Double Switch Procedure Research Articles

Surgical Outcomes in Patients Undergoing a Double Switch Operation for Corrected Transposition

BackgroundCongenitally corrected transposition of the great arteries (CC-TGA) is a rare and complex form of congenital heart disease. Results of physiologic repair proved disappointing due to late right ventricular dysfunction and/or tricuspid regurgitation. The current study was performed to evaluate surgical outcomes in patients undergoing a double switch for CC-TGA. MethodsThis was a retrospective review of 121 patients who underwent a double switch over a 2-decade time frame (2002-2023). Patients were a median age of 32 months. Before the double switch, 49 of 121 patients (40%) had undergone left ventricular retraining. ResultsSixty-seven patients underwent an arterial switch, and 54 underwent a Rastelli procedure. There were 4 in-hospital deaths (3.3%), including 3 who had a Rastelli procedure (5.6%) and 1 who had an arterial switch (1.5%). At a median follow-up of 30 months, there were 4 late deaths (2 Rastelli and 2 arterial switch). Combined early and late mortality was 9.3% for the Rastelli and 4.5% for arterial switch. Combined mortality was 2.0% for patients who required left ventricular retraining vs 9.7% for those who did not. For the 117 patients discharged from the hospital, 93% have normal or low-normal left ventricular function, and 96% have mild or less neoaortic insufficiency. ConclusionsSurgical outcomes in patients undergoing a double switch procedure have been excellent both in the short- and midterm. However, the Rastelli procedure was associated with a more than 2-fold increase in mortality risk compared with the arterial switch.

Results of the Double Switch Operation in Patients Who Previously Underwent Left Ventricular Retraining.

Congenitally corrected transposition of the great arteries (CC-TGA) is a complex form of congenital heart disease that has numerous subtypes. While most patients with CC-TGA have a large ventricular septal defect (VSD) and pulmonary stenosis, there are some patients who have either no VSD or a highly restrictive VSD. These patients will require left ventricular (LV) retraining prior to double switch. The purpose of this study was to review our experience with the double switch procedure in patients who had previously undergone LV retraining. This was a retrospective review of a single institution experience with the double switch procedure in patients who had undergone LV retraining (2002-present). Forty-five patients underwent double switch following LV retraining. Of these, 39 had an arterial switch with hemi-Mustard/bidirectional Glenn and six had a Senning. The median cross-clamp time was 135 min (range 71-272) and median bypass time was 202 min (range 140-430 min). Median hospital length of stay was eight days (range 4-108). There were no in-hospital deaths. Median duration of follow-up was 30 months (range 0-175). One patient subsequently underwent heart transplantation and died 65 months following double switch. At follow-up, 41 of the 44 survivors (93%) have normal or low normal LV function and 40 of the 44 survivors (91%) have no or trace mitral regurgitation. The data demonstrate early and mid-term survival of 100% and 97%. Ninety-three percent had preserved LV function. These results suggest that patients with CC-TGA who undergo LV retraining and double switch can have excellent clinical outcomes.

Outcomes of double switch procedure in patients with congenitally corrected transposition of the great arteries with or without associated VSD

Anatomical repair by double switch procedure (DS) for patients with congenitally corrected transposition of the great arteries (ccTGA) remains under debate. The outcomes after DS for ccTGA without LVOTO, with specific focus on the left ventricle (LV) performance in patients with intact ventricular septum (IVS) were assessed. Since 1994, 39 patients underwent DS: 9 had an IVS, and 30 had an associated VSD. Palliative procedure consisted in pulmonary artery banding (PAB) for 31 patients (79%): 9 (twice in 5) for tricuspid regurgitation (TR) and 22 for hemodynamically significant associated VSD. Survival and LV performance in follow up were analyzed. PAB was efficient to diminish TR significantly in all 9. The median age at repair was 22 months (12 days–13 years). Associated VSD closure was performed in 76% of patients ( n = 30). Four patients required peri-operative pacemaker implantation. Hospital mortality occurred in 1 (2.5%: 70% CI 1–8%) neonate who underwent DS and aortic arch repair: he died because of low output syndrome. Two (5%) patients required early reoperation for pulmonary venous pathway stenosis release. Median follow up was 13.7 years (range: 3 months–26 years). Two late deaths occurred 16 and 84 months postoperatively because of refractory LV dysfunction: both patients had IVS and benefited from LV retraining. Six patients (16%) required 7 reoperations during follow up. At last visit, 5 patients (14%) presented with LV dysfunction (LV ejection fraction < 50%) requiring medical treatment while remaining 31 (84%) were asymptomatic. Actuarial survival curves are shown in Fig. 1 . Mortality was significantly higher in the IVS group compared to the VSD group (33% vs. 0%, P = 0.01). The DS appeared to be the procedure of choice for ccTGA associated with VSD. PAB was efficient for the management of TR in early life. Our Results suggest that the strategy of LV retraining by PAB to achieve DS at any price remains questionable.

