Materiały źródłowe

• #1 Eisenmenger Syndrome: Causes, Symptoms and Treatment

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

  The only way to prevent Eisenmenger syndrome is to have congenital heart defects repaired as early in life as possible.

• #2 Eisenmenger syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/eisenmenger-syndrome/symptoms-causes/syc-20350580

  Early diagnosis and repair of congenital heart defects usually prevents Eisenmenger syndrome. […] If it does develop, treatment involves regular health checkups and medicines to improve symptoms.

• #3 Eisenmenger syndrome – UF Health

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

  Surgery as early as possible to correct the heart defect can prevent Eisenmenger syndrome.

• #4 Eisenmenger Syndrome – Adult Congenital Heart Disease | UCLA Health

  https://www.uclahealth.org/medical-services/heart/achd/conditions-treatments/eisenmenger-syndrome

  For children born with congenital heart defects, such as large holes or large connections between the great arteries, they are at risk for developing a progressive blood vessel disease in their lungs if their heart defects are not repaired early in life. […] For this reason, these large defects usually are repaired in the first 5-7 years of life, to avoid the life-long effects of pulmonary hypertension. […] In the past, it was believed that the use of medications to reduce pulmonary blood pressure would be too risky in patients with Eisenmenger syndrome, since they would be susceptible to a decrease in their body’s blood pressure which could make their oxygen levels drop dangerously. However, studies over the past decade have dispelled this myth and have led to wide-spread use of new drugs focused at reducing pulmonary blood pressure. Current studies have shown that these advanced therapies both improve quality of life, and may have a beneficial impact on survival.

• #5 Eisenmenger Syndrome Treatment & Management: Approach Considerations, Oxygen Therapy, Pulmonary Vasodilator Therapy

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

  Prevention of Eisenmenger syndrome is critical. When recognized in a timely fashion, congenital cardiac defects can be effectively treated with minimal morbidity and mortality. Eisenmenger syndrome is, by definition, an untreated lesion that has progressed to the point of inoperability. […] Many congenital heart defects can be identified in utero when families receive appropriate prenatal care. Continued perinatal care and routine follow-up with a qualified pediatrician lead to the identification of most lesions that are not identified prenatally. […] In addition to clinicians providing patients with instruction regarding infective endocarditis risk reduction, patients can discourage complications of Eisenmenger syndrome with the following measures: Avoiding situations that exacerbate vasodilation, Avoiding abrupt, strenuous, or isometric exercise, Restricting activities that may result in further oxygen desaturation, symptomatic arrhythmias, or right ventricular dysfunction, Stopping steady-state exercise at the onset of symptoms, Following good dental hygiene to prevent infectious concerns, such as infective endocarditis and brain abscess.

• #6 Eisenmenger syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Eisenmenger_syndrome

  Eisenmenger syndrome is defined as the process in which a long-standing left-to-right cardiac shunt caused by a congenital heart defect (typically by a ventricular septal defect, atrial septal defect, or less commonly, patent ductus arteriosus) causes pulmonary hypertension and eventual reversal of the shunt into a cyanotic right-to-left shunt. Because of the advent of fetal screening with echocardiography early in life, the incidence of heart defects progressing to Eisenmenger syndrome has decreased. […] If the inciting defect in the heart is identified before it causes significant pulmonary hypertension, it can normally be repaired through surgery, preventing the disease. […] Various medicines and therapies for pulmonary hypertension are under investigation for treatment of the symptoms. […] Antiarrhythmic drugs are important for many patients with Eisenmenger syndrome, as evidence suggests that arrhythmia-induced sudden cardiac death may be the leading cause of death among patients with the disease.

• #7 Eisenmenger syndrome

  https://adamcertificationdemo.adam.com/content.aspx?productid=139&pid=1&gid=007317

  Surgery as early as possible to correct the heart defect can prevent Eisenmenger syndrome. […] 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.

• #8 Eisenmenger Syndrome Treatment & Management: Approach Considerations, Oxygen Therapy, Pulmonary Vasodilator Therapy

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

  Prevention of Eisenmenger syndrome is critical. When recognized in a timely fashion, congenital cardiac defects can be effectively treated with minimal morbidity and mortality. Eisenmenger syndrome is, by definition, an untreated lesion that has progressed to the point of inoperability. […] Many congenital heart defects can be identified in utero when families receive appropriate prenatal care. Continued perinatal care and routine follow-up with a qualified pediatrician lead to the identification of most lesions that are not identified prenatally. […] In addition to clinicians providing patients with instruction regarding infective endocarditis risk reduction, patients can discourage complications of Eisenmenger syndrome with the following measures: Avoiding situations that exacerbate vasodilation, Avoiding abrupt, strenuous, or isometric exercise, Restricting activities that may result in further oxygen desaturation, symptomatic arrhythmias, or right ventricular dysfunction, Stopping steady-state exercise at the onset of symptoms, Following good dental hygiene to prevent infectious concerns, such as infective endocarditis and brain abscess.

• #9 2020 ESC Guidelines for the Management of ACHD: Focus on PH

  https://www.acc.org/Latest-in-Cardiology/Articles/2021/08/05/11/36/2020-ESC-Guidelines-for-the-Management-of-ACHD

  It is recommended that patients with PAH in CHD be cared for by a multidisciplinary team consisting of experts in cardiology, imaging, pulmonology, obstetrics, anesthesiology, neonatology, PH, cardiothoracic and thoracic surgery, nursing, and medical genetics. All patients with PAH in CHD should be given social and psychological support, be kept up to date on their vaccinations, and be advised to avoid excessive physical stress. […] All female patients with pre-capillary PH should be counseled against pregnancy (Class Ic recommendation). […] In patients with Eisenmenger syndrome, specifically, oxygen supplementation should be used only in cases in which there is a documented, consistent, and significant increase in oxygen saturation and improvement in symptoms. Routine phlebotomy should be avoided in these patients because secondary erythrocytosis is expected and, in fact, beneficial because this aides in oxygen transport and delivery.

