudden death,ithout a detectable anatomic or physiologic risk factor

nd without relation to the time interval from operativeorrection. Other frequent complications are presentedn Table 2, and are discussed more in detail in theubsequent sections.

Reoperation Rate Pacemaker

10.2% 3 transplantations 3.8%6.9% 7.5%

3.8% venous pathway complicationsand LVOTO relief

1.5%

4% 1 pneumonectomy for pulmonaryvein occlusion, 1 transplantation,2 tricuspid valve operations

24%

1.25% 1 reoperation for baffle leak 3.75%

0% 4.8%

12% for systemic and pulmonaryvenous complications

1.9%

3.7% LVOTO relief 0%

2.5%, 1 reoperation for pulmonaryvenous obstruction

5%

0% NR

right ventricle; SVC � superior vena cava; TI � tricuspid valve

ig 2. The right atrium has been opened, with the proposed incisionn the interatrial septum (dashed line) for the future septal flap.ote the extension of this incision into the mouth of the coronary

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inus. (AV � atrioventricular.)

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ong-Term Follow-Up IssuesXERCISE TOLERANCE. The adequacy of the right ventricle toustain the systemic circulation in the long-term can beuestioned by its relative inefficient response to stressnd effort, as illustrated by multiple studies enrollingatients after a successful atrial switch who are otherwisesymptomatic, in sinus rhythm, and without medication.ouard and colleagues performed bicycle ergometry in

3 asymptomatic patients at a mean follow-up of 11 � 2.8ears after a Senning operation. They found reducederobic capacity, shorter exercise times, and lower max-mal heart rates, indicating an impaired chronotropicesponse to effort [20]. Exercise capacity was inverselyorrelated with the time interval elapsed since surgery,uggesting that better functional results can be antici-ated when the Senning operation is performed early.hey also found an excessive ventilatory adaptation toxercise, reflected by an increased respiratory rate, a

ig 3. The septal flap is dropped down into the left atrium andewn over the orifices of the pulmonary veins. Note the pericardialatch that is sutured to the septal flap, thus filling the defect left byhe foramen ovale, and enlarging the pulmonary venous atrium.SVC � superior vena cava.)

ig 4. The pulmonary veins have been covered by the septal flap.he white arrows show the redirected systemic venous blood flow,

rom the two caval veins towards the mitral valve. (IVC � inferior
sena cava; SVC � superior vena cava.)
elative lesser increase in tidal volumes, and increasedotal ventilation, as compared to controls [20].

Matthys and colleagues pinpointed the lack of increasen stroke volume to be the underlying mechanism of annefficient response to effort, stressing that RV dysfunc-ion can exist without chronotropic impairment [21]. Alsosing bicycle ergometry, Gilljam and colleagues [22]emonstrated low oxygen uptake, low maximal heartate, abnormal stroke volume response, and high totaleripheral resistance in 17 adolescent patients after antrial switch. The authors suggest contributing factors tonclude small and noncompliant atria with subsequentnadequate filling of the ventricles, ventilation-perfusionnequality, intrapulmonary shunts, and oxygen diffusionimitation between the alveoli and pulmonary capillaries22].

Buheitel and colleagues [23] compared exercise perfor-ance of patients after a Senning operation or a Fontan

ompletion with normal controls. They measured peakonsumption of oxygen, maximal work rate, peak oxygenulse, and end-expiratory pressure of carbon dioxide and

ound the poorest results in Fontan patients. The reactiono exercise was qualitatively identical between Fontanatients and those after a Senning operation, and com-arable to that of patients with chronic heart failure.uantitatively, they found the results of Senning patients

o lie between controls and Fontan patients [23].