Left ventricular retraining in corrected transposition: Relationship between pressure and mass

BackgroundA subset of patients with corrected transposition of the great arteries (CC-TGA) will require left ventricular (LV) retraining before undergoing a double-switch procedure. LV retraining results in an immediate increase in LV pressure but not in LV mass. The purpose of this study was to evaluate the relationship between LV pressure and mass during LV retraining. MethodsThis was a retrospective review of 36 patients with CC-TGA who were enrolled in LV retraining. The median age at enrollment was 12 months. The majority (82%) had an Ebstenoid tricuspid valve or moderate to severe tricuspid valve regurgitation before pulmonary artery banding. ResultsTwenty-seven of the 36 patients underwent LV retraining and a double switch, including 14 patients with a single pulmonary artery band (PAB), 12 patients with 2 PABs, and 1 patient with 3 PABs. There was no early or late mortality associated with these procedures. Ninety percent of the patients demonstrated a linear relationship between LV pressure and mass; however, 3 patients (10%) demonstrated a different LV retraining pattern characterized by excess LV mass compared to LV pressure. Two of these 3 patients are LV retraining failures, whereas the third patient did undergo a double switch. ConclusionsThe data show that the majority of patients enrolled in LV retraining have a predictable relationship between LV mass and pressure. A minority demonstrate an excess accrual of mass with respect to pressure load. These results suggest there may be an important distinction between normal and pathological hypertrophy during the LV retraining process.

Promising Outcome of Anatomic Correction of Corrected Transposition of the Great Arteries

Anatomic correction of corrected transposition of the great arteries with associated lesions, utilizing the morphologic left ventricle as a systemic pumping chamber, is the preferred method in many centers. The purpose of this study was to analyze functional outcome after anatomic correction. Between Jan 1997 and May 2016, 63 patients with corrected transposition of the great arteries and associated lesions underwent anatomic correction. Forty-two patients (67%) underwent palliation before correction, including 14 patients (22%) who required training of systemic ventricle. The double switch procedure was performed in 37 patients; 25 patients underwent the Senning-Rastelli operation, and 1 patient underwent the Senning-Nikaidoh procedure. The median age at correction was 1.6 ± 3.7(SD) years (range, 0.2 to 17.8 years). The survival and freedom from any event was 95% and 71%, respectively, at 15-year follow-up. The combined freedom from death, failure of systemic ventricle, or heart transplant was 93% at 15-year follow-up regardless of procedure type. Sinus rhythm was present in 49 patients, with 14 patients requiring pacemaker (22%)-8 preoperatively, 4 early postoperatively, and 2 late postoperatively. Neurological development is normal in all patients. Fifty-four percent of the patients are not on medication. Anatomic correction of corrected transposition of the great arteries is a safe procedure that provides encouraging survival and functional benefits. Ninety-three percent preservation of morphological left ventricle function in 15 years of follow-up supports the concept of anatomic correction. Longer follow-up is needed to confirm superiority of this approach over other management strategies.

Left Ventricular Retraining and Double Switch in Patients With Congenitally Corrected Transposition of the Great Arteries

