Congenitally Corrected Transposition Of The Great Arteries Research Articles

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.

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

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. 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%). Three-dimensional-EAM-guided LVCSP preserved RV systolic function in paediatric patients with CCTGA and AVB after short-term follow-up.

Seeing into the Future: HeartMate 3 to the Systemic Right Ventricle in a Completely Blind Patient with Congenitally-corrected Transposition of the Great Arteries

A 24-year-old, blind male with congenitally-corrected transposition of the great arteries and systemic right ventricular failure presented with New York Heart Association class IV heart failure despite home inotropic therapy. He was not a transplant candidate due to psychosocial issues. He underwent a successful HeartMate 3 (Abbott Laboratories) placement with the implementation of techniques to guide home ventricular assist device care despite blindness.

Prenatal Diagnosis of Congenitally Corrected Transposition of the Great Arteries (CCTGA): A Case Report

CCTGA, also known as levo- or L-loop transposition (L-TGA), double discordance, or ventricular inversion, is a rare cardiovascular anomaly with inversion of the ventricles and great arteries. In this anomaly, the right atrium communicates with the morpho- logic left ventricle, which gives rise to the pulmonary artery, while the left atrium communicates with the morphologic right ventricle, which gives rise to the aorta. Thus, atrioventricular and ventriculoarterial discordance (double discordance) exist, and although the blood flows in the normal direction, it passes through the wrong ventricular chambers. It is a unique conotruncal anomaly, inwhich the four-chamber view is abnormal. It may be associated with other heart disorders. In most fetuses, TGA remains undiagnosed before birth. The diagnosis of TGA can be made by care- fully and appropriately evaluating the anatomic locationsof cardiac chambers and the connections between the atria, ventricles, and great arteries with high-resolution ultrasound. Patients with isolated CTGA generally present later in life with signs and symptoms related to either arrhythmias or heart failure. TGA is rarely associated with chromosomal abnormality and amniocentesis is usually not undertaken. We report a case of CTGA detected at 18 week’s gestation on screening ultrasound.

Genetics of congenitally corrected transposition of the great arteries: Whole-exome sequencing shows a mutation load effect in 156 genes involved in cardiac patterning

Congenital Heart Disease (CHD) is a major public health issue. About 20% of CHD correspond to a misalignment between cardiac segments. We focused on a rare CHD, the congenitally corrected transposition of the great arteries (CCTGA). The aim of this study was to understand the mechanisms and genes that control the alignment of cardiac chambers, and thus lead to the CCTGA. We analyzed a cohort of 44 CCTGA cases (42 sporadic cases and 2 familial cases) of non-syndromic CCTGA by next generation sequencing analysis (whole-genome and whole-exome sequencing). Under the hypothesis of Mendelian model with de novo genomic alterations, no major gene involved in the disease could be identified. Under the hypothesis of a complex model of inheritance with incomplete penetrance, a total gene set of 156 genes matched each of our selection criteria. The highly heterogeneous combinations of susceptibility rare variants, mostly inherited from the healthy mother and father respectively, functionally converge to give rise to the CCTGA phenotype. To prove that a given allele combination is associated with the CCTGA phenotype, we showed a significant enrichment of rare variants in cases, any given deleterious variant combination within the CCTGA gene set being specific to the affected individual. Taken together, the cases could be explained by a mutation load of segregated alleles at loci mostly involved in heart tube looping, outflow tract morphogenesis and establishment of left-right asymmetry. Our data contradict a monogenic mode of inheritance, favouring a multigenic origin of CCTGA, where both parents harbour genetic predisposition variants for the disease (susceptibility alleles) and both transmit this load of variants to the affected offspring, explaining the low recurrence risk.