IGHT VENTRICULAR FAILURE. After the atrial switch, the RVemains in the systemic circulation, similar to unoperatedatients with CCTGA. Numerous reports have demon-

ig 5. The systemic venous tunnel has been completed. The pedicledericardial flap is sutured to the opening in the left atrium, and itsree edge will be sutured to the opening in the right atrium, thusompleting the neo-pulmonary atrium. The white arrow shows theedirected flow of pulmonary venous blood from the left atrium to-ards the tricuspid valve, traveling over and around the systemicenous tunnel. The inset shows the completed repair, with the aug-ented pulmonary venous atrium, and branches of the pericardio-

hrenic artery. (IVC � inferior vena cava; SVC � superior venaava.)

trated the inadequacy of this ventricle to sustain the

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ystemic circulation in the intermediate and long term,ith RV dysfunction rates ranging between 4% and 16%

24–29]. RV failure seems to be more prominent andccurs earlier in patients with TGA plus VSD, than inhose with an intact interventricular septum [25, 27, 29].V failure is not a time-related event and can occur

nsidiously after a long period of apparent normal func-ion in an otherwise asymptomatic patient [26]. This haseen the major impetus towards not only abandoning theenning operation for TGA but also for converting antrial switch into an ASO and for promoting the anatomicepair in CCTGA, thus restoring the morphologic LV tohe systemic circulation.

Using radionuclide ventriculography in 99 patients at aedian of 13 years after an atrial switch, Reich and

olleagues [27] demonstrated systolic dysfunction notnly of the RV in 8% of patients, but also of the LV in 10%f patients. Diastolic dysfunction of the LV was present inp to 80% of patients and deteriorated with time [27].ubiszewska and colleagues [24] used myocardial perfu-ion imaging and radionuclide angiography to study 61atients at rest and at exercise at a mean of 10 years aftern atrial switch. Despite excellent exercise tolerance, RVystolic dysfunction was illustrated by a significantlyeduced RV ejection fraction in all patients, mild perfu-ion defects in 14.7% of patients, and extensive perfusionbnormalities in 54% of patients, more often in thenferior and anterior wall of the RV. Perfusion abnormal-ties were more pronounced in patients who were oldert the time of surgery and who had longer follow-upimes. Also, moderate-to-severe tricuspid valve insuffi-iency was more frequent in patients with abnormalerfusion [24].Confirming these results with a longer follow-up time

f between 10 and 20 years after an atrial switch opera-ion, Millane and colleagues [30] found perfusion defectsn 21 of 22 patients studied (95%) at rest, during dipyrid-mole stress testing, or both. More alarming, these per-usion defects were irreversible in 55% of patients, indi-ating infarction or fibrosis, more importantly so in thenterior, inferior, and septal segments of the systemicV. Concomitant wall-thickening abnormalities wereoted in 83% of segments with fixed perfusion defects,irrored by reduced wall motion [30].Labbe and colleagues reported similar results in 43

atients 11.3 � 3 years after a Senning operation by usinghallium myocardial scintigraphy [31]. In a study compar-ng patients undergoing either a Senning operation or anSO, Okuda and colleagues found reduced systolic

hortening of the anteroposterior diameter of the sys-emic RV only in the Senning patients [32].

In unoperated patients with CCTGA, a morphologicV sustains the systemic circulation and presents theame shortcomings as after a Senning correction. Hor-ung and colleagues [33] demonstrated reversible andxed perfusion defects in 5 unoperated patients withCTGA, correlating with regional wall motion, thicken-

ng abnormalities, and impaired RV contractility. Tu-evski and colleagues found similar results in 13 adult
atients with unoperated or physiologically repaired n
CTGA by using magnetic resonance imagery (MRI) andobutamine stress testing [34]. Both groups of authorsonclude that ischemia and infarction are importantauses of RV failure in patients with CCTGA, drawingarallels with the systemic RV after the atrial switchperation.Somewhat contrary to this evidence, Lorenz and col-

eagues [35], using cine MRI, found markedly elevatedV mass, normal RV size, and only mildly depressed RVjection fraction in 22 patients 8 to 23 years after an atrialwitch procedure. Only 1 patient had clinical RV dys-unction with increased RV mass, a finding also observedn only 1 out of 40 patients in the series from Milwaukee18]. They conclude that inadequate hypertrophy of theV is not the cause of late RV dysfunction in patientsfter an atrial switch [35].Using radionuclide cineangiography, Hochreiter and

olleagues [36] studied 22 patients 8 to 18 years after antrial switch and found not only normal resting RV andV ejection fractions, but also preserved exercise endur-nce with normal RV ejection fraction at stress in patientsaving undergone their repair before the age of 1 year.hey and others [18, 37] suggest that deleterious factorsuch as chronic hypoxia may explain the suboptimalesults observed in older patients who undergo the atrialaffle procedure [36].The cause of impaired RV function is presently un-

