2,199 results match your criteria Ebstein Anomaly


5 shades of grey in a Broad QRS tachycardia. What is the mechanism?

J Cardiovasc Electrophysiol 2021 May 2. Epub 2021 May 2.

Department of Cardiology, Christian Medical College, Vellore, Tamilnadu, India.

30-year-old gentleman with a mild form of Ebstein's anomaly complained of recurrent palpitations but had no documented tachycardia. His baseline electrocardiogram (ECG) showed pre-excitation which was suggestive of a right posterior accessory pathway This article is protected by copyright. All rights reserved. Read More

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Defibrillation Safety Margin Testing in Patients With Congenital Heart Disease: Results From the NCDR.

JACC Clin Electrophysiol 2021 Apr 22. Epub 2021 Apr 22.

Section of Cardiac Electrophysiology, Division of Cardiology, University of California-San Diego, La Jolla, California, USA.

Objectives: This study analyzed the predictors of defibrillation safety margin (DSM) testing at the time of implantable cardioverter-defibrillator (ICD) insertion and factors associated with inadequate DSM in patients with congenital heart disease (CHD).

Background: There are few data about the prevalence and safety of DSM testing in those with CHD.

Methods: A retrospective analysis was performed of all patients with atrial or ventricular septal defect, tetralogy of Fallot, transposition of the great vessels, Ebstein anomaly, or common ventricle undergoing a transvenous ICD procedure in the National Cardiovascular Data Registry (NCDR) ICD Registry from April 2010 to March 2016, and DSM testing was assessed. Read More

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Neonatal Ebstein anomaly national outcomes from 2000 to 2018 using the National Inpatient Sample.

Birth Defects Res 2021 Mar 31. Epub 2021 Mar 31.

Department of Neonatology, Cleveland Clinic Children's, Cleveland, Ohio, USA.

Background: The prevalence, morbidity, and mortality associated with Ebstein anomaly (EA) remains poorly characterized in neonates. EA is a rare form of congenital heart disease (CHD) with significant heterogeneity.

Objective: To determine the recent, 2000-2018, prevalence, mortality, outcomes, and healthcare utilization of infants admitted at ≤28 days of life with EA in comparison to other critical congenital heart defects (CCHD) in the United States using a national data set. Read More

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Uncommon mitral valve anomalies associated with Ebstein anomaly.

Rev Esp Cardiol (Engl Ed) 2021 Mar 27. Epub 2021 Mar 27.

Departamento de Ecocardiografía Pediátrica, Instituto Nacional de Cardiología Ignacio Chávez, Mexico City, Mexico. Electronic address:

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Ebstein's anomaly in the neonate.

Indian J Thorac Cardiovasc Surg 2021 Jan 21;37(Suppl 1):17-25. Epub 2020 Mar 21.

Department of Congenital Cardiothoracic Surgery, NYU Langone Health, New York, NY 10016 USA.

Ebstein's anomaly is a rare congenital heart defect characterized by apical displacement of the tricuspid valve and reduced volume of the true functional right ventricle. In its most severe form, the disease presents as right heart failure and cyanosis in the neonate. Although many of these neonates can be managed conservatively, surgical intervention may become necessary in the sickest of them. Read More

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January 2021

Selective His Bundle Pacing in a Patient With Ebstein's Anomaly and Atrioventricular Block.

JACC Clin Electrophysiol 2021 02;7(2):275-276

Geisinger Heart Institute, Geisinger Commonwealth School of Medicine, Wilkes Barre, Pennsylvania, USA. Electronic address:

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February 2021

Hemodynamics and Clinical Implications of Occult Left Ventricular Dysfunction in Adults Undergoing Ebstein Anomaly Repair.

Circ Cardiovasc Imaging 2021 Feb 15;14(2):e011739. Epub 2021 Feb 15.

Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN.

Background: Left ventricular global longitudinal strain (LVGLS) can detect early phases of LV systolic dysfunction, but its application has not been studied in Ebstein anomaly. We hypothesized that LVGLS can detect early phases of LV systolic dysfunction and that patients with occult LV systolic dysfunction will have worse hemodynamics, end-organ dysfunction, and suboptimal postoperative LV reverse remodeling after tricuspid valve surgery in comparison to patients with normal LV systolic function.

