791 results match your criteria Congenitally Corrected Transposition


Adverse impact of univentricular pacing for the patient with functional single ventricle: successful conversion to cardiac resynchronization therapy.

Surg Case Rep 2020 May 13;6(1):101. Epub 2020 May 13.

Division of Thoracic and Cardiovascular Surgery, Niigata University Graduate School of Medical and Dental Sciences, 1-757, Asahimachi-dori, Niigata City, 951-8510, Japan.

Background: In a Fontan candidate, univentricular pacing may cause delay in interventricular conduction, which induces asynchronous contraction. Cardiac resynchronization therapy is expected to be an effective mode of therapy in such a case.

Case Presentation: A 7-month-old girl, diagnosed with dextrocardia, congenitally corrected transposition of the great artery [situs solitus, L-loop, and L-transposition], ventricular septal defect, infundibular and pulmonary valvular stenosis, and straddling of the tricuspid valve, was considered as a candidate for the Fontan procedure. Read More

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http://dx.doi.org/10.1186/s40792-020-00863-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7221050PMC

Monitored Anesthesia Care of Two Patients with Highly Elevated Subpulmonic Ventricular Pressure due to Adult Congenital Heart Disease.

Case Rep Cardiol 2020 11;2020:2040561. Epub 2020 Jan 11.

Department of Anesthesiology, Yokohama City University Medical Center, 4-57 Urahune, Minami-ku, Yokohama 2320024, Japan.

Procedural sedation and analgesia for patients with adult congenital heart disease (ACHD) and highly elevated subpulmonic ventricular pressure require proper anesthesia care to prevent a pulmonary hypertensive crisis. We describe the monitored anesthesia care (MAC) of two patients with ACHD (a complete atrioventricular septal defect and congenitally corrected transposition of the great arteries) and highly elevated subpulmonic ventricular pressure. In both patients, preprocedural transthoracic echocardiography was useful for detecting severely elevated subpulmonic ventricular pressure. Read More

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http://dx.doi.org/10.1155/2020/2040561DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7201478PMC
January 2020

Incidental congenitally corrected transposition of the great arteries (ccTGA) in an adult with suspected coronary artery disease: review on radiological features and pathophysiology.

BMJ Case Rep 2020 Apr 22;13(4). Epub 2020 Apr 22.

Department of Radiology, Hospital Universiti Sains Malaysia, Kubang Kerian, Kelantan, Malaysia.

We report a case of a 46-year-old woman who has presented to a peripheral hospital with progressive exertional dyspnoea and chest discomfort. The resting ECG showed features of left-sided ventricular hypertrophy. The initial chest radiograph was reported as cardiomegaly. Read More

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http://dx.doi.org/10.1136/bcr-2019-234225DOI Listing

HeartMate 3 in a ccTGA patient.

World J Pediatr Congenit Heart Surg 2020 May;11(3):368-369

Cardiovascular Medicine, Vanderbilt University Medical Center, Nashville, TN, USA.

A 49-year-old female with congenitally corrected (or levo-) transposition of the great arteries complicated by nonischemic cardiomyopathy presented for worsening heart failure despite guideline-directed medical therapy and was found to be in cardiogenic shock. She successfully underwent ventricular assist device placement with a HeartMate III to her systemic right ventricle as a bridge to transplantation. Read More

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http://dx.doi.org/10.1177/2150135119897901DOI Listing

Effect of medical treatment in patients with systemic right ventricle.

Scand Cardiovasc J 2020 Apr 10:1-6. Epub 2020 Apr 10.

Department of Molecular and Clinical Medicine, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg and Sahlgrenska University Hospital, Goteborg, Sweden.

Congenitally corrected and surgical atrial redirected transposition of the great arteries (TGA) represents states where the morphological right ventricle serves as a systemic ventricle (S-RV). The S-RV is prone to failure, but data on medical treatment on this problem is limited. The purpose of this study was to evaluate the survival in adults with S-RV, with or without heart failure treatment. Read More

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http://dx.doi.org/10.1080/14017431.2020.1751266DOI Listing

Congenitally corrected transposition of the great arteries.

J Thorac Dis 2020 Mar;12(3):1213-1218

Department of Congenital Cardiothoracic Surgery, NYU Langone Health, New York, NY, USA.

Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital heart lesion with varied morphological presentation and can often by asymptomatic. A failing systemic right ventricle (RV) or increasing tricuspid regurgitation are generally indications for surgical intervention. The surgical approach depends upon the age of the patient and morphology of the lesion. Read More

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http://dx.doi.org/10.21037/jtd.2019.10.15DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7139005PMC

Implantable cardiac electronic device therapy for patients with a systemic right ventricle.

Heart 2020 Apr 8. Epub 2020 Apr 8.

Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, United Kingdom

The systemic right ventricle (SRV), defined as the morphological right ventricle supporting the systemic circulation, is relatively common in congenital heart disease (CHD). Our review aimed at examining the current evidence, knowledge gaps and technical considerations regarding implantable cardiac electronic device therapy in patients with SRV. The risk of sinus node dysfunction (SND) after atrial switch repair and/or complete heart block in congenitally corrected transposition of great arteries requiring permanent pacing increases with age. Read More

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http://dx.doi.org/10.1136/heartjnl-2019-316202DOI Listing

Permanent Conduction System Pacing for Congenitally Corrected Transposition of the Great Arteries.

Heart Rhythm 2020 Feb 10. Epub 2020 Feb 10.

University of Chicago Medicine, Chicago, IL.

Background: Congenitally corrected transposition of the great arteries (CCTGA) is associated with spontaneous AV block and pacing-induced cardiomyopathy. His bundle pacing (HBP) is a potential alternative to conventional CRT.

Objectives: To determine the outcomes of HBP for CCTGA. Read More

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http://dx.doi.org/10.1016/j.hrthm.2020.01.033DOI Listing
February 2020
5.076 Impact Factor

Permanent conduction system pacing for congenitally corrected transposition of the great arteries: A Pediatric and Congenital Electrophysiology Society (PACES)/International Society for Adult Congenital Heart Disease (ISACHD) Collaborative Study.

Heart Rhythm 2020 Mar 13. Epub 2020 Mar 13.

University of Chicago Medicine, Chicago, Illinois.

Background: Congenitally corrected transposition of the great arteries (CCTGA) is associated with spontaneous atrioventricular block and pacing-induced cardiomyopathy. Conduction system pacing is a potential alternative to conventional cardiac resynchronization therapy (CRT).

Objective: The purpose of this study was to determine the outcomes of conduction system pacing for CCTGA. Read More

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http://dx.doi.org/10.1016/j.hrthm.2020.01.033DOI Listing
March 2020
5.076 Impact Factor

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

Heart Rhythm 2020 Mar 20. Epub 2020 Mar 20.

Department of Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing, China.

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. Read More

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http://dx.doi.org/10.1016/j.hrthm.2020.03.010DOI Listing

Urgent double switch operation in a patient with congenitally corrected transposition of great arteries and an untrained systemic ventricle.

Turk Gogus Kalp Damar Cerrahisi Derg 2020 Jan 23;28(1):197-200. Epub 2020 Jan 23.

Department of Cardiovascular Surgery, Medicana International Istanbul Hospital, Istanbul, Turkey.

Congenitally corrected transposition of the great arteries is a rare cardiac abnormality. A nine-month-old male infant with the diagnosis of congenitally corrected transposition of the great arteries was admitted to our clinic with a history of pulmonary artery banding to train the left ventricle three months ago. On admission, he presented with bronchopneumonia, cyanosis, dyspnea, and severe biventricular heart failure associated with significant valvular regurgitation. Read More

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http://dx.doi.org/10.5606/tgkdc.dergisi.2020.18109DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7067021PMC
January 2020

IgA Vasculitis Triggered by Infective Endocarditis of Pulmonary Artery with Congenitally Corrected Transposition of the Great Arteries.

Int Heart J 2020 Mar 14;61(2):404-408. Epub 2020 Mar 14.

Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences.

A man in his 40s with a history of congenitally corrected transposition of the great arteries (CCTGA) and closure of ventricular septal defect was referred to our hospital with purpura and hematuria. Presence of purpura, renal damage, and pathological findings on skin biopsy led to the diagnosis of IgA vasculitis (IgAV). Oral prednisolone (PSL) was initiated. Read More

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http://dx.doi.org/10.1536/ihj.19-446DOI Listing

Effectiveness of Bidirectional Glenn Shunt Placement for Palliation in Complex Congenitally Corrected Transposed Great Arteries.

Tex Heart Inst J 2020 Feb 1;47(1):15-22. Epub 2020 Feb 1.