lear, and the available data are still inconclusive as to itsmplication. The etiology is probably multifactorial, ei-her related to a late operation after chronic preoperativeyanosis and resultant RV ischemia, to suboptimal intra-perative myocardial protection, as was certainly the case

n older series that used more primitive cardioprotectiveechniques, or to the inherent suboptimal geometry ofhe RV [28, 38]. Given the existence of adult patientshose RV volumes, function, and response to exercise

re normal long after an atrial baffle procedure, it seemsnreasonable to condemn the Senning or Mustard oper-tions on the basis of inevitable RV dysfunction alone.

AFFLE STENOSIS OR LEAK. Systemic vena cava stenosis cor-esponds to a pullback pressure difference of more thanmm Hg during catheterization [39]. Surprisingly, symp-

omatic caval obstruction is relatively rare, generallybserved within weeks to several months after an atrialwitch when it does occur, and rarely beyond 1 yearostoperatively [39]. It is observed more frequently inatients who were operated on as neonates [16, 40, 41].Superior caval obstruction is much more frequent than

he obstruction of the inferior vena cava. When present,ymptoms include puffiness of the eyelids or facialdema, pleural effusion, and even chylothorax [12]. Sys-emic venous obstruction has been reported more fre-uently after the Mustard operation (10% to 40% ) [15, 19]

han after the Senning operation (0% )[25].Pulmonary venous obstruction, contrary to systemic

tenosis, is usually symptomatic. The reported incidencef this complication is 0% to 27%, much less frequentlyfter the Senning operation [19, 39], although others have
ot found a statistical difference between the two proce-

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ures (10% after Mustard vs 13% after Senning) [40].ymptoms consisting of cough, wheezing, dyspnea, andxercise intolerance usually present during the first yearnd indicate surgical reintervention, not infrequently onn urgent basis [19].Baffle leaks (Fig 6) lead to residual interatrial shunts,

ither bidirectionnal or predominantly right to left. Theyre usually without hemodynamic significance and rarelyndicate surgical reintervention for this reason alone [19].ight-to-left shunting occurs in the absence of elevatedystemic venous pressures and has to do with the stream-ng of blood underneath the interatrial baffle. The inci-ence ranges from 20% to 73% after the Mustard opera-

ion and from 0% to 50% after the Senning operation [39].

TRIAL ARRHYTHMIAS. Arrhythmias are frequent after antrial switch operation, including sinus node dysfunction,inus rhythm with intermittent junctional escape, junc-ional rhythm, supraventricular tachycardia, atrial flutterr fibrillation, and ventricular tachycardia. Byrum andolleagues found sinus node dysfunction in 30% of oper-tive survivors, more frequently in patients younger thanmonths of age at the time of surgery, and relate this to

ntraoperative damage that is caused by the proximity ofutures lines to the sinus node in the smaller patient [42].inus node dysfunction is a progressive occurrence.Deanfield and colleagues reported normal sinus

hythm in 84% of their patients in the immediate post-perative phase of an atrial baffle procedure, falling to6% in stable sinus rhythm after a Senning correction,nd to 66% after a Mustard operation, at a mean fol-ow-up of 7 years [43]. They found no relation betweenhe loss of sinus rhythm or active arrhythmia and suddeneath, which occurs in up to 11% of patients as docu-ented by Holter recordings [43]. In a more recent study

rom the same institution comparing the Senning andustard operations, the incidence of postoperative atrial

utter was similar and was strongly associated with lateudden death [12].

Intraatrial reentry tachycardia occurs in 2% to 10% ofatients after the atrial switch operation [44]. It induces aapid ventricular response and is thought to be onexplanation for the 3% to 15% incidence of postoperativeudden death. Atrial tachyarrhythmias are induced byeentrant circuits that result from the extensive atrialuture lines involved in a Senning or Mustard operation45]. Concealed entrainment techniques can be used to

ap reentry sites, which are most often found in theouth of the coronary sinus and the tricuspid valve

nnulus, and in the atrial myocardium of right atrialrigin, whether they are part of the surgically createdulmonary or venous atrium [45]. These sites can beuccessfully silenced with radiofrequency catheter abla-ion, and recurrence at midterm follow-up is low. Thisreatment modality aims to eliminate the electrical sub-trate for the arrhythmia and is hence more attractivehan medication, which can result in breakthrough tachy-ardia or proarrhythmia, or both. Antitachycardia pacing
as been used to treat intraatrial reentry tachycardia, but i
t carries the risk of accelerating the tachycardia intotrial fibrillation [45].

