1,333 results match your criteria Eisenmenger Syndrome


An update on current and emerging treatments for pulmonary arterial hypertension in childhood and adolescence.

Expert Rev Respir Med 2019 Jan 5. Epub 2019 Jan 5.

d Pediatric Cardiology Unit , University Children's Hospital HUG, Pulmonary Hypertension Program HUG, Centre Universitaire Romand de Cardiologie et Chirurgie Cardiaque Pédiatrique (CURCCCP), University of Geneva and Lausanne , Geneva and Lausanne , Switzerland .

Introduction: Pulmonary hypertension is a severe condition that can develop during childhood due to several different etiologies. During the last two decades, based on a better understanding of the underlying pathobiology leading to pulmonary arterial hypertension, targeted therapies have been developed and have improved the dreadful prognosis of the condition. However, curative therapy remains elusive. Read More

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https://www.tandfonline.com/doi/full/10.1080/17476348.2019.1
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http://dx.doi.org/10.1080/17476348.2019.1565998DOI Listing
January 2019
2 Reads

Tale of 2 Endothelin Receptor Antagonists in Eisenmenger Syndrome.

Circulation 2019 Jan;139(1):64-66

Department of Pediatric Cardiology, University of Colorado School of Medicine, Aurora (D.I., N.W.).

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http://dx.doi.org/10.1161/CIRCULATIONAHA.118.037171DOI Listing
January 2019

Evaluation of Macitentan in Patients With Eisenmenger Syndrome.

Circulation 2019 Jan;139(1):51-63

Department of Experimental, Diagnostic and Specialty Medicine-DIMES, University of Bologna, Italy (N.G.).

Background: Eisenmenger syndrome describes congenital heart disease-associated severe pulmonary hypertension accompanied by right-to-left shunting. The multicenter, double-blind, randomized, placebo-controlled, 16-week, phase III MAESTRO study (Macitentan in Eisenmenger Syndrome to Restore Exercise Capacity) evaluated the efficacy and safety of the endothelin receptor antagonist macitentan in patients with Eisenmenger syndrome.

Methods: Patients with Eisenmenger syndrome aged ≥12 years and in World Health Organization functional class II-III were randomized 1:1 to placebo or macitentan 10 mg once daily for 16 weeks. Read More

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http://dx.doi.org/10.1161/CIRCULATIONAHA.118.033575DOI Listing
January 2019
1 Read

Review of evidence for bosentan therapy for treatment of Eisenmenger syndrome.

J Am Assoc Nurse Pract 2019 Jan;31(1):72-77

Oregon Health & Science University, School of Nursing, Adult-Gerontology Acute-Care Nurse Practitioner Program, Portland, Oregon.

Background And Purpose: Eisenmenger syndrome (ES) is a rare condition caused by a right-to-left cyanotic shunt. To date, only heart-lung transplant has been shown to be curative. Bosentan is the only medication studied with a double-blind placebo-controlled randomized trial for management of this condition. Read More

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http://dx.doi.org/10.1097/JXX.0000000000000104DOI Listing
January 2019
1 Read

Floating kidneys associated with Eisenmenger syndrome.

Kidney Int 2018 Nov;94(5):1029

3rd Department of Internal Medicine, Academic Teaching Hospital Feldkirch, Feldkirch, Austria.

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https://linkinghub.elsevier.com/retrieve/pii/S00852538183036
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http://dx.doi.org/10.1016/j.kint.2018.05.010DOI Listing
November 2018
3 Reads

The impact of trisomy 21 on treatment modalities and outcome in adults with congenital heart disease in Switzerland.

Pulm Circ 2019 Jan-Mar;9(1):2045894018811147. Epub 2018 Oct 19.

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

Trisomy 21 (T21) is associated in 40-45% of cases with heart defects, most commonly shunt lesions. These defects, if not repaired, can lead to irreversible shunt-induced pulmonary hypertension (i.e. Read More

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http://dx.doi.org/10.1177/2045894018811147DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6287318PMC
October 2018

Atrial septal defects and pulmonary arterial hypertension.

