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109doiart Defects4 defines a more physiologic classificationeme based on the site of venous drainage: the right atrium,left atrium, and both atria. Still another approach is foundSurgery of the Chest,5 which defines a classificationema based on embryologic origins.6 Most recently, theciety of Thoracic Surgeons-Congenital Heart Surgery Da-ase committee7 proposed a hierarchical scheme for classi-ation, again based on the IVC, the SVC, and the HVs. Thisssification systemic identified nine anomalies of the SVC,e of the IVC, and 10 of the HVs (Fig 1). The majority ofse variants are rare, the aim of this system being inclusive-ss.

the posterior and anterior branch on each side uniting toform a common cardinal vein. The umbilical, vitelline, andcommon cardinal veins unite to form the right and left hornsof the sinus venosus (Fig 2A). By the fourth week of fetal life,an invagination has developed from the left horn of the sinusvenosus, separating it from the left atrium (Fig 2B), whichserves as the conduit through which all blood enters the rightatrium. In the eighth week of gestation, the left innominatevein develops, connecting the right and left anterior cardinalveins. By the sixth month of fetal life, as flow through theinnominate increases, the left anterior cardinal vein decreasesin size and is obliterated (Fig 2C). The left common cardinalvein persists as the coronary sinus, to drain into the rightatrium. Failure of obliteration of the left anterior cardinalvein results in a persistent LSVC. Failure of the invaginationprocess of the left horn of the sinus venosus along with failurenford University, Department of Cardiothoracic Surgery, Stanford, CA.

ress reprint requests to Kimberly Gandy, MD, PhD, Division of Cardio-anagement of Systemic Vthe Pediatric Cardiovasc

mberly Gandy and Frank Hanley

Systemic venous anomalies are rare and heteare rare in the general population, they occcongenital heart disease. In and of themselvesignificance. However, in the setting of congicantly alter surgical treatment. This reviewSemin Thorac Cardiovasc Surg Pediatr Cardrights reserved.

KEYWORDS: Pediatric, cardiac, surgery, venous

he systemic venous system manifests a wide spectrum ofstructural abnormalities. These anomalies are relatively

e, occurring in approximately 0.3% to 0.4% of the generalpulation.1 In the subpopulation of patients requiring sur-y for congenital heart defects, however, these anomaliesmore prevalent, occurring in 2.8% to 4.8% of operativees.2 Although these lesions are most often physiologicallynign, they may significantly alter the surgical managementspecific lesions.Multiple classification schemes have been proposed foraracterization and organization of systemic venous anom-es. Mastery of Cardiothoracic Surgery3 offers an ana-ic classification scheme with the major categories basedofLS

thoracic Surgery, Arizona Health Sciences Center, PO Box 245071, Tuc-son, AZ 85724-5071; e-mail: kgandy@surgery.arizona.edu

2-9126/06/$-see front matter 2006 Elsevier Inc. All rights reserved.:10.1053/j.pcsu.2006.02.004ous Anomaliesr Surgical Patient

eous entities. Although these anomaliesre frequently in the subpopulation withst of these lesions have no physiologicheart disease these lesions may signif-icated to these lesions.Ann 9:63-74 2006 Elsevier Inc. All

aly

Most large series report that the majority of systemic ve-us anomalies are accounted for by a few specific abnormal-s. In a review of the last 40 anomalies seen in the congen-l cardiac surgery population at Stanford Universityanford, CA), the overall incidence was 3.8%. Forty-eightrcent of these anomalies were represented by bilateral SVC,% were represented by interrupted IVC, and 5% of pa-nts had anomalies for which the lesion alone was an indi-ion for surgery. These numbers are scarcely different fromt found in other series.The study of embryology lends some understanding to thequency and character of appreciated abnormalities. Theobliteration of the left anterior cardinal vein results in theCV draining directly into the left atrium.6

63

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64 K. Gandy and F. HanleyIVC development involves a supracardinal and subcar-al venous system that develops dorsal-medial and ven--medial, respectively, to the posterior cardinal veinsg 2D). Multiple anastomoses develop between the sup-ardinal and subcardinal systems, and eventually the lefttem undergoes regression. The left supracardinal sys-remains as the hemiazygos vein and the right becomesazygos (Fig 2E). The right subcardinal vein and the

Figure 1 Systemic venous anomalies as classified by GaynAbnormalities of IVC. (C) Abnormalities of hepatic veins.

