1,526 results match your criteria Eisenmenger Syndrome


Challenges in managing a critically ill patient with decompensated Eisenmenger syndrome.

BMJ Case Rep 2022 May 17;15(5). Epub 2022 May 17.

Anaesthesiology and Intensive Care Department, Faculty of Medicine and Health Sciences, Universiti Malaysia Sabah, Kota Kinabalu, Sabah, Malaysia

Eisenmenger syndrome (ES) is the most severe form of pulmonary arterial hypertension and is associated with congenital heart disease. ES itself is a challenging condition to manage, further compounded if the patient is critically ill and acutely decompensated. We share our experience of managing a critically ill adult patient with ES who presented with acute decompensation due to sepsis. Read More

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Long-Term Survival of Adult Patients With Atrial Septal Defect With Regards to Defect Closure and Pulmonary Hypertension.

Front Cardiovasc Med 2022 28;9:867012. Epub 2022 Apr 28.

Department of Cardiac Surgery, Na Homolce Hospital, Prague, Czechia.

Background: Atrial septal defect (ASD) is the most common congenital heart disease (CHD) in adults and pulmonary hypertension (PH) is an established risk factor. A decision whether to perform ASD closure, especially in elderly patients with PH, is a complex dilemma. The aim of our study was to compare long-term survival in patients with closed and open ASD. Read More

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Eisenmenger Syndrome Among Children with Unrepaired Congenital Heart Defects in Yunnan, China.

Pediatr Cardiol 2022 May 6. Epub 2022 May 6.

Susan and Henry Samueli College of Health Sciences, Program in Public Health, University of California Irvine, Irvine, CA, 92697, USA.

Eisenmenger syndrome is a life-threatening complication of congenital heart defects (CHD). Since Eisenmenger syndrome among children of repaired CHD is rare, very few studies have had the necessary data to investigate its distribution in children. The current study used data collected in rural China to investigate the prevalence of Eisenmenger syndrome in children with unrepaired CHD. Read More

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Maternal and fetal outcomes of pregnant women with pulmonary arterial hypertension associated with congenital heart disease in Beijing, China: A retrospective study.

Pulm Circ 2022 Apr 22;12(2):e12079. Epub 2022 Apr 22.

Department of Valvular Cardiac surgery center Beijing Anzhen Hospital Affiliated to Capital Medical University Chaoyang District Beijing China.

As pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) may increase maternal and fetal risk, this study explored the pregnancy outcomes of Chinese women with PAH-CHD. The clinical data of pregnant women with PAH-CHD admitted to the Beijing Anzhen Hospital from 2010 to 2019 were retrospectively analyzed; these patients and their offspring were followed up, with a mean period of 5.9 ± 2. Read More

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Spontaneous hyphema from iris microhemangioma in Eisenmenger syndrome.

Am J Ophthalmol Case Rep 2022 Jun 12;26:101536. Epub 2022 Apr 12.

Gold Coast University Hospital, 1 Hospital Blvd, Southport, QLD, 4215, Australia.

Purpose: We describe a patient with Eisenmenger syndrome and spontaneous hyphema from iris microhemangioma, two rare entities with a plausible pathophysiological connection.

Observations: A 56-year-old Caucasian female with a background of cyanotic congenital heart disease complicated by Eisenmenger syndrome presented with non-traumatic hyphema and blurred vision. Multiple vascular tufts consistent with iris microhemangiomas were observed around the pupil margins bilaterally, with no iris or retinal neovascularization. Read More

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Inferior sinus venosus defect and anomalous hepatic venous return to the coronary sinus leading to an Eisenmenger syndrome.

Cardiol Young 2022 Apr 28:1-2. Epub 2022 Apr 28.

Marie-Lannelongue Hospital, Paediatric and Congenital Cardiac Surgery Department, Centre de Référence des Malformations Cardiaques Congénitales Complexes M3C Groupe Hospitalier Saint-Joseph, Paris-Saclay University, Plessis-Robinson, France.

