Surgical Considerations for the Mitral Valve in Congenitally Corrected Transposition.

BACKGROUND: Systemic atrioventricular valve (morphologic tricuspid valve [TV]) regurgitation has been implicated in the development of systemic ventricular failure in congenitally corrected transposition of the great arteries (ccTGA), leading to timely referral for valve replacement. However, the surgical management of subpulmonary atrioventricular valve (morphologic mitral valve [MV]) regurgitation and outcomes has not been well studied. METHODS: Of 108 ccTGA patients undergoing atrioventricular valve surgery from 1979 to 2022, 22 patients (20%) underwent MV surgery. Demographics, etiology of valve regurgitation, operative details, and outcomes of these 22 patients were retrospectively reviewed. Follow-up at 1, 5, and 10 years was available for 18 (82%), 13 (59%), and 11 patients (50%), respectively. RESULTS: Median age was 37 years (interquartile range, 29-57 years). Intrinsic MV pathology was present in 18 individuals (82%). Operations included repair in 16 patients (73%), replacement in 6 (27%), and concomitant replacement of TV in 16 (73%). There was 1 perioperative death (5%) in a patient undergoing an emergent operation for severe acute-on-chronic heart failure due to worsening TV regurgitation. During a median follow-up of 12 years (interquartile range, 2-19 years), 7 patients (32%) died. Among the 16 patients who underwent repair, recurrent moderate or greater regurgitation was seen in 15%, 29%, and 43% of repairs in patients with annular, leaflet, and lead-induced regurgitation, respectively. CONCLUSIONS: Concomitant TV and MV disease occur much less frequently than isolated TV disease in ccTGA. Intrinsic MV disease is most commonly observed but appears less amenable to successful repair compared with mitral repair in the systemic position and suggests MV replacement may be preferred in ccTGA patients.

Systemic Atrioventricular Valve Surgery in Patients With Congenitally Corrected Transposition of the Great Vessels.

BACKGROUND: Limited data exist regarding the long-term outcomes of systemic atrioventricular valve (SAVV) intervention (morphologic tricuspid valve) in congenitally corrected transposition (ccTGA). OBJECTIVES: The goal of this study was to evaluate the mid- and long-term outcomes of SAVV surgery in ccTGA. METHODS: We performed a retrospective review of 108 ccTGA patients undergoing SAVV surgery from 1979 to 2022. The primary outcome was a composite endpoint of mortality, cardiac transplantation, or ventricular assist device implantation. The secondary outcome was long-term systemic right ventricular ejection fraction (SVEF). Cox proportional hazard and linear regression models were used to analyze survival and late SVEF data. RESULTS: The median age at surgery was 39.5 years (Q1-Q3: 28.8-51.0 years), and the median preoperative SVEF was 39% (Q1-Q3: 33.2%-45.0%). Intrinsic valve abnormality was the most common mechanism of SAVV regurgitation (76.9%). There was 1 early postoperative mortality (0.9%). Postoperative complete heart block occurred in 20 patients (18.5%). The actuarial 5-, 10-, and 20-year freedom from death or transplantation was 92.4%, 79.1%, and 62.9%. The 10- and 20-year freedom from valve reoperation was 100% and 93% for mechanical prosthesis compared with 56.6% and 15.7% for bioprosthesis (P < 0.0001). Predictors of postoperative mortality were age at operation (P = 0.01) and preoperative SVEF (P = 0.04). Preoperative SVEF (P < 0.001), complex ccTGA (P = 0.02), severe SAVV regurgitation (P = 0.04), and preoperative creatinine (P = 0.003) were predictors of late postoperative SVEF. CONCLUSIONS: SAVV surgery remains a valuable option for the treatment of patients with ccTGA, with low early mortality and satisfactory long-term outcomes, particularly in those with SVEF ≥40%. Timely referral and accurate patient selection are the keys to better long-term outcomes.

Anesthesia for cesarean delivery in a patient with congenitally corrected transposition of the great arteries: A case report.

Congenitally corrected transposition of the great arteries (CCTGA) is a rare form of congenital heart disease often associated with other cardiac defects. The adaptations and physiologic changes in pregnancy can present maternal challenges and complications; multidisciplinary care allows for the safest management of pregnancy and delivery in these patients. We present a case of the anesthetic management of cesarean delivery in a woman with CCTGA with her pregnancy complicated by recurrent volume overload, pulmonary hypertension, and dysrhythmias.