J Thorac Dis 2018 Sep;10(Suppl 24):S2953-S2965

Department of Adult Congenital Heart Disease, Royal Brompton and Harefield NHS Foundation Trust, National Heart and Lung Institute, Imperial College London, London, UK.

Atrial septal defects (ASD) are a common congenital heart defect. The majority of patient with ASDs often follow an uncomplicated course of events. However, a proportion of patients with ASDs, may have their condition complicated by pulmonary hypertension (PH), with a subsequent significant impact on management, morbidity and mortality. Read More

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http://jtd.amegroups.com/article/view/23699/18018
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http://dx.doi.org/10.21037/jtd.2018.08.92DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6174141PMC
September 2018
3 Reads

Atrial septal defect with pulmonary hypertension: when/how can we consider closure?

J Thorac Dis 2018 Sep;10(Suppl 24):S2890-S2898

Glenmark Cardiac Center, Mumbai, Maharashtra, India.

Patients having atrial septal defect (ASD) with moderate and more importantly severe pulmonary arterial hypertension (PAH) pose a clinical dilemma. Closing ASD in those with irreversible PAH and not closing it when the PAH is reversible can cost patients dearly, both in terms of quality of life and longevity. In our experience, there is no single parameter that can help in decision making in this difficult subset of patients and therefore we recommend a multi-dimensional approach, which takes into consideration clinical, radiological, electrocardiographic and hemodynamic variables as a whole. Read More

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http://dx.doi.org/10.21037/jtd.2018.07.112DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6174154PMC
September 2018
2 Reads

[Method selection and perioperative management of termination of pregnancy during the first and second trimester of pregnancy with severe cardiovascular disease].

Authors:
Z L Bao J Zhang

Zhonghua Fu Chan Ke Za Zhi 2018 Sep;53(9):608-612

Department of Obstetrics and Gynecology, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China.

To summarize and analyze the methods of termination of pregnancy in the first and second trimester of pregnancy with severe cardiovascular disease. A retrospective analysis of 27 cases of termination of pregnancy in the first and second trimester of pregnancy in Beijing Anzhen Hospital from January 1, 2016 to December 30, 2017. All of these pregnant women were pregnancy complicated with severe cardiovascular disease in grade Ⅴ pregnancy risk. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0529-567x.2018.09.005DOI Listing
September 2018
12 Reads

Potts Shunt to Be Preferred Above Atrial Septostomy in Pediatric Pulmonary Arterial Hypertension Patients: A Modeling Study.

Front Physiol 2018 10;9:1252. Epub 2018 Sep 10.

Pediatric Heart Center, Justus Liebig University Giessen, Giessen, Germany.

To quantitatively evaluate the basic pathophysiological process involved in the creation of Eisenmenger syndrome in pediatric pulmonary arterial hypertension (PAH) patients by either atrial septostomy (AS) or Potts shunt (PS) as well as to predict the effects of AS or PS in future PAH patients. The multi-scale lumped parameter CircAdapt model of the cardiovascular system was used to investigate the effects of AS and PS on cardiovascular hemodynamics and mechanics, as well as on oxygen saturation in moderate to severe PAH. The reference simulation, with cardiac output set to 2. Read More

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https://www.frontiersin.org/article/10.3389/fphys.2018.01252
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http://dx.doi.org/10.3389/fphys.2018.01252DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6139355PMC
September 2018
4 Reads

Congenital Left Ventricular-Right Atrial Communication Gerbode-Type Defect.

J Heart Valve Dis 2017 11;26(6):738-740

Department of Medicine, Division of Cardiology, Maimonides Medical Center, Brooklyn, NY, USA.

Adult congenital heart diseases present a unique challenge in assessing right-sided cardiac chambers, where pressures can be mistakenly calculated using standard echocardiographic formulae. A challenging case is presented of a combined inlet ventricular septal defect and ventriculo-atrial Gerbode defect. The diagnosis of such adult congenital heart diseases requires an in-depth understanding of cardiac pathophysiology and hemodynamics. Read More

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November 2017
14 Reads

Effect of Patent Foramen Ovale in Patients With Pulmonary Hypertension.

