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'Ebstein Anomaly' (1969)


Jan
2018

Ebstein anomaly is a congenital disorder of right ventricular myocardial development, which affects the tricuspid valve in addition to the right ventricular myocardium. Cardiac imaging by transthoracic echocardiography and cardiac magnetic resonance imaging are the key modalities used to assess timing and type of surgery. In this article, we review the current standards of echocardiographic and magnetic resonance imaging in Ebstein anomaly.

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Feb
2018

We aimed to quantify atrial and ventricular myocardial deformation in Ebstein's Anomaly (EA) in a case-control study with cardiovascular magnetic resonance (CMR) feature tracking and to correlate changes in cardiac performance with the severity of disease and clinical heart failure parameters.
Atrial and ventricular deformation was measured using CMR feature tracking in 30 EA and 20 healthy control subjects. Atrial performance was characterized using longitudinal strain and strain rate parameters for reservoir function, conduit function and booster pump function.

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Dec
2017

To investigate the early and mid-term clinical outcomes of the modified cone reconstruction in the treatment of Ebstein's anomaly (EA) which provide appropriate surgical treatment for clinical and reduce the incidence of re-operation and valve replacement.
Clinical data of 18 consecutive patients with EA in our hospital between May 2008 and August 2015 were analyzed retrospectively. All patients were diagnosed by echocardiography.

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Dec
1969

Yuan SM. Congenital heart defects in Williams syndrome. Turk J Pediatr 2017; 59: 225-232.

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Dec
2017

With improved surgical techniques and medical management for patients with congenital heart diseases, more patients are living longer and well into adulthood. This improved survival comes with a price of increased morbidity, mainly secondary to increased risk of tachyarrhythmias. One of the major arrhythmias commonly encountered in this subset of cardiac patients is AF.

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Jan
2018


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Jan
2018

The increased incidence of preoperative and postoperative arrhythmia in Ebstein anomaly (EA) prompted some clinicians to perform an electrophysiology study (EPS) in all patients prior to surgery for EA. The cone repair (CR) is the current surgical option of choice for most young patients with EA but the effect of the CR on arrhythmia is not well established.
To assess the burden of arrhythmia in young patients after CR and to assess the utility of selective preoperative EPS.

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Jan
2018

Ventricular dysfunction or structural alteration of either ventricle is a well-established risk factor for sudden death (SD). Ebstein anomaly (EA) can present with both right and left heart abnormalities; however, predictors of SD have not been described. We therefore sought to characterize the incidence and risk factors of SD among a large cohort of patients with EA.

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Jan
2018

Ebstein anomaly is a complex, congenital heart defect that is associated with a variety of cardiac abnormalities. Studies found a similar sarcomere gene mutation in patients with Ebstein anomaly (EA) and patients with isolated left ventricular non-compaction (LVNC).
We aimed to show the prevalence of LVNC and its potential relationship with severe cardiac events (VT - ventricular tachycardia, cardiac arrest) in adult patients with EA.

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Dec
2017

Familial Ebstein anomaly is a rare form of congenital heart disease. We report 7 individuals among 2 generations of 1 family with Ebstein anomaly. This family was first reported in 1991 by Balaji et al in which family members were also reported to have a mild skeletal phenotype.

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Dec
2017

Disease progression and heart failure development in Ebstein's Anomaly (EA) of the tricuspid valve is characterized by both right and left ventricular (LV) deterioration. The mechanisms underlying LV dysfunction and their role in heart failure development are incompletely understood. We hypothesized that LV dyssynchrony and impaired torsion and recoil mechanics induced by paradoxical movement of the basal septum may play a role in heart failure development.

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Dec
1969

Ebstein anomaly (EA) is a rare congenital defect characterized by apical displacement of the septal tricuspid leaflets and atrialization of the right ventricle. The etiology of EA is unclear; however, recurrence in families and the association of EA with genetic syndromes and copy number variants (CNVs) suggest a genetic component.
We performed a population-based study to search for recurrent and novel CNVs in a previously unreported set of EA cases.

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Jan
2018

Ebstein anomaly has heterogeneous anatomy and numerous operative techniques are described. Cone repair provides a near anatomic tricuspid valve repair. The purpose of this study was to examine our experience with cone repair.

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Nov
2017

This study aimed to determine factors associated with the need for extracorporeal membrane oxygenation (ECMO) in children with congenital heart disease (CHD) during admission for cardiac surgery (CS). A secondary aim was to determine how ECMO impacted length, cost, and mortality of the admission.
Data from the Kids' Inpatient Database (KIDS) were utilized.