Surgery for complex congenitally corrected transposed great arteries is one of the greatest challenges in cardiovascular surgery. We report our experience with bidirectional Glenn shunt placement as a palliative procedure for complex congenitally corrected transposition. We retrospectively identified 50 consecutive patients who had been diagnosed with congenitally corrected transposition accompanied by left ventricular outflow tract obstruction and ventricular septal defect and who had then undergone palliative bidirectional Glenn shunt placement at our institution from January 2005 through December 2014. Read More

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http://dx.doi.org/10.14503/THIJ-17-6555DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7046347PMC
February 2020

Ventricular fibrillation in congenitally corrected transposition of great arteries treated with pacing: a case report.

Eur Heart J Case Rep 2019 Dec 30;3(4):1-6. Epub 2019 Oct 30.

Department of Cardiology, Huaihe Hospital, Henan University College of Medicine, Kaifeng, Henan 475000, China.

Background: Congenitally corrected transposition of the great arteries (CCTGA) is a rare form of congenital heart disease which may present with sudden death from malignant arrhythmias including complete heart block and ventricular tachyarrhythmias as late complications. Only few cases about ventricular tachyarrhythmias, usually in those with markedly depressed systemic ventricular function, have been reported.

Case Summary: A 26-year-old woman with a known history of CCTGA presented to the emergency department with palpitations and breathlessness for 3-4 weeks and worsening symptoms for 8 h. Read More

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http://dx.doi.org/10.1093/ehjcr/ytz199DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7042131PMC
December 2019

Late Presentation of a Congenitally Corrected Transposition of Great Arteries and Hemodynamically Balanced Ventricles Associated with a Large Ventricular Septal Defect and Severe Pulmonary Stenosis: A Case Report and a Multi-Imaging Approach.

Am J Case Rep 2020 Feb 29;21:e920822. Epub 2020 Feb 29.

Cardiovascular Department, Rangueil Hospital, Toulouse, France.

BACKGROUND Congenitally corrected transposition of great arteries (ccTGA) represents a distinct rare group of congenital heart diseases. Survival of unoperated ccTGA in the presence of large ventricular septal defect (VSD) is exceptional. Furthermore, late presentation of such patients in the absence of severe pulmonary hypertension or severe systemic right ventricle dysfunction is unusual. Read More

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http://dx.doi.org/10.12659/AJCR.920822DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7064142PMC
February 2020

Congenitally corrected transposition of the great arteries, coronary anomaly and transplant.

Rev Esp Cardiol (Engl Ed) 2020 Feb 24. Epub 2020 Feb 24.

Departamento de Radiología, Servicio de Tomografía Cardiaca, Instituto Nacional de Cardiología Ignacio Chávez, Ciudad de México, Mexico.

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http://dx.doi.org/10.1016/j.rec.2019.11.012DOI Listing
February 2020

Exome-Based Case-Control Analysis Highlights the Pathogenic Role of Ciliary Genes in Transposition of the Great Arteries.

Circ Res 2020 Mar 11;126(7):811-821. Epub 2020 Feb 11.

From the State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China (Xuanyu Liu, W.C., W.L., Y.F., B.M., B.H., Xuewen Liu, S.H., Z.Z.).

Rationale: Transposition of the great arteries (TGA) is one of the most severe types of congenital heart diseases. Understanding the clinical characteristics and pathogenesis of TGA is, therefore, urgently needed for patient management of this severe disease. However, the clinical characteristics and genetic cause underlying TGA remain largely unexplored. Read More

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http://dx.doi.org/10.1161/CIRCRESAHA.119.315821DOI Listing

Serial changes of tricuspid regurgitation after anatomic repair for congenitally corrected transposition.

Eur J Cardiothorac Surg 2020 Feb 12. Epub 2020 Feb 12.

Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan.

Objectives: The aim of this study was to reveal the serial changes in tricuspid regurgitation (TR) after anatomic repair for congenitally corrected transposition of great arteries.

Methods: Between 1995 and 2018, 48 patients underwent anatomic repair (atrial/arterial switch in 14 patients, atrial switch and Rastelli in 34 patients). The mean age and weight of the patients during anatomic repair was 33 (interquartile range 21. Read More

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http://dx.doi.org/10.1093/ejcts/ezaa022DOI Listing
February 2020

The Cardiac Genome Clinic: implementing genome sequencing in pediatric heart disease.