Am J Cardiol 2018 Aug 1;122(3):505-510. Epub 2018 May 1.

Duke University Medical Center, Durham, North Carolina. Electronic address:

Septostomy reduces right ventricular (RV) workload at the expense of hypoxemia in patients with advanced pulmonary hypertension (PH). A patent foramen ovale (PFO) may serve as a "natural" septostomy, but the incidence and impact of a PFO in PH remains uncertain. We prospectively examined echocardiograms in 404 PH patients referred for initial hemodynamic assessment. Read More

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http://dx.doi.org/10.1016/j.amjcard.2018.04.014DOI Listing
August 2018
25 Reads

Dental considerations and recommendations in Eisenmenger syndrome: A report of an unusual paediatric case.

Eur Arch Paediatr Dent 2018 Dec 8;19(6):449-453. Epub 2018 Sep 8.

Paediatric Dentistry Postgraduate Programme, Faculty of Dentistry, San Luis Potosi University, San Luis Potosí, SLP, Mexico.

Background: Eisenmenger syndrome (ES) is a heart cyanotic condition characterised by elevated pulmonary vascular resistance and an intra-cardiac right-to-left shunting of blood through a systemic-to-pulmonary circulation connection. Affected children usually exhibit severe hypoxia, clubbing of fingers/toes, haemoptysis, anaemia, and organ damage.

Case Report: During autumn 2015, the patient and her parents arrived at the paediatric dentistry clinic. Read More

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http://link.springer.com/10.1007/s40368-018-0366-4
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http://dx.doi.org/10.1007/s40368-018-0366-4DOI Listing
December 2018
10 Reads

Eisenmenger Syndrome in Adults: Treatment Pattern and Prognostic Factors in the Advanced Pulmonary Vasodilator Era.

Pediatr Cardiol 2018 Aug 18. Epub 2018 Aug 18.

Department of Pediatrics, Sejong General Hospital, Bucheon, South Korea.

Patients with Eisenmenger syndrome (ES) have a higher mortality rate than patients with simple congenital heart disease (CHD). To determine factors associated with death in the era of advanced pulmonary vasodilator treatment, we analyzed the characteristics of adult ES patients depending on underlying CHD. Simple septal defects and patent ductus arteriosus were classified as simple CHD, and other conditions were classified as complex CHD. Read More

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http://dx.doi.org/10.1007/s00246-018-1956-yDOI Listing
August 2018
2 Reads

Management dilemmas in pulmonary arterial hypertension associated with congenital heart disease.

Pulm Circ 2018 Jul-Sep;8(3):2045894018792501. Epub 2018 Jul 23.

4 Department of Congenital Heart Disease, University Hospitals Bristol NHS Foundation Trust, UK.

There are few randomised controlled data to guide management of patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD). In this clinical review, common areas of uncertainty in the management of PAH-CHD are identified, the literature is summarised and discussed and a suggested approach offered for each clinical dilemma. Read More

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http://dx.doi.org/10.1177/2045894018792501DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6161209PMC

A Multifaceted Approach to Pulmonary Hypertension in Adults With Congenital Heart Disease.

Prog Cardiovasc Dis 2018 Sep - Oct;61(3-4):320-327. Epub 2018 Jul 18.

Department of Cardiovascular Medicine, Duke University Health System, Durham, NC. Electronic address:

Advances in the management of congenital heart disease (CHD) in children have resulted in growing numbers of adults with CHD. Pulmonary arterial hypertension related to CHD (PAH-CHD) is a common complication, affecting up to 10% of patients; and can arise even after successful and complete defect repair, with severe and potentially fatal consequences. Careful work-up in these patients is essential, particularly hemodynamic assessment, and can help define the most appropriate therapeutic approach. Read More

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http://dx.doi.org/10.1016/j.pcad.2018.07.017DOI Listing
December 2018
8 Reads

Eisenmenger syndrome - an electrocardiographic and echocardiographic assessment of the right ventricle.

