Materiały źródłowe

• #1 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. The disease can cause complications including permanent heart and lung damage. Nonsurgical treatments focus on easing symptoms, but there is no cure. […] Treatment for Eisenmenger syndrome is aimed at easing symptoms and preventing severe damage to the heart or lungs. Therapies may include: Antibiotics: Medication to prevent bacterial infections in the heart and lungs. Anticoagulants: Medication to prevent blood clots. Diuretics: Medication to reduce fluid buildup in the body. Dual endothelin receptor antagonists: Medication to lower high blood pressure in the lungs. Iron supplements: Additional iron to treat iron-deficiency anemia. Supplemental oxygen: Oxygen therapy to help you breathe better. […] You can reduce the risk of complications from Eisenmenger syndrome by: Eating a sodium-controlled diet. Getting vaccines to avoid infections. Staying hydrated. Taking all medications and other therapies prescribed by your cardiologist. Taking antibiotics before having dental work or surgery.

• #2 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.

  Eisenmenger syndrome frequently develops in childhood; in adults, the diagnosis is made mainly in patients with an atrioventricular septal defect, large interatrial shunt, nonrestrictive ventricular septal defects, or patent ductus arteriosus (PDA). […] Patients should be followed in specialized centers with expertise in care of adults with congenital heart disease and pulmonary hypertension. […] Pregnancy is a contraindication in women with Eisenmenger syndrome. […] 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.

• #3

  https://link.springer.com/article/10.1007/s00246-015-1184-7

  The Eisenmenger syndrome (ES) is a severe form of pulmonary arterial hypertension and arises in congenital heart disease with a systemic-to-pulmonary shunt. Patients with ES have multisystem involvement as a result of chronic hypoxemia with hematologic, skeletal, renal, and neurologic systems, causing significant morbidity and mortality. […] To date, there is no guideline for the perioperative care of ES patients in children and limited data available for adult patients. This review provides an overview of appropriate measures for the safe perioperative care of patients, based on an understanding of the pathophysiological changes that occur in ES.

• #4 What is Eisenmenger Syndrome in Children – Jupiter Hospital

  https://www.jupiterhospital.com/indore/blog/what-is-eisenmenger-syndrome-in-children/

  What is Eisenmenger Syndrome in Children […] Eisenmenger syndrome is an advanced form of heart problem that develops over time in the arteries. It occurs as a result of other congenital (before birth) defects in the heart (usually left heart-to-right heart shunts), where the arteries that carry blood from the heart to the lungs narrow. This results in high blood pressure of blood flow against the walls of the arteries, causing lung damage. It would help if you visited a pediatric cardiology hospital in Indore to treat your child as early as possible. Because, if the heart defects are not repaired or repaired later in life, it increases the risk for this medical condition. […] […] The best hospital in Indore Treatment will decide the treatment based on your child’s symptoms, age, and general health. The procedure also depends on how severe the condition is. By lowering the pressure in the pulmonary artery, the treatment aims to bring more oxygen to the lung tissues and ease the cyanosis. […] […] Most Medical treatment helps in managing symptoms. Treatments include: […] […] Oxygen will be given to your child continuously while he/she is sleeping or resting. […] […] Lung transplant or heart-lung transplant: These may be considered for your child in some severe cases.

• #5 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. […] Care in tertiary specialist cardiac centers with access to multidisciplinary subspecialities is required. […] 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 patients require life-long expert assessment and therapy at specialized centers. […] Preventing complications and avoiding management errors should be the primary goal of regular outpatient assessment.

• #5 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/

  It is recommended to meticulously encourage patients to obtain yearly influenza immunization as well as immunization against pneumococcal disease every 5 years. […] General anesthesia carries a high risk. […] 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.

