Materiały źródłowe

• #1 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. […] Although to date there is no causal therapy to reverse pulmonary vascular disease, a greater armamentarium of targeted therapies is available, which have been shown to be beneficial in patients with ES. […] The key principle in the clinical management of Eisenmenger syndrome patients remains to cause no harm to the fragile balanced pathophysiological state.

• #2 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. […] Although to date there is no causal therapy to reverse pulmonary vascular disease, a greater armamentarium of targeted therapies is available, which have been shown to be beneficial in patients with ES. […] The key principle in the clinical management of Eisenmenger syndrome patients remains to cause no harm to the fragile balanced pathophysiological state.

• #3 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. […] Although to date there is no causal therapy to reverse pulmonary vascular disease, a greater armamentarium of targeted therapies is available, which have been shown to be beneficial in patients with ES. […] The key principle in the clinical management of Eisenmenger syndrome patients remains to cause no harm to the fragile balanced pathophysiological state.

• #4 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. […] Although to date there is no causal therapy to reverse pulmonary vascular disease, a greater armamentarium of targeted therapies is available, which have been shown to be beneficial in patients with ES. […] The key principle in the clinical management of Eisenmenger syndrome patients remains to cause no harm to the fragile balanced pathophysiological state.

• #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 patients require life-long expert assessment and therapy at specialized centers. […] Since the advent of targeted therapies for pulmonary arterial hypertension approximately 20 years ago and subsequent positive results in Eisenmenger patients, these patients can be offered a therapeutic option that improves symptoms, exercise capacity and potentially outcome. […] Therapy is based on various pathophysiologic pathways. […] The majority (as well as the most robust) data exist on the effect of endothelin receptor antagonists in patients with Eisenmenger syndrome. […] This has sparked interest in various treatment approaches using also phosphodiesterase-5 inhibitors (mainly Sildenafil) in this setting. […] At most centers, endothelin receptor antagonist therapy remains the first choice for symptomatic Eisenmenger patients (especially those in NYHA class III and those deteriorating over time).

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

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

  The goals of Eisenmenger syndrome treatment are to: […] 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. […] Healthcare professionals don’t recommend surgery to repair the hole in the heart once Eisenmenger syndrome has developed. […] If you need treatment for Eisenmenger syndrome, get care at a medical center with healthcare professionals who have experience in congenital heart diseases.

• #7 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. […] Treatment methods may include, but are not limited to, the following: […] Phlebotomy […] Medications […] Oxygen […] Other treatments.

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

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

  The goals of Eisenmenger syndrome treatment are to: […] 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. […] Healthcare professionals don’t recommend surgery to repair the hole in the heart once Eisenmenger syndrome has developed. […] If you need treatment for Eisenmenger syndrome, get care at a medical center with healthcare professionals who have experience in congenital heart diseases.

• #9 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. […] Treatment methods may include, but are not limited to, the following: […] Phlebotomy […] Medications […] Oxygen […] Other treatments.

• #10 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. […] Although to date there is no causal therapy to reverse pulmonary vascular disease, a greater armamentarium of targeted therapies is available, which have been shown to be beneficial in patients with ES. […] The key principle in the clinical management of Eisenmenger syndrome patients remains to cause no harm to the fragile balanced pathophysiological state.

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

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

  The goals of Eisenmenger syndrome treatment are to: […] 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. […] Healthcare professionals don’t recommend surgery to repair the hole in the heart once Eisenmenger syndrome has developed. […] If you need treatment for Eisenmenger syndrome, get care at a medical center with healthcare professionals who have experience in congenital heart diseases.

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

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

  The goals of Eisenmenger syndrome treatment are to: […] 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. […] Healthcare professionals don’t recommend surgery to repair the hole in the heart once Eisenmenger syndrome has developed. […] If you need treatment for Eisenmenger syndrome, get care at a medical center with healthcare professionals who have experience in congenital heart diseases.

• #13 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/

  Therapy initiation or escalation is also useful in patients with low oxygen saturation and symptoms interfering with daily activities. […] 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.

