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https://www.readbyqxmd.com/read/28435681/a-hol-e-y-predicament
#1
Shaiva Ginoya Meka, Daniel Shelden, Amy Mertens, Paul Christensen, Meet Patel
Endocardial cushion defects are congenital abnormalities that result in valvular dysfunction as well as defects (or "holes") in the septa of the heart. They are typically diagnosed in early infancy; presentation late in life is rare. We present the case of a 72-year-old female admitted to the hospital with dyspnoea and palpitations. She was found to have multifocal atrial tachycardia. She suffered cardiac arrest associated with refractory hypoxaemia that required mechanical ventilation and vasodilator therapy with inhaled nitric oxide...
July 2017: Respirology Case Reports
https://www.readbyqxmd.com/read/28431027/eisenmenger-syndrome-a-rare-malady-that-continues-to-fascinate
#2
David S Celermajer
No abstract text is available yet for this article.
April 18, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28430906/past-and-current-cause-specific-mortality-in-eisenmenger-syndrome
#3
Cristel M Sørensen Hjortshøj, Aleksander Kempny, Annette Schophuus Jensen, Keld Sørensen, Edit Nagy, Mikael Dellborg, Bengt Johansson, Virginija Rudiene, Gu Hong, Alexander R Opotowsky, Werner Budts, Barbara J Mulder, Lidia Tomkiewicz-Pajak, Michele D'Alto, Katja Prokšelj, Gerhard-Paul Diller, Konstantinos Dimopoulos, Mette-Elise Estensen, Henrik Holmstrøm, Maila Turanlahti, Ulf Thilén, Michael A Gatzoulis, Lars Søndergaard
Aims: Eisenmenger syndrome (ES) is associated with considerable morbidity and mortality. Therapeutic strategies have changed during the 2000s in conjunction with an emphasis on specialist follow-up. The aim of this study was to determine the cause-specific mortality in ES and evaluate any relevant changes between 1977 and 2015. Methods and results: This is a retrospective, descriptive multicentre study. A total of 1546 patients (mean age 38.7 ± 15.4 years; 36% male) from 13 countries were included...
April 18, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28424444/a-successful-cesarean-delivery-without-fetal-or-maternal-morbidity-in-an-eisenmenger-patient-with-cor-triatriatum-sinistrum-double-orifice-mitral-valve-large-ventricular-septal-defect-and-single-ventricle-who-was-under-long-term-bosentan-treatment
#4
Hacer Ceren Tokgöz, Cihangir Kaymaz, Nertila Poci, Özgür Yaşar Akbal, Selçuk Öztürk
Presently described is successful cesarean delivery in a pregnancy superimposed on long-term bosentan treatment in an Eisenmenger syndrome patient with cor triatriatum sinistrum, double-orifice mitral valve, and large ventricular septal defect resulting in single functioning ventricle with double outlets. Cesarean delivery was performed at 27th week of gestation without maternal or fetal morbidity. The infant had no congenital cardiovascular abnormality or any probable teratogenic effect of bosentan treatment during pregnancy...
March 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28396379/improving-survival-in-patients-with-eisenmenger-syndrome-are-we-any-closer
#5
EDITORIAL
Richard A Lange, M Elizabeth Brickner
No abstract text is available yet for this article.
April 11, 2017: Circulation
https://www.readbyqxmd.com/read/28348949/impact-of-advanced-medical-therapy-for-the-outcome-of-an-adult-patient-with-eisenmenger-syndrome
#6
Eglė Ereminienė, Marija Kinderytė, Skaidrius Miliauskas
Eisenmenger syndrome (ES) is the most severe form of pulmonary arterial hypertension (PAH) associated with congenital heart disease. It is an extremely devastating condition with a serious impact on patients' life. Classical therapy of ES remains directed to avoid complications, such as erythrocytosis, treatment of congestive heart failure, prevention of infection, and secondary haematological abnormalities such as iron deficiency and coagulation disorders. However, the only effective treatment is heart-lung transplantation; still, morbidity and mortality after transplantation remain substantially high...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28318876/effects-of-reduction-of-pressure-overload-on-right-ventricular-function-in-patients-with-eisenmenger-syndrome
#7
EDITORIAL
Kazufumi Nakamura, Toshihiro Sarashina, Kentaro Ejiri, Satoshi Akagi
No abstract text is available yet for this article.
May 2017: Journal of Cardiology
https://www.readbyqxmd.com/read/28318659/cardiovascular-and-general-health-status-of-adults-with-trisomy-21
#8
Samuel A Hayes, Shelby Kutty, Joshua Thomas, Joyce T Johnson, Anji T Yetman
BACKGROUND: Patients with Trisomy 21 are now living well into adulthood. Little data exists to assist the cardiologist in the care of these patients. We sought to examine the cardiac and general health status of adults with Trisomy 21 undergoing cardiac evaluation. METHODS & RESULTS: A retrospective review of all affected adults >21years followed at 2 tertiary care institutions was performed. Of 193 patients identified, median age was 31 (range 21.1-60.5) years...
