1,462 results match your criteria Eisenmenger Syndrome


Young woman with recurrent pregnancy loss.

Heart 2021 May;107(10):821-854

Department of Cardiology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India.

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Risk stratification of adults with congenital heart disease during the COVID-19 pandemic: insights from a multinational survey among European experts.

Open Heart 2021 04;8(1)

Center for Congenital Heart Disease, Cardiology, Inselspital University Hospital Bern, University of Bern, Bern, Switzerland

Objective: Adults with congenital heart disease (ACHD) may be at a higher risk of a fatal outcome in case of COVID-19. Current risk stratification among these patients relies on personal experience and extrapolation from patients with acquired heart disease. We aimed to provide an expert view on risk stratification while awaiting results from observational studies. Read More

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Preventing disease progression in Eisenmenger syndrome.

Expert Rev Cardiovasc Ther 2021 May 6:1-18. Epub 2021 May 6.

Department of Cardiology, Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK.

: Eisenmenger syndrome describes a condition in which a congenital heart defect has caused severe pulmonary vascular disease, resulting in reversed (right-left) or bidirectional shunting and chronic cyanosis.: In this paper, the progression of congenital heart defects to Eisenmenger syndrome, including early screening, diagnosis and operability are covered. The mechanisms of disease progression in Eisenmenger syndrome and management strategies to combat this, including the role of pulmonary arterial hypertension therapies, are also discussed. Read More

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Brain abscess associated with ventricular septal defect and Eisenmenger syndrome: A case report.

Int J Surg Case Rep 2021 Apr 19;81:105799. Epub 2021 Mar 19.

Department of Neurosurgery, Zhongnan Hospital of Wuhan University, Wuhan, China. Electronic address:

Introduction And Importance: Brain abscess is a potentially fatal neurological infection, despite the development of new antimicrobial agents and modern neurosurgical techniques.

Case Presentation: We present an uncommon case where a large brain abscess was treated successfully in a patient with Eisenmenger syndrome. He was underwent neurosurgical treatment and eventually recovered. Read More

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Genetic deletion of CMG2 exacerbates systemic-to-pulmonary shunt-induced pulmonary arterial hypertension.

FASEB J 2021 Apr;35(4):e21421

Medical Research Center & Beijing Key Laboratory of Hypertension Research, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China.

Pulmonary arterial hypertension (PAH) secondary to congenital heart disease (CHD-PAH) with systemic-to-pulmonary shunt (SPS) is characterized by proliferative vascular remodeling. Capillary morphogenesis gene-2 (CMG2) plays a key role in cell proliferation and apoptosis. This study aimed to determine the role of CMG2 in the pathogenesis of SPS-induced PAH. Read More

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Peripheral microangiopathy in Eisenmenger syndrome: A nailfold video capillaroscopy study.

Int J Cardiol 2021 Mar 16. Epub 2021 Mar 16.

Fourth Department of Internal Medicine, Hippokration University Hospital, Medical School, Aristotle University of Thessaloniki, 49 Konstantinoupoleos Street, 54642 Thessaloniki, Greece.

Background: Eisenmenger syndrome (ES) comprises a severe phenotype of pulmonary arterial hypertension characterized by angiopathy of the lung circulation. The aim of the present study was to demonstrate the presence of systemic microvascular abnormalities in patients with ES using nailfold video-capillaroscopy (NVC) and to identify potential correlations of nailfold capillaroscopic characteristics with non-invasive markers of systemic organ function.

Methods: Α cross-sectional NVC study was performed in 17 consecutive patients with ES and 17 healthy controls matched for age and sex. Read More

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Idiopathic bilateral massive perirenal subcapsular effusion: The first case in the literature.

Urologia 2021 Mar 17:3915603211001252. Epub 2021 Mar 17.

Department of Urology, Bagcilar Training and Research Hospital, Istanbul, Turkey.

Introduction: Bilateral perirenal subcapsular effusion is a rare clinical condition and is associated with several underlying etiologies. We present a 33 years old male patient with idiopathic bilateral massive subcapsular effusion.

Case Presentation: A 33-year-old male patient presented to our outpatient clinic with bilateral flank pain and fever for 2 weeks. Read More

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Clinical outcome of COVID-19 in patients with adult congenital heart disease.