Congenitally corrected transposition of the great arteries (CC-TGA) is a complex form of congenital heart defect with numerous anatomic subgroups. The majority of patients with CC-TGA are excellent candidates for a double-switch procedure. However, in the absence of an unrestrictive ventricular septal defect or subpulmonary stenosis, the left ventricle (LV) may undergo involution and require retraining prior to double switch. The purpose of this study was to review our experience with patients having CC-TGA who required LV retraining prior to a double-switch procedure. This was a retrospective review of 24 patients with CC-TGA who were enrolled in an LV retraining program in preparation for a double-switch procedure. The median age at the time of enrollment for retraining was 11 months (range 1 month-24 years). The average left ventricle to right ventricle pressure ratio was 0.39 ± 0.07 prior to intervention. All 24 patients underwent placement of an initial pulmonary artery band (PAB) for LV retraining. Eighteen (75%) of the 24 patients underwent a double-switch procedure with no operative mortality. Of these 18 patients, 9 had a single PAB and 9 required a second band for retraining. Six patients have not undergone a double-switch procedure to date. Five patients are good candidates for a double switch and are 2 weeks, 3 weeks, 4 weeks, 8 months, and 35 months since their last PAB. One patient died from a noncardiac cause 26 months after PAB retightening. The 18 patients who underwent a double switch were followed for an average of 5 ± 1 years (range 0.1-10.3 years). There has been no late mortality, and only 2 patients required further reinterventions. The data demonstrate that LV retraining has been highly effective in this select group of patients with CC-TGA. The data also demonstrate that the results of the double-switch procedure have been excellent at midterm follow-up. These results suggest that LV retraining and double switch offer a reliable strategy option for patients with CC-TGA.

Double Switch Procedure and Surgical Alternatives for the Treatment of Congenitally Corrected Transposition of the Great Arteries.

We present our experience with the double switch operation in sixteen patients with congenitally corrected transposition of the great arteries. We enrolled 16 patients with congenitally corrected transposition of the great arteries operated by a single surgeon between 1995 and 2015. The mean age was 25 months (range 4 to 72 months) and the mean body weight was 8.9 kg (range 4.3 to 19 kg) at the time of operation. We encountered seven patients with moderate to severe tricuspid regurgitation, five of which had Ebstein anomaly. We performed a combination of atrial and arterial switch procedures in 11 cases, one of which had a concomitant coarctation of the aorta that was repaired along with the double switch procedure. Atrial switch and the Rastelli procedures were performed in three cases with concomitant pulmonary stenosis. A combination of arterial switch, Hemi-Mustard procedure, and bidirectional cavopulmonary anastomosis was performed in two cases. During a mean follow-up period of 67 months (range three months to 18 years), we encountered one early postoperative mortality related to intracerebral bleeding. All but one of the patients are now in NYHA class I-II. Congenitally corrected transposition of the great arteries is a rare congenital cardiac anomaly in which the results of the anatomical repair with double switch operation appear to be superior to that achieved by a physiological repair.

The results of a two-stage double switch operation for congenital corrected transposition of the great arteries with a deconditioned morphologically left ventricle.

The purpose of this retrospective study was to evaluate a two-stage double switch operation, morphological left ventricular (mLV) retraining followed by an atrial-arterial switch operation, in the management of patients with congenitally corrected transposition of the great arteries (CCTGA) and a deconditioned mLV. Between May 2005 and May 2011, 14 patients with CCTGA and a deconditioned mLV anomaly underwent the two-stage double switch operation. There were eight males and six females aged between 2.5 and 72 months (mean: 34.4 ± 24.0 months) old and weighing from 5 to 23 kg (mean: 12.7 ± 4.9 kg). The major associated malformations included: tricuspid regurgitation (TR, n = 13); restrictive ventricular septal defect (n = 10); atrial septal defect or patent foramen ovale (n = 7); mild pulmonary stenosis (n = 5) and patent ductus arteriosus (n = 4). These patients underwent morphological left ventricular retraining by means of pulmonary artery banding under general anaesthesia, which was then followed by a double switch operation under general anaesthesia and cardiopulmonary bypass. There were no deaths or complications during the hospital stay or follow-up for the mLV retraining. In comparison with preoperative conditions, the mLV end-diastolic diameter (mLVEDd), the posterior wall thickness of the mLV and the mLV/mRV pressure ratio were all increased; the interventricular septum had moved partially to the midline position and TR had decreased. After the atrial-arterial switch procedure, 2 patients died during the perioperative period. The causes of death included serious cardiac arrhythmia with circulatory collapse and sudden death. The others were followed up for 2-8 years: 1 patient died from serious cardiac arrhythmias with circulatory collapse in the follow-up period. With regard to the others, 8 were evaluated as New York Heart Association Functional Class I, and the other 3 as Class II. Moderate aortic valve regurgitation was noted in 3 patients and moderate mitral regurgitation in 1 patient. For CCTGA children with degraded mLV, the two-stage double switch procedure can be performed with low mortality and morbidity and may be an appealing alternative to conventional repair. mLV retraining should be performed as early as possible. The second-stage atrial-arterial switch procedure showed satisfactory early and mid-term results. More attention should be paid to the long-term function of the mLV and the aortic valve.

