Ventricular Function in Physiologically Repaired and Unrepaired Congenitally Corrected Transposition of the Great Arteries.

In patients with congenitally corrected transposition of the great arteries (ccTGA) and hemodynamically significant concomitant lesions, physiologic repair may be undertaken, in which the circulation is septated but the morphologic right ventricle (RV) remains the systemic ventricle. Patients without significant concomitant lesions may be observed without surgery, with a similar physiologic result. We compared cardiovascular magnetic resonance measures of ventricular size and function in patients with physiologically repaired and unrepaired ccTGA. Patients with ccTGA who underwent cardiovascular magnetic resonance at our center between September 2007 and July 2019 were analyzed. In 38 patients identified (12, physiologically repaired; 26, unrepaired; mean age 34.5 [18.7 to 52.0] years), there was a higher proportion of RV ejection fraction ≤45% in physiologically repaired (75% vs unrepaired 35%, p = 0.02). Physiologically repaired patients had worse left ventricle global longitudinal strain (-14.9% ± 5.0% vs unrepaired patients -18.4% ± 2.7%, p = 0.04). The difference in tricuspid regurgitant fraction between groups did not achieve statistical significance (physiologically repaired 27.4 ± 11.1% vs unrepaired patients 19.2 ± 13.0%, p = 0.08). Evaluation for late gadolinium enhancement was more commonly undertaken in physiologically repaired patients (8 of 12 vs unrepaired 7 of 26, p = 0.03) and present more frequently in the left ventricle in physiologically repaired patients in patients evaluated (6 of 8 vs unrepaired 0 of 7, p = 0.01). In conclusion, ventricular function is decreased in patients with ccTGA undergoing physiologic repair compared with those without previous surgery. These cohorts should be considered separately when using ventricular function as an outcome. RV dysfunction is concerning for long-term outcomes following physiologic repair.

Conventional Repair of an Intraoperatively Diagnosed Congenitally Corrected Transposition of the Great Arteries in an Adult.

BACKGROUND: Congenitally corrected transposition of the great arteries (ccTGA) is a rare condition that accounts for just 1% of all congenital heart disease. Diagnosis of ccTGA often is missed in adulthood, despite imaging and cardiology consultation. CASE REPORT: We present the case of an intraoperatively diagnosed ccTGA with severe tricuspid valve regurgitation and secundum atrial septal defect in a 54-year-old woman, who preoperatively was diagnosed with mitral valve regurgitation in atrioventricular and ventriculoarterial concordance heart. Intraoperatively, options considered were anatomical repair with atrial-arterial double switch operation after retraining the left ventricle or a conventional repair that focused on the associated defects without addressing the discordant connections. Considering our patient's age and condition, we decided to carry on with the conventional repair to prevent further systemic right ventricle dysfunction that may lead to poor outcome and decreased survival. She was discharged one week after surgery and resumed her normal activity at 3-month follow up. CONCLUSION: Although it rarely happens, CHD such as ccTGA in an adult must always be considered. Careful examination is essential. The treatment of ccTGA in an adult is challenging, with more limited options compared with pediatric patients. However, early management could still provide favorable outcomes.

Early Experience With Pulmonary Root Translocation in Transposition of the Great Arteries With Left Ventricular Outflow Tract Obstruction.

BACKGROUND: The optimal surgical management of patients with transposition of the great arteries (TGA), ventricular septal defect (VSD), and left ventricular outflow tract obstruction (LVOTO) is debatable. This is our initial experience with pulmonary root translocation (PRT), a technique that aims to preserve the pulmonary valve function. METHODS: From July 2012 to October 2019, 16 patients underwent anatomical repair for TGA, VSD, and LVOTO. The median age was 12 months (range: 7 months to 13 years), and the median weight was 7.75 kg (range: 5.6-29.5 kg). Thirteen patients had a diagnosis of d-TGA and three had congenitally corrected transposition of the great arteries (cc-TGA). The surgical technique involved PRT from the left ventricle (LV) to the right ventricle and routing the LV to the aorta. The left ventricular outflow tract orifice resulting from the pulmonary root extraction was closed with a pericardial patch. In patients with cc-TGA, an atrial switch operation was added. A bidirectional Glenn was necessary in four patients with a long LV to aorta tunnel. One patient required a transannular patch to reconstruct the right ventricular outflow tract (RVOT). RESULTS: The median follow-up was 27 months. There was one hospital death due to residual mitral regurgitation. One patient died at home four months after hospital discharge. The remaining patients are doing well with adequate RVOT function and no valve regurgitation. CONCLUSIONS: Complete correction of TGA, VSD, and LVOTO using PRT was achieved with acceptable risk in patients with pliable and nondysplastic pulmonary valve. The translocated pulmonary root performed well in this short follow-up.

