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Eisenmenger syndrome

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https://www.readbyqxmd.com/read/27866929/long-term-outcomes-of-pulmonary-arterial-hypertension-under-specific-drug-therapy-in-eisenmenger-syndrome
#1
Sébastien Hascoët, Alban-Elouen Baruteau, Marc Humbert, Gérald Simonneau, Xavier Jais, Jérôme Petit, Daniela Laux, Olivier Sitbon, Virginie Lambert, André Capderou
BACKGROUND: The long-term effectiveness of pulmonary arterial hypertension-specific drug therapy (PAH-SDT) in Eisenmenger syndrome is controversial. We investigated short-term and long-term hemodynamic changes under PAH-SDT and their associations with outcomes in a bicentric cohort. METHODS: Over 20 years, we included 69 patients with congenital heart disease, an indexed pulmonary vascular resistance (PVRi) >8 WU·m(2), and 292 standardized catheterizations at baseline and after PAH-SDT initiation or intensification...
October 15, 2016: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/27852228/pregnancy-outcome-in-women-with-eisenmenger-s-syndrome-a-case-series-from-west-china
#2
Ruiqi Duan, Xiumei Xu, Xiaodong Wang, Haiyan Yu, Yong You, Xinghui Liu, Aiyun Xing, Rong Zhou, Mingrong Xi
BACKGROUND: Eisenmenger's syndrome (ES) consists of pulmonary hypertension with a reversed or bidirectional shunt at the atrioventricular, or aortopulmonary level. The cardiovascular changes that occur during the pregnancy contribute to the high maternal morbidity and mortality in patients with ES. This study is to assess maternal and fetal outcomes in patients with ES. METHODS: This study is a retrospective analysis of 11 pregnancies in women with ES who delivered at a tertiary care center in west China between 2010 and 2014...
November 16, 2016: BMC Pregnancy and Childbirth
https://www.readbyqxmd.com/read/27849306/eisenmenger-syndrome-in-pregnancy
#3
Shi-Min Yuan
Eisenmenger syndrome is very rare in pregnant women. Debates remain concerning the management of Eisenmenger syndrome in this patient population and the prognosis is unclear in terms of maternal and fetoneonatal outcomes. Epidural analgesia is preferred for Cesarean section as it alleviates perioperative pain and reduces the pulmonary and systemic vascular resistances. Maternal mortality in the presence of Eisenmenger syndrome is reported as 30-50% and even up to 65% in those with Cesarean section. The major causes of death could be hypovolemia, thromboembolism and preeclampsia...
July 2016: Brazilian Journal of Cardiovascular Surgery
https://www.readbyqxmd.com/read/27846671/pulmonary-artery-dissection-in-a-patient-with-eisenmenger-s-syndrome-and-successful-conservative-medical-treatment
#4
Sun Hwa Hong, Kyung-Hee Kim
No abstract text is available yet for this article.
November 16, 2016: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/27816173/-sex-differences-in-congenital-heart-disease
#5
P Aubry, H Demian
Gender influences the clinical presentation and the management of some acquired cardiovascular diseases, such as coronary artery disease, resulting in different outcomes. Differences between women and men are also noticed in congenital heart disease. They are mainly related to the prevalence and severity of some congenital heart defects at birth, and in adulthood to the prognosis, incidence of Eisenmenger syndrome and risks of pregnancy. The role of gender on the risk of operative mortality of congenital heart surgery remains debated...
November 2, 2016: Annales de Cardiologie et D'angéiologie
https://www.readbyqxmd.com/read/27767324/brain-diffusion-changes-in-eisenmenger-syndrome
#6
Ferit Dogan, Dilek Sen Dokumaci, Ali Yildirim, Erol Bozdogan, Fatima Nurefsan Boyaci, Bulent Koca, Ekrem Karakas
OBJECTIVES: This preliminary study aimed to evaluate whether there are changes in the Apparent Diffusion Coefficient (ADC) values of the brain in patients presenting with Eisenmenger syndrome (ES). METHODS: This cross-sectional study included 10 consecutively recruited patients with ES and 10 healthy control subjects. In the patients and controls, 8 distinct neuroanatomical locations were selected for the analysis. Quantitative measurements of ADC values of the frontal and occiptal white matter, lentiform nucleus, thalamus, frontal cortex, anterior and posterior limbs of the internal capsule, caudate nucleus were measured...
October 21, 2016: British Journal of Radiology
https://www.readbyqxmd.com/read/27753979/os-12-06-effect-of-long-term-iloprost-treatment-on-the-right-ventricular-function-in-patients-with-eisenmenger-syndrome
#7
Kyoung Im Cho, Min Gu Chon
OBJECTIVE: Inhaled ilroprost can improve clinical status and symptoms in Eisenmenger syndrome (ES). Right ventricular (RV) function is important prognostic factor of pulmonary arterial hypertension. But there has been insufficient data in echocardiographic parameters related to RV function after long term iloprost therapy. So we evaluated effect of long term iloprost treatment on RV function and other parameters measured by echocardiography in this study. DESIGN AND METHOD: Eleven consecutive patients with ES associated with congenital heart disease underwent echocardiographic measurements at baseline and 48 weeks after iloprost therapy...