Transvenous cardiac resynchronization therapy in congenitally corrected transposition of the great arteries and progressive heart failure

Congenitally corrected transposition of the great arteries Transvenous cardiac resynchronization therapy Progressive heart failure A 24-year-old student with congenitally corrected transposition of the great arteries (ccTGA) who was unable to continue his study due to progressive signs of heart failure (NYHA III) was admitted to our department. He had a history of a double switch procedure including modified Senning- and arterial switch-operation as well as a surgical correction of the right pulmonary artery, Lecompte manoeuvre and resection of subpulmonary stenosis in the left ventricular outflow tract at the age of 6 years. Furthermore a ventricular septal defect was closed with a patch. During the surgical procedure the native systemic aortic valve was injured and replaced by a prosthetic aortic valve. Due to postsurgical third degree AV-block a DDD-pacemaker with epicardial leads was implanted in the abdomen. After exit-block of the epicardial right ventricular (RV) lead a transvenous RV-lead was implanted at the age of 16 years. At presentation NT pro-BNP levels were markedly elevated and 12lead-ECG showed permanent RV-stimulation and undersensing of the atrial lead (Panel A). Transthoracic echocardiography revealed a significantly dilatedand impairedsystemic ventricle with anejectionfraction of b25% and a normally functioning prosthetic aortic valve. Pacemaker interrogation exhibited malfunction (exit block and undersensing) of the atrial epicardial lead and increased thresholds of the transvenous RV-lead. Given the clinical presentation and the underlying ccTGA, the chronic dyssynchrony due to permanent RV-stimulation and atrio-ventricular dyssynchrony due to atrial undersensing were thought to be the most likely causes for the progression of heart failure. After a discussion with the patient and his family, a biventricular implantable cardioverter defibrillator (CRT-ICD) was implanted after complete removal of the abdominal pacemaker and partial removal of the transvenous RV-lead (Panel B; Film A): A defibrillator lead was placed in the apex of the systemic ventricle and an atrial lead was placed at the inferior free wall of the right atrium. Venography of the reconnected coronary sinus revealed a prominent infero-posterolateral vein (Panel C; Film B). Subsequently, a left ventricular pacing lead could be advanced successfully into this left ventricular vein and a CRT-ICD was implanted subpectorally (Panel D; Film C). Follow-up assessment at 6 months revealed a significant improvement in functional class (NYHA I; he was able to continue his studies), anincreaseofejectionfractionofthesystemicventricleandasignificant

Palliative pulmonary artery banding versus anatomic correction for congenitally corrected transposition of the great arteries with regressed morphologic left ventricle: Long-term results from a single center

We aimed to compare the long-term results between palliative pulmonary artery banding and anatomic correction for congenitally corrected transposition of the great arteries with regressed morphologic left ventricle. From 2003 to 2012, 40 consecutive patients underwent first-stage pulmonary artery banding. The second-stage operation-double switch-was performed in 15 patients (double-switch group). The other 25 patients retained pulmonary artery banding without further operation (pulmonary artery banding group). In-hospital mortality, long-term mortality, and heart function were studied as primary outcomes. The median time of follow-up was 3.4±0.7 years (range, 6 months-9.5 years). Overall survival rate was 66.7% in the double-switch group versus 96.0% in the pulmonary artery banding group (P=.03). The ratio of New York Heart Association functional class I-II (80.0% vs 95.9%; P=.02) and the mean functional left ventricle ejection fraction (51.4%±9.6% vs 61.0%±6.4%; P=.01) were higher in the pulmonary artery banding group at follow-up. In univariate analysis, age at pulmonary artery banding was the only risk factor for late deaths (odds ratio, 7.30; P=.01) and left ventricle dysfunction (odds ratio, 4.77; P=.03) after the double switch. For patients who experienced prolonged pulmonary artery banding, mean oxygen saturation was 95%±3.1% and the trans-banding pressure gradient was 46.9±21.5 mm Hg. In patients with congenitally corrected transposition of the great arteries with deconditioned morphologic left ventricle pulmonary artery banding may be considered an ideal procedure because it allows left ventricle training while improving tricuspid regurgitation. Compared with the double-switch procedure after pulmonary artery banding, prolonged palliative pulmonary artery banding provided a lower mortality rate and indicated better cardiac function.