• #10 Eisenmenger syndrome – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/eisenmenger-syndrome/diagnosis-treatment/drc-20350584

  The goals of Eisenmenger syndrome treatment are to: […] Prevent complications. […] Regular health checkups at least once a year are an important part of Eisenmenger syndrome treatment. […] Preventive antibiotics are only recommended in specific situations. Talk with your healthcare professional to learn if they are right for you. […] If you have Eisenmenger syndrome, it’s especially important to prevent respiratory infections such as the flu. Get a flu shot every year and a pneumonia vaccination every five years. […] Healthcare professionals say it’s critical that pregnancy be avoided if you have Eisenmenger syndrome. […] If you need treatment for Eisenmenger syndrome, get care at a medical center with healthcare professionals who have experience in congenital heart diseases.

• #11 Eisenmenger syndrome | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/sindrome-de-eisenmenger/

  Early diagnosis and repair of congenital heart defects usually prevents Eisenmenger syndrome. […] If you have Eisenmenger syndrome, you are usually sent to a doctor trained in heart diseases, called a cardiologist. It’s helpful to find a cardiologist who has experience treating people who have congenital heart defects. Regular health checkups at least once a year are an important part of Eisenmenger syndrome treatment. […] Some dental and medical procedures may let germs into the bloodstream. Some people need to take antibiotics before surgery or dental procedures to prevent a heart infection called endocarditis. Preventive antibiotics are only recommended in specific situations. Talk with your healthcare professional to learn if they are right for you. […] Healthcare professionals don’t recommend surgery to repair the hole in the heart once Eisenmenger syndrome has developed. […] If you have Eisenmenger syndrome, pregnancy poses serious health risks and can be life-threatening for you and the baby. Healthcare professionals say it’s critical that pregnancy be avoided if you have Eisenmenger syndrome.

• #12 Treatment of adults with Eisenmenger syndrome—state of the art in the 21st century: a short overview

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8410485/

  Eisenmenger syndrome (ES) develops in association with unrepaired, non-restrictive cardiac shunt lesions at the atrial, ventricular or arterial level over time. […] 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. […] Preventing complications and avoiding management errors should be the primary goal of regular outpatient assessment. […] It is recommended to meticulously encourage patients to obtain yearly influenza immunization as well as immunization against pneumococcal disease every 5 years. […] As per current guidelines, Eisenmenger patients should be offered appropriate antibiotic endocarditis prophylaxis before dental procedures. […] If general anaesthesia is required for any non-cardiac or cardiac diagnostic or therapeutic procedure, this should be supervised by an experienced Anaesthesist, ideally at a tertiary center with experience in ACHD.

• #13 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. […] 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. […] Preventing complications and avoiding management errors should be the primary goal of regular outpatient assessment. […] It is recommended to meticulously encourage patients to obtain yearly influenza immunization as well as immunization against pneumococcal disease every 5 years. Furthermore, respiratory infections should be pro-actively diagnosed and treated accordingly to avoid pulmonary or cardiac complications. As per current guidelines, Eisenmenger patients should be offered appropriate antibiotic endocarditis prophylaxis before dental procedures.

• #14 Eisenmenger syndrome – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/eisenmenger-syndrome/diagnosis-treatment/drc-20350584

  The goals of Eisenmenger syndrome treatment are to: […] Prevent complications. […] Regular health checkups at least once a year are an important part of Eisenmenger syndrome treatment. […] Preventive antibiotics are only recommended in specific situations. Talk with your healthcare professional to learn if they are right for you. […] If you have Eisenmenger syndrome, it’s especially important to prevent respiratory infections such as the flu. Get a flu shot every year and a pneumonia vaccination every five years. […] Healthcare professionals say it’s critical that pregnancy be avoided if you have Eisenmenger syndrome. […] If you need treatment for Eisenmenger syndrome, get care at a medical center with healthcare professionals who have experience in congenital heart diseases.

• #15 Treatment for Eisenmenger Syndrome | Stanford Health Care

  https://stanfordhealthcare.org/medical-conditions/blood-heart-circulation/eisenmenger-syndrome/treatments.html

  Specific treatment for Eisenmenger syndrome will be determined by your doctor based on: […] The goals of treatment for Eisenmenger syndrome are aimed at decreasing the pulmonary artery pressure, improving oxygenation, and decreasing degree of cyanosis and erythrocytosis. […] Pregnancy is not recommended for females of childbearing age with Eisenmenger syndrome. Pregnancy may pose a high risk of death for the mother as well as complications for the fetus due to effects of low oxygen levels in the blood. […] Any anesthesia and surgery is considered high risk and should be carefully planned. Collaboration with a cardiac specialist is recommended. […] Air travel and high altitude exposure requires adequate hydration (fluid intake) and supplemental oxygen to prevent complications. […] Smoking and alcohol intake are not recommended. […] Coughing should be controlled or prevented with a strong suppressant medication to prevent risk of pulmonary hemorrhage (bleeding from the lungs). […] Flu shots are recommended annually, and pneumococcal vaccine should be received according to the doctor’s recommendation.