Outcomes of treatment pathways in 240 patients with congenitally corrected transposition of great arteries.

OBJECTIVE: Congenitally corrected transposition of the great arteries (ccTGA) encompasses a diverse morphologic cohort, for which multiple treatment pathways exist. Understanding surgical outcomes among various pathways and their determinants are challenged by limited sample size and follow-up, and heterogeneity. We sought to investigate these questions with a large cohort of ccTGA patients presenting at different ages and representing the full therapeutic spectrum. METHODS: Retrospective review of 240 patients diagnosed with ccTGA from Cleveland Clinic coupled with prospective cross-sectional follow-up. Forty-six patients whose definitive procedure was completed elsewhere were excluded. Time-related survival was described among treatment pathways using actuarial, time-varying covariate, and competing risks analyses. Temporal trends in longitudinal valve and ventricular function were assessed using nonlinear mixed-effects models. RESULTS: Median follow-up was 10 years. Seventy-nine patients with ccTGA underwent anatomic repair, 45 physiologic repair, 24 Fontan palliation, and 6 primary transplant. Forty patients managed expectantly had excellent long-term survival when considered from time of presentation, but benefited from failures captured following transition to physiologic repair or transplant. Morphologic right ventricular dysfunction after physiologic repair increased from 68% to 85% after 5 years, whereas morphologic left ventricular function was stable in anatomic repair, especially with early surgery. Transplant-free survival at 15 years for anatomic and physiologic repair was 80% and 71%, respectively. CONCLUSIONS: Early anatomic repair may be preferable to physiologic repair for select ccTGA patients. Late attrition after physiologic repair represents failure of expectant management and progressive tricuspid valve and morphologic right ventricular dysfunction compared with anatomic repair, where morphologic left ventricular function is relatively preserved.

Continuous-Flow Left Ventricular Assist Device Therapy in Adults with Transposition of the Great Vessels.

An increasing number of children with congenital heart disease are surviving into adulthood and subsequently developing end-stage heart failure. Two example populations are adults who have been previously operated on for congenitally corrected transposition of the great arteries (CCTGA) and transposition of the great arteries (TGA). Implantation of a continuous flow left ventricular assist device (CF-LVAD) in these patients can present unusual anatomical and physiologic challenges. In this report, we describe outcomes of CF-LVAD implantation in three such patients. These cases demonstrate the feasibility of implanting a CF-LVAD in patients who have undergone surgery for CCTGA and/or TGA.

Senning With Aortic Translocation and Anatomic Repair for Congenitally Corrected Transposition.

BACKGROUND: Anatomic repair for congenitally corrected transposition of the great arteries with ventricular septal defect (VSD) and pulmonic stenosis has been accomplished with atrial switch and Rastelli. Aortic translocation offers a direct left ventricular outflow without an extraanatomic right ventricular-to-pulmonary conduit, which may lead to decreased reoperations. We reviewed our entire experience performing Senning with aortic translocation (SAT). METHODS: From 2007 to 2017, 8 patients (mean age, 14.1 months; size, 8.86 kg) underwent SAT. Associated anomalies included situs inversus (n = 2), dextrocardia (n = 6), multiple muscular VSDs (n = 2), abnormal or straddling atrioventricular valve chords (n = 5), and branch pulmonary artery stenosis (n = 3). Four of 8 had previous systemic arterial shunts. Mean cardiopulmonary bypass was 487 minutes, and mean cardiac ischemic time was 307 minutes. Additional procedures included repair of branch pulmonary artery stenoses and closure of multiple muscular VSDs. RESULTS: There was no hospital death. One patient was supported with extracorporeal membrane oxygenation because of junctional tachycardia on postoperative day 5. One patient required pacemaker placement for first-degree heart block. Median hospital length of stay was 31 days. Mean length of follow-up was 52 months. All patients remain well with mild or no aortic regurgitation. The first patient underwent a repeat surgical operation for pulmonary venous baffle obstruction 2 years after SAT. CONCLUSIONS: Despite the technical complexity, patient outcomes have been satisfactory. We believe SAT provides a superior anatomic repair in these complex defects. Longer-term follow-up is needed regarding late intervention.