Percutaneous Edge To Edge Repair for the treatment of severe Tricuspid valve Regurgitation in high-surgical risk patients with Systemic Right Ventricle: A Multicentric French Cohort (PETER TRESRIV)

Patients with systemic right ventricle (SRV) (mostly congenitally-corrected transposition of thegreat arteries or transposition of the great arteries corrected by atrial switch) commonly developsignificant systemic tricuspid valve regurgitation and systemic right ventricular dysfunction inadulthood, both of which being a therapeutic dilemma for the care team. Until recently, the onlytherapeutic option has been surgery but the rate of complications is high. Percutaneous edge to edgerepair could be an alternative, especially for high-surgical risk patients. To describe clinical and paraclinical follow up of high-surgical risk patients with SRV and severe TR undergoing PETER. 10 high-risk surgical patients with severe systemic tricuspid regurgitation (TR) undergoing apercutaneous repair with the Mitraclip or Triclip system (Abbott) were included between May 2019and October 2021. We collected and compared clinical (symptoms, quality of life questionnaires), biological (renal function, BNP/ntproBNP levels), VO2 max testing, echocardiographic, and crosssectionnalimaging (tricuspid regurgitation severity, right ventricle dilatation and dysfunction) dataat baseline and 6 months after the procedure. We report a percutaneous implant success of 80%, one early death due to septal leaflet tear and 2early common flutters successfully ablated. Clinically, we describe a median improvement after 6months of functional status as well as physical (+10,3 points) and mental (+12,2 points) quality oflife scores. Biologically, BNP and ntproBNP median blood levels decreased (respectively of 162and 178 μmol/L). VO2 max testing parameters did not change significantly. TTE TR severityparameters EROA and regurgitation volume improved of respectively 20 mm 2 and 11 mL while MRIor TDM SRV volumes decreased in median of 25 ml/m 2 for diastolic and 22 ml/m 2 for systolicvolume, with an increase in RVEF of 18,5%. Percutaneous edge to edge repair of systemic tricuspid regurgitation might be a safe and effectivetherapeutic option in high-risk surgical adult patients.

Double Switch in Congenitally Corrected Transposition of the Great Arteries With Incomplete Atrioventricular Septal Defect, Ebsteinoid Tricuspid Valve, Ventricular Outflow Tract Obstructions, and Multiple VSDs.

We describe the management of a 2 month-old male (BSA 0.27 m2) with congenitally corrected transposition of the great arteries (CCTGA), left and right ventricular outflow tract obstruction, incomplete atrioventricular (AV) canal defect, Ebsteinoid left AV valve, and multiple VSDs. In short, the patient presented with respiratory failure, metabolic acidosis with worsening AV valve regurgitation, and acute decompensation from pulmonary overcirculation. We performed a complex double switch operation described below. Despite the unique anatomical complexities, the patient's successful course demonstrates the importance of multidisciplinary discourse, preoperative imaging and planning, and intraoperative decision making from patient selection to anatomic repair.

Congenitally Corrected Transposition of the Great Arteries in the Adult.

IntroductionCongenitally corrected transposition of the great arteries (CCTGA) is a rare anomaly. Current data available regarding adult cases is derived from small series, information simultaneously presented in pediatric publications, and one classical multicenter study. This review, not aimed to exhaust the subject, has the purpose to examine the literature addressing presentation, diagnostic methodology, and management of afflicted adult patients.MethodsA comprehensive search was undertaken in three major databases (PubMed, Cochrane, SciELO), using the keywords “congenitally corrected transposition of the great arteries” and “adults”. Relevant articles in English, Spanish, and Portuguese were extracted and critically appraised in this review. Steps for study selection were: (1) identification of titles of records through databases searching, (2) removal of duplicates, (3) screening and selection of abstracts, (4) final inclusion in the study.ResultsFour hundred sixty-five publications on CCTGA in adult patients were retrieved, and 166 were excluded; 299 studies were used for this review including 76 full-text articles, 70 studies related to general aspects of the subject, and, due to the small number of publications, 153 case reports. Sixty-one articles referring to combined experiences in pediatric and adult patients and judged to be relevant, but retrieved from another sources, were also included.ConclusionAlbeit clinical presentation and diagnostic criteria have been well stablished, there seems to be room for discussion related to clinical and surgical management of CCTGA in adults. Considering the rarity of the disease, well designed multicenter studies may provide answers.

