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Eisenmenger syndrome

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https://www.readbyqxmd.com/read/28882496/the-echocardiographic-characteristics-and-prognostic-significance-of-pericardial-effusions-in-eisenmenger-syndrome
#1
Clare Arnott, Rachael Cordina, David S Celermajer
BACKGROUND AND OBJECTIVES: Pericardial effusion (PE) confers a poor prognosis in non-congenital pulmonary arterial hypertension (PAH). The clinical significance and prognostic implications of PE in Eisenmenger syndrome (ES) is much less well characterised. Accordingly, we aimed to define the frequency, echocardiographic characteristics, natural history and clinical sequelae of PE in those with ES. PATIENTS AND METHODS: We retrospectively studied 55 consecutive individuals presenting since 2000, age >18 years, with ES...
June 15, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28874283/mortality-in-adults-with-congenital-heart-disease
#2
Pavithra Naidu, Leeanne Grigg, Dominica Zentner
AIM: Retrospective ascertainment of the causes of mortality in the adult congenital heart disease (ACHD) cohort of the Royal Melbourne Hospital (RMH). METHODS: Deceased patients (n=73) of the 2519 ACHD patients in the Royal Melbourne Hospital registry (commenced in 1991) were identified. Retrospective analysis was undertaken. Age, gender of deceased individuals, and frequency and cause of death in different congenital diagnosis groups was explored. RESULTS: Between 1991 and 2015, death occurred in 3...
October 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28794135/pulmonary-vasodilator-therapy-is-associated-with-greater-survival-in-eisenmenger-syndrome
#3
Clare Arnott, Geoff Strange, Andrew Bullock, Adrienne C Kirby, Clare O'Donnell, Dorothy J Radford, Leeanne E Grigg, David S Celermajer
OBJECTIVE: Eisenmenger syndrome (ES) is a severe form of pulmonary hypertension in adults with congenital heart disease (CHD) and has a poor prognosis. We aimed to understand factors associated with survival in ES and particularly to assess the potential benefits of advanced pulmonary vasodilator therapy (AT). METHODS: From January 2004, when AT became generally available for patients with ES, we followed 253 ES adults from 12 adult congenital heart disease centres across Australia and New Zealand...
August 9, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28704136/factors-influencing-outcomes-in-patients-with-eisenmenger-syndrome-a-nine-year-follow-up-study
#4
Mariana M Clavé, Nair Y Maeda, Claudia R P Castro, Sergio P Bydlowski, Antonio A Lopes
In patients with Eisenmenger syndrome, life expectancy is usually longer than in patients with other forms of pulmonary arterial hypertension (PAH). We conducted a cohort study in which patients were followed over a long period of time in an attempt to identify potential predictors of clinical outcomes. Sixty-seven treatment-naïve patients were enrolled (age range = 12-60 years; median age = 33 years). Baseline demographic, diagnostic, and functional parameters, plasma levels of endothelial dysfunction markers, and treatment-related data were tested for possible correlations with event-free survival...
July 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28663364/platelet-count-and-mean-platelet-volume-predict-outcome-in-adults-with-eisenmenger-syndrome
#5
Agustin C Martin-Garcia, Deepa Rj Arachchillage, Aleksander Kempny, Rafael Alonso-Gonzalez, Ana Martin-Garcia, Anselm Uebing, Lorna Swan, Stephen J Wort, Laura C Price, Colm McCabe, Pedro Luis Sanchez, Konstantinos Dimopoulos, Michael A Gatzoulis
OBJECTIVES: Although a significant proportion of patients with cyanotic congenital heart disease are thrombocytopaenic, its prevalence and clinical significance in adults with Eisenmenger syndrome (ES) is not well studied. Accordingly, we examined the relationship of thrombocytopaenia and mean platelet volume (MPV) to bleeding or thrombotic complications and survival in a contemporary cohort of patients with ES, including patients with Down syndrome. METHODS: Demographics, laboratory and clinical data were analysed from 226 patients with ES under active follow-up over 11 years...