Genet Med 2020 Feb 10. Epub 2020 Feb 10.

Ted Rogers Centre for Heart Research, Cardiac Genome Clinic, The Hospital for Sick Children, Toronto, ON, Canada.

Purpose: This study investigated the diagnostic utility of nontargeted genomic testing in patients with pediatric heart disease.

Methods: We analyzed genome sequencing data of 111 families with cardiac lesions for rare, disease-associated variation.

Results: In 14 families (12. Read More

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http://dx.doi.org/10.1038/s41436-020-0757-xDOI Listing
February 2020
7.329 Impact Factor

Prenatal diagnosis of congenitally corrected transposition of the great arteries.

J Ultrason 2019 12 31;19(79):314-317. Epub 2019 Dec 31.

Department of Obstetrics, Paulista School of Medicine - Federal University of São Paulo (EPM-UNIFESP), São Paulo-SP, Brazil.

Congenitally corrected transposition of the great arteries is a rare and complex congenital heart disease. It essentially occurs with atrioventricular and ventriculoarterial discordance, in which the great vessels become parallel. Cases of corrected transposition of the great arteries are more frequently associated with other cardiac anomalies. Read More

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http://dx.doi.org/10.15557/JoU.2019.0048DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6988459PMC
December 2019

Global longitudinal strain correlates to systemic right ventricular function.

Cardiovasc Ultrasound 2020 Jan 27;18(1). Epub 2020 Jan 27.

Department of Cardiology, Skåne University Hospital, Lund University, SE-221 85, Lund, Sweden.

Background: The aim of this retrospective study was to evaluate the relationship between right ventricular function derived from cardiac magnetic resonance imaging (CMR), echocardiography and exercise stress test performance, NT-proBNP (N-terminal proB-type natriuretic peptide) level and NYHA class in patients with a systemic right ventricle.

Methods: All patients with congenitally corrected transposition of the great arteries (ccTGA), or transposition of the great arteries after Mustard or Senning procedures, (TGA) followed at our centre who had undergone CMR, echocardiography, an exercise stress test and blood sampling, were included in the study.

Results: We examined 11 patients (six after the Senning procedure, one after the Mustard procedure, and four ccTGA) who have a median age of 32 years (22-67 years). Read More

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http://dx.doi.org/10.1186/s12947-020-0186-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6986040PMC
January 2020

Assessment and management of heart failure in the systemic right ventricle.

Heart Fail Rev 2020 Jan 22. Epub 2020 Jan 22.

Department of Cardiology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.

Transposition of the great arteries (TGA) is a common congenital cardiac malformation, in which many patients survive into adulthood. From the 1960s through much of the 1980s, the majority of those with D-TGA were repaired with an atrial switch procedure, in which the right ventricle (RV) remains the systemic ventricle. Congenitally corrected TGA patients often remain with a systemic RV. Read More

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http://dx.doi.org/10.1007/s10741-020-09914-2DOI Listing
January 2020

Systematic Evaluation of Systemic Right Ventricular Function.

J Clin Med 2019 Dec 31;9(1). Epub 2019 Dec 31.

Department of Internal Medicine II, cardiology, Medical University of Vienna, 1090 Vienna, Austria.

Background: The right ventricle serves as the subaortic systemic ventricle (sysRV) in patients with congenitally corrected transposition of the great arteries (ccTGA) and in patients with transposition of the great arteries (TGA) surgically repaired by an atrial switch. SysRV can lead to late complications, primarily heart failure, significant regurgitation of the systemic atrioventricular (AV) valve, and ventricular arrhythmias with sudden cardiac death. We sought to investigate the value of 2D- and 3D-echocardiographic parameters of sysRV function. Read More

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http://dx.doi.org/10.3390/jcm9010107DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7019841PMC
December 2019

Mechanisms for heart failure in systemic right ventricle.

Heart Fail Rev 2019 Dec 19. Epub 2019 Dec 19.

Royal Melbourne Hospital, Melbourne, VIC, Australia.

