Materiały źródłowe

• #1 Eisenmenger syndrome: current perspectives | RRCC

  https://www.dovepress.com/eisenmenger-syndrome-current-perspectives-peer-reviewed-fulltext-article-RRCC

  Eisenmenger syndrome (ES) is the most severe form of pulmonary arterial hypertension (PAH) related to congenital heart disease (CHD). […] With advances in pediatric cardiology and surgery, the prevalence of ES is steadily falling in developed countries; nonetheless, there will always be patients who are unsuitable for repair at the time of diagnosis, or emigrating from countries with less advanced healthcare, who will develop ES. […] The exact prevalence of ES is not known. According to historical data and before the advent of timely intervention, ~8% of patients with CHD and 11% of those with left-to-right shunts developed ES. […] Enhanced understanding and timely surgical or interventional repair of defects has resulted in a substantial reduction in the numbers of patients developing ES. Nevertheless, this condition will continue to occur in patients unsuitable for early repair and individuals from countries where availability of tertiary pediatric cardiology and cardiothoracic surgery is limited.

• #2 Eisenmenger syndrome

  https://www.redalyc.org/journal/5760/576068163002/html/

  Eisenmenger syndrome (ES) stands for pulmonary hypertension, reversal of the central shunts flow, and cyanosis secondary to any congenital heart defect (CHD) associated with non-repaired intra- or extra-cardiac communication. […] The population of adults with congenital heart disease (known under the acronym of GUCH: grown-up congenital heart disease) is growing at an acceleration rate of 5% per year. In the USA, more than 1 million adults have CHD. 10% of the cases present pulmonary hypertension, 30% of them due to unrepaired defects. ES will develop in half of these patients with unrepaired cardiovascular defects. […] Although long-term survival has been reported, patients with ES usually die between 30 and 35 years old.

• #3 [Eisenmenger syndrome. Epidemiology and natural history] – PubMed

  https://pubmed.ncbi.nlm.nih.gov/20141995/

  Eisenmenger syndrome is the last-stage of congenital heart diseases with untreated left to right shunt. […] However, patients follow-up is often performed in inappropriate non-specialized care centres which makes the collection of epidemiological data difficult. […] Consequently, population registries are necessary and would increase knowledge of natural history.

• #4 Eisenmenger syndrome: current perspectives | RRCC

  https://www.dovepress.com/eisenmenger-syndrome-current-perspectives-peer-reviewed-fulltext-article-RRCC

  Eisenmenger syndrome (ES) is the most severe form of pulmonary arterial hypertension (PAH) related to congenital heart disease (CHD). […] With advances in pediatric cardiology and surgery, the prevalence of ES is steadily falling in developed countries; nonetheless, there will always be patients who are unsuitable for repair at the time of diagnosis, or emigrating from countries with less advanced healthcare, who will develop ES. […] The exact prevalence of ES is not known. According to historical data and before the advent of timely intervention, ~8% of patients with CHD and 11% of those with left-to-right shunts developed ES. […] Enhanced understanding and timely surgical or interventional repair of defects has resulted in a substantial reduction in the numbers of patients developing ES. Nevertheless, this condition will continue to occur in patients unsuitable for early repair and individuals from countries where availability of tertiary pediatric cardiology and cardiothoracic surgery is limited.

• #5 Postoperative Management of Eisenmenger’s Syndrome

  https://www.oatext.com/postoperative-management-of-eisenmengers-syndrome.php

  Eisenmengers Syndrome (ES) is defined by the triad of systemic-to-pulmonary congenital cardiovascular communication, pulmonary arterial hypertension, and cyanosis. Modern advances, enhanced understanding of pediatric cardiology, and timely surgical intervention in the management of congenital heart defects (CHDS) has resulted in the decreased prevalence of ES by 50%. An estimated 8% of patients with congenital defects will develop ES and 11% with CHDS plus left to right shunt physiology. CHDS commonly associated with ES includes atrial septal defects (ASDs), atrioventricular septal defects, ventricular septal defects (VSDs) and patent ductus arteriosus (PDA). Most patients with ES survive for 20 to 30 years. The survival of patients with pulmonary hypertension and ES into the 5th and 6th decades of life increase the likelihood of exposure to non-cardiac surgery. Eisenmengers Syndrome is associated with perioperative complications including early and sudden postoperative death. Non-cardiac surgeries amongst ES patients carry a mortality risk as high as 30%.

• #6 Eisenmenger’s syndrome epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Eisenmenger%E2%80%99s_syndrome_epidemiology_and_demographics

  The incidence and prevalence of Eisenmenger’s syndrome has been decreasing gradually over the years. In the general population, the prevalence decreased from 24.6 to 11.9/million inhabitants in 2012. About 8% of patents with congenital heart diseases develop Eisenmenger’s syndrome. The mortality rate of Eisenmenger’s syndrome is about 27%. The risk of Eisenmenger’s syndrome increases in patients with large cardiac defects or who live in developing countries due to poor healthcare access. […] The incidence of Eisenmenger’s syndrome decreased from 2.5/million individuals/year in 1977 to 0.2/million individuals/year in 2012. […] Overall, about 8% of patients with congenital heart disease develop Eisenmenger’s syndrome. […] In the general population, the prevalence decreased from 24.6 to 11.9/million individuals in 2012.

• #7 Eisenmenger’s syndrome epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Eisenmenger%E2%80%99s_syndrome_epidemiology_and_demographics

  The incidence and prevalence of Eisenmenger’s syndrome has been decreasing gradually over the years. In the general population, the prevalence decreased from 24.6 to 11.9/million inhabitants in 2012. About 8% of patents with congenital heart diseases develop Eisenmenger’s syndrome. The mortality rate of Eisenmenger’s syndrome is about 27%. The risk of Eisenmenger’s syndrome increases in patients with large cardiac defects or who live in developing countries due to poor healthcare access. […] The incidence of Eisenmenger’s syndrome decreased from 2.5/million individuals/year in 1977 to 0.2/million individuals/year in 2012. […] Overall, about 8% of patients with congenital heart disease develop Eisenmenger’s syndrome. […] In the general population, the prevalence decreased from 24.6 to 11.9/million individuals in 2012.