RICUSPID VALVE INSUFFICIENCY. Various degrees of tricuspidalve insufficiency (TI) have been reported after the atrialwitch, with an incidence that reaches as high as 52% inome series [8]. Relevant TI occurs more frequently afterhe Mustard correction than after a Senning operation12]. It is more frequent in patients with TGA plus VSD,nd may be related to intrinsic abnormalities of thericuspid valve in these patients [25, 29, 46] or to intraop-rative injury or distortion of the valve during VSDlosure [25, 29, 39]. The incidence varies from 5% whenhe interventricular septum is intact to 30% with anssociated VSD [19].The degree of severity is usually mild, and symptoms

r hemodynamic relevance are rare when TI occurs in thebsence of RV failure [14,15, 39]. Accordingly, few reop-rations are needed for isolated TI (see Table 2). Accord-ng to Poirier and Mee [47], differences in outcome andventual failure of the RV after a Senning procedure areelated to the degree of TI in the immediate postopera-ive period, particularly in patients with TGA plus VSD.n the series from Melbourne [25], tricuspid valve dam-ge at VSD closure or by jet lesions contributed to theifference in outcomes, suggesting that mild postopera-

ive (post-Senning) TI could become significant and po-entially lethal because it adds to the RV workload,urther precipitating RV failure [25, 47].

When severe TI occurs, it is a precursor and nearurrogate of impending RV failure, which it precedes byears [8, 29]. This may be addressed by tricuspid valveepair or replacement, although the results are disap-ointing [26, 28, 29, 37, 48], with minimum improvement

ig 6. Cardiac angiography with contrast injection of the systemicenous tunnel and the bend it performs around the septal flap.

n hemodynamics. When TI is associated with RV failure,

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trial switch takedown, pulmonary artery (PA) bandingnd conversion to an ASO [28, 29], or transplantation,ay be better options [8, 28, 48].

ULMONARY VASCULAR OBSTRUCTIVE DISEASE. The appearancend progression of pulmonary vascular obstructive dis-ase and resultant pulmonary hypertension (PHN) innoperated patients with TGA is related to age; theegree to which it persists, stabilizes, or regresses aftern atrial switch is also a function of age at correction [39].ild-to-severe PHN occurs in 4% to 35% of patients with

GA plus an intact ventricular septum after an ASO [39].his incidence is only 1% to 3% when the repair iserformed before 1 year of age, and increases steadily

hereafter. As a corollary, risk factors to develop PHNnclude older age at repair, the preoperative presence of

large patent ductus arteriosus, and a large VSD [39].he progression of PHD after an atrial baffle procedure isare but has been reported, as well as the even more rareecrease in pulmonary arteriolar resistance after surgicalorrection [39].

sychosocial Outcomes and Neurodevelopmentfter the various surgical repairs for TGA, children haveore neurologic impairment, learning disabilities, be-

avioral disorders, and poorer motor and vocabularybilities than their healthy peers [49–51]. Although thisas been extensively documented after the ASO by theroup from Boston Children’s Hospital [49–51], the def-

cits found in this cohort do not seem specific to childrenith TGA, but are similar to those found in othersndergoing repair of a congenital or acquired heartefect [51].Alden and colleagues [52] studied 31 children whoere operated on in one institution at a mean of 11.5

ears after a Senning or Mustard repair, with varyingardiac functional status at last follow-up. Nineteen per-ent had a psychiatric diagnosis, mostly of an internaliz-ng nature that tended to be predicted by the severity ofhe cardiac condition. This is still considerably lower thanhat has been reported after cardiac surgery for other

yanotic cardiac conditions. These children had goodsychosocial functioning, and only one in five had severemotional or behavioral problems. Intelligence quotientcores were marginally lower than the general popula-ion, but only one child was mentally retarded (3%) [52].