Methods: In this retrospective cohort study, 371 Ebstein patients that underwent tricuspid valve surgery were divided into 3 groups: normal LV systolic function (normal LVGLS and LV ejection fraction; n=244, 77%), occult LV systolic dysfunction (abnormal LVGLS with normal LV ejection fraction; n=44, 14%), and overt LV systolic dysfunction (abnormal LVGLS and LV ejection fraction; n=27, 9%). Read More

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February 2021

From Safety to Benefit in Cell Delivery During Surgical Repair of Ebstein Anomaly: Initial Results.

Ann Thorac Surg 2021 Feb 1. Epub 2021 Feb 1.

Wanek HLHS Consortium Clinical Pipeline, Mayo Clinic, Rochester, Minnesota, USA.

Background: The objective of this study is to assess the safety and early impact of intramyocardial delivery of autologous bone marrow-derived mononuclear cells (BM-MNC) at time of surgical Ebstein repair.

Methods: Patients with EA (ages 6 months to 30 years) scheduled to undergo repair of the TV were eligible to participate in this open label, non-randomized Phase I clinical trial. BM-MNC target dose was 1-3 million cells/kg. Read More

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February 2021

Balloon Valvuloplasty for Congenital Aortic Stenosis: Experience at a Tertiary Center in a Developing Country.

J Interv Cardiol 2021 12;2021:6681693. Epub 2021 Jan 12.

Department of Pediatrics and Adolescent Medicine, American University of Beirut Medical Center (AUBMC), Beirut, Lebanon.

Background: Aortic valve stenosis accounts for 3-6% of congenital heart disease. Balloon aortic valvuloplasty (BAV) is the preferred therapeutic intervention in many centers. However, most of the reported data are from developed countries. Read More

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January 2021

Successful Radiofrequency Ablation of an Atrial Flutter in an Elderly Patient With Uncorrected Ebstein's Anomaly.

Cureus 2020 Dec 23;12(12):e12242. Epub 2020 Dec 23.

Department of Cardiology, St. Vincet Hospital, Ascension Medical Group, Evansville, USA.

Ebstein's anomaly is a rare congenital heart disorder that causes displacement of the tricuspid valve towards the apex of the heart. Patients with this anomaly rarely survive beyond the age of 70 without surgical correction. When such patients survive till adulthood, they tend to present with atrial arrhythmias. Read More

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December 2020

Preoperative percutaneous oxygen saturation is a predictor of postoperative adverse events after Ebstein's anomaly reconstruction.

J Card Surg 2021 Mar 27;36(3):1012-1017. Epub 2021 Jan 27.

Pediatric Cardiac Center, Beijing Anzhen Hospital, Capital Medical University, Beijing, China.

Background: Ebstein's anomaly (EA) is a kind of congenital heart disease, which is currently widely treated by cone reconstruction. However, the prediction of postoperative recovery is still challenging.

Methods: A retrospective analysis was performed on EA cases undergoing cone reconstruction from January 2010 to January 2016. Read More

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Commentary: Ventricular function improvement after the cone for Ebstein anomaly: It is time to incorporate magnetic resonance studies into every long-term postoperative protocol.

J Thorac Cardiovasc Surg 2020 Dec 31. Epub 2020 Dec 31.

Division of Pediatric and Congenital Cardiac Surgery, Department of Cardiac, Thoracic, and Vascular Surgery, Columbia University Irving Medical Center, Morgan Stanley Children's Hospital/NewYork-Presbyterian, New York, NY.

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December 2020

Cone repair for Ebstein's anomaly and atrial fibrillation ablation in an adult patient.

Multimed Man Cardiothorac Surg 2020 11 30;2020. Epub 2020 Nov 30.

Department of Cardiovascular Surgery, Institut Cardiovascular, Hospital Clìnic, 170th Villarroel St., 08036 Barcelona, Spain.

We present a 52-year-old woman with Ebstein's anomaly not previously treated. In this subset of patients, there are no clear guidelines regarding the best surgical strategy for treating the tricuspid valve: replace it or repair it.  In this case, extensive repair of the tricuspid valve and the right ventricle is achieved using the cone repair technique popularized by Dr. Read More

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November 2020

Commentary: Cone reconstruction for Ebstein's anomaly is here to stay.

Authors:
Pedro J Del Nido

J Thorac Cardiovasc Surg 2021 03 5;161(3):1110-1111. Epub 2020 Dec 5.

Department of Cardiac Surgery, Boston Children's Hospital, and Harvard Medical School, Boston, Mass. Electronic address:

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Transcatheter pulmonary valve implantation in tetralogy of Fallot and Ebstein's anomaly with one and a half ventricular repair.

Cardiol Young 2021 Apr 11;31(4):658-660. Epub 2021 Jan 11.

