2,284 results match your criteria Ebstein Anomaly


Transcatheter Systemic AV Valve-in-Valve Implantation in a Patient With LTGA and Ebstein Anomaly.

JACC Case Rep 2022 May 4;4(9):551-555. Epub 2022 May 4.

Division of Pediatric Cardiology, Loma Linda University Children's Hospital and Medical Center, Loma Linda, California, USA.

We present the first in-human transcatheter systemic atrioventricular valve-in-valve implantation in a 37-year-old patient with Ebstein anomaly, levo-transposition of the great arteries, and prior systemic valve replacement. She had severe bioprosthetic valve regurgitation and reduced systolic function. She had high surgical risk and was planned for transcatheter intervention. Read More

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Moyamoya Disease Complicating Ebstein's Anomaly.

Intern Med 2022 May 14. Epub 2022 May 14.

Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, Japan.

Ebstein's anomaly is an uncommon congenital disorder affecting the tricuspid valve. We herein report a 38-year-old woman who experienced consciousness and sensory disturbance during treatment for heart failure caused by Ebstein's anomaly. Urgent magnetic resonance imaging and cerebral angiography demonstrated acute cerebral infarction and internal carotid artery obstruction with the development of collateral arteries. Read More

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Pulmonary Artery Development Over Time in Severe Ebstein Anomaly.

Pediatr Cardiol 2022 May 13. Epub 2022 May 13.

Department of Pediatric Cardiology, Fukuoka Children's Hospital, 5-1-1 Kashiiteriha Higashi-ku, Fukuoka, 813-0017, Japan.

Recently, the outcome of severe Ebstein anomaly (EA) has improved with the prevalence of the Starnes procedure. However, time-dependent changes in the size of the central pulmonary artery (PA) have not been fully understood. A retrospective chart review of patients with EA who underwent the Starnes procedure during the neonatal period and those with pulmonary atresia with intact ventricular septum (PAIVS) was performed at Fukuoka Children's Hospital. Read More

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Implantation of a leadless pacemaker in a patient with mechanical tricuspid valve.

HeartRhythm Case Rep 2022 Apr 28;8(4):284-287. Epub 2022 Jan 28.

Department of Thoracic and Cardiovascular Surgery, Keimyung University Dongsan Hospital, Keimyung University School of Medicine, Daegu, Republic of Korea.

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MYH7 variants cause complex congenital heart disease.

Am J Med Genet A 2022 May 2. Epub 2022 May 2.

Division of Human Genetics, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.

MYH7, encoding the myosin heavy chain sarcomeric β-myosin heavy chain, is a common cause of both hypertrophic and dilated cardiomyopathy. Additionally, families with left ventricular noncompaction cardiomyopathy (LVNC) and congenital heart disease (CHD), typically septal defects or Ebstein anomaly, have been identified to have heterozygous pathogenic variants in MHY7. One previous case of single ventricle CHD with heart failure due to a MYH7 variant has been identified. Read More

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Left, but not right, ventricular status determines heart failure in adults with Ebstein anomaly - A case-control study based on magnetic resonance.

Int J Cardiol 2022 Jul 26;358:39-44. Epub 2022 Apr 26.

1(st) Department of Cardiology, University of Medical Sciences, Poznan, Poland; 1-2 Dluga Street, 61-848 Poznan, Poland.

Background: Ebstein anomaly (EA) is a congenital heart defect affecting the right heart. Heart failure (HF) is a significant complication in adults with EA. It may result not only from the right ventricle (RV), but also from the left ventricle (LV) abnormalities. Read More

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Canadian Cardiovascular Society 2022 Guidelines for Cardiovascular Interventions in Adults with Congenital Heart Disease.

Can J Cardiol 2022 Apr 20. Epub 2022 Apr 20.

Cincinnati Children's Hospital Heart Institute, Cincinnati, Ohio, USA.