Bratisl Lek Listy 2018;119(6):321-329

Background: Eisenmenger syndrome represents severe, irreversible, and end-stage pulmonary arterial hypertension (PAH) associated with congenital heart defects. For long-term outcome optimal right ventricular (RV) adaptation is crucial with precise assessment of its hypertrophy, dilatation and function.

Objectives: Associations of electrocardiographic (ECG) and echocardiographic (ECHO) RV characteristics were analyzed. Read More

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http://dx.doi.org/10.4149/BLL_2018_060DOI Listing
November 2018
1 Read

Eisenmenger Syndrome in Pregnancy: When Is It Time for ECMO?: A Case Report.

A A Pract 2018 Nov;11(10):270-272

From the Departments of Anesthesiology.

We report the case of a 21-year-old primiparous woman at 22 weeks gestation who presented with a large uncorrected ventricular septal defect, severe pulmonary hypertension, and Eisenmenger syndrome. The patient elected for termination of pregnancy, which was performed under regional anesthesia. Hemodynamic changes apparently associated with uterine contraction immediately after termination resulted in increased right to left shunting across the ventricular septal defect requiring urgent venovenous extracorporeal membrane oxygenation. Read More

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http://Insights.ovid.com/crossref?an=02054229-201811150-0000
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http://dx.doi.org/10.1213/XAA.0000000000000806DOI Listing
November 2018
19 Reads

Use of intravenous iron in cyanotic patients with congenital heart disease and/or pulmonary hypertension.

Int J Cardiol 2018 Sep 19;267:79-83. Epub 2018 May 19.

Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK; National Heart and Lung Institute, Imperial College School of Medicine, London, UK. Electronic address:

Background: Secondary erythrocytosis is common in patients with cyanosis secondary to congenital heart disease (CHD) and/or pulmonary hypertension (PH). This compensatory mechanism aims at increasing oxygen delivery to the tissues, but it requires adequate iron stores. Optimal methods of iron supplementation in this setting remain controversial, with fears of excessive erythropoiesis and hyperviscosity symptoms. Read More

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http://dx.doi.org/10.1016/j.ijcard.2018.05.062DOI Listing
September 2018
3 Reads

Dual endothelin-1 receptor antagonism attenuates platelet-mediated derangements of blood coagulation in Eisenmenger syndrome.

J Thromb Haemost 2018 May 26. Epub 2018 May 26.

School of Medicine and Medical Science, University College Dublin, Dublin, Ireland.

Essentials Eisenmenger syndrome is characterised by thrombotic and hemorrhagic risks of unclear aetiology. Calibrated automated thrombography was used to assess these coagulation derangements. Platelet activity supported abnormalities in procoagulant and anticoagulant pathway function. Read More

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http://dx.doi.org/10.1111/jth.14159DOI Listing

Pulmonary hypertension in congenital heart disease.

Future Cardiol 2018 07 24;14(4):343-353. Epub 2018 May 24.

Department of Congenital Heart Disease, Bristol Heart Institute, Upper Maudlin Street, Bristol, BS2 8BJ, UK.

Pulmonary hypertension is defined as a mean pulmonary arterial pressure ≥25 mmHg. We focus on its relevance in congenital heart disease, reviewing pathophysiology, diagnosis and management. Pulmonary hypertension is a relatively common complication of congenital heart disease, with adult prevalence between 5 and 10%. Read More

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http://dx.doi.org/10.2217/fca-2017-0065DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6136120PMC
July 2018
4 Reads

Arrhythmias in adult patients with congenital heart disease and pulmonary arterial hypertension.

Heart 2018 Dec 18;104(23):1963-1969. Epub 2018 May 18.

Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, National Heart and Lung Institute, Imperial College London, London, UK.

Objectives: Approximately 5%-10% of adults with congenital heart disease (CHD) develop pulmonary arterial hypertension (PAH), which affects life expectancy and quality of life. Arrhythmias are common among these patients, but their incidence and impact on outcome remains uncertain.