• #14 2020 ESC Guidelines for the Management of ACHD: Focus on PH

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

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

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

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

  The goals of Eisenmenger syndrome treatment are to: […] 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. […] Healthcare professionals don’t recommend surgery to repair the hole in the heart once Eisenmenger syndrome has developed. […] If you need treatment for Eisenmenger syndrome, get care at a medical center with healthcare professionals who have experience in congenital heart diseases.

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

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

  Eisenmenger patients require life-long expert assessment and therapy at specialized centers. […] Since the advent of targeted therapies for pulmonary arterial hypertension approximately 20 years ago and subsequent positive results in Eisenmenger patients, these patients can be offered a therapeutic option that improves symptoms, exercise capacity and potentially outcome. […] Therapy is based on various pathophysiologic pathways. […] The majority (as well as the most robust) data exist on the effect of endothelin receptor antagonists in patients with Eisenmenger syndrome. […] This has sparked interest in various treatment approaches using also phosphodiesterase-5 inhibitors (mainly Sildenafil) in this setting. […] At most centers, endothelin receptor antagonist therapy remains the first choice for symptomatic Eisenmenger patients (especially those in NYHA class III and those deteriorating over time).

• #17 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 patients require life-long expert assessment and therapy at specialized centers. […] Since the advent of targeted therapies for pulmonary arterial hypertension approximately 20 years ago and subsequent positive results in Eisenmenger patients, these patients can be offered a therapeutic option that improves symptoms, exercise capacity and potentially outcome. […] Therapy is based on various pathophysiologic pathways. […] The majority (as well as the most robust) data exist on the effect of endothelin receptor antagonists in patients with Eisenmenger syndrome. […] This has sparked interest in various treatment approaches using also phosphodiesterase-5 inhibitors (mainly Sildenafil) in this setting. […] At most centers, endothelin receptor antagonist therapy remains the first choice for symptomatic Eisenmenger patients (especially those in NYHA class III and those deteriorating over time).

• #18 Eisenmenger syndrome: current perspectives | RRCC

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

  However, evidence in this is limited and ambiguous. […] The only definitive treatment for ES is lung transplantation (LT) with shunt closure or heart and lung transplantation (HLT). […] The mainstay of PAH treatment is, nowadays, specific disease-targeting pulmonary therapies, which have only been in use since the 1990s. […] So far, only three pathomechanistic pathways in PAH have been translated into clinical practice: prostacyclin, nitric oxide, and endothelin pathways. […] Despite the associated functional limitations and multitude of comorbidities, survival prospects in adults with ES are generally considered better than in other forms of PAH, but mortality remains high. […] Bosentan is currently endorsed as the first-line choice of treatment in patients with ES in the WHO functional class IIIIV.

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

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

  Medications to treat pulmonary arterial hypertension (eg, prostacyclin analogs, endothelin antagonists, phosphodiesterase-5 inhibitors) […] There is no specific treatment once the syndrome develops, other than heart and lung transplantation, but drugs that may lower pulmonary vascular resistance (eg, prostacyclin analogs, endothelin antagonists, phosphodiesterase-5 inhibitors) are useful. […] Supportive treatment includes avoidance of conditions that may exacerbate the syndrome (eg, pregnancy, volume depletion, isometric exercise, high altitudes, smoking). Supplemental oxygen may provide some benefit. […] Heart and lung transplantation or lung transplantation with cardiac repair may be an option, but transplantation is reserved for patients with severe symptoms and unacceptable quality of life. Long-term prognosis after transplantation is guarded. […] All patients should be given endocarditis prophylaxis before dental or surgical procedures that are likely to cause bacteremia.

• #20 Eisenmenger syndrome: current perspectives | RRCC

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

  However, evidence in this is limited and ambiguous. […] The only definitive treatment for ES is lung transplantation (LT) with shunt closure or heart and lung transplantation (HLT). […] The mainstay of PAH treatment is, nowadays, specific disease-targeting pulmonary therapies, which have only been in use since the 1990s. […] So far, only three pathomechanistic pathways in PAH have been translated into clinical practice: prostacyclin, nitric oxide, and endothelin pathways. […] Despite the associated functional limitations and multitude of comorbidities, survival prospects in adults with ES are generally considered better than in other forms of PAH, but mortality remains high. […] Bosentan is currently endorsed as the first-line choice of treatment in patients with ES in the WHO functional class IIIIV.