Interventions in Adults with Congenital Heart Disease (ACHD) focus on surgical and percutaneous interventions in light of rapidly evolving ACHD clinical practice. To bring rigor to our process and amplify the cumulative nature of evidence ACHD care we used the ADAPTE process; we systematically adjudicated, updated, and adapted existing guidelines by Canadian, American, and European cardiac societies between from 2010 to 2020. We applied this to interventions related to right and left ventricular outflow obstruction, tetralogy of Fallot, coarctation, aortopathy associated with bicuspid aortic valve, atrioventricular canal defects, Ebstein anomaly, complete and congenitally corrected transposition, and patients with the Fontan operation. Read More

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Ebstein's Anomaly: From Fetus to Adult-Literature Review and Pathway for Patient Care.

Pediatr Cardiol 2022 Apr 23. Epub 2022 Apr 23.

McGovern Medical School, Children's Heart Institute, University of Texas Health, Houston, TX, USA.

Ebstein's anomaly, first described in 1866 by Dr William Ebstein, accounts for 0.3-0.5% of congenital heart defects and represents 40% of congenital tricuspid valve abnormalities. Read More

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Right ventricular outflow tract anomalies: Neonatal interventions and outcomes.

Semin Perinatol 2022 Mar 12:151583. Epub 2022 Mar 12.

Heart Institute, UPMC Children's Hospital of Pittsburgh, Department of Pediatrics, University of Pittsburgh School of Medicine.

Right ventricular outflow tract (RVOT) anomalies comprise a wide spectrum of congenital heart disease, typically characterized by obstruction to flow from the right ventricle to pulmonary arteries. This review highlights important considerations surrounding management strategy as well as clinical outcomes for the neonate with RVOT anomaly, including: pulmonary atresia with intact ventricular septum, congenital pulmonary valve stenosis, tetralogy of Fallot, and Ebstein anomaly with anatomic or physiologic RVOT obstruction. Read More

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Successful Radiofrequency Ablation of the Right Lateral Accessory Pathway with Modified Carpentier Reconstruction Surgery in a Patient with Ebstein Anomaly Concomitant of Secundum Atrial Septal Defect, Atrial Fibrillation, and Wolff-Parkinson-White Syndrome.

Case Rep Vasc Med 2022 4;2022:8343943. Epub 2022 Apr 4.

Hanoi Heart Hospital, Hanoi Medical University, Vietnam.

Ebstein anomaly (EA) results from the failure of proper delamination of the tricuspid valve leaflets from the right ventricle (RV) myocardium. The severity of EA occurs on a spectrum that results in varying degrees of tricuspid regurgitation, atrial dilation, RV dilation, and dysfunction. These effects have the potential to create substrates that can give rise to atrial arrhythmia, ventricular arrhythmia, and a greater incidence of Wolff-Parkinson-White (WPW) syndrome Wackel et al. Read More

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Case Report: Residual Atrial Shunt Lesions in Aging Adults With Congenital Heart Disease: An Underestimated Risk of Stroke?

Front Cardiovasc Med 2022 16;9:847244. Epub 2022 Mar 16.

Department of Internal Medicine and Cardiology, Charité Universitätsmedizin Berlin, Berlin, Germany.

We report two cases of paradoxical cerebral embolism in adults with congenital heart disease (ACHD) with residual atrial shunt lesions, a 59 year-old male patient with partial detachment of a surgical ASD closure patch, and a 57 year-old male patient with Ebstein's anomaly and a large patent foramen ovale. Considering these mechanisms and the increasing incidence of venous thrombosis with age, a higher prevalence of paradoxical embolism in ACHD patients with residual atrial shunts may be suspected. Regular follow-up of patients with ACHD remains important throughout life even in seemingly stable lesions. Read More

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New trend: Surgical correction of Ebstein's anomaly using modified cone repair and a combination of different techniques.

J Card Surg 2022 Mar 20. Epub 2022 Mar 20.

Pediatric Cardiovascular Surgery Department, Kartal Koşuyolu High Speciality Educational and Research Hospital, Istanbul, Turkey.