Methods: All adult patients with PAH associated with CHD (PAH-CHD) seen in a tertiary centre between 2007 and 2015 were followed for new-onset atrial or ventricular arrhythmia. Read More

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http://dx.doi.org/10.1136/heartjnl-2017-312881DOI Listing
December 2018
4 Reads

Congenital heart disease in adults: Assessmentof functional capacity using cardiopulmonary exercise testing.

Rev Port Cardiol 2018 May;37(5):399-405

Cardiology Department, Santa Marta Hospital, Lisbon, Portugal.

Aim: The aim of the study was to compare functional capacity in different types of congenital heart disease (CHD), as assessed by cardiopulmonary exercise testing (CPET).

Methods: A retrospective analysis was performed of adult patients with CHD who had undergone CPET in a single tertiary center. Diagnoses were divided into repaired tetralogy of Fallot, transposition of the great arteries (TGA) after Senning or Mustard procedures or congenitally corrected TGA, complex defects, shunts, left heart valve disease and right ventricular outflow tract obstruction. Read More

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http://dx.doi.org/10.1016/j.repc.2017.09.020DOI Listing
May 2018
1 Read

Pulmonary arterial hypertension in adult congenital heart disease.

Heart 2018 Oct 2;104(19):1568-1574. Epub 2018 May 2.

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

Pulmonary arterial hypertension (PAH) is commonly associated with congenital heart disease (CHD) and relates to type of the underlying cardiac defects and repair history. Large systemic to pulmonary shunts may develop PAH if untreated or repaired late. PAH, when present, markedly increases morbidity and mortality in patients with CHD. Read More

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http://dx.doi.org/10.1136/heartjnl-2017-312106DOI Listing
October 2018
1 Read

A Focus on Macitentan in the Treatment of Pulmonary Arterial Hypertension.

Basic Clin Pharmacol Toxicol 2018 Aug 5;123(2):103-113. Epub 2018 Jun 5.

Clinic for Plastic, Aesthetic and Hand Surgery, Otto-von-Guericke-University Magdeburg, Magdeburg, Germany.

The approval of macitentan has increased the number of pharmacological treatments of pulmonary arterial hypertension (PAH). Here, we review the effect on PAH of macitentan compared to other endothelin receptor antagonists. Drugs targeting the endothelin (ET) pathway include the selective ET receptor antagonist ambrisentan, the ET /ET receptor antagonists, bosentan and macitentan, which were recently approved for PAH treatment. Read More

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http://dx.doi.org/10.1111/bcpt.13033DOI Listing
August 2018
2 Reads

Case Report of an Awake Craniotomy in a Patient With Eisenmenger Syndrome.

A A Pract 2018 May;10(9):219-222

From the Department of Anesthesiology, Perioperative and Pain Medicine, Stanford University School of Medicine, Stanford, California.

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. Read More

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http://dx.doi.org/10.1213/XAA.0000000000000664DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6309536PMC
May 2018
1 Read

Contemporary survival of patients with pulmonary arterial hypertension and congenital systemic to pulmonary shunts.

PLoS One 2018 17;13(4):e0195092. Epub 2018 Apr 17.

Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.

Objective: To compare survival of patients with newly diagnosed pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) according to various clinical classifications with classifications of anatomical-pathophysiological systemic to pulmonary shunts in a single-center cohort.

Methods: All prevalent cases of PAH-CHD with hemodynamic confirmation by cardiac catheterization in 1995-2015 were retrospectively reviewed. Patients who were younger than three months of age, or with single ventricle following surgery were excluded. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0195092PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5903600PMC
July 2018
16 Reads

Increased microvolt T-wave alternans in children and adolescents with Eisenmenger syndrome.

Anatol J Cardiol 2018 May 10;19(5):303-310. Epub 2018 Apr 10.

Department of Pediatric Cardiology, Faculty of Medicine, Mersin University; Mersin-Turkey.

Objective: To determine the values of microvolt T-wave alternans (MTWA) in children and adolescents with Eisenmenger syndrome (ES) and controls.

Methods: Thirteen were included in the study. After analyzing the 24-h ECG recordings, MTWA was considered using three leads (V5, V1, and aVF). Read More

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http://dx.doi.org/10.14744/AnatolJCardiol.2018.60487DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6280262PMC
May 2018
1 Read

Adult Congenital Heart Disease with Pregnancy.

