645 results match your criteria Ebstein Anomaly Imaging

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

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

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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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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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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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

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

Tricuspid Valve Dysplasia at Fetal Autopsy.

Fetal Pediatr Pathol 2020 Oct 16:1-4. Epub 2020 Oct 16.

Dr R.K. Hospital for Women and Children, Thanjavur, India.

Introduction: Tricuspid valve abnormalities detected in fetal life include Ebstein anomaly and tricuspid valve dysplasia. The differentiation between these 2 entities can sometimes be challenging in the 2nd trimester fetus. We report a case of tricuspid valve dysplasia diagnosed on fetal autopsy. Read More

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October 2020

Ebstein's Anomaly of the Tricuspid Valve: an Overview of Pathology and Management.

Curr Cardiol Rep 2020 10 9;22(12):157. Epub 2020 Oct 9.

Division of Cardiology, Department of Medicine, University of Washington, 1959 Pacific Street, Box 356422, Seattle, WA, 98195, USA.

Purpose Of Review: Ebstein's anomaly (EA) is a rare, but complex form of congenital heart disease consisting of a right ventricular myopathy and morphologic tricuspid valve disease leading to a high incidence of right ventricular dysfunction and arrhythmias. This review offers an updated overview of the current understanding and management of patients with EA with a focus on the adult population.

Recent Findings: Increased understanding of anatomic accessory atrioventricular pathways in EA has resulted in an improvement in ablation techniques and long-term freedom of atrial arrhythmia recurrence. Read More

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October 2020

Strain and Strain Rate To Evaluate Right Heart Function of Ebstein Anomaly (EA) Patients Before and After Operation.

Heart Surg Forum 2020 Sep 8;23(5):E636-E640. Epub 2020 Sep 8.

Heart Center, The First Hospital of Tsinghua University (Beijing Huaxin Hospital), Beijing, China.

Objective: This study was aimed to elucidate the feasibility of using right ventricular (RV) strain and strain rate to evaluate right heart function of Ebstein anomaly (EA) patients before and after operation.

Methods: Sixty EA patients and 30 healthy controls underwent echocardiography (UCG) for evaluation of right heart function. Preoperative UCG and 1-week and 3-month postoperative UCG were performed in EA patients. Read More

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September 2020

Elderly Woman With Cough and Palpitations.

Jun Huang

Ann Emerg Med 2020 09;76(3):e39-e40

Department of Echocardiography, the Affiliated Changzhou No. 2 People's Hospital with Nanjing Medical University, Changzhou, China.

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September 2020

Congenitally corrected transposition and mitral atresia complicated by restrictive atrial septum.

J Clin Ultrasound 2020 Oct 4;48(8):510-512. Epub 2020 Aug 4.

The Congenital Heart Collaborative, Rainbow Babies and Children's Hospital, Case Western Reserve University School of Medicine, Cleveland, Ohio, USA.

Congenitally corrected transposition (ccTGA), also known as L-transposition of the great arteries (L-TGA), is a rare cardiac malformation accounting for approximately 0.05% of congenital heart disease, characterized by ventricular inversion, discordant ventriculo-arterial connections with a normal visceroatrial relationship. It was first described by Baron Rokitansky in 1875, and prenatal sonographic diagnosis can be difficult. Read More

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October 2020

Differential diagnosis of arrhythmogenic cardiomyopathy: phenocopies versus disease variants.

Minerva Med 2021 Apr 22;112(2):269-280. Epub 2020 Jul 22.

Department of Cardiac-Thoracic-Vascular Sciences and Public Health, University of Padua, Padua, Italy -

Arrhythmogenic cardiomyopathy (ACM) is a genetic heart muscle disease caused by mutations of desmosomal genes in about 50% of patients. Affected patients may have defective non-desmosomal genes. The ACM phenotype may occur in other genetic cardiomyopathies, cardio-cutaneous syndromes or neuromuscular disorders. Read More

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Commentary: Should the cone repair be the only option to consider for all patients with Ebstein's anomaly? Definitely not.

David Kalfa

J Thorac Cardiovasc Surg 2020 12 24;160(6):1557-1558. Epub 2020 Jun 24.

Section of Pediatric and Congenital Cardiac Surgery, Division of Cardiac, Thoracic and Vascular Surgery, New York-Presbyterian Morgan Stanley Children's Hospital, Columbia University Medical Center, New York, NY. Electronic address:

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December 2020

Diagnosis of Ebstein Anomaly with Atrial Septal Defect and Persistent Left Superior Vena Cava Using Cardiac Magnetic Resonance Imaging.