• #8 Eisenmenger’s Effects

  http://www.rnceus.com/vsd/eisen25.html

  Eisenmenger syndrome (ES) is a severe form of pulmonary arterial hypertension (PAH) associated with congenital heart defects (CHD). The prevalence of ES varies: it is encountered in approximately 1% to 5.6% of large tertiary CHD cohorts. Among patients with PAH associated with CHD, the prevalence of ES ranges from 1.1% to 12.3%, depending on the registry and population studied. The incidence of ES has declined in high-income countries due to early diagnosis and surgical correction of CHD. However, in low- and middle-income countries, ES remains more prevalent due to limited access to timely medical care and surgical interventions (Arvanitaki 2022).

• #9 Eisenmenger’s Effects

  http://www.rnceus.com/vsd/eisen25.html

  Eisenmenger syndrome (ES) is a severe form of pulmonary arterial hypertension (PAH) associated with congenital heart defects (CHD). The prevalence of ES varies: it is encountered in approximately 1% to 5.6% of large tertiary CHD cohorts. Among patients with PAH associated with CHD, the prevalence of ES ranges from 1.1% to 12.3%, depending on the registry and population studied. The incidence of ES has declined in high-income countries due to early diagnosis and surgical correction of CHD. However, in low- and middle-income countries, ES remains more prevalent due to limited access to timely medical care and surgical interventions (Arvanitaki 2022).

• #10 The Belgian Eisenmenger syndrome registry: Implications for treatment strategies?

  https://lirias.kuleuven.be/97056

  OBJECTIVE: Pulmonary arterial hypertension (PAH), associated with congenital heart disease (CHD), usually results from a systemic-to-pulmonary shunt. […] A national registry of ES patients was initiated to optimise patient care and to provide epidemiological information regarding PAH and CHD in Belgium. […] Through the national Eisenmenger registry, 91 adult patients with ES were identified (estimated prevalence II per million inhabitants).

• #11 Eisenmenger Syndrome: Causes, Symptoms and Treatment

  https://my.clevelandclinic.org/health/diseases/17921-eisenmenger-syndrome

  Eisenmenger syndrome is rare. It occurs in 1% to 6% of adults born with a heart defect. […] People with Eisenmenger syndrome have shorter life expectancies due to the wide range of complications that can accompany the disease. However, some people survive until middle age. Survival rates vary based on the severity of the symptoms and the underlying congenital heart defect.

• #12 Treatment of adults with Eisenmenger syndrome—state of the art in the 21st century: a short overview – Diller – Cardiovascular Diagnosis and Therapy

  https://cdt.amegroups.org/article/view/74370/html

  Eisenmenger syndrome (ES) develops in association with unrepaired, non-restrictive cardiac shunt lesions at the atrial, ventricular or arterial level over time. […] The prevalence of Eisenmenger syndrome is not well defined but traditional estimates suggest that around 5% of ACHD patients under follow-up at large supra-regional centers have Eisenmenger syndrome. […] The leading reasons for mortality are sudden cardiac death, progressive heart failure, and infectious diseases. […] Follow-up and care at specialist congenital heart disease centers is highly recommended to prevent, to early diagnose and to timely manage complications of ES. […] In patients presenting late with established Eisenmenger syndrome, shunt closure is contraindicated as this will worsen prognosis in this setting.

• #13

  https://step2.medbullets.com/cardiovascular/120014/eisenmenger-syndrome

  Epidemiology […] Risk factors […] congenital heart defect […] […] […] Eisenmenger syndrome is associated with congenital heart defects that lead to pulmonary arterial hypertension.

• #14 Eisenmenger Syndrome: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/154555-overview

  Eisenmenger syndrome usually develops before puberty, but it may also start manifesting in adolescence and early adulthood. Approximately 8% of patients with congenital heart disease develop Eisenmenger syndrome. […] The prevalence of Eisenmenger syndrome is difficult to quantify, but it is declining in the developed world with the identification and surgical correction of congenital heart conditions. Patients from underdeveloped countries are more likely to have late presentations of Eisenmenger syndrome due to uncorrected congenital cardiac lesions. […] The frequency of pulmonary hypertension and the subsequent development of reversed shunting vary depending on the heart defect and operative intervention. Such variations include the following: Large, nonrestrictive ventricular septal defect (VSD) or patent ductus arteriosus (PDA): Approximately 50% of infants with one of these defects develop pulmonary hypertension by early childhood. VSD or PDA and transposition of the great arteries: About 40% of patients develop pulmonary hypertension within the first year of life. Large secundum atrial septal defect (ASD): The natural history of a large secundum ASD differs in that the 10% of cases that progress to pulmonary hypertension do so more slowly and usually not until after the third decade of life. Persistent truncus arteriosus and unrestricted pulmonary blood flow: All patients develop severe pulmonary hypertension by the second year of life. Common atrioventricular canal: Almost all patients develop severe pulmonary hypertension by the second year of life. Surgically-created systemic-to-pulmonary shunt: The frequency of pulmonary hypertension varies depending on size and anatomy.

• #15 Eisenmenger Syndrome: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/154555-overview

  Eisenmenger syndrome usually develops before puberty, but it may also start manifesting in adolescence and early adulthood. Approximately 8% of patients with congenital heart disease develop Eisenmenger syndrome. […] The prevalence of Eisenmenger syndrome is difficult to quantify, but it is declining in the developed world with the identification and surgical correction of congenital heart conditions. Patients from underdeveloped countries are more likely to have late presentations of Eisenmenger syndrome due to uncorrected congenital cardiac lesions. […] The frequency of pulmonary hypertension and the subsequent development of reversed shunting vary depending on the heart defect and operative intervention. Such variations include the following: Large, nonrestrictive ventricular septal defect (VSD) or patent ductus arteriosus (PDA): Approximately 50% of infants with one of these defects develop pulmonary hypertension by early childhood. VSD or PDA and transposition of the great arteries: About 40% of patients develop pulmonary hypertension within the first year of life. Large secundum atrial septal defect (ASD): The natural history of a large secundum ASD differs in that the 10% of cases that progress to pulmonary hypertension do so more slowly and usually not until after the third decade of life. Persistent truncus arteriosus and unrestricted pulmonary blood flow: All patients develop severe pulmonary hypertension by the second year of life. Common atrioventricular canal: Almost all patients develop severe pulmonary hypertension by the second year of life. Surgically-created systemic-to-pulmonary shunt: The frequency of pulmonary hypertension varies depending on size and anatomy.

