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ebsteins anomaly

Colleen M Carlston, Steven B Bleyl, Ashley Andrews, Lindsay Meyers, Sara Brown, Pinar Bayrak-Toydemir, James F Bale, Lorenzo D Botto
The NONO gene encodes a nuclear protein involved in RNA metabolism. Hemizygous loss-of-function NONO variants have been associated with syndromic intellectual disability and with left ventricular noncompaction (LVNC). A two-year-old boy presented to the University of Utah's Penelope Undiagnosed Disease Program with developmental delay, nonfamilial features, relative macrocephaly, and dilated cardiomyopathy with LVNC and Ebstein anomaly. Brain MRI showed a thick corpus callosum, mild Chiari I malformation, and a flattened pituitary...
February 17, 2019: American Journal of Medical Genetics. Part A
Aseem Ranjan Srivastava, Amol Bhoje, Shyamveer Khangraut, Shubhra Gupta
We describe a change in our technique for plication of the atrialised right ventricle during a Cone repair. The plication was performed from the 'Outside' -on a beating heart. Advantages that we expect are- all major coronaries are better seen when full of blood and can be avoided, if a major vessel is compromised - EKG changes should point it out immediately, there is a small but significant saving in terms of arrest time and if the plication needs to be taken down because of an adverse coronary event- this can be done without the need of another cardioplegia...
February 5, 2019: Seminars in Thoracic and Cardiovascular Surgery
Brandon D Morrical, Joseph A Dearani, Crystal R Bonnichsen, Nathaniel W Taggart
Repair of Ebstein anomaly has evolved over the last decade, and timing of repair remains variable. There have been no studies of exercise or functional capacity in patients who have had tricuspid valve surgery for Ebstein anomaly in adulthood. We aimed to compare exercise capacity before and after tricuspid valve repair or replacement for Ebstein anomaly in adults at Mayo Clinic. We performed a retrospective chart review of all patients with Ebstein anomaly who underwent tricuspid valve surgery at Mayo Clinic between June 2007 and January 2015...
January 30, 2019: Pediatric Cardiology
Guilherme Viotto, Leonardo Paim, Renato Souza, Joaquim Aprígio, Lucas Lacerda, Pablo Pomerantzeff, Marcelo B Jatene, José Honório Palma, Fabio B Jatene
OBJECTIVES: In this study, we sought to evaluate early outcomes of transcatheter valve-in-valve implantation in patients with a degenerated bioprosthesis in the tricuspid position. METHODS: We used a prospective study, which included patients with a degenerated bioprosthesis in the tricuspid position who were considered high-risk by our heart team and who underwent a valve-in-valve implantation. The procedures were performed via transjugular venous access and were done with the patient under general anaesthesia with transoesophageal echocardiographic and fluoroscopic guidance...
January 25, 2019: Interactive Cardiovascular and Thoracic Surgery
Costantina Prota, Giovanni Di Salvo, Jolanda Sabatino, Manjit Josen, Josefa Paredes, Domenico Sirico, Marisol Uy Pernia, Andreas Hoschtitzky, Guido Michielon, Rodolfo Citro, Alain Fraisse, Olivier Ghez
BACKGROUND: Accurate risk stratification of patients with Ebstein's anomaly (EA) is crucial. Aim of the study was to assess the prognostic value of echocardiography, including 2D speckle tracking (STE) derived myocardial deformation indices, for predicting outcome in pediatric and young adult unrepaired EA patients. METHODS: Fifty consecutive EA patients (1 day-18 years, 52% males) underwent echocardiography and were followed for a mean follow-up of 60 ± 41 months for clinical outcome (ventricular tachyarrhythmia, heart failure, need for surgery and/or death)...