March 10, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28286190/outcome-of-adults-with-eisenmenger-syndrome-treated-with-drugs-specific-to-pulmonary-arterial-hypertension-a-french-multicentre-study
#9
Sebastien Hascoet, Emmanuelle Fournier, Xavier Jaïs, Lauriane Le Gloan, Claire Dauphin, Ali Houeijeh, Francois Godart, Xavier Iriart, Adelaïde Richard, Jelena Radojevic, Pascal Amedro, Gilles Bosser, Nathalie Souletie, Yvette Bernard, Pamela Moceri, Hélène Bouvaist, Pierre Mauran, Elise Barre, Adeline Basquin, Clement Karsenty, Damien Bonnet, Laurence Iserin, Olivier Sitbon, Jérôme Petit, Elie Fadel, Marc Humbert, Magalie Ladouceur
BACKGROUND: The relationship between pulmonary arterial hypertension-specific drug therapy (PAH-SDT) and mortality in Eisenmenger syndrome (ES) is controversial. AIMS: To investigate outcomes in patients with ES, and their relationship with PAH-SDT. METHODS: Retrospective, observational, nationwide, multicentre cohort study. RESULTS: We included 340 patients with ES: genetic syndrome (n=119; 35.3%); pretricuspid defect (n=75; 22...
March 9, 2017: Archives of Cardiovascular Diseases
https://www.readbyqxmd.com/read/28237697/heart-transplantation-in-adults-with-congenital-heart-disease
#10
REVIEW
Lucile Houyel, Ngoc-Tram To-Dumortier, Yannick Lepers, Jérôme Petit, Régine Roussin, Mohamed Ly, Emmanuel Lebret, Elie Fadel, Jürgen Hörer, Sébastien Hascoët
With the advances in congenital cardiac surgery and postoperative care, an increasing number of children with complex congenital heart disease now reach adulthood. There are already more adults than children living with a congenital heart defect, including patients with complex congenital heart defects. Among these adults with congenital heart disease, a significant number will develop ventricular dysfunction over time. Heart failure accounts for 26-42% of deaths in adults with congenital heart defects. Heart transplantation, or heart-lung transplantation in Eisenmenger syndrome, then becomes the ultimate therapeutic possibility for these patients...
February 22, 2017: Archives of Cardiovascular Diseases
https://www.readbyqxmd.com/read/28237536/early-experience-of-macitentan-for-pulmonary-arterial-hypertension-in-adult-congenital-heart-disease
#11
S Herbert, W Gin-Sing, L Howard, R M R Tulloh
BACKGROUND: Endothelin receptor antagonists (ERA) have been recognised as effective therapy for pulmonary arterial hypertension in congenital heart disease (CHD-PH), and Eisenmenger syndrome (ES) since the Breathe 5 study. A new dual receptor antagonist - Macitentan - is currently undergoing trials to determine its efficacy in simple ES. To date there is little information on this therapy in CHD and we report our first experience, some with more complex diseases. METHODS: Data was collected prospectively from September 2014...
February 6, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28197278/patent-ductus-arteriosus-with-eisenmenger-syndrome-difficult-diagnosis-made-easily-with-saline-contrast-echocardiography
#12
Prakash Kumar, Santosh Kumar Sinha, Umeshwar Pandey, Ramesh Thakur, Chandra Mohan Varma, Mohit Sachan, Amit Goel
The diagnosis of patent ductus arteriosus (PDA) with Eisenmenger syndrome is difficult. We report a case of 35-year-old male who came to our outpatient department (OPD) for evaluation of repeated hemoptysis and dyspnea on exertion. He had already completed two courses of ATT. On examination, grade 3 ejection systolic murmur was audible over precordium. Transthoracic echocardiography (TTE) showed enlargement of right atrium (RA) and right ventricular (RV) with severe tricuspid regurgitation (TR). On agitated saline contrast injection, agitated saline was seen in pulmonary artery followed by filling of abdominal aorta without filling of ascending aorta, thus confirming the diagnosis of right to left shunt with PDA...
June 2016: Cardiology Research
https://www.readbyqxmd.com/read/28195885/eisenmenger-syndrome-and-pregnancy-novel-ecmo-configuration-as-a-bridge-to-delivery-and-recovery-utilizing-a-multidisciplinary-team
#13
Erika B Rosenzweig, Darryl Abrams, Mauer Biscotti, Diane Kerstein, Daphnie Drassinower, Daniel Brodie, Matthew Bacchetta
Pregnancy is typically contraindicated in Eisenmenger syndrome because of its association with excessively high maternal and fetal morbidity and mortality. We report on our novel approach to successfully managing a 29 year-old pregnant woman with an unrepaired patent ductus arteriosus (PDA) with Eisenmenger syndrome through delivery and recovery. Venovenous ECMO was utilized during induction of labor to stabilize maternal hemodynamics and optimize fetal oxygenation by creating an oxygenated right-to-left shunt through the PDA...