Prognostic significance of subpulmonary left ventricular size and function in patients with a systemic right ventricle.

AIMS: To assess the additional prognostic significance of echocardiographic parameters of subpulmonary left ventricular (LV) size and function in patients with a systemic right ventricle (SRV). METHODS AND RESULTS: All adults with an SRV who underwent transthoracic echocardiography in 2010-18 at a large tertiary centre were identified. Biventricular size and function were assessed at the most recent examination. The study endpoint was all-cause mortality or heart/heart-lung transplantation. We included 180 patients, with 100 (55.6%) males, with a mean age of 42.4 ± 12.3 years, of whom 103 (57.2%) had undergone Mustard/Senning operations and 77 (42.8%) had congenitally corrected transposition of great arteries. Over 4.9 (3.8-5.7) years, 28 (15.6%) patients died and 4 (2.2%) underwent heart or heart-lung transplantation. Univariable predictors of the study endpoint included age, New York Heart Association functional Class III or IV, history of atrial arrhythmias, presence of a pacemaker or cardioverter defibrillator, high B-type natriuretic peptide, and echocardiographic markers of SRV and subpulmonary LV size and function. On multivariable Cox analysis of echocardiographic variables, indexed LV end-systolic diameter [ESDi; hazard ratio (HR) 2.77 (95% confidence interval, CI) 1.35-5.68, P = 0.01], LV fractional area change [FAC; HR 0.7 (95% CI 0.57-0.85), P = 0.002), SRV basal diameter [HR 1.66 (95% CI 1.21-2.29), P = 0.005], and SRV FAC [HR 0.65 (95% CI 0.49-0.87), P = 0.008] remained predictive of mortality or transplantation. On receiver-operating characteristic analysis, subpulmonary LV parameters performed better than SRV markers in predicting adverse events. CONCLUSION: SRV basal diameter, SRV FAC, LV ESDi, and LV FAC are significantly and independently associated with mortality and transplantation in adults with an SRV. Accurate echocardiographic assessment of both SRV and subpulmonary LV is, therefore, essential to inform risk stratification and management.

Hypertrophic cardiomyopathy in the systemic right ventricle in a patient with congenitally corrected transposition of the great arteries: A case report.

Congenitally corrected transposition of the great arteries is a rare clinical entity, which usually presents during adulthood with associated defects; atrioventricular block, heart failure, systemic valve failure, and arrhythmias usually complicate the clinical course. Even rarer is associated hypertrophic cardiomyopathy, which complicates the disease course and clinical decision-making. Herein, we present a patient with this condition who underwent heart transplantation, with adequate clinical resolution.

Management of congenitally corrected transposition from fetal diagnosis to adulthood.

INTRODUCTION: Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart defect characterized by atrioventricular and ventriculo-arterial discordance. CcTGA can be diagnosed at any stage of life. The natural history of the disease depends on concomitant anomalies present in most of the cases, progression of systemic ventricular dysfunction and conduction disturbances. AREAS COVERED: This review describes diagnosis of the anomaly and summarizes the current knowledge on etiology and prognosis in ccTGA patients. Furthermore, interventional and pharmacological approaches to ccTGA management are discussed. The areas requiring further research are highlighted. EXPERT OPINION: Although advances in diagnosis and treatment continue to improve outcomes for ccTGA, patients are burdened with significant morbidity and mortality. Optimal approaches to surgical management of the anomaly, as well as prevention and management of heart failure, are still not established. Future research should focus on the long-term effect of anatomic repair, potential benefits of novel pharmacological strategies for heart failure therapy, and the optimal mode of pacing in ccTGA patients. However, the issues might be difficult to address due to rarity of the disease and its heterogenous clinical presentation. As the life-expectancy of ccTGA patients improves, acquired cardiovascular disorders will become another serious concern.

Congenitally corrected transposition: not correct at all.

