Materiały źródłowe

• #1 Eisenmenger syndrome: diagnosis, prognosis and clinical management | Heart

  https://heart.bmj.com/content/106/21/1638.abstract

  Eisenmenger syndrome (ES) represents the most severe phenotype of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) and occurs in patients with large unrepaired shunts. […] Close follow-up is needed to early diagnose and timely manage these complications. […] Impaired exercise tolerance, decreased arterial oxygen saturation, iron deficiency, pre-tricuspid shunts, arrhythmias, increased brain natriuretic peptide, echocardiographic indices of right ventricular dysfunction and hospitalisation for heart failure predict mortality. […] Finally, the development of a risk score is of utmost importance to guide clinical therapy.

• #2 Eisenmenger Syndrome – StatPearls – NCBI Bookshelf

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

  Eisenmenger syndrome (ES) is a constellation of symptoms that arise from a congenital heart defect and result in large anatomic shunts. […] This activity discusses the evaluation and treatment of Eisenmenger syndrome and further defines the role of the interprofessional team in managing patients with this condition. […] All patients with suspicion for Eisenmenger syndrome should undergo a detailed history and physical, with particular attention on determining triggers for PAH. Patients should also undergo an evaluation to determine the degree of PAH as well as ventricular function. […] Workup should include pulse oximetry, chest radiograph, EKG, pulmonary function tests, complete blood count, iron studies, and cytidine monophosphate testing. Complete cardiac catheterization should occur at centers that specialize in the management of adult congenital heart disease and PAH. […] Eisenmenger syndrome is best managed by an interprofessional team that includes a cardiologist, pulmonologist, cardiac surgeon, internist, thoracic surgeon, and intensivist. […] Once Eisenmenger syndrome develops, the prognosis is poor.

• #3 Diagnosing Eisenmenger Syndrome | Stanford Health Care

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

  A child or adolescent with a history of an atrial septal defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA), or atrioventricular canal defect (AV canal) that has been unrepaired or repaired after one year of age is at risk for developing Eisenmenger syndrome. […] The doctor may note a change or increase in intensity of a cardiac murmur (a blowing or rasping sound heard while listening to the heart that may or may not indicate problems within the heart or circulatory system) during a routine physical examination. Complaints of change in exercise tolerance or any associated shortness of breath, chest pain, or palpitations, especially with activity, should be reported to the doctor for further investigation. […] The doctor will perform a physical examination, listening to the heart and lungs, and make other observations that help in the diagnosis. […] Other tests that may be performed to help with the diagnosis include the following: Blood Test, Cardiac Catheterization, Chest X-Ray, Echocardiogram, Electrocardiogram (ECG or EKG), Magnetic Resonance Imaging (MRI).

• #4 Eisenmenger Syndrome: Causes, Symptoms and Treatment

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

  Eisenmenger syndrome is the development of pulmonary hypertension (high blood pressure in the lungs) due to an untreated congenital heart defect. […] Eisenmenger syndrome typically develops in children and young adults who have an uncorrected ventricular septal defect (VSD). […] A healthcare provider typically notices signs of Eisenmenger syndrome during a physical examination. They may order the following tests to confirm the diagnosis: Blood oxygen level, Cardiac catheterization, Chest X-ray, Complete blood count (CBC), Echocardiogram (echo), Electrocardiogram (EKG), Heart MRI, Pulmonary function testing. […] Treatment for Eisenmenger syndrome is aimed at easing symptoms and preventing severe damage to the heart or lungs. […] The only way to prevent Eisenmenger syndrome is to have congenital heart defects repaired as early in life as possible. […] Symptoms tend to develop during puberty or early adulthood and may include cyanosis, exercise intolerance and heart rhythm abnormalities. Treatment is nonsurgical and focuses on easing symptoms and preventing severe heart and lung damage.

• #5 Eisenmenger Syndrome: Background, Pathophysiology, Etiology

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

  Eisenmenger syndrome refers to any untreated congenital cardiac defect with intracardiac communication that leads to pulmonary hypertension, reversal of flow, and cyanosis. […] The previous left-to-right shunt is converted into a right-to-left shunt secondary to elevated pulmonary artery pressures and associated pulmonary vascular disease. […] Advances in the medical treatment of patients with severe pulmonary hypertension may improve survival in patients with Eisenmenger syndrome and may potentially reverse the process in selected patients to a point at which they again become candidates for surgical repair. […] The size of the intracardiac shunt plays an important role in the likelihood of development of the syndrome. […] About 3% of patients with a small ventricular septal defect (VSD) (1.5 cm) and 50% of patients with a large VSD (1.5cm) can develop Eisenmenger syndrome.

• #6 Eisenmenger Syndrome: Background, Pathophysiology, Etiology

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

  Failure to reduce pulmonary pressures in the first 2 years of life may result in the irreversible progression of endothelial dysfunction and pulmonary vascular remodeling. […] The condition then advances to irreversible pulmonary hypertension. […] The usual life expectancy of a patient with Eisenmenger syndrome is 20-40 years if the syndrome is diagnosed promptly and treated with vigilance. […] A systematic review by Diller et al was able to estimate 10-year mortality rates of 30-40%. […] In a study of 153 German patients with Eisenmenger syndrome, 10-year mortality approached 60-70% in those who did not receive disease-directed therapy. […] A study by Salehian et al reported that left ventricular (LV) dysfunction (defined as LV ejection fraction [LVEF] 50%), right ventricular (RV) hypertrophy, arrhythmias, low serum albumin, and signs and symptoms of heart failure predict mortality in patients with Eisenmenger syndrome. […] A simple echocardiographic score that relies on RV and right atrial characteristics was found to predict adverse outcomes in patients with Eisenmenger syndrome that is not associated with complex congenital heart disease.

• #7 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 you have any symptoms of Eisenmenger syndrome, see your healthcare professional. Make an appointment even if you have never been diagnosed with a heart condition. […] Eisenmenger syndrome: Diagnosis, prognosis and clinical management.

• #8 Eisenmenger Syndrome – StatPearls – NCBI Bookshelf

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

  Eisenmenger syndrome (ES) is a constellation of symptoms that arise from a congenital heart defect and result in large anatomic shunts. […] This activity discusses the evaluation and treatment of Eisenmenger syndrome and further defines the role of the interprofessional team in managing patients with this condition. […] All patients with suspicion for Eisenmenger syndrome should undergo a detailed history and physical, with particular attention on determining triggers for PAH. Patients should also undergo an evaluation to determine the degree of PAH as well as ventricular function. […] Workup should include pulse oximetry, chest radiograph, EKG, pulmonary function tests, complete blood count, iron studies, and cytidine monophosphate testing. Complete cardiac catheterization should occur at centers that specialize in the management of adult congenital heart disease and PAH. […] Eisenmenger syndrome is best managed by an interprofessional team that includes a cardiologist, pulmonologist, cardiac surgeon, internist, thoracic surgeon, and intensivist. […] Once Eisenmenger syndrome develops, the prognosis is poor.