Heart 2021 Mar 8. Epub 2021 Mar 8.

Departament of Cardiology, University Heart Center, University Hospital Zurich, Zurich, Switzerland.

Aims: Patients with adult congenital heart disease (ACHD) are a potentially vulnerable patient cohort in case of COVID-19. Some cardiac defects may be associated with a poor COVID-19 outcome. Risk estimation in ACHD is currently based on expert opinion. Read More

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Hybrid retrieval of embolized device in abdominal aorta after transcatheter closure of large patent ductus arteriosus.

J Vasc Surg Cases Innov Tech 2021 Mar 6;7(1):56-60. Epub 2020 Nov 6.

Department of Vascular Surgery, Faculty of Medicine, Mansoura University, Mansoura, Egypt.

Patients with a large patent ductus arteriosus (PDA) can have several presentations. Many will be asymptomatic, some could develop severe pulmonary hypertension, and others can develop Eisenmenger syndrome. We have presented a case in which a PDA correction device was embolized to the abdominal aorta, 2 months after transcatheter closure of a large PDA. Read More

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Simple equations to predict the effects of veno-venous ECMO in decompensated Eisenmenger syndrome.

ESC Heart Fail 2021 Apr 25;8(2):1637-1642. Epub 2021 Feb 25.

The Service of Adult Intensive Care Medicine, University Hospital and Faculty of Biology and Medicine, University of Lausanne, Rue du Bugnon 46, Lausanne, CH-1011, Switzerland.

Adult patients with uncorrected congenital heart diseases and chronic intracardiac shunt may develop Eisenmenger syndrome (ES) due to progressive increase of pulmonary vascular resistance, with significant morbidity and mortality. Acute decompensation of ES in conditions promoting a further increase of pulmonary vascular resistance, such as pulmonary embolism or pneumonia, can precipitate major arterial hypoxia and death. In such conditions, increasing systemic oxygenation with veno-venous extracorporeal membrane oxygenation (VV-ECMO) could be life-saving, serving as a bridge to treat a potential reversible cause for the decompensation, or to urgent lung transplantation. Read More

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Oxygen and pulmonary arterial hypertension: effects, mechanisms, and therapeutic benefits.

Eur J Prev Cardiol 2021 Mar;28(1):127-136

School of Health Sciences, Locked Bag 1797 Penrith, Sydney, NSW 2751, Australia.

Oxygen is a pulmonary vasodilator. Although treatment of pulmonary arterial hypertension (PAH) is focused on pulmonary vasodilation, treatment guidelines do not recommend O2 therapy for patients unless they develop hypoxaemia. 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. Read More

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Congenital Heart Disease-Associated Pulmonary Hypertension.

Clin Chest Med 2021 Mar 12;42(1):9-18. Epub 2021 Jan 12.

Division of Pediatric Cardiology, Columbia University Irving Medical Center - New York Presbyterian Hospital, 3959 Broadway-CH-2N, New York, NY 10032, USA.

Presently, with increasing survival of patients with congenital heart disease (CHD), pulmonary arterial hypertension (PAH) associated with CHD is commonly encountered in children and adults. This increased prevalence is seen despite significant advances in early diagnosis and surgical correction of patients with structural CHD. PAH is the cause of significant morbidity and mortality in these patients and comes in many forms. Read More

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Use of the Prostacyclin Receptor Agonist Selexipag in Patients With Pulmonary Arterial Hypertension Associated With Eisenmenger Syndrome.

Can J Cardiol 2021 Feb 2. Epub 2021 Feb 2.

Department of Cardiology, Onassis Cardiac Surgery Center, Athens, Greece. Electronic address:

Eisenmenger syndrome is a multisystem disorder and the most severe form of pulmonary arterial hypertension in adult congenital heart disease. Pulmonary arterial hypertension represents a fatal disease, characterized by increased pulmonary vascular resistance, right heart failure, and death. Although therapeutic management has rapidly advanced in recent years, these patients were not included in randomized controlled trials for specific pulmonary arterial hypertension drugs, except for bosentan. Read More

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February 2021

Management of Eisenmenger syndrome by cardiac defect closure combined bilateral lung transplantation with intraoperative venoarterial ECMO support.