Culbert and colleagues [53] compared patients havingndergone an ASO operation, a Senning or Mustardperation, and a Rastelli operation with healthy age-atched children. After TGA repair, children and ado-

escents functioned well both physically, and psychoso-ially. The complete patient population scored higher onhe Child Health Questionnaires than control norms inll categories except self-esteem. Patients achievedigher scores after an ASO [53] than both subsets ofatients undergoing an atrial baffle procedure. Contrary

o this study, Ellerbeck and colleagues found no differ-nce in cognitive and motor development, neurologic
mpairment, learning disabilities, behavior disorders, or w
otor, vocabulary and acquired abilities, between chil-ren after an ASO and an atrial switch operation [54].Given the vast list of pre-, intra-, and postoperative

ariables that may affect the mid- to long-term neurode-elopmental status of a patient, it is currently difficult tostablish whether the underlying disease itself, the typef surgical correction, or the technical aspects of cardio-ulmonary bypass are responsible for the adverse out-omes [51].

enning Versus Mustardfter the Senning procedure was abandoned in theid-1960s and early 1970s in favor of the Mustard oper-

tion, renewed interest in the Senning procedure wasained after the technical modifications introduced andromoted by Quaegebeur and colleagues [7].The theoretical and practical relative advantages of the

enning operation include avoidance of foreign material,otential for growth of native tissues forming the neo-hambers, potential functional capacity with muscularontraction of the atrial chambers, and avoidance ofkinetic patches that can scar, shrink, thicken and furtherbstruct atrial inflow, such as that seen with the Mustardperation [41]. In a population-based cohort study that

ooked at mortality 25 years after surgery for congenitaleart diseases, Morris and Menasche found an improve-ent in survival with the Senning operation comparedith the Mustard operation (late cardiac mortality 2% at

0 years, and 15% at 15 years, respectively) [55]. Arrhyth-ias were a major cause of morbidity and mortality in

urvivors of the Mustard operation, but not with theenning operation, after which no arrhythmia-relatedeaths were noted [55].The recent multicenter study from Belgium compared

he long-term outcome in 339 patients up to 30 years afterne of the two atrial switch procedures [56]. Both groupsad a relatively high early mortality rate, but actuarialurvival at 10, 20, and 30 years was satisfactory at 91.7%,8.6%, and 79.3%, respectively. This was slightly betteror the Senning group, although not significantly. At lateollow-up, Senning patients had better functional status,articipated more actively in sports, and had feweraffle-related problems than did the Mustard group [56].Sarkar and colleagues [12] compared their series of 141

atients who underwent a Senning operation with 249atients who underwent the Mustard operation during

he same time period. Survival was significantly betteror the Senning group, reinterventions for baffle-relatedroblems or left outflow tract obstruction were signifi-antly lower, and pacemaker insertion was less frequent.he loss of stable sinus rhythm was comparable in the

wo groups and unrelated to death. The incidence oftrial flutter was similar in both groups and stronglyssociated with late sudden death. The authors con-luded that the Senning operation had superior results,ith good late functional status, and argued that elective

trial baffle takedown and conversion to an ASO cannote justified in asymptomatic post-Senning patients [12].During the same historical period in which patients
ere enrolled to undergo either of the atrial baffle pro-

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edures, Helbing and colleagues [14] compared 60 pa-ients after a Mustard operation with 62 patients after aenning procedure. At respective median follow-up

imes of 16 and 11 years postoperatively, there were noifferences with regards to baffle-associated problems,V failure, sudden death, or functional status between

he Mustard and Senning patients. Satisfactory long-erm survival was similar, and excluding pacemakermplantation, no reoperations were necessary in eitherubset of patients. The only significant risk factor for theccurrence of sinus node dysfunction was the Mustardperation [14].A technical pitfall of the Mustard operation involves

he difficulty in shaping an appropriate baffle withoutreating systemic or pulmonary venous obstruction, par-icularly in neonates. In a meta-analysis that reviewed

ulticentric postoperative angiographic data, Graham39] found both caval obstruction and pulmonary venoustenosis to be more frequent after the Mustard operationhan after a Senning operation. Risk factors to developystemic venous obstruction included the use of a DacronDuPont, Wilmington, DE) baffle, operation in early in-ancy (� 6 months), and the use of a “trouser-shaped”affle instead of a “dumbbell-shaped” baffle, such as thatriginally described by Mustard [39].Contrary to these reports, The Congenital Heart Sur-