June 29, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28660556/the-effect-of-endothelin-receptor-antagonists-in-patients-with-eisenmenger-syndrome-a-systematic-review
#6
REVIEW
Abdelrahman Elshafay, Duy Hieu Truong, Mohamed M AboElnas, Hossam Idrees, Hatem G Metwali, Nguyen Lam Vuong, Omar Ahmed Saad, Kenji Hirayama, Nguyen Tien Huy
INTRODUCTION: The efficacy of endothelin receptor antagonists (ERAs) in the management of Eisenmenger syndrome (ES) remains controversial. The aim of this study is to systemically review the safety and effects of ERAs in improving the quality of life and basic cardiac functions of these patients. METHODS: Twelve databases were searched, including PubMed, Web of Science, Scopus, Virtual Health Library, World Health Organization (WHO) Global Health Library, Google Scholar, POPLINE, Systems for Information of Grey Literature in Europe, New York Academy of Medicine, ClinicalTrials...
June 28, 2017: American Journal of Cardiovascular Drugs: Drugs, Devices, and Other Interventions
https://www.readbyqxmd.com/read/28652631/-stenting-of-multiple-peripheral-pulmonary-stenosis-with-the-coronary-technique-in-an-adul-patient-with-eisenmenger-like-syndrome
#7
Andrea Amaducci, Patrizio Vitulo
Peripheral pulmonary artery stenosis is a quite rare condition that can lead to misdiagnosis, particularly in adults. We describe the case of a 48-year-old woman with multiple stenoses, challenging anatomy and Eisenmenger-like presentation, who was treated percutaneously with a coronary technique.
June 2017: Giornale Italiano di Cardiologia
https://www.readbyqxmd.com/read/28651656/operable-patent-ductus-arteriosus-even-with-differential-cyanosis-a-case-of-patent-ductus-arteriosus-and-mitral-stenosis
#8
Anunay Gupta, Shyam S Kothari
Patients with patent ductus arteriosus and significant left-to-right shunt develop Eisenmenger syndrome at an early age and are not operable after development of irreversible pulmonary artery hypertension. Patients with mitral stenosis, however, are treatable even with suprasystemic pulmonary artery pressures. A combination of these two lesions is rare. We document a patient with differential cyanosis who improved after corrective surgery of both the lesions. The importance of post-capillary pulmonary artery hypertension in shunt lesions needs to be better appreciated...
June 27, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28622145/clinical-analyses-of-383-cases-with-maternal-cardiac-diseases
#9
Emine Aydin, Ozgur Ozyuncu, Dila Kasapoglu, Gokcen Orgul, Necla Ozer, Murat Yurdakok, M Sinan Beksac
AIM: To evaluate the pregnancy outcomes of women with heart disease. MATERIALS AND METHODS: In this retrospective study, 383 pregnant women with cardiac diseases were examined. The cases were classified according to the World Health Organization (WHO) classification. The distribution of the cases according to class, congenital heart diseases, mean birthweight, mean gestational week at delivery, type of delivery [cesarean section (CS) or vaginal delivery], and cardivascular events (during pregnancy and puerperium) were evaluated...
June 5, 2017: Journal of Perinatal Medicine
https://www.readbyqxmd.com/read/28614229/familial-clustering-of-congenital-deafness-patent-ductus-arteriosus-eisenmenger-complex-and-differential-cyanosis-a-case-report
#10
Ting-Wei Lin, Chih-Wei Tseng, Chi-Yao Huang, Kuo-Yang Wang, Kae-Woei Liang
RATIONALE: Few studies had reported syndromes that include patent ductus arteriosus (PDA) with Eisenmenger syndrome and congenital deafness clustered in male siblings without facial, skeletal, or mental abnormalities. PATIENT CONCERNS: Two brothers, who were deaf and had PDA with Eisenmenger complex, were first seen at our Cardiology clinic at the ages of 25 and 41, respectively. They presented with progressive dyspnea on exertion. Upon physical examination, both brothers had clubbing and/or cyanotic toes, normal fingers, and without facial, skeletal, ophthalmological, or mental abnormalities...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28597783/reverse-cardiac-remodelling-after-lung-transplantation-in-eisenmenger-syndrome
#11
Nejc Pavsic, Barbara Salobir, Katja Prokselj
No abstract text is available yet for this article.