• #21 Eisenmenger Syndrome – StatPearls – NCBI Bookshelf

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

  Cardiopulmonary transplantation is curative for Eisenmenger syndrome. However, this is impractical in most settings. Pharmacology has improved symptoms but not mortality. Potential pharmaceuticals for the treatment of Eisenmenger syndrome include diuretics, antiarrhythmics, and anticoagulation in some patients. Supplemental oxygen has not definitively shown to have a mortality benefit. Vasodilator therapies may provide an opportunity for clinical research, and studies have shown some symptomatic improvement. The Bosentan Randomized Trial of Endothelin Antagonist Therapy-5 trial showed improved exercise capacity and symptomatic improvement with endothelin antagonists in patients with ASD, VSD, and PDA with Eisenmenger syndrome. […] Clinical follow-up should focus on checking annual CBC, iron studies, kidney function, and uric acid, with a focus on correcting any abnormalities. Patients also should be assessed with pulse oximetry, both with and without supplemental O2. Any abnormalities suggesting hypoxemia warrant further evaluation. Surgical correction of the causative heart defect in adult patients is generally contraindicated.

• #22 Bosentan: a new therapy for Eisenmenger syndrome? | Nature Reviews Cardiology

  https://www.nature.com/articles/ncpcardio0649

  Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Bosentan is a dual endothelin-1-receptor antagonist that effectively treats idiopathic PAH and PAH related to connective tissue disease. Following preliminary trials of bosentan for the treatment of Eisenmenger syndrome, the Bosentan Randomized Trial of Endothelin Antagonist Therapy-5 (BREATHE-5), was designed to confirm the drug’s safety and efficacy.

• #23 Eisenmenger syndrome: current perspectives | RRCC

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

  However, evidence in this is limited and ambiguous. […] The only definitive treatment for ES is lung transplantation (LT) with shunt closure or heart and lung transplantation (HLT). […] The mainstay of PAH treatment is, nowadays, specific disease-targeting pulmonary therapies, which have only been in use since the 1990s. […] So far, only three pathomechanistic pathways in PAH have been translated into clinical practice: prostacyclin, nitric oxide, and endothelin pathways. […] Despite the associated functional limitations and multitude of comorbidities, survival prospects in adults with ES are generally considered better than in other forms of PAH, but mortality remains high. […] Bosentan is currently endorsed as the first-line choice of treatment in patients with ES in the WHO functional class IIIIV.

• #24 Eisenmenger syndrome: current perspectives | RRCC

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

  Ambrisentan is a selective mono-antagonist of the ETA receptor with a longer half-life allowing for once-a-day dosing. […] Macitentan, similar to bosentan, is a dual ET receptor antagonist and has been evaluated in a large event-driven RCT on patients with PAH (not ES). […] Two small RCTs have been published in ES evaluating the effect of PDE-5i in a total of 48 patients. […] Riociguat is a relatively new drug in PAH. […] Selexipag is one of the latest pulmonary therapies to be tested in PAH. […] There is limited information on the efficacy of combination therapy in ES, even though most experts feel that combining therapies and targeting different pathomechanistic pathways improve results. […] In a prospective open-label study, DAlto et al also studied the combined effect of bosentan and sildenafil in ES patients and reported an improvement in functional class. […] However, patients on combination therapy likely represented a sicker population with more advanced symptoms, hence making direct comparison unreliable.

• #25 Eisenmenger Syndrome Medication: Diuretics, Cardiac Glycosides, Prostaglandins, Iron Products, Rheumatologics, Other, Endothelin Antagonists, Phosphodiesterase-5 Enzyme Inhibitors