• #16 Treatment of adults with Eisenmenger syndrome—state of the art in the 21st century: a short overview

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8410485/

  Eisenmenger syndrome (ES) develops in association with unrepaired, non-restrictive cardiac shunt lesions at the atrial, ventricular or arterial level over time. […] 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. […] Preventing complications and avoiding management errors should be the primary goal of regular outpatient assessment. […] It is recommended to meticulously encourage patients to obtain yearly influenza immunization as well as immunization against pneumococcal disease every 5 years. […] As per current guidelines, Eisenmenger patients should be offered appropriate antibiotic endocarditis prophylaxis before dental procedures. […] If general anaesthesia is required for any non-cardiac or cardiac diagnostic or therapeutic procedure, this should be supervised by an experienced Anaesthesist, ideally at a tertiary center with experience in ACHD.

• #17 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. […] 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. […] Preventing complications and avoiding management errors should be the primary goal of regular outpatient assessment. […] It is recommended to meticulously encourage patients to obtain yearly influenza immunization as well as immunization against pneumococcal disease every 5 years. Furthermore, respiratory infections should be pro-actively diagnosed and treated accordingly to avoid pulmonary or cardiac complications. As per current guidelines, Eisenmenger patients should be offered appropriate antibiotic endocarditis prophylaxis before dental procedures.

• #18 Eisenmenger Syndrome – Congenital Heart Disease in Adults – Cardiovascular Diseases – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.2.12.5.

  1) Administration of influenza vaccine every year, updated vaccination against coronavirus disease 2019 (COVID-19) as per the national guidelines, and pneumococcal vaccine every 5 years. […] 10. Endocarditis prophylaxis is indicated in all patients with cyanotic congenital heart disease. […] Routine anticoagulation: Although patients with Eisenmenger syndrome are at risk of thrombosis (especially in the dilated pulmonary arteries), currently available data do not support the routine use of anticoagulation in these patients due to the inherent increased risk of bleeding as well as difficulty with the routine monitoring of anticoagulation.

• #19 Eisenmenger Syndrome – ACHA

  https://www.achaheart.org/your-heart/educational-qas/types-of-heart-defects/eisenmenger-syndrome/

  Management of complications include: […] Prevent infections by keep excellent oral health (mouth, gums, teeth) […] Take antibiotics to prevent subacute bacterial endocarditis when indicated […] Get your Covid-19, flu and pneumovax vaccines.

• #20 Eisenmenger Syndrome – Congenital Heart Disease in Adults – Cardiovascular Diseases – Diseases – McMaster Textbook of Internal Medicine

  https://empendium.com/mcmtextbook/chapter/B31.II.2.12.5.

  1) Administration of influenza vaccine every year, updated vaccination against coronavirus disease 2019 (COVID-19) as per the national guidelines, and pneumococcal vaccine every 5 years. […] 10. Endocarditis prophylaxis is indicated in all patients with cyanotic congenital heart disease. […] Routine anticoagulation: Although patients with Eisenmenger syndrome are at risk of thrombosis (especially in the dilated pulmonary arteries), currently available data do not support the routine use of anticoagulation in these patients due to the inherent increased risk of bleeding as well as difficulty with the routine monitoring of anticoagulation.

• #21 Eisenmenger Syndrome – Pediatrics – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/pediatrics/congenital-cardiovascular-anomalies/eisenmenger-syndrome

  Endocarditis prophylaxis is recommended. […] All patients should be given endocarditis prophylaxis before dental or surgical procedures that are likely to cause bacteremia. […] Doing a corrective operation for the underlying cardiac anomaly at the appropriate age should prevent Eisenmenger syndrome. […] There is no specific treatment once the syndrome develops, other than heart and lung transplantation, but drugs that may lower pulmonary vascular resistance (eg, prostacyclin analogs, endothelin antagonists, phosphodiesterase-5 inhibitors) are useful.

• #22 Eisenmenger syndrome: current perspectives | RRCC

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

  Current guidelines in the management of patients with ES recommend regular consultations with experienced physicians in CHD and PH. Areas that require routine consideration are detailed in the following sections, but also include adequate contraception, endocarditis prophylaxis, up-to-date immunization against influenza, and pneumococcal infections. […] The anticipation and timely management of potentially specific complications arising from cyanosis due to the right-to-left shunt and associated morbidities, such as iron deficiency, thrombosis, gout, renal dysfunction, cholelithiasis, and cerebral infections are just as important to consider as the specific treatment of the PAH with therapies. […] Patients with an underlying cardiac condition, which includes any cyanotic CHD, are at higher risk of infective endocarditis and should receive prophylaxis. This, however, only applies to high-risk procedures, which are mainly invasive dental procedures, and not for respiratory tract procedures, gastrointestinal/urogenital, or skin/soft tissue procedures.

• #23 Eisenmenger syndrome: current perspectives | RRCC

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

  Current guidelines in the management of patients with ES recommend regular consultations with experienced physicians in CHD and PH. Areas that require routine consideration are detailed in the following sections, but also include adequate contraception, endocarditis prophylaxis, up-to-date immunization against influenza, and pneumococcal infections. […] The anticipation and timely management of potentially specific complications arising from cyanosis due to the right-to-left shunt and associated morbidities, such as iron deficiency, thrombosis, gout, renal dysfunction, cholelithiasis, and cerebral infections are just as important to consider as the specific treatment of the PAH with therapies. […] Patients with an underlying cardiac condition, which includes any cyanotic CHD, are at higher risk of infective endocarditis and should receive prophylaxis. This, however, only applies to high-risk procedures, which are mainly invasive dental procedures, and not for respiratory tract procedures, gastrointestinal/urogenital, or skin/soft tissue procedures.