• #16 Eisenmenger syndrome | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/eisenmenger-syndrome?embed_domain=hackmd.io%252525252525252F%2525252525252540yIPUAFeCSL2JsU8smR5nJQ%252525252525252Fbnjhjgjghjghjghfavicon.icoradiopaedia-icon-144.png&lang=us

  In general, the shunts that lead to Eisenmenger syndrome share high pressure and high flow. As such the three lesions that account for most cases are: […] In this group of patients, the gender distribution is equal, and it is uncommon to develop Eisenmenger syndrome before the age of 2. […] In contrast, atrial septal defects (ASD) which are commonly not apparent until adulthood, are generally well-tolerated due to the low pressures involved. In this setting Eisenmenger syndrome is uncommon, but when it does occur is more common in females.

• #17 Eisenmenger syndrome | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/eisenmenger-syndrome?embed_domain=hackmd.io%252525252525252F%2525252525252540yIPUAFeCSL2JsU8smR5nJQ%252525252525252Fbnjhjgjghjghjghfavicon.icoradiopaedia-icon-144.png&lang=us

  In general, the shunts that lead to Eisenmenger syndrome share high pressure and high flow. As such the three lesions that account for most cases are: […] In this group of patients, the gender distribution is equal, and it is uncommon to develop Eisenmenger syndrome before the age of 2. […] In contrast, atrial septal defects (ASD) which are commonly not apparent until adulthood, are generally well-tolerated due to the low pressures involved. In this setting Eisenmenger syndrome is uncommon, but when it does occur is more common in females.

• #18 Eisenmenger syndrome: current perspectives | RRCC

  https://www.dovepress.com/eisenmenger-syndrome-current-perspectives-peer-reviewed-fulltext-article-RRCC

  Eisenmenger syndrome (ES) is the most severe form of pulmonary arterial hypertension (PAH) related to congenital heart disease (CHD). […] With advances in pediatric cardiology and surgery, the prevalence of ES is steadily falling in developed countries; nonetheless, there will always be patients who are unsuitable for repair at the time of diagnosis, or emigrating from countries with less advanced healthcare, who will develop ES. […] The exact prevalence of ES is not known. According to historical data and before the advent of timely intervention, ~8% of patients with CHD and 11% of those with left-to-right shunts developed ES. […] Enhanced understanding and timely surgical or interventional repair of defects has resulted in a substantial reduction in the numbers of patients developing ES. Nevertheless, this condition will continue to occur in patients unsuitable for early repair and individuals from countries where availability of tertiary pediatric cardiology and cardiothoracic surgery is limited.

• #19 Eisenmenger Syndrome: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/154555-overview

  Eisenmenger syndrome usually develops before puberty, but it may also start manifesting in adolescence and early adulthood. Approximately 8% of patients with congenital heart disease develop Eisenmenger syndrome. […] The prevalence of Eisenmenger syndrome is difficult to quantify, but it is declining in the developed world with the identification and surgical correction of congenital heart conditions. Patients from underdeveloped countries are more likely to have late presentations of Eisenmenger syndrome due to uncorrected congenital cardiac lesions. […] The frequency of pulmonary hypertension and the subsequent development of reversed shunting vary depending on the heart defect and operative intervention. Such variations include the following: Large, nonrestrictive ventricular septal defect (VSD) or patent ductus arteriosus (PDA): Approximately 50% of infants with one of these defects develop pulmonary hypertension by early childhood. VSD or PDA and transposition of the great arteries: About 40% of patients develop pulmonary hypertension within the first year of life. Large secundum atrial septal defect (ASD): The natural history of a large secundum ASD differs in that the 10% of cases that progress to pulmonary hypertension do so more slowly and usually not until after the third decade of life. Persistent truncus arteriosus and unrestricted pulmonary blood flow: All patients develop severe pulmonary hypertension by the second year of life. Common atrioventricular canal: Almost all patients develop severe pulmonary hypertension by the second year of life. Surgically-created systemic-to-pulmonary shunt: The frequency of pulmonary hypertension varies depending on size and anatomy.

• #20 Eisenmenger’s Effects

  http://www.rnceus.com/vsd/eisen25.html

  Eisenmenger syndrome (ES) is a severe form of pulmonary arterial hypertension (PAH) associated with congenital heart defects (CHD). The prevalence of ES varies: it is encountered in approximately 1% to 5.6% of large tertiary CHD cohorts. Among patients with PAH associated with CHD, the prevalence of ES ranges from 1.1% to 12.3%, depending on the registry and population studied. The incidence of ES has declined in high-income countries due to early diagnosis and surgical correction of CHD. However, in low- and middle-income countries, ES remains more prevalent due to limited access to timely medical care and surgical interventions (Arvanitaki 2022).

• #21 Eisenmenger’s Syndrome | Doctor

  https://patient.info/doctor/eisenmengers-syndrome

  Eisenmenger’s syndrome epidemiology […] In developed countries, cardiac defects are being operated on in a timely manner, before pulmonary vascular disease develops. However, in lower income countries, shunt lesions that are not amenable for repair, as pulmonary vascular disease has already established, are more common.

• #22 Eisenmenger’s Effects

  http://www.rnceus.com/vsd/eisen25.html

  Eisenmenger syndrome (ES) is a severe form of pulmonary arterial hypertension (PAH) associated with congenital heart defects (CHD). The prevalence of ES varies: it is encountered in approximately 1% to 5.6% of large tertiary CHD cohorts. Among patients with PAH associated with CHD, the prevalence of ES ranges from 1.1% to 12.3%, depending on the registry and population studied. The incidence of ES has declined in high-income countries due to early diagnosis and surgical correction of CHD. However, in low- and middle-income countries, ES remains more prevalent due to limited access to timely medical care and surgical interventions (Arvanitaki 2022).

• #23 Eisenmenger Syndrome: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/154555-overview

  Eisenmenger syndrome usually develops before puberty, but it may also start manifesting in adolescence and early adulthood. Approximately 8% of patients with congenital heart disease develop Eisenmenger syndrome. […] The prevalence of Eisenmenger syndrome is difficult to quantify, but it is declining in the developed world with the identification and surgical correction of congenital heart conditions. Patients from underdeveloped countries are more likely to have late presentations of Eisenmenger syndrome due to uncorrected congenital cardiac lesions. […] The frequency of pulmonary hypertension and the subsequent development of reversed shunting vary depending on the heart defect and operative intervention. Such variations include the following: Large, nonrestrictive ventricular septal defect (VSD) or patent ductus arteriosus (PDA): Approximately 50% of infants with one of these defects develop pulmonary hypertension by early childhood. VSD or PDA and transposition of the great arteries: About 40% of patients develop pulmonary hypertension within the first year of life. Large secundum atrial septal defect (ASD): The natural history of a large secundum ASD differs in that the 10% of cases that progress to pulmonary hypertension do so more slowly and usually not until after the third decade of life. Persistent truncus arteriosus and unrestricted pulmonary blood flow: All patients develop severe pulmonary hypertension by the second year of life. Common atrioventricular canal: Almost all patients develop severe pulmonary hypertension by the second year of life. Surgically-created systemic-to-pulmonary shunt: The frequency of pulmonary hypertension varies depending on size and anatomy.