Figure 2 Embryological development of the systemic veinInvagination of the left horn of the sinus venosus. (C) Obliand subcardinal venous systems. (E) Azygos and hemiazyg

hemiazygos continuation. (Adapted and reprinted with permissCompany.)s join to form the IVC. Failure to do so results in anerrupted IVC that can then drain into the SVC by azygoshemiazygos continuation (Fig 2F), azygos continuationing the more common of the two drainage anomalies.omalies of situs are frequently associated with theseions.Our discussion of these anomalies will be divided into twoups: those that are physiologically benign and those that

einberg, and Spray. (A) Abnormalities of SVC. (B)ted with permission.7)

Formation of the horns of the sinus venosus. (B)n of the left anterior cardinal vein. (D) Supracardinalelopment. (F) Development of interrupted IVC withs. (A)teratioos devHVintorbeAnles

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Management of systemic venous anomalies 65pathophysiologic. Emphasis is given to common clinicalsentations and a treatment algorithm is proposed, thendation being presented in Figure 3. There are two phys-ogically benign entities that will be discussed: bilateralC and interrupted IVC with azygos continuation. Theretwo pathophysiologic entities that will be discussed: un-fed coronary sinus syndromes and baffle obstruction intients with complete TGA (transposition of the great arter-) that have undergone atrial switch procedures.The most common systemic venous anomaly is the persis-t left SVC (LSVC). This anomaly can be recognized at bothdiac catheterization and cardiac echocardiography.8 Aht SVC (RSVC) is usually present, but may be absent in aall number of cases. The LSVC typically originates at thegin of the internal jugular vein and the subclavian vein.e vein then courses anteriorly over the aorta, pierces thericardium, and enters the coronary sinus. In a small num-r of cases, the SVC may enter the left atrium, a conditionociated with unroofed coronary sinus (discussed below).most instances, there is no accompanying innominaten, although there may be multiple small connecting veins.the presence of a LSVC, the RSVC is often smaller thanal and the presence of a smaller than expected RSVC at thee of pediatric cardiac surgical procedure should raise sus-ion of the presence of an LSVC. However, because most ofse patients have undergone echocardiography and/or car-c catheterization, it would be rare for an LSVC to haveen previously unappreciated.In the absence of abnormalities of situs, the LSVC usuallyins into the coronary sinus, a scenario present in 2% to 4%all cardiac defects (Fig 4).9 Indeed, a dilated coronaryus, on echocardiography or upon gross examination of theart, should raise suspicion of an LSVC. However, some

Figure 3 Treatment algorithm for patiVC may drain into the RSVC, right atrium, or left atriumg 1A).7 Suspicion of LSVC should not necessarily prompt

Figutwosection in a search for it. The left phrenic courses along thee of the LSVC and can be injured during dissection, espe-lly if the procedure being performed is a secondary oper-on, with alteration of native tissue textures and disruptionnative tissue planes. A particular scenario has been de-ibed that may mimic cor triatriatum echocardiographi-ly and functionally.3 In patients with pulmonary hyper-sion and elevated right atrial pressure, LSVC draining intoilated coronary sinus can impinge upon the wall of the leftium and obstruct flow from the left atrium across the mi-l valve. Knowledge of this particular functional scenarioprevent unnecessary exploration of the left atrium. Some

ve suggested that such an abnormality may alter fetal he-dynamics in such a way as to foster hypoplastic develop-nt of left-sided structures, such as mitral valve stenosis

ith systemic venous anomalies.hamomere 4 Treatment algorithm for patients with bilateral SVC on-ventricle pathways.