• #24 Eisenmenger’s Syndrome | Doctor

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

  Prevention of infective endocarditis: The National Institute for Health and Care Excellence (NICE) recommends that if a person at risk of infective endocarditis is receiving antimicrobial therapy because they are undergoing a gastrointestinal or genitourinary procedure at a site where there is a suspected infection, the person should receive an antibiotic that covers organisms that cause infective endocarditis. […] Any episodes of infection in people at risk of infective endocarditis should be investigated and treated promptly to reduce the risk of endocarditis developing. […] Routine therapeutic venesections have no place in treatment, as they cause or exacerbate iron deficiency and reduce oxygen tissue delivery, thus increasing the risk of cerebrovascular events. Venesection should only be considered for patients with significantly elevated haemoglobin concentration and hematocrit.

• #25 Eisenmenger Syndrome – ACHA

  https://www.achaheart.org/your-heart/educational-qas/types-of-heart-defects/eisenmenger-syndrome/

  Management of complications include: […] Prevent infections by keep excellent oral health (mouth, gums, teeth) […] Take antibiotics to prevent subacute bacterial endocarditis when indicated […] Get your Covid-19, flu and pneumovax vaccines.

• #26 Eisenmenger Syndrome Treatment & Management: Approach Considerations, Oxygen Therapy, Pulmonary Vasodilator Therapy

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

  Prevention of Eisenmenger syndrome is critical. When recognized in a timely fashion, congenital cardiac defects can be effectively treated with minimal morbidity and mortality. Eisenmenger syndrome is, by definition, an untreated lesion that has progressed to the point of inoperability. […] Many congenital heart defects can be identified in utero when families receive appropriate prenatal care. Continued perinatal care and routine follow-up with a qualified pediatrician lead to the identification of most lesions that are not identified prenatally. […] In addition to clinicians providing patients with instruction regarding infective endocarditis risk reduction, patients can discourage complications of Eisenmenger syndrome with the following measures: Avoiding situations that exacerbate vasodilation, Avoiding abrupt, strenuous, or isometric exercise, Restricting activities that may result in further oxygen desaturation, symptomatic arrhythmias, or right ventricular dysfunction, Stopping steady-state exercise at the onset of symptoms, Following good dental hygiene to prevent infectious concerns, such as infective endocarditis and brain abscess.

• #27 Eisenmenger syndrome – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/eisenmenger-syndrome/diagnosis-treatment/drc-20350584

  The goals of Eisenmenger syndrome treatment are to: […] Prevent complications. […] Regular health checkups at least once a year are an important part of Eisenmenger syndrome treatment. […] Preventive antibiotics are only recommended in specific situations. Talk with your healthcare professional to learn if they are right for you. […] If you have Eisenmenger syndrome, it’s especially important to prevent respiratory infections such as the flu. Get a flu shot every year and a pneumonia vaccination every five years. […] Healthcare professionals say it’s critical that pregnancy be avoided if you have Eisenmenger syndrome. […] If you need treatment for Eisenmenger syndrome, get care at a medical center with healthcare professionals who have experience in congenital heart diseases.

• #28 Treatment for Eisenmenger Syndrome | Stanford Health Care

  https://stanfordhealthcare.org/medical-conditions/blood-heart-circulation/eisenmenger-syndrome/treatments.html

  Specific treatment for Eisenmenger syndrome will be determined by your doctor based on: […] The goals of treatment for Eisenmenger syndrome are aimed at decreasing the pulmonary artery pressure, improving oxygenation, and decreasing degree of cyanosis and erythrocytosis. […] Pregnancy is not recommended for females of childbearing age with Eisenmenger syndrome. Pregnancy may pose a high risk of death for the mother as well as complications for the fetus due to effects of low oxygen levels in the blood. […] Any anesthesia and surgery is considered high risk and should be carefully planned. Collaboration with a cardiac specialist is recommended. […] Air travel and high altitude exposure requires adequate hydration (fluid intake) and supplemental oxygen to prevent complications. […] Smoking and alcohol intake are not recommended. […] Coughing should be controlled or prevented with a strong suppressant medication to prevent risk of pulmonary hemorrhage (bleeding from the lungs). […] Flu shots are recommended annually, and pneumococcal vaccine should be received according to the doctor’s recommendation.

• #29

  https://www.banglajol.info/index.php/UHJ/article/view/78483

  Women with diagnosed case of Eisenmenger syndrome are usually advised to avoid pregnancy because of high mortality rate 30% to 50%, which increase up to 65% in case of caesarean section. […] Prevention and early termination is advisable. […] carefully coordinated multidisciplinary care involving experienced specialists from obstetrics, anesthesiology, cardiology, neonatology and critical care medicine is necessary to optimize the chances of survival for both mother and baby.

• #30 Eisenmenger syndrome | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/sindrome-de-eisenmenger/

  Early diagnosis and repair of congenital heart defects usually prevents Eisenmenger syndrome. […] If you have Eisenmenger syndrome, you are usually sent to a doctor trained in heart diseases, called a cardiologist. It’s helpful to find a cardiologist who has experience treating people who have congenital heart defects. Regular health checkups at least once a year are an important part of Eisenmenger syndrome treatment. […] Some dental and medical procedures may let germs into the bloodstream. Some people need to take antibiotics before surgery or dental procedures to prevent a heart infection called endocarditis. Preventive antibiotics are only recommended in specific situations. Talk with your healthcare professional to learn if they are right for you. […] Healthcare professionals don’t recommend surgery to repair the hole in the heart once Eisenmenger syndrome has developed. […] If you have Eisenmenger syndrome, pregnancy poses serious health risks and can be life-threatening for you and the baby. Healthcare professionals say it’s critical that pregnancy be avoided if you have Eisenmenger syndrome.