• #24 Orphanet: Eisenmenger syndrome

  https://www.orpha.net/en/disease/detail/97214

  A rare respiratory disease associated with unoperated congenital heart disease and characterized by congenital heart malformations with reversed or bi-directional shunting through an intra-cardiac or intervascular (usually aorto-pulmonary) communication with the development of PAH. […] The disease is thought to be rare and affects both males and females equally. The incidence of the syndrome is decreasing in the developed world as the vast majority of causative cardiac lesions are now readily treatable. Incidence may still be higher in the less developed regions of the world as perinatal survival is higher but surgical treatment remains unaffordable for many. […] Life expectancy depends on the type and severity of the underlying defect and right ventricular function, and ranges from 20 to 50 years. In patients with the syndrome the maternal mortality rate exceeds 50%. The chances of preterm delivery and low birth weight are extremely high and the likelihood of livebirth drops dramatically with lower maternal oxygen saturation levels (to below 10% in some series) if maternal resting oxygen saturations are less than 85%.

• #25 Eisenmenger Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK507800/

  Eisenmenger syndrome is a relatively rare disorder that is usually seen in persons with poor healthcare access (ie, rural/underserved areas), in whom large anatomical defects may go undetected for many years. […] Eisenmenger syndrome is best managed by an interprofessional team that includes a cardiologist, pulmonologist, cardiac surgeon, internist, thoracic surgeon, and intensivist. The disorder has many causes, and the key is to treat the primary disorder. Once Eisenmenger syndrome develops, the prognosis is poor. Heart and lung transplants are an option, but the lack of donors is a problem. Medications to dilate the pulmonary vasculature are unreliable or consistent in efficacy. Most patients are dead within 12 to 24 months after the diagnosis is made.

• #26 Eisenmenger Syndrome: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/154555-overview

  Eisenmenger syndrome usually develops before puberty, but it may also start manifesting in adolescence and early adulthood. Approximately 8% of patients with congenital heart disease develop Eisenmenger syndrome. […] The prevalence of Eisenmenger syndrome is difficult to quantify, but it is declining in the developed world with the identification and surgical correction of congenital heart conditions. Patients from underdeveloped countries are more likely to have late presentations of Eisenmenger syndrome due to uncorrected congenital cardiac lesions. […] The frequency of pulmonary hypertension and the subsequent development of reversed shunting vary depending on the heart defect and operative intervention. Such variations include the following: Large, nonrestrictive ventricular septal defect (VSD) or patent ductus arteriosus (PDA): Approximately 50% of infants with one of these defects develop pulmonary hypertension by early childhood. VSD or PDA and transposition of the great arteries: About 40% of patients develop pulmonary hypertension within the first year of life. Large secundum atrial septal defect (ASD): The natural history of a large secundum ASD differs in that the 10% of cases that progress to pulmonary hypertension do so more slowly and usually not until after the third decade of life. Persistent truncus arteriosus and unrestricted pulmonary blood flow: All patients develop severe pulmonary hypertension by the second year of life. Common atrioventricular canal: Almost all patients develop severe pulmonary hypertension by the second year of life. Surgically-created systemic-to-pulmonary shunt: The frequency of pulmonary hypertension varies depending on size and anatomy.

• #27 Eisenmenger syndrome – UF Health

  https://ufhealth.org/conditions-and-treatments/eisenmenger-syndrome

  Eisenmenger syndrome may begin to develop before a child reaches puberty. However, it also can develop in young adulthood, and may progress throughout young adulthood. […] The number of cases of this condition in the United States has dropped because providers are now able to diagnose and correct the defect sooner. Therefore, the problem can be corrected before irreversible damage occurs to the small lung arteries. […] Surgery as early as possible to correct the heart defect can prevent Eisenmenger syndrome.

• #28

  https://step1.medbullets.com/cardiovascular/108028/eisenmenger-syndrome

  A 26-year-old man presents to his cardiologist for exercise intolerance and blue discoloration of the lips and fingernails. He also reports occasionally coughing up blood. He reports having been diagnosed with a congenital heart defect when he was young but was lost to follow-up after moving cities. A Doppler echocardiogram shows a ventricular septal defect, right ventricular hypertrophy, and elevated pulmonary vascular resistance. […] Epidemiology risk factors congenital heart defect. […] Age of onset depends on type and severity of the defect. […] Can present as early as childhood. […] Death can result from decompensated cor pulmonale.

• #29 Eisenmenger’s syndrome epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Eisenmenger%E2%80%99s_syndrome_epidemiology_and_demographics

  At follow-up of 6 years, the mortality rate of Eisenmenger’s syndrome was 27%. […] Death usually occurs between 30 and 35 years old. […] Eisenmenger’s syndrome usually manifests before puberty. […] The prevalence of Eisenmenger’s syndrome is less common in developed countries due to better healthcare access. […] The prevalence of Eisenmenger’s syndrome is more common in developing countries due to poor healthcare access.

• #30 Eisenmenger syndrome: current perspectives | RRCC

  https://www.dovepress.com/eisenmenger-syndrome-current-perspectives-peer-reviewed-fulltext-article-RRCC

  ES patients should be followed in specialized centers, by means of an interdisciplinary approach by clinicians experienced in PAH and CHD. […] Current guidelines in the management of patients with ES recommend regular consultations with experienced physicians in CHD and PH. […] Despite the associated functional limitations and multitude of comorbidities, survival prospects in adults with ES are generally considered better than in other forms of PAH, but mortality remains high. […] However, registry data have shown better survival prospects in ES patients on pulmonary therapies disease when compared to those who were treatment nave.

• #31 Eisenmenger syndrome: current perspectives | RRCC

  https://www.dovepress.com/eisenmenger-syndrome-current-perspectives-peer-reviewed-fulltext-article-RRCC

  ES patients should be followed in specialized centers, by means of an interdisciplinary approach by clinicians experienced in PAH and CHD. […] Current guidelines in the management of patients with ES recommend regular consultations with experienced physicians in CHD and PH. […] Despite the associated functional limitations and multitude of comorbidities, survival prospects in adults with ES are generally considered better than in other forms of PAH, but mortality remains high. […] However, registry data have shown better survival prospects in ES patients on pulmonary therapies disease when compared to those who were treatment nave.