The Fate of Congenitally Corrected Transposition of the Great Arteries Unoperated Before Adulthood.

BACKGROUND: The outcomes, therapeutic strategies, and risk factors of congenital corrected transposition of great arteries (ccTGA) unoperated before adulthood are unclear. METHODS: From October 2009 to January 2018, 117 adult ccTGA patients, classified into ccTGA with intact ventricular septum, ventricular septum defect, and pulmonary valve or subpulmonary outflow tract stenosis (PS) groups, were reviewed. Statistical analysis was performed with SPSS 19.0 (IBM, Armonk, NY). RESULTS: At the first visit, no patients suffered operation. The PS group had the least systemic atrioventricular valve regurgitation and the greatest systemic ventricular ejection fraction. All 49 patients underwent surgery. From the first visit to last follow-up, systemic ventricular ejection fraction of unoperated ccTGA decreased significantly. In the intact ventricular septum group, patients receiving systemic atrioventricular valve replacement/valvuloplasty had a significantly increased systemic ventricular ejection fraction and statistically more freedom from death and transplant than unoperated. In the ventricular septum defect group the late systemic ventricular ejection fraction of operated patients was not statistically different from their basic data at first visit. In the PS group patients receiving physiologic repair had significantly decreased systemic ventricular ejection fractions. Severe systemic atrioventricular valve regurgitation, physiologic repair, and systemic ventricular dysfunction (ejection fraction <40%) were risk factors for mortality, transplant, and congestive heart failure. CONCLUSIONS: PS protects against systemic atrioventricular valve regurgitation and ventricular dysfunction. Systemic atrioventricular valve replacement/valvuloplasty improved systemic ventricular function for ccTGA with an intact ventricular septum. Physiologic repair was not ideal for ccTGA with PS. Severe systemic atrioventricular valve regurgitation and systemic ventricular dysfunction were associated with suboptimal outcomes.

Long-term outcomes of arrhythmia and distinct electrophysiological features in congenitally corrected transposition of the great arteries in an Asian cohort.

Congenitally corrected transposition of the great arteries (ccTGA) is associated with various types of arrhythmia, including supraventricular tachycardia (SVT) and complete atrioventricular block (cAVB). Our study aims to characterize the arrhythmia burden, associated risk factors, arrhythmia mechanisms, and the long-term follow-up results in patients with ccTGA in a large Asian cohort. METHODS: We enrolled 104 patients (43 women and 61 men) diagnosed with ccTGA at our institution. The mean age at last follow-up was 20.8 years. RESULTS: For 40 patients (38%) with tachyarrhythmia, paroxysmal SVT (PSVT) and atrial arrhythmia were observed in 17 (16%) and 27 (26%) patients, respectively, with 4 patients (4%) having both types of SVT. The 20-year and 30-year SVT-free survival rates were 68% and 54%, respectively. Seven patients (7%) developed cAVB: 2 (2%) developed spontaneously, and the other 5 (5%) was surgically complicated (surgical risk of cAVB: 7%, all associated with ventricular septal defect repair surgery). PSVT was mostly associated with accessory pathways (5/9) but also related to twin atrioventricular nodal reentry tachycardia (3/9) and atrioventricular nodal reentry tachycardia (1/9). Most of the accessory pathways were located at tricuspid valve (9/10). Catheter ablation successfully eliminated all PSVT substrates (10/10) and most of the atrial arrhythmia substrates (3/5), with low recurrence rate. CONCLUSIONS: The arrhythmia burden in patients with ccTGA is high and increases over time. However, cAVB incidence was relatively low and kept stationary in this Asian cohort. The mechanisms of SVT are complicated and can be controlled through catheter ablation.