• #31 Conditions – Leeds Congenital Hearts

  https://leedscongenitalhearts.com/adult/conditions/view/4/17/eisenmenger-s-syndrome

  Eisenmengers Syndrome occurs when a hole in the heart (or other defect) allows too much blood to reach the lungs in childhood, damaging the lung arteries and leading to high blood pressure in the blood vessels in the lungs. This high blood pressure in the lungs is called pulmonary hypertension. […] There is no cure at present for Eisenmengers syndrome. Treatment is therefore aimed at controlling symptoms and preventing complications. It is tailored to each individual. […] We may prescribe you blood thinning medication to decrease your chance of forming clots. […] Pregnancy is not recommended in patients with Eisenmengers syndrome. It carries a risk to the mothers life of up to 50%. You should therefore use a reliable method of contraception. The combined oral contraceptive pill should be avoided but there are plenty of other progrestogen -only preparations which are very effective.

• #32 Conditions – Leeds Congenital Hearts

  https://www.leedscongenitalhearts.com/adult/conditions/view/4/17/eisenmenger-s-syndrome

  Eisenmengers Syndrome occurs when a hole in the heart (or other defect) allows too much blood to reach the lungs in childhood, damaging the lung arteries and leading to high blood pressure in the blood vessels in the lungs. This high blood pressure in the lungs is called pulmonary hypertension. As a result of the high pressure it is difficult for the heart to pump blood round your lungs and some blue blood leaks into the red side, often causing you to be a little blue (cyanosed). […] There is no cure at present for Eisenmengers syndrome. Treatment is therefore aimed at controlling symptoms and preventing complications. It is tailored to each individual. […] We may prescribe you blood thinning medication to decrease your chance of forming clots. […] Pregnancy is not recommended in patients with Eisenmengers syndrome. It carries a risk to the mothers life of up to 50%. You should therefore use a reliable method of contraception. The combined oral contraceptive pill should be avoided but there are plenty of other progrestogen -only preparations which are very effective.

• #33 2020 ESC Guidelines for the Management of ACHD: Focus on PH

  https://www.acc.org/Latest-in-Cardiology/Articles/2021/08/05/11/36/2020-ESC-Guidelines-for-the-Management-of-ACHD

  It is recommended that patients with PAH in CHD be cared for by a multidisciplinary team consisting of experts in cardiology, imaging, pulmonology, obstetrics, anesthesiology, neonatology, PH, cardiothoracic and thoracic surgery, nursing, and medical genetics. All patients with PAH in CHD should be given social and psychological support, be kept up to date on their vaccinations, and be advised to avoid excessive physical stress. […] All female patients with pre-capillary PH should be counseled against pregnancy (Class Ic recommendation). […] In patients with Eisenmenger syndrome, specifically, oxygen supplementation should be used only in cases in which there is a documented, consistent, and significant increase in oxygen saturation and improvement in symptoms. Routine phlebotomy should be avoided in these patients because secondary erythrocytosis is expected and, in fact, beneficial because this aides in oxygen transport and delivery.

• #34 Postoperative Management of Eisenmenger’s Syndrome

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

  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% [4,5]. […] Understanding Eisenmengers physiology and anticipating the hemodynamic changes during the surgical intervention with targeted treatment could lead to a reduction in significant mortality. […] The fundamental principles in perioperative management of patients with ES involve preventing systemic hypotension and avoiding elevation of pulmonary vascular resistance. […] Therefore, measures should be in place to detect and manage alteration in hemodynamics during induction of anesthesia and surgery [3,4,7]. […] Therefore, it is generally recommended to use vasopressors during induction to maintain SVR and cardiac output.

• #35 Treatment of adults with Eisenmenger syndrome—state of the art in the 21st century: a short overview

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8410485/

  Eisenmenger syndrome (ES) develops in association with unrepaired, non-restrictive cardiac shunt lesions at the atrial, ventricular or arterial level over time. […] 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. […] Preventing complications and avoiding management errors should be the primary goal of regular outpatient assessment. […] It is recommended to meticulously encourage patients to obtain yearly influenza immunization as well as immunization against pneumococcal disease every 5 years. […] As per current guidelines, Eisenmenger patients should be offered appropriate antibiotic endocarditis prophylaxis before dental procedures. […] If general anaesthesia is required for any non-cardiac or cardiac diagnostic or therapeutic procedure, this should be supervised by an experienced Anaesthesist, ideally at a tertiary center with experience in ACHD.

• #36 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

  If general anaesthesia is required for any non-cardiac or cardiac diagnostic or therapeutic procedure, this should be supervised by an experienced Anaesthesist, ideally at a tertiary center with experience in ACHD. […] In case of progressive deterioration discussion of heart lung transplantation should be initiated early and patients be offered referral to a transplantation service experienced in congenital heart disease. […] 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.

• #37 Postoperative Management of Eisenmenger’s Syndrome

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

  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% [4,5]. […] Understanding Eisenmengers physiology and anticipating the hemodynamic changes during the surgical intervention with targeted treatment could lead to a reduction in significant mortality. […] The fundamental principles in perioperative management of patients with ES involve preventing systemic hypotension and avoiding elevation of pulmonary vascular resistance. […] Therefore, measures should be in place to detect and manage alteration in hemodynamics during induction of anesthesia and surgery [3,4,7]. […] Therefore, it is generally recommended to use vasopressors during induction to maintain SVR and cardiac output.