• #32 Eisenmenger’s syndrome epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Eisenmenger%E2%80%99s_syndrome_epidemiology_and_demographics

  At follow-up of 6 years, the mortality rate of Eisenmenger’s syndrome was 27%. […] Death usually occurs between 30 and 35 years old. […] Eisenmenger’s syndrome usually manifests before puberty. […] The prevalence of Eisenmenger’s syndrome is less common in developed countries due to better healthcare access. […] The prevalence of Eisenmenger’s syndrome is more common in developing countries due to poor healthcare access.

• #33 Eisenmenger’s syndrome epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Eisenmenger%E2%80%99s_syndrome_epidemiology_and_demographics

  At follow-up of 6 years, the mortality rate of Eisenmenger’s syndrome was 27%. […] Death usually occurs between 30 and 35 years old. […] Eisenmenger’s syndrome usually manifests before puberty. […] The prevalence of Eisenmenger’s syndrome is less common in developed countries due to better healthcare access. […] The prevalence of Eisenmenger’s syndrome is more common in developing countries due to poor healthcare access.

• #34 Eisenmenger syndrome

  https://www.redalyc.org/journal/5760/576068163002/html/

  Eisenmenger syndrome (ES) stands for pulmonary hypertension, reversal of the central shunts flow, and cyanosis secondary to any congenital heart defect (CHD) associated with non-repaired intra- or extra-cardiac communication. […] The population of adults with congenital heart disease (known under the acronym of GUCH: grown-up congenital heart disease) is growing at an acceleration rate of 5% per year. In the USA, more than 1 million adults have CHD. 10% of the cases present pulmonary hypertension, 30% of them due to unrepaired defects. ES will develop in half of these patients with unrepaired cardiovascular defects. […] Although long-term survival has been reported, patients with ES usually die between 30 and 35 years old.

• #35 Orphanet: Eisenmenger syndrome

  https://www.orpha.net/en/disease/detail/97214

  A rare respiratory disease associated with unoperated congenital heart disease and characterized by congenital heart malformations with reversed or bi-directional shunting through an intra-cardiac or intervascular (usually aorto-pulmonary) communication with the development of PAH. […] The disease is thought to be rare and affects both males and females equally. The incidence of the syndrome is decreasing in the developed world as the vast majority of causative cardiac lesions are now readily treatable. Incidence may still be higher in the less developed regions of the world as perinatal survival is higher but surgical treatment remains unaffordable for many. […] Life expectancy depends on the type and severity of the underlying defect and right ventricular function, and ranges from 20 to 50 years. In patients with the syndrome the maternal mortality rate exceeds 50%. The chances of preterm delivery and low birth weight are extremely high and the likelihood of livebirth drops dramatically with lower maternal oxygen saturation levels (to below 10% in some series) if maternal resting oxygen saturations are less than 85%.

• #36 Survival comparison in adults with congenital systemic to pulmonary shunt and borderline elevated pulmonary vascular resistance versus Eisenmenger syndrome | Scientific Reports

  https://www.nature.com/articles/s41598-024-81834-9

  The survival rates of adult patients with ES at 5, 10, and 15 years were 93.2%, 77.8%, and 71.2%, respectively. […] For patients with PAH-CHD, predominantly L-R shunts, at the median follow-up (5.6 years; range 0.124.3 years), the survival rates at 5, 10, and 15 years were 93.6%, 86.1%, and 86.1%, respectively. […] The survival of patients who received treat-and-repair was slightly better than that of ES patients, though the difference was not statistically significant (p=0.19). […] The independent mortality risk factors for patients with PAH-CHD with predominantly L-R shunts were functional class III-IV at initial presentation and oxygen saturation94% at the recent visit.

• #37 Long-Term Study on Therapeutic Strategy for Treatment of Eisenmenger Syndrome Patients: A Case Series Study

  https://www.mdpi.com/2227-9067/9/8/1217

  Eisenmenger syndrome (ES) refers to congenital heart diseases (CHD) with reversal flow associated with increased pulmonary pressure and irreversible pulmonary vascular remodeling. […] The prevalence of ES in PAH-CHD patients was reported to be between 30 and 40 %. […] The long-term prognosis of patients with ES remains unsatisfactory even under the treatment of advanced therapies. […] The leading cause of death was heart failure, which accounted for 34.3%, followed by infection and sudden cardiac death. […] Although previous studies demonstrated that 5-year survival rates were around 74–83%, no patient mortality was seen within 5 years of follow-up in our studies. […] The life span of the mortality cases was 53.3 ± 8.2 years, similar to the previous reports. […] A number of studies reported that 21–71% of ES patients had intrapulmonary thrombosis.

• #38 Survival comparison in adults with congenital systemic to pulmonary shunt and borderline elevated pulmonary vascular resistance versus Eisenmenger syndrome | Scientific Reports

  https://www.nature.com/articles/s41598-024-81834-9

  The survival rates of adult patients with ES at 5, 10, and 15 years were 93.2%, 77.8%, and 71.2%, respectively. […] For patients with PAH-CHD, predominantly L-R shunts, at the median follow-up (5.6 years; range 0.124.3 years), the survival rates at 5, 10, and 15 years were 93.6%, 86.1%, and 86.1%, respectively. […] The survival of patients who received treat-and-repair was slightly better than that of ES patients, though the difference was not statistically significant (p=0.19). […] The independent mortality risk factors for patients with PAH-CHD with predominantly L-R shunts were functional class III-IV at initial presentation and oxygen saturation94% at the recent visit.

• #39 Survival comparison in adults with congenital systemic to pulmonary shunt and borderline elevated pulmonary vascular resistance versus Eisenmenger syndrome | Scientific Reports

  https://www.nature.com/articles/s41598-024-81834-9

  Pulmonary arterial hypertension (PAH) associated with congenital heart disease (PAH-CHD) is a consequence of unrepaired large systemic-to-pulmonary shunts. […] The long-term data of adult patients who have PAH-CHD with elevated pulmonary vascular resistance (PVR) are limited. […] The PVR in the ES group was 21.013.1 WU. […] Most ES patients (97%) received pulmonary vasodilator therapy. […] The survival rate of patients who underwent treat-and-repair was slightly better than that of patients who underwent ES, although the difference was not statistically significant (p=0.19). […] Independent mortality risk factors were functional class III-IV at initial presentation (hazard ratio 5.7, 95% CI 1.226.6; p=0.02) and oxygen saturation94% at the most recent visit (hazard ratio 9.4, 95% CI 2.142.9; p=0.004).

