**7.2 Surgical management**

Indications for surgical management in ccTGA patients of all ages continue to evolve and most often are determined on a case-by-case basis. Beauchanese et al. (2002) described a cohort of 44 unrepaired adult ccTGA patients. Of these, the 30 patients who required surgical intervention had significantly larger pre-operative cardiothoracic ratios on chest

Cardiopulmonary exercise testing by treadmill is an important adjunct for ccTGA patient evaluation and management. In those patients able to perform treadmill tests, exercise capacity is determined through minute ventilation, carbon dioxide production, and oxygen consumption. Impaired exercise capacity in ccTGA patients has been shown to correlate with diastolic dysfunction in the form of increased RV filling pressures as measured by tissue Doppler imaging (Tay et al., 2011). Cardiopulmonary exercise testing in combination with gadolinium-enhanced MRI has been utilized to demonstrate RV myocardial fibrosis hypothesized to be responsible for RV dysfunction (Giardini et al., 2006). Systemic RV function can also be evaluated by dobutamine stress testing, in which MRI is performed at baseline and with dobutamine infusion. Objectively defining the capacity of the systemic RV to respond to stress may guide treatment on both initial and follow-up evaluations (Dodge-Khatami et al., 2002; Fratz et al., 2008). Sequential testing, performed either by exercise testing or by dobutamine stress test, is useful to assess overall cardiopulmonary function

CHF medical management for the ccTGA patient with systemic RV has been extrapolated from CHF therapy for LV failure. This primarily includes β-adrenergic receptor blockade (βblockers), diuretics and afterload-reducing agents with an angiotensin-converting enzyme (ACE) inhibitor (Winter et al., 2009). Digoxin may also be useful for its inotropic and antiarrhythmic effects. Angiotensin receptor blockade with losartan was evaluated in a multicenter, randomized, placebo-controlled clinical trial by Dore and colleagues (2005) but found to have no improvement on exercise capacity and no reduction in neurohormonal levels in patients with systemic right ventricles. Overall, evidence-based therapy for optimal CHF treatment in patients with systemic RV is lacking. Beyond medication, cardiac resynchronization has emerged as a therapy for patients with impaired systemic RV function and widened QRS morphology on ECG. Increased QRS duration as a result of bundle branch block or conventional pacemaker is typically greater than 120-140 ms with some patients having QRS duration >200 ms. Such electromechanical dyssynchrony creates inefficiency in ventricular ejection, whereas restoring synchrony has been shown to decrease QRS duration with improvement in RV filling time, ejection fraction, and overall CHF symptoms (Diller et al., 2006; Janousek et al., 2004; Kordybach et al., 2009). Takemoto et al. (2010) reports the use of transvenous permanent para-Hisian pacing in an 8 year old with ccTGA. Restoration of cardiac synchrony decreased the QRS duration from 198 ms to 94 ms, decreased interventricular conduction delay from 137 ms to 37 ms, and improved the patient's CHF symptoms from NYHA (New York Heart Association) class III to NYHA class II over a period of 6 months. Limitations in cardiac resynchronization therapy include difficulty in percutaneous lead delivery, although this has successfully been accomplished

Indications for surgical management in ccTGA patients of all ages continue to evolve and most often are determined on a case-by-case basis. Beauchanese et al. (2002) described a cohort of 44 unrepaired adult ccTGA patients. Of these, the 30 patients who required surgical intervention had significantly larger pre-operative cardiothoracic ratios on chest

**6.6 Exercise and stress testing** 

and response to medical or surgical therapy.

even in ccTGA cases of dextrocardia (Malecka et al., 2010).