Effect of medical treatment in patients with systemic right ventricle.

Objective: Congenitally corrected and surgical atrial redirected transposition of the great arteries (TGA) represents states where the morphological right ventricle serves as a systemic ventricle (S-RV). The S-RV is prone to failure, but data on medical treatment on this problem is limited. The purpose of this study was to evaluate the survival in adults with S-RV, with or without heart failure treatment. Design: The SWEDCON registry was used to collect data. All adults with S-RV and minimum follow-up of 1 year were included retrospectively. Medical treatment was defined as taking beta-blockers and/or ACE inhibitors and/or ARBs for more than 50% of the time. Results: We identified 343 patients with S-RV (median age: 21 years). Surgical atrial redirected TGA was present in 58% and congenitally corrected TGA in 42% of patients. The medically treated group (n = 126) had higher rates of impaired S-RV function, use of diuretics, pacemaker and higher NYHA functional class at baseline compared to controls. The proportion of patients with impaired functional class did not change over time in the medically treated group, but increased in controls (21% vs. 30%, p = .015). In Kaplan-Meier analysis, the mean follow-up was 10.3 years, no difference in survival was seen between the groups. Conclusions: Medical treatment may be beneficial in patients with S-RV and impaired functional class and appears to be safe in the long term. The treatment group had equal survival to controls, despite worse baseline characteristics, which might be a result of slower progression of disease in this group.

Effectiveness of Bidirectional Glenn Shunt Placement for Palliation in Complex Congenitally Corrected Transposed Great Arteries.

Surgery for complex congenitally corrected transposed great arteries is one of the greatest challenges in cardiovascular surgery. We report our experience with bidirectional Glenn shunt placement as a palliative procedure for complex congenitally corrected transposition. We retrospectively identified 50 consecutive patients who had been diagnosed with congenitally corrected transposition accompanied by left ventricular outflow tract obstruction and ventricular septal defect and who had then undergone palliative bidirectional Glenn shunt placement at our institution from January 2005 through December 2014. Patients were divided into 3 groups according to subsequent surgeries: Fontan completion (total cavopulmonary connection, 13 patients) (group 1), anatomic repair (hemi-Mustard and Rastelli procedures without Glenn takedown, 11 patients) (group 2), and prolonged palliation (no further surgery, 26 patients) (group 3). After shunt placement, no patient died or had ventricular dysfunction. Overall, mean oxygen saturation increased significantly from 79.5% ± 13.5% preoperatively to 94.1% ± 7.3% (P <0.001). The median time from shunt placement to Fontan completion and anatomic repair, respectively, was 2.1 years (range, 1.6-5.2 yr) and 1.1 years (range, 0.6-2.4 yr). Only 2 late deaths occurred, both in group 1. In group 3, time from shunt placement to latest follow-up was 4.5 years (range, 2.3-8 yr). At latest follow-up, mean oxygen saturation was 91.6% ± 10.3%, and no patients had impaired ventricular function. Bidirectional Glenn shunt placement as an optional palliative procedure for complex congenitally corrected transposition has favorable outcomes. Later, patients can feasibly be treated by Fontan completion or anatomic repair. Use of a bidirectional Glenn shunt for open-ended palliation is also acceptable.

The impact of pulmonary artery banding and cardiac resynchronization therapy for the adult patient with congenitally corrected transposition of the great arteries.

It has been controversial how to manage severe dysfunction of the systemic right ventricle and severe tricuspid regurgitation for adult patients with congenitally corrected transposition of the great arteries. We reported a 43-year-old man with these disorders, who received pulmonary artery banding and cardiac resynchronization therapy without cardiopulmonary bypass. The degree of the tricuspid regurgitation was improved to mild-moderate and systemic right ventricular ejection fraction was improved from 28 to 45% after surgery. Cardiopulmonary exercise testing showed that the predictive value of anaerobic threshold and peak oxygen uptake improved from 65 to 99% and 59 to 92%, respectively. In conclusion, pulmonary artery banding and cardiac resynchronization therapy can be the first choice of surgery for severe tricuspid regurgitation with severely impaired systemic right ventricular function. After improving systemic right ventricular dysfunction tricuspid surgery could be the next choice as a surgical intervention in the future.

