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'Congenitally Corrected Transposition' (682)



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Feb
2018

Congenitally corrected transposition of the great arteries is a rare form of CHD. Situs inversus is a much less common variant of a congenitally corrected transposition of the great arteries. In rare cases, transposition events may be accompanied by various cardiac anomalies.

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Feb
2018

A 25-year-old female with congenitally corrected transposition of great arteries (CCTGAs), atrial septal defect, and severe pulmonary stenosis underwent lower segment cesarean section at 34 weeks of gestation using combined spinal epidural anesthesia (CSEA). We used transthoracic echocardiography (TTE) for intraoperative monitoring of the cardiovascular system because these patients are reported to have a high prevalence of myocardial perfusion defects, regional wall motion abnormalities, and impaired ventricular contractility. Scanning was done at four different time intervals; preoperatively, after initiation of CSEA, after delivery of child and postoperatively (6 and 24 h postdelivery) to detect regional wall motion and valvular abnormalities, calculate ejection fractions and optimize fluid administration.

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Jan
2018

The systemic right ventricle (SRV) is commonly encountered in congenital heart disease representing a distinctly different model in terms of its anatomic spectrum, adaptation, clinical phenotype, and variable, but overall guarded prognosis. The most common clinical scenarios where an SRV is encountered are complete transposition of the great arteries with previous atrial switch repair, congenitally corrected transposition of the great arteries, double inlet right ventricle mostly with previous Fontan palliation, and hypoplastic left heart syndrome palliated with the Norwood-Fontan protocol. The reasons for the guarded prognosis of the SRV in comparison with the systemic left ventricle are multifactorial, including distinct fibromuscular architecture, shape and function, coronary artery supply mismatch, intrinsic abnormalities of the tricuspid valve, intrinsic or acquired conduction abnormalities, and varied SRV adaptation to pressure or volume overload.

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Jan
2018

Congenitally corrected transposition of the great arteries (ccTGA) can occur in isolation, or in combination with other structural cardiac anomalies, most commonly ventricular septal defect, pulmonary stenosis and tricuspid valve disease. Clinical recognition can be challenging, so echocardiography is often the means by which definitive diagnosis is made. The tricuspid valve and right ventricle are on the systemic arterial side of the ccTGA circulation, and are therefore subject to progressive functional deterioration.

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Mar
2018

We present a case of a 57-year-old man with congenitally corrected transposition of great vessels who was found to have coronary ostial atresia with cranial flow through left superior vena cava on CT with contrast injection in coronary tributaries. As such, he was ineligible for cardiac resynchronization therapy.

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Mar
2018

The life expectancy of adults with congenital heart disease (CHD) has significantly improved in recent decades, with non-cardiovascular causes of death now competing with traditional cardiovascular causes. The risk of sudden cardiac death (SCD), a devastating event, still remains elevated above that of the general population.
We reviewed 2935 patients in our adult CHD database (age≥16years, seen at least once in our centre) and documented all cases of SCD between 2000-2015.

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Jan
2018

Congenitally corrected transposition of great arteries (CCTGA) is a rare anomaly described by atrioventricular and ventriculoarterial discordance. On the other hand, mesocardia that is extremely rare includes two relatively well-defined apexes defined by each ventricle with the major axis of the heart lies in the midline. We describe a rare case of an asymptomatic 20-year-old woman with mesocardia, CCTGA, and severe subvalvular pulmonic stenosis due to a ball-shaped accessory tissue in left ventricular outflow tract.

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Nov
2017

The double-switch operation for congenitally corrected transposition of the great arteries, in which the morphologic left ventricle is restored to the systemic circulation, is an effective surgical option. This case report describes an atrial switch technique without using supplemental material during the double-switch operation for congenitally corrected transposition of the great arteries associated with persistent left superior vena cava, with the systemic venous blood flow rerouted through the enlarged coronary sinus.

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Feb
2018

Injuries to the superior vena cava (SVC) during transvenous lead extraction (TLE) procedures are a rare but life-threatening complication. The Bridge Occlusion Balloon (BOB) is specifically designed for temporary SVC occlusion in TLE procedures. We report the first case of a 27-year-old man using the BOB as a safety net in a high-risk TLE procedure.

