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Ebstein Malformation Surgery

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Examination of pathologic features of the right atrioventricular groove in hearts with Ebstein anomaly and correlation with arrhythmias.

Luciana Marcondes, Stephen P Sanders, Pedro J Del Nido, Edward P Walsh,

BACKGROUND:Catheter ablation of accessory pathways (APs) in patients with Ebstein anomaly (EA) has a higher recurrence rate than in subjects with normal hearts. Anatomic features could account for suboptimal ablation outcomes. OBJECTIVE:The purpose of this study was to examine the right atrioventricular (AV) groove in autopsy hearts with EA, correlate ... Read more >>

Heart Rhythm (Heart rhythm)
[2020, :]

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

Risa Shimbori, Jun Takaki, Yasuhito Hosoda, Hideaki Hidaka, Ken Okamoto, Toshihiro Fukui,

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 ... Read more >>

J Card Surg (Journal of cardiac surgery)
[2020, 35(3):675-678]

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Which one predominates in Ebstein anomaly: tricuspid regurgitation or right ventricular dysfunction? (Reply).

Kimberly A Holst, M Yasir Qureshi, Joseph A Dearani,

Ann. Thorac. Surg. (The Annals of thoracic surgery)
[2020, :]

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Novel KLHL26 variant associated with a familial case of Ebstein's anomaly and left ventricular noncompaction.

Sai Suma K Samudrala, Lauren M North, Karl D Stamm, Michael G Earing, Michele A Frommelt, Richard Willes, Swarnendu Tripathi, Nikita R Dsouza, Michael T Zimmermann, Donna K Mahnke, Huan Ling Liang, Michael Lund, Chien-Wei Lin, Gabrielle C Geddes, Michael E Mitchell, Aoy Tomita-Mitchell,

BACKGROUND:Ebstein's anomaly (EA) is a rare congenital heart disease of the tricuspid valve and right ventricle. Patients with EA often manifest with left ventricular noncompaction (LVNC), a cardiomyopathy. Despite implication of cardiac sarcomere genes in some cases, very little is understood regarding the genetic etiology of EA/LVNC. Our study describes ... Read more >>

Mol Genet Genomic Med (Molecular genetics & genomic medicine)
[2020, :e1152]

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Left Ventricular Noncompaction and Congenital Heart Disease Increases the Risk of Congestive Heart Failure.

Keiichi Hirono, Yukiko Hata, Nariaki Miyao, Mako Okabe, Shinya Takarada, Hideyuki Nakaoka, Keijiro Ibuki, Sayaka Ozawa, Naoki Yoshimura, Naoki Nishida, Fukiko Ichida, Lvnc Study Collaborators,

BACKGROUND:Left ventricular noncompaction (LVNC) is a hereditary cardiomyopathy that is associated with high morbidity and mortality rates. Recently, LVNC was classified into several phenotypes including congenital heart disease (CHD). However, although LVNC and CHD are frequently observed, the role and clinical significance of genetics in these cardiomyopathies has not been ... Read more >>

J Clin Med (Journal of clinical medicine)
[2020, 9(3):]

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Mortality burden in patients born with Ebstein's anomaly: a 40-year nationwide cohort study.

Filip Eckerström, Peter Eriksson, Mikael Dellborg, Georgios Lappas, Annika Rosengren, Vibeke Elisabeth Hjortdal, Zacharias Mandalenakis,

AIMS:Survival rates for unoperated patients with Ebstein's anomaly (EA) are unknown. We estimated overall long-term mortality in operated and unoperated EA patients, compared with the general population in Sweden. METHODS AND RESULTS:Using national medical registries, Swedish individuals born 1970-1993 and diagnosed with EA between 1970 and 2011 were included. The ... Read more >>

Eur Heart J Qual Care Clin Outcomes (European heart journal. Quality of care & clinical outcomes)
[2020, :]

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Pseudopolycystic Atrialized Right Ventricle in Ebstein's Anomaly.