J Cardiovasc Imaging 2020 Oct 19;28(4):283-285. Epub 2020 Mar 19.

Department of Radiology, Soonchunhyang University Hospital, Seoul, Korea.

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October 2020

Does addition of bi-directional cavo-pulmonary shunt to tricuspid repair in advanced cases of Ebstein anomaly result in better outcomes?

Gen Thorac Cardiovasc Surg 2020 Dec 20;68(12):1388-1396. Epub 2020 May 20.

Department of Cardiac Anesthesia, U. N. Mehta Institute of Cardiology and Research Center, Ahmedabad, India.

Objectives: Ebstein anomaly can be managed by single ventricular, bi-ventricular and one and a half ventricular repairs. We present midterm results of Comprehensive Tricuspid Valve repair (CTVR) with bi-directional cavo-pulmonary shunt (BCPS).

Methods: In this prospective observational study (Jan2012-July2018), 69 patients underwent surgery for Ebstein anomaly. Read More

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December 2020

Percutaneous Atrial Septal Defect Closure in Adult Ebstein's Anomaly with Exertional Hypoxemia.

Int Heart J 2020 May 15;61(3):620-623. Epub 2020 May 15.

Department of Pediatrics, Jichi Medical University.

In Ebstein's anomaly, percutaneous atrial septal defect (ASD) closure for the treatment of hypoxemia due to a right-to-left interatrial shunt remains controversial. We report the case of a 40-year-old woman with Ebstein's anomaly who developed cyanosis and shortness of breath on exercise. Her symptoms improved after percutaneous ASD closure and her clinical course has been good during follow-up. Read More

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Epidemiological, clinical, and echocardiographic features, and outcome of dogs with Ebstein's anomaly: 32 cases (2002-2016).

J Vet Cardiol 2020 Jun 3;29:11-21. Epub 2020 Apr 3.

Ecole Nationale Vétérinaire d'Alfort, Univ Paris Est Creteil, INSERM, IMRB, F-94700 Maisons-Alfort, France; Department of Biostatistics and Clinical Epidemiology, Université Paris-Est, École Nationale Vétérinaire d'Alfort, 7 Avenue Du Général de Gaulle, Maisons-Alfort, F-94700, France.

Introduction/objectives: Ebstein's anomaly (EA) is a congenital heart disease characterized by apical displacement of the tricuspid valve leaflets in the right ventricle. The objective of this retrospective study was to investigate the signalment, clinical features, echocardiographic findings, and outcome of dogs with EA.

Animals, Materials And Methods: Medical records of 40 dogs with EA were reviewed. Read More

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Right Ventricular Systolic Function After the Cone Procedure for Ebstein's Anomaly: Comparison Between Echocardiography and Cardiac Magnetic Resonance.

Pediatr Cardiol 2020 Jun 25;41(5):985-995. Epub 2020 Apr 25.

Echocardiography Laboratory, Hospital Israelita Albert Einstein, Av. Albert Einstein, 627/701, Morumbi, São Paulo, SP, 05652-900, Brazil.

Although the Cone procedure has improved outcomes for patients with Ebstein´s anomaly (EA), neither RV systolic function recovery in long-term follow-up nor the best echocardiographic parameters to assess RV function are well established. Thus, we evaluated RV performance after the Cone procedure comparing two-dimensional (2DEcho) and three-dimensional (3DEcho) echocardiography to cardiac magnetic resonance (CMR). We assessed 27 EA patients after the Cone procedure (53% female, median age of 20 years at the procedure, median post-operative follow-up duration of 8 years). Read More

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Unusual Location of a Ventricular Tachycardia: Let the Cat Out of the Bag.

JACC Clin Electrophysiol 2020 01;6(1):127-128

Department of Cardiology (Division D), University Hospital Southampton, Southampton, United Kingdom.

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January 2020

Enlarged coronary sinus thrombosis after repair of Ebstein's anomaly.

J Card Surg 2020 Mar 16;35(3):675-678. Epub 2020 Jan 16.

Department of Cardiovascular Surgery, Kumamoto University Hospital, Kumamoto, Japan.

A 58-year-old woman was referred to our hospital with progressively increasing breathlessness. She reported a history of bioprosthetic valve implantation for tricuspid valve replacement and direct closure of an atrial septal defect for Ebstein's anomaly, 31 years before presentation. Transthoracic echocardiography revealed prosthetic valve failure, an enlarged coronary sinus, and severe mitral regurgitation. Read More

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Ebstein's anomaly with 'reversible' functional pulmonary atresia.