**7. Management** 

**7.1 Medical management** 

**7.2 Surgical management** 

radiographs, and had moderate to severe or severe systemic AV valve regurgitation. The ejection fraction of the systemic ventricle between the operated and unoperated groups was not statistically significant (Beauchesne et al., 2002). As discussed previously and depicted in Figure 2, nearly 2/3 of unrepaired ccTGA patients with associated defects will have developed CHF by the age of 45 years. Even asymptomatic adults with ccTGA have been shown by echocardiography to have RV dysfunction through the use of tissue Doppler quantification techniques (Bos et al. 2006). Thus the natural evolution of ccTGA for the majority of patients is eventual RV dysfunction and TV regurgitation. It is postulated that progression to failure in a systemic RV is unavoidable because the RV and TV are not anatomically suited to withstand the systemic pressure for which the LV and MV are intended. One mechanism thought to contribute to progressive RV decompensation is worsening TR from annular dilation and/or displacement of the septal leaflet of the TV as the RV remodels to accommodate systemic afterload.


Table 1. ccTGA {S,L,L} Surgical Repair and Palliation. VSD, ventricular septal defect; PS, pulmonary stenosis; PA, pulmonary artery; PV, pulmonary valve; RV, right ventricle; TR, tricuspid regurgitation; BDG, Bidirectional Glenn

Depending on the age of presentation and extent of associated lesions, surgical repair may include one or more of several approaches (Table 1). In patients with a VSD and no LVOTO, "classic" or "physiologic" repair may include VSD closure only. Specific techniques must be employed in ccTGA patients to avoid damage to the conduction system during VSD closure. Because the AV conduction bundle descends along the anterior rim of the VSD and travels along the septal side of the right-sided morphologic LV, it is recommended to suture the VSD patch along the morphological right ventricular aspect of the septum. The surgical approach should be via right atriotomy and right-sided mitral valve. Ideally the VSD patch will lie partially on the morphologic LV septal aspect (to avoid damage to the TV superiorly) and partially on the morphologic RV aspect of the septum inferiorly (to avoid damage to the main conduction bundle) (Jonas, 2004). Physiologic repair may also include relief of

Congenitally Corrected Transposition of the Great Arteries 173

The "anatomic" or "Double Switch" (DS) operation was developed in response to unsatisfactory outcomes after the classic repair. Components of the DS (Figure 7A) include arterial switch with coronary artery transfer, VSD closure if necessary, and interatrial baffle by Senning or Mustard procedure. The Senning and Mustard operations, referred to as an "atrial switch," serve to direct systemic venous flow to the TV and RV and pulmonary venous flow to the mitral valve and LV. The purpose of the DS is to improve long term outcome by restoring the LV and MV to the systemic circulation. Requirements for this repair before committing the LV to the systemic workload include pre-operative LV pressure that is 80-100% systemic and normal LV wall thickness and function for a systemic LV (Duncan & Mee, 2005; Poirier et al., 2004). In the absence of LVOTO, pulmonary hypertension, or an unrestrictive VSD, the morphologic LV requires training prior to committing it to the systemic ventricle in the DS. LV training has been performed by placement of a pulmonary artery band (PAB) which is then serially tightened to introduce a greater pressure load nearing that of systemic pressure to the naïve LV. Median banding time for the purpose of LV retraining has been reported on average to be 13-14 months (Ly et al., 2009; Poirier et al., 2004; Winlaw et al., 2005). Morphologic LV reconditioning with PAB in patients with systemic RV after atrial switch for dextrotransposition of the great arteries (dTGA) has been described by Poirier et al (2004). PAB was performed in this population prior to anatomic correction or as bridge to transplant, and the success rate of completing adequate LV retraining was significantly less in patients beyond 12 years of age (20% of patients over 12 years completed the protocol, whereas 62% of patients less than 12 years were able to complete the PAB protocol, p = 0.02). Although a well defined standard for age of PAB placement in this setting is yet to be realized, it is apparent that candidacy for LV training with PAB beyond adolescence is questionable. Also concerning is report of late LV dysfunction in ccTGA patients who underwent DS operation after successful LV