• #9 Eisenmenger Syndrome | Stanford Health Care

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

  Patients that are children or adolescents with a congenital heart disease that has not been repaired, or was repaired after age 1 are most at risk of developing Eisenmenger syndrome. […] Along with a physical examination of the heart and lungs your doctor may utilize the following tools to help diagnose Eisenmenger syndrome: Blood tests, Chest X-ray, Electrocardiogram (EKG or ECG), Echocardiogram, Cardiac catheterization, Magnetic resonance imaging (MRI). […] Eisenmenger Syndrome Our experts at Stanford diagnose and treat Eisenmenger syndrome using cardiac catheterization, phlebotomy and lung transplant.

• #10 Diagnosing Eisenmenger Syndrome | Stanford Health Care

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

  A child or adolescent with a history of an atrial septal defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA), or atrioventricular canal defect (AV canal) that has been unrepaired or repaired after one year of age is at risk for developing Eisenmenger syndrome. […] The doctor may note a change or increase in intensity of a cardiac murmur (a blowing or rasping sound heard while listening to the heart that may or may not indicate problems within the heart or circulatory system) during a routine physical examination. Complaints of change in exercise tolerance or any associated shortness of breath, chest pain, or palpitations, especially with activity, should be reported to the doctor for further investigation. […] The doctor will perform a physical examination, listening to the heart and lungs, and make other observations that help in the diagnosis. […] Other tests that may be performed to help with the diagnosis include the following: Blood Test, Cardiac Catheterization, Chest X-Ray, Echocardiogram, Electrocardiogram (ECG or EKG), Magnetic Resonance Imaging (MRI).

• #11 Eisenmenger Syndrome: Causes, Symptoms and Treatment

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

  Eisenmenger syndrome is the development of pulmonary hypertension (high blood pressure in the lungs) due to an untreated congenital heart defect. […] Eisenmenger syndrome typically develops in children and young adults who have an uncorrected ventricular septal defect (VSD). […] A healthcare provider typically notices signs of Eisenmenger syndrome during a physical examination. They may order the following tests to confirm the diagnosis: Blood oxygen level, Cardiac catheterization, Chest X-ray, Complete blood count (CBC), Echocardiogram (echo), Electrocardiogram (EKG), Heart MRI, Pulmonary function testing. […] Treatment for Eisenmenger syndrome is aimed at easing symptoms and preventing severe damage to the heart or lungs. […] The only way to prevent Eisenmenger syndrome is to have congenital heart defects repaired as early in life as possible. […] Symptoms tend to develop during puberty or early adulthood and may include cyanosis, exercise intolerance and heart rhythm abnormalities. Treatment is nonsurgical and focuses on easing symptoms and preventing severe heart and lung damage.

• #12 Diagnosing Eisenmenger Syndrome | Stanford Health Care

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

  A child or adolescent with a history of an atrial septal defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA), or atrioventricular canal defect (AV canal) that has been unrepaired or repaired after one year of age is at risk for developing Eisenmenger syndrome. […] The doctor may note a change or increase in intensity of a cardiac murmur (a blowing or rasping sound heard while listening to the heart that may or may not indicate problems within the heart or circulatory system) during a routine physical examination. Complaints of change in exercise tolerance or any associated shortness of breath, chest pain, or palpitations, especially with activity, should be reported to the doctor for further investigation. […] The doctor will perform a physical examination, listening to the heart and lungs, and make other observations that help in the diagnosis. […] Other tests that may be performed to help with the diagnosis include the following: Blood Test, Cardiac Catheterization, Chest X-Ray, Echocardiogram, Electrocardiogram (ECG or EKG), Magnetic Resonance Imaging (MRI).

• #13 Eisenmenger Syndrome: Causes, Symptoms, and Treatment

  https://www.medicoverhospitals.in/diseases/eisenmenger-syndrome/

  A thorough medical history and physical examination are the first steps in diagnosing Eisenmenger Syndrome. The physician will look for signs such as cyanosis, clubbing, and heart murmurs. […] Several diagnostic tests are crucial in confirming the diagnosis: […] Diagnosis is made through echocardiograms, chest X-rays, and other heart function tests.

• #14 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

  Currently, increased understanding of the pathophysiology of ES together with the reported success of disease-specific treatment for pulmonary arterial hypertension (PAH) using advanced therapies (ATs), including phosphodiesterase type 5 inhibitors, endothelin receptor antagonists, and prostacyclin analogs, have offered new hope for patients with ES. […] By adopting similar strategies for the treatment of ES, our institution has now seen a significant improvement in survival for these patients to 50–60 years. […] The signs and symptoms of ES in advanced stages include central cyanosis, dyspnea, fatigue, dizziness, hemoptysis, syncope, and reduced quality and expectancy of life. […] In our study, five out of six patients died during follow-up. Among them, three (60%) died of irreversible right heart failure, and one each died of brain abscess and sudden cardiac death. […] The life span of the mortality cases was 53.3 ± 8.2 years, similar to the previous reports.

• #15 Eisenmenger Syndrome – StatPearls – NCBI Bookshelf

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

  Eisenmenger syndrome (ES) is a constellation of symptoms that arise from a congenital heart defect and result in large anatomic shunts. […] This activity discusses the evaluation and treatment of Eisenmenger syndrome and further defines the role of the interprofessional team in managing patients with this condition. […] All patients with suspicion for Eisenmenger syndrome should undergo a detailed history and physical, with particular attention on determining triggers for PAH. Patients should also undergo an evaluation to determine the degree of PAH as well as ventricular function. […] Workup should include pulse oximetry, chest radiograph, EKG, pulmonary function tests, complete blood count, iron studies, and cytidine monophosphate testing. Complete cardiac catheterization should occur at centers that specialize in the management of adult congenital heart disease and PAH. […] Eisenmenger syndrome is best managed by an interprofessional team that includes a cardiologist, pulmonologist, cardiac surgeon, internist, thoracic surgeon, and intensivist. […] Once Eisenmenger syndrome develops, the prognosis is poor.

• #16 Eisenmenger Syndrome Workup: Approach Considerations, Laboratory Studies, Chest Radiography and MRI

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

  Laboratory studies used in the diagnosis of Eisenmenger syndrome include complete blood cell count, biochemical profiles, and iron studies, in addition to blood gas assessments. Electrocardiography can also reveal signs of an underlying cardiac defect and of right ventricular hypertrophy. Imaging studies can reveal cardiac structural defects and pulmonary changes, including irreversible alterations in the pulmonary system. Histologic findings can be used to determine the stage of pulmonary vascular pathology. The updated 2018 AHA/ACC guidelines for adults with congenital heart disease (ACHD) emphasize the need for advanced cardiac imaging and cardiac catheterization data accuracy to exclude other etiologies of the right-to-left shunt physiology (Class IC recommendation). […] Preoperatively, the combination of 100% oxygen or nitric oxide are used to evaluate pulmonary vasculature reactivity in pulmonary hypertension. If the pulmonary vascular resistance (PVR) does not decrease with this test, the PVR is considered irreversible, and the patient may not be a good surgical candidate for corrective cardiac surgery.