Background: Ebstein's anomaly,a rare cardiac disease that accounts for <1% of all congenital heart diseases, is a right ventricular myopathy with varying degrees of delamination in the endocardium under the tricuspid valve. Cone reconstruction, first described by da Silva in 2004, is a modification of the Carpentier technique. An alternative surgical treatment for Ebstein's anomaly, it is now the preferred surgical corrective technique. Read More

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Sudden cardiac death in congenital heart disease.

Eur Heart J 2022 Mar 18. Epub 2022 Mar 18.

Hôpital Européen Georges Pompidou, Université de Paris, Paris, France.

Sudden cardiac death (SCD) accounts for up to 25% of deaths in patients with congenital heart disease (CHD). To date, research has largely been driven by observational studies and real-world experience. Drawbacks include varying definitions, incomplete taxonomy that considers SCD as a unitary diagnosis as opposed to a terminal event with diverse causes, inconsistent outcome ascertainment, and limited data granularity. Read More

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Displacement of all three leaflets of tricuspid valve: A rare variant of Ebstein anomaly.

Clin Case Rep 2022 Mar 8;10(3):e05555. Epub 2022 Mar 8.

Rajaie Cardiovascular Medical and Research Center Iran University of Medical Sciences Tehran Iran.

Ebstein anomaly, a rare congenital heart disease, is defined as displacement of hinge points of septal or posterior tricuspid leaflets but not anterior leaflet. Here, we report the case of a 35-year-old woman with an extremely rare pattern of Ebstein anomaly (EA) with all three tricuspid leaflets displaced downward to the apex. Read More

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[Successful Surgical Repair for Adult Ebstein Disease Using Cone Reconstruction Combined with One and a Half Ventricle Repair].

Kyobu Geka 2022 Feb;75(2):92-97

Second Department of Surgery, Yamagata University Faculty of Medicine, Yamagata, Japan.

A 67-year-old woman presented with dyspnea on effort and cyanosis due to massive tricuspid regurgitation and an atrial septal defect with right to left shunt. She was diagnosed with Ebstein disease at the age of 53 when she underwent surgery for varicose veins. Echocardiography showed the severe apical displacement of the septal and posterior leaflet. Read More

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February 2022

The Mid-Term Outcomes of Cone Repair or Replacement of Tricuspid Valve in Patients with Ebstein's Anomaly: Our Experience.

Heart Surg Forum 2022 Jan 17;25(1):E030-E036. Epub 2022 Jan 17.

Department of Cardiac Surgery, National Heart Foundation Hospital and Research Institute, Mirpur, Dhaka-1216, Bangladesh.

Background: Cone repair of the tricuspid valve (TV) is a contemporary reproducible technique for surgical reconstruction of Ebstein's anomaly. Different authorities have shown that this technique restores excellent tricuspid valve function. In Bangladesh, this technique still is unfamiliar to many. Read More

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January 2022

Ebstein anomaly associated with retinal venular dilatation, migraine, and visual snow syndrome: a case report.

BMC Ophthalmol 2022 Feb 14;22(1):75. Epub 2022 Feb 14.

Department of Pediatric and Congenital Cardiology, Amsterdam University Medical Center, AMC, Meibergdreef 9, 1105, AZ, Amsterdam, The Netherlands.

Background: To present a case with Ebstein anomaly, a rare congenital heart disorder, with ophthalmological and neurophthalmological signs and symptoms. To date, retinal venous dilatation and visual snow syndrome have not been previously been published in this anomaly.

Case Presentation: A 10-year-old white girl was diagnosed with Ebstein anomaly. Read More

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February 2022

Accuracy of Deep Learning Echocardiographic View Classification in Patients with Congenital or Structural Heart Disease: Importance of Specific Datasets.

J Clin Med 2022 Jan 28;11(3). Epub 2022 Jan 28.

Department of Cardiology III-Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Albert-Schweitzer-Campus 1, 48149 Muenster, Germany.

Introduction: Automated echocardiography image interpretation has the potential to transform clinical practice. However, neural networks developed in general cohorts may underperform in the setting of altered cardiac anatomy.