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

  Bosentan is an endothelin receptor antagonist indicated for the treatment of pulmonary arterial hypertension (PAH) in patients with WHO class III or IV symptoms. It is used to improve exercise ability and decrease the rate of clinical worsening. […] Ambrisentan is an endothelin receptor antagonist indicated for PAH in patients with WHO class II or III symptoms. It improves exercise ability and decreases the progression of clinical symptoms. […] The antiproliferative effects of the phosphodiesterase type-5 (PDE5) pathway, which regulates cyclic guanosine monophosphate hydrolysis, may be significant in the chronic treatment of pulmonary hypertension with PDE5 inhibitors such as sildenafil. […] Sildenafil promotes selective smooth muscle relaxation in the lung vasculature, possibly by inhibiting PDE5. The inhibition of PDE5 increases cyclic guanosine monophosphate (cGMP) activity, which increases the vasodilatory effects of nitric oxide. […] Tadalafil is a PDE5 selective inhibitor. Inhibition of PDE5 increases cGMP activity, which increases the vasodilatory effects of nitric oxide. Tadalafil promotes selective smooth muscle relaxation in the lung vasculature, possibly by inhibiting PDE5.

• #26 Eisenmenger syndrome: current perspectives | RRCC

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

  Ambrisentan is a selective mono-antagonist of the ETA receptor with a longer half-life allowing for once-a-day dosing. […] Macitentan, similar to bosentan, is a dual ET receptor antagonist and has been evaluated in a large event-driven RCT on patients with PAH (not ES). […] Two small RCTs have been published in ES evaluating the effect of PDE-5i in a total of 48 patients. […] Riociguat is a relatively new drug in PAH. […] Selexipag is one of the latest pulmonary therapies to be tested in PAH. […] There is limited information on the efficacy of combination therapy in ES, even though most experts feel that combining therapies and targeting different pathomechanistic pathways improve results. […] In a prospective open-label study, DAlto et al also studied the combined effect of bosentan and sildenafil in ES patients and reported an improvement in functional class. […] However, patients on combination therapy likely represented a sicker population with more advanced symptoms, hence making direct comparison unreliable.

• #27 Eisenmenger Syndrome Medication: Diuretics, Cardiac Glycosides, Prostaglandins, Iron Products, Rheumatologics, Other, Endothelin Antagonists, Phosphodiesterase-5 Enzyme Inhibitors

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

  Bosentan is an endothelin receptor antagonist indicated for the treatment of pulmonary arterial hypertension (PAH) in patients with WHO class III or IV symptoms. It is used to improve exercise ability and decrease the rate of clinical worsening. […] Ambrisentan is an endothelin receptor antagonist indicated for PAH in patients with WHO class II or III symptoms. It improves exercise ability and decreases the progression of clinical symptoms. […] The antiproliferative effects of the phosphodiesterase type-5 (PDE5) pathway, which regulates cyclic guanosine monophosphate hydrolysis, may be significant in the chronic treatment of pulmonary hypertension with PDE5 inhibitors such as sildenafil. […] Sildenafil promotes selective smooth muscle relaxation in the lung vasculature, possibly by inhibiting PDE5. The inhibition of PDE5 increases cyclic guanosine monophosphate (cGMP) activity, which increases the vasodilatory effects of nitric oxide. […] Tadalafil is a PDE5 selective inhibitor. Inhibition of PDE5 increases cGMP activity, which increases the vasodilatory effects of nitric oxide. Tadalafil promotes selective smooth muscle relaxation in the lung vasculature, possibly by inhibiting PDE5.

• #28 Oral sildenafil treatment for Eisenmenger syndrome: a prospective, open-label, multicentre study | Heart

  https://heart.bmj.com/content/97/22/1876

  Oral sildenafil treatment for Eisenmenger syndrome: a prospective, open-label, multicentre study […] To investigate whether long-term treatment (12months) with the phosphodiesterase type 5 inhibitor sildenafil improves clinical and haemodynamic parameters in patients with Eisenmenger syndrome. […] Twelve months of oral sildenafil treatment was well tolerated and appeared to improve exercise capacity, systemic arterial oxygen saturation and haemodynamic parameters in patients with Eisenmenger syndrome.