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

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

  To diagnose Eisenmenger syndrome, your healthcare professional examines you and asks questions about your symptoms and medical history. […] Tests to diagnose Eisenmenger syndrome may include: […] A complete blood cell count is often done. The number of red blood cells may be high in Eisenmenger syndrome. […] An ECG can show how fast or slow the heart is beating. […] A chest X-ray shows the condition of the heart and lungs. […] An echocardiogram shows blood flow through the heart and heart valves. […] This type of CT scan uses X-rays to create detailed images of the lungs and lung arteries. […] This test uses magnetic fields and radio waves to create detailed images of the blood vessels in the lungs. […] This test shows how blood flows through the heart arteries. […] You might be asked to walk for several minutes to see how your body reacts to mild exercise.

• #18 Eisenmenger syndrome | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/%D9%85%D8%AA%D9%84%D8%A7%D8%B2%D9%85%D8%A9-%D8%A3%D9%8A%D8%B2%D9%86%D9%85%D9%8A%D9%86%D8%AC%D8%B1?content_id=CON-20155125

  To diagnose Eisenmenger syndrome, your healthcare professional examines you and asks questions about your symptoms and medical history. […] Tests to diagnose Eisenmenger syndrome may include: […] A complete blood cell count is often done. The number of red blood cells may be high in Eisenmenger syndrome. Blood tests also are done to see how well the kidneys and liver are working. […] An electrocardiogram (ECG or EKG) measures the electrical activity of the heart. […] A chest X-ray shows the condition of the heart and lungs. […] An echocardiogram shows blood flow through the heart and heart valves. […] A computerized tomography (CT) scan uses X-rays to create detailed images of the lungs and lung arteries. […] A magnetic resonance imaging (MRI) scan uses magnetic fields and radio waves to create detailed images of the blood vessels in the lungs. […] Cardiac catheterization shows how blood flows through the heart arteries. […] You might be asked to walk for several minutes to see how your body reacts to mild exercise.

• #19 Orphanet: Eisenmenger syndrome

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

  Diagnosis is based on the clinical features, pulse oximetry findings, an abnormal electrocardiogram (right axis deviation, right ventricular hypertrophy and right atrial enlargement), echocardiography (underlying lesion, site of shunt and estimating pulmonary arterial pressure) and imaging (MRI and CT). […] Right-heart catheterization (saturation, hemodynamics and pulmonary vasoreactivity study if appropriate) is usual to establish the diagnosis and may be clinically useful in planning treatment / assessing efficacy. […] Routine lab testing (total blood count, liver function test, urea, creatinine, electrolytes, uric acid and iron status) is sought to detect emerging complications.

• #20 Eisenmenger syndrome: diagnosis, prognosis and clinical management – PubMed

  https://pubmed.ncbi.nlm.nih.gov/32690623/

  Eisenmenger syndrome (ES) represents the most severe phenotype of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) and occurs in patients with large unrepaired shunts. […] Close follow-up is needed to early diagnose and timely manage these complications. […] Impaired exercise tolerance, decreased arterial oxygen saturation, iron deficiency, pre-tricuspid shunts, arrhythmias, increased brain natriuretic peptide, echocardiographic indices of right ventricular dysfunction and hospitalisation for heart failure predict mortality. […] Due to the lack of evidence, current guidelines do not provide a clear therapeutic strategy regarding treatment escalation. […] Finally, the development of a risk score is of utmost importance to guide clinical therapy.

• #21 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.

  Laboratory tests reveal abnormalities that are secondary to hypoxemia; hemoglobin oxygen saturation (SaO2) is usually 90% (measured at rest for at least 5 minutes). […] Electrocardiography (ECG): Features of right atrial enlargement (if in sinus rhythm) and features of right ventricular hypertrophy (often with strain pattern) and overload are the dominant features of ECG. […] Chest radiography: The size of the heart may vary depending on the primary defect and severity of Eisenmenger syndrome. […] Echocardiography: Right ventricular hypertrophy with color and spectral Doppler evidence of bidirectional flow through the shunt, and severe elevation in right ventricular systolic pressures. […] Other abnormalities such as mitral or tricuspid regurgitation can be present and depend on the severity of Eisenmenger syndrome. […] Exercise capacity should be assessed with the 6-minute walk test.

• #22 Eisenmenger syndrome: diagnosis, prognosis and clinical management – PubMed

  https://pubmed.ncbi.nlm.nih.gov/32690623/

  Eisenmenger syndrome (ES) represents the most severe phenotype of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) and occurs in patients with large unrepaired shunts. […] Close follow-up is needed to early diagnose and timely manage these complications. […] Impaired exercise tolerance, decreased arterial oxygen saturation, iron deficiency, pre-tricuspid shunts, arrhythmias, increased brain natriuretic peptide, echocardiographic indices of right ventricular dysfunction and hospitalisation for heart failure predict mortality. […] Due to the lack of evidence, current guidelines do not provide a clear therapeutic strategy regarding treatment escalation. […] Finally, the development of a risk score is of utmost importance to guide clinical therapy.

• #23 Eisenmenger Syndrome – StatPearls – NCBI Bookshelf

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

  Eisenmenger syndrome (ES) is a constellation of symptoms that arise from a congenital heart defect and result in large anatomic shunts. […] This activity discusses the evaluation and treatment of Eisenmenger syndrome and further defines the role of the interprofessional team in managing patients with this condition. […] All patients with suspicion for Eisenmenger syndrome should undergo a detailed history and physical, with particular attention on determining triggers for PAH. Patients should also undergo an evaluation to determine the degree of PAH as well as ventricular function. […] Workup should include pulse oximetry, chest radiograph, EKG, pulmonary function tests, complete blood count, iron studies, and cytidine monophosphate testing. Complete cardiac catheterization should occur at centers that specialize in the management of adult congenital heart disease and PAH. […] Eisenmenger syndrome is best managed by an interprofessional team that includes a cardiologist, pulmonologist, cardiac surgeon, internist, thoracic surgeon, and intensivist. […] Once Eisenmenger syndrome develops, the prognosis is poor.