There are two predominant scenarios where the right ventricle acts as the systemic ventricle, namely congenitally corrected transposition of the great arteries (CC-TGA) and patients with D-transposition of the great arteries (D-TGA) who have undergone an atrial switch operation. There is evidence that having a right ventricle in the systemic position predisposes to the development of heart failure. In this review, we aim to explore the proposed mechanisms and pathophysiology for heart failure in patients with a systemic right ventricle in these two subsets of patients. Read More

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http://dx.doi.org/10.1007/s10741-019-09902-1DOI Listing
December 2019

A Case of Transposition of Great Arteries with Ventricular Septal Defect and Mesocardia Presenting for Neurosurgery.

J Neurosci Rural Pract 2019 Oct 11;10(4):715-717. Epub 2019 Dec 11.

Division of Neuroanesthesia, Department of Anaesthesiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India.

Congenitally corrected transposition of great arteries (CC-TGAs) associated with a ventricular septal defect (VSD) presents with cyanotic spells and systemic complications such as brain abscess. In mesocardia, the heart lies in the midline with no apex seen on the left side. We report the anesthetic management of a child with CC-TGA, VSD, and mesocardia presenting with parietal brain abscess for neurosurgery. Read More

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http://dx.doi.org/10.1055/s-0039-3399601DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6906105PMC
October 2019

A unique indication for the senning procedure: Atrioventricular discordance with ventriculoarterial concordance.

J Card Surg 2020 Feb 3;35(2):444-446. Epub 2019 Dec 3.

Division of Cardiothoracic Surgery, American Family Children's Hospital, University of Wisconsin, Madison, Wisconsin.

Objective: We describe experience treating a patient with atrioventricular (AV) discordance with ventriculoarterial (VA) concordance, ventricular septal defect (VSD), and situs inversus. This is a rare congenital lesion in which closing the VSD would septate D-transposition physiology and performing an arterial switch and VSD closure would produce congenitally corrected transposition of the great arteries physiology. As such, it is the only lesion for which an isolated atrial switch (and VSD closure) remains the preferred correction. Read More

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http://dx.doi.org/10.1111/jocs.14342DOI Listing
February 2020

Insights from examination of hearts from adults dying suddenly to the understanding of congenital cardiac malformations.

Clin Anat 2020 Apr 9;33(3):394-404. Epub 2019 Dec 9.

Cardiology Clinical Academic Group, Department of Cardiovascular Pathology, Molecular and Clinical Sciences Research Institute, St George's University of London, London, United Kingdom.

Congenital heart disease is a rare but important finding in adults who experience sudden death. Examination of the congenitally malformed heart has historically been considered esoteric and best left to those with expertise. The Cardiac Risk in the Young cardiovascular pathology laboratory based at St George's University of London has now received over 6,000 cases. Read More

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http://dx.doi.org/10.1002/ca.23531DOI Listing

EDUCATIONAL SERIES IN CONGENITAL HEART DISEASE: Echocardiographic assessment of transposition of the great arteries and congenitally corrected transposition of the great arteries

Echo Res Pract 2019 Dec 1;6(4):R107-R119. Epub 2019 Dec 1.

Division of Cardiology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.

Echocardiographic assessment of patients with transposition of the great arteries and congenitally corrected transposition requires awareness of the morphology and commonly associated lesions. The pre-operative echocardiography should include a full segmental and sequential analysis. Post-operative assessment is not possible without awareness of the type of surgical procedure performed and consists of assessing surgical connections and residual lesions. Read More

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http://dx.doi.org/10.1530/ERP-19-0047DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6865365PMC
December 2019

Concordant location of accessory pathways and tricuspid valve in AV discordance.

J Cardiovasc Electrophysiol 2020 Jan 27;31(1):100-102. Epub 2019 Nov 27.

The Electrophysiology Program, Penn State University Heart and Vascular Institute, Penn State Health Children's Hospital, Milton S. Hershey Medical Center, Penn State University College of Medicine, Hershey, Pennsylvania.

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http://dx.doi.org/10.1111/jce.14274DOI Listing
January 2020

In congenitally corrected transposition of great arteries following Fontan surgery, a left ventricle with suprasystemic systolic and high end-diastolic pressures paradoxically preserves right ventricular and tricuspid valve function.

Cardiol Young 2019 Dec 31;29(12):1522-1523. Epub 2019 Oct 31.

Department of Pediatric Cardiology, Madras Medical Mission, Chennai, India.

Systemic right ventricular function in congenitally corrected transposition depends on septal geometry. Suprasystemic left ventricular systolic pressures and high end-diastolic pressures after Fontan surgery paradoxically preserve right ventricular function. Read More

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http://dx.doi.org/10.1017/S1047951119002324DOI Listing
December 2019

A case of 15-year misdiagnosed heart failure: congenitally corrected transposition of the great arteries.