• #40 Treatment of adults with Eisenmenger syndrome—state of the art in the 21st century: a short overview – Diller – Cardiovascular Diagnosis and Therapy

  https://cdt.amegroups.org/article/view/74370/html

  Eisenmenger syndrome (ES) develops in association with unrepaired, non-restrictive cardiac shunt lesions at the atrial, ventricular or arterial level over time. […] The prevalence of Eisenmenger syndrome is not well defined but traditional estimates suggest that around 5% of ACHD patients under follow-up at large supra-regional centers have Eisenmenger syndrome. […] The leading reasons for mortality are sudden cardiac death, progressive heart failure, and infectious diseases. […] Follow-up and care at specialist congenital heart disease centers is highly recommended to prevent, to early diagnose and to timely manage complications of ES. […] In patients presenting late with established Eisenmenger syndrome, shunt closure is contraindicated as this will worsen prognosis in this setting.

• #41 Long-Term Study on Therapeutic Strategy for Treatment of Eisenmenger Syndrome Patients: A Case Series Study

  https://www.mdpi.com/2227-9067/9/8/1217

  Eisenmenger syndrome (ES) refers to congenital heart diseases (CHD) with reversal flow associated with increased pulmonary pressure and irreversible pulmonary vascular remodeling. […] The prevalence of ES in PAH-CHD patients was reported to be between 30 and 40 %. […] The long-term prognosis of patients with ES remains unsatisfactory even under the treatment of advanced therapies. […] The leading cause of death was heart failure, which accounted for 34.3%, followed by infection and sudden cardiac death. […] Although previous studies demonstrated that 5-year survival rates were around 74–83%, no patient mortality was seen within 5 years of follow-up in our studies. […] The life span of the mortality cases was 53.3 ± 8.2 years, similar to the previous reports. […] A number of studies reported that 21–71% of ES patients had intrapulmonary thrombosis.

• #42 Orphanet: Eisenmenger syndrome

  https://www.orpha.net/en/disease/detail/97214

  A rare respiratory disease associated with unoperated congenital heart disease and characterized by congenital heart malformations with reversed or bi-directional shunting through an intra-cardiac or intervascular (usually aorto-pulmonary) communication with the development of PAH. […] The disease is thought to be rare and affects both males and females equally. The incidence of the syndrome is decreasing in the developed world as the vast majority of causative cardiac lesions are now readily treatable. Incidence may still be higher in the less developed regions of the world as perinatal survival is higher but surgical treatment remains unaffordable for many. […] Life expectancy depends on the type and severity of the underlying defect and right ventricular function, and ranges from 20 to 50 years. In patients with the syndrome the maternal mortality rate exceeds 50%. The chances of preterm delivery and low birth weight are extremely high and the likelihood of livebirth drops dramatically with lower maternal oxygen saturation levels (to below 10% in some series) if maternal resting oxygen saturations are less than 85%.

• #43 Pulmonary Vascular Disease | UKOSS | NPEU

  https://www.npeu.ox.ac.uk/ukoss/completed-surveillance/pvd

  What is the current incidence of Eisenmenger’s syndrome and pulmonary hypertension in pregnancy in the UK? […] Eisenmenger’s syndrome is estimated to carry a maternal mortality rate of 40% per pregnancy, with an infant mortality rate of 10-15%. […] This prospective study through UKOSS will provide an appropriate national case series with good ascertainment and allow a comprehensive study of the epidemiology and current management of Eisenmenger’s syndrome and pulmonary hypertension.

• #44 Orphanet: Eisenmenger syndrome

  https://www.orpha.net/en/disease/detail/97214

  A rare respiratory disease associated with unoperated congenital heart disease and characterized by congenital heart malformations with reversed or bi-directional shunting through an intra-cardiac or intervascular (usually aorto-pulmonary) communication with the development of PAH. […] The disease is thought to be rare and affects both males and females equally. The incidence of the syndrome is decreasing in the developed world as the vast majority of causative cardiac lesions are now readily treatable. Incidence may still be higher in the less developed regions of the world as perinatal survival is higher but surgical treatment remains unaffordable for many. […] Life expectancy depends on the type and severity of the underlying defect and right ventricular function, and ranges from 20 to 50 years. In patients with the syndrome the maternal mortality rate exceeds 50%. The chances of preterm delivery and low birth weight are extremely high and the likelihood of livebirth drops dramatically with lower maternal oxygen saturation levels (to below 10% in some series) if maternal resting oxygen saturations are less than 85%.

• #45 Eisenmenger syndrome: current perspectives | RRCC

  https://www.dovepress.com/eisenmenger-syndrome-current-perspectives-peer-reviewed-fulltext-article-RRCC

  ES patients should be followed in specialized centers, by means of an interdisciplinary approach by clinicians experienced in PAH and CHD. […] Current guidelines in the management of patients with ES recommend regular consultations with experienced physicians in CHD and PH. […] Despite the associated functional limitations and multitude of comorbidities, survival prospects in adults with ES are generally considered better than in other forms of PAH, but mortality remains high. […] However, registry data have shown better survival prospects in ES patients on pulmonary therapies disease when compared to those who were treatment nave.

• #46 Eisenmenger Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK507800/

  Eisenmenger syndrome is a relatively rare disorder that is usually seen in persons with poor healthcare access (ie, rural/underserved areas), in whom large anatomical defects may go undetected for many years. […] Eisenmenger syndrome is best managed by an interprofessional team that includes a cardiologist, pulmonologist, cardiac surgeon, internist, thoracic surgeon, and intensivist. The disorder has many causes, and the key is to treat the primary disorder. Once Eisenmenger syndrome develops, the prognosis is poor. Heart and lung transplants are an option, but the lack of donors is a problem. Medications to dilate the pulmonary vasculature are unreliable or consistent in efficacy. Most patients are dead within 12 to 24 months after the diagnosis is made.

• #47 Treatment of adults with Eisenmenger syndrome—state of the art in the 21st century: a short overview – Diller – Cardiovascular Diagnosis and Therapy

  https://cdt.amegroups.org/article/view/74370/html

  Eisenmenger syndrome (ES) develops in association with unrepaired, non-restrictive cardiac shunt lesions at the atrial, ventricular or arterial level over time. […] The prevalence of Eisenmenger syndrome is not well defined but traditional estimates suggest that around 5% of ACHD patients under follow-up at large supra-regional centers have Eisenmenger syndrome. […] The leading reasons for mortality are sudden cardiac death, progressive heart failure, and infectious diseases. […] Follow-up and care at specialist congenital heart disease centers is highly recommended to prevent, to early diagnose and to timely manage complications of ES. […] In patients presenting late with established Eisenmenger syndrome, shunt closure is contraindicated as this will worsen prognosis in this setting.