eons Society [11] found better early and late survivalfter the Mustard operation than after a Senning opera-ion in a prospective cohort of patients with TGA whoere destined to have either an ASO, or one of the two

trial switch procedures. Twenty-one patients who werentended to have an ASO had a Senning operationnstead owing to unfavorable conditions or anatomy thatere discovered in the operating room. This cross-overith higher risk patients undergoing the Senning oper-

tion may have influenced the difference in early survivaln favor of the Mustard operation, but does not explainhe difference in late survival. In the atrial switch sub-roup, risk factors for long-term pacemaker requirement

ncluded patients with TGA plus VSD undergoing aenning operation, and previous surgical atrial septec-

omy [11]. Institutional preference or experience couldartially explain the better early and late results with theustard variation, although this is purely speculative.

V Retraining and Senning Takedown En Route to anSOhen RV failure after a Senning correction reaches an

dvanced stage, treatment options are limited to tricus-id valve replacement, orthotopic cardiac transplanta-

ion, or atrial baffle takedown and conversion to an ASO.s the first two procedures have their own set of disap-ointing results and long-term complications [25, 28, 29,7], more groups advocate restoring the morphologic LVo the systemic circulation [25, 29, 57]. Most often, thisannot be done in one step, as the LV has accustomedtself to the low pressures found in the pulmonaryirculation. Before a Senning or Mustard takedown and auccessful ASO are attempted, the LV must be retrained.
ulmonary artery (PA) banding is required to achieve 2
dequate LV muscle mass, as was first described by Mee29].

Currently, there are no clear indications or discrimi-ating points to decide when a patient should no longere treated medically for heart failure, whether transplan-

ation is deemed a better option, or whether one shouldirectly proceed to LV retraining. This controversial topicnds proponents and adversaries for each therapeuticrm and may be institutional-based; its answer is beyondhe scope of this review. As medical treatment andransplantation are well described in the literature, LVetraining en route to an ASO is briefly reviewed here.

Foremost, contraindications to LV retraining includerreversible LV dysfunction, pulmonary valve abnormal-ties that render it unsuitable as a future neo-aortic valve,V outflow tract obstruction that cannot be relieved, andncontrolled arrhythmias [47]. The response to LV re-

raining is poorer in patients who are older than 15 years,lthough a successful Senning takedown and ASO wereerformed in a 28-year-old patient [47]. The degree ofreexisting RV failure does influence the response to LVetraining, owing to the common interventricular septumhat bulges towards the LV that induces LV outflow tractbstruction and eventual LV failure at lower than ex-ected LV pressures [25, 47]. For these reasons, earlier PAanding is advocated, before decongestive therapy forV failure becomes necessary [25, 28].The aims of PA banding are to achieve a LV/RV

ressure ratio of 0.7 or greater. One or more bandingsay be required over a period of approximately 1 year to

nduce adequate LV hypertrophy, although this period isenerally shorter in younger children [47]. The prepara-ory stage of retraining is better tolerated in patients afterprevious atrial baffle procedure than in patients with annoperated TGA who present late [48]. The former doot require systemic-to-pulmonary shunts in addition toPA band to maintain adequate saturations, as they

lready have a physiologic circulation [48]. PA bandingan induce neo-aortic valve insufficiency [28, 48, 57, 58],nd the relative cumbersome need to perform multipleperations to tighten or loosen a band before adequateV retraining is achieved may promote wider applica-

ions for the new adjustable and teleguidable FloWatch--PAB (EndoArt SA, Lausanne, Switzerland) band [59].Before debanding, Senning takedown, and conversion

o an ASO, transthoracic echocardiography, cardiac cath-terization, and MRI are performed. These seek to con-rm a LV that generates more than 80% of systemic bloodressures at rest, suprasystemic pressures with isopro-

erenol, or normal LV mass and wall thickness, indexedor weight and age [47, 58]. The size of the coronaryrteries, and namely, that of the left coronary arteryefore PA debanding, may influence the success of aubsequent ASO with regards to the increase in coronaryow reserve that is required to adequately perfuse the

uture systemic LV [60].In appropriately selected patients, the results of the LV

etraining protocol after a failed Senning en route to anSO are good to excellent in prepubescent patients [25,