Thomas Martens, Kristof Vandekerckhove, Katrien François, Thierry Bové,

World J Pediatr Congenit Heart Surg (World journal for pediatric & congenital heart surgery)
[2020, 11(1):110-111]

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

Mehmet Salih Bilal, Arda Özyüksel, Mustafa Kemal Avşar, Özgür Yıldırım,

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 ... Read more >>

Turk Gogus Kalp Damar Cerrahisi Derg (Turk gogus kalp damar cerrahisi dergisi)
[2020, 28(1):197-200]

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Multimodality imaging of an asymptomatic giant right atrial appendage aneurysm.

Fatih Uzun, Ahmet Güner, Serkan Kahraman, Ali Kemal Kalkan, Ezgi Gültekin Güner, Mehmet Ertürk,

Right atrial appendage (RAA) aneurysm is a rare congenital cardiac abnormality thought to be caused by dysplasia of the muscular wall of the RA and RAA. Structural, contractile, or electrical atrial changes are associated with the onset and progression of atrial fibrillation. The RAA aneurysm can have various symptoms, such ... Read more >>

Echocardiography (Echocardiography (Mount Kisco, N.Y.))
[2020, 37(2):347-350]

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Early and Long-Term Outcomes of Surgical Treatment of Ebstein's Anomaly.

Guilherme Viotto Rodrigues da Silva, Leonardo Augusto Miana, Luiz Fernando Caneo, Aída Luiza Ribeiro Turquetto, Carla Tanamati, Juliano G Penha, Fabio B Jatene, Marcelo B Jatene,

OBJECTIVE:This study aimed to evaluate Ebstein's anomaly surgical correction and its early and long-term outcomes. METHODS:A retrospective analysis of 62 consecutive patients who underwent surgical repair of Ebstein's anomaly in our institution from January 2000 to July 2016. The following long-term outcomes were evaluated: survival, reoperations, tricuspid regurgitation, and postoperative ... Read more >>

Braz J Cardiovasc Surg (Brazilian journal of cardiovascular surgery)
[2019, 34(5):511-516]

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Successful catheter ablation for ventricular tachycardia after cone procedure in Ebstein anomaly.

Kyung Jin Ahn, Mi Kyoung Song, Gi Beom Kim, Woong Han Kim, Eun Jung Bae,

HeartRhythm Case Rep (HeartRhythm case reports)
[2020, 6(2):106-109]

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Indian Guidelines for Indications and Timing of Intervention for Common Congenital Heart Diseases: Revised and Updated Consensus Statement of the Working Group on Management of Congenital Heart Diseases. Abridged Secondary Publication.

Anita Saxena, Jay Relan, Ravi Agarwal, Neeraj Awasthy, Sushil Azad, Manisha Chakrabarty, Kulbhushan S Dagar, Velayoudam Devagourou, Baiju S Dharan, Saurabh K Gupta, Krishna S Iyer, M Jayranganath, Raja Joshi, B R J Kannan, Ashish Katewa, Vikas Kohli, Nageswara Rao Koneti, Shyam S Kothari, K M Krishnamoorthy, Snehal Kulkarni, Rohit Manoj Kumar, Raman Krishna Kumar, Sunita Maheshwari, Krishna Manohar, Ashutosh Marwah, Smita Mishra, Smruti R Mohanty, Kona Samba Murthy, P V Suresh, S Radhakrishnan, Palleti Rajashekar, Sivasubramanian Ramakrishnan, Nitin Rao, Suresh G Rao, Chinnaswamy Hm Reddy, Rajesh Sharma, Krishnanaik Shivaprakasha, Raghavan Subramanyan, R Suresh Kumar, Sachin Talwar, Munesh Tomar, Sudeep Verma, Vijayakumar Raju, ,

JUSTIFICATION:A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However, these guidelines are for patients living in high income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for congenital heart diseases, as often these patients present late ... Read more >>

Indian Pediatr (Indian pediatrics)
[2020, 57(2):143-157]

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Electrocardiography findings and clinical presentation in Ebstein's anomaly.