J Card Surg 2021 Apr 24;36(4):1560-1562. Epub 2021 Jan 24.

Department of Thoracic Surgery, Wuxi Lung Transplant Center, Wuxi People's Hospital Affiliated to Nanjing Medical University, Wuxi, China.

Background: Optimal surgical treatment for Eisenmenger syndrome in adult congenital heart disease remains in debate.

Case Report: We report a case of a 22-year-old female with Eisenmenger syndrome secondary to ventricular septum defect (VSD), who underwent cardiac defect closure combined with bilateral lung transplantation in our center. The patient had an uncorrected peri-membranous VSD and subsequently developed severe pulmonary hypertension. Read More

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A Rare Case of Adult Aortopulmonary Window Combined with Anomalous Origin of the Right Pulmonary Artery from the Aorta Leading to Eisenmenger Syndrome.

J Int Med Res 2021 Jan;49(1):300060520984656

Center for Cardiovascular Medicine, The First Hospital of Jilin University, Changchun, China.

Aortopulmonary window is a rare congenital heart disease that can increase pulmonary vascular resistance, exacerbate left-to-right shunt and lead to heart failure and respiratory tract infections. Most patients die during childhood. We report a 53-year-old male patient with a large aortopulmonary window combined with anomalous origin of the right pulmonary artery from the aorta, with Eisenmenger syndrome and without surgery. Read More

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January 2021

Midterm outcomes of the Potts shunt for pediatric pulmonary hypertension, with comparison to lung transplant.

J Thorac Cardiovasc Surg 2021 Mar 9;161(3):1139-1148. Epub 2020 Dec 9.

Section of Pediatric Cardiothoracic Surgery, Washington University School of Medicine, St Louis Children's Hospital, St Louis, Mo. Electronic address:

Objective: For children with severe pulmonary hypertension, addition of Potts shunt to a comprehensive palliation strategy might improve the outcomes afforded by medications and delay lung transplantation.

Methods: A prospective analysis was conducted of all children undergoing Potts shunt (first performed in 2013) or bilateral lung transplant for pulmonary hypertension from 1995 to present.

Results: A total of 23 children underwent Potts shunt (20 surgical, 3 transcatheter), and 31 children underwent lung transplant. Read More

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Contemporary use of Selexipag in pulmonary arterial hypertension associated with congenital heart disease: a case series.

Eur Heart J Case Rep 2020 Dec 7;4(6):1-7. Epub 2020 Nov 7.

Division of Cardiology, Department of Medicine, University of California San Francisco, 505 Parnassus Avenue, L524, UCSF Box 0103, San Francisco, CA 94117, USA.

Background: There are significant risks of parenteral prostacyclin use in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), which may limit their use. Selexipag is an oral, selective prostacyclin analogue that has been shown to reduce disease progression and improve exercise capacity in patients with PAH-CHD. Administering Selexipag in patients with PAH-CHD could potentially overcome some of the risks of parenteral therapy while improving clinical outcomes. Read More

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December 2020

Patent foramen ovale and atrial septal defect.

Echocardiography 2020 12;37(12):2172-2184

Division of Cardiology, Loma Linda University Medical Center, Loma Linda, CA, USA.

Atrial septal defects (ASD) are among the most common congenital heart diseases encountered in adulthood. Patent foramen ovale (PFO) is present in up to 25% of the population. ASD could present as isolated lesion or in association with more complex congenital heart disease form as tetralogy of Fallot, or Ebstein's anomaly of tricuspid valve. Read More

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December 2020

Clinical and hemodynamic effect of endothelin receptor antagonists in Eisenmenger Syndrome.

Ann Pediatr Cardiol 2020 Oct-Dec;13(4):309-319. Epub 2020 Jul 24.

Department of Cardiology and Vascular Medicine, Faculty of Medicine Universitas Indonesia, National Cardiovascular Center Harapan Kita, Jakarta, Indonesia.