• #38 Postoperative Management of Eisenmenger’s Syndrome

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

  Therefore, careful monitoring of fluid balance and replacement is necessary for maintaining normo-tension is critical for effective management of this patient population2. […] It is advisable to refer patients with ES to specialized centers capable of applying strategies aimed at the perioperative hemodynamic changes during surgical interventions [8-13]. […] As discussed in our case, close monitoring using standard tools or invasive monitoring, appropriate anesthetic use for induction, maintenance of SVR, oxygenation, pain control, use of vasopressors, and pulmonary therapies are important tools to consider in the perioperative management of ES.

• #39 Eisenmenger Syndrome – Everything you should know

  https://ckbirlahospitals.com/bmb/blog/eisenmenger-syndrome-meaning

  To prevent Eisenmenger syndrome, the only way is early diagnosis and congenital heart defects repair. There might be a need for frequent visits and medications when it comes to treatment if even after early diagnosis Eisenmenger develops. The treatment options help in improving the symptoms. […] The options for Eisenmenger syndrome treatment focus on improving the symptoms and averting any serious harm to the lungs or heart. Here are some of the options: […] Antibiotics are recommended to avert the risk of bacterial infections in the lungs or heart. […] Anticoagulants are given for the prevention of blood clotting risk. […] Diuretic medications are recommended as they help in reducing fluid accumulation in the body. […] Dual endothelin receptor antagonists are prescribed as they benefit in lowering the blood pressure in the lungs.

• #40 Eisenmenger syndrome – Leeds Teaching Hospitals NHS Trust

  https://www.leedsth.nhs.uk/patients/resources/eisenmenger-syndrome/

  This leaflet aims to give you an understanding of Eisenmenger syndrome and it’s management. […] Treatment is therefore aimed at controlling symptoms and preventing complications. It is tailored to each individual. […] We may prescribe you blood thinning medication to decrease your chance of forming clots. […] All patients with congenital heart disease are at risk of infection in the heart (endocarditis). The most common way bacteria can get into the blood stream and to the heart is through the mouth so it is important to practice good dental hygiene and visit the dentist every 12 months. If you need invasive dental treatment, we recommend antibiotic cover to be taken shortly before your dental appointment. […] Pregnancy is not recommended in patients with Eisenmenger syndrome. It carries a risk to the mother’s life of up to 50%. You should therefore use a reliable method of contraception. […] Remember to declare your heart condition when booking holiday insurance, to ensure you are properly covered.

• #41 Conditions – Leeds Congenital Hearts

  https://leedscongenitalhearts.com/adult/conditions/view/4/17/eisenmenger-s-syndrome

  Eisenmengers Syndrome occurs when a hole in the heart (or other defect) allows too much blood to reach the lungs in childhood, damaging the lung arteries and leading to high blood pressure in the blood vessels in the lungs. This high blood pressure in the lungs is called pulmonary hypertension. […] There is no cure at present for Eisenmengers syndrome. Treatment is therefore aimed at controlling symptoms and preventing complications. It is tailored to each individual. […] We may prescribe you blood thinning medication to decrease your chance of forming clots. […] Pregnancy is not recommended in patients with Eisenmengers syndrome. It carries a risk to the mothers life of up to 50%. You should therefore use a reliable method of contraception. The combined oral contraceptive pill should be avoided but there are plenty of other progrestogen -only preparations which are very effective.

• #42 Eisenmenger Syndrome – Everything you should know

  https://ckbirlahospitals.com/bmb/blog/eisenmenger-syndrome-meaning

  To prevent Eisenmenger syndrome, the only way is early diagnosis and congenital heart defects repair. There might be a need for frequent visits and medications when it comes to treatment if even after early diagnosis Eisenmenger develops. The treatment options help in improving the symptoms. […] The options for Eisenmenger syndrome treatment focus on improving the symptoms and averting any serious harm to the lungs or heart. Here are some of the options: […] Antibiotics are recommended to avert the risk of bacterial infections in the lungs or heart. […] Anticoagulants are given for the prevention of blood clotting risk. […] Diuretic medications are recommended as they help in reducing fluid accumulation in the body. […] Dual endothelin receptor antagonists are prescribed as they benefit in lowering the blood pressure in the lungs.

• #43 Eisenmenger Syndrome – Everything you should know

  https://ckbirlahospitals.com/bmb/blog/eisenmenger-syndrome-meaning

  To prevent Eisenmenger syndrome, the only way is early diagnosis and congenital heart defects repair. There might be a need for frequent visits and medications when it comes to treatment if even after early diagnosis Eisenmenger develops. The treatment options help in improving the symptoms. […] The options for Eisenmenger syndrome treatment focus on improving the symptoms and averting any serious harm to the lungs or heart. Here are some of the options: […] Antibiotics are recommended to avert the risk of bacterial infections in the lungs or heart. […] Anticoagulants are given for the prevention of blood clotting risk. […] Diuretic medications are recommended as they help in reducing fluid accumulation in the body. […] Dual endothelin receptor antagonists are prescribed as they benefit in lowering the blood pressure in the lungs.

• #44 Eisenmenger Syndrome – Everything you should know

  https://ckbirlahospitals.com/bmb/blog/eisenmenger-syndrome-meaning

  Iron supplements are given to patients with iron deficiency or anemia. […] Supplemental oxygen therapy is prescribed so that the patient can breathe better. […] People with serious symptoms and organ harm might require a heart or lung transplantation. […] Surgery might be recommended for people with serious symptoms of Eisenmenger syndrome complications. Phlebotomy is often recommended to treat and manage the symptoms.