• #24 Eisenmenger Syndrome Workup: Approach Considerations, Laboratory Studies, Chest Radiography and MRI

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

  Laboratory studies used in the diagnosis of Eisenmenger syndrome include complete blood cell count, biochemical profiles, and iron studies, in addition to blood gas assessments. Electrocardiography can also reveal signs of an underlying cardiac defect and of right ventricular hypertrophy. Imaging studies can reveal cardiac structural defects and pulmonary changes, including irreversible alterations in the pulmonary system. Histologic findings can be used to determine the stage of pulmonary vascular pathology. The updated 2018 AHA/ACC guidelines for adults with congenital heart disease (ACHD) emphasize the need for advanced cardiac imaging and cardiac catheterization data accuracy to exclude other etiologies of the right-to-left shunt physiology (Class IC recommendation). […] Preoperatively, the combination of 100% oxygen or nitric oxide are used to evaluate pulmonary vasculature reactivity in pulmonary hypertension. If the pulmonary vascular resistance (PVR) does not decrease with this test, the PVR is considered irreversible, and the patient may not be a good surgical candidate for corrective cardiac surgery.

• #25 Orphanet: Eisenmenger syndrome

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

  Diagnosis is based on the clinical features, pulse oximetry findings, an abnormal electrocardiogram (right axis deviation, right ventricular hypertrophy and right atrial enlargement), echocardiography (underlying lesion, site of shunt and estimating pulmonary arterial pressure) and imaging (MRI and CT). […] Right-heart catheterization (saturation, hemodynamics and pulmonary vasoreactivity study if appropriate) is usual to establish the diagnosis and may be clinically useful in planning treatment / assessing efficacy. […] Routine lab testing (total blood count, liver function test, urea, creatinine, electrolytes, uric acid and iron status) is sought to detect emerging complications.

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

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

  Eisenmenger syndrome is a complication of uncorrected large intracardiac or aortic to pulmonary artery left-to-right shunts. […] Diagnosis is based on echocardiography or advanced imaging and cardiac catheterization. […] Diagnosis of Eisenmenger syndrome is suspected by history of uncorrected cardiac anomalies, supported by chest x-ray and ECG findings, and established by 2-dimensional echocardiography with color flow and Doppler studies. Cardiac catheterization is often done to measure pulmonary artery pressure, pulmonary vascular resistance, and response to pulmonary vasodilators. […] Chest x-ray usually shows prominent central pulmonary arteries, peripheral pulmonary vessel pruning, and right heart enlargement. […] ECG shows right ventricular hypertrophy, right axis deviation, and, occasionally, right atrial enlargement.

• #27 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.

  Laboratory tests reveal abnormalities that are secondary to hypoxemia; hemoglobin oxygen saturation (SaO2) is usually 90% (measured at rest for at least 5 minutes). […] Electrocardiography (ECG): Features of right atrial enlargement (if in sinus rhythm) and features of right ventricular hypertrophy (often with strain pattern) and overload are the dominant features of ECG. […] Chest radiography: The size of the heart may vary depending on the primary defect and severity of Eisenmenger syndrome. […] Echocardiography: Right ventricular hypertrophy with color and spectral Doppler evidence of bidirectional flow through the shunt, and severe elevation in right ventricular systolic pressures. […] Other abnormalities such as mitral or tricuspid regurgitation can be present and depend on the severity of Eisenmenger syndrome. […] Exercise capacity should be assessed with the 6-minute walk test.

• #28 Image diagnosis: Eisenmenger’s syndrome in patients with simple congenital heart disease | BMC Cardiovascular Disorders | Full Text

  https://bmccardiovascdisord.biomedcentral.com/articles/10.1186/s12872-020-01489-y

  Early identification of congenital heart disease (CHD) allows detection of the pulmonary arteriopathy in an early stage, and timely shunt closure can permanently reverse pulmonary arterial hypertension (PAH). […] The diagnosis of pulmonary hypertension can be confirmed only by right heart catheterization. […] Echocardiography is the mainstay of non-invasive diagnostic tool during the early screening that depicts pulmonary hypertension or right heart overload. […] Although echocardiographic diagnosis has been described, cardiac catheterization is still considered the gold standard method of diagnosis. […] The main purposes are: (1) To accurately measure the right atrial and right ventricular pressure, PAP, PAWP, cardiac output and other hemodynamic parameters, which are helpful to assess the severity of PAH.

• #29 Eisenmenger syndrome | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/%D9%85%D8%AA%D9%84%D8%A7%D8%B2%D9%85%D8%A9-%D8%A3%D9%8A%D8%B2%D9%86%D9%85%D9%8A%D9%86%D8%AC%D8%B1?content_id=CON-20155125

  To diagnose Eisenmenger syndrome, your healthcare professional examines you and asks questions about your symptoms and medical history. […] Tests to diagnose Eisenmenger syndrome may include: […] A complete blood cell count is often done. The number of red blood cells may be high in Eisenmenger syndrome. Blood tests also are done to see how well the kidneys and liver are working. […] An electrocardiogram (ECG or EKG) measures the electrical activity of the heart. […] A chest X-ray shows the condition of the heart and lungs. […] An echocardiogram shows blood flow through the heart and heart valves. […] A computerized tomography (CT) scan uses X-rays to create detailed images of the lungs and lung arteries. […] A magnetic resonance imaging (MRI) scan uses magnetic fields and radio waves to create detailed images of the blood vessels in the lungs. […] Cardiac catheterization shows how blood flows through the heart arteries. […] You might be asked to walk for several minutes to see how your body reacts to mild exercise.

• #30 Eisenmenger Syndrome in Children | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions—pediatrics/e/eisenmenger-syndrome.html

  Eisenmenger syndrome is an advanced form of pulmonary artery hypertension. […] How is Eisenmenger syndrome diagnosed in a child? Your child’s healthcare provider will ask about your childs symptoms. The provider may see signs when examining your child. Your provider may hear an abnormal heart sound when listening to your child’s heart with a stethoscope. […] Your child may need tests. These may include: Pulse oximetry. A small probe is used to check the amount of oxygen in the blood. Blood tests. Tests may be done to check for a high or low red blood cell counts. Chest X-ray. A chest X-ray shows the heart and lungs. There may be changes in the lungs because of extra blood flow. Electrocardiogram (ECG). An ECG records the electrical activity of the heart. It also shows abnormal rhythms and detects heart muscle stress. Echocardiogram (echo). An echo uses sound waves to make a moving picture of the heart and heart valves. An echo can show the direction of blood flow. It can find out the size of a defect. Different types of echo tests may be done. Pulmonary (lung) function testing. Lung function testing checks how well your child’s lungs are working. Cardiac cath (catheterization). A cardiac cath gives very detailed information about the structures inside the heart. The healthcare provider puts a small, flexible tube (catheter) into a large blood vessel in the groin. The healthcare provider guides the catheter to the heart. They measure blood pressure and oxygen. In some cases, the healthcare provider may inject contrast dye to more clearly see the blood flow and structures inside the heart. Cardiovascular MRI (CMRI). This test makes detailed images of the heart and blood vessels. MRI may be used to check the amount and direction of blood shunting. CT scan and CT angiography. These tests may be used to check for blood clots in the lung arteries and lung. Stress test. This is an ECG done while the child is exercising.