• #29 Eisenmenger Syndrome Medication: Diuretics, Cardiac Glycosides, Prostaglandins, Iron Products, Rheumatologics, Other, Endothelin Antagonists, Phosphodiesterase-5 Enzyme Inhibitors

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

  Bosentan is an endothelin receptor antagonist indicated for the treatment of pulmonary arterial hypertension (PAH) in patients with WHO class III or IV symptoms. It is used to improve exercise ability and decrease the rate of clinical worsening. […] Ambrisentan is an endothelin receptor antagonist indicated for PAH in patients with WHO class II or III symptoms. It improves exercise ability and decreases the progression of clinical symptoms. […] The antiproliferative effects of the phosphodiesterase type-5 (PDE5) pathway, which regulates cyclic guanosine monophosphate hydrolysis, may be significant in the chronic treatment of pulmonary hypertension with PDE5 inhibitors such as sildenafil. […] Sildenafil promotes selective smooth muscle relaxation in the lung vasculature, possibly by inhibiting PDE5. The inhibition of PDE5 increases cyclic guanosine monophosphate (cGMP) activity, which increases the vasodilatory effects of nitric oxide. […] Tadalafil is a PDE5 selective inhibitor. Inhibition of PDE5 increases cGMP activity, which increases the vasodilatory effects of nitric oxide. Tadalafil promotes selective smooth muscle relaxation in the lung vasculature, possibly by inhibiting PDE5.

• #30 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. […] Vasodilator therapy improves symptoms in patients with Eisenmenger syndrome and should be used routinely in the management of this patient population. […] Long-term prostacyclin therapy has been shown to improve hemodynamics and the quality of life in patients with congenital heart disease and PAH. […] Bosentan therapy resulted in improved oxygen saturation and improved NYHA class. […] A systematic review of the effect of endothelin-receptor antagonists in patients with Eisenmenger syndrome found that bosentan was safe and improved hemodynamics, but results were mixed regarding exercise capacity. […] Anticoagulation is still not routinely recommended for these patients. […] Pregnancy should therefore be avoided by women with Eisenmenger syndrome.

• #31 Eisenmenger Syndrome Medication: Diuretics, Cardiac Glycosides, Prostaglandins, Iron Products, Rheumatologics, Other, Endothelin Antagonists, Phosphodiesterase-5 Enzyme Inhibitors

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

  The medical treatment of Eisenmenger syndrome is directed toward the improvement of symptoms related to heart failure and pulmonary hypertension and the prevention and management of complications related to cyanotic congenital heart disease. […] A partial list of medications used in the management of Eisenmenger syndrome includes aspirin, to prevent thrombotic complications; allopurinol, for gout; iron supplementation, for microcytosis; and digitalis and diuretics, for symptoms of heart failure. […] These drugs can be effective in reversing reactive pulmonary vasoconstriction and can, therefore, lower pulmonary vascular resistance, decrease afterload, reduce the right ventricle, and reduce right-to-left shunting. In some patients, chronic prostacyclin analogue therapy (epoprostenol) can be of benefit, particularly as a bridge to heart-lung transplantation.

• #32 Current therapy of Eisenmenger syndrome – Kim – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/10757/html

  Other recent randomized controlled trials in ES with phosphodiesterase type-5 inhibitors have shown improvements in exercise capacity and hemodynamics. […] Several studies have reported beneficial effects of inhaled iloprost, resulting in improved WHO functional class, hemodynamics, an increased 6-minute walking distance, and quality of life. A retrospective study of adult patients with PAH to CHD in Korea also suggested that inhaled iloprost as a perioperative medical intervention for patients with PAH-CHD is safe and effective in improving the systemic oxygen saturation and for early recovery in the postoperative course. […] Also, a prospective single-arm study which 18 patients with ES and exertional dyspnea according to WHO functional class III or IV were prospectively recruited had showed that 24 weeks of inhaled iloprost therapy in patients with ES led to significant improvements in exercise capacity, quality of life, and right ventricular function.

• #33 Eisenmenger syndrome: current perspectives | RRCC

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

  Ambrisentan is a selective mono-antagonist of the ETA receptor with a longer half-life allowing for once-a-day dosing. […] Macitentan, similar to bosentan, is a dual ET receptor antagonist and has been evaluated in a large event-driven RCT on patients with PAH (not ES). […] Two small RCTs have been published in ES evaluating the effect of PDE-5i in a total of 48 patients. […] Riociguat is a relatively new drug in PAH. […] Selexipag is one of the latest pulmonary therapies to be tested in PAH. […] There is limited information on the efficacy of combination therapy in ES, even though most experts feel that combining therapies and targeting different pathomechanistic pathways improve results. […] In a prospective open-label study, DAlto et al also studied the combined effect of bosentan and sildenafil in ES patients and reported an improvement in functional class. […] However, patients on combination therapy likely represented a sicker population with more advanced symptoms, hence making direct comparison unreliable.