• #48 Treatment of adults with Eisenmenger syndrome—state of the art in the 21st century: a short overview – Diller – Cardiovascular Diagnosis and Therapy

  https://cdt.amegroups.org/article/view/74370/html

  Assessing prognosis is paramount as it may help to guide specific pulmonary arterial hypertension therapy in this setting. […] Given the degree of pulmonary arterial hypertension, survival prospects of Eisenmenger patients are relatively preserved compared to patients with idiopathic pulmonary hypertension. […] The key principle in the clinical management of Eisenmenger syndrome patients remains to cause no harm to the fragile balanced pathophysiological state. […] Eisenmenger patients require life-long expert assessment and therapy at specialized centers. […] All Eisenmenger syndrome patients need to be referred to an ACHD expert center with access to pulmonary hypertension specialists so that this fragile population is early evaluated for specific disease targeted therapy.

• #49 Treatment of adults with Eisenmenger syndrome—state of the art in the 21st century: a short overview – Diller – Cardiovascular Diagnosis and Therapy

  https://cdt.amegroups.org/article/view/74370/html

  Assessing prognosis is paramount as it may help to guide specific pulmonary arterial hypertension therapy in this setting. […] Given the degree of pulmonary arterial hypertension, survival prospects of Eisenmenger patients are relatively preserved compared to patients with idiopathic pulmonary hypertension. […] The key principle in the clinical management of Eisenmenger syndrome patients remains to cause no harm to the fragile balanced pathophysiological state. […] Eisenmenger patients require life-long expert assessment and therapy at specialized centers. […] All Eisenmenger syndrome patients need to be referred to an ACHD expert center with access to pulmonary hypertension specialists so that this fragile population is early evaluated for specific disease targeted therapy.

• #50 The Belgian Eisenmenger syndrome registry: Implications for treatment strategies?

  https://lirias.kuleuven.be/97056

  OBJECTIVE: Pulmonary arterial hypertension (PAH), associated with congenital heart disease (CHD), usually results from a systemic-to-pulmonary shunt. […] A national registry of ES patients was initiated to optimise patient care and to provide epidemiological information regarding PAH and CHD in Belgium. […] Through the national Eisenmenger registry, 91 adult patients with ES were identified (estimated prevalence II per million inhabitants).

• #51 [Eisenmenger syndrome. Epidemiology and natural history] – PubMed

  https://pubmed.ncbi.nlm.nih.gov/20141995/

  Eisenmenger syndrome is the last-stage of congenital heart diseases with untreated left to right shunt. […] However, patients follow-up is often performed in inappropriate non-specialized care centres which makes the collection of epidemiological data difficult. […] Consequently, population registries are necessary and would increase knowledge of natural history.

• #52 Pulmonary Vascular Disease | UKOSS | NPEU

  https://www.npeu.ox.ac.uk/ukoss/completed-surveillance/pvd

  What is the current incidence of Eisenmenger’s syndrome and pulmonary hypertension in pregnancy in the UK? […] Eisenmenger’s syndrome is estimated to carry a maternal mortality rate of 40% per pregnancy, with an infant mortality rate of 10-15%. […] This prospective study through UKOSS will provide an appropriate national case series with good ascertainment and allow a comprehensive study of the epidemiology and current management of Eisenmenger’s syndrome and pulmonary hypertension.

• #53 Long-Term Study on Therapeutic Strategy for Treatment of Eisenmenger Syndrome Patients: A Case Series Study

  https://www.mdpi.com/2227-9067/9/8/1217

  In our study, four ES patients (80%) had intrapulmonary thrombosis despite warfarin treatment. […] Previous studies suggested that progressive RV failure was closely related to mortality in ES patients, and it was associated with RV remodeling and metabolic alterations that could augment glucose uptake. […] Although the clinical outcomes improved after advanced therapies, long-term mortality and morbidity remain high in patients with ES. […] Based on our experience, we regularly followed up with these patients every 1 to 2 months and applied management according to the guidelines of the European Society of Cardiology and American Heart Association. […] The collaborative work from our multidisciplinary team, as well as our regular follow-up studies, improved the 5-year survival of our patients.

• #54 Long-Term Study on Therapeutic Strategy for Treatment of Eisenmenger Syndrome Patients: A Case Series Study

  https://www.mdpi.com/2227-9067/9/8/1217

  In our study, four ES patients (80%) had intrapulmonary thrombosis despite warfarin treatment. […] Previous studies suggested that progressive RV failure was closely related to mortality in ES patients, and it was associated with RV remodeling and metabolic alterations that could augment glucose uptake. […] Although the clinical outcomes improved after advanced therapies, long-term mortality and morbidity remain high in patients with ES. […] Based on our experience, we regularly followed up with these patients every 1 to 2 months and applied management according to the guidelines of the European Society of Cardiology and American Heart Association. […] The collaborative work from our multidisciplinary team, as well as our regular follow-up studies, improved the 5-year survival of our patients.

• #55 Long-Term Study on Therapeutic Strategy for Treatment of Eisenmenger Syndrome Patients: A Case Series Study

  https://www.mdpi.com/2227-9067/9/8/1217

  In our study, four ES patients (80%) had intrapulmonary thrombosis despite warfarin treatment. […] Previous studies suggested that progressive RV failure was closely related to mortality in ES patients, and it was associated with RV remodeling and metabolic alterations that could augment glucose uptake. […] Although the clinical outcomes improved after advanced therapies, long-term mortality and morbidity remain high in patients with ES. […] Based on our experience, we regularly followed up with these patients every 1 to 2 months and applied management according to the guidelines of the European Society of Cardiology and American Heart Association. […] The collaborative work from our multidisciplinary team, as well as our regular follow-up studies, improved the 5-year survival of our patients.

• #56 Treatment of adults with Eisenmenger syndrome—state of the art in the 21st century: a short overview – Diller – Cardiovascular Diagnosis and Therapy

  https://cdt.amegroups.org/article/view/74370/html

  Assessing prognosis is paramount as it may help to guide specific pulmonary arterial hypertension therapy in this setting. […] Given the degree of pulmonary arterial hypertension, survival prospects of Eisenmenger patients are relatively preserved compared to patients with idiopathic pulmonary hypertension. […] The key principle in the clinical management of Eisenmenger syndrome patients remains to cause no harm to the fragile balanced pathophysiological state. […] Eisenmenger patients require life-long expert assessment and therapy at specialized centers. […] All Eisenmenger syndrome patients need to be referred to an ACHD expert center with access to pulmonary hypertension specialists so that this fragile population is early evaluated for specific disease targeted therapy.