February 9, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/28168751/easily-measurable-noninvasive-and-novel-finding-for-pulmonary-hypertension-hypertrophy-of-the-basal-segment-of-septomarginal-trabeculation-of-right-ventricle
#14
Gultekin Karakus, Ertugrul Zencirci, Aleks Degirmencioglu, Tolga Sinan Güvenc, Hale Unal Aksu, Aydin Yildirim
BACKGROUND: Effect of pulmonary hypertension (PH) on right ventricular (RV) geometry constitutes an ideal target to assess both pulmonary artery pressure (PAP) and its physiological importance. In this study, we evaluated the diagnostic power of the basal segment of septomarginal trabeculation (SMT) in predicting the PH and RV hypertrophy by cardiovascular magnetic resonance (CMR) in patients with idiopathic pulmonary arterial hypertension (IPAH) and Eisenmenger's syndrome (ES). METHODS: Eleven patients with IPAH, seven patients with ES, and 20 healthy controls were enrolled...
February 2017: Echocardiography
https://www.readbyqxmd.com/read/28148318/utility-of-4d-flow-mapping-in-eisenmenger-syndrome-with-pulmonary-atresia
#15
Soha Romeih, Heba Aguib, Magdi Yacoub
Management of patients with Eisenmenger syndrome with pulmonary atresia is challenging because of the complexity of the structure-function relationship of the components of the syndrome. Multi-modality imaging including cardiac magnetic resonance (CMR) 4D Flow offers unprecedented opportunities to unravel, at least in part, some of these components, and thus help in the management of these patients. In this study, we describe the use of these integrated methods with particular reference to CMR 4D Flow in a patient with Eisenmenger syndrome and pulmonary atresia and outline both the utility and the limitations...
December 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/28110830/treatment-of-pulmonary-arterial-hypertension-in-eisenmenger-syndrome-practice-makes-almost-perfect
#16
EDITORIAL
Jean-Luc Vachiery
No abstract text is available yet for this article.
December 23, 2016: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/28066570/current-therapy-of-eisenmenger-syndrome
#17
EDITORIAL
Minsu Kim, Wook-Jin Chung
No abstract text is available yet for this article.
November 2016: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28027769/brain-metabolite-alterations-in-eisenmenger-syndrome-evaluation-with-mr-proton-spectroscopy
#18
Dilek Şen Dokumacı, Ferit Doğan, Ali Yıldırım, Fatıma Nurefşan Boyacı, Erol Bozdoğan, Bülent Koca
OBJECTIVE: Eisenmenger syndrome (ES) is a life-threatening disease characterized by pulmonary hypertension and cyanosis in patients with congenital heart diseases. The aim of this study was to determine the brain metabolite changes in Eisenmenger syndrome compared with a control group using MR proton spectroscopy. METHODS AND MATERIALS: The study included 10 children (3 male, 7 female) with congenital heart diseases and a diagnosis of Eisenmenger syndrome. The control group consisted of 10 healthy volunteer children...
January 2017: European Journal of Radiology
https://www.readbyqxmd.com/read/28011668/cardiac-remodelling-amongst-adults-with-various-aetiologies-of-pulmonary-arterial-hypertension-including-eisenmenger-syndrome-implications-on-survival-and-the-role-of-right-ventricular-transverse-strain
#19
Pamela Moceri, Priscille Bouvier, Delphine Baudouy, Konstantinos Dimopoulos, Pierre Cerboni, Stephen J Wort, Denis Doyen, Elie-Dan Schouver, Pierre Gibelin, Roxy Senior, Michael A Gatzoulis, Emile Ferrari, Wei Li
AIMS: Survival in pulmonary arterial hypertension (PAH) and Eisenmenger syndrome (ES) relates to right ventricular (RV) function. Little is known about differences of ventricular function between ES patients and those suffering from other PAH aetiologies. In this study, we compared global ventricular function assessed by speckle-tracking in adult patients with ES, other PAH aetiologies, or healthy controls; and assessed the relationship between ventricular function and survival. METHODS AND RESULTS: We performed a prospective cohort study recruiting 83 adult PAH patients (43 ES and 40 other PAH aetiologies patients) and 37 controls between March 2011 and June 2015...
December 23, 2016: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/27979875/predictors-of-death-in-contemporary-adult-patients-with-eisenmenger-syndrome-a-multicentre-study
#20
Aleksander Kempny, Cristel S Hjortshøj, Hong Gu, Wei Li, Alexander R Opotowsky, Michael Landzberg, Annette S Jensen, Lars Søndergaard, Mette-Elise Estensen, Ulf Thilén, Werner Budts, Barbara J Mulder, Ilja M Blok, Lidia Tomkiewicz-Pajak, Kamil Szostek, Michele D'Alto, Giancarlo Scognamiglio, Katja Prokšelj, Gerhard-Paul Diller, Konstantinos Dimopoulos, Stephen J Wort, Michael A Gatzoulis
BACKGROUND: -Eisenmenger syndrome (ES) is associated with substantial morbidity and mortality. There is no consensus, however, on mortality risk stratification. We aimed to investigate survival and predictors of death in a large, contemporary cohort of ES patients. METHODS: -We identified in a multicentre approach adults with ES under follow-up between 2000 and 2015. We examined survival and its association with clinical, electrocardiographic, echocardiographic and laboratory parameters...
December 15, 2016: Circulation
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