859 results match your criteria Congenitally Corrected Transposition


Effect of medical treatment on heart failure incidence in patients with a systemic right ventricle.

Heart 2021 May 6. Epub 2021 May 6.

Adult Congenital Heart Centre, National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK.

Background: To date, clinical trials have been underpowered to demonstrate a benefit from ACE inhibitors (ACEis) or angiotensin II receptor blockers (ARBs) in preventing systemic right ventricle (sRV) failure and disease progression in patients with transposition of the great arteries (TGA). This observational study aimed to estimate the effect of ACEi and ARB on heart failure (HF) incidence and mortality in a large population of patients with an sRV.

Methods: Data on all patients with an sRV under active follow-up at two tertiary centres between January 2007 and September 2018 were studied. Read More

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Aspergillus Endocarditis of Pulmonary Homograft in a Congenital Heart Disease Patient.

World J Pediatr Congenit Heart Surg 2021 May 6:2150135120988640. Epub 2021 May 6.

Department of Cardiac Surgery, Mitera Hospital, Athens, Greece.

Aspergillus endocarditis (AE) is a life-threatening condition with mortality rates approximating 80%. Herein, we describe the case of a 19-year-old patient with congenitally corrected transposition of great arteries, ventricular septal defect, and pulmonary atresia, who underwent seven cardiac surgical procedures in the past. The patient was operated for AE of a previously implanted pulmonary valve homograft associated with septic pulmonary embolism and right heart failure but succumbed to multi-organ failure three months later. Read More

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Pregnancy outcomes in women with a systemic right ventricle and transposition of the great arteries results from the ESC-EORP Registry of Pregnancy and Cardiac disease (ROPAC).

Heart 2021 Apr 28. Epub 2021 Apr 28.

Department of Cardiology, Erasmus Medical Center Rotterdam, Rotterdam, The Netherlands

Objective: Cardiac disease is a major cause of maternal mortality. Data regarding pregnancy outcomes in women with a systemic right ventricle (sRV) are scarce. We studied pregnancy outcomes in women with an sRV after the atrial switch procedure for transposition of the great arteries (TGA) or congenitally corrected TGA (CCTGA). Read More

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Role of Transesophageal and Epicardial Echocardiography to Assess Surgical Repair in Double-Outlet Left Ventricle.

J Cardiothorac Vasc Anesth 2021 Mar 27. Epub 2021 Mar 27.

Department Cardiothoracic and Vascular Anaesthesia, Sree Chitra Tirunal Institute for Medical Sciences & Technology, Trivandrum, India.

A double-outlet left ventricle (DOLV) is a congenital cardiac anomaly that rarely is encountered. This case report demonstrates the echocardiographic features of DOLV in the form of the pulmonary artery arising completely from the left ventricle with D-looped ventricles, especially when evaluated by intraoperative transesophageal echocardiography (TEE), along with the correlation of characteristic features with cardiac computerized tomography. The features pertinent to the differentiation of DOLV from double-outlet right ventricle and congenitally corrected transposition of the great arteries by echocardiography have been described. Read More

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Temporary pacemaker implantation through umbilical vein in a low birth weight neonate with congenital complete heart block.

Cardiol Young 2021 Apr 23:1-3. Epub 2021 Apr 23.

Department of Pediatric Cardiology, Max Superspeciality Hospital, Saket, Delhi, India.

Congenital heart block is a rare and lethal condition in paediatric population associated with maternal connective tissue disorders and rarely with structural cardiac disease like atrioventricular canal defects with or without left isomerism and congenitally corrected transposition of great arteries. Pacing in neonate if indicated is generally accomplished by epicardial pacing systems. However, in cases of significant bradycardia and haemodynamic instability, temporary pacemaker implantation via transvenous approach remains as a suitable option. Read More

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Atrial flutter after surgeries of congenitally corrected transposition of great arteries in total visceral inversion.

J Arrhythm 2021 Apr 17;37(2):445-447. Epub 2021 Jan 17.