Rather than performing pulmonary artery banding in symptomatic ccTGA patients with intention of anatomic repair, Metton and associates (2010) advocate the use of PAB in asymptomatic ccTGA neonates and infants with intact ventricular septum to maintain rather than train the LV. In Metton's group the TV was not repaired at PAB placement, as it was thought that PAB placement may improve TR that was present prior to banding (Ly et al., 2009). This mechanism is described by Kral Kollars et al. (2010) in 14 patients who underwent PAB for LV retraining (median age 1.1 years, range 0 to 12 years). Eleven of the 14 patients had an increase in LV pressure of ≥2/3 systolic RV pressure with PAB and demonstrated significantly decreased TR as the LV geometry became more spherical and the interventricular septum shifted toward the morphologic RV. Patients who underwent classic ccTGA repair with procedures that reduced LV pressure below that of the RV, such as VSD closure with LV to PA conduit placement, demonstrated significantly increased TR

Although it is reasonable to medically manage mild TR with anticongestive therapy and afterload reduction, surgical intervention is indicated in cases of moderate or moderate to severe TR. TV repair for ccTGA patients is rarely successful, and most patients require valve replacement, which can be problematic in young children because of the relatively large prosthesis needed to allow for growth. Palliation with PAB may therefore be reasonable in infants and young children, since it has been shown that severe TV insufficiency leading to RV dysfunction has the greatest impact on long-term survival (Kral Kollars et al., 2010;

retraining by PAB placement (Quinn et al., 2008).

postoperatively.

pulmonary stenosis (PS) and/or LV to PA conduit placement. There is, however, the possibility that decreasing LV pressure by VSD closure and/or PS relief may allow the ventricular septum to realign towards the LV, resulting in displacement of the TV septal leaflet and increasing TR (Kral Kollars et al. 2010; Said et al. 2011). In a cohort of 123 patients with ccTGA presenting for classic biventricular repair over 33 years, the surgical group undergoing repair of VSD + PS demonstrated the greatest survival whereas patients requiring TV replacement at their initial operation exhibited the shortest survival. Risk factors for death in the VSD +/- PS relief groups included pre-operative RV end diastolic pressure greater than 17mmHg and complete heart block. Survival rates at 1-, 5-, 10-, and 15 years for patients who underwent classic repair were 84%, 75%, 68%, and 61%, respectively, although 17 of the 113 patients in this subgroup underwent Fontan and achieved 100% survival in short-term follow-up (Figure 6). The univentricular pathway with Fontan was assigned to ccTGA patients for which biventricular repair was contraindicated, as in patients with straddling AV valve tissue, inaccessible or multiple VSDs, or unbalanced complete AV canals (Hraska et al. 2005). More recently Bogers et al. (2010) confirmed that classic repair in which the RV remains the systemic ventricle results in significant incidence of reoperation and overall suboptimal survival.

Fig. 6. Operative survival in ccTGA patients undergoing Fontan pathway (*dotted line*; n = 17), VSD surgery (*solid line*; n = 76), and TV surgery (*dashed line*; n = 14). Numbers of patients at risk are in parentheses. Error bars indicate 70% confidence limits. VSD, ventricular septal defect; TV, tricuspid valve. (Reprinted from *The Journal of Thoracic and Cardiovascular Surgery,* Vol. 129, No. 1, Long-term outcome of surgically treated patients with corrected transposition of the great arteries, pp. 182-191, Copyright 2005 with permission from Elsevier).

