Eisenmenger Complex (Eisenmenger's Syndrome)

A condition associated with VENTRICULAR SEPTAL DEFECT and other congenital heart defects that allow the mixing of pulmonary and systemic circulation, increase blood flow into the lung, and subsequent responses to low oxygen in blood. This complex is characterized by progressive PULMONARY HYPERTENSION; HYPERTROPHY of the RIGHT VENTRICLE; CYANOSIS; and ERYTHROCYTOSIS.

Also Known As:

Networked: 434 relevant articles (33 outcomes, 36 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Pulmonary Arterial Hypertension
2.	Familial Primary Pulmonary Hypertension
3.	Congenital Heart Defects (Congenital Heart Defect)
4.	Pulmonary Hypertension
5.	Atrial Heart Septal Defects (Atrial Septal Defect)

Experts

1.	Gatzoulis, Michael A: 16 articles (01/2020 - 07/2006)
2.	Dimopoulos, Konstantinos: 13 articles (10/2021 - 08/2007)
3.	Diller, Gerhard-Paul: 10 articles (12/2021 - 08/2007)
4.	Wort, Stephen J: 6 articles (10/2021 - 01/2010)
5.	Alonso-Gonzalez, Rafael: 6 articles (01/2020 - 06/2011)
6.	Kempny, Aleksander: 5 articles (10/2021 - 05/2012)
7.	Beghetti, Maurice: 5 articles (01/2019 - 02/2005)
8.	Inuzuka, Ryo: 5 articles (04/2014 - 01/2010)
9.	Swan, Lorna: 5 articles (10/2013 - 01/2010)
10.	Giannakoulas, Georgios: 5 articles (05/2012 - 01/2010)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Eisenmenger Complex:

1.	Bosentan (Tracleer)FDA Link 05/01/2010 - "In Eisenmenger syndrome, treatment with bosentan significantly improved walking distance, pulmonary blood flow, and PVR. " 07/04/2006 - "In this first placebo-controlled trial in patients with Eisenmenger syndrome, bosentan was well tolerated and improved exercise capacity and hemodynamics without compromising peripheral oxygen saturation." 05/01/2006 - "This study showed a clear functional improvement in patients with Eisenmenger's syndrome treated with bosentan. " 09/15/2021 - "The presence of a randomized controlled trial of bosentan BREATHE-5 and uncontrolled trials of other drugs became the basis for a higher class and level of evidence of bosentan (IB) compared to other drugs (IIaC) for Eisenmenger syndrome in the current European (ERS/ESC 2015) and updated Russian (2020) guidelines. " 09/01/2009 - "In the only randomised controlled trial dedicated to end-stage PAH-CHD, bosentan significantly reduced pulmonary vascular resistance and significantly increased 6-minute walk distance without compromising peripheral oxygen saturation, in patients with Eisenmenger syndrome. "
2.	Sildenafil Citrate (Viagra)FDA Link 12/01/2011 - "Sildenafil is an effective and safe agent for patients with Eisenmenger's syndrome. " 04/01/2006 - "In this randomized placebo-controlled study, we evaluated the efficacy of oral sildenafil in idiopathic PAH and PAH caused by Eisenmenger syndrome. " 11/01/2011 - "Oral sildenafil treatment for Eisenmenger syndrome: a prospective, open-label, multicentre study." 07/01/2014 - "Nine Eisenmenger syndrome patients [mean age=26 (SD=1.6) years] underwent 6MWT and an exercise test before and 6 weeks after oral sildenafil (20mg 3× D). " 06/01/2013 - "The favorable effects of short-term use of sildenafil on patients with Eisenmenger syndrome have been reported. "
3.	Endothelin Receptor AntagonistsIBA 04/01/2018 - "The efficacy of endothelin receptor antagonists (ERAs) in the management of Eisenmenger syndrome (ES) remains controversial. " 10/01/2020 - "Clinical and hemodynamic effect of endothelin receptor antagonists in Eisenmenger Syndrome." 02/05/2019 - "Correction to: A Tale of Two Endothelin Receptor Antagonists in Eisenmenger Syndrome: It was the best of times, It was the Worst of Times." 01/01/2019 - "Tale of 2 Endothelin Receptor Antagonists in Eisenmenger Syndrome." 04/01/2018 - "The Effect of Endothelin Receptor Antagonists in Patients with Eisenmenger Syndrome: A Systematic Review."
4.	Oxygen (Dioxygen)IBA 10/15/2021 - "Patients with IPAH and EIS have exaggerated ventilatory responses to stair-climbing compared to the controls with more severe levels of dyspnoea perception in Eisenmenger syndrome for equivalent oxygen uptake and work." 03/15/2017 - "Effects of acute oxygen supplementation on functional capacity and heart rate recovery in Eisenmenger syndrome." 10/12/2013 - "Six-minute walk test distance and resting oxygen saturations but not functional class predict outcome in adult patients with Eisenmenger syndrome." 03/11/2013 - "The mean oxygen drop index in Eisenmenger syndrome group was 9.0±6.2 and in the control group was 8.0±5.9 (p=0.63). " 12/01/2007 - "Patients with Eisenmenger syndrome had a raised p50 of the oxygen-haemoglobin dissociation curve (mean (SD) 29.4 (2.6) mm Hg vs 27 (3) mm Hg in laboratory controls, p<0.01). "
5.	Iloprost (Ventavis)FDA Link 06/01/2012 - "Iloprost is well tolerated and appears to be beneficial in the management of patients with Eisenmenger syndrome." 06/01/2012 - "In this study, 12 patients with Eisenmenger syndrome (mean age, 33.2 ± 12.1 years; 75% female) started receiving iloprost 10 μg/dose administered six times a day. " 06/01/2012 - "This study aimed to assess the efficacy and safety of iloprost used for patients with Eisenmenger syndrome. " 01/01/2020 - "A single-centre, placebo-controlled, double-blind randomised cross-over study of nebulised iloprost in patients with Eisenmenger syndrome: A pilot study." 01/01/2017 - "We evaluated the effect of long-term iloprost treatment on RV function in patients with Eisenmenger syndrome (ES). "
6.	Vasodilator Agents (Vasodilators)IBA 04/01/2021 - "This study evaluated the efficacy and safety of pulmonary vasodilators in patients with Eisenmenger syndrome. " 04/01/2021 - "Efficacy and safety of pulmonary vasodilators in the patients with Eisenmenger syndrome: a meta-analysis of randomized controlled trials." 04/01/2021 - "The present meta-analysis indicated that pulmonary vasodilators decrease mortality and improve hemodynamics and exercise capacity in patients with Eisenmenger syndrome. " 04/01/2021 - "Few meta-analyses evaluated the efficacy and safety of pulmonary vasodilators in patients with Eisenmenger syndrome. " 01/01/2019 - "Eisenmenger Syndrome in Adults: Treatment Pattern and Prognostic Factors in the Advanced Pulmonary Vasodilator Era."
7.	Tadalafil (Cialis)FDA Link 07/01/2007 - "In this blinded, cross over study, 11 patients with severe PAH related to congenital left to right shunt lesions (Eisenmenger syndrome) were randomly assigned to tadalafil (20 mg daily) or placebo for 4 weeks period, separated by a wash out period of at least 2 weeks. " 10/24/2006 - "Preliminary evaluation of tadalafil has shown efficacy and safety in selected patients with Eisenmenger syndrome, warranting further investigation in this subgroup of patients." 10/24/2006 - "Sixteen symptomatic Eisenmenger syndrome patients (mean age, 25+/-8.9 years) were assessed hemodynamically at baseline and 90 minutes after a single dose of tadalafil (1 mg/kg body weight up to a maximum of 40 mg). " 09/01/2011 - "In a randomized double-blind crossover trial, we compared the efficacy of phosphodiesterase-5 (PDE-5) inhibitor tadalafil with placebo in patients of Eisenmenger Syndrome (ES). " 09/01/2011 - "Clinical efficacy of phosphodiesterase-5 inhibitor tadalafil in Eisenmenger syndrome--a randomized, placebo-controlled, double-blind crossover study."
8.	Type 5 Cyclic Nucleotide PhosphodiesterasesIBA 10/24/2006 - "Phosphodiesterase-5 inhibitor in Eisenmenger syndrome: a preliminary observational study." 09/01/2011 - "In a randomized double-blind crossover trial, we compared the efficacy of phosphodiesterase-5 (PDE-5) inhibitor tadalafil with placebo in patients of Eisenmenger Syndrome (ES). " 09/01/2011 - "Clinical efficacy of phosphodiesterase-5 inhibitor tadalafil in Eisenmenger syndrome--a randomized, placebo-controlled, double-blind crossover study." 10/24/2006 - "We studied the effects of tadalafil, a phosphodiesterase-5 inhibitor, on short-term hemodynamics, tolerability, and efficacy over a 12-week period in patients of Eisenmenger syndrome having a pulmonary vascular pathology similar to idiopathic pulmonary arterial hypertension. "
9.	Nitric Oxide (Nitrogen Monoxide)FDA Link 01/01/2022 - "Successful Bronchial Artery Embolization after Stabilization with Nitric Oxide for the Treatment of Haemoptysis in a Patient with Eisenmenger Syndrome." 10/01/2006 - "Peri-operative trans-oesophageal echocardiography and nitric oxide during general anaesthesia in a patient with Eisenmenger's syndrome." 09/01/2000 - "Value of inhaled nitric oxide in Eisenmenger syndrome during pregnancy." 08/01/1999 - "Management of labor in Eisenmenger syndrome with inhaled nitric oxide." 01/01/1999 - "Favorable response of Eisenmenger syndrome to inhaled nitric oxide during pregnancy."
10.	selexipagIBA 12/01/2020 - "In the third case, initiation of Selexipag improved symptoms and objective exercise capacity in a patient with Eisenmenger syndrome. " 11/01/2018 - "Selexipag in Congenital Heart Disease-Associated Pulmonary Arterial Hypertension and Eisenmenger Syndrome: First Report." 01/01/2021 - "We present 3 patients with Eisenmenger syndrome and their improvement after starting treatment with selexipag, an oral selective IP prostacyclin receptor agonist." 01/01/2021 - "Use of the Prostacyclin Receptor Agonist Selexipag in Patients With Pulmonary Arterial Hypertension Associated With Eisenmenger Syndrome." 09/15/2021 - "Only three (bosentan, macitentan and selexipag) out of seven specific pulmonary vasodilators registered in the Russian Federation have indications for pulmonary arterial hypertension associated with congenital heart disease and Eisenmenger syndrome or pulmonary arterial hypertension associated with corrected simple congenital heart disease in the instructions for use. "