PURPOSE OF REVIEW: Congenitally corrected transposition of the great arteries is a rare congenital defect with several management options. Disagreement continues on strategies, such as anatomic repair, physiologic repair or observation-only. This review discusses recent data that provide further guidance for clinical decision-making. RECENT FINDINGS: New data provide greater insights into practice patterns and outcomes. Recent data from high-volume centers show progressively high rates of systemic right ventricle dysfunction over time with lower rates of systemic left ventricle dysfunction following anatomic repair; there is a statistical trend towards better survival of anatomic repair patients. Data comparing anatomic repair to observation showed that anatomic repair patients had a lower hazard of reaching a composite adverse outcome. These complex operations are predominantly performed at a small subset of congenital heart surgery centers. SUMMARY: Anatomic repair compared with physiologic repair may have better outcomes, although there are relatively high rates of morbidity for both approaches. In the patient without associated lesions, nonsurgical management can have excellent outcomes but is complicated by right ventricular failure over time. Multicenter research will help determine risk factors for bad outcomes; management at high volume, experienced centers will probably be beneficial for this complex patient population.

Congenitally corrected transposition of the great arteries and implantation of a leadless pacemaker: a case report.

BACKGROUND: Congenitally corrected transposition of the great arteries (ccTGA) is a rare cardiac anomaly and can lead to abnormal electrical activity of the heart. The implant of a pacemaker in such patients is more complicated than conventional operations. This case report of an adult with ccTGA who had a leadless pacemaker implant will provide a reference for diagnosing and treating such patients. CASE PRESENTATION: A 50-year-old male patient was admitted to hospital having experienced intermittent vision loss for a month. An electrocardiogram and Holter monitoring showed intermittent third-degree atrioventricular block, and echocardiography, cardiac computed tomography and cardiac magnetic resonance imaging confirmed a diagnosis of ccTGA. A leadless pacemaker was successfully implanted into the patient's anatomical left ventricle, and the postoperative parameters were stable. CONCLUSION: Implanting a leadless pacemaker into a patient with a rare anatomical and electrophysiological abnormality, such as ccTGA, is feasible and efficacious, but preoperative imaging evaluation is of considerable importance.

Pitfalls and Possibilities of Ventricular Assist Device Support in Congenitally Corrected Transposition of the Great Arteries in Children.

Congenitally corrected transposition of the great arteries (ccTGAs) represents a complex form of congenital heart disease that is associated with several cardiac complications. Herein is a case series of three children with ccTGA and ventricular assist device (VAD) inserted for systemic right ventricle failure at a single institution. All patients remained hemodynamically stable postimplant and were successfully discharged from the intensive care unit to undergo postoperative rehabilitation. All three patients received an orthotopic heart transplant with uneventful posttransplant courses. This case series provides insight into the medical management and technical feasibility of VAD support in children with ccTGA with end-stage heart failure.

Can an operation provide superior outcomes for corrected transposition of the great arteries with left ventricular outflow tract obstruction? A multi-institutional study.

OBJECTIVES: Our goal was to evaluate the risk-adjusted effects of operative and non-operative repair on long-term outcomes in patients with congenitally corrected transposition of the great arteries and left ventricular outflow tract obstruction (CCTGA/LVOTO). METHODS: We retrospectively reviewed 391 patients with CCTGA/LVOTO from 2001 to 2020 (operative group, 282; non-operative group, 109) in 3 centres in China. The operative group included 73 patients who underwent anatomical repair and 209 patients who underwent non-anatomical repair. The median follow-up time was 8.5 years. The inverse probability of treatment weighted-adjusted Cox regression and a Kaplan-Meier analysis were used to evaluate long-term outcomes. RESULTS: Operative repair did not reduce the hazard ratio (HR) of death, tricuspid regurgitation or New York Heart Association functional class III/IV, but a significantly increased HR was observed for pulmonary valve regurgitation [HR, 2.84; 95% confidence interval (CI), 1.10-7.33; P = 0.031]. Compared with that in the non-operative group, anatomical repair resulted in significantly increased HRs for death (HR, 2.94; 95% CI, 1.10-7.87; P = 0.032) and pulmonary valve regurgitation (HR, 9.71; 95% CI, 3.66-25.77; P < 0.001). Subgroup analysis showed that in patients with CCTGA/LVOTO with moderate or worse tricuspid regurgitation (≥ moderate), anatomical repair significantly reduced the HR of death. An inverse probability of treatment weighting-adjusted Kaplan-Meier analysis showed that the survival rates at 5 and 10 days postoperatively were 88.2 ± 4.0% and 79.0 ± 7.9%, respectively, in the anatomical repair group; these rates were significantly lower than the rates in the non-operative group (95.4 ± 2.0% and 91.8 ± 2.8%; P = 0.032). CONCLUSIONS: For patients with CCTGA/LVOTO, operative repair does not provide superior long-term outcomes, and anatomical repair results in a higher incidence of death. However, in patients with CCTGA/LVOTO with tricuspid regurgitation ≥ moderate, anatomical repair can reduce the risk of death in the long term.