pulmonary stenosis (PS) and/or LV to PA conduit placement. There is, however, the possibility that decreasing LV pressure by VSD closure and/or PS relief may allow the ventricular septum to realign towards the LV, resulting in displacement of the TV septal leaflet and increasing TR (Kral Kollars et al. 2010; Said et al. 2011). In a cohort of 123 patients with ccTGA presenting for classic biventricular repair over 33 years, the surgical group undergoing repair of VSD + PS demonstrated the greatest survival whereas patients requiring TV replacement at their initial operation exhibited the shortest survival. Risk factors for death in the VSD +/- PS relief groups included pre-operative RV end diastolic pressure greater than 17mmHg and complete heart block. Survival rates at 1-, 5-, 10-, and 15 years for patients who underwent classic repair were 84%, 75%, 68%, and 61%, respectively, although 17 of the 113 patients in this subgroup underwent Fontan and achieved 100% survival in short-term follow-up (Figure 6). The univentricular pathway with Fontan was assigned to ccTGA patients for which biventricular repair was contraindicated, as in patients with straddling AV valve tissue, inaccessible or multiple VSDs, or unbalanced complete AV canals (Hraska et al. 2005). More recently Bogers et al. (2010) confirmed that classic repair in which the RV remains the systemic ventricle results in significant incidence

Fig. 6. Operative survival in ccTGA patients undergoing Fontan pathway (*dotted line*; n = 17), VSD surgery (*solid line*; n = 76), and TV surgery (*dashed line*; n = 14). Numbers of patients at risk are in parentheses. Error bars indicate 70% confidence limits. VSD, ventricular septal defect; TV, tricuspid valve. (Reprinted from *The Journal of Thoracic and Cardiovascular Surgery,* Vol. 129, No. 1, Long-term outcome of surgically treated patients with corrected transposition of the

great arteries, pp. 182-191, Copyright 2005 with permission from Elsevier).

of reoperation and overall suboptimal survival.

The "anatomic" or "Double Switch" (DS) operation was developed in response to unsatisfactory outcomes after the classic repair. Components of the DS (Figure 7A) include arterial switch with coronary artery transfer, VSD closure if necessary, and interatrial baffle by Senning or Mustard procedure. The Senning and Mustard operations, referred to as an "atrial switch," serve to direct systemic venous flow to the TV and RV and pulmonary venous flow to the mitral valve and LV. The purpose of the DS is to improve long term outcome by restoring the LV and MV to the systemic circulation. Requirements for this repair before committing the LV to the systemic workload include pre-operative LV pressure that is 80-100% systemic and normal LV wall thickness and function for a systemic LV (Duncan & Mee, 2005; Poirier et al., 2004). In the absence of LVOTO, pulmonary hypertension, or an unrestrictive VSD, the morphologic LV requires training prior to committing it to the systemic ventricle in the DS. LV training has been performed by placement of a pulmonary artery band (PAB) which is then serially tightened to introduce a greater pressure load nearing that of systemic pressure to the naïve LV. Median banding time for the purpose of LV retraining has been reported on average to be 13-14 months (Ly et al., 2009; Poirier et al., 2004; Winlaw et al., 2005). Morphologic LV reconditioning with PAB in patients with systemic RV after atrial switch for dextrotransposition of the great arteries (dTGA) has been described by Poirier et al (2004). PAB was performed in this population prior to anatomic correction or as bridge to transplant, and the success rate of completing adequate LV retraining was significantly less in patients beyond 12 years of age (20% of patients over 12 years completed the protocol, whereas 62% of patients less than 12 years were able to complete the PAB protocol, p = 0.02). Although a well defined standard for age of PAB placement in this setting is yet to be realized, it is apparent that candidacy for LV training with PAB beyond adolescence is questionable. Also concerning is report of late LV dysfunction in ccTGA patients who underwent DS operation after successful LV retraining by PAB placement (Quinn et al., 2008).

Rather than performing pulmonary artery banding in symptomatic ccTGA patients with intention of anatomic repair, Metton and associates (2010) advocate the use of PAB in asymptomatic ccTGA neonates and infants with intact ventricular septum to maintain rather than train the LV. In Metton's group the TV was not repaired at PAB placement, as it was thought that PAB placement may improve TR that was present prior to banding (Ly et al., 2009). This mechanism is described by Kral Kollars et al. (2010) in 14 patients who underwent PAB for LV retraining (median age 1.1 years, range 0 to 12 years). Eleven of the 14 patients had an increase in LV pressure of ≥2/3 systolic RV pressure with PAB and demonstrated significantly decreased TR as the LV geometry became more spherical and the interventricular septum shifted toward the morphologic RV. Patients who underwent classic ccTGA repair with procedures that reduced LV pressure below that of the RV, such as VSD closure with LV to PA conduit placement, demonstrated significantly increased TR postoperatively.