Introduction: Endothelin receptor antagonists (ERAs) are widely accepted as a specific treatment for pulmonary arterial hypertension. Unfortunately, consensus and recommendations are lacking for the treatment of patients who suffer from pulmonary arterial hypertension and congenital heart disease, including Eisenmenger syndrome.

Objective: This meta-analysis aimed to compare the effect of ERA on patients with Eisenmenger syndrome. Read More

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Amyloid and Tau PET Imaging of Alzheimer Disease and Other Neurodegenerative Conditions.

Semin Ultrasound CT MR 2020 Dec 29;41(6):572-583. Epub 2020 Aug 29.

Department of Radiology, University of Wisconsin, Madison, WI. Electronic address:

Although diagnosing the syndrome of dementia is largely a clinical endeavor, neuroimaging plays an increasingly important role in accurately determining the underlying etiology, which extends beyond its traditional role in excluding other causes of altered cognition. New neuroimaging methods not only facilitate the diagnosis of the most common neurodegenerative conditions (particularly Alzheimer Disease [AD]) after symptom onset, but also show diagnostic promise even in the very early or presymptomatic phases of disease. Positron emission tomography (PET) is increasingly recognized as a key clinical tool for differentiating normal age-related changes in brain metabolism (using F-fluorodeoxyglucose [FDG]) from those seen in the earliest stages of specific forms of dementia. Read More

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December 2020

Pulmonary hypertension associated with congenital heart disease; clinical decision scenario.

Respir Med Case Rep 2020 11;31:101286. Epub 2020 Nov 11.

Consultant Pediatric Cardiologist, Prince Sultan Cardiac Center-Qassim, Saudi Arabia.

Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is one type under group 1 PH. Undiagnosed or delayed diagnosis of significant CHD might lead to significant PAH and at the end might lead to Eisenmenger syndrome. We could expect the degree of PAH in patients with CHD by proper clinical assessment as well as by the basic assessment tools including the chest x-ray (CXR), ECG, and transthoracic echocardiography (TTE). Read More

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November 2020

A 26-Year-Old Woman with Worsening Dyspnea: Look Closer, Think Critically.

J Cardiovasc Echogr 2020 Apr-Jun;30(2):116-118. Epub 2020 Aug 17.

Department of Cardiology, Ataturk University Faculty of Medicine, Erzurum, Turkey.

Pulmonary hypertension due to congenital heart disease continues to be a diagnostic challenge despite modern diagnostic modalities. Herein, we report a 26-year-old woman with an incidentally documented patent ductus arteriosus and Eisenmenger syndrome. She presented with progressive dyspnea and exercise intolerance which was initially attributed to pulmonary embolus. Read More

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Thrombocytopaenia in cyanotic CHD.

Cardiol Young 2021 Mar 2;31(3):429-434. Epub 2020 Dec 2.

Department of Cardiology, University Heart Center, University Hospital Zurich, Zurich, Switzerland.

Introduction: Thrombocytopaenia is common in adults with cyanotic heart disease. Our aim was to explore potential mechanisms for thrombocytopaenia in these vulnerable patients.

Methods: Adults with cyanotic heart defects were identified from our clinical database. Read More

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Transcatheter closure for patent ductus arteriosus in patients with Eisenmenger syndrome: to do or not?

BMC Cardiovasc Disord 2020 12 1;20(1):505. Epub 2020 Dec 1.

Department of Cardiology, Shanghai East Hospital, Shanghai Tongji University School of Medicine, No. 150, Jimo Road, Pudong District, Shanghai, 200120, China.

Background: Patent ductus arteriosus (PDA) complicated by Eisenmenger syndrome (ES) remains to be a major cause of morbidity and mortality worldwide. Giving increasing evidences of benefit from targeted therapies, ES patients once thought to be inoperable may have increasing options for management. This study aims to explore whether PDA in patients with ES can be treated with transcatheter closure (TCC). Read More

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December 2020

[Dynamics of the clinical functional and hemodynamic profile of patients with pulmonary arterial hypertension with initial monotherapy with endothelin receptor antagonists: bosentan vs. macitentan].

Kardiologiia 2020 Aug 11;60(7):28-35. Epub 2020 Aug 11.

A.L. Myasnikov Research Institute of Clinical Cardiology, National Medical Research Center of Cardiology, Moscow, Russia.