Authors:
Koichiro Niwa

Korean Circ J 2018 Apr;48(4):251-276

Department of Cardiology, Cardiovascular Center, St. Luke's International Hospital, Tokyo, Japan.

The number of women with congenital heart disease (CHD) at risk of pregnancy is growing because over 90% of them are grown-up into adulthood. The outcome of pregnancy and delivery is favorable in most of them provided that functional class and systemic ventricular function are good. Women with CHD such as pulmonary hypertension (Eisenmenger syndrome), severe left ventricular outflow stenosis, cyanotic CHD, aortopathy, Fontan procedure and systemic right ventricle (complete transposition of the great arteries [TGA] after atrial switch, congenitally corrected TGA) carry a high-risk. Read More

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http://dx.doi.org/10.4070/kcj.2018.0070DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5889976PMC
April 2018
5 Reads

Selexipag in Congenital Heart Disease-Associated Pulmonary Arterial Hypertension and Eisenmenger Syndrome: First Report.

Am J Ther 2018 Nov/Dec;25(6):e714-e715

Department of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, The Ohio State University Wexner Medical Center, Columbus, OH.

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http://dx.doi.org/10.1097/MJT.0000000000000727DOI Listing
March 2018
4 Reads

International Trade Drives Global Resource Use: A Structural Decomposition Analysis of Raw Material Consumption from 1990-2010.

Environ Sci Technol 2018 Apr 14;52(7):4190-4198. Epub 2018 Mar 14.

Institute of Social Ecology , Schottenfeldgasse 29 , A-1070 Vienna , Austria.

Globalization led to an immense increase of international trade and the emergence of complex global value chains. At the same time, global resource use and pressures on the environment are increasing steadily. With these two processes in parallel, the question arises whether trade contributes positively to resource efficiency, or to the contrary is further driving resource use? In this article, the socioeconomic driving forces of increasing global raw material consumption (RMC) are investigated to assess the role of changing trade relations, extended supply chains and increasing consumption. Read More

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http://dx.doi.org/10.1021/acs.est.7b06133DOI Listing
April 2018
11 Reads

Evaluation of Tissue Doppler Echocardiographic Imaging findings in children with pulmonary hypertension.

Turk J Pediatr 2017 ;59(3):244-253

Division of Pediatric Cardiology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Akın A, Alehan D, Aykan HH, Özkutlu S, Özer S, Karagöz T. Evaluation of Tissue Doppler Echocardiographic Imaging findings in children with pulmonary hypertension. Turk J Pediatr 2017; 59: 244-253. Read More

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http://dx.doi.org/10.24953/turkjped.2017.03.003DOI Listing
December 2018
16 Reads

Percutaneous Coronary Intervention for a Patient with Left Main Coronary Compression Syndrome.

Intern Med 2018 May 11;57(10):1421-1424. Epub 2018 Jan 11.

Department of Cardiovascular Biology and Medicine, Niigata University Graduate School of Medical and Dental Sciences, Japan.

Left main coronary compression syndrome rarely occurs in patients with severe pulmonary hypertension. A 65-year-old woman with severe pulmonary hypertension due to an atrial septal defect suffered from angina on effort. Cardiac computed-tomography and coronary angiography revealed considerable stenosis of the left main coronary artery (LMA) caused by compression between the dilated main pulmonary artery trunk and the sinus of valsalva. Read More

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http://dx.doi.org/10.2169/internalmedicine.9534-17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5995705PMC
May 2018
6 Reads

Secundum atrial septal defect with Eisenmenger syndrome: A scarcely reported disease in Nigeria.

Niger Med J 2017 Mar-Apr;58(2):81-83

Department of Physiotherapy, University of Nigeria Teaching Hospital, Ituku-Ozalla, Enugu, Nigeria.