Although it is reasonable to medically manage mild TR with anticongestive therapy and afterload reduction, surgical intervention is indicated in cases of moderate or moderate to severe TR. TV repair for ccTGA patients is rarely successful, and most patients require valve replacement, which can be problematic in young children because of the relatively large prosthesis needed to allow for growth. Palliation with PAB may therefore be reasonable in infants and young children, since it has been shown that severe TV insufficiency leading to RV dysfunction has the greatest impact on long-term survival (Kral Kollars et al., 2010;

Congenitally Corrected Transposition of the Great Arteries 175

cardiopulmonary bypass and aortic cross-clamp times, and there is an increased risk of complete heart block and ventricular dysfunction if the existing VSD requires enlargement (Gaies et al., 2009; Shin'oka et al., 2007). Nevertheless, intermediate results in a small group of ccTGA patients with VSD and LVOTO who underwent this form of anatomic repair suggest good biventricular function and mild or no AV valve insufficiency up to 17 years

An additional variation in the DS for patients with severe RV dysfunction, hypoplasia of the RV, or abnormal right atrial anatomy includes a modified atrial switch termed the "hemi-Mustard/bidirectional Glenn," which is performed in combination with either an arterial switch or a Rastelli procedure. In this operation the interatrial baffle only includes the IVC return, as the SVC is reimplanted into the pulmonary artery to create a cavo-pulmonary Glenn shunt, and the SVC portion of the RA is oversewn. Midterm outcomes from the hemi-Mustard/Glenn as reported by Malhotra et al. (2011) are favorable and hold several advantages over the traditional Senning or Mustard atrial switch. The authors report a prolonged lifespan of the RV to PA conduit due to volume-unloading the RV, increased intra-atrial space for pulmonary venous return (and therefore less risk of pulmonary venous obstruction), and less risk for arrhythmia with the reduction in intra-atrial suture lines. It remains to be seen if the hemi-Mustard / bidirectional Glenn variant of the DS will prove

Alghamdi and associates (2006) published a meta-analysis of 11 nonrandomized studies totalling 124 ccTGA patients and compared in-hospital mortality between physiologic and anatomic repair. Patient age at time of repair ranged from 3 months to 55 years with 41% of patients undergoing definitive repair prior to 1995. Thirty patients underwent physiologic repair, 69 underwent Rastelli-type anatomic repair, and 25 received anatomic repair with arterial switch. The Rastelli-type anatomic repair had significantly lower hospital mortality while era of operation before 1995 demonstrated an increased mortality risk. A large risk analysis performed by Shin'oka et al. (2007) combined ccTGA patients with a group of systemic RV patients with discordant AV connections, (n=189) and compared long-term results of definitive surgical repair with respect to hospitalization, late mortality, and reoperation. Risk factors for hospital death included preoperative moderate TR and intraoperative cardiopulmonary bypass time of over 240 minutes. The presence of TR was also a risk factor for late mortality. Reoperation risks included preoperative cardiomegaly (cardiothoracic ratio of >0.6) and presence of TR, operative need for VSD enlargement, and patient size of <10 kg. Although survival of classic repair in patients without TR was satisfactory in comparison to anatomic repair, patients with ccTGA and discordant AV connections with TR demonstrated improved survival with anatomic repair. More recently Lim and colleagues (2010) report results from a multicenter study including 167 patients who underwent biventricular ccTGA repair. Of the patients studied, 123 underwent physiologic repair (ASD or VSD closure, TV surgery, and/or pulmonary ventricle to PA conduit placement), and 44 underwent anatomic repair (atrial + arterial switch or atrial + interventricular re-routing procedure) over the years 1983 – 2009. Long-term results of biventricular repair revealed an estimated survival of 83.3% ± 0.05% at 25 years. The incidence of complete heart block was lower for the anatomic repair group, and there was a late mortality of 5.9% after physiologic repair in comparison to 0% after anatomic repair.

post-operatively (Hörer et al., 2007).

favorable in long-term studies.