Eur Heart J Case Rep 2019 Sep 16;3(3):ytz153. Epub 2019 Sep 16.

Department of Cardiology, Istanbul University, Istanbul Faculty of Medicine, Turgut Ozal Street, No. 118, Fatih, Istanbul, Turkey.

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http://dx.doi.org/10.1093/ehjcr/ytz153DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6764573PMC
September 2019

Congenitally corrected transposition of the great arteries: is it really a transposition? An anatomical study of the right ventricular septal surface.

J Anat 2020 Feb 27;236(2):325-333. Epub 2019 Oct 27.

Laboratory of Anatomy of Congenital Heart Disease - M3C, Hôpital Marie Lannelongue, Le Plessis-Robinson, France.

Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital malformation which associates discordant atrioventricular and ventriculo-arterial connections. Although frequently associated with a ventricular septal defect (VSD), its anatomy remains controversial. This could be due in hearts with usual atrial arrangement to the apparently different anatomy of the left-sided right ventricle compared with a right-sided right ventricle. Read More

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http://dx.doi.org/10.1111/joa.13097DOI Listing
February 2020
1 Read

Prognostic power of anaerobic threshold parameters in patients with transposition of the great arteries and systemic right ventricle.

Cardiol Young 2019 Dec 18;29(12):1445-1451. Epub 2019 Oct 18.

Department of Cardiology, Hospital de Santa Marta, Centro Universitário Hospitalar de Lisboa Central, Lisbon, Portugal.

Introduction: Both transposition of the great arteries (TGA) previously submitted to a Senning/Mustard procedure and congenitally corrected TGA (cc-TGA) have the systemic circulation supported by the morphological right ventricle, thereby rendering these patients to heart failure events risk. The aim of this study was to evaluate cardiopulmonary exercise test parameters for stratifying the risk of heart failure events in TGA patients.

Methods: Retrospective evaluation of adult TGA patients with systemic circulation supported by the morphological right ventricle submitted to cardiopulmonary exercise test in a tertiary centre. Read More

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http://dx.doi.org/10.1017/S1047951119002361DOI Listing
December 2019
1 Read

A de novo splicing variant supports the candidacy of TLL1 in ASD pathogenesis.

Eur J Hum Genet 2020 Apr 30;28(4):525-528. Epub 2019 Sep 30.

Department of Pediatrics, Prince Sultan Military Medical City, Riyad, Saudi Arabia.

Congenital heart disease (CHD) is the most common type of birth defects with family- and population-based studies supporting a strong hereditary component. Multifactorial inheritance is the rule although a growing number of Mendelian forms have been described including candidates that have yet to be confirmed independently. TLL1 is one such candidate that was proposed in the etiology of atrial septal defect (ASD). Read More

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http://dx.doi.org/10.1038/s41431-019-0524-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7080731PMC
April 2020
1 Read
4.349 Impact Factor

Exploring the Prognostic Value of Novel Markers in Adults With a Systemic Right Ventricle.

J Am Heart Assoc 2019 09 21;8(17):e013745. Epub 2019 Aug 21.

Department of Cardiology Erasmus MC University Medical Centre Rotterdam Rotterdam The Netherlands.

Background Adults with a systemic right ventricle (sRV) have a high risk of cardiac complications. This study aimed to identify prognostic markers in adults with sRV based on clinical evaluation, echocardiography, and blood biomarkers. Methods and Results In this prospective cohort study, consecutive clinically stable adults with sRV caused by Mustard- or congenitally corrected transposition of the great arteries were included (2011-2013). Read More

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http://dx.doi.org/10.1161/JAHA.119.013745DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6755830PMC
September 2019
6 Reads

Successful staged tricuspid valve replacement following cardiac resynchronization therapy in a congenitally corrected transposition of the great arteries.

Clin Case Rep 2019 Aug 24;7(8):1484-1488. Epub 2019 Jun 24.

Department of Cardiovascular Surgery Tokyo Women's Medical University Tokyo Japan.