Atrial septal defect (ASD) may be rarely associated with Eisenmenger syndrome (ES), the most advanced form of pulmonary vascular disease to complicate a congenital heart disease. In spite of availability of pediatric cardiologists and two-dimensional-echochardiography in many tertiary health care centers in Nigeria, late referral and late diagnosis of ASD still occur leading to the development of ES. We describe a case of a 19-year-old male with ASD who presented late with ES. Read More

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http://dx.doi.org/10.4103/0300-1652.219346DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5726179PMC
December 2017
3 Reads

Incidence and clinical characteristics of sudden cardiac death in adult congenital heart disease.

Int J Cardiol 2018 03 5;254:101-106. Epub 2017 Dec 5.

University of Sydney Medical School and Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia. Electronic address:

Background: The life expectancy of adults with congenital heart disease (CHD) has significantly improved in recent decades, with non-cardiovascular causes of death now competing with traditional cardiovascular causes. The risk of sudden cardiac death (SCD), a devastating event, still remains elevated above that of the general population.

Methods: We reviewed 2935 patients in our adult CHD database (age≥16years, seen at least once in our centre) and documented all cases of SCD between 2000-2015. Read More

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http://dx.doi.org/10.1016/j.ijcard.2017.11.117DOI Listing
March 2018
6 Reads

A Case of Advanced Glaucoma with Increased Episcleral Venous Pressure in a 17-Year-Old with Eisenmenger Syndrome.

Case Rep Ophthalmol Med 2017 23;2017:5808047. Epub 2017 Oct 23.

Mater Dei Hospital, Msida, Malta.

Eisenmenger syndrome refers to reversal of shunt and central cyanosis due to pulmonary hypertension induced by congenital heart disease with a large systemic-to-pulmonary shunt. We report a case of a 17-year-old man with Eisenmenger syndrome who presented with gradual deterioration in visual acuity and was diagnosed with advanced secondary open angle glaucoma. There have been reports of patients suffering from thrombosis due to hyperviscosity associated with this syndrome; however, to our knowledge, the association of secondary open angle glaucoma with Eisenmenger syndrome has not yet been documented. Read More

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http://dx.doi.org/10.1155/2017/5808047DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5672606PMC
October 2017
15 Reads

Eisenmenger Syndrome Revisited.

Authors:
Gaetano Thiene

World J Pediatr Congenit Heart Surg 2017 11;8(6):726-734

1 Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, Padua, Italy.

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http://dx.doi.org/10.1177/2150135117727259DOI Listing
November 2017
3 Reads

Feasibility of real-time molecular profiling for patients with newly diagnosed glioblastoma without MGMT promoter hypermethylation-the NCT Neuro Master Match (N2M2) pilot study.

Neuro Oncol 2018 05;20(6):826-837

Department of Neuropathology, Institute of Pathology, University Hospital Heidelberg, Heidelberg, Germany.

Background: O6-methylguanine-DNA methyltransferase (MGMT) promoter methylation status is a predictive biomarker in glioblastoma patients. Glioblastoma without hypermethylated MGMT promoter is largely resistant to treatment with temozolomide. These patients are in particular need of new treatment approaches, which are offered by biomarker-driven clinical trials with targeted drugs based on molecular characterization of individual tumors. Read More

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https://academic.oup.com/neuro-oncology/article/20/6/826/464
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http://dx.doi.org/10.1093/neuonc/nox216DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5961072PMC
May 2018
66 Reads

Non-invasive assessment of liver changes in Eisenmenger patients.

Int J Cardiol 2017 Dec;249:140-144

Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Centre Munich, Technical University of Munich (TUM), Munich, Germany. Electronic address:

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. Read More

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http://dx.doi.org/10.1016/j.ijcard.2017.07.029DOI Listing
December 2017
17 Reads

A Rare Case of Hemopericardium From a Ruptured Dissecting Pulmonary Artery Aneurysm: Postmortem Computed Tomography Scan and Autopsy Findings.