**8. Outcomes: Physiologic vs. anatomic repair** 

Prieto et al., 1998). Several groups have concluded that TV replacement should be considered at the earliest sign of RV dysfunction, with recommendations to consider operation before systemic ventricular ejection fraction (EF) decreases below 40 and 44% (Mongeon et al., 2011; Van Son et al., 1995).

Fig. 7. The Double Switch operation for ccTGA. (A.) The double-switch anatomic surgical repair for ccTGA with VSD consists of an arterial switch, VSD closure, and atrial venous switch (not shown) by intra-atrial baffle operation (i.e., Senning or Mustard repair). (B.) The double-switch for ccTGA with left ventricle (LV) outflow tract obstruction includes an anatomical LV - aorta (Ao) baffle (i.e., Rastelli repair) and anatomical right ventricle (RV) pulmonary artery (PA) conduit. Although not pictured, the baffle and conduit repair are also in combination with an atrial switch. (With permission from Springer Science + Business Media: *Current Treatment Options in Cardiovascular Medicine*, Congenitally Corrected Transposition of the Great Arteries: An Update, Vol. 9, 2007, pp. 405-413, Graham, T.P., Markham, L., Parra, D.P., & Bichell, D., Figure 1).

The combination of progressive systemic RV dysfunction and TR has lead to the consideration of a variation in DS operation for patients with LVOTO. Rather than combining the atrial and arterial switches, the Senning or Mustard atrial switch procedure is combined with a Rastelli operation, in which the LV outflow is channeled from the LV through a large VSD to the aorta and an RV to PA conduit is placed (Figure 7B). This operation is technically challenging and subject to the need for conduit replacements as well as possible reoperation for interatrial or interventricular baffle obstructions. Specific to the Senning / Rastelli operation, risk factors associated with death include longer

Prieto et al., 1998). Several groups have concluded that TV replacement should be considered at the earliest sign of RV dysfunction, with recommendations to consider operation before systemic ventricular ejection fraction (EF) decreases below 40 and 44%

Fig. 7. The Double Switch operation for ccTGA. (A.) The double-switch anatomic surgical repair for ccTGA with VSD consists of an arterial switch, VSD closure, and atrial venous switch (not shown) by intra-atrial baffle operation (i.e., Senning or Mustard repair). (B.) The double-switch for ccTGA with left ventricle (LV) outflow tract obstruction includes an anatomical LV - aorta (Ao) baffle (i.e., Rastelli repair) and anatomical right ventricle (RV) pulmonary artery (PA) conduit. Although not pictured, the baffle and conduit repair are also in combination with an atrial switch. (With permission from Springer Science + Business Media: *Current Treatment Options in Cardiovascular Medicine*, Congenitally

Corrected Transposition of the Great Arteries: An Update, Vol. 9, 2007, pp. 405-413, Graham,

The combination of progressive systemic RV dysfunction and TR has lead to the consideration of a variation in DS operation for patients with LVOTO. Rather than combining the atrial and arterial switches, the Senning or Mustard atrial switch procedure is combined with a Rastelli operation, in which the LV outflow is channeled from the LV through a large VSD to the aorta and an RV to PA conduit is placed (Figure 7B). This operation is technically challenging and subject to the need for conduit replacements as well as possible reoperation for interatrial or interventricular baffle obstructions. Specific to the Senning / Rastelli operation, risk factors associated with death include longer

T.P., Markham, L., Parra, D.P., & Bichell, D., Figure 1).