Mayank Dalakoti, Devinder Singh, Wee Tiong Yeo, Lik Wui Edgar Tay, Kian Keong Poh,

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. Patients may first ... Read more >>

Singapore Med J (Singapore medical journal)
[2019, 60(11):560-564]

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Cone reconstruction is feasible in babies under 2 kg of weight.

Carles Bautista-Rodriguez, Alain Fraisse, Margarita Burmester, Oliver Ghez,

Symptomatic neonates with Ebstein anomaly present with intractable heart failure. We present the first successful cone reconstruction in an ex 30 + 4-week premature below 2 kg with a favourable outcome. ... Read more >>

Interact Cardiovasc Thorac Surg (Interactive cardiovascular and thoracic surgery)
[2019, 29(4):652-653]

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Which one predominates in Ebstein anomaly: tricuspid regurgitation or right ventricular dysfunction?

Ismail Yurekli, Mert Kestelli, Habib Cakir,

Ann. Thorac. Surg. (The Annals of thoracic surgery)
[2019, :]

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Is Carotid Intima-Media Thickness Increased in Adults With Congenital Heart Disease?

Barbara Reiner, Renate Oberhoffer, Anna-Luisa Häcker, Peter Ewert, Jan Müller,

Background Because of the increasing numbers of congenital patients surviving into adulthood, early diagnosis and prevention of acquired cardiovascular disease is reasonable. The aim of this study was to detect diagnostic subgroups of adults with congenital heart disease (ACHD) that have increased carotid intima-media thickness (cIMT), a subclinical marker of ... Read more >>

J Am Heart Assoc (Journal of the American Heart Association)
[2020, 9(3):e013536]

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Cerebrovascular accidents in Ebstein's anomaly.

Nicholas Y Tan, Christine H Attenhofer Jost, Murray D Polkinghorne, Emily R Vargas, David O Hodge, Joseph A Dearani, Samuel J Asirvatham, Heidi M Connolly, Christopher J McLeod,

INTRODUCTION:Mechanisms and risk factors for cerebrovascular accidents (CVAs) in Ebstein's anomaly (EA) are not well understood; hence, we aimed to clarify these in a large cohort of EA patients. METHODS:Patients with a confirmed diagnosis of EA were retrospectively reviewed. Baseline characteristics were compared between patients with and without a prior ... Read more >>

Congenit Heart Dis (Congenital heart disease)
[2019, 14(6):1157-1165]

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Results for tricuspid valve surgery in adults with congenital heart disease other than Ebstein's anomaly†.

Mauro Lo Rito, Maria Grandinetti, Giulia Muzio, Alessandro Varrica, Alessandro Frigiola, Angelo Micheletti, Massimo Chessa, Alessandro Giamberti,

OBJECTIVES:Tricuspid valve (TV) surgery in the adult with congenital heart disease (ACHD) is a frequently performed procedure. The aim of this study was to analyse postoperative and medium-term outcomes. METHODS:We conducted a single-centre retrospective study of patients with ACHD who underwent TV surgery (January 2000-December 2016); patients with Ebstein's anomalies ... Read more >>

Eur J Cardiothorac Surg (European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery)
[2019, 56(4):706-713]

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Arrhythmia Mechanisms and Outcomes of Ablation in Pediatric Patients With Congenital Heart Disease.

Charlotte A Houck, Stephanie F Chandler, Ad J J C Bogers, John K Triedman, Edward P Walsh, Natasja M S de Groot, Dominic J Abrams,

BACKGROUND:In contrast to the adult population with congenital heart disease (CHD), arrhythmia mechanisms and outcomes of ablation in pediatric patients with CHD in recent era have not been studied in detail. Aims of this study were to determine arrhythmia mechanisms and to evaluate procedural and long-term outcomes in pediatric patients ... Read more >>

Circ Arrhythm Electrophysiol (Circulation. Arrhythmia and electrophysiology)
[2019, 12(11):e007663]

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Surgical Repair of Ebstein's Anomaly Using a Bicuspidization Approach.