Methods: Consecutive echocardiographic studies of patients with congenital or structural heart disease (C/SHD) were used to validate an existing convolutional neural network trained on 14,035 echocardiograms for automated view classification. Read More

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January 2022

Left Ventricular Dysfunction in Neonatal Ebstein's Anomaly and Tricuspid Valve Dysplasia.

J Am Soc Echocardiogr 2022 May 31;35(5):503-512.e3. Epub 2022 Jan 31.

Fetal & Neonatal Cardiology Program, Division of Cardiology, Department of Pediatrics, Women & Children Health Research Institute, University of Alberta, Edmonton, Alberta, Canada.

Background: The mechanisms and prognostic importance of left ventricular (LV) dysfunction in neonatal Ebstein's anomaly (EA) and tricuspid valve dysplasia (TVD) are not well understood. The authors recently demonstrated reduced cardiac output and dyssynchrony to be common in fetal EA/TVD and therefore hypothesized that LV dysfunction may be associated with worse outcomes in neonatal EA/TVD.

Methods: A multicenter retrospective case-control study was conducted among neonatal patients with EA/TVD (n = 32) and a healthy control cohort (n = 17) encountered from 2004 to 2019. Read More

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Impact of fetal haemodynamics on surgical and neurodevelopmental outcomes in patients with Ebstein anomaly and tricuspid valve dysplasia.

Cardiol Young 2022 Jan 6:1-12. Epub 2022 Jan 6.

Division of Pediatric Cardiology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, ON, Canada, Toronto.

Objectives: To evaluate the impact of fetal haemodynamics on surgical and neurodevelopmental outcomes in severe Ebstein anomaly and tricuspid valve dysplasia.

Methods: Thirty-four fetuses with Ebstein anomaly/tricuspid valve dysplasia were referred from 2013 to 2019 for fetal echocardiography and clinical management. Nineteen fetuses with Ebstein anomaly/tricuspid valve dysplasia and 30 controls underwent cardiovascular magnetic resonance to quantify the fetal blood flow and to calculate cerebral oxygen delivery (cDO2) and consumption (cVO2). Read More

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January 2022

Simple, reproducible, and consistent physiology: The argument for single-ventricle repair in critically ill neonates with Ebstein anomaly.

JTCVS Tech 2021 Dec 3;10:428-432. Epub 2021 Apr 3.

Division of Cardiac Surgery, Department of Surgery, Children's Hospital Los Angeles, Los Angeles, Calif.

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December 2021

A case of redo tricuspid valve repair after neonatal cone operation for Ebstein's anomaly.

Gen Thorac Cardiovasc Surg 2022 Mar 29;70(3):289-291. Epub 2021 Nov 29.

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

Cone reconstruction for Ebstein's anomaly, although effective, is challenging in neonatal cases. Very few reports have discussed recurrent regurgitation. Herein, we report a successful redo tricuspid valve repair for recurrent regurgitation 5 years after cone reconstruction. Read More

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Ebstein Anomaly and Right Aortic Arch in Patient with Charge Syndrome.

Medicina (Kaunas) 2021 Nov 13;57(11). Epub 2021 Nov 13.

Department of Pediatrics, Riga Stradins University, LV-1007 Riga, Latvia.

Ebstein anomaly is a rare congenital heart disease characterized by a varying degree of anatomical and functional abnormalities of tricuspid valve and right ventricle. It often coexists with other congenital cardiac malformations. Up to 79-89% of patients with Ebstein anomaly have interatrial communication in the form of patent oval foramen or atrial septal defect and more than one-third has other types of cardiac malformations. Read More

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November 2021

Transcatheter tricuspid valve-in-valve implantation for degenerative surgical bio-prosthesis using SAPIEN 3: A case series.

Clin Case Rep 2021 Nov 19;9(11):e05029. Epub 2021 Nov 19.

Faculty of Medicine Ramathibodi Hospital Mahidiol University Bangkok Thailand.

We evaluated early outcomes of transcatheter valve-in-valve (ViV) implantation in patients with degenerated bio-prosthesis in tricuspid position. Total of 5 patients were included in our case series. Baseline native tricuspid valve etiology were highly varied ranging from chest wall trauma, Ebstein anomaly, rheumatic heart disease, infective endocarditis and complex congenital heart disease. Read More

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November 2021

Modified Starnes procedure as a bridge to 2-ventricle repair in neonatal Ebstein's anomaly: A paradigm shift.