Department of Cardiovascular Medicine Kyoto Prefectural University of Medicine Kyoto Japan.

A 51-year-old man, who had a history of open heart surgery for corrected transposition of great arteries, presented with palpitation due to atrial tachycardia. A propagation map using three-dimensional electroanatomical mapping (CARTO3) showed atrial flutter and underwent linear ablation successfully. This case highlights the difficulty of diagnosis before mapping following a complicated cardiac operation and the usefulness of three-dimensional mapping. Read More

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Congenitally Malformed Hearts: Aspects of Teaching and Research Involving Medical Students.

J Cardiovasc Dev Dis 2021 Mar 28;8(4). Epub 2021 Mar 28.

Department of Paediatric Cardiac Surgery, Birmingham Women's and Children's Hospital, Birmingham B4 6NH, UK.

To appreciate congenital heart disease fully, a detailed understanding of the anatomical presentation, as well as the physiology, is required. This is often introduced at an advanced stage of training. Professor Anderson has been influential in the Clinical Anatomy Intercalated BSc programme at the University of Birmingham, in particular in his teaching on Sequential Segmental Analysis. Read More

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Validation and Feasibility of Echocardiographic Assessment of Systemic Right Ventricular Function: Serial Correlation With MRI.

Front Cardiovasc Med 2021 16;8:644193. Epub 2021 Mar 16.

Department of Cardiology, Leiden University Medical Center, Leiden, Netherlands.

Inherent to its geometry, echocardiographic imaging of the systemic right ventricle (RV) is challenging. Therefore, echocardiographic assessment of systemic RV function may not always be feasible and/or reproducible in daily practice. Here, we aim to validate the usefulness of a comprehensive range of 32 echocardiographic measurements of systemic RV function in a longitudinal cohort by serial assessment of their correlations with cardiac magnetic resonance (CMR)-derived systemic RV ejection fraction (RVEF). Read More

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Pregnancy in a woman with congenitally corrected transposition of the great arteries.

Cleve Clin J Med 2021 04 1;88(4):228-236. Epub 2021 Apr 1.

Professor of Medicine and Pediatrics, Director of the Adult Congenital Heart Disease Center, Duke University Medical Center, Durham, NC

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

J Electrocardiol 2021 Mar 25;66:77-78. Epub 2021 Mar 25.

Department of Cardiology, Royal Melbourne Hospital, Australia.

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. Read More

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

World J Pediatr Congenit Heart Surg 2021 Mar;12(2):197-203

Cardiovascular and Thoracic Surgery, 30093Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.

Background: The optimal surgical management of patients with transposition of the great arteries (TGA), ventricular septal defect (VSD), and left ventricular outflow tract obstruction (LVOTO) is debatable. This is our initial experience with pulmonary root translocation (PRT), a technique that aims to preserve the pulmonary valve function.

Methods: From July 2012 to October 2019, 16 patients underwent anatomical repair for TGA, VSD, and LVOTO. Read More

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Defining transposition: What have we learnt?

Ann Pediatr Cardiol 2021 Jan-Mar;14(1):63-66. Epub 2020 Sep 23.

Department of Paediatric Cardiac Surgery, Birmingham Children's Hospital, Birmingham, United Kingdom.

Understanding transposition is important for all who hope to effectively treat patients with the condition. The variants of the condition are frequently debated in the literature. We describe an unusual variant of transposition, in which despite the arterial roots being supported by morphologically inappropriate ventricles, the roots themselves were normally related, with the intrapericardial arterial trunks spiraling as they extended into the mediastinum. Read More

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September 2020

Heart-lung transplant in congenitally corrected transposition of the great arteries and dextrocardia patient.

SAGE Open Med Case Rep 2021 12;9:2050313X20987449. Epub 2021 Feb 12.

Department of Cardiothoracic Surgery, Mayo Clinic, Jacksonville, FL, USA.