Inferior sinus venosus defect associated with left hepatic vein drainage to the coronary sinus is an extremely rare condition. We report the case of a 41-year-old man suffering from pulmonary arterial hypertension related to this unusual CHD. Planning of heart-lung transplantation in this case required accurate anatomical description. Read More

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Pregnancy in Congenital Heart Disease, Complicated by Pulmonary Arterial Hypertension-A Challenging Issue for the Pregnant Woman, the Foetus, and Healthcare Professionals.

Medicina (Kaunas) 2022 Mar 25;58(4). Epub 2022 Mar 25.

Clinic of Cardiac and Vascular Diseases, Faculty of Medicine, Institute of Clinical Medicine, Vilnius University, LT-08661 Vilnius, Lithuania.

: Pregnancy and delivery in patients with congenital heart disease (CHD) and pulmonary arterial hypertension (PAH) carry a very high risk for maternal and foetal complications and are contraindicated according to the guidelines. In the last decades, when an available modern PAH-targeted medication therapy and a new management concept improved patients' well-being and survival, some PAH-CHD females decided to conceive. Of note, despite advanced treatment and modern healthcare system possibilities, dealing with pregnancy in a diverse PAH-CHD population is still challenging. Read More

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Evaluation of Liver Stiffness After Atrial Septal Defect Closure.

Ultrasound Q 2022 Apr 14. Epub 2022 Apr 14.

Internal Medicine, University of Health Sciences-Adana Health Practice and Research Center, Adana, Turkey.

Abstract: In this study, we aimed to evaluate the liver stiffness (LS) values and the right ventricle (RV) functions after atrial septal defect (ASD) closure treatment. Sixty-six patients were included (38 female, 28 male) in the study. Patients were grouped into 3 subgroups (group I = 21 patients without ASD closure, group II = 38 patients who underwent ASD closure, and group III = 11 patients with ASD and Eisenmenger syndrome). Read More

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Pheochromocytoma and multiple paragangliomas associated with Eisenmenger syndrome: An autopsy case.

Pathol Int 2022 Apr 8. Epub 2022 Apr 8.

Department of Diagnostic Pathology, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Hakodate, Japan.

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Transcatheter leadless permanent pacemaker in complex congenital heart disease with interrupted inferior vena cava: A challenging implantation.

Indian Pacing Electrophysiol J 2022 May-Jun;22(3):165-168. Epub 2022 Mar 29.

Department of Cardiology, Eternal Hospital, Jaipur, India.

31 years lady with complete atrioventricular canal defect, large primum atrial septal defect (ASD), inlet ventricular septal defect (VSD) and Eisenmenger syndrome, presented with atrial flutter and complete heart block. She was not suitable for corrective cardiac surgery and not yet indicated for heart-lung transplantation. She was advised single chamber permanent pacemaker and eventually Micra VR transcatheter leadless pacemaker was finalised for her. Read More

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Role of high-flow nasal oxygen therapy in COVID-19 pneumonia with Eisenmenger syndrome: A case report.

Int J Cardiol Congenit Heart Dis 2022 Mar 12;7:100325. Epub 2022 Jan 12.

Department of Anesthesiology and Intensive Care, Universiti Sains Malaysia, School of Medical Sciences, 16150, Kubang Kerian, Kelantan, Malaysia.

Since the coronavirus disease 2019 (COVID-19) pandemic emerged in November 2019, various international guidelines and local protocols have been published to assist clinicians face the pandemic effectively. Medical and ventilatory strategies have evolved and researchers have come out with multiple studies and solutions within a short period of time. The patient's best interest is always the goal of the management. Read More

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A Case of Multifocal Extramedullary and Non-Hepatosplenic Extramedullary Hematopoiesis in a 43-Year-Old Man with a History of Congenital Eisenmenger Syndrome.

Am J Case Rep 2022 Mar 25;23:e935141. Epub 2022 Mar 25.

Department of Pathology, Military Institute of Medicine, Warsaw, Poland.

BACKGROUND Extramedullary hematopoiesis in organs outside the bone marrow most commonly occurs in the liver and spleen. This report is of a case of multifocal extramedullary and non-hepatosplenic extramedullary hematopoiesis a 43-year-old man with a history of congenital Eisenmenger syndrome. CASE REPORT We present the case of a 43-year-old patient with complex heart disease and full-blown Eisenmenger syndrome associated with ventricular septal defect, bicuspid right ventricle, and pulmonary hypertension. Read More

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Eisenmenger Syndrome: JACC State-of-the-Art Review.