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66 K. Gandy and F. Hanleyd left ventricular hypoplasia (personal communication, M.Leval, December 2005).The presence of LSVC has been evaluated in the context ofociated cardiovascular defects. There is an associated VSDup to 17% of patients. In 10% to 15% of patients there istic coarctation, tetralogy of Fallot, or an atrioventricularV) septal defect. Finally, in a small percentage of patients,atrial septal defect, double outlet right ventricle, TGA, or am of single ventricle may be present.

plications of BilateralVC in Two-Ventricle Repairsschematic of the decision making process for operativeervention in patients with two-ventricle repairs is pre-ted in Figure 4. As noted, in and of itself, this lesion isnign. In the face of necessary open-heart surgery and theed for cardiopulmonary bypass, however, the anomalyy need to be addressed. In the presence of a sizable cross-vein, the LSVC can be ligated, with flow simply beingerted across the innominate vein into the RSVC. It is ofue to note that when the innominate is present, it is notcommonly retroaortic.10

There may be multiple small connecting veins, but theominate is absent or hypoplastic in 75% of cases (Fig 5).11

re 5 Bilateral SVC with no innominate.such cases, the decision algorithm is more complex. Inple cases, where an absolutely dry field is not necessary,

Figucavous drainage may be provided by cannulation of the righterior cava and use of the cardiotomy sucker in the region

the coronary sinus (Fig 6). However, care must be taken inof the cardiotomy sucker in the coronary sinus becauseessive tension in this area can cause heart block secondarytraction on the adjacent AV node. For a similar reason,ect cannulation of the coronary sinus is avoided.In the repair of moderately complex and complex lesions,recommend cannulation of both the LSVC and RSVC (FigInmost cases, each cava is smaller than the single cava thatuld typically be present in a child of a given weight and itecessary to downsize the cannula by at least one size.In a select group of patients without a crossing vein, theVC can still be temporarily occluded. Occlusion shouldays be avoided in premature infants, long cases, or caseswhich cerebral perfusion could have been significantlyered preoperatively secondary to hemodynamic compro-se. Before occlusion of the cava, the pressure in the SVC isessed after test occlusion by palpation or direct measure. Ifcerebral AV pressure gradient is reduced by more than

%, the LSVC is not ligated and alternative operative man-ment is considered. We strongly prefer separate cannula-n of the LSVC in this case. If this is not possible, however,ht atrial cannulation and deep hypothermic circulatoryest may be necessary. It is important to note, however, thatre are investigators that use the technique of circulatoryest in most of their cases with bilateral SVC.3

There are two other specific anatomic instances that de-ve further comment. A LSVC draining to the coronaryus has specific implications for repair of AV canals. Such anario should predispose the surgeon to incorporate theonary sinus on the right side of the patch, a deviation frompreference of a large number of surgeons. Similarly, there 6 Cardiotomy sucker in coronary sinus for left superior venaa drainage.

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Management of systemic venous anomalies 67VC to coronary sinus drainage pattern complicates atrialffle procedures, which are still used for patients with AVcordance because the coronary sinus is usually incorpo-ed into the pulmonary venous atrium.

plications of BilateralVC in Single-Ventricle Repairse presence of bilateral SVC has multiple implications intients with single-ventricle physiology. Patterns of cannu-ion for cardiopulmonary bypass, operative plan of recon-uction, and management of anticoagualation are all al-ed. Bilateral SVC may also be more common in patientst are undergoing single-ventricle repairs. For instance, up15% of patients with hypoplastic left heart syndrome mayve bilateral SVC.12 A summary of the most importantints is presented in Figure 8. In patients undergoing a stageeconstruction for hypoplastic left heart syndrome withh reconstruction and creation of controlled pulmonaryod flow, the principle issue is related to management ofcavae for future bilateral cavo-pulmonary anastomosesPA). We recommend that no deep lines be placed preop-tively before construction of BCPA. These lines predisposese vessels to thrombosis and may preclude their use insequent cavo-pulmonary reconstruction. Patients withluded cavae may undergo BCPA if they have developedge collateral vessels that can be used in their stead, but theg-term patency of reconstructions performed with suchsels is in question. Single-ventricle physiology candidatesuld be surveyed upon presentation to an institution andring their presence in the NICU so that such lines are notd. Deep lines may be placed in the lower extremities ifsolutely necessary, but we recommend that such lines haveinfusion of heparin 5 U/mL to enhance efforts to maintaintency. Similarly, we recommend that such lines be moni-ed intermittently with ultrasound to avoid the accumula-

re 7 Cannulation of both LSVC and RSVC.n of occlusive clot along the course of the line. IVC occlu-n in these infants can have disastrous consequences,