A 53-year-old male patient was presented to our institution with the clinical picture of biventricular failure. The echocardiogram revealed congenitally corrected transposition of the great arteries, dextrocardia with situs solitus, atrioventricular discordance and ventriculoatrial discordance, severe systemic and mitral valves regurgitation, and severe pulmonary hypertension (mean pulmonary artery pressure: 51 mm Hg). He underwent heart-lung transplant. Read More

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February 2021

Durable ventricular assist device implantation for systemic right ventricle: a case series.

Eur Heart J Case Rep 2020 Dec 18;4(6):1-9. Epub 2020 Nov 18.

Department of Cardiovascular Surgery, National Cerebral and Cardiovascular Center, 6-7 Kishibeshinmachi, Suita, Osaka 564-8565, Japan.

Background: A systemic right ventricle (RV) after atrial switch in transposition of the great arteries (TGA) or congenitally corrected TGA (ccTGA) often results in advanced heart failure in adulthood.

Case Summary: Four patients with INTERMACS Class III underwent durable ventricular assist device (VAD) implantation for a systemic RV. Two patients were diagnosed with ccTGA and underwent tricuspid valve replacement, and two were diagnosed with TGA in childhood and underwent Mustard repair. Read More

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December 2020

Multimodality Imaging of Transposition of the Great Arteries.

Radiographics 2021 Mar-Apr;41(2):338-360. Epub 2021 Jan 22.

From the Department of Radiology, UT Southwestern Medical Center, Dallas, Tex (A.C.); Department of Cardiology, University of Iowa Stead Family Children's Hospital, Iowa City, Iowa (R.A.); Department of Radiology, University of Michigan, Ann Arbor, Mich (P.P.A.); and Department of Radiology, Mayo Clinic, 200 1st St SW, Rochester, MN 55905 (C.F., P.R.).

Transposition of the great arteries (TGA) is a congenital conotruncal abnormality characterized by discordant connections between the ventricles and great arteries, with the aorta originating from the right ventricle (RV), and the pulmonary artery (PA) originating from the left ventricle (LV). The two main types of TGA are complete transposition or dextro-transposition of the great arteries (D-TGA), commonly referred to as d-loop, and congenitally corrected transposition (CCTGA), commonly referred to as l-loop or L-TGA. In D-TGA, the connections between the ventricles and atria are concordant, whereas in CCTGA they are discordant, with the left atrium connected to the RV, and the right atrium connected to the LV. Read More

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January 2021

[Asymptomatic congenitally corrected transposition of the great arteries. Report of two cases].

Rev Med Chil 2020 Jun;148(6):868-874

Departamento de Medicina Interna, Escuela de Medicina, Universidad de Valparaíso, Valparaíso, Chile.

Congenitally corrected transposition of the great arteries is a rare congenital anomaly that can remain asymptomatic until adulthood, especially when there are no other associated congenital anomalies. We report two patients in their sixth decade of life with corrected transposition of the great arteries incidentally diagnosed by transthoracic echocardiography in a preventive medical check-up. The complementary use of cardiac computed tomography confirmed the diagnosis. Read More

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Sacubitril/valsartan in the treatment of systemic right ventricular failure.

Heart 2021 Jan 15. Epub 2021 Jan 15.

CAHAL, Center for Congenital Heart Disease Amsterdam Leiden, Leiden University Medical Center, Leiden, The Netherlands

Objective: Pharmacological options for patients with a failing systemic right ventricle (RV) in the context of transposition of the great arteries (TGA) after atrial switch or congenitally corrected TGA (ccTGA) are not well defined. This study aims to investigate the feasibility and effects of sacubitril/valsartan treatment in a single-centre cohort of patients.

Methods: Data on all consecutive adult patients (n=20, mean age 46 years, 50% women) with a failing systemic RV in a biventricular circulation treated with sacubitril/valsartan in our centre are reported. Read More

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January 2021

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

J Thorac Cardiovasc Surg 2021 Mar 13;161(3):1080-1093.e4. Epub 2020 Dec 13.