• #31 Eisenmenger Syndrome in Children | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions—pediatrics/e/eisenmenger-syndrome.html

  Eisenmenger syndrome is an advanced form of pulmonary artery hypertension. […] How is Eisenmenger syndrome diagnosed in a child? Your child’s healthcare provider will ask about your childs symptoms. The provider may see signs when examining your child. Your provider may hear an abnormal heart sound when listening to your child’s heart with a stethoscope. […] Your child may need tests. These may include: Pulse oximetry. A small probe is used to check the amount of oxygen in the blood. Blood tests. Tests may be done to check for a high or low red blood cell counts. Chest X-ray. A chest X-ray shows the heart and lungs. There may be changes in the lungs because of extra blood flow. Electrocardiogram (ECG). An ECG records the electrical activity of the heart. It also shows abnormal rhythms and detects heart muscle stress. Echocardiogram (echo). An echo uses sound waves to make a moving picture of the heart and heart valves. An echo can show the direction of blood flow. It can find out the size of a defect. Different types of echo tests may be done. Pulmonary (lung) function testing. Lung function testing checks how well your child’s lungs are working. Cardiac cath (catheterization). A cardiac cath gives very detailed information about the structures inside the heart. The healthcare provider puts a small, flexible tube (catheter) into a large blood vessel in the groin. The healthcare provider guides the catheter to the heart. They measure blood pressure and oxygen. In some cases, the healthcare provider may inject contrast dye to more clearly see the blood flow and structures inside the heart. Cardiovascular MRI (CMRI). This test makes detailed images of the heart and blood vessels. MRI may be used to check the amount and direction of blood shunting. CT scan and CT angiography. These tests may be used to check for blood clots in the lung arteries and lung. Stress test. This is an ECG done while the child is exercising.

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

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

  To diagnose Eisenmenger syndrome, your healthcare professional examines you and asks questions about your symptoms and medical history. […] Tests to diagnose Eisenmenger syndrome may include: […] A complete blood cell count is often done. The number of red blood cells may be high in Eisenmenger syndrome. […] An ECG can show how fast or slow the heart is beating. […] A chest X-ray shows the condition of the heart and lungs. […] An echocardiogram shows blood flow through the heart and heart valves. […] This type of CT scan uses X-rays to create detailed images of the lungs and lung arteries. […] This test uses magnetic fields and radio waves to create detailed images of the blood vessels in the lungs. […] This test shows how blood flows through the heart arteries. […] You might be asked to walk for several minutes to see how your body reacts to mild exercise.

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

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

  Eisenmenger syndrome is a complication of uncorrected large intracardiac or aortic to pulmonary artery left-to-right shunts. […] Diagnosis is based on echocardiography or advanced imaging and cardiac catheterization. […] Diagnosis of Eisenmenger syndrome is suspected by history of uncorrected cardiac anomalies, supported by chest x-ray and ECG findings, and established by 2-dimensional echocardiography with color flow and Doppler studies. Cardiac catheterization is often done to measure pulmonary artery pressure, pulmonary vascular resistance, and response to pulmonary vasodilators. […] Chest x-ray usually shows prominent central pulmonary arteries, peripheral pulmonary vessel pruning, and right heart enlargement. […] ECG shows right ventricular hypertrophy, right axis deviation, and, occasionally, right atrial enlargement.

• #34 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.

  Laboratory tests reveal abnormalities that are secondary to hypoxemia; hemoglobin oxygen saturation (SaO2) is usually 90% (measured at rest for at least 5 minutes). […] Electrocardiography (ECG): Features of right atrial enlargement (if in sinus rhythm) and features of right ventricular hypertrophy (often with strain pattern) and overload are the dominant features of ECG. […] Chest radiography: The size of the heart may vary depending on the primary defect and severity of Eisenmenger syndrome. […] Echocardiography: Right ventricular hypertrophy with color and spectral Doppler evidence of bidirectional flow through the shunt, and severe elevation in right ventricular systolic pressures. […] Other abnormalities such as mitral or tricuspid regurgitation can be present and depend on the severity of Eisenmenger syndrome. […] Exercise capacity should be assessed with the 6-minute walk test.

• #35 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.

  Laboratory tests reveal abnormalities that are secondary to hypoxemia; hemoglobin oxygen saturation (SaO2) is usually 90% (measured at rest for at least 5 minutes). […] Electrocardiography (ECG): Features of right atrial enlargement (if in sinus rhythm) and features of right ventricular hypertrophy (often with strain pattern) and overload are the dominant features of ECG. […] Chest radiography: The size of the heart may vary depending on the primary defect and severity of Eisenmenger syndrome. […] Echocardiography: Right ventricular hypertrophy with color and spectral Doppler evidence of bidirectional flow through the shunt, and severe elevation in right ventricular systolic pressures. […] Other abnormalities such as mitral or tricuspid regurgitation can be present and depend on the severity of Eisenmenger syndrome. […] Exercise capacity should be assessed with the 6-minute walk test.

• #36 Eisenmenger syndrome: diagnosis, prognosis and clinical management – PubMed

  https://pubmed.ncbi.nlm.nih.gov/32690623/

  Eisenmenger syndrome (ES) represents the most severe phenotype of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) and occurs in patients with large unrepaired shunts. […] Close follow-up is needed to early diagnose and timely manage these complications. […] Impaired exercise tolerance, decreased arterial oxygen saturation, iron deficiency, pre-tricuspid shunts, arrhythmias, increased brain natriuretic peptide, echocardiographic indices of right ventricular dysfunction and hospitalisation for heart failure predict mortality. […] Due to the lack of evidence, current guidelines do not provide a clear therapeutic strategy regarding treatment escalation. […] Finally, the development of a risk score is of utmost importance to guide clinical therapy.

• #37 Eisenmenger Syndrome – StatPearls – NCBI Bookshelf

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

  Eisenmenger syndrome (ES) is a constellation of symptoms that arise from a congenital heart defect and result in large anatomic shunts. […] This activity discusses the evaluation and treatment of Eisenmenger syndrome and further defines the role of the interprofessional team in managing patients with this condition. […] All patients with suspicion for Eisenmenger syndrome should undergo a detailed history and physical, with particular attention on determining triggers for PAH. Patients should also undergo an evaluation to determine the degree of PAH as well as ventricular function. […] Workup should include pulse oximetry, chest radiograph, EKG, pulmonary function tests, complete blood count, iron studies, and cytidine monophosphate testing. Complete cardiac catheterization should occur at centers that specialize in the management of adult congenital heart disease and PAH. […] Eisenmenger syndrome is best managed by an interprofessional team that includes a cardiologist, pulmonologist, cardiac surgeon, internist, thoracic surgeon, and intensivist. […] Once Eisenmenger syndrome develops, the prognosis is poor.