Peritoneal Dialysis for Refractory Heart Failure from a Congenitally Corrected Transposition of the Great Arteries who has not Undergone Definitive Surgery: A Case Report

Heart failure (HF) is a major cause of significant morbidity, mortality, and hospitalization worldwide including the Philippines. Congenitally corrected transposition of the great arteries (C-TGA) occurs when the right atrium enters the morphological left ventricle which gives rise to the pulmonary artery and the left atrium communicates with the right ventricle which gives rise to the aorta. Heart failure can occur in C-TGA especially if associated with other heart defects. Ideal management is anatomic correction via surgery to prevent or address heart failure. Peritoneal dialysis has been used as a therapeutic intervention for patients with refractory heart failure and kidney injury with or without kidney failure due to its gentler fluid removal compared to conventional ultrafiltration resulting in less myocardial stunning and neurohormonal activation. We present the case of a patient with heart failure who started on peritoneal dialysis (PD) as an adjunct therapy for fluid management after failing to satisfactorily achieve volume control with diuretics. The patient is a 56-year-old man with C-TGA admitted for decompensated heart failure. He was initially treated with intravenous diuretics on the first admission but was readmitted after 3 months for decompensation this time with borderline low blood pressure making diuresis difficult. The patient was given loop diuretics, tolvaptan, and angiotensin receptor neprilysin inhibitor (ARNI) but still with decreasing trends in urine output and inadequate symptom control. PD was initiated before discharge with subsequent improvement in heart failure symptoms. The patient was on regular follow-up for PD maintenance and titration of heart failure medication. In this case report, we have shown how PD can be an effective adjunct to guideline-directed medical therapy in patients with severely symptomatic heart failure who have an unstable hemodynamic status and for which volume management cannot be satisfactorily achieved with diuretics.

Percutaneous edge to edge systemic tricuspid valve repair for the treatment of severe tricuspid valve regurgitation in patients with systemic right ventricle: The first descriptive cohort

Patients with systemic right ventricle (mostly congenitally-corrected transposition of the great arteries or transposition of the great arteries corrected by atrial switch) commonly develop significant systemic tricuspid valve regurgitation and systemic right ventricular dysfunction in adulthood, both of which presenting a therapeutic dilemma for the care team. Percutaneous edge to edge repair could be a alternative to surgery. Seven high-risk surgical patients with severe systemic tricuspid regurgitation undergoing a percutaneous repair were included between July 2020 and March 2021. Our study is a prospective analysis of short and mid-term clinical, biological, echocardiographic and MRI outcomes with an expected minimum follow-up of 2 years. The first data tend to show a significant benefit of the repair on clinical status (dyspnea severity, quality of life, test exercise performance), a decrease of BNP level and an improvement of tricuspid regurgitation and right ventricular volume and function measured by echocardiography and MRI. Besides, the rate of failure and complications seems to be very low. Percutaneous edge to edge repair of systemic tricuspid regurgitation might be a safe and effective therapeutic option in high-risk surgical adult patients.

Case report: Congenitally corrected transposition of the great arteries (CCTGA)

Case report: Congenitally corrected transposition of the great arteries (CCTGA)

Abstract 11736: Anatomic Repair of Congenitally Corrected Transposition of the Great Arteries is Associated With Risk of Heart Block and ECMO, but Not Mortality

Introduction: Congenitally corrected transposition of the great arteries (CC-TGA) is characterized by atrioventricular and ventriculoarterial discordance with circulation remaining in series. The double switch operation and atrial switch with Rastelli are anatomic repairs of CC-TGA, achieving a systemic morphologic left ventricle. In contrast, physiologic repair (addressing associated lesions like ventricular septal defects and pulmonary stenosis) or no repair leave the right ventricle in the systemic position. Anatomic repair may confer superior longevity with increased upfront risk. We sought to determine if anatomic repair was associated with increased short-term risk of mortality, heart block, or extracorporeal membrane oxygenation (ECMO). Methods: Single-center retrospective cohort study of CC-TGA patients from 1990-2017. Cox proportional hazard analyses and Fisher’s exact test were performed. Results: Inclusion criteria were met by 54 patients, 21 anatomic repair and 33 physiologic/no repair. Median follow-up was 8.9 years (IQR 1.4-18.0) with no difference between cohorts (p=0.38). The anatomic repair cohort was more likely to have ventricular septal defects, 86% versus 33% (p<0.001) and pulmonary stenosis, 43% versus 15% (p=0.02). Mortality was low and did not differ significantly between cohorts (p=0.07). Probability of developing complete heart block was higher in the anatomic repair cohort (p=0.006), with no significant difference after adjusting for anatomy (HR 3.8, 95% CI 0.7-20.5). The anatomic repair cohort had an increased risk of ECMO (p=0.006). There was no difference in quantity of iterative operations (p=0.64). Conclusions: Anatomic repair of CC-TGA conferred a higher short-term risk of heart block and ECMO compared to the physiologic/no repair cohort, though risk of heart block was linked with anatomy. This upfront risk did not result in higher mortality in the anatomic repair cohort but long-term risk remains unknown.

