Corrected Transposition Of Great Arteries Research Articles

Diastolic disfunction in systemic right ventricle: a cardiac magnetic resonance study on atrial function in congenital corrected transposition of great arteries patients

Abstract Background congenital corrected transposition of great arteries (ccTGA) leads to systemic position of the right ventricle (sRV). Patients with ccTGA are usually asymptomatic in the first decades of life but over time sRV will fail to support elevated systemic pressure, possibly leading to systolic heart failure (HF) in adulthood. Several factors have been associated with sRV failure and worse prognosis such as tricuspid valve dysfunction, conduction abnormalities, previous surgery due to associated lesions. However, little is known about diastolic function in sRV. We hypothesize that diastolic dysfunction characterizes sRV pathophysiology, therefore an accurate evaluation of diastolic function would allow a better assessment and risk stratification of patients. The present study aims to investigate the diastolic function in patients with ccTGA by assessing left atrial (LA) morpho-functional parameters. Methods we retrospectively examined Cardiac Magnetic Resonance (CMR) imaging of 33 ccTGA patients clinically referred to our Center between April 2014 and December 2022. ccTGA patients were matched based on sex and age with a healthy volunteer population (control group) and a group of patients with Hypertrophic Cardiomyopathy (HCM) diagnosed with HFpEF according to HF guidelines criteria. LA morpho-functional parameters were compared among the three groups: LA volumes (obtained by the length-area method), ejection fraction (EF) and, by means of feature tracking analysis, strain reservoir (εs), conduit (εe) and booster pump (εa). All patients were in sinus rhythm at the time of CMR. Results In comparison with the control group, ccTGA patients had a significant impairment in LA function (LA EF: 41,6 ± 15,1 vs 63,9 ± 7,8, p < 0,001, εs: 19,0 ± 8,3 vs 38,0 ± 10,3, p < 0,001, εe: 11,7 ± 5,9 vs 21,1 ± 8,3 p < 0,001, εa: 7,6 ± 4,1 vs 16,8 ± 6,1, p < 0,001). Conversely, no significant difference was found between ccTGA and HCM group regarding LA function (EF: 41,6 ± 15,1 vs 42,0 ± 10,1, εs: 19,0 ± 8,3 vs 22,6 ± 8,8, εe: 11,7 ± 5,9 vs 12,6 ± 7,0, εa: 7,6 ± 4,1 vs 10 ± 3,7). As for morphological parameters, ccTGA patients had an increase of LA volumes compared to both the control group and HCM patients. Conclusion ccTGA patients present an impaired LA function and a significative increase in LA volume consistent with the presence of diastolic disfunction. Further studies are needed to assess the clinical impact of morpho-functional LA impairment in ccTGA patients.

A rare form of complex cardiac anomaly

Congenital corrected Transposition Of Great Arteries (TGA) is one of the rarest complex cardiac anomalies, comprising 1% of Congenital Heart Disease (CHD) and 20% of cases of fetal TGA [1,2]. It is characterized by an atrioventricular discordance with a concurrent ventricular-arterial discordance. Its features include the aorta, which arises from the left-sided morphologic right ventricle, anterior and left of the pulmonary artery.

Comparison of blood pool and myocardial 3D printing in the diagnosis of types of congenital heart disease

The study aimed to evaluate the effectiveness of blood pool and myocardial models made by stereolithography in the diagnosis of different types of congenital heart disease (CHD). Two modeling methods were applied in the diagnosis of 8 cases, and two control groups consisting of experts and students diagnosed the cases using echocardiography with computed tomography, blood pool models, and myocardial models. The importance, suitability, and simulation degree of different models were analyzed. The average diagnostic rate before and after 3D printing was used was 88.75% and 95.9% (P = 0.001) in the expert group and 60% and 91.6% (P = 0.000) in the student group, respectively. 3D printing was considered to be more important for the diagnosis of complex CHDs (very important; average, 87.8%) than simple CHDs (very important; average, 30.8%) (P = 0.000). Myocardial models were considered most realistic regarding the structure of the heart (average, 92.5%). In cases of congenital corrected transposition of great arteries, Williams syndrome, coronary artery fistula, tetralogy of Fallot, patent ductus arteriosus, and coarctation of the aorta, blood pool models were considered more effective (average, 92.1%), while in cases of double outlet right ventricle and ventricular septal defect, myocardial models were considered optimal (average, 80%).

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

The outcomes, therapeutic strategies, and risk factors of congenital corrected transposition of great arteries (ccTGA) unoperated before adulthood are unclear. 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). 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. 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.

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

BackgroundCongenitally 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 presentationHere 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.ConclusionWe 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.

Jarvik 2000 Implantation in Anatomical Right Ventricle on Patient with Corrected Transposition of Great Arteries

The use of durable continuous-flow device for right ventricular (RV) remains limited and is evolving to date. We experienced a patient with corrected transposition of great arteries (CC-TGA) who needed ventricular assist device (VAD) for end-stage heart failure, was properly treated by Jarvik 2000 implantation in anatomical right ventricular.