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

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

  Eisenmenger syndrome is an end-stage complication of congenital heart disease that occurs when a left to right shunt causes pulmonary over-circulation, leading to vascular remodeling, increased vascular resistance, and ultimately even shunt reversal. […] Evaluation for Eisenmenger syndrome should include a comprehensive history, physical exam, ECG, echocardiogram, cardiac catheterization, and laboratory work to identify multi-system complications of cyanosis and secondary erythrocytosis. […] The most definitive means to diagnose Eisenmenger syndrome in a patient with a prior left-to-right shunt lesion is with a right heart cardiac catheterization showing right to left shunting (Qp:Qs 1). […] Remember: You must have had a right heart cardiac catheterization to diagnose someone with Eisenmenger syndrome.

• #39 Eisenmenger Syndrome Workup: Approach Considerations, Laboratory Studies, Chest Radiography and MRI

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

  Cardiac catheterization can be of value in patients with Eisenmenger syndrome, after collecting clinical and noninvasive data, to confirm and/or demonstrate the following: Severity of pulmonary arterial hypertension, Conduit patency and pressure gradient, Coexisting coronary artery anomalies (rare), Degree of shunting. […] In patients with Eisenmenger syndrome and severe pulmonary vascular disease, histologic analysis reveals abnormal extension of muscle into small peripheral arteries, severe medial smooth muscle hypertrophy of existing muscular arteries, plexiform lesions and increased intercellular material, and a reduction in the overall concentration and size of arteries.

• #40 Image diagnosis: Eisenmenger’s syndrome in patients with simple congenital heart disease | BMC Cardiovascular Disorders | Full Text

  https://bmccardiovascdisord.biomedcentral.com/articles/10.1186/s12872-020-01489-y

  Early identification of congenital heart disease (CHD) allows detection of the pulmonary arteriopathy in an early stage, and timely shunt closure can permanently reverse pulmonary arterial hypertension (PAH). […] The diagnosis of pulmonary hypertension can be confirmed only by right heart catheterization. […] Echocardiography is the mainstay of non-invasive diagnostic tool during the early screening that depicts pulmonary hypertension or right heart overload. […] Although echocardiographic diagnosis has been described, cardiac catheterization is still considered the gold standard method of diagnosis. […] The main purposes are: (1) To accurately measure the right atrial and right ventricular pressure, PAP, PAWP, cardiac output and other hemodynamic parameters, which are helpful to assess the severity of PAH.

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

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

  Eisenmenger syndrome is an end-stage complication of congenital heart disease that occurs when a left to right shunt causes pulmonary over-circulation, leading to vascular remodeling, increased vascular resistance, and ultimately even shunt reversal. […] Evaluation for Eisenmenger syndrome should include a comprehensive history, physical exam, ECG, echocardiogram, cardiac catheterization, and laboratory work to identify multi-system complications of cyanosis and secondary erythrocytosis. […] The most definitive means to diagnose Eisenmenger syndrome in a patient with a prior left-to-right shunt lesion is with a right heart cardiac catheterization showing right to left shunting (Qp:Qs 1). […] Remember: You must have had a right heart cardiac catheterization to diagnose someone with Eisenmenger syndrome.

• #42 Eisenmenger Syndrome Workup: Approach Considerations, Laboratory Studies, Chest Radiography and MRI

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

  Laboratory studies used in the diagnosis of Eisenmenger syndrome include complete blood cell count, biochemical profiles, and iron studies, in addition to blood gas assessments. Electrocardiography can also reveal signs of an underlying cardiac defect and of right ventricular hypertrophy. Imaging studies can reveal cardiac structural defects and pulmonary changes, including irreversible alterations in the pulmonary system. Histologic findings can be used to determine the stage of pulmonary vascular pathology. The updated 2018 AHA/ACC guidelines for adults with congenital heart disease (ACHD) emphasize the need for advanced cardiac imaging and cardiac catheterization data accuracy to exclude other etiologies of the right-to-left shunt physiology (Class IC recommendation). […] Preoperatively, the combination of 100% oxygen or nitric oxide are used to evaluate pulmonary vasculature reactivity in pulmonary hypertension. If the pulmonary vascular resistance (PVR) does not decrease with this test, the PVR is considered irreversible, and the patient may not be a good surgical candidate for corrective cardiac surgery.

• #43 Image diagnosis: Eisenmenger’s syndrome in patients with simple congenital heart disease | BMC Cardiovascular Disorders | Full Text

  https://bmccardiovascdisord.biomedcentral.com/articles/10.1186/s12872-020-01489-y

  According to current guidelines, assessment of reversibility is limited to hemodynamic variables: those in favor of reversible PAH-CHD are a left-to-right shunt and a PVR index 4 Woods units. […] Shunt closure is contraindicated when the net shunt is directed right-to-left, and is discouraged when the PVR index is 8 Woods units. […] The assessment of reversibility is nowadays primarily based on clinical judgement and measurements of hemodynamic variables, which have limitations as surrogates for the stage of the arteriopathy. […] In our study, the surgical correction was not performed in all patients who had fixed pulmonary hypertension.

• #44 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.

  Laboratory tests reveal abnormalities that are secondary to hypoxemia; hemoglobin oxygen saturation (SaO2) is usually 90% (measured at rest for at least 5 minutes). […] Electrocardiography (ECG): Features of right atrial enlargement (if in sinus rhythm) and features of right ventricular hypertrophy (often with strain pattern) and overload are the dominant features of ECG. […] Chest radiography: The size of the heart may vary depending on the primary defect and severity of Eisenmenger syndrome. […] Echocardiography: Right ventricular hypertrophy with color and spectral Doppler evidence of bidirectional flow through the shunt, and severe elevation in right ventricular systolic pressures. […] Other abnormalities such as mitral or tricuspid regurgitation can be present and depend on the severity of Eisenmenger syndrome. […] Exercise capacity should be assessed with the 6-minute walk test.