April 2017: Acta Cardiologica
https://www.readbyqxmd.com/read/28566473/declining-incidence-and-prevalence-of-eisenmenger-syndrome-in-the-developed-world-a-triumph-of-modern-medicine
#12
EDITORIAL
Aleksander Kempny, Konstantinos Dimopoulos, Michael A Gatzoulis
No abstract text is available yet for this article.
September 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28536680/eisenmenger-syndrome
#13
Fernando Peixoto Ferraz de Campos, Luiz Alberto Benvenuti
No abstract text is available yet for this article.
January 2017: Autopsy & case reports
https://www.readbyqxmd.com/read/28524032/-preoperative-risk-factors-analysis-of-pulmonary-hypertension-crisis-during-perioperative-period-for-caesarean-section-of-woman-with-severe-pulmonary-hypertension
#14
Chunlei Zhang, Yaguang Liu, Enming Qing, Jun Ma
OBJECTIVE: To analyze preoperative risk factors of perioperative pulmonary hypertension crisis (PHC) for pregnant woman with severe pulmonary artery hypertension (PAH), and approach its clinical value. METHODS: A retrospective analysis was conducted. The clinical data from 152 pregnant women with severe PAH underwent cesarean delivery admitted to Beijing Anzhen Hospital from January 1st 2008 to December 31st 2016 was collected. The patients were divided into two groups according to with perioperative PHC or not...
May 2017: Zhonghua Wei Zhong Bing Ji Jiu Yi Xue
https://www.readbyqxmd.com/read/28523857/arrhythmia-burden-and-related-outcomes-in-eisenmenger-syndrome
#15
Shankar Baskar, Philippa Horne, Samantha Fitzsimmons, Philip R Khoury, Joseph Vettukattill, Koichiro Niwa, Teiji Agaki, Mark Spence, Hisanori Sakazaki, Gruschen Veldtman
BACKGROUND: Patients with Eisenmenger syndrome have a shorter lifespan than the general population. A significant proportion develop arrhythmia and some, sudden death. OBJECTIVE: The aims of this study were to characterize the frequency, type and effects of arrhythmias in adult patients with Eisenmenger's syndrome and to identify risk factors for arrhythmias. METHODS: This retrospective study included patients aged ≥ 18 years of age with Eisenmenger's syndrome from three institutions...
July 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28515531/anaesthetic-management-of-a-pregnant-woman-with-preeclampsia-and-eisenmenger-s-syndrome-role-of-advanced-haemodynamic-monitoring
#16
Muthapillai Senthilnathan, Savitri Velayudhan, Anusha Cherian, Pankaj Kundra
No abstract text is available yet for this article.
April 2017: Indian Journal of Anaesthesia
https://www.readbyqxmd.com/read/28450553/epidemiological-changes-in-eisenmenger-syndrome-in-the-nordic-region-in-1977-2012
#17
MULTICENTER STUDY
Cristel Sørensen Hjortshøj, Annette Schophuus Jensen, Keld Sørensen, Edit Nagy, Bengt Johansson, Thomas Kronvall, Mikael Dellborg, Mette-Elise Estensen, Henrik Holmstrøm, Maila Turanlahti, Ulf Thilén, Lars Søndergaard
OBJECTIVE: Improved diagnostic tools, timely closure of the shunt and a better understanding of the complexity of Eisenmenger syndrome (ES) have led to improved care and treatment in tertiary centres. These may have decreased the incidence of ES and improved survival of patients with ES, although evidence is still lacking. The aim of this study was to investigate temporal changes in incidence, prevalence and mortality in patients with ES for 35 years in the Nordic region. METHODS: This was a retrospective population-based study including 714 patients with ES...
September 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28435681/a-hol-e-y-predicament
#18
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
#19
David S Celermajer
No abstract text is available yet for this article.
July 7, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28430906/past-and-current-cause-specific-mortality-in-eisenmenger-syndrome
#20
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...
July 7, 2017: European Heart Journal
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