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

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

  Our caring team of Mayo Clinic experts can help you with your Eisenmenger syndrome-related health concerns […] If you need treatment for Eisenmenger syndrome, get care at a medical center with healthcare professionals who have experience in congenital heart diseases. […] Regular health checkups at least once a year are an important part of Eisenmenger syndrome treatment. […] Medicines are the main treatment for Eisenmenger syndrome. Medicines can’t cure Eisenmenger syndrome, but they can help improve quality of life. […] 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. […] If you have Eisenmenger syndrome, these tips may help improve your quality of life. […] Some people find that talking with others who have been through similar situations brings comfort and encouragement. Ask your healthcare team if there are any local support groups.

• #7 Eisenmenger Syndrome | Norton Children’s

  https://nortonchildrens.com/services/cardiology/conditions/heart-disease/eisenmenger-syndrome/

  Eisenmenger syndrome is a complication from a congenital heart disease lesion. It happens with a congenital heart defect that includes a hole between two chambers, causing increased blood flow to the lungs (left to right shunt). Eisenmenger syndrome occurs when the increased flow to the lungs causes pulmonary hypertension (high pressure in the blood vessels of the lungs), and flow between the two chambers eventually reverses and goes backward because of worsening permanent pulmonary hypertension. […] The specialists at Norton Children’s Heart Institute — the leading providers of pediatric heart care in Louisville and Southern Indiana — can help your child with Eisenmenger syndrome. […] Eisenmenger syndrome treatment focuses on: […] Treatments may include: […] Medications: These can be given to lower high pressure in the lungs. […] Oxygen: It can help with breathing during sleep and rest. […] Heart or lung transplant: When other treatments are no longer effective, a transplant may be considered and would depend on a large number of factors that the Norton Children’s Heart Institute specialists would guide you through.

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

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

  Initiation of vasodilator therapy may require inpatient hospitalization and observation. Patients do not require hospitalization for therapeutic erythropheresis, but they may need attention for infectious disease complications and/or other hematologic concerns. […] Patients should follow up at a specialized cardiology clinic within 2 weeks of discharge from inpatient care and every 3 months when stable. It is recommended that patients are referred to and followed by specialized centers. […] Smoking is absolutely contraindicated in patients with Eisenmenger syndrome because of its deleterious effects on the heart, blood vessels, and lungs. […] Alcohol may exacerbate myocardial dysfunction, hypovolemia, and worsening hyperviscosity, and it can result in systemic hypotension with an exacerbation of the right-to-left shunt.

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

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

  The use of oxygen supplementation in patients with Eisenmenger syndrome is controversial. It has previously been shown to have no impact on exercise capacity and survival in adult patients with this condition. […] Vasodilator therapy improves symptoms in patients with Eisenmenger syndrome and should be used routinely in the management of this patient population. However, a single medical therapy has not been consistently shown to reduce mortality in patients with Eisenmenger syndrome. […] Patients with Eisenmenger syndrome are at very high risk for endocarditis. Therefore, emphasize endocarditis prophylaxis, and give patients repeat instructions about this issue. […] Erythrocytosis is almost always present in patients with Eisenmenger syndrome. This can result in symptoms of hyperviscosity, including visual disturbances, fatigue, headache, dizziness, and paresthesia.

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

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

  Right heart failure is often present in patients with Eisenmenger syndrome, but limited treatment options are available for this complication. Typically, digoxin and diuretics have been used. Diuretics, specifically loop diuretics, are used for symptomatic relief of congestion. However, diuretics should be used cautiously in these patients, given their preload-dependent state. […] Central nervous system (CNS) events can occur secondary to paradoxical embolus, CNS venous thrombosis, intracranial hemorrhage, or brain abscesses in the setting of endocarditis. Endocarditis prophylaxis, the use of air filters with all intravenous catheters during hospital admissions, and adequate management of hyperviscosity can help to decrease these potentially fatal complications. […] Surgical palliation or repair should be performed early in patients with congenital heart disease to prevent progression to Eisenmenger syndrome. No surgical care is available to correct the congenital cardiac defect that caused the pathologic pulmonary vascular changes once Eisenmenger syndrome has developed to the irreversible stage.