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Dec
1969

Congenitally corrected transposition of great vessels (CCTGV) is a rare congenital heart disease (CHD) accounting for <1% of CHDs. CCTGV with infundibular pulmonary stenosis (PS) with ventricular septal defect (VSD) is part of Fallot's physiology. It is known to be associated with bradyarrhythmias like atrioventricular (AV) blocks, and acquired complete AV block occurs at a rate of 2% per year.

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Dec
2017

We present our experience for patients who have undergone an anatomic repair (AR) for congenitally corrected transposition of the great arteries (CCTGA) at the Children's Hospital of Wisconsin. A retrospective chart review of patients who underwent AR for CCTGA from 2001 to 2015 was performed. The cohort consisted of 15 patients (74% male).

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Nov
2017

Stem cell therapy for the systemic right ventricle.

Expert Rev Cardiovasc Ther 2017 Nov 17;15(11):813-823. Epub 2017 Sep 17.
Ming-Sing Si, Richard G Ohye
In specific forms of congenital heart defects and pulmonary hypertension, the right ventricle (RV) is exposed to systemic levels of pressure overload. The RV is prone to failure in these patients because of its vulnerability to chronic pressure overload. As patients with a systemic RV reach adulthood, an emerging epidemic of RV failure has become evident.

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Dec
2017

Cardiac resynchronisation therapy (CRT) is a well-recognised treatment in systolic heart failure. There is limited evidence in congenital patients with univentricular hearts or systemic right ventricles. In 2014 PACES/HRS published a consensus statement recommending CRT if ventricular ejection fraction (EF)≤35%, QRS duration≥150ms (with RBBB in systemic RV), NYHA II-IV and ventricular dilatation.

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Oct
2017

Retrospective ascertainment of the causes of mortality in the adult congenital heart disease (ACHD) cohort of the Royal Melbourne Hospital (RMH).
Deceased patients (n=73) of the 2519 ACHD patients in the Royal Melbourne Hospital registry (commenced in 1991) were identified. Retrospective analysis was undertaken.

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Sep
2017

Transcatheter pulmonary valve replacement (TPVR) is an established therapy for dysfunctional right ventricular (RV) outflow tract conduits. TPVR in patients with congenitally corrected transposition of the great arteries, subpulmonary left ventricle, and left ventricular outflow tract (LVOT) conduit dysfunction has not been studied. Unique anatomic and physiological aspects of this population may contribute to distinct risks and outcomes.

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Jan
2017

We report the use of three-dimensional (3D) modeling to plan surgery for physiologic repair of congenitally corrected transposition of the great arteries with pulmonary atresia, dextrocardia, and complex intra cardiac anatomy. Based on measurements made from the 3D printed model of the actual patient's anatomy, we anticipated using a composite valved conduit (Dacron tube graft, decellularized bovine jugular vein, and aortic homograft) to establish left ventricle-to-pulmonary artery continuity with relief of stenosis involving the pulmonary artery confluence and bilateral branch pulmonary arteries.

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Dec
2017

In a cohort of congenitally corrected transposition of the great arteries (cc-TGA) and transposition of the great arteries after atrial switch procedure (d-TGA) the study objectives were: 1) to assess the change of quantitative systemic right ventricle (sRV) parameters over time and; 2) to examine the relationship of quantitative sRV parameters with adverse clinical outcomes.
Single-center cohort study that included 49 (39%) cc-TGA and 76 (61%) d-TGA patients >18years who had at least one MUGA sRV assessment, 18/39 had more than one respectively. The primary clinical endpoint was all-cause mortality, heart transplantation and/or heart failure hospitalization.

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Jan
2018

There is relatively sparse literature on the use of administrative datasets for research in patients with adult congenital heart disease (ACHD). The goal of this analysis is to examine the accuracy of administrative data for identifying patients with ACHD who died.
A list of the International Classification of Diseases codes representing ACHD of moderate- or great-complexity was created.