Figusinulting in renal failure and/or gastrointestinal ischemia ift propagates to the origin of venous drainage for thesean systems. If nothing else, occlusive IVC clot can com-cate Fontan completion at a later date. Operative place-nt of common atrial lines in these patients can be helpful,d should be used for longer periods in patients in whomous access has been problematic. We begin anticoagula-n of these lines within 48 hours of operation. Commonial lines can be left in long after chest tubes have beencontinued, and removed once tracts around the lines arell-developed.For most stage I operations on neonates, we do not recom-nd cannulation of the cava. The caval condition has directects on subsequent stages of reconstruction, and every ef-t is made to avoid caval manipulation. Because complexcedures are typically not performed on the right side ofheart in most stage I procedures, it is possible to performoperation with right atrial cannulation and sucker bypassistance when necessary.The presence of bilateral SVC has more significant impli-ions for the construction of the BCPA, a procedure thatrepresent either the primary or secondary stage of a pal-

tive procedure. The underlying principle of the BCPA isdiversion of upper body systemic venous blood flow di-tly into the pulmonary arterial tree. Physiologically, thiscedure should increase effective pulmonary blood flowile unloading the volume load on the systemic ventricle.13

most patients, this means a single anastomosis of the RSVCthe right pulmonary artery. In patients with bilateral SVC,ernative decisions are made in terms of both the decisionsupper limb cannulation as well as the number of anasto-ses for reconstruction. It has been reported that the pres-ce of bilateral SVC increases the risk of the BCPA.14 How-r, this has not been our experience15 and bilateral SVC hast altered our recommendations for timing or candidacy forPA. We recommend bilateral BCPA in patients with bilat-l SVC without crossing veins (Fig 9). Some perform suchastomoses with temporary occlusion of each cava duringanastomosis, without cannulating the cavae, relying onsumed small vessel collaterals vessels for decompression.re 8 Treatment algorithm for patients with bilateral SVC ongle-ventricle pathways.

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68 K. Gandy and F. Hanleych procedures have proven safe in the hands of experi-ced surgeons, but recommend that significant attention beid to monitoring for the development of venous hyperten-n in these incidences. In patients with sizable crossingns, the LSVC can be ligated and a single BCPA can bestructed on the right. In patients with balanced pulmo-

ry blood flow in whom the BCPA may represent that pri-ry procedure, the BCPA is performed between 2 and 4nths of age.16 In patients who have required neonatal in-vention and present for BCPA as a second-stage palliation,PA is performed between 3 and 6 months of age. Analysisa small set of patients undergoing BCPA less than 6monthsage has shown that bilateral SVC is not a risk factor for poortcome.17 It has previously been noted that central pulmo-ry arterial hypoplasia can develop in this patient popula-n, (Fig 10A), and it was hypothesized that this could bedressed by maintenance of antegrade ventriculo-pulmo-ry arterial flow.17 Preservation of antegrade pulmonaryod flow is a controversial subject in patients withPA.18-22 Some have proposed that the maintenance of an-rade ventriculo-pulmonary blood flow may increase com-cations, and may even prevent the full effect of the volumeloading for which the BCPA was originally intended. It hasen our practice to preserve some degree of antegrade ven-ulo-pulmonary flow in patients in whom the ventriculo-lmonary outflow tract is patent (Fig 10B) and significanttral pulmonary arterial hypoplasia has been avoided.15 Intients that have not previously undergone a pulmonaryery band, a band may be placed. In patients that havedergone a pulmonary artery band, the band may be tight-ed. In patients in whom a central-pulmonary artery shunts been placed, this is usually taken down because of theximity of the shunt to the region of proposed anastomo-, and only replaced if arterial saturations are unsatisfactoryer completion of the BCPA. Even if the shunt is on the side