Three-dimensional-mapping-guided permanent conduction system pacing in paediatric patients with congenitally corrected transposition of the great arteries.

AIMS: In congenitally corrected transposition of the great arteries (CCTGA) the right ventricle (RV) is systemic. Atrioventricular block (AVB) and systolic dysfunction are frequently observed. Permanent pacing of the subpulmonary left ventricle (LV) may worsen RV dysfunction. The aim of this study was to seek out if LV conduction system pacing (LVCSP) guided by three-dimensional-electroanatomic mapping systems (3D-EAMs) can preserve RV systolic function in paediatric CCTGA patients with AVB. METHODS AND RESULTS: Retrospective analysis of CCTGA patients who underwent 3D-EAM-guided LVCSP. Three-dimensional-pacing map guided lead implantation towards septal sites with narrower paced QRS. Electrocardiograms (ECGs), echocardiograms, and lead parameters (threshold, sensing, and impedance) were compared at baseline (pre-implantation) and at 1-year follow-up. Right ventricle function was evaluated by 3D ejection fraction (EF), fractional area change (FAC), RV global longitudinal strain (GLS). Data are reported as median (25th-75th centiles). Seven CCTGA patients aged 15 (9-17) years, with complete/advanced AVB (4 with prior epicardial pacing), underwent 3D-guided LVCSP (5 DDD, 2 VVIR). Baseline echocardiographic parameters were impaired in most patients. No acute/chronic complications occurred. Ventricular pacing was >90%. At 1-year follow-up QRS duration showed no significant changes compared with baseline; however, QRS duration shortened in comparison with prior epicardial pacing. Lead parameters remained acceptable despite ventricular threshold increased. Systemic RV function was preserved: FAC and GLS improved significantly, and all patients showed normal RV EF (>45%). CONCLUSION: Three-dimensional-EAM-guided LVCSP preserved RV systolic function in paediatric patients with CCTGA and AVB after short-term follow-up.

Safety and Efficacy of Sacubitril/Valsartan in Patients With a Failing Systemic Right Ventricle: A Prospective Single-Center Study.

BACKGROUND: Sacubitril/valsartan was demonstrated to reduce hospitalization rate and mortality in patients with heart failure with reduced ejection fraction. Data on the effects of sacubitril/valsartan in patients with a systemic right ventricle are still lacking. METHODS: Patients with transposition of the great arteries following Senning/Mustard procedure or congenitally corrected transposition of the great arteries with impaired systemic right ventricle systolic function were prospectively included. Primary end points included sacubitril/valsartan safety and efficacy. Primary efficacy end points were NT-proBNP (N-terminal pro-B-type natriuretic peptide) and systolic function improvement. Secondary end points included New York Heart Association class, 6-minute walking distance, and quality of life change. RESULTS: Fifty patients (38±12 years, 60% male, 35% congenitally corrected transposition of the great arteries) were included and followed for 1 year. No major adverse events occurred. Two (4%) patients ceased treatment due to hypotension and 1 (2%) developed a nephrotic syndrome. The target dose was reached in 20 (42%) patients. NT-proBNP values decreased significantly immediately after treatment initiation, while returned to baseline at 1 year. Echocardiography showed progressive fractional area change increase (29.2±5.8 versus 34.9±5.1%; P<0.001), and right ventricle global longitudinal strain (-13.9 [-15.1, -11.8] versus -15.3 [-17.2, -13.4]%; P<0.001) and free-wall global longitudinal strain (-14.3 [-17.3, -12.3] versus -17.2 [-19.3, -15.8]%; P<0.001) raise, whereas tricuspid regurgitation severity improved only in transposition of the great arteries patients (P=0.006). Moreover, 3-dimensional echocardiography demonstrated right ventricle volumes reduction (end-diastolic volume: 181±63 versus 156±50 mL; P=0.002; end-systolic volume: 117±48 versus 89±33 mL; P<0.001), and significantly increased systemic right ventricle ejection fraction (35.6±8.1 versus 41.5±7.5%; P<0.001). Clinical improvement was suggested by New York Heart Association class change (P<0.001), increased 6-minute walking distance (425 [333, 480] versus 500 [443, 560] m; P<0.001) as well as improved quality of life at 1-year follow-up. Beneficial effects were observed irrespective of the underlying anatomy and were more pronounced in those on target dose. CONCLUSIONS: Our data showed that sacubitril/valsartan is well tolerated and is associated with systemic right ventricle remodeling and improved systolic function as well as improved clinical status, supporting its use in this complex population.