September 2016: Journal of Hypertension
https://www.readbyqxmd.com/read/27727969/eisenmenger-syndrome-in-an-adult-patient-with-aortopulmonary-window
#8
Nerusu Hari Babu
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27713195/-cardiac-function-after-lung-transplantation-of-eisenmenger-syndrome
#9
Soichiro Funaki, Yasushi Shintani, Masato Minami, Takayoshi Ueno, Koichi Toda, Yoshiki Sawa, Meinoshin Okumura
Eisenmenger syndrome(ES) could occur at the end-stage of congenital heart disease and is one of the indications for lung transplantation. Survival following heart-lung transplantation is better than that following bilateral lung transplantation (LT). However, in Japan, because of limitations in regard to the numbers of donors available, LT with cardiac repair is usually chosen. Following LT with cardiac repair, careful management of the repaired heart is necessary, though there are few reports available regarding such postoperative cardiac management...
October 2016: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/27648950/long-term-outcome-of-patients-with-perimembranous-ventricular-septal-defect-results-from-the-belgian-registry-on-adult-congenital-heart-disease
#10
Charlien Gabriels, Julie De Backer, Agnes Pasquet, Bernard P Paelinck, Marielle Morissens, Frederik Helsen, Alexander Van De Bruaene, Werner Budts
OBJECTIVES: Studies evaluating the long-term outcome of adults with ventricular septal defect (VSD) are important to inform patients about prognosis. This study investigated the long-term outcome of patients with perimembranous VSD (pmVSD) followed in the Belgian Registry on Adult Congenital Heart Disease. METHODS: All pmVSD patients in the registry were analyzed. RESULTS: Two hundred and sixty-six patients were studied. Fifteen patients had Eisenmenger syndrome...
September 21, 2016: Cardiology
https://www.readbyqxmd.com/read/27643376/os-12-06-effect-of-long-term-iloprost-treatment-on-the-right-ventricular-function-in-patients-with-eisenmenger-syndrome
#11
Kyoung Im Cho, Min Gu Chon
OBJECTIVE: Inhaled ilroprost can improve clinical status and symptoms in Eisenmenger syndrome (ES). Right ventricular (RV) function is important prognostic factor of pulmonary arterial hypertension. But there has been insufficient data in echocardiographic parameters related to RV function after long term iloprost therapy. So we evaluated effect of long term iloprost treatment on RV function and other parameters measured by echocardiography in this study. DESIGN AND METHOD: Eleven consecutive patients with ES associated with congenital heart disease underwent echocardiographic measurements at baseline and 48 weeks after iloprost therapy...
September 2016: Journal of Hypertension
https://www.readbyqxmd.com/read/27568461/elevated-serum-hmgb1-in-pulmonary-arterial-hypertension-secondary-to-congenital-heart-disease
#12
Yi-Yuan Huang, Wei Su, Zhao-Wei Zhu, Liang Tang, Xin-Qun Hu, Sheng-Hua Zhou, Zhen-Fei Fang, Jiang Li
AIMS: This study investigated the potential value of serum high mobility group box-1 (HMGB1) level in the diagnosis, staging and treatment response of patients with pulmonary arterial hypertension secondary to congenital heart disease (PAH-CHD). METHODS AND RESULTS: This was a single-center prospective study in 106 CHD patients. Serum HMGB1 levels were measured by enzymelinked immunosorbent assay. HMGB1 levels were significantly increased in patients with PAH compared to patients without PAH (P<0...
October 2016: Vascular Pharmacology
https://www.readbyqxmd.com/read/27564658/decreased-circulating-thrombomodulin-is-improved-by-tadalafil-therapy-in-hypoxemic-patients-with-advanced-pulmonary-arterial-hypertension
#13
N Y Maeda, M M Clavé, S P Bydlowski, A A Lopes
INTRODUCTION: Advanced pulmonary arterial hypertension (PAH) in patients with congenital cardiac communications and right-to-left shunting (Eisenmenger syndrome - PAH-ES) is associated with hypoxemia and decreased circulating levels of thrombomodulin (TM), probably reflecting decreased endothelial TM production. The combination of these two factors has been shown to induce fibrin deposition, with increased risk of thrombosis, a well known complication in this syndrome. PATIENTS AND METHODS: We tested the hypothesis that vasodilator therapy with the phosphodiesterase-5 inhibitor tadalafil, an approved drug for management of PAH could improve endothelial dysfunction markers, in particular plasma TM, in addition to improving the physical capacity (expected effect of pulmonary vasodilatation) in PAH-ES patients...