Department of Pediatric Cardiology, Istanbul University of Health Sciences, Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, Istanbul, Turkey.

The coexistence of tetralogy of Fallot and Ebstein's anomaly is extremely rare. There are only a few case reports in the literature, and surgical options for the treatment are controversial. There is insufficient data on long-term follow-up of patients and management of complications. Read More

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Commentary: Gold or silver? Value of cardiac magnetic resonance imaging over echocardiography in Ebstein's anomaly.

J Thorac Cardiovasc Surg 2021 03 19;161(3):1109-1110. Epub 2020 Nov 19.

Department of Cardiovascular Surgery, Mayo Clinic, Rochester, Minn. Electronic address:

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Cor triatriatum dexter associated to Ebstein anomaly with tricuspid double lesion and atrial septal defect.

Arch Cardiol Mex 2020 Dec 21. Epub 2020 Dec 21.

Department of Nuclear cardiology, National Institute of Cardiology Ignacio Chávez, Mexico City; Department of Echocardiography, ABC Medical Center, I.A.P, Mexico City, Mexico.

Female 23-year-old patient with heart murmur diagnosed in the first year of life. She attended to our institute with progressive dyspnea and palpitations of 7-month evolution. Physical examination showed perioral and distal cyanosis with digital clubbing, oxygensaturation of 79%, jugular plethora, arrhythmic heartsounds of upper limbs, fixed second heart sound, systolic tricuspid murmur, and edema. Read More

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December 2020

Prognostic Role of Hepatorenal Function Indexes in Patients With Ebstein Anomaly.

J Am Coll Cardiol 2020 12;76(25):2968-2976

Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota, USA.

Background: Hepatorenal dysfunction is a risk factor for mortality in patients with chronic tricuspid regurgitation due to acquired heart disease. Ebstein anomaly is the most common cause of primary tricuspid regurgitation in adults with congenital heart disease, but the prevalence and prognostic implications of hepatorenal dysfunction are unknown in this population.

Objectives: The purpose of this study was to determine the risk factors and prognostic implications of hepatorenal dysfunction, as measured primarily by the use of model for end-stage liver disease excluding international normalized ratio (MELD-XI score), as well as looking at other associated factors. Read More

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December 2020

Surgical management of Ebstein anomaly: impact of the adult congenital heart disease anatomical and physiological classifications.

Interact Cardiovasc Thorac Surg 2021 Apr;32(4):593-600

Department of Congenital Heart Surgery - Pediatric Heart Surgery, German Heart Center Berlin, Berlin, Germany.

Objectives: Our goal was to evaluate the impact of the adult congenital heart disease anatomical and physiological (ACHD AP) classification system on the surgical management of Ebstein anomaly (EA) in adult patients.

Methods: From February 2000 through August 2017, data of patients aged at least 16 years, who underwent primary EA surgery, were retrospectively evaluated. The cohort was divided in 2 groups according to their ACHD AP classification: the moderate EA group (IIB, IIC) and the severe EA group (IID). Read More

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Cone reconstruction for Ebstein anomaly: Late biventricular function and possible remodeling.

J Thorac Cardiovasc Surg 2021 03 13;161(3):1097-1108. Epub 2020 Nov 13.

Department of Cardiology, Great Ormond Street Hospital for Children, London, United Kingdom; Centre for Cardiovascular Imaging, Great Ormond Street Hospital for Children, London, United Kingdom.

Objectives: To evaluate late-term tricuspid valve competence and biventricular function following cone reconstruction for Ebstein anomaly, and to explore biventricular remodeling.

Methods: Consecutive adult and pediatric patients who underwent cone reconstruction from 2009 to 2019 were reviewed for inclusion in this retrospective cardiac magnetic resonance imaging study. Tricuspid valve competence was assessed with tricuspid regurgitation fraction. Read More

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Extracardiac Doppler indices predict perinatal mortality in fetuses with Ebstein anomaly and tricuspid valve dysplasia.

Prenat Diagn 2021 Feb 9;41(3):332-340. Epub 2021 Jan 9.

Department of Pediatrics, Division of Cardiology, Children's Hospital Los Angeles, University of Southern California Keck School of Medicine, Los Angeles, California, USA.

Objectives: Ebstein anomaly and tricuspid valve dysplasia (EA/TVD) carry high perinatal mortality. Past studies have focused on cardiac predictors of mortality; we sought to describe the fetal echo (FE) extracardiac Dopplers in this cohort and determine their association with perinatal mortality.