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.

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.

Congenitally corrected transposition of the great arteries.

Congenitally corrected transposition of the great arteries (cc-TGA, also known as L-transposition) is a rare condition that represents approximately 0.5% of congenital heart disease. It is characterized by atrioventricular and ventriculoarterial discordance. Patients with cc-TGA are at risk for cardiac arrhythmias due to abnormalities of the conduction system. We present an autopsy case of a 49-year-old man with cc-TGA who died unexpectedly, likely as a result of a fatal arrhythmia. This case illustrates the cardiac pathology characteristic of cc-TGA and describes how to grossly identify inversion of the atrioventricular valves.

Congenitally corrected transposition and mitral atresia complicated by restrictive atrial septum.

Congenitally corrected transposition (ccTGA), also known as L-transposition of the great arteries (L-TGA), is a rare cardiac malformation accounting for approximately 0.05% of congenital heart disease, characterized by ventricular inversion, discordant ventriculo-arterial connections with a normal visceroatrial relationship. It was first described by Baron Rokitansky in 1875, and prenatal sonographic diagnosis can be difficult. Symptomatology and clinical presentation of this malformation are related to the associated intracardiac defects. We report a rare case of severe, complex cardiac disease: prenatally diagnosed ccTGA with atrial restriction, mitral atresia, Ebsteinoid tricuspid valve, and severe pulmonary valve stenosis, who subsequently developed left pulmonary vein stenosis.

A rare case report: tricuspid valve prolapse due to spontaneous chordae rupture in a congenitally corrected transposition of the great arteries patient.

BACKGROUND: Congenitally corrected transposition of great arteries (CCTGA) is caused by atrioventricular and ventriculoarterial discordance. Cases of CCTGA with spontaneous chordae rupture of tricuspid valve have not been reported before. CASE PRESENTATION: Here we diagnosed a 38-year-old man, who was found CCTGA 14 years ago, as spontaneous chordae rupture by real-time three dimentional transesophageal echocardiography (RT-3D-TEE). The present case is the first report to describe a CCTGA patient combine with spontaneous chordae rupture in tricuspid valve. After tricuspid valve replacement, the patient was uneventful after 6 years' follow-up. CONCLUSION: We reported a rare case with spontaneous chordae rupture of tricuspid valve in a CCTGA patient and explored its etiology here. RT-3D-TEE is an important supplement to 2-dimentional transthoracic echocardiography and can provide more accurate detections in tricuspid valve diseases in CCTGA.

Atrial tachycardia eliminated at the ventricular side in patients with congenitally corrected transposition of the great arteries: Electrophysiological findings and anatomical concerns.

BACKGROUND: The unique malformation of congenitally corrected transposition of the great arteries (cc-TGA) makes the pulmonary outflow tract (POT) a possible origin of atrial tachycardia (AT). OBJECTIVE: The purpose of this study was to investigate the mapping characteristics of ATs successfully ablated at the POT in patients with cc-TGA. METHODS: Patients with cc-TGA with AT eliminated at the POT were analyzed. Activation mapping of the atria and POT was performed under the guidance of a 3-dimensional electroanatomic mapping system. The activation pattern of these chambers was investigated, with the local activation time (LAT; using coronary sinus ostium as a reference) of the earliest activation site (EAS) being compared. RESULTS: AT eliminated at the POT was documented in 5 of 6 patients with cc-TGA. The EAS was at the right anteroseptal region with a LAT of 33 (21-120) ms in the right atrium and at the septal wall with a comparable LAT (26, 47, and 26 ms; P = .604) in the left atrium. The EAS of the POT was in the vicinity of the left-facing pulmonary sinus cusp in 3 cases and the nonfacing pulmonary sinus cusp in 2 cases, with a LAT of 106 (28-134) ms preceding both atria. Ablation at this site successfully eliminated AT in all 5 cases. CONCLUSION: AT arising adjacent to the POT is not an uncommon tachycardia in patients with situs solitus-type cc-TGA and can be safely eliminated by ablation targeting the EAS in the POT.