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Jan
2018

We sought to describe the acute results and short- to medium-term durability of transcatheter tricuspid valve-in-valve (TVIV) implantation within surgical bioprostheses among patients with Ebstein anomaly (EA). Cases were identified from a voluntary, multicenter, international registry of 29 institutions that perform TVIV. Demographic, clinical, procedural, and follow-up data were analyzed.

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Nov
2017

In 2013, a prospective registry for adults with congenital heart disease (CHD) was established in Switzerland, providing detailed data on disease characteristics and outcomes: Swiss Adult Congenital HEart disease Registry (SACHER). Its aim is to improve the knowledge base of outcomes in adults with CHD. The registry design and baseline patient characteristics are reported.

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Oct
2017

Extracorporeal membrane oxygenation (ECMO) can be used as rescue intervention for cardiac and/or respiratory failure. High-risk adult patients with adult congenital heart disease (ACHD) may require pre- and post-operative ECMO support.
Retrospective data collection within a five-year time period from 2011 to 2016, at a single-centre study at a tertiary university hospital and regional unit for ACHD.

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Jan
2018

Left ventricular non-compaction (cardiomyopathy) (LVN(C)) is a rare hereditary cardiac condition, resulting from abnormal embryonic myocardial development. While it mostly occurs as an isolated condition, association with other cardiovascular manifestations such as Ebstein anomaly (EA) has been reported. This congenital heart defect is characterized by downward displacement of the tricuspid valve and leads to diminished ventricular size and function.

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Oct
2017

We report a case of a 7-year-old girl with atriofascicular Mahaim (AFM) pathway concomitant with Ebstein's anomaly. The QRS wave showed left bundle branch block pattern on electrocardiogram. Holter electrocardiogram showed prolongation of the PR interval and QRS morphological change during sinus tachycardia.

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Oct
2017

Ebstein's anomaly is a rare congenital heart disease characterized by apical displacement of the septal and posterior tricuspid valve leaflets. It is often complicated with left ventricular (LV) dysfunction as well as right-sided abnormalities. On the other hand, in the presence of LV dysfunction, right ventricular pacing is likely to aggravate the diseased LV function, which is termed pacemaker-induced cardiomyopathy.

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Oct
2017

Neonatal Ebstein Anomaly.

Semin Thorac Cardiovasc Surg 2017 Autumn;29(3):331-337. Epub 2017 Sep 25.
T K Susheel Kumar, Umar S Boston, Christopher J Knott-Craig

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Sep
2017

Ebstein Anomaly and Sudden Childhood Death.

J Forensic Sci 2017 Sep 20. Epub 2017 Sep 20.
Amanda Freeman, Roger W Byard
A 13-year-old girl is reported who died suddenly and unexpectedly in her sleep from previously undiagnosed Ebstein anomaly. At autopsy, there was dilatation of the right atrium with marked dilatation of the right auricle and apical displacement of the tricuspid valve into the right ventricular cavity with atrialization of the upper portion of the right ventricle. There were also prominent dysplastic changes in both the septal and posterior leaflets of the tricuspid valve with thickening of the valve and fusion of leaflets to the wall of the ventricle.

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Sep
2017

Perinatal mortality remains high among fetuses diagnosed with Ebstein's anomaly of the tricuspid valve. The subgroup of patients with pulmonary valve regurgitation is at particularly high risk. In the setting of pulmonary valve regurgitation, early constriction of the ductus arteriosus may be a novel perinatal management strategy to reduce systemic steal resulting from circular shunt physiology.

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Dec
1969

Management of Neonatal Ebstein Anomaly: To Thine Own Self Be True.

Semin Thorac Cardiovasc Surg 2017 19;29(2):213-214. Epub 2017 Apr 19.
David M Overman

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Dec
1969

This study aimed to review our 30-year, single-center experience of neonates admitted with Ebstein anomaly. Between January 1985 and August 2015, 80 neonates with Ebstein anomaly were managed. The primary outcome measures were early and late survival, freedom from reoperation, and functional status.

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Jun
2017

The Two Anomalies of Wilhelm Ebstein.

Tex Heart Inst J 2017 Jun 1;44(3):198-201. Epub 2017 Jun 1.
Magdalena Mazurak, Jacek Kusa
The year 2016 was the 180th anniversary of Wilhelm Ebstein's birth and the 150th anniversary of his description of the congenital tricuspid valve malformation that came to be known as Ebstein anomaly. We present a brief history of the life and work of Dr. Wilhelm Ebstein.