• #45 Eisenmenger syndrome | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/%D9%85%D8%AA%D9%84%D8%A7%D8%B2%D9%85%D8%A9-%D8%A3%D9%8A%D8%B2%D9%86%D9%85%D9%8A%D9%86%D8%AC%D8%B1?content_id=CON-20155125

  To diagnose Eisenmenger syndrome, your healthcare professional examines you and asks questions about your symptoms and medical history. […] Tests to diagnose Eisenmenger syndrome may include: […] A complete blood cell count is often done. The number of red blood cells may be high in Eisenmenger syndrome. Blood tests also are done to see how well the kidneys and liver are working. […] An electrocardiogram (ECG or EKG) measures the electrical activity of the heart. […] A chest X-ray shows the condition of the heart and lungs. […] An echocardiogram shows blood flow through the heart and heart valves. […] A computerized tomography (CT) scan uses X-rays to create detailed images of the lungs and lung arteries. […] A magnetic resonance imaging (MRI) scan uses magnetic fields and radio waves to create detailed images of the blood vessels in the lungs. […] Cardiac catheterization shows how blood flows through the heart arteries. […] You might be asked to walk for several minutes to see how your body reacts to mild exercise.

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

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

  To diagnose Eisenmenger syndrome, your healthcare professional examines you and asks questions about your symptoms and medical history. […] Tests to diagnose Eisenmenger syndrome may include: […] A complete blood cell count is often done. The number of red blood cells may be high in Eisenmenger syndrome. […] An ECG can show how fast or slow the heart is beating. […] A chest X-ray shows the condition of the heart and lungs. […] An echocardiogram shows blood flow through the heart and heart valves. […] This type of CT scan uses X-rays to create detailed images of the lungs and lung arteries. […] This test uses magnetic fields and radio waves to create detailed images of the blood vessels in the lungs. […] This test shows how blood flows through the heart arteries. […] You might be asked to walk for several minutes to see how your body reacts to mild exercise.

• #47 Eisenmenger syndrome: diagnosis, prognosis and clinical management | Heart

  https://heart.bmj.com/content/106/21/1638.abstract

  Eisenmenger syndrome (ES) represents the most severe phenotype of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) and occurs in patients with large unrepaired shunts. […] Close follow-up is needed to early diagnose and timely manage these complications. […] Impaired exercise tolerance, decreased arterial oxygen saturation, iron deficiency, pre-tricuspid shunts, arrhythmias, increased brain natriuretic peptide, echocardiographic indices of right ventricular dysfunction and hospitalisation for heart failure predict mortality. […] Finally, the development of a risk score is of utmost importance to guide clinical therapy.

• #48 Eisenmenger’s syndrome differential diagnosis – wikidoc

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

  Eisenmenger’s syndrome should be differentiated from idiopathic pulmonary hypertension, pulmonary infarction, respiratory failure, tricuspid atresia, persistent truncus arteriosus, and other congenital heart diseases. […] The main diagnostic studies are CT pulmonary angiography, ventilation/perfusion scanning. However, echocardiography may diagnose underlying cardiac conditions.

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

  https://radiopaedia.org/articles/eisenmenger-syndrome-1?lang=us

  The Eisenmenger syndrome is a complication of an uncorrected high-flow, high-pressure congenital heart anomaly leading to chronic pulmonary arterial hypertension and shunt reversal. […] In general by the time true Eisenmenger syndrome develops patients are already symptomatic with easy fatigue, dyspnea, chest pain and syncope being common. […] No specific imaging findings can distinguish between a patient with severe pulmonary arterial hypertension and one with superimposed Eisenmenger syndrome. […] Treatment for Eisenmenger phenomenon has historically been palliative, and closure of the underlying shunt is contraindicated. […] The radiographic differential is essentially that of pulmonary arterial hypertension.

• #50 Eisenmenger’s syndrome differential diagnosis – wikidoc

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

  Eisenmenger’s syndrome should be differentiated from idiopathic pulmonary hypertension, pulmonary infarction, respiratory failure, tricuspid atresia, persistent truncus arteriosus, and other congenital heart diseases. […] The main diagnostic studies are CT pulmonary angiography, ventilation/perfusion scanning. However, echocardiography may diagnose underlying cardiac conditions.

• #51 Eisenmenger Syndrome Workup: Approach Considerations, Laboratory Studies, Chest Radiography and MRI

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

  Cardiac catheterization can be of value in patients with Eisenmenger syndrome, after collecting clinical and noninvasive data, to confirm and/or demonstrate the following: Severity of pulmonary arterial hypertension, Conduit patency and pressure gradient, Coexisting coronary artery anomalies (rare), Degree of shunting. […] In patients with Eisenmenger syndrome and severe pulmonary vascular disease, histologic analysis reveals abnormal extension of muscle into small peripheral arteries, severe medial smooth muscle hypertrophy of existing muscular arteries, plexiform lesions and increased intercellular material, and a reduction in the overall concentration and size of arteries.

• #52 Eisenmenger syndrome Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/eisenmenger-syndrome

  Eisenmenger syndrome is a condition that affects blood flow from the heart to the lungs in some people who were born with structural problems of the heart. […] The provider will diagnose Eisenmenger syndrome by looking at the person’s history of heart problems. Tests may include: […] 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.

• #53 Image diagnosis: Eisenmenger’s syndrome in patients with simple congenital heart disease – Congenital Heart International Professionals (CHiP) Network

  https://thechipnetwork.org/image-diagnosis-eisenmengers-syndrome-in-patients-with-simple-congenital-heart-disease/

  Early identification of congenital heart disease (CHD) allows detection of the pulmonary arteriopathy in an early stage, and timely shunt closure can permanently reverse pulmonary arterial hypertension (PAH). […] From January 2017 to November 2018, a total of 8 CHD patients (3 ventricular septal defects (VSD), 2 atrial septal defects (ASD), and 3 patent ductus arteriosus (PDA), median age, 15.5 years [range, 3-18 years]) with PAH were detected by chest X-ray, electrocardiogram, transthoracic echocardiography (TTE), computed tomographic angiography (CTA) and cardiac catheterization. […] The surgical correction was not performed in all patients who had fixed PAH and were referred to medical treatment. […] PAH in CHD can be reversed by early shunt closure, but this potential is lost beyond a certain point of no return. […] Failure to accurately and timely assess PAH will delay effective treatment past optimal treatment time, and even lead to death.

• #54 Eisenmenger syndrome: diagnosis, prognosis and clinical management | Heart

  https://heart.bmj.com/content/106/21/1638.abstract

  Eisenmenger syndrome (ES) represents the most severe phenotype of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) and occurs in patients with large unrepaired shunts. […] Close follow-up is needed to early diagnose and timely manage these complications. […] Impaired exercise tolerance, decreased arterial oxygen saturation, iron deficiency, pre-tricuspid shunts, arrhythmias, increased brain natriuretic peptide, echocardiographic indices of right ventricular dysfunction and hospitalisation for heart failure predict mortality. […] Finally, the development of a risk score is of utmost importance to guide clinical therapy.

• #55 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. […] The key principle in the clinical management of Eisenmenger syndrome patients remains to cause no harm to the fragile balanced pathophysiological state. […] Eisenmenger syndrome represents a clinical diagnosis based on physiologic factors. […] Assessing prognosis is paramount as it may help to guide specific pulmonary arterial hypertension therapy in this setting. […] 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.

