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Eisenmenger syndrome

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https://www.readbyqxmd.com/read/29135526/case-report-of-an-awake-craniotomy-in-a-patient-with-eisenmenger-syndrome
#1
Boris D Heifets, Erin Crawford, Ethan Jackson, Jessica Brodt, Richard A Jaffe, Mark A Burbridge
We present a detailed report of an awake craniotomy for recurrent third ventricular colloid cyst in a patient with severe pulmonary arterial hypertension in the setting of Eisenmenger syndrome, performed 6 weeks after we managed the same patient for a more conservative procedure. This patient has a high risk of perioperative mortality and may be particularly susceptible to perioperative hemodynamic changes or fluid shifts. The risks of general anesthesia induction and emergence must be balanced against the risks inherent in an awake craniotomy on a per case basis...
November 9, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/29121718/non-invasive-assessment-of-liver-changes-in-eisenmenger-patients
#2
Siegrun Mebus, Nicole Nagdyman, Johanna Kügel, Reinhart Zachoval, Siegmund Lorenz Braun, Guido Haverkämper, Bernd Opgen-Rhein, Felix Berger, Sophia Horster, Jörg Schoetzau, Claudia Pujol Salvador, Ulrike Bauer, John Hess, Peter Ewert, Harald Kaemmerer
BACKGROUND: Eisenmenger syndrome as a severe form of cyanotic congenital heart disease results in a complex multisystemic disorder. Due to increased systemic venous pressure and the inability to ensure systemic perfusion and metabolic requirements, the liver may develop congestion, fibrosis or cirrhosis. This study aimed to assess hepatic abnormalities in Eisenmenger patients non-invasively. METHODS AND RESULTS: 10 adults with Eisenmenger syndrome (six female; median age 44...
December 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/29061688/managing-the-eisenmenger-syndrome-a-call-to-arms
#3
EDITORIAL
Edgar L W Tay
No abstract text is available yet for this article.
October 23, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/29021475/successful-pulmonary-artery-embolization-for-the-management-of-hemoptysis-in-a-patient-with-eisenmenger-syndrome-caused-by-patent-ductus-arteriosus
#4
Naoki Tamada, Kazuhiko Nakayama, Yuto Shinkura, Ken-Ichi Yanaka, Naoto Katayama, Takuya Okada, Masato Yamaguchi, Kensuke Matsumoto, Hidekazu Tanaka, Toshiro Shinke, Noriaki Emoto, Ken-Ichi Hirata
The patient was a 19-year-old woman who was diagnosed with patent ductus arteriosus complicating Eisenmenger syndrome at a previous medical institution. She was referred to our hospital and arranged for lung transplantation. She developed hemoptysis after the introduction of i.v. epoprostenol, which was administered as a bridging treatment while the patient awaited lung transplantation. She continued to suffer from recurrent hemoptysis, even after switching from i.v. epoprostenol to i.v. treprostinil. Angiography of the systemic and pulmonary arteries revealed the vessel responsible for the recurrent hemoptysis and pulmonary artery embolization was successfully performed...
October 11, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29016730/microrna-424-322-as-a-new-marker-of-disease-progression-in-pulmonary-arterial-hypertension-and-its-role-in-right-ventricular-hypertrophy-by-targeting-smurf1
#5
Rui Baptista, Carla Marques, Steve Catarino, Francisco J Enguita, Marina C Costa, Paulo Matafome, Mónica Zuzarte, Graça Castro, Abílio Reis, Pedro Monteiro, Mariano Pêgo, Paulo Pereira, Henrique Girão
MicroRNAs (miRNAs) have been implicated in the pathogenesis of pulmonary hypertension (PH), a multifactorial and progressive condition associated with an increased afterload of the right ventricle leading to heart failure and death. The main aim of this study was to correlate the levels of miR-424(322) with the severity and prognosis of PH and with right ventricle hypertrophy progression. Additionally, we intended to evaluate the mechanisms and signaling pathways whereby miR-424(322) secreted by pulmonary endothelial cells (PAECs) impacts cardiomyocytes...
September 12, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28970634/severe-pulmonary-hypertension-and-right-ventricular-failure
#6
REVIEW
Ajay Kumar, Praveen Kumar Neema
Perioperative management of severe pulmonary hypertension (PH) is challenging. Anaesthesiologists come across perioperative management of such cases during incidental surgeries, surgery for various congenital heart diseases and valvular heart diseases and for caesarean section or painless labour in pregnant patient with Eisenmenger syndrome. Knowledge of pathophysiology of PH and novel drugs acting through different mechanisms is paramount in managing such patients. This review will help understanding pathophysiology of PH, anaesthetising patients with PH, use of novel drugs for PH and use of new mechanical devices for rescue of failing right ventricle...
September 2017: Indian Journal of Anaesthesia
https://www.readbyqxmd.com/read/28966315/prognostic-value-of-pulmonary-artery-compliance-in-patients-with-pulmonary-arterial-hypertension-associated-with-adult-congenital-heart-disease
#7
Xiao-Ling Cheng, Zhi-Hong Liu, Qing Gu, Xin-Hai Ni, Qin Luo, Zhi-Hui Zhao, Jian-Guo He, Chang-Ming Xiong
In congenital heart disease (CHD), the presence of pulmonary arterial hypertension (PAH) is associated with a poor prognosis. In this study, we aim to investigate the role of pulmonary artery compliance (Cp) in predicting the mortality of PAH associated with adult congenital heart disease (APAH-CHD). One-hundred and seventy-five patients of APAH-CHD who underwent a comprehensive clinical evaluation were included in this study. All patients were followed up in a 6-month interval and the primary end point was all cause of death...
October 21, 2017: International Heart Journal
https://www.readbyqxmd.com/read/28957530/rv-pressure-overload-from-hypertrophy-to-failure
#8
Cathelijne E E van der Bruggen, Ryan J Tedford, Martin Louis Handoko, Jolanda van der Velden, Frances S de Man
In pulmonary arterial hypertension (PAH), right ventricular (RV) adaptation is essential to overcome the chronic increases in RV pressure overload. Ultimately, RV compensatory mechanisms are not sufficient and patients succumb to RV failure. The processes underlying the transition of RV adaptation to RV failure are not well understood. In this review, we propose that important insights in RV adaptation processes can be obtained by comparing different etiologies of PAH, namely patients with PAH secondary to Eisenmenger syndrome, patients with PAH secondary to systemic sclerosis and patients where no cause is identified: idiopathic PAH...
October 1, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28882496/the-echocardiographic-characteristics-and-prognostic-significance-of-pericardial-effusions-in-eisenmenger-syndrome
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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