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

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

  Patients with Eisenmenger syndrome are prone to thrombotic events as part of their hyperviscosity syndrome. At the same time, they are susceptible to bleeding because their platelets are dysfunctional. […] Among pregnant women with Eisenmenger syndrome, the fetal mortality rate is approximately 25% and the maternal mortality rate is about 50%. Pregnancy should therefore be avoided by women with Eisenmenger syndrome. […] Heart-lung transplantation is the procedure of choice if repair of the underlying cardiac defect is not possible in Eisenmenger syndrome. […] Repair of the primary defect in patients with Eisenmenger syndrome is contraindicated in the context of established severe pulmonary arterial hypertension (PAH). However, corrective surgery may be possible in certain individuals if there remains a significant degree of left-to-right shunting and if responsiveness of the pulmonary circulation to vasodilator therapy can be demonstrated.

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

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

  Patients with right-sided congestive heart failure should follow a no-salt-added or salt-restricted diet. However, sodium restriction must be balanced against the need to maintain intravascular volume. […] Prevention of Eisenmenger syndrome is critical. When recognized in a timely fashion, congenital cardiac defects can be effectively treated with minimal morbidity and mortality.

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

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

  The treatment of Eisenmenger syndrome varies widely and depends on the patient’s age, degree of cyanosis, and subsequent polycythemia. Asymptomatic patients require periodic evaluation, with anticipation of potential needs. All patients with intracardiac right-to-left shunts have potential for the following: […] The therapeutic approach of Eisenmenger syndrome encompasses medical therapy with pulmonary vasodilators, close specialist follow-up, hematologic complication management, avoidance of high-risk situations and, ultimately, lung and heart transplantation (or lung transplantation with cardiac defect repair). High-risk situations for these patients include pregnancy, volume depletion, isometric exercise, endocardial pacing, and high altitudes. […] Patients should avoid sudden fluid shifts or dehydration, which may increase right-to-left shunting. They should also avoid very hot or humid conditions, which may exacerbate vasodilation, causing syncope and increased right-to-left shunting.

• #9 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. […] Care in tertiary specialist cardiac centers with access to multidisciplinary subspecialities is required. […] 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 patients require life-long expert assessment and therapy at specialized centers. […] Preventing complications and avoiding management errors should be the primary goal of regular outpatient assessment.

• #9 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

  It is recommended to meticulously encourage patients to obtain yearly influenza immunization as well as immunization against pneumococcal disease every 5 years. […] 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.

• #10 Eisenmenger syndrome | PPT

  https://www.slideshare.net/slideshow/eisenmenger-syndrome-29637358/29637358

  Eisenmenger syndrome is a condition where a congenital heart defect causes pulmonary hypertension and a reversal of blood flow, leading to hypoxemia. […] Treatment focuses on managing symptoms and complications through lifestyle modifications and medications, as surgery to repair the underlying defect poses high risks. […] It is essential to appreciate that their hemodynamic state is very delicately balanced. This balance is easily upset, often with disastrous results. […] Recommendations for Medical Therapy of Eisenmenger Physiology Class I 1. It is recommended that patients with Eisenmenger syndrome avoid the following activities or exposures, which carry increased risks: a. Pregnancy. (Level of Evidence: B) […] 6. These pts should undergo noncardiac surgery and cardiac catheterization only in centers with expertise in the care of such patients (Level of Evidence: C).

• #10 Eisenmenger syndrome | PPT

  https://www.slideshare.net/slideshow/eisenmenger-syndrome-29637358/29637358

  Non-cardiac surgery in Eisenmenger patients carries a high morbidity and mortality risk (up to 19%). Surgery should be avoided when possible, but is commonly needed for acute cholecystitis (due to bilirubin stone formation from the hyperbilirubinemia). Necessary operations should be done in a center familiar with the high risks of performing surgery on these patients. […] For quality of life, complications must be managed.