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Jun
2017

Ebstein anomaly is a rare congenital heart disease that has been described in domestic dogs, a meerkat, a pygmy goat, and a lion. An 11-mo-old Tsushima leopard cat presented to Osaka Prefecture University Veterinary Hospital for diagnosis and treatment of right-sided congestive heart failure. Echocardiography showed a dilated right atrium and ventricle with an enlarged tricuspid valve annulus and apical displacement of the tricuspid valve leaflets.

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Jan
2018

 Maternal hyperoxygenation (MH) during fetal ultrasound can characterize fetal pulmonary vasoreactivity (PVr) and its associations with postnatal physiology.
 We explored MH testing to facilitate perinatal risk stratification for fetuses with congenital heart disease (CHD).
 MH was performed in 12 fetuses: 2 with Ebstein anomaly, 2 with total anomalous pulmonary venous connection (TAPVC), 4 with hypoplastic left heart syndrome (HLHS) with (a) restrictive atrial septum (RAS) or (b) intact atrial septum (IAS) with decompressing vertical vein (VV), and 4 with D-loop transposition of the great arteries (TGA).

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May
2017

Management of Ebstein's anomaly.

Ann Cardiothorac Surg 2017 May;6(3):266-269
Irving L Kron, Mark Elliot Roeser
Ebstein malformation is a rare disease that presents with a spectrum of severity. We use a management paradigm that breaks the anomaly into two groups: neonates and children/adults. This leads to a discussion of management and operative techniques as well as pitfalls.

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Aug
2017

Objective Only limited clinical and electrophysiological data concerning patients (pts) with multiple accessory pathways (MAP) in comparison to large control groups are available. The aim of our study was to analyse these data from the largest cohort of patients with multiple accessory pathways and a large control group. Method and results We analysed data from pts with MAP (group 1) and pts with a single accessory pathway (AP) (group 2) referred for radiofrequency catheter ablation (RFCA) at our tertiary centre.

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Dec
1969

Patients with Ebstein's anomaly of the tricuspid valve (EA) are at risk of tachyarrhythmia, congestive heart failure and sudden cardiac death. We sought to determine the value of cardiovascular magnetic resonance (CMR) for predicting these outcomes.
Seventy-nine consecutive adult patients (aged 37±15 years) with unrepaired EA underwent CMR and were followed prospectively for a median 3.

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Dec
1969

Ebstein's anomaly is the most commonly occurring congenital defect associated with the Wolff-Parkinson-White syndrome. In patients with Ebstein's anomaly and supraventricular tachycardia, the absence of right bundle branch block (RBBB) in sinus rhythm is a highly sensitive and specific indicator of the presence of an ipsilateral accessory AP.
We present an electrophysiology case of a patient with Ebstein's anomaly and 3 ipsilateral accessory pathways.

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Jul
2017

Ebstein's anomaly in combination with tetralogy of Fallot (TOF) is rare. We report a male infant with these combined anomalies who presented with severe cyanosis, heart failure, and respiratory distress after birth. Biventricular repair was successfully performed with 1-stage correction of his ventricular septal defect (VSD), right ventricular outflow tract (RVOT) obstruction, and tricuspid regurgitation.

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May
2017

Ebstein's anomaly is a complex malformation. Justification of a procedure in mildly symptomatic adults is debatable: repair techniques are demanding and valve replacement is associated with poorer outcome.
We report our initial experience with the cone procedure versus medical follow-up.

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Jan
2017

Ebstein's anomaly is defined as the significant apical displacement of the tricuspid valve causing tricuspid regurgitation. Although a variety of concomitant lesions have been previously described, we herein introduce an unusual presentation. Our patient was an 86-year-old man with a primary presentation of typical chest pain in the setting of recently diagnosed coronary artery disease with concomitant Ebstein's anomaly.

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Jun
2017

Patients with repaired or unrepaired congenital heart anomalies are at increased risk for arrhythmia development throughout their lives, often paralleling the need for reoperations for hemodynamic residua. The ability to incorporate arrhythmia surgery into reoperations can result in improvement in functional class and decreased need for antiarrhythmic medications. Every reoperation for congenital heart disease can be viewed as an opportunity to assess the electrical and arrhythmia substrates and to intervene to improve the arrhythmias and the hemodynamic condition of the patient.

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Jun
2017

Ebstein Anomaly.

Card Electrophysiol Clin 2017 Jun;9(2):245-254
Elizabeth D Sherwin, Dominic J Abrams
Ebstein anomaly is a rare form of congenital heart disease with a uniquely high prevalence of arrhythmias. The most prevalent arrhythmia mechanisms are intrinsic to the underlying embryologic defects and may manifest at any stage. Current electrophysiological and surgical strategies are well equipped to address these arrhythmia mechanisms, yet despite available technology and a robust understanding of the mechanisms, these cases remain challenging.