• #56 Eisenmenger syndrome: diagnosis, prognosis and clinical management – PubMed

  https://pubmed.ncbi.nlm.nih.gov/32690623/

  Eisenmenger syndrome (ES) represents the most severe phenotype of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) and occurs in patients with large unrepaired shunts. […] Close follow-up is needed to early diagnose and timely manage these complications. […] Impaired exercise tolerance, decreased arterial oxygen saturation, iron deficiency, pre-tricuspid shunts, arrhythmias, increased brain natriuretic peptide, echocardiographic indices of right ventricular dysfunction and hospitalisation for heart failure predict mortality. […] Due to the lack of evidence, current guidelines do not provide a clear therapeutic strategy regarding treatment escalation. […] Finally, the development of a risk score is of utmost importance to guide clinical therapy.

• #57 Eisenmenger Syndrome: Background, Pathophysiology, Etiology

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

  Failure to reduce pulmonary pressures in the first 2 years of life may result in the irreversible progression of endothelial dysfunction and pulmonary vascular remodeling. […] The condition then advances to irreversible pulmonary hypertension. […] The usual life expectancy of a patient with Eisenmenger syndrome is 20-40 years if the syndrome is diagnosed promptly and treated with vigilance. […] A systematic review by Diller et al was able to estimate 10-year mortality rates of 30-40%. […] In a study of 153 German patients with Eisenmenger syndrome, 10-year mortality approached 60-70% in those who did not receive disease-directed therapy. […] A study by Salehian et al reported that left ventricular (LV) dysfunction (defined as LV ejection fraction [LVEF] 50%), right ventricular (RV) hypertrophy, arrhythmias, low serum albumin, and signs and symptoms of heart failure predict mortality in patients with Eisenmenger syndrome. […] A simple echocardiographic score that relies on RV and right atrial characteristics was found to predict adverse outcomes in patients with Eisenmenger syndrome that is not associated with complex congenital heart disease.

• #58 Eisenmenger syndrome: diagnosis, prognosis and clinical management | Heart

  https://heart.bmj.com/content/106/21/1638.abstract

  Eisenmenger syndrome (ES) represents the most severe phenotype of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) and occurs in patients with large unrepaired shunts. […] Close follow-up is needed to early diagnose and timely manage these complications. […] Impaired exercise tolerance, decreased arterial oxygen saturation, iron deficiency, pre-tricuspid shunts, arrhythmias, increased brain natriuretic peptide, echocardiographic indices of right ventricular dysfunction and hospitalisation for heart failure predict mortality. […] Finally, the development of a risk score is of utmost importance to guide clinical therapy.

• #59 Eisenmenger Syndrome: Background, Pathophysiology, Etiology

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

  Failure to reduce pulmonary pressures in the first 2 years of life may result in the irreversible progression of endothelial dysfunction and pulmonary vascular remodeling. […] The condition then advances to irreversible pulmonary hypertension. […] The usual life expectancy of a patient with Eisenmenger syndrome is 20-40 years if the syndrome is diagnosed promptly and treated with vigilance. […] A systematic review by Diller et al was able to estimate 10-year mortality rates of 30-40%. […] In a study of 153 German patients with Eisenmenger syndrome, 10-year mortality approached 60-70% in those who did not receive disease-directed therapy. […] A study by Salehian et al reported that left ventricular (LV) dysfunction (defined as LV ejection fraction [LVEF] 50%), right ventricular (RV) hypertrophy, arrhythmias, low serum albumin, and signs and symptoms of heart failure predict mortality in patients with Eisenmenger syndrome. […] A simple echocardiographic score that relies on RV and right atrial characteristics was found to predict adverse outcomes in patients with Eisenmenger syndrome that is not associated with complex congenital heart disease.

• #60 Patent Ductus Arteriosus With Eisenmenger Syndrome: Difficult Diagnosis Made Easily With Saline Contrast Echocardiography | Kumar | Cardiology Research

  https://cardiologyres.org/index.php/Cardiologyres/article/view/447

  The diagnosis of patent ductus arteriosus (PDA) with Eisenmenger syndrome is difficult. […] On agitated saline contrast injection, agitated saline was seen in pulmonary artery followed by filling of abdominal aorta without filling of ascending aorta, thus confirming the diagnosis of right to left shunt with PDA.

• #61 Eisenmenger Syndrome Workup: Approach Considerations, Laboratory Studies, Chest Radiography and MRI

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

  Laboratory studies used in the diagnosis of Eisenmenger syndrome include complete blood cell count, biochemical profiles, and iron studies, in addition to blood gas assessments. Electrocardiography can also reveal signs of an underlying cardiac defect and of right ventricular hypertrophy. Imaging studies can reveal cardiac structural defects and pulmonary changes, including irreversible alterations in the pulmonary system. Histologic findings can be used to determine the stage of pulmonary vascular pathology. The updated 2018 AHA/ACC guidelines for adults with congenital heart disease (ACHD) emphasize the need for advanced cardiac imaging and cardiac catheterization data accuracy to exclude other etiologies of the right-to-left shunt physiology (Class IC recommendation). […] Preoperatively, the combination of 100% oxygen or nitric oxide are used to evaluate pulmonary vasculature reactivity in pulmonary hypertension. If the pulmonary vascular resistance (PVR) does not decrease with this test, the PVR is considered irreversible, and the patient may not be a good surgical candidate for corrective cardiac surgery.

• #62 Eisenmenger Syndrome – ACHA

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

  The diagnosis of ES is based on the presence of three symptoms: […] In addition to blood tests, EKG and chest x-ray, other tests are needed to confirm the diagnosis. These include an Echocardiogram, cardiac catheterization and a 6 minute walk test. […] The prognosis for patients with Eisenmenger Syndrome is improving with better diagnosis and care.

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

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

  Eisenmenger syndrome is an end-stage complication of congenital heart disease that occurs when a left to right shunt causes pulmonary over-circulation, leading to vascular remodeling, increased vascular resistance, and ultimately even shunt reversal. […] Evaluation for Eisenmenger syndrome should include a comprehensive history, physical exam, ECG, echocardiogram, cardiac catheterization, and laboratory work to identify multi-system complications of cyanosis and secondary erythrocytosis. […] The most definitive means to diagnose Eisenmenger syndrome in a patient with a prior left-to-right shunt lesion is with a right heart cardiac catheterization showing right to left shunting (Qp:Qs 1). […] Remember: You must have had a right heart cardiac catheterization to diagnose someone with Eisenmenger syndrome.

• #64 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 you have any symptoms of Eisenmenger syndrome, see your healthcare professional. Make an appointment even if you have never been diagnosed with a heart condition. […] Eisenmenger syndrome: Diagnosis, prognosis and clinical management.

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