J Am Coll Cardiol 2022 03;79(12):1183-1198

Department of Cardiology, Monaldi Hospital - "L. Vanvitelli" University, Naples, Italy. Electronic address:

Although major breakthroughs in the field of pediatric cardiology, cardiac surgery, intervention, and overall care improved the outlook of congenital heart disease, Eisenmenger syndrome (ES) is still encountered and remains a complex clinical entity with multisystem involvement, including secondary erythrocytosis, increased thrombotic and bleeding diathesis, high arrhythmogenic risk, progressive heart failure, and premature death. Clearly, care for ES is best delivered in multidisciplinary expert centers. In this review, we discuss the considerable recent progress in understanding the complex pathophysiology of ES, means of prognostication, and improvement in clinical outcomes achieved with pulmonary arterial hypertension-targeted therapies. Read More

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Pulmonary Hypertension in Adult Congenital Heart Disease in Asia: A Distinctive Feature of Complex Congenital Heart Disease.

J Am Heart Assoc 2022 Apr 14;11(7):e022596. Epub 2022 Mar 14.

Department of Pediatrics National Taiwan University Hospital and Medical CollegeNational Taiwan University Taipei Taiwan.

Background The epidemiology of pulmonary hypertension (PH) in patients with adult congenital heart disease in Western countries is already known. We investigate clinical characteristics of PH in adult congenital heart disease with emphasis on complex congenital heart disease (CHD) from an Asian cohort in Taiwan. Methods and Results All adult patients (aged >18 years) diagnosed with CHD between January 2007 and July 2018 qualified for the study. Read More

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Eisenmenger syndrome with left main compression syndrome: a case report.

BMC Cardiovasc Disord 2022 03 5;22(1):89. Epub 2022 Mar 5.

Cardiology Intervention Division, Department of Cardiology and Vascular Medicine, University of Padjadjaran, Hasan Sadikin General Hospital, Bandung, Indonesia.

Background: Left main coronary artery disease secondary to pulmonary artery compression related to Eisenmenger syndrome is an under-suspected condition that can cause fatal outcomes if left untreated. It presents with typical angina but is frequently mistaken for pulmonary hypertension (PH) symptoms. It is now recognized as one of the few important causes of angina in PH. Read More

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Central retinal artery occlusion with cilioretinal sparing in a patient with Eisenmenger syndrome.

BMJ Case Rep 2022 Feb 28;15(2). Epub 2022 Feb 28.

Department of Ophthalmology, All India Institute of Medical Sciences - Patna, Patna, Bihar, India

A young boy in his 20s presented with sudden diminution of vision in right eye 2 days ago. Best-corrected visual acuity was hand movement in right eye and 20/20 in left eye. Funduscopy demonstrated diffuse retinal opacification and swelling at the posterior pole with a cherry red spot at the macula. Read More

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February 2022

Anaesthetic management in neurosurgery in a patient with Eisenmenger syndrome and Down syndrome. A case report.

Rev Esp Anestesiol Reanim (Engl Ed) 2022 02 12;69(2):109-113. Epub 2022 Feb 12.

Servicio de Anestesiología, Reanimación y Tratamiento del Dolor, Hospital Clínic de Barcelona, Barcelona, Spain.

Adults patients with congenital heart disease increasingly present for non cardiac surgery. The anesthetic management this type of patients in neurosurgery requires a meticulous surgical anesthetic planning. The need for urgent intervention, with the presence of a congenital heart disease evolved to Eisenmenger syndrome, associated to a difficult airway, is a challenge for the anesthesiologist. Read More

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February 2022

Haemoptysis in Pulmonary Arterial Hypertension Associated with Congenital Heart Disease: Insights on Pathophysiology, Diagnosis and Management.

J Clin Med 2022 Jan 26;11(3). Epub 2022 Jan 26.

First Department of Cardiology, AHEPA University Hospital, Aristotle University of Thessaloniki, St. Kiriakidi 1, 54636 Thessaloniki, Greece.

