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Journal of the American College of... Mar 2022Although major breakthroughs in the field of pediatric cardiology, cardiac surgery, intervention, and overall care improved the outlook of congenital heart disease,... (Review)
Review
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. Additionally, we delineate areas of uncertainty in various aspects of care, discuss gaps in current evidence, and review current status in less privileged countries and propose initiatives to reduce disease burden. Finally, we propose the application of emerging technologies to enhance the delivery and quality of health care related to ES and beyond.
Topics: Child; Eisenmenger Complex; Heart Defects, Congenital; Humans; Hypertension, Pulmonary; Pulmonary Arterial Hypertension
PubMed: 35331414
DOI: 10.1016/j.jacc.2022.01.022 -
Heart (British Cardiac Society) Nov 2020Eisenmenger syndrome (ES) represents the most severe phenotype of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) and occurs in... (Review)
Review
Eisenmenger syndrome (ES) represents the most severe phenotype of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) and occurs in patients with large unrepaired shunts. Despite early detection of CHD and major advances in paediatric cardiac surgery, ES is still prevalent and requires a multidisciplinary approach by adult CHD experts in tertiary centres. Central cyanosis is the primary clinical manifestation leading to secondary erythrocytosis and various multiorgan complications that increase morbidity and affect quality of life. Close follow-up is needed to early diagnose and timely manage these complications. The primary goal of care is to maintain patients' fragile stability. Although the recent use of advanced PAH therapies has substantially improved functional capacity and increased life expectancy, long-term survival remains poor. Progressive heart failure, infectious diseases and sudden cardiac death comprise the main causes of death in patients with ES. Impaired exercise tolerance, decreased arterial oxygen saturation, iron deficiency, pre-tricuspid shunts, arrhythmias, increased brain natriuretic peptide, echocardiographic indices of right ventricular dysfunction and hospitalisation for heart failure predict mortality. Endothelin receptor antagonists are used as first-line treatment in symptomatic patients, while phosphodiesterase-5 inhibitors may be added. Due to the lack of evidence, current guidelines do not provide a clear therapeutic strategy regarding treatment escalation. Additional well-designed trials are required to assess the comparative efficacy of various PAH agents and the benefit of combination therapy. Finally, the development of a risk score is of utmost importance to guide clinical therapy.
Topics: Disease Management; Echocardiography; Eisenmenger Complex; Humans; Prognosis; Quality of Life; Risk Factors
PubMed: 32690623
DOI: 10.1136/heartjnl-2020-316665 -
American Heart Journal Aug 1971
Topics: Adult; Angiocardiography; Arteriosclerosis; Autopsy; Cardiac Catheterization; Ductus Arteriosus, Patent; Eisenmenger Complex; Electrocardiography; Female; Heart Failure; Humans; Indicator Dilution Techniques; Lung; Myocardium; Pancreatitis; Pulmonary Artery; Pulmonary Circulation; Thrombosis; Vectorcardiography
PubMed: 4933981
DOI: 10.1016/0002-8703(71)90272-9 -
Brazilian Journal of Cardiovascular... 2016Eisenmenger syndrome is very rare in pregnant women. Debates remain concerning the management of Eisenmenger syndrome in this patient population and the prognosis is... (Review)
Review
Eisenmenger syndrome is very rare in pregnant women. Debates remain concerning the management of Eisenmenger syndrome in this patient population and the prognosis is unclear in terms of maternal and fetoneonatal outcomes. Epidural analgesia is preferred for Cesarean section as it alleviates perioperative pain and reduces the pulmonary and systemic vascular resistances. Maternal mortality in the presence of Eisenmenger syndrome is reported as 30-50% and even up to 65% in those with Cesarean section. The major causes of death could be hypovolemia, thromboembolism and preeclampsia. Pregnancy should ideally be avoided in a woman with Eisenmenger syndrome concerning the high maternal mortality rate and probable poor prognosis of the baby. A short labour and an atraumatic delivery under epidural block are preferred in the women with a strong desire of pregnancy. The purpose of this article is to discuss the debates of Eisenmenger syndrome in pregnancy and the possible resolutions.