Multimed Man Cardiothorac Surg 2021 Nov 24;2021. Epub 2021 Nov 24.

Division of Pediatric Cardiovascular Surgery, Masonic Children's Hospital, University of Minnesota, Minneapolis, MN, 55454.

Severe tricuspid valve regurgitation secondary to Ebstein's anomaly represents several challenges in neonates. It can result in significant respiratory and/or hemodynamic compromise that mandates urgent interventions. When conservative management fails, 2 surgical options are available: tricuspid valve repair or single ventricle palliation. Read More

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November 2021

Successful One-and-a-Half Ventricle Repair of Right Ventricle Dysfunction Due to Lymphoblastic Leukemia Treatment in a Patient with Restrictive Cardiomyopathy.

Am J Case Rep 2021 Nov 24;22:e933677. Epub 2021 Nov 24.

Department of Congenital Heart Diseases, A.N. Bakoulev National Medical Research Center of Cardiovascular Surgery, Moscow, Russian Federation.

BACKGROUND The cardiotoxic effects of chemotherapy in cancer treatment can damage cardiomyocytes. A common link in the pathogenesis is the proliferation of fibroblasts and the increase of collagen synthesis, leading to development of common endomyocardial fibrosis. The walls of ventricles become rigid and their inability to relax prevents them from carrying the required amount of blood. Read More

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November 2021

Imaging for Transcatheter Edge-to-Edge Repair for Tricuspid Regurgitation in Ebstein Anomaly.

Circ Cardiovasc Imaging 2021 11 11;14(11):e013327. Epub 2021 Nov 11.

Li Ka Shing Institute of Health Science, The Chinese University of Hong Kong, Hong Kong SAR, China (K.K., K.S., A.P.-W.L.).

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November 2021

Accessory pathway ablation during atrial fibrillation in Ebstein anomaly.

Pacing Clin Electrophysiol 2022 Mar 23;45(3):431-434. Epub 2021 Nov 23.

The Second Department of Internal Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan.

An 84-year-old woman with type B Wolff-Parkinson-White (WPW) with Ebstein anomaly was admitted with heart failure. She had rapid wide QRS tachycardia due to accessory pathway (AP) conduction associated with atrial fibrillation (AF). Since transesophageal echocardiography before catheter ablation showed a left atrial thrombus, ablation was performed using a 3D mapping system under AF. Read More

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The Association of Pulmonary Flow Characteristics With Cardiac Function in Tricuspid Valve Malformation Fetuses: Study With Two-Dimensional Speckle Tracking Echocardiography.

J Ultrasound Med 2021 Nov 2. Epub 2021 Nov 2.

Department of Ultrasound Medicine, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.

Objectives: To explore the relationship between cardiac function and pulmonary flow characteristics in tricuspid valve malformation (TVM) fetuses by conventional and speckle tracking echocardiography.

Methods: Eighty-eight TVM fetuses were retrospectively included and compared with 80 normal controls. TVM fetuses in each trimester were divided into two subgroups according to pulmonary flow characteristics: those with normal pulmonary flow (TVM-N) and those with reduced or absent pulmonary flow (TVM-R/A). Read More

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November 2021

Improved Tricuspid Valve Function, Preload Recruitment and Ventricular Efficiency During Submaximal Exercise in Patients with Unoperated Ebstein's Anomaly: An MRI Study.

J Magn Reson Imaging 2022 06 15;55(6):1843-1850. Epub 2021 Oct 15.

Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich, Technical University of Munich, Munich, Germany.

Background: Adolescents and adults with native Ebstein's anomaly (EA) are at the benign part of the Ebstein spectrum, having survived infancy without surgery. In this population, surgical indication and timing remain objects of controversy and depend, among other factors, on exercise capacity.

Purpose: To better understand the pathophysiology of exercise adaptation in native EA. Read More

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