Haemoptysis represents one of the most severe major bleeding manifestations in the clinical course of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD). Accumulating evidence indicates that dysfunction of the pulmonary vascular bed in the setting of PAH predisposes patients to increased hemorrhagic diathesis, resulting in mild to massive and life-threatening episodes of haemoptysis. Despite major advances in PAH targeted treatment strategies, haemoptysis is still correlated with substantial morbidity and impaired quality of life, requiring a multidisciplinary approach by adult CHD experts in tertiary centres. Read More

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January 2022

Fruitful Pregnancy Outcome in a Case of Eisenmenger Syndrome With Severe Pulmonary Hypertension: A Rare Case Report.

Cureus 2022 Jan 10;14(1):e21068. Epub 2022 Jan 10.

Medicine, Alfaisal University, Riyadh, SAU.

Eisenmenger syndrome (ES) is considered an absolute contraindication for pregnancy. ES is characterized by a congenital heart abnormality that results in a significant anatomical shunt. Hemodynamic forces generate a left-right shunt, leading to severe pulmonary arterial hypertension (PAH). Read More

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January 2022

Catheter Ablation of Atrioventricular Nodal Reentrant Tachycardia in Patients With Congenital Heart Disease.

Circ Arrhythm Electrophysiol 2022 02 28;15(2):e010631. Epub 2022 Jan 28.

Royal Brompton Hospital, Guys and St. Thomas' NHS Foundation Trust, London, United Kingdom (S.E.).

Atrioventricular (AV) nodal reentrant tachycardia represents the most common regular supraventricular arrhythmia in humans, and catheter ablation of the so called slow AV nodal pathway has been effectively performed for decades. In patients with congenital heart disease, a combination of different factors makes catheter ablation of AV nodal reentrant tachycardia substrate particularly challenging, including abnormal venous access to intracardiac structures, abnormal intracardiac anatomy, potentially deviant and often unpredictable sites of the specific conduction system, loss of traditional anatomic landmarks, and congenital cardiac surgery that may complicate the access to the AV nodal area. Published experiences have confirmed the efficacy and the relative safety of such procedures when performed by experts, but the risk of complications, in particular AV block, remains non-negligible. Read More

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February 2022

Medical treatment of pulmonary hypertension in adults with congenital heart disease: updated and extended results from the International COMPERA-CHD Registry.

Cardiovasc Diagn Ther 2021 Dec;11(6):1255-1268

Universitätsspital Zürich, Klinik für Pneumologie, Zürich, Switzerland.

Background: Pulmonary arterial hypertension (PAH) is common in congenital heart disease (CHD). Because clinical-trial data on PAH associated with CHD (PAH-CHD) remain limited, registry data on the long-term course are essential. This analysis aimed to update information from the COMPERA-CHD registry on management strategies based on real-world data. Read More

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December 2021

The use of pulmonary arterial hypertension therapies in Eisenmenger syndrome.

Expert Rev Cardiovasc Ther 2021 Dec 27;19(12):1053-1061. Epub 2021 Dec 27.

Department of Cardiology III - Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Albert‑Schweitzer‑Campus 1, Muenster, Germany.

Introduction: For many years, treatment options for patients with Eisenmenger physiology had been restricted to conservative measures to alleviate multi-system complications. The use of pulmonary arterial hypertension (PAH)-targeted therapies in patients with Eisenmenger syndrome (ES) changed the course of the disease, since they substantially improved clinical outcomes and increased survival.

Areas Covered: In this review, we primarily focus on the use of PAH pharmacotherapies in ES. Read More

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December 2021

Angiography-guided mid/high septal implantation of ventricular leads in patients with congenital heart disease.

J Arrhythm 2021 Dec 17;37(6):1512-1521. Epub 2021 Sep 17.

Department of Biomedical Engineering Maastricht UMC+ Maastricht The Netherlands.

Background: Conduction system pacing prevents pacing-induced cardiomyopathy, but it can be challenging to perform in patients with congenital heart disease (CHD), and mid/high septal lead implantation is an alternative. This study aimed to assess intraprocedural angiography's utility as a guide for mid/high-septal lead implantation in CHD patients.

Methods: The study subjects were CHD patients with Class I/IIa indications for permanent pacemaker implantation. Read More

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December 2021

Effect of oxygen therapy on exercise performance in patients with cyanotic congenital heart disease: Randomized-controlled trial.