Percutaneous edge to edge systemic tricuspid valve repair for the treatment of severe tricuspid valve regurgitation in patients with systemic right ventricle: The first descriptive cohort

Patients with systemic right ventricle (mostly congenitally-corrected transposition of the great arteries or transposition of the great arteries corrected by atrial switch) commonly develop significant systemic tricuspid valve regurgitation and systemic right ventricular dysfunction in adulthood, both of which presenting a therapeutic dilemma for the care team. Percutaneous edge-to-edge repair could be a alternative to surgery. Seven high-risk surgical patients with severe systemic tricuspid regurgitation undergoing a percutaneous repair were included between July 2020 and March 2021. Our study is a prospective analysis of short and mid-term clinical, biological, echocardiographic and MRI outcomes with an expected minimum follow-up of 2 years. The first data tend to show a significant benefit of the repair on clinical status (dyspnea severity, quality of life, test exercise performance), a decrease of BNP level and an improvement of tricuspid regurgitation and right ventricular volume and function measured by echocardiography and MRI. Besides, the rate of failure and complications seems to be very low. Percutaneous edge-to-edge repair of systemic tricuspid regurgitation might be a safe and effective therapeutic option in high-risk surgical adult patients.

Anaesthetic management of a case of congenitally corrected transposition of great arteries for non cardiac surgery: A case report

Congenitally corrected transposition of the great arteries (CCTGA) is a rare form of congenital heart diseases, present with or without associated anomalies. It is a complex congenital heart disease with both atrioventricular and ventriculoarterial discordance. Such patients tend to develop systemic ventricular dysfuction with the stress of surgery. Patients with CCTGA are usually diagnosed at early stages of life due to associated anomalies, but they may even remain asymptomatic till later decades of their life. Literature search revealed very few reported cases of anaesthetic management of such high risk cases for non cardiac surgery. These patients have the tendency to develop cardiac dysrhythmias and left ventricular failure during intraoperative and postoperative period. We report anaesthetic management of a case of a 24 years old male with the congenitally corrected transposition of great arteries(CCTGA) who was operated for fracture left distal radius and ulna and right sided galeazzi fracture.

The Electrocardiogram in Nonsystemic Ventricular Pacing in a Patient with Congenitally Corrected Transposition of the Great Arteries and Dextrocardia

Congenitally corrected transposition of the great arteries (CCTGA) has a high incidence of dextrocardia and complete heart block. We describe a 34 year-old male with CCTGA, dextrocardia, and third degree atrioventricular block status-post dual chamber pacemaker that presented with acute heart failure syndrome. Cardiac CT Angiography confirmed CCTGA with situs solitus and dextrocardia. EKG with standard frontal leads showed a normal P wave axis and right-sided precordial leads showed a right bundle branch block pattern. With situs solitus there is a normal P wave axis on standard frontal leads. With nonsystemic ventricular pacing in CCTGA with dextrocardia left- and right-sided precordial leads show a left bundle branch block pattern and right bundle branch block pattern respectively. This case highlights electrocardiographic patterns seen with dextrocardia and morphologic reversal of ventricles.

Left bundle branch area pacing in congenitally corrected transposition of great arteries - The obvious choice?

Patients with congenitally corrected transposition of the great arteries (CCTGA) are susceptible to acquired atrioventricular conduction disease in early life. Emerging studies propose that conduction system pacing either by His bundle pacing or bundle branch pacing is advantageous in this population. The anatomical abnormality of CCTGA conveniently positions the left bundle branches on the easily accessible right ventricular septal side. We present a case of a young female CCTGA patient with exercise-induced heart block who received left bundle branch area pacing with the ventricular lead implanted at the conventional right ventricular septal approach without the need for 3-dimensional electroanatomical mapping.

Transcatheter valve repair in congenitally corrected transposition of the great arteries.