re 9 Bilateral BCPA with preservation of antegrade pulmonaryod flow.tralateral to a single cavo-pulmonary anastomoses, wed it is usually still necessary to take this shunt down to

ceinarvide for adequate mobilization of structures to provide asionless anastomosis. We close the azygos when perform-BCPA in all circumstances except those in which azygostinuation of IVC flow is present. In a study of 42 infantso underwent BCPA less than 6months of age, 10 of whomd bilateral SVCs, no increase in shunt failure, complica-ns, or decrease in survival was found in patients with ac-sory pulmonary blood flow as compared with those with-t accessory pulmonary blood flow.17 These patients did,wever, have higher early pulmonary artery pressures,nds toward a higher incidence of postoperative effusions,d an increased period of mechanical ventilatory depen-nce. There was no note of central pulmonary artery hyp-lasia when any of these patients presented for the Fontan.her experiences differ, however. In a retrospective analysisa group of patients who underwent BCPA at another insti-ion, antegrade flow did not affect the incidence of centrallmonary arterial hypoplasia.14 It is also notable that a sig-cant number of these patients experienced thrombosis ine or both of the SVC, a circumstance that may in some waylain the disparate results.Postoperatively, we place all patients with bilateral BCPA

re 10 Development of central arterial hypoplasia in patients re-

ving bilateral BCPA without maintenance of antegrade pulmo-y blood flow.

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Management of systemic venous anomalies 69aspirin. This is especially important given the catastrophicults of thrombosis in these patients. In a series of patientst had undergone bilateral BCPA, there was 100% earlyrtality in nine of nine patients that developed thrombosisthe BCPA system as verified by catheterization.14 In theure, it may be important to screen these patients for aspirinistance. In patients in whom there is a family history ofctor V Leiden or other heritable coagulation disorders,sideration should also be given to screening for coagula-n abnormalities. Alternative anticoagulation strategiesmayin order, although there is currently no data upon whichmake such recommendations.

plications of Bilateral SVCTransplantation

ateral SVC can also affect the conduct of heart transplan-ion. The issue of management of bilateral SVC is not un-

re 11 Treatment algorithm for patient with interrupted IVC on-ventricle pathways.re 12 Snare position in SVC cannulation with interrupted IVC.Figusinmon in the transplant population. For instance, up to% of patients with hypoplastic left heart syndrome mayve bilateral SVC.12 Some patients may present with novious surgical procedures involving the SVC. In these pa-nts with the usual LSVC drainage to the coronary sinus, itsimplest to alter the recipient cardiectomy such that therse of the LSVC into the right atrium is preserved. Theision in the inferolateral wall of the left atrium is moved toAV groove.12 Another group of patients may present after

re 13 Separate entry of hepatic veins into the atrium.re 14 Treatment algorithm for patient with interrupted IVC ongle-ventricle pathways.

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70 K. Gandy and F. Hanleyy have already undergone either BCPA or completionntan. In these patients, cannulation is performed moreximal on each affected cava. Additionally, more innomi-te vein is harvested with the donor cardiectomy. Harvest ofong segment of innominate vein will allow placement ofth LSVC and RSVC anastomoses to this venous segmentthout the need for additional reconstruction.

plications of Interrupted IVCerrupted IVC is rare, occurring in only 0.6% of patientsth congenital heart defects.23 This anomaly, however, ist uncommon in patients with abnormalities of situs. Anorithm for management of two-ventricle patients with in-rupted IVC is presented in Figure 11. In simple cases,re are no unusual issues. It is necessary, however, for there around the SVC cannula to be distal to azygos or hemi-gos entrance so that this flow is not excluded from drain-(Fig 12). In more complicated cases, it may be necessary