Simple tricuspid valve surgery for complex heart disease with systemic right ventricular dysfunction is a high-risk procedure; however, staged tricuspid valve surgery following cardiac resynchronization therapy seems to be a beneficial method to expect reverse systemic ventricular remodeling. Read More

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http://dx.doi.org/10.1002/ccr3.2272DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6693055PMC
August 2019
2 Reads

Ventricular assist device implantation in patients with a failing systemic right ventricle: a call to expand current practice.

Neth Heart J 2019 Dec;27(12):590-593

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

Ventricular assist device (VAD) implantation is an established treatment modality for patients with end-stage heart failure, and improves symptoms and survival. In the Netherlands, it is not yet routinely considered in patients with congenital heart disease and failing systemic right ventricle (SRV). Recently, a VAD was implanted in 2 SRV patients, one who underwent a Mustard procedure during infancy for transposition of the great arteries (male, 47 years old) and one with a congenitally corrected transposition of the great arteries (male, 54 years old). Read More

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http://dx.doi.org/10.1007/s12471-019-01314-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6890896PMC
December 2019
3 Reads

Successful ablation for premature ventricular contraction originating from moderator band of morphologic right ventricle in congenitally corrected transposition of great arteries.

J Electrocardiol 2019 Sep - Oct;56:106-108. Epub 2019 Jul 25.

Division of Cardiology, Osaka Rosai Hospital, Sakai, Osaka, Japan.

A 54-year-old man with congenitally corrected transposition of great arteries (CCTGA) was referred to our hospital for palpitation. 24-hour Holter ECG showed frequent premature ventricular contraction (PVC) and we performed catheter ablation for this PVC. Pace-mapping was performed in morphologic right ventricle (RV) by transaortic approach. Read More

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http://dx.doi.org/10.1016/j.jelectrocard.2019.07.010DOI Listing
July 2019
4 Reads

Commentary: Choosing complex biventricular repair versus Fontan-the unmet needs.

Authors:
Shu-Chien Huang

J Thorac Cardiovasc Surg 2019 10 10;158(4):1171-1172. Epub 2019 Jul 10.

Department of Surgery, National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei, Taiwan. Electronic address:

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http://dx.doi.org/10.1016/j.jtcvs.2019.06.059DOI Listing
October 2019

Virtual dissection and endocast three-dimensional reconstructions: maximizing computed tomographic data for procedural planning of an obstructed pulmonary venous baffle.

Cardiol Young 2019 Aug 26;29(8):1104-1106. Epub 2019 Jun 26.

Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.

We present a case of pulmonary venous baffle obstruction in a child with a history of congenitally corrected transposition status post double switch repair. We highlight two forms of volume rendering three-dimensional reconstructions from computed tomographic data which allowed for detailed pre-surgical planning. These reconstructions emphasise the concept of maximizing previously obtained two-dimensional data in a time-efficient and cost-effective manner. Read More

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http://dx.doi.org/10.1017/S1047951119001501DOI Listing
August 2019
4 Reads

Postnatal Outcome Following Prenatal Diagnosis of Discordant Atrioventricular and Ventriculoarterial Connections.

Pediatr Cardiol 2019 Oct 25;40(7):1509-1515. Epub 2019 Jul 25.

Fetal Cardiology Unit, Department of Congenital Heart Disease, Evelina London Children's Healthcare, Guy's and St Thomas' NHS Foundation Trust, Westminster Bridge Road, London, UK.

Discordant atrioventricular and ventriculoarterial connection(s) (DAVVAC) are a rare group of congenital heart lesions. DAVVAC can be isolated or associated with a variety of other cardiac abnormalities. Previous studies examining the outcome of prenatally diagnosed DAVVAC have described only fetal and early postnatal outcome in small cohorts. Read More

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http://dx.doi.org/10.1007/s00246-019-02176-2DOI Listing
October 2019
3 Reads

Hemodynamically balanced congenitally corrected transposition of the great arteries with a large ventricular septal defect, and subvalvular pulmonic stenosis: a case report.

J Med Case Rep 2019 Jul 19;13(1):219. Epub 2019 Jul 19.

Department of Pathology, Seoul National University College of Medicine, Seoul, Republic of Korea.

Background: Adults with unoperated congenitally corrected transposition of the great arteries are rare but form a distinct group among adults with congenital heart disease. Patients with congenitally corrected transposition of the great arteries often have one or more associated cardiac anomalies that dictate the need for, and timing of, surgical intervention in childhood. However, in a proportion of patients, the hemodynamics does not require surgical attention during childhood, and, in some patients, a correct diagnosis is not established until adulthood. Read More

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http://dx.doi.org/10.1186/s13256-019-2145-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6639941PMC
July 2019
6 Reads

Congenitally corrected transposition of the great arteries in a 69-year-old woman.