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Sep
2017

Neonates with severe forms of Ebstein anomaly present a surgical challenge, and the Starnes operation as single ventricle palliation is highly advocated. Cone reconstruction for tricuspid valvuloplasty (TVP) has become a widely accepted technique, although very few cases of TVP have been reported in neonates. This report describes a surgical strategy for neonatal Ebstein anomaly, with an aim toward biventricular repair.

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Apr
2017

A 2-year-old boy presented with severe hypotension and acute kidney injury after a prodrome of non-bloody diarrhoea and fever in the preceding 3 days. He had a mild Ebstein cardiac anomaly but otherwise a normal past history and growth. On examination, he looked ill, his temperature was 37.

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Jun
2017

In severe right heart obstruction (RHO), redistribution of cardiac output to the left ventricle (LV) is well tolerated by the fetal circulation. Although the same should be true of severely regurgitant tricuspid valve disease (rTVD) with reduced or no output from the right ventricle, affected fetuses more frequently develop hydrops or suffer intrauterine demise. We hypothesized that right atrium (RA) function is altered in rTVD but not in RHO, which could contribute to differences in outcomes.

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Apr
2017

The aim of this study was to clarify the feasibility of myocardial strain using cardiovascular magnetic resonance (CMR) for the evaluation of left ventricular (LV) deformation in patients with Ebstein's anomaly (EA).
We recruited 32 patients with EA and 30 controls for CMR examination and measured LV function, dimension and tissue tracking parameters (the global and regional radial, circumferential and longitudinal peak strain), together with the right ventricle (RV) dimension. LV strain parameters were compared among the controls, patients with preserved LV ejection fraction (LVEF; ≥55%), and patients with reduced LVEF (<55%).

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Feb
2017

The cone reconstruction technique, first described by da Silva and modified by Dearani and by others, has become the repair method of choice in patients with Ebstein anomaly of the tricuspid valve. This report details the outcome of the modified cone reconstruction technique in 6 children who underwent surgical correction of Ebstein anomaly at the Tomsk Institute of Cardiology in Siberia. From 2012 through 2015, 4 boys and 2 girls (age range, 11 mo-12 yr) underwent surgery to correct Ebstein anomaly.

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Dec
1969

The purpose of our study is (1) to characterise patients with congenital heart disease undergoing heart transplantation by adult cardiac surgeons in a large academic medical centre and (2) to describe successful outcomes associated with our multidisciplinary approach to the evaluation and treatment of adults with congenital heart disease (ACHD) undergoing orthotopic heart transplantation (OHT).
Heart failure is the leading cause of death in patients with ACHD leading to increasing referrals for OHT.
The Penn Congenital Transplant Database comprises a cohort of patients with ACHD who underwent OHT between March 2010 and April 2016.

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Dec
1969

Ebstein Anomaly With QRS Fragmentation on Electrocardiogram.

J Investig Med High Impact Case Rep 2017 Jan-Mar;5(1):2324709616688710. Epub 2017 Jan 1.
Prakash Acharya, Jonathan Ross Ang, Bernard Gitler
Ebstein anomaly is a rare congenital disorder that involves the tricuspid valve and the right ventricle. It is associated with interatrial communication, which allows for paradoxical embolization causing unilateral blindness. Abnormal conduction through the atrialized right ventricle leads to QRS fragmentation on electrocardiogram.

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Dec
1969

Ebstein's anomaly is a congenital heart defect rarely revealed by fetal-placental anasarca. Our study reports an original case of Ebstein's anomaly diagnosed during fetal-placental anasarca assessment, revealed by antenatal ultrasound, objectifying hydramnios, ascites and pericardial effusion. Echocardiography allowed the identification of Ebstein's disease with significant tricuspid insufficiency, mitral regurgitation (grade 3) and patent ductus arteriosus.

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Feb
2017

Ebstein's anomaly is a benign and stable congenital heart disease for asymptomatic patients. Despite a low incidence of Ebstein's anomaly (EA), patients' quality of life can be badly affected by EA without positive surgical intervention. Especially EA is associated with other congenital heart disease, such as the atrial septal defect, patent foramen ovale, and arterial embolism exclude other reasons, it is often considered to be the consequence of paradoxical embolism, and surgical intervention must be conducted.

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Oct
2016

A 27-year-old primiparous woman with 28 weeks gestational age was admitted to our hospital with worsening shortness of breath. She was diagnosed with Ebstein's anomaly three years ago, but preferred to be left untreated. The patient was not cyanotic and her vital signs were stable.

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