• #57 Treatment of adults with Eisenmenger syndrome—state of the art in the 21st century: a short overview – Diller – Cardiovascular Diagnosis and Therapy

  https://cdt.amegroups.org/article/view/74370/html

  Assessing prognosis is paramount as it may help to guide specific pulmonary arterial hypertension therapy in this setting. […] Given the degree of pulmonary arterial hypertension, survival prospects of Eisenmenger patients are relatively preserved compared to patients with idiopathic pulmonary hypertension. […] The key principle in the clinical management of Eisenmenger syndrome patients remains to cause no harm to the fragile balanced pathophysiological state. […] Eisenmenger patients require life-long expert assessment and therapy at specialized centers. […] All Eisenmenger syndrome patients need to be referred to an ACHD expert center with access to pulmonary hypertension specialists so that this fragile population is early evaluated for specific disease targeted therapy.

• #58 Long-Term Study on Therapeutic Strategy for Treatment of Eisenmenger Syndrome Patients: A Case Series Study

  https://www.mdpi.com/2227-9067/9/8/1217

  Eisenmenger syndrome (ES) refers to congenital heart diseases (CHD) with reversal flow associated with increased pulmonary pressure and irreversible pulmonary vascular remodeling. […] The prevalence of ES in PAH-CHD patients was reported to be between 30 and 40 %. […] The long-term prognosis of patients with ES remains unsatisfactory even under the treatment of advanced therapies. […] The leading cause of death was heart failure, which accounted for 34.3%, followed by infection and sudden cardiac death. […] Although previous studies demonstrated that 5-year survival rates were around 74–83%, no patient mortality was seen within 5 years of follow-up in our studies. […] The life span of the mortality cases was 53.3 ± 8.2 years, similar to the previous reports. […] A number of studies reported that 21–71% of ES patients had intrapulmonary thrombosis.

• #59 Long-Term Study on Therapeutic Strategy for Treatment of Eisenmenger Syndrome Patients: A Case Series Study

  https://www.mdpi.com/2227-9067/9/8/1217

  In our study, four ES patients (80%) had intrapulmonary thrombosis despite warfarin treatment. […] Previous studies suggested that progressive RV failure was closely related to mortality in ES patients, and it was associated with RV remodeling and metabolic alterations that could augment glucose uptake. […] Although the clinical outcomes improved after advanced therapies, long-term mortality and morbidity remain high in patients with ES. […] Based on our experience, we regularly followed up with these patients every 1 to 2 months and applied management according to the guidelines of the European Society of Cardiology and American Heart Association. […] The collaborative work from our multidisciplinary team, as well as our regular follow-up studies, improved the 5-year survival of our patients.

• #60 Long-Term Study on Therapeutic Strategy for Treatment of Eisenmenger Syndrome Patients: A Case Series Study

  https://www.mdpi.com/2227-9067/9/8/1217

  In our study, four ES patients (80%) had intrapulmonary thrombosis despite warfarin treatment. […] Previous studies suggested that progressive RV failure was closely related to mortality in ES patients, and it was associated with RV remodeling and metabolic alterations that could augment glucose uptake. […] Although the clinical outcomes improved after advanced therapies, long-term mortality and morbidity remain high in patients with ES. […] Based on our experience, we regularly followed up with these patients every 1 to 2 months and applied management according to the guidelines of the European Society of Cardiology and American Heart Association. […] The collaborative work from our multidisciplinary team, as well as our regular follow-up studies, improved the 5-year survival of our patients.

• #61 Postoperative Management of Eisenmenger’s Syndrome

  https://www.oatext.com/postoperative-management-of-eisenmengers-syndrome.php

  Eisenmengers Syndrome (ES) is defined by the triad of systemic-to-pulmonary congenital cardiovascular communication, pulmonary arterial hypertension, and cyanosis. Modern advances, enhanced understanding of pediatric cardiology, and timely surgical intervention in the management of congenital heart defects (CHDS) has resulted in the decreased prevalence of ES by 50%. An estimated 8% of patients with congenital defects will develop ES and 11% with CHDS plus left to right shunt physiology. CHDS commonly associated with ES includes atrial septal defects (ASDs), atrioventricular septal defects, ventricular septal defects (VSDs) and patent ductus arteriosus (PDA). Most patients with ES survive for 20 to 30 years. The survival of patients with pulmonary hypertension and ES into the 5th and 6th decades of life increase the likelihood of exposure to non-cardiac surgery. Eisenmengers Syndrome is associated with perioperative complications including early and sudden postoperative death. Non-cardiac surgeries amongst ES patients carry a mortality risk as high as 30%.

• #62 Postoperative Management of Eisenmenger’s Syndrome

  https://www.oatext.com/postoperative-management-of-eisenmengers-syndrome.php

  Understanding Eisenmengers physiology and anticipating the hemodynamic changes during the surgical intervention with targeted treatment could lead to a reduction in significant mortality. It is also important to consider the severity of pulmonary hypertension, right ventricular dysfunction, severity of tricuspid regurgitation, type and duration of surgery, other cardiac disease, and other comorbidities prior to any intervention. The fundamental principles in perioperative management of patients with ES involve preventing systemic hypotension and avoiding elevation of pulmonary vascular resistance. An abrupt reduction in the systemic vascular resistance (SVR) will worsen the right to left shunting, leading to cyanosis, systemic hypoxemia, significant bradycardia, and cardiovascular collapse. Decreased SVR also leads to RV encroachment of the LV cavity causing diastolic impairment and decreased cardiac output. Therefore, measures should be in place to detect and manage alteration in hemodynamics during induction of anesthesia and surgery.

• #63 Postoperative Management of Eisenmenger’s Syndrome

  https://www.oatext.com/postoperative-management-of-eisenmengers-syndrome.php

  Several authors have proposed different techniques of monitoring ES patient intraoperatively. While some suggested the use of frequent blood pressure monitoring by non-invasive techniques, others advocate the combination of intra-arterial monitoring, central venous catheterization and use of Swan Ganz catheter in monitoring of PVR. It is advisable to refer patients with ES to specialized centers capable of applying strategies aimed at the perioperative hemodynamic changes during surgical interventions.

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