cannulate the HVs. If the HVs enter with the IVC as a trunkg 12), they can be cannulated through the atria, with careen to assure that the cannula tip is above the HV bifurca-n.4 If the HVs enter the right atrium separately (Fig 13), ity be necessary to cannulate both HVs. If one of the veins isaller than the other, however, drainage from this vein maycontrolled by cardiotomy suction. If HV anatomy is un-ar, it may be necessary to establish cannulation open, un-r low-flow conditions. The remainder of the procedurey then be conducted under standard cardiopulmonarypass. It is not uncommon to have hepatic venous drainagetering opposing corners of the atrium in cases of rightmerism. Inability to incorporate such venous drainageo the Fontan circuit may result in significant postoperativeerial desaturation. It may even be necessary to cross-clampaorta for a brief period in such cases to appropriately

rform the inferior anastomosis.A significant number of patients with interrupted IVC andnormalities of situs will move on to single ventricular pal-tive procedures because associated anomalies. An algo-

re 15 Treatment algorithm for patients with LSVC and unroofedonary sinus.m for management of such patients is presented in Figure. In stage I of such repairs, there are no specific issues and

Figutacht atrial cannulation should proceed as usual. Cardiotomytion can be used for HV flow if necessary during the atrialtectomy. Issues related to proceeding to the next palliativege are primarily related to timing. The Kawashima opera-n,24 in which the SVC flow and flow from the azygoustinuation are diverted to the pulmonary arteries, resultsdiversion of over 75% of systemic venous return into thelmonary arterial tree. The pulmonary vascular resistancest be able to accept this volume load at the time of theeration. We prefer to perform this operation between 9d 12 months of age. Although it was originally hoped thatKawashima operation would be the final palliative proce-re for these patients, it subsequently became clear thatse patients developed significant pulmonary arterio-ve-us malformation (AVMs).25-29 In 1995, Srivastava et al26

monstrated that these AVMs were the result of the lack of astance called hepatic factor in the pulmonary circulation.erefore, efforts were made to direct the remaining hepaticous effluent to the pulmonary circulation. Most com-nly, this is performed by directing hepatic venous flow topulmonary circulation by an extracardiac conduit or a

eral tunnel. Others have devised procedures in which thepatic venous flow is directed to the azygous vein.30-33 Wefer to obtain an atrial cuff with the attached HVs and tohion an anastomosis to an extracardiac conduit directed topulmonary artery. Studies have suggested that redirectionre 16 Most severe form of absent coronary sinus: left SVC at-hed to superior aspect of left atrium.

Management of systemic venous anomalies 71Figure 17 Operative repairs of unroofed coronary sinus. (A) Plication of the backwall of the left atrium. (B) LSVC to rightatrial appendage. (C) LSVC to RSVC. (D) LSVC to left pulmonary artery.

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72 K. Gandy and F. Hanleythis flow may prevent and even result in the resolution oflmonary AVM.34,35 Analysis of a series of 21 patients withterotaxy syndrome and interrupted IVC with azygous con-uation determined that bilateral SVCs and an interval ofre than 2 years between BCPA and redirection of hepaticous flow were independent risk factors for developmentAVM.34 Therefore, we recommend redirection of hepaticw before this critical period if possible. In keeping with ourommendations regarding all Fontan procedures,36 we rec-mend that the Fontan not be fenestrated, lest the benefitsthe procedure be lessened.

nroofed Coronary Sinusroofed coronary sinus syndrome refers to a spectrum ofomalies with varying degrees of absence of the septumtween the coronary sinus and the left atrium, the manage-nt of which is oulined in Fig 15. In its most extreme form,entire septum is absent, creating the absence of the cor-

ary sinus and resulting in the LSVC being attached to theerior aspect of the left atrium (Fig 16). An intra-atrialmunication is present in the location that the coronary