Introduction In congenitally corrected transposition of the great arteries (CCTGA) the right ventricle (RV) is the systemic ventricle. RV dysfunction and significant regurgitation of the systemic tricuspid valve are common and associated with worse outcomes. Management of tricuspid regurgitation (TR) in CCTGA is challenging. In acquired cardiovascular disease, percutaneous repair of mitral valve regurgitation (MR) is established. Data on CCTGA with TR are limited1. We report our experience with this technique in these patients. Methods CCTGA patients with moderate-severe TR were eligible if surgery was deemed high-risk. Interventional therapy was offered, and patients provided written informed consent. ...

Congenitally Corrected Transposition of the Great Arteries: Conduction Anomalies: A Case Report

Introduction: Congenitally corrected transposition of the great arteries (CCTGA) is a rare heart disease that encompasses an atrioventricular and ventriculoarterial discordance and accounts for less than 1 percent of congenital heart diseases. Objective: To present an atypical case of a man with complex congenital heart disease and conduction anomalies. Case Presentation: This is a case of a 34-year-old patient who came to the hospital with 1 week of dyspnea on exertion and episodes of lipothymia. The patient was referred to our hospital after an electrocardiogram from his primary care with evidence of blocked atrial fibrillation. During the initial evaluation blocked atrial fibrillation was confirmed with a rescue ventricular rate of 38 bpm. A magnetic resonance confirmed the presence of the atrioventricular and ventriculoarterial discordance, the aorta had a left anterior position, perimembranous ventricular septal defect with a right to left shunt, biventricular systolic dysfunction, moderate tricuspid, and mitral regurgitation, interventricular septal intramyocardial and biatrial fibrosis, left atrial dilation, and dilation of the pulmonary artery. After pacemaker placement, the patient has an improvement in his clinical symptoms and quality of life. Conclusions: Cardiac arrhythmias are CCTGA’s leading cause of death, mostly ventricular tachycardia, and atrial fibrillation. Right bundle branch block is a previously unreported and potentially very rare presentation of this disease. This, added to the fact that our patient was diagnosed at an advanced age, but without symptoms of heart failure, makes him an atypical case of CCTGA, with new potential treatment options.

Low Dose Combined Spinal-Epidural Anesthesia: An Anesthesia Technique for Cesarean Section in a Patient Suffering from Congenitally Corrected Transposition of Great Arteries and Severe Pulmonary Stenosis

A primigravida, aged 22 years, at 39th gestational week was hospitalized with dyspnea since onset of labor. On systemic evaluation and echocardiography, she was diagnosed to have congenitally corrected transposition of the great arteries (CCTGA) along with severe pulmonic stenosis and bidirectional ventricular and atrial septal defects. A multidisciplinary team provided initial symptomatic management, stratified the risk and planned for a cesarean section. A low-dose combined sequential spinal-epidural anesthesia with invasive monitoring was used which provided excellent surgical condition with stable intraoperative and postoperative hemodynamics.

Congenital Corrected Transposition of the Great Artery (ccTGA): A Complex Cardiac Anomaly: Challenges in Management in a Resource Poor Country: A Review Article

Background: Congenitally corrected transposition of the great arteries (CCTGA) is a rare complex cardiac anomaly with a unique anatomical lesion such that despite certain anatomical aberrations there could be physiologically normal circulation without any admixture. They could also be anatomical lesions and various clinical evolutions. Surgical intervention has continued to change and updated. Methods: A search for published works on ccTGA was made using Google and Pub Med as well as searching through the Cochrane Database of Systematic Reviews, institute for Scientific Information (ISI) “Web of Science,” and Medline. The areas of focus were historical facts, patho-anatomy and patho-physiological correlates of ccTGA, management options and challenges in management in Nigeria. Results and conclusion: Congenitally corrected transposition of the great artery is a complex cardiac anomaly with a complex anatomy and presents with constellations of features ranging from heart failure, heart block to deaths. The management of ccTGA is a huge challenge in a developing world in general and Nigeria in particular. This is worsened by the harsh economic situation, lack of political will, the current COVID-19 pandemic and lack of state- of-the-art facility and human resources. The cases in Nigeria are been underreported and so are the mortality caused by this cardiac anomaly.