Topics: Cesarean Section; Eisenmenger Complex; Female; Humans; Pregnancy; Pregnancy Complications, Cardiovascular; Pregnancy Outcome; Prognosis
PubMed: 27849306
DOI: 10.5935/1678-9741.20160062 -
Expert Review of Cardiovascular Therapy Jun 2021: Eisenmenger syndrome describes a condition in which a congenital heart defect has caused severe pulmonary vascular disease, resulting in reversed (right-left) or... (Review)
Review
: 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.: Patients with congenital heart disease (CHD) are at increased risk of developing pulmonary arterial hypertension with Eisenmenger syndrome being its extreme manifestation. All CHD patients should be regularly assessed for pulmonary hypertension. Once Eisenmenger syndrome develops, shunt closure should be avoided. The clinical manifestations of Eisenmenger syndrome are driven by the systemic effects of the pulmonary hypertension, congenital defect and long-standing cyanosis. Expert care is essential for avoiding pitfalls and preventing disease progression in this severe chronic condition, which is associated with significant morbidity and mortality. Pulmonary arterial hypertension therapies have been used alongside supportive care to improve the quality of life, exercise tolerance and the outcome of these patients, although the optimal timing for their introduction and escalation remains uncertain.
Topics: Disease Management; Disease Progression; Eisenmenger Complex; Familial Primary Pulmonary Hypertension; Humans; Practice Guidelines as Topic
PubMed: 33853494
DOI: 10.1080/14779072.2021.1917995 -
The Veterinary Record Jan 1992
Topics: Animals; Cattle; Cattle Diseases; Eisenmenger Complex; Female; Heart Failure
PubMed: 1539437
DOI: 10.1136/vr.130.1.19-c -
Respiratory Care Sep 2012Recently, advanced therapies for pulmonary arterial hypertension have become available, and have been effective in reducing pulmonary vascular resistance and symptoms in... (Review)
Review
Recently, advanced therapies for pulmonary arterial hypertension have become available, and have been effective in reducing pulmonary vascular resistance and symptoms in patients with Eisenmenger syndrome, previously thought to be inoperable. This review summarizes the current knowledge on the pathophysiology and treatment of Eisenmenger syndrome. The recent introduction of targeted therapies in pulmonary arterial hypertension has led to a renewed insight in the pathophysiology and treatment of Eisenmenger syndrome. Patients with Eisenmenger syndrome using a diagnostic-treatment-and-repair strategy are amenable to surgery after successful treatment with advanced therapy. With continued improvements in the diagnosis, preoperative management, refinement of surgical techniques and intra- and postoperative management strategies, the patients with Eisenmenger syndrome selected using a diagnostic-treatment-and-repair strategy are operable with safety and efficacy in the current era with advanced pulmonary arterial hypertension therapy. Future directions of Eisenmenger syndrome may be the combination of reversal of pulmonary vascular remodeling and correction.
Topics: Eisenmenger Complex; Endothelins; Familial Primary Pulmonary Hypertension; Humans; Hypertension, Pulmonary; Vasodilator Agents
PubMed: 22348268
DOI: 10.4187/respcare.01418 -
American Practitioner and Digest of... May 1953
Topics: Cardiovascular Abnormalities; Cardiovascular System; Eisenmenger Complex
PubMed: 13050920
DOI: No ID Found -
JAMA May 2020
Topics: Child Psychiatry; Down Syndrome; Eisenmenger Complex; Female; Heart Failure; Hope; Humans; Patient Acceptance of Health Care; Psychology, Child
PubMed: 32396184
DOI: 10.1001/jama.2020.4508 -
Progress in Cardiovascular Diseases 2002Eisenmenger's syndrome describes the elevation of pulmonary arterial pressure to the systemic level caused by increased pulmonary vascular resistance with reversal or... (Review)
Review
Eisenmenger's syndrome describes the elevation of pulmonary arterial pressure to the systemic level caused by increased pulmonary vascular resistance with reversal or bi-directional shunting through a large intracardiac or extracardiac congenital heart defect. This article reviews the natural history and pathophysiology of Eisenmenger's syndrome untreated and medical and surgical treatment options presently available. Although there is no cure for this condition at present, recent advances in management have improved the quality of life for many patients with Eisenmenger's syndrome.
Topics: Antihypertensive Agents; Eisenmenger Complex; Epoprostenol; Humans; Hypertension, Pulmonary; Pulmonary Artery; Vascular Resistance
PubMed: 12411974
DOI: 10.1053/pcad.2002.127492