The palliative pulmonary banding may provide better outcomes than anatomical correction of congenitally corrected transposition of the great arteries with involuted morphologic left ventricle

Background: Recently sub-optimal outcomes were reported after the second stage anatomical correction of congenitally corrected transposition of the great arteries (cc-TGA) with morphologic Left Ventricular (mLV) regression. We followed up all the patients underwent Pulmonary Artery Banding (PAB) with/without the second stage Double Switch (DS) operation to compare the outcomes between the DS and the prolonged palliative PAB. Methods: From 2005 to 2012, 40 consecutive patients diagnosed with cc-TGA with morphological LV regression were included in this retrospective cohort study. Every patient underwent the first stage PAB. The second stage DS was performed in 15 patients (group I) while the palliative PAB was prolonged in the other 25 patients (group II). The median age at PAB was 3.1 years (range, 3 months to 8 years) and the mean weight at PAB was 11.5kg (range, 5.0-21.5kg). Mean follow-up was 3.4±0.7years (range, 6 months to 9.5 years). Results: Overall survival rate was 66.7% in group I vs. 96.0% in group II (P=0.03). Three patients had at least moderate neo-aortic regurgitation at latest echocardiography and 2 patients had severe post-double switch operative arrhythmias in group I. The ratio of NYHA I–II (80.0% in group I vs. 95.9% in group II, P=0.02) and the mean systemic ventricular ejection fraction (51.4±9.6% in group I vs. 61.0±6.4% in group II, P=0.006) were higher in group II at follow up. No PAB relating cyanosis or ventricular dysfunction was found in group II. The trans-banding pressure gradient was 46.9±21.5mmHg in group II. View this table: Outcomes between groups Conclusions: The results of the mLV retraining followed by a DS procedure for cc-TGA with morphological LV regression were unsatisfactory. Compared to the DSO, the prolonged pallative PAB provided a lower mortality and indicated a better cardiac function. As a result, performing the DSO on these patients needs careful consideration and the prolonged pallative PAB may be considered as an ideal procedure. Further follow-up is warranted with a focus on the exercise tolerance.

The Atrial Switch Component of the Double-Switch Procedure: Management of Venous Anomalies and Role of the Superior Cavopulmonary Connection

The atrial switch procedures were once amongst the standard armamentarium of the cardiac surgeon. However, since the advent of the arterial switch, the Senning and Mustard procedures would almost have been consigned to history— were it not for their role in anatomical repair of congenitally corrected transposition of the great arteries (ccTGA) as part of the double-switch (and Rastelli-Senning) procedures. The double-switch procedure has brought about the renaissance of the atrial inversion procedures over recent years, with the Senning procedure being the most popular version. This poses particular problems because (1) it has little in common with other cardiac procedures and so is difficult to learn and (2) ccTGA is associated with unusual cardiac positions and venous connections that require adaptations and variations on the standard procedure.

Congenital Complete Heart Block: Single Tertiary Centre Experience

Congenital complete heart block (CCHB) is an uncommon although important and potentially serious condition contributing to neonatal morbidity and mortality. To study the characteristics and outcomes of infants born with CCHB at a single tertiary centre. A retrospective review of all infants with CCHB over the last 20 years was carried out to determine the outcomes, and the indications and timing of pacemaker insertion. Fifteen live born infants (10 male, 5 female) with CCHB were identified. Their mean (and SD) gestation and birth weight were 37 (3.3) weeks and 3100 (448) grams respectively. Maternal systemic lupus erythematosus (SLE) antibodies were present in eight (53%) pregnancies and two infants had congenitally corrected transposition of the great arteries (cCTGA). The median heart rate/minute at birth was 60 (range 40-80). Thirteen (87%) patients to date required a pacemaker. The median age of insertion of a pacemaker device was six months (range 2 days-16 years). All patients were paced epicardially - six initially with a single chamber and five with a dual chamber pacemaker. At the time of generator change, dual chamber pacemakers were used. The median life of an implanted pacemaker was six years (3-10 years). Except for a patient with cCTGA who has undergone a double switch procedure, all the patients had good systemic ventricular function. There was one death in the group unrelated to CCHB. CCHB is a uncommon but potentially serious condition in infancy. While a significant number of infants need a pacemaker, the overall outcome of infants with CCHB in our experience is good.