March 1, 2019: International Journal of Cardiology
Fusahiko Ito, Masazumi Watanabe, Go Kataoka, Haruna Seki, Dai Tasaki, Masanori Hirota
We experienced an explantation of Wada-Cutter prosthetic tilting disk valve of 47 years after implantation. The patient was 53 years old female who underwent the 1st operation for Ebstein's anomaly, which included tricuspid valve replacement (TVR), closure of atrial septal defect (ASD) and posterior annulorrhaphy when she was 6 years old. She was doing well after the 1st operation without symptom,but dyspnea and systemic edema worsened recently though increased admission of diuretics. Echocardiography revealed severe tricuspid valve stenosis, severe tricuspid valve insufficiency, and increased right atrium volume...
December 2018: Kyobu Geka. the Japanese Journal of Thoracic Surgery
Kariem Mrad Agua, Melchior Burri, Julie Cleuziou, Elisabeth Beran, Christian Meierhofer, Nicole Nagdyman, Rüdiger Lange
OBJECTIVES: Postoperative right ventricular (RV) failure is a severe complication after tricuspid valve (TV) surgery in patients with Ebstein's anomaly. We investigated the preoperative predictability of postoperative mortality and morbidity by assessing the influence of age, RV size and RV function on in-hospital mortality and on the clinical course during the intensive care unit (ICU) stay. METHODS: We retrospectively analysed 189 patients who had undergone TV surgery for Ebstein's anomaly at our centre...
December 15, 2018: European Journal of Cardio-thoracic Surgery
Tobias Rutz, Andreas Kühn
No abstract text is available yet for this article.
December 6, 2018: International Journal of Cardiology
Jayshree V Gite, Gauri R Gangakhedkar, Manali Nadkarni
No abstract text is available yet for this article.
November 2018: Indian Journal of Anaesthesia
Edgar Francisco Carrizales-Sepúlveda, Raymundo Vera-Pineda, Ramiro Flores-Ramírez, Alejandro Ordaz-Farías
No abstract text is available yet for this article.
December 6, 2018: Internal and Emergency Medicine
Lin Liu, Hong-Dan Wang, Cun-Ying Cui, Hui-Mei Yao, Lei Huang, Tao Li, Tai-Bing Fan, Bang-Tian Peng, Lian-Zhong Zhang
The prognosis of right heart enlargement varies according to different etiologies. The purpose of this study was to investigate the characteristics of echocardiogram, surgical treatment, chromosome and prognosis for fetal right heart enlargement.The foetal echocardiogram was performed on 3987 pregnant women, and then 88 fetuses with right heart enlargement were identified. The data about prenatal and postnatal echocardiograms, postnatal cardiac surgical treatment, karyotype analysis and autopsy after induced labor were analyzed in the 88 fetuses...
November 2018: Medicine (Baltimore)
Eva Maria Delmo Walter, Mariano Francisco Del Maria Javier, Roland Hetzer
No abstract text is available yet for this article.
October 26, 2018: Journal of Thoracic and Cardiovascular Surgery
Rengarajan Rajagopal, Niraj Nirmal Pandey, Rishabh Khurana, Sanjeev Kumar
No abstract text is available yet for this article.
November 10, 2018: Journal of Cardiovascular Computed Tomography
Jun Zeng, Wei Wei
We presented a 39-year-old female patient with life-threatening hypoxemia after tricuspid valve replacement because of Ebstein's anomaly. And the severe cyanosis is due to bioprosthetic valve stenosis and atrial septal defect. Anesthetic management of a patient with severe obstructive prosthetic valve dysfunction can be challenging. Similar considerations should be given to patients with Ebstein's anomaly to maintain the pressure equalized between the right and left atrial. Transesophageal echocardiography and cerebral oxygen saturation provided real time information in perioperative care...