October 2016: Thrombosis Research
https://www.readbyqxmd.com/read/27554331/anaesthetic-management-of-breast-surgery-in-a-patient-with-eisenmenger-syndrome
#14
J C Galán Gutiérrez, F E Fernández Suárez, P Miranda García, L A Sopena Zubiria
Eisenmenger syndrome (ES) is a complex combination of cardiovascular abnormalities defined as pulmonary hypertension with investment or bidirectional flow through an intracardiac or aortopulmonary communication, usually secondary to a congenital heart disease not resolved promptly. It carries a significant risk of perioperative mortality, with an incidence close to 30% for non-cardiac surgery. We report the anaesthetic management in a ES patient undergoing breast surgery, which was successfully performed under general anaesthesia combined with thoracic analgesic blocks...
August 20, 2016: Revista Española de Anestesiología y Reanimación
https://www.readbyqxmd.com/read/27552240/preoperative-assessment-of-the-impact-of-positive-pressure-ventilation-with-noninvasive-positive-pressure-ventilation-in-a-patient-with-eisenmenger-syndrome-a-case-study
#15
Kazutomo Saito, Hiroaki Toyama, Yutaka Ejima, Masanori Yamauchi
In Eisenmenger syndrome (ES), positive pressure ventilation (PPV) during general anesthesia may lead to an increase in pulmonary vascular resistance and potentially to hypoxemia. In an attempt to predict the patient's hemodynamic response to intraoperative ventilation, we tested preoperatively the hemodynamic effects of noninvasive PPV with continuous positive pressure in a woman with ES scheduled for oophorectomy. The surgery was performed without complications, and the patient was discharged on postoperative day 8...
August 22, 2016: A & A Case Reports
https://www.readbyqxmd.com/read/27492657/effects-of-long-term-iloprost-treatment-on-right-ventricular-function-in-patients-with-eisenmenger-syndrome
#16
Min Ku Chon, Kyoung Im Cho, Kwang Soo Cha, Jeong Sook Seo, Dong Soo Kim
BACKGROUND: Right ventricular (RV) function is an important prognostic factor of pulmonary arterial hypertension (PAH), but there is insufficient data regarding RV function after long-term inhaled iloprost treatment. We evaluated the effect of long-term iloprost treatment on RV function in patients with Eisenmenger syndrome (ES). METHODS: Eleven consecutive patients with ES associated with congenital heart disease underwent echocardiographic measurements at baseline and 48 weeks after iloprost therapy...
August 1, 2016: Journal of Cardiology
https://www.readbyqxmd.com/read/27326222/survival-outcomes-in-severe-congenital-versus-non-congenital-pulmonary-hypertension
#17
Clare Arnott, Christiane Boehm, Edmund Lau, David S Celermajer
OBJECTIVE: Since 2006, our institution has cared for 70 patients with pulmonary arterial hypertension (PAH) and severe right ventricular (RV) hypertension; 44 with congenital heart disease (CHD) had Eisenmenger syndrome (ES) and 26 had PAH without CHD. We sought to determine and compare 'real-world' survival outcomes and cause of death in these two distinct groups. METHODS: An observational study of consecutive adult patients from our CHD or PAH clinics with RV systolic pressure ≥80 mm Hg (on echocardiography or right-heart catheter)...
2016: Heart Asia
https://www.readbyqxmd.com/read/27325973/left-main-coronary-artery-compression-in-a-young-woman-with-eisenmenger-syndrome
#18
Tobias Koppara, Julinda Mehilli, Alfred Hager, Harald Kaemmerer
No abstract text is available yet for this article.
2011: Heart Asia
https://www.readbyqxmd.com/read/27325590/eisenmenger-syndrome-and-long-term-survival-in-patients-with-down-syndrome-and-congenital-heart-disease
#19
Marc-André Körten, Paul C Helm, Hashim Abdul-Khaliq, Helmut Baumgartner, Deniz Kececioglu, Christian Schlensak, Ulrike M M Bauer, Gerhard-Paul Diller
OBJECTIVE: To characterise patients with trisomy 21 (Down syndrome, DS) based on the data of the German National Register for Congenital Heart Defects, to identify changes in the availability of surgical therapy over time and to analyse the impact of these changes on developing Eisenmenger syndrome (ES) as well as survival. METHODS: Out of 1549 patients with DS with congenital heart disease in the National Register for Congenital Heart Defects, 894 patients (55% female, mean age 17...
October 1, 2016: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/27287060/electrocardiogram-in-a-young-woman-with-the-eisenmenger-syndrome
#20
David Luke Glancy, Dana C Aiello, Prashanthi Atluri
A young woman with an atrial septal defect and the Eisenmenger syndrome has worsening symptoms and electrocardiographic changes of right ventricular hypertrophy five years later.
July 15, 2016: American Journal of Cardiology
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