• #45 Orphanet: Eisenmenger syndrome

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

  The disease can be avoided if appropriate repair of the underlying heart defect is undertaken prior to the establishment of irreversible changes to the pulmonary vascular bed. […] Management includes maintaining fluid balance, prophylaxis against infective endocarditis, iron supplementation and avoiding precipitating factors (pregnancy, isometric exercise, high altitude).

• #46 Review of Eisenmenger Syndrome

  https://www.acc.org/Latest-in-Cardiology/ten-points-to-remember/2022/03/30/17/59/Eisenmenger-Syndrome-JACC

  Eisenmenger syndrome (ES) is defined as pulmonary hypertension at a systemic level, caused by a high pulmonary vascular resistance (PVR), with reversed or bidirectional shunt at the aortopulmonary, ventricular, or atrial level. […] Routine anticoagulation is not indicated for patients with ES. Anticoagulation should be reserved for selected patients with atrial arrhythmias or known pulmonary artery thrombus or embolism. […] Advanced pulmonary arterial hypertension therapies have an important role in the management of patients with ES. The authors propose a staged approach beginning with monotherapy with an endothelin receptor antagonist or phosphodiesterase type 5 inhibitor. The next steps would include combination therapy, then triple therapy, and then assessment for lung or heart-lung transplantation. […] For this reason, women with ES should continue to be counseled against pregnancy. […] Patients require expert care at the time of noncardiac surgery, with the rate of major complications ranging from 5-25%. Expert anesthetic care and patient optimization are important in the perioperative period.

• #47 2020 ESC Guidelines for the Management of ACHD: Focus on PH

  https://www.acc.org/Latest-in-Cardiology/Articles/2021/08/05/11/36/2020-ESC-Guidelines-for-the-Management-of-ACHD

  Anticoagulation with vitamin K antagonists should be considered only in patients with concomitant atrial arrhythmias, mechanical heart valves, or vascular prostheses. There are no data on the use of non-vitamin K antagonist oral anticoagulant therapy in this patient population. The risk of bleeding does increase in cyanotic patients, so the use of oral anticoagulation and antiplatelet agents should be made on a case-by-case basis and with great consideration. […] Advanced therapies for PAH have been shown to benefit patients with Eisenmenger syndrome and likely will benefit other patients with PAH in CHD. Endothelin-receptor antagonist (ERA) therapy, specifically with bosentan, has been shown to improve objective measures as well as quality of life in patients with Eisenmenger syndrome, but its effect on mortality is less well known.

• #48 Review of Eisenmenger Syndrome

  https://www.acc.org/Latest-in-Cardiology/ten-points-to-remember/2022/03/30/17/59/Eisenmenger-Syndrome-JACC

  Eisenmenger syndrome (ES) is defined as pulmonary hypertension at a systemic level, caused by a high pulmonary vascular resistance (PVR), with reversed or bidirectional shunt at the aortopulmonary, ventricular, or atrial level. […] Routine anticoagulation is not indicated for patients with ES. Anticoagulation should be reserved for selected patients with atrial arrhythmias or known pulmonary artery thrombus or embolism. […] Advanced pulmonary arterial hypertension therapies have an important role in the management of patients with ES. The authors propose a staged approach beginning with monotherapy with an endothelin receptor antagonist or phosphodiesterase type 5 inhibitor. The next steps would include combination therapy, then triple therapy, and then assessment for lung or heart-lung transplantation. […] For this reason, women with ES should continue to be counseled against pregnancy. […] Patients require expert care at the time of noncardiac surgery, with the rate of major complications ranging from 5-25%. Expert anesthetic care and patient optimization are important in the perioperative period.

• #49 2020 ESC Guidelines for the Management of ACHD: Focus on PH

  https://www.acc.org/Latest-in-Cardiology/Articles/2021/08/05/11/36/2020-ESC-Guidelines-for-the-Management-of-ACHD

  Most treatment centers use a symptom-oriented treatment strategy, starting with an oral ERA or phosphodiesterase type 5 inhibitor in low- to intermediate-risk patients and then escalating therapy if symptoms persist or there is clinical deterioration (Class Ia recommendation). However, it must be remembered that most patients with PAH in CHD, including patients with Eisenmenger syndrome, are generally considered moderate- to high-risk patients and may require treatment with combination therapy and possible use of parenteral prostacyclins (Class Ia recommendation).

• #50 Eisenmenger Syndrome Treatment & Management: Approach Considerations, Oxygen Therapy, Pulmonary Vasodilator Therapy

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

  Prevention of Eisenmenger syndrome is critical. When recognized in a timely fashion, congenital cardiac defects can be effectively treated with minimal morbidity and mortality. Eisenmenger syndrome is, by definition, an untreated lesion that has progressed to the point of inoperability. […] Many congenital heart defects can be identified in utero when families receive appropriate prenatal care. Continued perinatal care and routine follow-up with a qualified pediatrician lead to the identification of most lesions that are not identified prenatally. […] In addition to clinicians providing patients with instruction regarding infective endocarditis risk reduction, patients can discourage complications of Eisenmenger syndrome with the following measures: Avoiding situations that exacerbate vasodilation, Avoiding abrupt, strenuous, or isometric exercise, Restricting activities that may result in further oxygen desaturation, symptomatic arrhythmias, or right ventricular dysfunction, Stopping steady-state exercise at the onset of symptoms, Following good dental hygiene to prevent infectious concerns, such as infective endocarditis and brain abscess.