• #11

  https://www.amerikanhastanesi.org/mayo-clinic-care-network/mayo-clinic-health-information-library/diseases-conditions/eisenmenger-syndrome

  Eisenmenger syndrome is a long-term complication of an unrepaired heart problem present at birth (congenital heart defect). Eisenmenger syndrome is life-threatening. […] Early diagnosis and repair of congenital heart defects usually prevents Eisenmenger syndrome. If it does develop, treatment involves regular medical visits and medications to improve symptoms. […] If you are diagnosed with Eisenmenger syndrome, you’ll be referred to a heart specialist (cardiologist). Regular health checkups at least once a year are an important part of Eisenmenger syndrome treatment. It’s helpful to find a cardiologist who has experience treating people who have congenital heart defects. […] Medications are the main treatment for Eisenmenger syndrome. They can’t cure the condition, but they can help improve your quality of life. You need regular medical checkups when taking medications. Your provider checks for any changes in blood pressure, pulse rate and fluid levels.

• #11

  https://www.amerikanhastanesi.org/mayo-clinic-care-network/mayo-clinic-health-information-library/diseases-conditions/eisenmenger-syndrome

  Health care providers don’t recommend surgery to repair the hole (shunt) in the heart once Eisenmenger syndrome has developed. […] If you’re diagnosed with Eisenmenger syndrome, proper treatment and precautions can help improve quality of life. Try these strategies: Avoid dehydration. Ask your provider how much fluid you need each day. […] If you have Eisenmenger syndrome, becoming pregnant poses serious health risks and can be life-threatening for you and the baby. It’s critical that pregnancy be avoided if you have Eisenmenger syndrome. […] A diagnosis of Eisenmenger syndrome can be scary. Although treatments can help symptoms and improve prognosis, you may feel worried or anxious. […] Eisenmenger syndrome is a complicated condition. There’s often a lot to discuss at a medical appointment. So it’s a good idea to be prepared.

• #12 A Case of Patient with Eisenmenger Syndrome Admitted to Intensive Care Unit Due to Septic Shock Complicated with Newlydiagnosed Down Syndrome and Moyamoya Disease

  https://www.accjournal.org/journal/view.php?doi=10.4266/kjccm.2013.28.3.204

  Eisenmenger syndrome is a severe form of pulmonary arterial hypertension related to congenital cardiac defects. […] The treatment of primary pulmonary hypertension is being applied to Eisenmenger syndrome such as endothelin receptor antagonists, phosphodiesterase-5 blockers, and prostacyclin. […] After the combination treatment with iloprost and sildenafil within the intensive care unit, the patient was able to wean mechanical ventilation without further applications of invasive rescue therapy such as extracorporeal membrane oxygenator. […] She maintained bosentan therapy for 34 months continuously without aggravations of symptom but eventually died with intracranial hemorrhage, a complication of Moyamoya disease.

• #13 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. 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). […] 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. […] Water tablets (diuretics) make you pass urine more frequently and therefore decrease the amount of excess fluid in your body. This takes pressure off the heart allowing it to pump more easily. This should make you less breathless and should also decrease the swelling in your ankles and abdomen.

• #14 Treatment of Eisenmenger Syndrome With UPTRAVI

  https://www.jnjmedicalconnect.com/products/uptravi/medical-content/treatment-of-eisenmenger-syndrome-with-uptravi

  UPTRAVI is not approved by the regulatory agencies for the treatment of patients with Eisenmenger syndrome (ES). Johnson Johnson does not recommend the use of UPTRAVI in a manner that is inconsistent with the approved labeling. […] Studies specifically designed to investigate the safety and/or efficacy of UPTRAVI for the treatment of patients with ES have not been performed. […] A search of the scientific literature retrieved limited information from few case series and case reports describing the administration of UPTRAVI to patients with ES; relevant information from these publications is summarized in this letter. […] In addition, Van Dissel et al (2020), in a congress abstract, have described the data of a multicenter, prospective study involving 34 adult patients with pulmonary arterial hypertension associated with congenital heart disease who were treated with UPTRAVI. A total of 60% of these patients had ES; however, the study results refer to the entire cohort, and no safety or efficacy outcomes specific to the subgroup of patients with ES are included.