Division of Pediatric Cardiac Surgery, Heart, Vascular, and Thoracic Institute, Cleveland Clinic, Cleveland, Ohio. Electronic address:

Objective: Congenitally corrected transposition of the great arteries (ccTGA) encompasses a diverse morphologic cohort, for which multiple treatment pathways exist. Understanding surgical outcomes among various pathways and their determinants are challenged by limited sample size and follow-up, and heterogeneity. We sought to investigate these questions with a large cohort of ccTGA patients presenting at different ages and representing the full therapeutic spectrum. Read More

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Transvenous pacing through the pulmonary valve in a patient with cyanotic congenital heart disease after Glenn shunt - A case report.

Indian Pacing Electrophysiol J 2021 Mar-Apr;21(2):132-136. Epub 2021 Jan 9.

Department of Cardiology, Nizam's Institute of Medical Sciences, Hyderabad, India. Electronic address:

Bradyarrhythmia requiring pacing is infrequently encountered in patients with complex cyanotic congenital heart disease. Even though epicardial pacing is the preferred mode, rarely, a need for endocardial lead implantation arises. Patients with cavopulmonary shunts limit access to the venous atria and ventricles, necessitating alternate methods of pacemaker implantation. Read More

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January 2021

Commentary: Management of congenitally corrected transposition: Different strokes for different folks.

Authors:
Joseph B Clark

J Thorac Cardiovasc Surg 2021 03 5;161(3):1094-1095. Epub 2020 Dec 5.

Division of Pediatric Cardiac Surgery, Department of Pediatrics, Penn State Children's Hospital, Hershey, Pa. Electronic address:

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Commentary: Incomplete data and inertia: Neither silences the tolling bell of corrected transposition.

J Thorac Cardiovasc Surg 2021 03 1;161(3):1095-1096. Epub 2020 Dec 1.

Division of Pediatric Cardiothoracic Surgery, Department of Surgery, Herma Heart Institute, Children's Wisconsin, Medical College of Wisconsin, Milwaukee, Wis.

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Pulmonary root translocation in corrected transposition of great arteries with ventricular septal defect and pulmonary stenosis with dextrocardia.

J Card Surg 2021 Feb 16;36(2):748-751. Epub 2020 Dec 16.

Department of Cardiovascular and Thoracic Surgery, All India Institute of Medical Sciences, New Delhi, India.

We present 13 years old with history of cyanosis and diagnosis of congenitally corrected transposition of great arteries with ventricle septal defect and pulmonary stenosis with dextrocardia. He underwent modified Senning procedure with pulmonary root translocation. Postoperative recovery was uneventful and was extubated on postoperative day 1. Read More

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February 2021

Impact of electrophysiological features acquired after anatomical repair of congenital corrected transposition of the great arteries on late mortality and ventricular dysfunction.

Eur J Cardiothorac Surg 2021 Apr;59(4):839-846

Paediatric Cardiac Surgery Center, State Key Laboratory of Cardiovascular Disease, National Center for Cardiovascular Disease, Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.

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Objectives: In patients with anatomically repaired congenitally corrected transposition of the great arteries, the impact of electrophysiological features on postoperative ventricular dysfunction remains less well known. Our goal was to investigate the role of fragmented QRS and QRS duration in mortality and systemic ventricular dysfunction after anatomical repair of corrected transposed great arteries.

Methods: Consecutive patients who underwent anatomical repair in our institution from January 2005 to December 2017 were enrolled in this retrospective analysis. Read More

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Congenitally Corrected Transposition of the Great Arteries at Age 88 Years.

CJC Open 2020 Nov 18;2(6):726-728. Epub 2020 Aug 18.

Department of Cardiology, Kurashiki Central Hospital, Kurashiki, Japan.