• #51 Eisenmenger Syndrome: Symptoms, Cause, Treatment

  https://www.webmd.com/heart-disease/eisenmenger-syndrome-facts

  The choices you make are important in managing Eisenmenger syndrome. For example, pregnancy is extremely dangerous for women who have it. It can lead to the death of the mother or the child. […] Here are some things you can do to stay healthy: […] Never smoke or drink alcohol. […] Don’t overdo it with exercise. Don’t play competitive sports. Never scuba dive. […] Avoid salt, which can raise your blood pressure. […] Drink plenty of water. […] Don’t use drugs that put stress on your kidneys unless your doctor says to. That includes anti-inflammatory pain relievers. Aspirin is also dangerous because of the risk of bleeding. […] Avoid high altitudes. […] Get a flu shot and pneumonia vaccine. […] Take good care of your teeth, gums, and skin, and take antibiotics before dental visits. Germs that get in your bloodstream can damage your heart.

• #52 Eisenmenger Syndrome: Symptoms, Cause, Treatment

  https://www.webmd.com/heart-disease/eisenmenger-syndrome-facts

  The choices you make are important in managing Eisenmenger syndrome. For example, pregnancy is extremely dangerous for women who have it. It can lead to the death of the mother or the child. […] Here are some things you can do to stay healthy: […] Never smoke or drink alcohol. […] Don’t overdo it with exercise. Don’t play competitive sports. Never scuba dive. […] Avoid salt, which can raise your blood pressure. […] Drink plenty of water. […] Don’t use drugs that put stress on your kidneys unless your doctor says to. That includes anti-inflammatory pain relievers. Aspirin is also dangerous because of the risk of bleeding. […] Avoid high altitudes. […] Get a flu shot and pneumonia vaccine. […] Take good care of your teeth, gums, and skin, and take antibiotics before dental visits. Germs that get in your bloodstream can damage your heart.

• #53 Eisenmenger Syndrome | Baptist Health

  https://www.baptisthealth.com/care-services/conditions-treatments/eisenmenger-syndrome

  Eisenmenger Syndrome prevention: […] Your doctor may suggest steps you can take to help prevent future problems with Eisenmenger Syndrome. […] Ask your doctor about restrictions to your physical activity. […] Limit exposure to high-altitude environments due to lower oxygen levels. […] Stay hydrated, especially in warmer rooms or environments. […] Stop using any form of tobacco products. […] To avoid sudden drops in blood pressure, take shorter baths and showers, especially when the water is hot. […] Use warm water instead of hot water when taking baths and showers. […] Limit time in hot tubs. […] Check with your doctor before taking any medications or supplements. […] Protect yourself from infections by getting vaccinated. […] Check with your doctor before traveling by plane. […] Use contraceptives as pregnancy can be life-threatening.

• #54 Eisenmenger syndrome: current perspectives | RRCC

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

  Prophylactic or routine venesection to maintain a hematocrit level within an arbitrary predetermined level (hematocrit 65%) is not indicated. This is one of the major misconceptions in the management of patients with cyanotic CHD and routine venesection can be harmful to these patients, as they can result in iron deficiency, reduced exercise tolerance, impaired oxygen transport capacity due to relative anemia, and may increase the risk of cerebrovascular events. […] Venesection should only be offered to patients with moderate-to-severe hyperviscosity symptoms due to significant secondary erythrocytosis in the absence of volume depletion/dehydration.

• #55 Eisenmenger syndrome: current perspectives | RRCC

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

  Prophylactic or routine venesection to maintain a hematocrit level within an arbitrary predetermined level (hematocrit 65%) is not indicated. This is one of the major misconceptions in the management of patients with cyanotic CHD and routine venesection can be harmful to these patients, as they can result in iron deficiency, reduced exercise tolerance, impaired oxygen transport capacity due to relative anemia, and may increase the risk of cerebrovascular events. […] Venesection should only be offered to patients with moderate-to-severe hyperviscosity symptoms due to significant secondary erythrocytosis in the absence of volume depletion/dehydration.

• #56 Eisenmenger’s Syndrome | Doctor

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

  Prevention of infective endocarditis: The National Institute for Health and Care Excellence (NICE) recommends that if a person at risk of infective endocarditis is receiving antimicrobial therapy because they are undergoing a gastrointestinal or genitourinary procedure at a site where there is a suspected infection, the person should receive an antibiotic that covers organisms that cause infective endocarditis. […] Any episodes of infection in people at risk of infective endocarditis should be investigated and treated promptly to reduce the risk of endocarditis developing. […] Routine therapeutic venesections have no place in treatment, as they cause or exacerbate iron deficiency and reduce oxygen tissue delivery, thus increasing the risk of cerebrovascular events. Venesection should only be considered for patients with significantly elevated haemoglobin concentration and hematocrit.

• #57 328. ACHD: Eisenmenger Syndrome with Dr. Alexander Sasha Opotowsky

  https://www.cardionerds.com/328-achd-eisenmenger-syndrome-with-dr-alexander-sasha-opotowsky/

  Eisenmenger syndrome is a multi-organ disease and many manifestations occur due to secondary erythrocytosis. Prevention and treatment of these complications are the major goals of care in this population. […] Routine phlebotomy is no longer used in this population in the absence of moderate to severe hyperviscosity symptoms. Instead, the focus has shifted to iron deficiency. Repleting iron can improve symptoms of hyperviscosity, increases exercise capacity, and reduces stroke risk. […] It is contraindicated to close a shunt when Eisenmenger syndrome has developed. […] Among the few cardiac conditions where pregnancy is contraindicated are pulmonary arterial hypertension and Eisenmenger syndrome. In a pregnant woman with a resting oxygen saturation of less than 90%, babies are born small for gestational age, and most are born premature. In a pregnant woman with a resting oxygen saturation of less than 85%, less than 10% of babies are born alive. The maternal death rate is approximately 30% in these patients. Of note, some PAH medications are contraindicated in pregnancy.

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