8, 47, 58], but give unpredictable results in patients older

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han 15 or 16 years [47, 58]. LV retraining has failed whennadequate LV hypertrophy or LV dysfunction occurs, orf atrial arrhythmias progress [47]. When LV retraining isnsuccessful with uncontrollable ongoing RV dysfunc-

ion before an ASO, or LV deterioration after a secondarySO, then early transplantation should be considered

28, 48, 57, 58]. The results of transplantation for a failedtrial switch have been satisfactory when performed in aimely fashion, although the long-term consequenceshat are general to all posttransplant patients, namelyssues pertaining to a lifelong immunosuppressive regi-

en, are of concern [28, 48].

ebirth of the Atrial Baffle Procedures for Patientsith CCTGA

lthough the Senning operation seems outdated and issed only in exceptional cases to treat patients with TGA,

ncreasing interest and experience is being gained withhis procedure in patients with congenitally correctedransposition as part of the double switch or Senning-astelli procedures. These operations reposition theorphologic LV in the systemic circulation, also referred

o as the “anatomic repairs” of CCTGA, and are currentlyhe treatment of choice in patients with this anomaly [28,7, 61–66]. Most authors recommend anatomic repairhen tricuspid valve regurgitation or RV dysfunction areresent [62, 64]. Others are more aggressive and recom-end anatomic repair for all patients with an adequate

r trainable LV, although until which age this is feasibler gives acceptable results is still controversial [62, 65, 66].Proponents of the anatomic repair have demonstrated

etter results when the double switch is performed, withr without prior LV training, before the age of 15 to 16ears [47, 62]. Results have been less satisfactory in olderatients, and in some instances, the LV is simply no

onger trainable, leaving transplantation as the only sal-age alternative. Some controversy concerning the dou-le switch still revolves around asymptomatic patients,ith or without associated intracardiac defects [67]. In-eed, drawing parallels between unoperated patientsith CCTGA and patients after atrial correction for TGA,ormal or near normal RV function in the long-term haseen demonstrated in minimally symptomatic or asymp-

omatic adult patients with CCTGA [67].When an anatomic repair of CCTGA is performed, the

enning operation is the preferred atrial baffle procedureor most [47, 61, 63], although in the presence of dextro-ardia, the Mustard operation may be technically easiero perform [10, 61]. The timing of an anatomic repair isased on the size of the VSD. When the VSD is restric-

ive, LV pressures remain low (infra-systemic), resultingn an untrained LV, and the procedure should be per-ormed before 1 month of age [61]. If it is performed later,reliminary PA banding may be required to redevelop

he LV. With a large VSD that results in unrestrictedulmonary blood flow and systemic PA pressures, theepair should be performed by 6 months of age torevent the development of pulmonary vascular disease

61].
The results of this complex procedure are good to v
xcellent, with mortality rates ranging from 0% to 15%62–65]. Long-term follow-up of the anatomic repair forCTGA is still required for patients with valved conduitsho have undergone a Senning-Rastelli procedure and

or the aortic valve and the morphologic LV in patientsfter the double switch [64]. Although the LV is restoredo the systemic circulation, the long-term complicationselated to the atrial part of the Senning operation, namelyhe venous pathway problems and atrial arrhythmias,

ay still be expected [10].

ndications to Perform an Atrial Switch Operation forGAhere are still instances where the Senning operationay be indicated for patients with TGA. These include

omplex coronary anatomy precluding an ASO, or lateeferral in patients with TGA plus VSD, which is veryommonplace in developing countries. In this situation,NH and a LV that is inadequate or untrainable mayoth contraindicate an ASO [16]. Even in older infantsith an intact ventricular septum and low LV pressures,

here still may be a place for the atrial baffle procedure37].