Int J Cardiol 2022 Feb 30;348:65-72. Epub 2021 Nov 30.

Clinic of Pulmonology, University Hospital of Zurich, Switzerland; Center for Human Integrative Physiology, and Zurich Center for Interdisciplinary Sleep Research, University of Zurich, Switzerland. Electronic address:

Background: Patients with unrepaired cyanotic congenital heart disease (CHD) suffer from aggravated hypoxemia during exercise. We tested the hypothesis that supplemental oxygen improves exercise performance in these patients.

Methods: In this randomized, sham-controlled, single-blind, cross-over trial cyanotic CHD-patients underwent four cycle exercise tests to exhaustion, while breathing either oxygen-enriched (FiO 0. Read More

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February 2022

Stridor as a Harbinger of Congenital Cardiovascular Anomaly.

Acta Cardiol Sin 2021 Nov;37(6):608-617

Departments of Pediatrics; College of Medicine, National Taiwan University Hospital, Taipei, Taiwan.

Background: There is an intimate spatial relationship between the cardiovascular and airway structures. Central airway compression related to congenital cardiovascular anomalies should be considered in neonates, infants, and young children presenting with stridor.

Methods: From July 31, 1990 to December 31, 2018, 24 pediatric patients, including 18 males and 6 females, aged 1 day to 11. Read More

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November 2021

Pulmonary Hypertension in Adults with Congenital Heart Disease.

Cardiol Clin 2022 Feb;40(1):55-67

Section of Adult Congenital Heart Disease, Division of Cardiology, Duke University Medical Center, Box 3331, Durham, NC 27710, USA. Electronic address:

Pulmonary arterial hypertension related to congenital heart disease (PAH-CHD) affects 5% to 10% of adults with CHD and is associated with significant morbidity and mortality. PAH-CHD develops as a consequence of intracardiac or extracardiac systemic-to-pulmonary shunts that lead to pulmonary vascular remodeling through a pathologic process that is similar to other causes of PAH. Eisenmenger syndrome is the most severe phenotype of PAH-CHD and is characterized by severe elevation in pulmonary vascular resistance, with shunt reversal causing hypoxemia and central cyanosis. Read More

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February 2022

The use of point-of-care assessments and advanced hemodynamic monitoring in a patient with Eisenmenger syndrome for cesarean section: A case report.

Int J Surg Case Rep 2021 Dec 12;89:106601. Epub 2021 Nov 12.

Department of Anesthesiology, University of the Philippines - Philippine General Hospital, Taft Avenue, Manila, Philippines. Electronic address:

Introduction: Eisenmenger syndrome should be diagnosed before pregnancy and surgically corrected if possible. Cesarean section in a patient with Eisenmenger syndrome is high risk as morbidity and mortality are very high. Delivery in hospitals with intensive care units should be recommended. Read More

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December 2021

Pulmonary hypertension in adults with congenital heart disease. Clinical phenotypes and outcomes in the advanced pulmonary vasodilator era.

Heart Lung 2022 Jan-Feb;51:75-81. Epub 2021 Nov 9.

Unidad de Cardiopatías Congénitas del Adulto, Hospital General Universitario Virgen del Rocio, Instituto de BioMedicina de Sevilla (IBIS) y CIBERCV, Avenida Manuel Siurot S/N, Sevilla 41013, Spain. Electronic address:

Background: Mortality of pulmonary hypertension associated with congenital heart disease (PAH-CHD) in adults remains high.

Objectives: To identify predictors of death and to assess the impact of treatment on outcome.

Methods: Retrospective, multicenter cohort study of 103 adults with PAH-CHD followed-up for 8. Read More

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Use of venous-venous extracorporeal membrane oxygenation in the treatment of postpartum pulmonary hypertension crisis.

Perfusion 2021 Nov 11:2676591211043705. Epub 2021 Nov 11.

Department of Critical Care Medicine, Taizhou Hospital of Zhejiang Province Affiliated to Wenzhou Medical University, Taizhou, Zhejiang, China.

The incidence of heart disease in pregnancy ranges from 0.5% to 3.0% and is regarded as one of the top three causes of maternal death. Read More

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November 2021