Therapies and Procedures

1.	Therapeutics 07/15/2013 - "DTT therapy improved clinical status in many patients with Eisenmenger's syndrome, but no significant impact on survival could be shown." 08/10/2013 - "To examine long-term efficacy of disease targeting therapies (DTT) in patients with Eisenmenger syndrome. " 01/01/2021 - "These guidelines point to a lack of evidence of benefit of O2 therapy from randomized controlled trials (RCTs) and to evidence of lack of benefit in a single RCT involving patients with Eisenmenger syndrome. " 12/01/2021 - "Medical Therapy for Eisenmenger Syndrome: A Case Report and Review of Literature." 07/17/2021 - "Presence of Eisenmenger syndrome compelled us to offer medical therapy for the patient. "
2.	Heart-Lung Transplantation 02/28/1999 - "Magnetic resonance imaging can be used repetitively in patients with Eisenmenger syndrome which may be helpful for better timing of combined heart-lung transplantation as ultimate therapeutic strategy because deterioration of right ventricular function can be monitored." 06/15/2022 - "Eisenmenger Syndrome in Case of Extended Goldenhar Complex Treated With Heart-Lung Transplantation." 01/01/2020 - "Heart-lung transplantation in Eisenmenger syndrome (two for the price of ?)." 12/01/2019 - "Heart-lung transplantation with concomitant aortic arch reconstruction for Eisenmenger syndrome and type B interrupted aortic arch." 01/01/2013 - "The number of patients needing heart-lung transplantation (especially for Eisenmenger's syndrome) has decreased in the last years. "
3.	Cesarean Section (Caesarean Section) 10/01/2023 - "This paper describes two clinical cases of pregnancy in patients (28 and 20 years old) with aortopulmonary window defect, severe PAH, and Eisenmenger syndrome that ended in preterm delivery by caesarean section. " 01/15/2023 - "Pregnancy in patients with Eisenmenger syndrome (ES) is associated with high maternal mortality rates of 30%‒50%, or even up to 65% in the case of a cesarean section (Yuan, 2016). " 12/01/2021 - "Cesarean section in a patient with Eisenmenger syndrome is high risk as morbidity and mortality are very high. " 12/01/2021 - "The use of point-of-care assessments and advanced hemodynamic monitoring in a patient with Eisenmenger syndrome for cesarean section: A case report." 09/01/2021 - "Women with Eisenmenger syndrome are usually advised to avoid pregnancy because of the high maternal mortality rate of 30-50% which increases up to 65% in the case of a cesarean section. "
4.	Epidural Anesthesia 11/01/1974 - "Epidural anesthesia in Eisenmenger's syndrome: a case report." 01/01/2002 - "We conclude that carefully titrated epidural anesthesia may be safe, appropriate, and effective for patients with Eisenmenger's syndrome for cesarean section." 01/01/2002 - "Epidural anesthesia for cesarean section in a patient with Eisenmenger's syndrome." 01/01/1989 - "[Epidural anesthesia for cesarean section in a patient with Eisenmenger's syndrome]." 05/14/1981 - "Eisenmenger's syndrome in pregnancy: epidural anesthesia for elective cesarean section."
5.	Hemodilution 06/01/1989 - "Significant hemodilution may lead to a marked improvement in platelet abnormalities in patients with Eisenmenger's complex." 12/01/2003 - "Thus, we studied 17 patients with the Eisenmenger syndrome, aged from 15 to 53 years, in the basal condition, studying 12 of them both before and after hemodilution. "