• #34 Eisenmenger Syndrome Medication: Diuretics, Cardiac Glycosides, Prostaglandins, Iron Products, Rheumatologics, Other, Endothelin Antagonists, Phosphodiesterase-5 Enzyme Inhibitors

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

  The medical treatment of Eisenmenger syndrome is directed toward the improvement of symptoms related to heart failure and pulmonary hypertension and the prevention and management of complications related to cyanotic congenital heart disease. […] A partial list of medications used in the management of Eisenmenger syndrome includes aspirin, to prevent thrombotic complications; allopurinol, for gout; iron supplementation, for microcytosis; and digitalis and diuretics, for symptoms of heart failure. […] These drugs can be effective in reversing reactive pulmonary vasoconstriction and can, therefore, lower pulmonary vascular resistance, decrease afterload, reduce the right ventricle, and reduce right-to-left shunting. In some patients, chronic prostacyclin analogue therapy (epoprostenol) can be of benefit, particularly as a bridge to heart-lung transplantation.

• #35 Eisenmenger syndrome: current perspectives | RRCC

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

  Interestingly, in a study of 153 patients with ES, Diller et al noted a trend toward better survival in patients on -blockers in their analysis. […] Aldosterone antagonists (eg, spironolactone) and loop diuretics are often used in these patients. […] However, the use of diuretics should be carefully monitored as dehydration and significant fluid shifts may exacerbate a right-to-left shunt, thus worsening the cyanosis, potentiating renal dysfunction, and increasing the risk of thromboembolism. […] Traditionally, angiotensin-converting enzyme (ACE) inhibitors and angiotensin II receptor blockers (ARBs) have been avoided in patients with ES because of the potential worsening of cyanosis from an increase in a right-to-left shunt as a consequence of the reduction of the systemic vascular resistance (SVR).

• #36 Eisenmenger syndrome: current perspectives | RRCC

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

  Interestingly, in a study of 153 patients with ES, Diller et al noted a trend toward better survival in patients on -blockers in their analysis. […] Aldosterone antagonists (eg, spironolactone) and loop diuretics are often used in these patients. […] However, the use of diuretics should be carefully monitored as dehydration and significant fluid shifts may exacerbate a right-to-left shunt, thus worsening the cyanosis, potentiating renal dysfunction, and increasing the risk of thromboembolism. […] Traditionally, angiotensin-converting enzyme (ACE) inhibitors and angiotensin II receptor blockers (ARBs) have been avoided in patients with ES because of the potential worsening of cyanosis from an increase in a right-to-left shunt as a consequence of the reduction of the systemic vascular resistance (SVR).

• #37 Eisenmenger syndrome – Wikipedia

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

  Supportive measures, transplant surgery […] If the inciting defect in the heart is identified before it causes significant pulmonary hypertension, it can normally be repaired through surgery, preventing the disease. After pulmonary hypertension is sufficient to reverse the blood flow through the defect, however, the maladaptation is considered irreversible, and a heart-lung transplant or a lung transplant with repair of the heart is the only curative option. Transplantation is the final therapeutic option and only for patients with poor prognosis and quality of life. Timing and appropriateness of transplantation remain difficult decisions. […] Various medicines and therapies for pulmonary hypertension are under investigation for treatment of the symptoms. […] Antiarrhythmic drugs are important for many patients with Eisenmenger syndrome, as evidence suggests that arrhythmia-induced sudden cardiac death may be the leading cause of death among patients with the disease. These therapies generally aim to restore and maintain sinus rhythm, but the specific interventions chosen will depend on the nature of the patient’s arrhythmia.