Aortic Translocation Using the Hemi-Mustard Procedure for Corrected Transposition

The management of congenitally corrected transposition of the great arteries and associated lesions is frequently challenging. Restrictive ventricular septal defect and mild pulmonary stenosis are contraindications to the double switch procedure, including the atrial-Rastelli switch procedure, due to the production of postoperative left ventricular outflow tract obstruction. We describe a case of aortic translocation using the hemi-Mustard procedure after left ventricular training in order to prevent postoperative left ventricular outflow obstruction.

306 20 years of follow-up in 132 Senning procedures: late results

Senning procedure was performed for the first time in France at Bordeaux University Hospital in 1965. Follow up have come out focusing on failing systemic right ventricle and rhythmic complication. The aim of this work is to identify prognosis factors of reoperation, arrhythmias and right ventricular dysfunction. This single institution study is a retrospective analysis of 132 patients, with simple (105 patients) and complex (27 patients) transposition of the great arteries, after a Senning procedure between 1977 and 2004. The mean follow-up time was 19.5 ± 6.6 years. Conventional follow up and testing were performed in the same unit. An isotopic ventriculography or MRI was done for 70 patients to investigate the systemic right ventricular function. Operative mortality was 5.3%. Late mortality was 9.6%. 9 patients underwent a single reoperation and one needs heart transplantation. Actuarial survival rate was 91.5%, 91%, 89% and 88% at respectively 1, 5, 10, and 20 years. There is no statistically difference between simple and complex transposition for actuarial survival rate, maintaining permanent sinus rhythm or arrhythmias occurrence. After 20 years of 98% of patients in simple transposition group have ejection fraction > 40% versus 58% in complex transposition (p<0.001). Risk factors of ventricular dysfunction were complex transposition (p<0.001) and absence of cardioplegia (p<0.001). Last follow-up showed 91% in NYHA class 1. Imaging systemic right ventricular dysfunction (FEVD<40%) was yet uncommon at 20 years of follow-up but is not sufficient to predict a good response of this ventricle to stress and effort. Long term follow-up after the Senning operation shows frequent and increasing incidence of sinus node dysfunction and others arrhythmias: these complications are expected in double switch procedure.

Early prophylactic pulmonary artery banding in isolated congenitally corrected transposition of the great arteries☆

Mid-term evaluation of an aggressive surgical management of isolated congenitally corrected transposition of the great arteries (ccTGA) by pulmonary artery (PA) banding in early infancy. Between 2001 and 2009, 11 asymptomatic patients (seven neonates and four infants) underwent a dilatable, partially adjustable, homemade PA banding for ccTGA with intact ventricular septum. PA band circumference was correlated to body weight (22 mm+1 mm kg(-1)) and ideally adjusted to obtain flat septal geometry. Mean age at operation was 1.5±1.4 months. There was no hospital mortality. Mean ventilation time and intensive care unit (ICU) stay were 20±9 h and 2.6±1.5 days, respectively. Five patients required postoperative inotropic support. One late death occurred suddenly at 4 months; normal biventricular function and no tricuspid regurgitation were noted at last follow-up, 1 week before death. Mean follow-up was 21.5±26 months. Mean band velocity increased over time from 2.65±0.7 m s(-1) postoperatively to 3.7±0.3 at 6 months and 4.5±0.4 m s(-1) at 2 years. Tricuspid regurgitation remained stable in seven patients, decreased in three and worsened in one. Flat septal geometry was obtained in all patients after the third postoperative month. One patient underwent a double-switch procedure at 7 years due to suprasystemic morphologically left ventricular pressure. The postoperative course was uneventful. In neonates with isolated ccTGA, prophylactic PA banding is safe and carries a low morbidity. At mid-term evaluation, tricuspid valve function is stabilised or improved and systemic competence of the left ventricle is maintained, thus allowing double switch if indicated.

Results of the double switch operation for congenitally corrected transposition of the great arteries☆