A certain subset of patients may be more common thaneported, mostly in developing countries with subopti-al medical control and access to diagnosis, namely

hose with TGA plus an intact ventricular septum, andevere PHN without a correctable cause. In the absencef overt left-to-right shunting, idiopathic PHN tends to

ast well beyond the neonatal period, if it regresses at all,nd is more difficult to manage with medical therapy.Successful surgical correction has been achieved in

eonates with TGA plus an intact septum and PHN withn ASO [68], at the cost of a lengthy and stormy postop-rative course that required inhaled nitric oxide or evenxtracorporeal membrane oxygenation (ECMO) [69, 70].n these patients, Sharma and colleagues reported 75%

ortality with an attempted ASO [68]. Despite whateemed to be a “prepared” LV preoperatively, RV failuren the face of systemic pulmonary artery pressures re-ulted in death. In addition, neopulmonary valve insuf-ciency is also a well-documented possibility after anSO and will worsen with poor right-sided hemodynam-

cs in the face of PHN. In 6 similar infants presentingonsecutively, they opted for a Senning repair that re-ulted in early extubation and hospital discharge as wells 100% survival. Four of the patients had normal pul-onary artery pressures at 1 year postoperatively. Aorphologic LV is better suited to face systemic pulmo-

ary pressures in the setting of patients with PHN, anday give better chances for survival [69].The group from Great Ormond Street, London, has

ecently presented their evolving practice to expand thendications for an ASO, either for late referral or diagno-is, prematurity, or intercurrent illness [70]. In theseifficult patients, increased experience and the availabil-

ty of postoperative ECMO has allowed post-ASO sur-
ival in selected patients up to 6 months of age [70].

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onclusions

fter being the only viable surgical solution for patientsith TGA, the Senning operation successfully enjoyedopularity, followed by abandonment in favor of theustard operation, then an initial revival after modifica-

ions introduced by Quaegebeur, and colleagues [7],efore finally finding its most frequent current indications part of the anatomic repair for patients with CCTGA.Surgeons can perform the Senning operation low mor-

ality and minimal morbidity by applying technical mod-fications and paying meticulous attention to large andnobstructed venous pathways. The results of this pro-edure may be compared with the newer ASO for TGAith regards to initial operative success, although long-

erm complications of the atrial baffle procedure cur-ently speak in favor of the ASO. Eventual RV failure isot a time-related event [26], and still hampers the late

ollow-up of patients after the Senning operation. Theres some evidence that the onset or degree of RV failure18, 24, 36–38] or exercise intolerance [20, 36] may beeduced when the Senning operation is performed ear-ier, particularly before the age of 1 year [36]. Currently,o diagnostic tool exists that allows for prediction ofventual RV failure in patients after an atrial correctionor TGA. Long-term arrhythmias remain a problem afterhe atrial switch. In some instances this may be treatedonservatively, although more invasive radiofrequencyatheter ablation is required in others. The insidiousature of the various arrhythmias and their potential butnproven relation to sudden death emphasizes the need

or closer arrhythmia follow-up.It is noteworthy that reports from Europe and Australia

hat compare the Senning and the Mustard operationsoint to better immediate and long-term results with theenning operation. However, the successive meta-nalyses from the Congenital Heart Surgeons Society,hich enrolled North American centers, report betterbjective outcomes with the Mustard operation. Thisay only reflect schools of training that have historically

avored one operation over the other, leading to in-reased and improved experience with each respectiveurgical procedure. Despite the theoretical advantage ofvoiding foreign material in the Senning operation, onehould ultimately proceed with what works best for eachnstitution.

The superior results of the double switch or Senning-astelli operation compared with the “classic” or “phys-

ologic” repair make the former the preferred surgicalreatment in patients with CCTGA. Theoretically, even-ual RV failure or tricuspid valve insufficiency should bevoided, as the morphologic LV and mitral valve areestored to the systemic circulation [63]. As the follow-upf the more modern anatomic repair is still short, atrialrrhythmias and venous pathway obstructions or leaksay still be expected [10], although the management of

hese problems may relatively be straightforward andithout heavy dire consequences.In the current era, the Senning operation in patients
ith TGA is reserved for those with unfavorable coro- 1
ary anatomy, for late referral, or for patients with TGAnd pulmonary vascular obstructive disease, even wheneferred at an earlier age. These situations and the choiceor a Senning operation may be particularly frequent andertinent in developing countries without access to nitricxide or ECMO. In patients with CCTGA, the Senningperation is an integral part of the double switch orenning-Rastelli operation, whose long-term follow-up istill awaited.

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in the footsteps of senning

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