Richard D Mainwaring, Tatiana R Rosenblatt, George K Lui, Michael Ma, Frank L Hanley,

BACKGROUND:Ebstein's anomaly of the tricuspid valve is a rare and heterogeneous form of congenital heart defect. The purpose of this study was to review the surgical experience with Ebstein's anomaly at a single institution. METHODS:This was a retrospective review of 47 patients with Ebstein's anomaly who were previously unrepaired and ... Read more >>

Ann. Thorac. Surg. (The Annals of thoracic surgery)
[2019, 108(6):1875-1882]

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Outcome of Care Provided in Neonatal Surgery Intensive Care Unit of a Public Sector Tertiary Care Teaching Hospital of India.

Vijaydeep Siddharth, Shakti Kumar Gupta, Sandeep Agarwala, Sidhartha Satpathy, Prabudh Goel,

Aims:There is limited literature on the outcome of care in intensive care units (ICUs), especially when it comes to neonatal surgical units. Hence, this study was aimed to observe the outcome of care provided in the neonatal surgery ICU (NSICU) at an apex tertiary care teaching institute of North India. ... Read more >>

J Indian Assoc Pediatr Surg (Journal of Indian Association of Pediatric Surgeons)
[2019, 24(4):257-263]

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Cone Type Repair Has Become Our First Option in the Treatment of Adult Ebstein Anomaly.

Baburhan Ozbek, Nursen Tanrikulu,

PURPOSE:Different surgical procedures were defined due to degree of Ebstein anomaly. In this study, we are reporting our surgical experience of adult patients with Ebstein anomaly. METHODS:We analyzed the patients, who had operated with the diagnosis of Ebstein anomaly from March 2011 through February 2018, retrospectively. We evaluated patients in ... Read more >>

Ann Thorac Cardiovasc Surg (Annals of thoracic and cardiovascular surgery : official journal of the Association of Thoracic and Cardiovascular Surgeons of Asia)
[2019, 25(5):260-264]

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Postoperative persistent diastolic dyssynchronous expansion in patients with Ebstein's anomaly.

Kyung-Jin Kim, Kyung-Hwan Kim, Woong-Han Kim, Dae-Won Sohn,

In Ebstein's anomaly, maximal expansion in the atrialized right ventricle (RV) occurs during early diastole, whereas that of the functional RV occurs in late diastole, resulting in diastolic dyssynchronous expansion (DSE). We quantitatively assessed DSE and identified preoperative factors correlated with persistent DSE after surgery. Seventeen patients diagnosed with Ebstein's ... Read more >>

PLoS ONE (PloS one)
[2019, 14(8):e0220890]

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Radiofrequency catheter ablation of accessory pathways at the site of prior valve surgery.

Jae-Sun Uhm, Jun Kim, Moo-Nyun Jin, In-Soo Kim, Min Soo Cho, Pil-Sung Yang, Hee Tae Yu, Tae-Hoon Kim, Boyoung Joung, Hui-Nam Pak, Gi-Byoung Nam, Kee-Joon Choi, You-Ho Kim, Chun Hwang, Moon-Hyoung Lee,

Background:Radiofrequency catheter ablation (RFCA) for accessory pathways (APs) at the site of prior valve surgery (VS) remains challenging. We aimed to clarify the factors associated with successful RFCA for such APs. Methods:Upon reviewing a RFCA registry and previous case reports, we included nine patients who underwent RFCA of APs at ... Read more >>

J Arrhythm (Journal of arrhythmia)
[2019, 35(4):645-653]

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Utility of three-dimensional echocardiography and magnetic resonance imaging in the diagnosis of double-orifice tricuspid valve.

Sandeep Mohanty, Sreeja Pavithran, Ravi Agarwal, Kothandam Sivakumar,

Duplication of atrioventricular valves involves the mitral valve more often than the tricuspid valve and is often associated with other cardiac defects. Double-orifice tricuspid valve (DOTV) is often identified in surgery or autopsy and missed on echocardiography, as the two orifices are orthogonal to the imaging plane. If suspected on ... Read more >>

Ann Pediatr Cardiol (Annals of pediatric cardiology)
[2019, 12(3):339-341]

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