**A. B.**

(Mongeon et al., 2011; Van Son et al., 1995).

cardiopulmonary bypass and aortic cross-clamp times, and there is an increased risk of complete heart block and ventricular dysfunction if the existing VSD requires enlargement (Gaies et al., 2009; Shin'oka et al., 2007). Nevertheless, intermediate results in a small group of ccTGA patients with VSD and LVOTO who underwent this form of anatomic repair suggest good biventricular function and mild or no AV valve insufficiency up to 17 years post-operatively (Hörer et al., 2007).

An additional variation in the DS for patients with severe RV dysfunction, hypoplasia of the RV, or abnormal right atrial anatomy includes a modified atrial switch termed the "hemi-Mustard/bidirectional Glenn," which is performed in combination with either an arterial switch or a Rastelli procedure. In this operation the interatrial baffle only includes the IVC return, as the SVC is reimplanted into the pulmonary artery to create a cavo-pulmonary Glenn shunt, and the SVC portion of the RA is oversewn. Midterm outcomes from the hemi-Mustard/Glenn as reported by Malhotra et al. (2011) are favorable and hold several advantages over the traditional Senning or Mustard atrial switch. The authors report a prolonged lifespan of the RV to PA conduit due to volume-unloading the RV, increased intra-atrial space for pulmonary venous return (and therefore less risk of pulmonary venous obstruction), and less risk for arrhythmia with the reduction in intra-atrial suture lines. It remains to be seen if the hemi-Mustard / bidirectional Glenn variant of the DS will prove favorable in long-term studies.

#### **8. Outcomes: Physiologic vs. anatomic repair**

Alghamdi and associates (2006) published a meta-analysis of 11 nonrandomized studies totalling 124 ccTGA patients and compared in-hospital mortality between physiologic and anatomic repair. Patient age at time of repair ranged from 3 months to 55 years with 41% of patients undergoing definitive repair prior to 1995. Thirty patients underwent physiologic repair, 69 underwent Rastelli-type anatomic repair, and 25 received anatomic repair with arterial switch. The Rastelli-type anatomic repair had significantly lower hospital mortality while era of operation before 1995 demonstrated an increased mortality risk. A large risk analysis performed by Shin'oka et al. (2007) combined ccTGA patients with a group of systemic RV patients with discordant AV connections, (n=189) and compared long-term results of definitive surgical repair with respect to hospitalization, late mortality, and reoperation. Risk factors for hospital death included preoperative moderate TR and intraoperative cardiopulmonary bypass time of over 240 minutes. The presence of TR was also a risk factor for late mortality. Reoperation risks included preoperative cardiomegaly (cardiothoracic ratio of >0.6) and presence of TR, operative need for VSD enlargement, and patient size of <10 kg. Although survival of classic repair in patients without TR was satisfactory in comparison to anatomic repair, patients with ccTGA and discordant AV connections with TR demonstrated improved survival with anatomic repair. More recently Lim and colleagues (2010) report results from a multicenter study including 167 patients who underwent biventricular ccTGA repair. Of the patients studied, 123 underwent physiologic repair (ASD or VSD closure, TV surgery, and/or pulmonary ventricle to PA conduit placement), and 44 underwent anatomic repair (atrial + arterial switch or atrial + interventricular re-routing procedure) over the years 1983 – 2009. Long-term results of biventricular repair revealed an estimated survival of 83.3% ± 0.05% at 25 years. The incidence of complete heart block was lower for the anatomic repair group, and there was a late mortality of 5.9% after physiologic repair in comparison to 0% after anatomic repair.