November 13, 2018: Revista Brasileira de Anestesiologia
Tsukasa Torigoe, Wadi Mawad, Mike Seed, Greg Ryan, Davide Marini, Fraser Golding, Tim van Mieghem, Edgar Jaeggi
A circular shunt (CS) is a life-threatening condition consisting of massive shunting of systemic arterial blood via the ductus arteriosus to the left ventricle without traversing the lungs. In the prenatal circulation, it mainly occurs in severe Ebstein anomaly (EA) from unrestricted ductal flow and significant pulmonary and tricuspid regurgitation. We aimed to improve the fetal hemodynamics and chances of survival of affected fetuses by inducing ductal constriction with transplacental non-steroidal anti-inflammatory drugs (NSAIDs)...
October 31, 2018: Ultrasound in Obstetrics & Gynecology
Moyu Hasegawa, Shigemitsu Iwai, Sanae Yamauchi, Yosuke Kugo, Futoshi Kayatani, Kunihiko Takahashi, Hiroaki Kawata
We present a case of critical Ebstein's anomaly with circular shunting, diagnosed in utero. The fetal cardiothoracic area ratio was elevated; tricuspid regurgitation and pulmonary regurgitation worsened with fetal hydrops. At 35 weeks and 6 days of gestation, elective caesarean section delivery was performed. Planned bilateral pulmonary artery banding and pulmonary trunk ligation were performed as a palliative operation 4 hours after birth, with the infant in a stable condition. At age 5 days, we performed Starnes operation...
October 22, 2018: Annals of Thoracic Surgery
Shankar Baskar, Haikun Bao, Karl E Minges, David S Spar, Richard J Czosek
Background Implantable cardioverter defibrillators (ICDs) are an important part of therapy for many patients, yet there is little data on population characteristics, complications, or system survival in pediatric patients. Methods A retrospective review of ICD recipients in the National Cardiovascular Data Registry ICD Registry was performed from 2010 to 2016. Patient characteristics and complications between pediatric (≤21 years) and adult populations (>21 years) were compared. Variables associated with complications and early device interventions within the pediatric cohort were evaluated using multivariate modeling...
September 2018: Circulation. Arrhythmia and Electrophysiology
Jeremy P Moore, Kevin M Shannon, Roberto G Gallotti, Christopher J McLeod, Anca Chiriac, Edward P Walsh, Narayanswami Sreeram, Akash R Patel, Natasja M De Groot, Johannes von Alvensleben, Seshadri Balaji, David S Frankel, Christina Y Miyake, James C Perry, Kalyanam Shivkumar
OBJECTIVES: The purpose of this study was to determine the ventricular arrhythmia (VA) substrates in patients with unoperated and post-surgical Ebstein's Anomaly (EA). BACKGROUND: EA is associated with variable atrialization of the right ventricle and a propensity for VA and sudden death. There are scant data on catheter ablation for VA in this population. METHODS: This was a retrospective study involving 11 congenital heart disease centers...
October 2018: JACC. Clinical Electrophysiology
Edward P Walsh
Ebstein's anomaly of the tricuspid valve is a relatively rare form of congenital heart disease that has long been a challenge to electrophysiologists and cardiac surgeons. In addition to the hemodynamic burden of the actual valve defect, Ebstein's patients must also contend with an extraordinarily high incidence of tachyarrhythmias, most of which can be attributed to accessory atrioventricular pathways (APs) located along the posterior and septal border of the tricuspid valve where the valve leaflets are most abnormal...
October 2018: JACC. Clinical Electrophysiology
Haleema Akhter Hashmi, Hafeeza Khatoon, Syed Ijlal Ahmed
Ebstein anomaly is a rare malformation of the tricuspid valve of the heart. The malformed tricuspid valve may be incompetent, stenotic, or rarely, imperforate. Pregnant patients with Ebstein anomaly become a challenge for the physician in terms of management. We report a case in which patient delivered successfully with Ebstein anomaly. The only complication was breathlessness. Authors concluded that women with Ebstein anomaly can have successful outcomes of pregnancy with close monitoring.
September 2018: JPMA. the Journal of the Pakistan Medical Association
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