us would otherwise occupy.37 Individual coronary veinsnect to the inferior aspect of the left atrium or the rightium.38 This defect is usually associated with other anom-es, the majority of which will physiologically overshadoweffects of the lesion itself. When the lesion is present as anlated defect, cyanosis is present in early infancy. Stroked thrombotic risk complicate 10% to 25% of cases, and lifeectancy is reduced.The goal of operative therapy is the rerouting of systemicous blood into the right atrium.Multiple operative repairsve been proposed for this condition. Historically, this con-ion has been corrected by creating a roof for the coronaryus with plication of the back wall of the left atrium16 (FigA) or by using a pericardial,39,40 synthetic,40 or atrial ap-ndage patch to baffle LSVC flow to the right atrium afterision of the atrial septum. Most series report few compli-ions with these procedures. There may be circumstances,wever, in which extracardiac techniquesmay bemore ben-cial and several techniques have been proposed.41 Closeximity of pulmonary veins to the SVC can make intracar-c baffles difficult. Additionally, extracardiac techniquesow procedures to be performed during rewarming, provid-for a reduction in the time on cardiopulmonary bypass.ree recently described techniques41 are described below.some circumstances, the LSVC can be transected at itsertion into the left atrium, mobilized anteriorly over theart, and anastomosed to the tip of the right atrial append-(Fig 17B). The LSVC can also be transected at its atrial

trance and moved through a tunnel created between theta and pulmonary artery to be anastomosed end-to-side toRSVC (Fig 17C). Finally, in older patients, the LSVC can

anastomosed directly to the left pulmonary artery in and-to-side fashion (Fig 17D).

atrartVC Obstruction Aftertrial Baffle in TGA Correctionother anomaly with pathophysiologic consequences in-lves SVC obstruction after creation of an atrial baffle inair of d-TGA. In past eras, anomalies of venous returnre sometimes necessarily of concern in repair, as the Sen-g or Mustard repair was the common reparative proce-re from the early 1960s to the mid 1980s. The reader iserred to other references to address issues related to atrialffle repairs.4,42 Because complete TGA is most commonlyrected with an arterial switch procedure in the current era,surgeon will more often be approached regarding prob-s from baffle obstruction. Baffle obstruction in Senning

d Mustard repairs can range from 1.4% to 13% and 4% to%, respectively.43,44 A recent series reports a rate of 5% atyears when both populations are evaluated together,45

r approach to such obstruction is illustrated in Figure 18.st line therapy is percutaneous catheter intervention, acedure that is currently highly successful in most in-nces. The complication rate from such intervention is very. The reader is referred to a recent series in which noplications are reported and which reviews the literature

these interventions.46 However, if percutaneous measuresineffective or if another surgical procedure is warrantedch as a valve repair, a baffle leak repair, or a maze proce-re), surgical revision of the patch may be necessary. Baffleks may be closed by percutaneous intervention, but theerience is rare,46-52 and treatment will be dependent onexperience of the center. When surgical patch repair isrranted, we use a generous ovoid piece of savage patch,hioned such that no undue tension is placed on the suturees and such that no redundancy is left in the patch totentially cause subsequent obstruction (Fig 19). If intra-diac repair is for some reason not possible, there are alter-tives. The SVC can be connected to the left-atrial append-or the innominate vein to the pulmonary artery.53 The

ter approachwas used in a case of acute SVC obstruction toow hemodynamic recovery before eventual SVC-right

re 18 Treatment algorithm in patients with SVC baffle obstruc-n in complete TGA.ial patch revision. If concurrent left ventricular-pulmonaryery obstruction is encountered, it may be necessary to

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Management of systemic venous anomalies 73rform a bidirectional Glenn, a procedure only possible iflmonary artery pressures are low.In summary, systemic venous anomalies are not uncom-n in patients undergoing congenital heart surgery. Suchomalies often dictate significant modifications in the con-ct and timing of cardiac surgical procedures.

knowledgmente authors thank Jos Domen for figure modification.

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Management of Systemic Venous Anomalies in the Pediatric Cardiovascular Surgical PatientImplications of Bilateral SVC in Two-Ventricle RepairsImplications of Bilateral SVC in Single-Ventricle RepairsImplications of Bilateral SVC in TransplantationImplications of Interrupted IVCUnroofed Coronary SinusSVC Obstruction After Atrial Baffle in TGA CorrectionAcknowledgmentReferences