Congenital corrected transposition of great arteries with situs solitus, dextrocardia and pulmonary stenosis

A 12 years female presented with dyspnea on exertion. NYHA class-II for last 3 years with reduced effort tolerance and cyanosis since child hood was reported. On examination apex in 5 th intercostal space was shifted to the right of mid clavicular line, first heart sound was single (S1), the pulmonary component of second heart sound was loud (P2). Grade 3/6 pan systolic murmur over right parasternal area was present. Chest X-ray, echocardiogram, CT cardiac angiography and cardiac catheterization affirmed situs solitus, dextrocardia, CCTGA with valvular and infundibular pulmonary stenosis, bilateral superior venacavae, large subaortic ventricular septal defect (VSD) with left sided aortic arch and normal biventricular function (Figure 1and 2).

Effectiveness of Simulator‐Based Echocardiography Training of Noncardiologists in Congenital Heart Diseases

Congenital heart diseases (CHD) are responsible for substantial morbidity and mortality in neonates. The preliminary diagnosis often is made by noncardiologists. For this reason, there is a huge demand of training in echocardiography of CHD. This is difficult to achieve due to limited resources of specialized centers. The goal of this study was to investigate the training effect of the echocardiography simulator EchoCom on trainee's ability to diagnose CHD. We enrolled 10 residents for simulator-based training in echocardiography of CHD. All participants were instructed on the simulator's basic handling and had one hour to scan the first 9 datasets information (ventricular septal defect, atrial septal defect, atrioventricular septal defect, Tetralogy of Fallot, transposition of great arteries, congenital corrected transposition of great arteries, common arterial trunk, hypoplastic left heart syndrome, normal anatomy) and establish a diagnosis. No help was given except for support regarding simulator related issues. Afterward, 2 rounds of structured simulator based echocardiography training focused on echocardiographic anatomy, spatial orientation, standard views, and echocardiographic anatomy of different CHD followed. All participants completed a standardized questionnaire containing 10 multiple-choice (MC) questions focusing on basic theoretical knowledge in echocardiographic anatomy and common CHD. Almost all of the residents invited from the affiliated children's hospital had little (20%) or no experience (80%) in echocardiography of CHD. Their Pretest and Posttest scores showed significant improvement for both, MC test and performance test, respectively. Our study showed that simulator-based training in echocardiography in CHD could be very effective and may assist with training outside the scope of CHD.

Congenital corrected transposition of great arteries with atrio-ventricular canal defect

Congenital corrected transposition of great arteries with atrio-ventricular canal defect

Atrial flutter in grown-up congenital heart (GUCH) patients: Clinical characteristics of affected population

Objective: To define occurrence, lesions and clinical characteristics of grown-up congenital heart (GUCH) patients who develop atrial flutter (AFL). Design: All GUCH patients who presented as inpatients or outpatients with documented sustained AFL between 1996 and 1998 were studied prospectively. Retrospective review of case notes for basic data relating to underlying anomaly, prior surgery and age at onset of AFL, enquiry into events before the first attack. Clinical state was assessed by Ability Index before AFL and at last visit. Setting: Designated quaternary service for GUC in a tertiary referral centre. Results: From October 1996 to April 1998, 100 consecutive patients (49 female) aged 17–77 (mean 35) years, who presented to the GUCH Unit at Royal Brompton Hospital with a sustained attack of AEL documented by a 12 lead electrocardiogram were studied. Four basic cardiac anomalies accounted for 75% patients: one ventricle (26), atrial septal defect (ASD) (19), transposition of great arteries (TGA) (17) and Tetralogy of Fallot (13). AFL occurred occasionally in small ventricular septal defect (VSD), congenital corrected TGA (CC-TGA), pulmonary stenosis and pulmonary atresia with or without VSD. 86/100 patients had undergone cardiac surgery: Fontan 19 (22%), reconstruction of right ventricular outflow tract 17 (20%), closure of ASD 15 (17%), Mustard for TGA 13 (15%), and other palliative surgery 22 (26%). AFL occurred in ‘natural history’ (unoperated) in 14 (14%) mostly in CC-TGA, ASD and Fallot. Age at first attack was 6–64 (mean 28) years with the first attack occurring at younger age after Mustard (22±7 years) and Fontan (24±7), than in un-operated ASD (46±13) and CCTGA (31±10). Haemodynamic abnormalities from anatomical causes were present in 62/74 (84%) patients who had undergone reparative surgery and included venous pathway obstruction, pulmonary regurgitation and pulmonary hypertension. Additional factors which could have precipitated AFL in prone patients were present in 63. New symptoms appeared in 96 patients with the first attack of AFL. Ability Index prior to onset in 90 patients who have been followed-up for more than 1 year since the first onset was 1 in 52, 2 in 31, 3 in 6 and 4 in 1 patients. At the last visit (mean time from the first onset 6.6±4.7 years), only 9 patients remained with Ability Index 1, 43 in 2, 20 in 3 and 18 in 4 despite return to sinus rhythm. Conclusion: One ventricle heart, ASD, transposition of great arteries and Tetrology of Fallot are the most common underlying anomalies in GUCH patients who develop AFL. It is less commonly seen in unoperated patients. When occurs AFL compromises patients’ activities and deteriorates the clinical condition. Residual or developed haemodynamic abnormalities and precipitating factor are often present in this patients, hence full investigation and close follow up are necessary once AFL develops.

Isolated Corrected Transposition of Great Arteries and Double Left Anterior Descending Artery Originating from the Left and Right Coronary Artery A Rare Combination of Coronary Artery Anomaly and Congenital Heart Disease

A case of double left anterior descending coronary artery in a patient with isolated corrected transposition of great arteries is presented. The double artery originated from the left main stem and the right coronary artery. There were no stenoses on these two arteries. This anomaly seems to be very rare.