Most patients with congenital heart disease are diagnosed shortly after birth; however, some patients are overlooked and diagnosed in adulthood. We present the case of a man in whom congenitally corrected transposition of the great arteries (CCTGA) was first diagnosed at the age of 88 years. CCTGA is a rare disease; patients without frequently associated cardiac anomalies are considered to have long life expectancies; however, their prognosis remains unclear. Read More

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November 2020

Percutaneous pulmonary artery debanding.

J Cardiol 2021 Mar 2;77(3):307-312. Epub 2020 Dec 2.

Pediatric Heart Disease & Adult Congenital Heart Disease Center, Showa University, Tokyo, Japan.

Background: There is a paucity of data on palliative or total percutaneous pulmonary artery debanding (p-debanding), particularly with use of a stent.

Methods: Twelve p-debandings in eight patients were included in this study. Age at pulmonary artery banding (PAB) ranged from 3 days to 1 year (median, 13 days), while p-debanding was performed at 2-157 (7) months. Read More

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[Tricuspid Valve Replacement Preserving the Leaflets in a Congenitally Corrected Transposition of the Great Arteries;Report of a Case].

Kyobu Geka 2020 Dec;73(13):1109-1112

Department of Cardiovascular Surgery, Akita University, Akita, Japan.

In patients with a congenitally corrected transposition of the great arteries (ccTGA), the regurgitation of the systemic atrioventricular valve (SAVV) increases and anatomical right ventricular (ARV) dysfunction often progressively develops. A low systemic ventricular ejection fraction( SVEF) is a risk factor for mortality. However, in patients with a low ejection fraction of ARV, it is unclear how to best perform valve replacement for patients with regurgitation of SAVV. Read More

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December 2020

Mechanical circulatory support as a bridge to candidacy in adults with transposition of the great arteries and a systemic right ventricle.

Eur J Cardiothorac Surg 2020 Nov 23. Epub 2020 Nov 23.

Center for Congenital Heart Defects, Heart and Diabetes Centre, North Rhine Westphalia, Bad Oeynhausen, Germany.

Objectives: Clinical experience with continuous flow ventricular assist devices (VADs) in patients with transposition of the great arteries (TGA) including dextro-TGA and congenitally corrected TGA is rare, and indications as well as potential benefits or specific hurdles remain unclear. Therefore, our goal was to report on our experience regarding VAD therapy in adult patients with TGA as a bridge to candidacy.

Methods: We performed a single-centre retrospective study of all adult patients with TGA with systemic right ventricular failure who had continuous flow VAD implants between 2010 and 2018. Read More

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November 2020

Prenatal diagnosis, associated findings and postnatal outcome in fetuses with congenitally corrected transposition of the great arteries.

Arch Gynecol Obstet 2021 Jun 20;303(6):1469-1481. Epub 2020 Nov 20.

Department of Obstetrics and Prenatal Medicine, University of Bonn, Venusberg-Campus 1, 53127, Bonn, Germany.

Purpose: To analyze anatomic features and associated malformations in 37 prenatally detected cases of congenitally corrected transposition of the great arteries (ccTGA) and to evaluate the prenatal course, neonatal outcome and mid-term follow-up.

Methods: Retrospective analysis of prenatal ultrasound of 37 patients with ccTGA in two tertiary centers between 1999 and 2019. All fetuses received fetal echocardiography and a detailed anomaly scan. Read More

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The fate of congenitally corrected transposition of the great arteries unoperated before adulthood.

Ann Thorac Surg 2020 Nov 11. Epub 2020 Nov 11.

Division of Pediatric Cardiac Surgical Centre, Chinese Academy of Medical Sciences and Peking Union Medical College, Fuwai Hospital, Beijing, China.

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

Methods: From October 2009 to January 2018, 117 adult ccTGA patients, classified into ccTGA with intact ventricular septum, ventricular septum defect and pulmonary valve or subpulmonary outflow tract stenosis (PS) groups, were reviewed. Statistical analysis was performed with SPSS 19. Read More

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November 2020