Aim To compare results of 24-h treatments with bosentan and macitentan by the clinical functional status and indexes of pulmonary hemodynamics in patients with pulmonary arterial hypertension (PAH).Materials and methods Based on the Russian National Registry (NCT03707561), 44 patients older than 18 years with PAH (34 patients with idiopathic pulmonary hypertension (IPH) and 10 patients with Eisenmenger syndrome) were retrospectively included into this study. Based on the statistical method of pairwise comparison, two groups were formed and matched by age, gender, WHO functional class (FC), and 6-min walk distance (6MWD). Read More

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Cardiovascular Care for Pregnant Women With Cardiovascular Disease.

J Am Coll Cardiol 2020 11;76(18):2102-2113

Division of Cardiology, Department of Medicine, Columbia University Irving Medical Center, New York, New York. Electronic address:

Background: Cardio-obstetrics refers to a team-based approach to maternal care that includes multidisciplinary collaboration among maternal fetal medicine, cardiology, and others.

Objectives: This study sought to describe clinical characteristics, maternal and fetal outcomes, and cardiovascular readmissions in a cohort of pregnant women with underlying cardiovascular disease (CVD) followed by a cardio-obstetrics team.

Methods: We identified patients evaluated by our cardio-obstetrics team from January 1, 2010, through December 31, 2019, at a quaternary care hospital in New York City. Read More

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November 2020

Pregnancy outcome in women with an uncorrected single ventricle: A single-centre experience from South India.

Obstet Med 2020 Sep 1;13(3):137-141. Epub 2019 May 1.

Department of Cardiology, Jawaharlal Institute of Post-graduate Medical Education & Research (JIPMER), Puducherry, India.

Background: Women with an uncorrected single ventricle heart are at increased risk of adverse maternal and perinatal outcomes.

Methods: We report our experience of managing pregnant women with uncorrected single ventricles, during the time period 2011 to 2017, in a low-resource setting and compare pregnancy outcome with healthy concurrent controls. Outcomes assessed include the mode of delivery, maternal complications, neonatal death and birth weight. Read More

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September 2020

Pregnancy and pulmonary arterial hypertension-improving surveillance and outcomes with multidisciplinary care and N terminal pro-brain natriuretic peptide trends.

J Matern Fetal Neonatal Med 2020 Oct 18:1-7. Epub 2020 Oct 18.

Department of Pediatric Cardiology, Amrita Institute of Medical Sciences, Amrita Vishwa Vidyapeetham University, Kochi, India.

Objective: To describe maternal and fetal outcomes and N Terminal pro-brain natriuretic peptide (NT-proBNP) trends in pregnancy with pulmonary arterial hypertension (PAH).

Methods: The medical records of all pregnant women with PAH referred to Pulmonary Hypertension Clinic were retrospectively reviewed and analyzed.

Results: We identified 35 pregnancies in 22 women (mean age 27. Read More

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October 2020

Galectin-3 plasma levels in adult congenital heart disease and the pressure overloaded right ventricle: reason matters.

Biomark Med 2020 09;14(13):1197-1205

Department of Congenital Heart Disease, National Institute of Cardiology, Alpejska, Warsaw 04-628, Poland.

To assess galectin-3 (Gal-3) levels and their relationship with clinical status and right ventricular (RV) performance in adults with RV pressure overload of various mechanisms due to congenital heart disease. A cross-sectional study was conducted. Patients underwent clinical examination, blood testing and transthoracic echocardiography. Read More

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September 2020

Evolving Paradigms in the Treatment of Atrial Septal Defects with Pulmonary Arterial Hypertension (ASD-PAH).

Cardiol Rev 2020 Sep 18. Epub 2020 Sep 18.

Division of Cardiology, University of British Columbia, St. Paul's Hospital, Vancouver, BC.

Atrial septal defects are one of the most frequently diagnosed congenital heart defects in adulthood. The presence of concurrent moderate or severe pulmonary arterial hypertension without Eisenmenger Syndrome at the time of diagnosis can make for a challenging clinical scenario. There is continually evolving literature to determine the ideal approach to this subset of patients. Read More

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September 2020