Am J Forensic Med Pathol 2018 Mar;39(1):50-53

Nontraumatic hemopericardium is a potentially fatal pathology that is most commonly caused by myocardial wall rupture, ruptured aortic dissection, aortic aneurysm rupture, or a neoplastic process. A rare potential cause of hemopericardium is pulmonary artery dissection, with less than 100 reported cases in the literature. Pulmonary artery dissection is associated with pulmonary artery aneurysm, pulmonary artery hypertension, and congenital heart disease. Read More

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http://dx.doi.org/10.1097/PAF.0000000000000362DOI Listing
March 2018
17 Reads

Age is not a good predictor of irreversibility of pulmonary hypertension in congenital cardiac malformations with left-to-right shunt.

Congenit Heart Dis 2018 Mar 25;13(2):210-216. Epub 2017 Oct 25.

Department of Pediatric Cardiology, University Hospital of Vaud, Lausanne, Switzerland.

Objective: Congenital cardiac malformations with high pulmonary blood flow and pressure due to left-to-right shunts are usually repaired in early infancy for both the benefits of early relief of heart failure and the fear that the concomitant pulmonary hypertension may become irreversible unless these defects are corrected at an early age. Age, however, has been a poor predictor of irreversibility of pulmonary hypertension in our experience, which is presented here.

Design: A retrospective observational study. Read More

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http://dx.doi.org/10.1111/chd.12545DOI Listing
March 2018
17 Reads

Managing the Eisenmenger syndrome: a call to arms.

Authors:
Edgar L W Tay

Heart 2018 05 23;104(9):714-716. Epub 2017 Oct 23.

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http://dx.doi.org/10.1136/heartjnl-2017-312215DOI Listing
May 2018
9 Reads

Successful Pulmonary Artery Embolization for the Management of Hemoptysis in a Patient with Eisenmenger Syndrome Caused by Patent Ductus Arteriosus.

Intern Med 2017 Dec 11;56(24):3299-3304. Epub 2017 Oct 11.

Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine, Japan.

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. Read More

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http://dx.doi.org/10.2169/internalmedicine.9071-17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5790717PMC
December 2017
15 Reads

The Efficacy of a Genetic Analysis of the BMPR2 Gene in a Patient with Severe Pulmonary Arterial Hypertension and an Atrial Septal Defect Treated with Bilateral Lung Transplantation.

Intern Med 2017 Dec 11;56(23):3193-3197. Epub 2017 Oct 11.

Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine, Japan.

Severe pulmonary arterial hypertension (PAH) rarely develops in children with an atrial septal defect (ASD), even those with a large defect. We herein report the case of a 27-year-old man with a moderate-sized secundum ASD and right ventricular failure due to severe PAH, which developed in his early teens. He was diagnosed as having a genetic mutation of the bone morphogenetic protein receptor-2 (BMPR2) gene and was successfully treated with bilateral lung transplantation with ASD path closure. Read More

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http://dx.doi.org/10.2169/internalmedicine.8686-16DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5742392PMC
December 2017
19 Reads

MicroRNA-424(322) as a new marker of disease progression in pulmonary arterial hypertension and its role in right ventricular hypertrophy by targeting SMURF1.

Cardiovasc Res 2018 Jan;114(1):53-64

CNC.IBILI, University of Coimbra, Coimbra, Portugal.

Aims: 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 signalling pathways whereby miR-424(322) secreted by pulmonary arterial endothelial cells (PAECs) impacts cardiomyocytes. Read More

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http://dx.doi.org/10.1093/cvr/cvx187DOI Listing
January 2018
30 Reads

Severe pulmonary hypertension and right ventricular failure.

Indian J Anaesth 2017 Sep;61(9):753-759

Department of Anaesthesiology, All India Institute of Medical Sciences, Raipur, Chhattisgarh, India.

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. Read More

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http://dx.doi.org/10.4103/ija.IJA_420_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5613601PMC
September 2017
11 Reads

Prognostic Value of Pulmonary Artery Compliance in Patients with Pulmonary Arterial Hypertension Associated with Adult Congenital Heart Disease.

Int Heart J 2017 Oct 30;58(5):731-738. Epub 2017 Sep 30.

State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College.

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. Read More

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http://dx.doi.org/10.1536/ihj.16-449DOI Listing
October 2017
20 Reads