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Dec
2017

In patients with transposition of the great arteries corrected by interatrial baffle (TGA) and those with congenitally corrected transposition of the great arteries (ccTGA) the right ventricle (RV) is subjected to systemic pressure and fails prematurely. Previous studies have demonstrated RV dysfunction may be more pronounced in patients with TGA. The present study sought to compare patients with TGA and ccTGA using three-dimensional (3D) techniques to comprehensively analyze the shape, volume, global and regional function in the systemic RV.

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Oct
2017

In biventricular hearts the filling and contractility of one ventricle affects the performance of the other. In this study, we compared right ventricular systolic function and exercise capacity in patients with a subaortic right ventricle (RV) in relation to the presence of a left ventricular outflow tract obstruction (LVOTO).
Retrospective chart review of adults with congenitally corrected transposition of the great arteries (ccTGA) or with a previous atrial switch procedure for complete TGA (D-TGA).

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Jul
2017

Background Successful anatomic repair of congenitally corrected transposition of the great arteries achieves excellent outcomes. Several centers report excellent long-term survival with the Fontan pathway as well. We have selectively applied both approaches depending on individual patient morphology, with anatomic repair preferred but utilizing the Fontan pathway when high technical complexity or operative risk is anticipated.

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Aug
2017

Congenitally corrected transposition of the great arteries is a rare form of congenital heart disease. Management is controversial; options include observation, physiologic repair, and anatomic repair. Assessment of morphologic left ventricle preparedness is key in timing anatomic repair.

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Aug
2017

Three-dimensional. (3D) printing is an innovative manufacturing process that allows computer-assisted conversion of 3D imaging data into physical "printouts" Healthcare applications are currently in evolution.
The objective of this study was to explore the feasibility and impact of using patient-specific 3D-printed cardiac prototypes derived from high-resolution medical imaging data (cardiac magnetic resonance imaging/computed tomography [MRI/CT]) on surgical decision-making and preoperative planning in selected cases of complex congenital heart diseases (CHDs).

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Sep
2017

Patients with a systemic right ventricle (RV) have a compromised late outcome caused by ventricular dysfunction. Standard medical heart failure therapy has not been shown to improve RV function and survival in these patients. Phosphodiesterase (PDE)-5 inhibition increases contractility in experimental models of RV hypertrophy, but not in the normal RV.

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Nov
2017

We report the placement of a permanent transvenous nonselective His bundle pacing lead in conjunction with a transvenous pacemaker/implantable cardioverter-defibrillator in an adult with Levo-Transposition of the Great Arteries (L-TGA) and a stenotic coronary sinus (CS) ostium, which would not accommodate a transvenous left ventricular (LV) pacing lead. Nonselective His bundle pacing provided a nearly identical ventricular activation pattern in this previously unpaced patient. Many L-TGA patients will have an eventual need for permanent pacing and, given the challenges of CS cannulation, His bundle pacing may represent a preferred modality rather than pure morphologic LV pacing or surgical systemic ventricular lead placement to achieve optimal electrical synchrony.

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Sep
2017

Patients with complete and congenitally corrected transposition of the great arteries commonly survive into adulthood and present with a vast array of clinical residua.
Echocardiography remains the primary imaging modality in the routine assessment of the adult with transposition of the great arteries. It provides a comprehensive anatomic and hemodynamic evaluation.

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Dec
1969

Adults with congenital heart disease (ACHD) undergoing systemic atrioventricular valve (SAVV) surgery are a complex, understudied population. We assessed midterm outcomes and prognostic factors in ACHD undergoing SAVV surgery.
We performed retrospective evaluation of ACHD undergoing SAVV surgery from January 2005 to February 2016: 14 (33%) patients with congenital mitral valve stenosis/regurgitation, 15 (35%) with atrioventricular septal defect (AVSD), and 14 (33%) with congenitally corrected transposition of the great arteries (ccTGA) with systemic tricuspid valve regurgitation.

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Jul
2017

The surgical indication, timing, strategy, and surgical technique for anatomic correction of congenitally corrected transposition of the great arteries are challenging. We evaluated the long-term results at The Royal Children's Hospital Melbourne.
Review of 32 successive anatomic corrections between 1996 and 2015.