• #15 Eisenmenger syndrome

  https://www.mymlc.com/health-information/diseases-and-conditions/e/eisenmenger-syndrome/?section=Causes

  If your red blood cell count becomes too high and is causing symptoms such as headache, difficulty concentrating or vision problems, your doctor may recommend having blood drawn to help decrease your blood cell counts. The blood draw procedure is called phlebotomy. It should not be done routinely and should only be done after consultation with a congenital heart disease expert. […] Some people who have Eisenmenger syndrome may eventually need a heart and lung transplant or a lung transplant with repair of the hole in the heart if other treatments don’t control your symptoms. […] If you’re diagnosed with Eisenmenger syndrome, you can still lead an active life with proper treatment and precautions. […] Avoid dehydration. Ask your doctor how much fluid you need each day. You may need more fluids if you’re sick, in a heated room or traveling on an airplane.

• #15 Eisenmenger syndrome

  https://www.mymlc.com/health-information/diseases-and-conditions/e/eisenmenger-syndrome/?section=Causes

  Check with your doctor about exercise restrictions. While you shouldn’t perform strenuous exercise or sports, you may be able to do less intense physical activities. Talk to your doctor about what type of physical activity is appropriate for you. […] Avoid situations that can excessively lower blood pressure. These include sitting in a hot tub or sauna or taking long hot baths or showers. These activities lower your blood pressure and cause fainting or even death. […] Be cautious with any medications and supplements. Many prescription and over-the-counter medications or supplements may increase or decrease blood pressure, increase risk of bleeding or blood clots, or affect kidney function in patients who have Eisenmenger syndrome. […] If you have Eisenmenger syndrome, becoming pregnant poses serious health risks — and can be life threatening — for the mother and baby. It’s critical that women who have Eisenmenger syndrome avoid becoming pregnant.

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

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

  Surgery should be performed with an experienced cardiac anesthesiologist. Operative risk in Eisenmenger syndrome is lower than in pulmonary arterial hypertension as patients with Eisenmenger syndrome are able to increase their cardiac output by increasing right to left shunting at the expense of their oxygenation. […] 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. […] Among the few cardiac conditions where pregnancy is contraindicated are pulmonary arterial hypertension and Eisenmenger syndrome. […] Transplantation in Eisenmenger syndrome requires both heart and lung transplant. The number of heart-lung transplant for congenital heart disease is a small proportion and even less so for Eisenmenger syndrome.

• #17 Postoperative Management of Eisenmenger’s Syndrome

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

  Therefore, measures should be in place to detect and manage alteration in hemodynamics during induction of anesthesia and surgery. […] Therefore, careful monitoring of fluid balance and replacement is necessary for maintaining normo-tension is critical for effective management of this patient population. […] It is advisable to refer patients with ES to specialized centers capable of applying strategies aimed at the perioperative hemodynamic changes during surgical interventions. […] 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.

• #18 Monitored anesthesia care for craniotomy in a patient with Eisenmenger syndrome: A case report

  https://www.wjgnet.com/2307-8960/full/v10/i27/9859.htm

  Eisenmenger syndrome (ES) is an uncorrected congenital cardiac defect with a left-to-right shunt, leading to pulmonary arterial hypertension. […] Patients with ES are susceptible to hemodynamic alterations during noncardiac surgery with general anesthesia, which increases perioperative morbidity and mortality. […] Monitored anesthesia care (MAC) is often used during minor procedures in patients with cardiac disease. […] However, few reports on MAC in patients with ES exist. […] The main anesthetic goals in patients with ES are as follows: (1) Maintain euvolemia; (2) optimize systematic vascular resistance; and (3) avoid the elements that increase pulmonary vascular resistance (pain, hypoxia, and hypercapnia). […] MAC with sedation has been implemented in anesthesia management of patients with ES, even for minor surgical procedures.