BMJ Case Rep 2019 Dec 29;12(12). Epub 2019 Dec 29.

Pediatric Cardiology, Hospital Santa Cruz, Carnaxide, Portugal.

We present the case of an infant with prenatal diagnosis, at 32 weeks gestation, of Ebstein's anomaly without anterograde flow from right ventricular to pulmonary atresia (PA)-functional PA with flow reversal in the ductus arteriosus. Prostaglandin E1 was started after birth. Chest X-ray showed severe cardiomegaly and echocardiogram confirmed Ebstein's anomaly with a thickened non-opening pulmonary valve without anterograde flow but with mild regurgitation. Read More

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December 2019

Heart failure with reduced and preserved ejection fraction in adult congenital heart disease.

Heart Fail Rev 2020 07;25(4):569-581

Department of Medicine, Division of Cardiovascular Medicine, Stanford University School of Medicine, Palo Alto, CA, USA.

Heart failure with reduced ejection fraction (HFrEF) is common in patients with adult congenital heart disease. Many of the most common congenital defects have a high prevalence of HFrEF, including left-sided obstructive lesions (aortic stenosis, coarctation of the aorta, Shone complex), tetralogy of Fallot, Ebstein anomaly, lesions in which there is a systemic right ventricle, and lesions palliated with a Fontan circulation. However, heart failure with preserved ejection fraction (HFpEF) is also prevalent in all these lesions. Read More

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Current and future role of fetal cardiovascular MRI in the setting of fetal cardiac interventions.

Prenat Diagn 2020 01 19;40(1):71-83. Epub 2019 Dec 19.

Division of Pediatric Cardiology, Department of Pediatrics, University of Toronto and Hospital for Sick Children, Toronto, Ontario, Canada.

Over recent years, technical developments resulting in the feasibility of fetal cardiovascular magnetic resonance (CMR) have provided a new diagnostic tool for studying the human fetal heart and circulation. During the same period, we have witnessed the arrival of several minimally invasive fetal cardiac interventions (FCI) as a possible form of treatment in selected congenital heart diseases (CHDs). The role of fetal CMR in the planning and monitoring of FCI is not yet clear. Read More

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January 2020

Electrocardiography findings and clinical presentation in Ebstein's anomaly.

Singapore Med J 2019 Nov;60(11):560-564

Department of Cardiology, National University Heart Centre Singapore, Singapore.

Ebstein's anomaly is a congenital malformation characterised by tricuspid valve pathology with right heart enlargement. Cases of Ebstein's anomaly can vary widely in severity, anatomy and presentation. In this article, we presented three cases of Ebstein's anomaly and discussed the presentation as well as electrocardiographic (ECG) changes. Read More

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November 2019

Severity Scores for Ebstein Anomaly: Credibility and Usefulness of Echocardiographic vs Magnetic Resonance Assessments of the Celermajer Index.

Can J Cardiol 2019 Dec 14;35(12):1834-1841. Epub 2019 Aug 14.

1st Department of Cardiology, Poznan University of Medical Sciences, Poznań, Poland; Cardiac Magnetic Resonance Unit at 1st Department of Cardiology, Poznan University of Medical Sciences, Poznań, Poland.

Background: The severity score of Ebstein anomaly (EA) that corresponds to clinical status is still under research, with the Celermajer index (Cel-ind) being one of those. The agreement between echocardiographic and cardiac magnetic resonance (CMR) assessment of Cel-ind is not known. We determined the agreement between echocardiography- and CMR-derived Cel-ind and its relationship with heart failure markers. Read More

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December 2019

Arrhythmias in Adults With Congenital Heart Disease: What the Practicing Cardiologist Needs to Know.

Paul Khairy

Can J Cardiol 2019 Dec 16;35(12):1698-1707. Epub 2019 Jul 16.

Electrophysiology Service and Adult Congenital Heart Disease Center, Montreal Heart Institute, Université de Montréal, Montreal, Québec, Canada. Electronic address:

The expanding population of adults with congenital heart disease (CHD) combined with the pervasiveness of arrhythmias has resulted in the rapid growth of a dedicated sector of cardiology at the intersection between 2 subspecialties: electrophysiology and adult CHD. Herein, practical considerations are offered regarding urgent referral for catheter ablation of atrial arrhythmias, anticoagulation, and primary prevention implantable cardioverter-defibrillators (ICDs). Patients with Ebstein anomaly and ventricular pre-excitation should be referred promptly due to the high prevalence of multiple accessory pathways and increased incidence of atrial tachyarrhythmias, which may be poorly tolerated. Read More

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December 2019