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Jan
2017

A 29-year-old woman was referred to our hospital due to exacerbation in dyspnea on exertion and easy fatigability. A known case of congenitally corrected transposition of the great vessels and congenital complete heart block, she had already received a permanent single-chamber pacemaker. Decision was made to implant a biventricular pacemaker for the treatment of the failing heart.

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Jan
2017

Right ventricular pacing can cause pacing-induced ventricular dysfunction (PIVD) correctable with biventricular pacing (BiVP). Factors associated with PIVD are poorly understood.
We reviewed children receiving epicardial dual-chamber pacemakers for complete heart block (CHB) after congenital heart disease (CHD) surgery.

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Mar
2017

The aims of this study were to determine the early and late outcomes of anatomic repair of congenitally corrected transposition of the great arteries (ccTGA) and to evaluate effectiveness of the hemi-Mustard procedure.
We conducted a retrospective, single-center study of patients who underwent anatomic repair for ccTGA between July 1996 and December 2013. Sixteen patients were included in the study.

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Sep
2017

A 59-year-old man who had been previously diagnosed with congenitally corrected transposition of the great arteries at the age of 35 years became a candidate for heart transplantation. At the age of 57 years, he was referred to our hospital and underwent implantation of a left ventricular assist device (EVAHEART; Sun Medical Technology Research Corp., Suwa City, Japan) because of worsening ventricular function and was listed as a heart transplant candidate.

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Dec
1969

Congenitally corrected transposition of the great arteries (CC-TGA) is a complex form of congenital heart defect with numerous anatomic subgroups. The majority of patients with CC-TGA are excellent candidates for a double-switch procedure. However, in the absence of an unrestrictive ventricular septal defect or subpulmonary stenosis, the left ventricle (LV) may undergo involution and require retraining prior to double switch.

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Jun
2017

The hemi-Mustard and bidirectional Glenn (BDG) procedures combined with the Rastelli procedure have been applied to selected cases of congenitally corrected transposition of the great arteries (ccTGA) for potential benefit over the classic atrial switch procedure. The aim of this study was to analyse our experience with the hemi-Mustard, BDG and Rastelli procedures as an anatomical correction for patients with ccTGA/left ventricular outflow tract obstruction (LVOTO) with positional heart anomalies.
In this retrospective study, 31 consecutive patients with corrected transposition underwent the hemi-Mustard/BDG procedures with the Rastelli operation between 2011 and 2015.

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Jun
2017

Congenitally corrected transposition of the great arteries is a rare form of congenital heart disease in which the persistence of the right ventricle as the systemic ventricle leads to heart failure, tricuspid valve insufficiency, and arrhythmia. Supraventricular arrhythmias are especially common in these patients. We discuss the anesthetic management of a 33-year-old patient with congenitally corrected transposition of the great arteries who required a ventricular assist device to maintain cardiac output during ablation of supraventricular tachyarrythmia.

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Aug
2017

High sensitive troponin T (hsTnT), a marker of myocardial injury, appears to be a promising diagnostic tool in patients with congenital heart disease. However, little is known about its distribution among adults with systemic right ventricle (sRV). We aimed to assess the distribution of hsTnT concentrations in patients with congenitally corrected transposition of the great arteries (ccTGA) and to evaluate its relationship with sRV function and NT-proBNP.

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Nov
2016

Congenitally corrected transposition of the great arteries (CTGA) is a rare congenital heart disease. In patients with functional CTGA with circumflex artery occlusion and mitral regurgitation (MR), the right ventricle functions as the left ventricle. Coronary artery bypass grafting with mitral valve replacement is an effective treatment for CTGA with concomitant myocardial infarction (MI) and MR.

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Dec
1969

In congenitally corrected transposition of the great arteries the right ventricle (RV) supports systemic circulation, and patients are prone to develop heart failure over time. Chronic volume overload secondary to obesity may contribute to premature dysfunction of the systemic RV.
The aim of our study was to assess the systemic RV function in overweight/obese adult patients with congenitally corrected transposition of the great arteries.

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Dec
1969

Congenitally corrected transposition of the great arteries (CCTGA) is rare form of congenital heart diseases. It may be present with or without associated anomalies. 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.

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