Congenitally Corrected Transposition of the Great Arteries 177

pursue. The age and eligibility of pulmonary artery banding for LV retraining is yet to be standardized, and as pulmonary banding for maintenance of LV function in the asymptomatic infant is further evaluated, individualized decisions such as these are sure to

Alghamdi, A.A., McCrindle, B.W., & Van Arsdell, G.S. (2006). Physiologic Versus Anatomic

Anderson, R.H., Becker, A.E., Arnold, R., & Wilkinson, J.L. (1974). The Conducting Tissues

Beauchesne, L.M., Warnes, C.A., Connolly, H.M., Ammash, N.M., Tajik, A.J., & Danielson,

Bogers, A.J., Head, S.J., de Jong, P.L., Witsenburg, M., & Kappetein, A.P. (2010). Long term

Bos, J.M., Hagler, D.J., Silvilairat, S., Cabalka, A., O'Leary, P., Daniels, O., Miller, F.A., &

Bottega, N.A., Kapa, S., Edwards, W.D., Connolly, H.M., Munger, T.M., Warnes, C.A., &

Carey, L.S. & Ruttenberg, H.D. (1964). Roentgenographic features of congenital corrected

*Medicine*, Vol. 92, No. 3, (September 1964), pp. 623-651, ISSN 0002-9580. Caso, P., Ascione, L., Lange, A., Palka, P., Mininni, N., & Sutherland, G.R. (1998). Diagnostic

Chang, H-Y., Yin, W-H., Hsiung, M-C., & Young, M-S. (2009). A Heart Reversed Triply: Situs

*Allied Techniques*, Vol. 26, No. 5, (May 2009), pp. 617-621, ISSN 1540-8175. Connolly, H.M., Grogan, M., & Warnes, C.A. (1999). Pregnancy among women with

*Journal*, Vol. 135, No. 1, (January 1998), pp. 43-50, ISSN 0002-8703.

*Cardiology*, Vol. 40, No. 2, (July 2002), pp. 285-290, ISSN 1558-3597.

Vol. 5, Article No. 74, (September 2010), ISSN 1749-8090.

Vol. 19, No. 8, (August 2006), pp. 1033-1037, ISSN 0894-7317.

(October 2009), pp. 1450-1456, ISSN 1547-5271.

Repair of Congenitally Corrected Transposition of the Great Arteries: Meta-Analysis of Individual Patient Data. *Annals of Thoracic Surgery*, Vol. 81, No. 4, (April

in Congenitally Corrected Transposition. *Circulation*, Vol 50, (1974), pp. 911-923,

G.K. (2002). Outcome of the Unoperated Adult Who Presents With Congenitally Corrected Transposition of the Great Arteries. *Journal of the American College of* 

follow up after surgery in congenitally corrected transposition of the great arteries with a right ventricle in the systemic circulation. *Journal of Cardiothoracic Surgery,* 

Abraham, T.P. (2006). Right Ventricular Function in Asymptomatic Individuals with a Systemic Right Ventricle. *Journal of the American Society of Echocardiography*,

Asirvatham, S.J. The cardiac veins in congenitally corrected transposition of the great arteries: Delivery options for cardiac devices. *Heart Rhythm*, Vol. 6, No. 10,

transposition of the great vessels: A comparative study of 33 cases with a roentgenographic classification based on the associated malformations and hemodynamic states. *American Journal of Roentgenology, Radium Therapy, and Nuclear* 

value of transesophageal echocardiography in the assessment of congenitally corrected transposition of the great arteries in adult patients. *American Heart* 

Inversus Totalis with Congenitally Corrected Transposition of the Great Arteries in a Middle-Aged Woman. *Echocardiography: A Journal of Cardiovascular Ultrasound and* 

congenitally corrected transposition of the great arteries. *Journal of the American College of Cardiology*, Vol. 33., No. 6, (May 1999), pp. 1692-1695, ISSN 1558-3597.

produce much debate.

ISSN 1524-4539.

2006), pp. 1529-1535, ISSN 0003-4975.

**11. References** 

Freedom from systemic AV valve regurgitation and ventricular dysfunction was significantly higher after anatomic repair. The authors concluded that anatomic is superior to physiologic repair in patients with two adequately sized ventricles. However high risk groups such as those patients with RV dysfunction or the need for LV training warrant careful selection prior to undergoing anatomic repair. Taken together, these outcomes favor anatomic over classic /physiologic repair with careful preoperative assessment of TR for the purpose of risk stratification.