Method: Fetuses with EA/TVD at 23 centers from 2005-2011 were included for retrospective study. Read More

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February 2021

Surgical management for an adult, female patient, with Ebstein Anomaly on Tricuspid Valve that has Subvalvular membrane with severe PS and multiple VSDs-A case report.

Heart Surg Forum 2020 Oct 16;23(6):E781-E785. Epub 2020 Oct 16.

Senior Consultant, Department of Cardiac Surgery, National Heart Foundation Hospital & Research Institute, Dhaka, Bangladesh.

Ebstein's anomaly is a rare and complexed heart defect that affects the tricuspid valve and is accountable for around 1% of congenital cardiac abnormalities. It is one of the most common congenital causes of tricuspid valve regurgitation. Ebstein's anomaly is often diagnosed prenatally due to its severe cardiomegaly. Read More

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October 2020

Mitral Ebstein's Anomaly Modified with a Scarred Rhabdomyoma in Tuberous Sclerosis: An Extremely Rare Cause of Mitral Insufficiency.

Intern Med 2021 Apr 23;60(8):1225-1229. Epub 2020 Nov 23.

Division of Cardiovascular Medicine, Department of Internal Medicine, Japan.

We present an extremely rare case of mitral Ebstein's anomaly that resulted in severe mitral regurgitation (MR). A 41-year-old woman with a history of tuberous sclerosis underwent surgery. Preoperatively, it was assumed that MR had occurred due to leaflet tethering related to left ventricular posterior wall motion asynergy due to a scarred rhabdomyoma. Read More

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Effect of In Utero Non-Steroidal Anti-Inflammatory Drug Therapy for Severe Ebstein Anomaly or Tricuspid Valve Dysplasia (NSAID Therapy for Fetal Ebstein anomaly).

Am J Cardiol 2021 02 18;141:106-112. Epub 2020 Nov 18.

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

Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD) are rare congenital malformations associated with nearly 50% mortality when diagnosed in utero. The diseases often produce severe tricuspid regurgitation (TR) in the fetus and in some cases, pulmonary regurgitation (PR) and circular shunting ensue. Since the ductus arteriosus (DA) plays a critical role in the circular shunt and may be constricted by transplacental nonsteroidal anti-inflammatory drugs (NSAIDs), we sought to assess the effect of NSAIDs on fetuses with EA/TVD. Read More

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February 2021

Risk Assessment in Patients With Tricuspid Valve Regurgitation: MELD and Beyond.

J Am Coll Cardiol 2020 12 9;76(25):2977-2979. Epub 2020 Nov 9.

Department of Cardiovascular Surgery, Icahn School of Medicine at Mount Sinai, New York, New York, USA.

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December 2020

Transcatheter tricuspid valve-in-valve replacement in two patients with Ebstein anomaly: technical considerations.

Clin Res Cardiol 2021 Mar 9;110(3):472-477. Epub 2020 Nov 9.

Klinikum Oldenburg - Universitätsklinik für Innere Medizin - Kardiologie, Oldenburg, Germany.

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The Congenital Tricuspid Valve Spectrum: From Ebstein to Dysplasia.

World J Pediatr Congenit Heart Surg 2020 11;11(6):783-791

Department of Pathology, 4352Mayo Clinic, Rochester, MN, USA.

Ebstein anomaly has a breadth of presentations, including "typical" and "atypical," and can be confused with congenital tricuspid dysplasia. We summarize how to differentiate within this spectrum of disease. Both typical and atypical Ebstein have an underlying failure of delamination, but atypical Ebstein does not have ≥8mm/m apical septal leaflet displacement. Read More

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November 2020

Haemodynamic determinants of improved aerobic capacity after tricuspid valve surgery in Ebstein anomaly.

Heart 2020 Oct 29. Epub 2020 Oct 29.

Cardiovascular Medicine, Mayo Clinic Minnesota, Rochester, Minnesota, USA.

Background: Although tricuspid valve surgery improves functional capacity in patients with Ebstein anomaly, it is not always associated with improvement in aerobic capacity. The purpose of this study was to identify the determinants of improved aerobic capacity after tricuspid valve surgery in adults with Ebstein anomaly with severe tricuspid regurgitation.

Methods: Retrospective study of patients with severe tricuspid regurgitation due to Ebstein anomaly that had tricuspid valve surgery at Mayo Clinic Rochester (2000-2019) and had preoperative and postoperative cardiopulmonary exercise tests and echocardiograms. Read More

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October 2020