Diagn Interv Imaging 2020 Mar 10;101(3):187-188. Epub 2019 Jul 10.

Department of Radiology, Cardiac MR/CT Unit, Rouen University Hospital, 1, rue de Germont, 76031 Rouen cedex, France; Inserm U1096, Normandie University UNIROUEN, 22, boulevard Gambetta, 76000 Rouen, France. Electronic address:

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http://dx.doi.org/10.1016/j.diii.2019.06.008DOI Listing
March 2020
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Commentary: Black or white: The false dichotomy argument inherent in comparing biventricular repair versus Fontan completion.

J Thorac Cardiovasc Surg 2019 10 15;158(4):1168-1170. Epub 2019 Jun 15.

Division of Pediatric Cardiac Surgery, Heart Vascular Institute, Cleveland Clinic, Cleveland, Ohio.

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http://dx.doi.org/10.1016/j.jtcvs.2019.05.074DOI Listing
October 2019

The impact of pulmonary artery banding and cardiac resynchronization therapy for the adult patient with congenitally corrected transposition of the great arteries.

Gen Thorac Cardiovasc Surg 2019 Jul 8. Epub 2019 Jul 8.

Department of Cardiovascular Surgery, Okayama University, 2-5-1 Shikata, Okayama, 700-8558, Japan.

It has been controversial how to manage severe dysfunction of the systemic right ventricle and severe tricuspid regurgitation for adult patients with congenitally corrected transposition of the great arteries. We reported a 43-year-old man with these disorders, who received pulmonary artery banding and cardiac resynchronization therapy without cardiopulmonary bypass. The degree of the tricuspid regurgitation was improved to mild-moderate and systemic right ventricular ejection fraction was improved from 28 to 45% after surgery. Read More

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http://dx.doi.org/10.1007/s11748-019-01171-xDOI Listing
July 2019
4 Reads

Postoperative and short-term atrial tachyarrhythmia burdens after transcatheter vs surgical pulmonary valve replacement among congenital heart disease patients.

Congenit Heart Dis 2019 Sep 7;14(5):838-845. Epub 2019 Jul 7.

Division of Cardiology, Ahmanson/UCLA Adult Congenital Heart Disease Center, Los Angeles, California.

Objective: We examined the atrial tachyarrhythmia (AT) burden among patients with congenital heart disease (CHD) following transcatheter (TC-) or surgical (S-) pulmonary valve replacement (PVR).

Design/setting: This was a retrospective observational study of patients who underwent PVR from 2010 to 2016 at UCLA Medical Center.

Patients: Patients of all ages who had prior surgical repair for CHD were included. Read More

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http://dx.doi.org/10.1111/chd.12818DOI Listing
September 2019
4 Reads

[Congenitally Corrected Transposition of the Great Arteries( TGA) in Adult].

Kyobu Geka 2019 Apr;72(4):269-274

Department of Cardiovascular Surgery, Kyushu University, Fukuoka, Japan.

Objectives: In patients with congenitally corrected transposition of the great arteries( cc-TGA), the morphological right ventricle (RV) sustains the systemic circulation and is frequently associated with progressive tricuspid regurgitation and declining RV function.

Methods: Thirty-four patients with cc-TGA and systemic RV were enrolled in this retrospective study to examine long-term outcome.

Results: Age was averaged 38. Read More

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April 2019
7 Reads

Factors associated with exercise capacity in patients with a systemic right ventricle.

Int J Cardiol 2019 10 13;292:230-235. Epub 2019 Jun 13.

Paediatric and Congenital Cardiology Department, M3C Regional Reference Centre, Montpellier University Hospital, Montpellier, France; PhyMedExp, CNRS, INSERM, University of Montpellier, Montpellier, France. Electronic address:

Background: Systemic right ventricle (RV) is a rare and complex congenital heart disease (CHD). Patients with a systemic RV present with a significant decrease of their exercise capacity. We aimed at identifying clinical and paraclinical factors associated with maximum oxygen uptake (VO2) in adults with a systemic RV. Read More

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http://dx.doi.org/10.1016/j.ijcard.2019.06.030DOI Listing
October 2019
7 Reads