Pulmonary Artery Banding for Congenitally Corrected Transposition of the Great Arteries With Hydrops Fetalis: A Case Report.

At 32 weeks of gestation, a male fetus with congenitally corrected transposition of the great arteries developed hydrops fetalis caused by a combination of mitral valve regurgitation and tricuspid valve regurgitation (TR). We performed a pulmonary artery banding (PAB) at 108 days old for gradually progressing TR, after confirming that a balloon dilatation test in the main pulmonary artery reduced TR. As the patient grew, the PAB became tighter and systolic blood pressure in the morphological left ventricle increased. At present, the patient is waiting for a double switch operation.

Double Outlet Right Ventricle With Right-Sided Aorta From the Left-Sided Morphologically Right Ventricle in the Setting of Discordant Atrioventricular Connections.

We describe the anatomic findings in a 2-year-old patient with double outlet right ventricle with right-sided aorta in the setting of usual atrial arrangement and discordant atrioventricular connections, making comparison with a specimen from the pathological archive of the Birmingham Children's Hospital in the United Kingdom having this rare combination of anatomic features. We discuss the challenges involved in diagnosis and management.

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

BACKGROUND: A 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. METHODS: This 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. RESULTS: Twenty-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. CONCLUSIONS: The 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.

In congenitally corrected transposition of great arteries following Fontan surgery, a left ventricle with suprasystemic systolic and high end-diastolic pressures paradoxically preserves right ventricular and tricuspid valve function.

Systemic right ventricular function in congenitally corrected transposition depends on septal geometry. Suprasystemic left ventricular systolic pressures and high end-diastolic pressures after Fontan surgery paradoxically preserve right ventricular function.

Postnatal Outcome Following Prenatal Diagnosis of Discordant Atrioventricular and Ventriculoarterial Connections.

Discordant atrioventricular and ventriculoarterial connection(s) (DAVVAC) are a rare group of congenital heart lesions. DAVVAC can be isolated or associated with a variety of other cardiac abnormalities. Previous studies examining the outcome of prenatally diagnosed DAVVAC have described only fetal and early postnatal outcome in small cohorts. We aimed to describe the medium-term outcome of these fetuses. Cases were identified by searching the fetal cardiac databases of two centers. Follow-up data were collected from the electronic patient records. We identified 98 fetuses with DAVVAC. 39 pregnancies were terminated and 51 resulted in a liveborn infant. Postnatal data were available for 43 patients. The median length of follow-up was 9.5 years (range 36 days to 22.7 years). The overall 5-year survival of the cohort was 80% (95% confidence interval 74-86%), no deaths were seen after this period. Associated cardiac lesions had a significant effect on both survival and surgery-free survival. Isolated DAVVAC and DAVVAC with pulmonary stenosis ± ventricular septal defect had a low mortality (89% and 100% 5-year survival, respectively). Poorer survival was seen in the group with Ebstein's anomaly of the tricuspid valve, and other complex cardiac abnormalities. Antenatal tricuspid regurgitation had a significant negative impact on postnatal survival. In conclusion, the short- and medium-term outlook for fetuses with isolated DAVVAC, and those with DAVVAC and pulmonary stenosis are good. Antenatal risk factors for postnatal mortality include Ebstein's anomaly of the tricuspid valve, especially if associated with tricuspid regurgitation, and the presence of complex associated lesions.

Congenitally corrected transposition of the great vessels: evaluation with cardiac magnetic resonance imaging after double-switch repair. / Transposición congénitamente corregida de grandes arterias: valoración por resonancia magnética cardíaca tras corrección anatómica tipo "doble switch".

Congenitally corrected transposition of the great vessels implies double discordance: atrioventricular and ventriculoarterial. We present cardiac magnetic resonance images from a 9-year-old girl with congenitally corrected transposition of the great vessels, interventricular communication, and coarctation of the aorta who was treated with pulmonary artery banding, correction of coarctation, and posterior double switch. We also review the disease and the complications that should be evaluated after the surgical intervention.