Congenitally corrected TGA (CC-TGA) is characterized by discordant atrioventricular and ventriculo arterial connections. In absence of right ventricular outflow tract obstruction (RVOTO), repair by atrial and arterial switches remains a challenging procedure for which long term follow-up is uncertain. From 1995 to 2007, 20 patients (median age: 26 months) with CC-TGA had double switch procedure. Segmental anatomy was {SLL} in all patients, dextrocardia in two patients, mesocardia in two patients. Ventricular septal defect was present in 17 patients, aortic coarctation in 2 patients and interrupted aortic arch (IAoA) in 1 patient. Five patients had tricuspid valve regurgitation. Six patients had AV blocks, 4 patients had pacemaker implantation prior to repair. Pulmonary artery banding was performed in 17 patients, for congestive heart failure (14 patients) or left ventricular retraining (3 patients). Three patients, including one patient with IAoA had primary repair. After LV retraining, repair was performed when indexed LV mass to LV volume ratio was above 1.5. A median follow-up of 60 months was achieved in all. There were no deaths. Postoperative pacemaker implantation was required in four patients. Reoperation for Senning obstruction was necessary in one patient, and pacemaker battery replacement in another patient. One patient had mild neoaortic insufficiency, two had mild tricuspid regurgitation and two had mild mitral regurgitation. All were in NYHA I-II. Actuarial survival at 10 years was 100% and freedom from reoperation at 5 and 10 years were 93% and 77.4%, respectively. Double switch for CC-TGA without RVOTO can be performed with no mortality and low morbidity. Since these results seem to last for several years, it should be considered as the optimal procedure.

The morphologic left ventricle that requires training by means of pulmonary artery banding before the double-switch procedure for congenitally corrected transposition of the great arteries is at risk of late dysfunction

The morphologic left ventricle that requires training by means of pulmonary artery banding before the double-switch procedure for congenitally corrected transposition of the great arteries is at risk of late dysfunction

The morphologic left ventricle that requires training by means of pulmonary artery banding before the double-switch procedure for congenitally corrected transposition of the great arteries is at risk of late dysfunction

The aim of this study was to compare the outcome of the double-switch procedure for congenitally corrected transposition of the great arteries for patients completing morphologic left ventricle training by means of pulmonary artery banding with the outcome of patients whose morphologic left ventricle did not require training. A retrospective study of all patients undergoing the double-switch procedure from 1991 through 2004 was performed. Patients were divided into 2 groups: those not requiring morphologic left ventricle training (n = 33) and those completing morphologic left ventricle training by means of pulmonary artery banding (n = 11). The time spent with the morphologic left ventricle conditioned at systemic pressures was longer for the group not requiring morphologic left ventricle training (median, 730 days; interquartile range, 399-1234 vs median, 436 days; interquartile range, 411-646; P = .19). The overall mortality (not requiring morphologic left ventricle training, 12.1%; requiring morphologic left ventricle training, 9.1%; P = 1) and rate of death/transplantation, development of moderate-to-severe morphologic left ventricle dysfunction, or both (not requiring morphologic left ventricle training, 21.2%; requiring morphologic left ventricle training, 45.5%; P = .14) were similar between groups. Actuarial freedom from death/transplantation with good morphologic left ventricular function was superior for patients whose morphologic left ventricle did not require training (P = .04). The follow-up was not different between groups (not requiring training: median, 1435 days [interquartile range, 285-2570 days]; requiring morphologic left ventricle training: median, 568 days [interquartile range, 399-1465 days]; P = .14). On multivariate analysis, the completion of morphologic left ventricle training predicted death/transplantation, development of moderate-to-severe morphologic left ventricle dysfunction, or both (P = .02). The early results of the double-switch procedure in patients whose morphologic left ventricle required training compare favorably with those of patients whose morphologic left ventricle required no training. There is an increased risk of deterioration of morphologic left ventricle function over time in patients whose morphologic left ventricle requires training, and these patients need to be followed up regularly to detect this.

The Fate of the Retrained Left Ventricle After Double Switch Procedure for Congenitally Corrected Transposition of the Great Arteries

In congenitally corrected transposition of the great arteries (CCTGA) there is atrioventricular and ventriculoarterial discordance so that the systemic circulation is supported by the morphologic right ventricle (RV). These may require repair when there are associated cardiac anomalies such as ventricular septal defect, pulmonary stenosis, and tricuspid valve anomalies. In addressing only the associated anomalies, conventional or physiologic repair leaves the morphologic RV in the systemic circulation. Longterm follow-up by many centers has documented deteriorating right ventricular function with tricuspid regurgitation with this approach and survival at 1 year of 84%, declining to 60% at 15 years. 1 The morphologic left ventricle (LV) can be restored to the systemic circulation with an atrial, arterial switch procedure, the so called “double switch.” For this to be successful, the morphologic LV may need retraining by application of a pulmonary artery band. We were interested in the fate of this retrained LV and wondered whether it would be as robust as a LV subjected to systemic pressures from birth. Thus, we reviewed our patients with CCTGA who had undergone a double switch procedure to see if we could detect a difference in outcome in ventricular function between the trained and untrained LV.