• #18 Monitored anesthesia care for craniotomy in a patient with Eisenmenger syndrome: A case report

  https://www.wjgnet.com/2307-8960/full/v10/i27/9859.htm

  The major concerns in MAC are (1) hypoxia and hypercarbia due to oversedation and lack of adequate airway support, and (2) potential agitation and uncontrollable behaviors due to the paradoxical reaction of sedative agents. […] Dexmedetomidine, an alpha-2 adrenoreceptor agonist, has sedative and analgesic effects. […] It can induce sedation without respiratory depression and could, therefore, be a good choice to sedate patients with Eisenmenger features, while avoiding hypoxemia or hypercapnia. […] MAC with dexmedetomidine and remifentanil may be effective for craniotomy in patients with ES. […] Supplementing oxygen via HFNC is advisable to avoid hypoxia.

• #19 Eisenmenger’s Syndrome | Conditions | UCSF Health

  https://www.ucsfhealth.org/conditions/eisenmengers-syndrome

  Eisenmenger’s syndrome refers to this combination of reversed blood flow with pulmonary hypertension. […] UCSF provides comprehensive, highly specialized care for adults living with heart defects such as Eisenmenger’s syndrome. […] Adult patients with Eisenmenger’s syndrome should be seen by a cardiologist specializing in the care of adults with congenital heart disease to monitor for late complications. […] All patients will need to take antibiotics during subsequent surgeries or dental procedures to protect against endocarditis. […] It is important to eat a nutritious diet and avoid alcohol and salt. […] Overexertion and smoking also should be avoided. […] The only other surgical option is heart/lung transplant or lung transplant along with a repair of the defect.

• #20 Pregnancy in Eisenmenger syndrome: a case series from a tertiary care hospital of Northern India | Cardiology in the Young | Cambridge Core

  https://www.cambridge.org/core/journals/cardiology-in-the-young/article/pregnancy-in-eisenmenger-syndrome-a-case-series-from-a-tertiary-care-hospital-of-northern-india/BA8A934F7E2DC7537DB8C3DA2E4249D7

  Despite advances in medical care, we still come across pregnancy in Eisenmenger syndrome. […] Due to very high maternal and perinatal morbidity and mortality, pregnancy is contraindicated among these women. […] Current guidelines also recommend that the women who become pregnant should opt for early termination of pregnancy. […] Women with Eisenmenger syndrome require effective contraception, preconceptional counselling, early termination of pregnancy, and multidisciplinary care.

• #21 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. […] Considerations related to Eisenmenger syndrome include, but are not limited to, the following: 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. […] 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.

• #22 Eisenmenger syndrome // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/eisenmenger-syndrome

  Eisenmenger syndrome is a life-threatening condition. […] 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. […] Medicines are the main treatment for Eisenmenger syndrome. Medicines can’t cure Eisenmenger syndrome, but they can help improve quality of life. […] If you need treatment for Eisenmenger syndrome, get care at a medical center with healthcare professionals who have experience in congenital heart diseases. […] If you have Eisenmenger syndrome, these tips may help improve your quality of life. […] 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. […] If you have Eisenmenger syndrome, you may find that joining a support group is helpful. Some people find that talking with others who have been through similar situations brings comfort and encouragement.

• #23 Eisenmenger Syndrome – ACHA

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

  The prognosis for patients with Eisenmenger Syndrome is improving with better diagnosis and care. […] If you have Eisenmenger Syndrome, you should: Be seen on a regular basis by an ACHD specialist. […] Have non-cardiac and cardiac surgery at centers with expertise in Eisenmenger Syndrome. […] It is essential that you are treated by an ACHD doctor and a team with expertise in managing your condition and associated symptoms. Care from doctors who understand the challenges you face and can provide the high level treatment you need is crucial. […] Education and the right care will help assure you have the longest and best quality of life.

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

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

  With advances in the recognition of risks confronted by adults with Eisenmenger syndrome, and evolving modalities to minimize the risks of bleeding, and minimize the evolution of heart failure, many of these adults are living well into their 40’s, 50’s, and even into their 60’s. […] 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. […] The Ahmanson/UCLA Adult Congenital Heart Disease Center is beginning a research study to evaluate the impact of inhaled ventavis on exercise capacity in our Eisenmenger syndrome patients.

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