• #38 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 patients require life-long expert assessment and therapy at specialized centers. […] Since the advent of targeted therapies for pulmonary arterial hypertension approximately 20 years ago and subsequent positive results in Eisenmenger patients, these patients can be offered a therapeutic option that improves symptoms, exercise capacity and potentially outcome. […] Therapy is based on various pathophysiologic pathways. […] The majority (as well as the most robust) data exist on the effect of endothelin receptor antagonists in patients with Eisenmenger syndrome. […] This has sparked interest in various treatment approaches using also phosphodiesterase-5 inhibitors (mainly Sildenafil) in this setting. […] At most centers, endothelin receptor antagonist therapy remains the first choice for symptomatic Eisenmenger patients (especially those in NYHA class III and those deteriorating over time).

• #39 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/

  Therapy initiation or escalation is also useful in patients with low oxygen saturation and symptoms interfering with daily activities. […] 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.

• #40 2020 ESC Guidelines for the Management of ACHD: Focus on PH

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

  Advanced therapies for PAH have been shown to benefit patients with Eisenmenger syndrome and likely will benefit other patients with PAH in CHD. Endothelin-receptor antagonist (ERA) therapy, specifically with bosentan, has been shown to improve objective measures as well as quality of life in patients with Eisenmenger syndrome, but its effect on mortality is less well known. […] Most treatment centers use a symptom-oriented treatment strategy, starting with an oral ERA or phosphodiesterase type 5 inhibitor in low- to intermediate-risk patients and then escalating therapy if symptoms persist or there is clinical deterioration. […] Heart-lung transplantation or lung transplantation with heart surgery is an option in rare patients who are unresponsive to medical treatment, but it must be evaluated on a case-by-case basis because surgical complexity and organ availability are both limiting.

• #41 Review of Eisenmenger Syndrome

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

  Eisenmenger syndrome (ES) is defined as pulmonary hypertension at a systemic level, caused by a high pulmonary vascular resistance (PVR), with reversed or bidirectional shunt at the aortopulmonary, ventricular, or atrial level. […] Advanced pulmonary arterial hypertension therapies have an important role in the management of patients with ES. The authors propose a staged approach beginning with monotherapy with an endothelin receptor antagonist or phosphodiesterase type 5 inhibitor. The next steps would include combination therapy, then triple therapy, and then assessment for lung or heart-lung transplantation. […] Routine anticoagulation is not indicated for patients with ES. Anticoagulation should be reserved for selected patients with atrial arrhythmias or known pulmonary artery thrombus or embolism.

• #42 Eisenmenger syndrome: current perspectives | RRCC

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

  Ambrisentan is a selective mono-antagonist of the ETA receptor with a longer half-life allowing for once-a-day dosing. […] Macitentan, similar to bosentan, is a dual ET receptor antagonist and has been evaluated in a large event-driven RCT on patients with PAH (not ES). […] Two small RCTs have been published in ES evaluating the effect of PDE-5i in a total of 48 patients. […] Riociguat is a relatively new drug in PAH. […] Selexipag is one of the latest pulmonary therapies to be tested in PAH. […] There is limited information on the efficacy of combination therapy in ES, even though most experts feel that combining therapies and targeting different pathomechanistic pathways improve results. […] In a prospective open-label study, DAlto et al also studied the combined effect of bosentan and sildenafil in ES patients and reported an improvement in functional class. […] However, patients on combination therapy likely represented a sicker population with more advanced symptoms, hence making direct comparison unreliable.

• #43 Eisenmenger syndrome: current perspectives | RRCC

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

  Ambrisentan is a selective mono-antagonist of the ETA receptor with a longer half-life allowing for once-a-day dosing. […] Macitentan, similar to bosentan, is a dual ET receptor antagonist and has been evaluated in a large event-driven RCT on patients with PAH (not ES). […] Two small RCTs have been published in ES evaluating the effect of PDE-5i in a total of 48 patients. […] Riociguat is a relatively new drug in PAH. […] Selexipag is one of the latest pulmonary therapies to be tested in PAH. […] There is limited information on the efficacy of combination therapy in ES, even though most experts feel that combining therapies and targeting different pathomechanistic pathways improve results. […] In a prospective open-label study, DAlto et al also studied the combined effect of bosentan and sildenafil in ES patients and reported an improvement in functional class. […] However, patients on combination therapy likely represented a sicker population with more advanced symptoms, hence making direct comparison unreliable.

• #44 Advanced therapies in Eisenmenger syndrome | Journal of Congenital Cardiology | Full Text

  https://jcongenitalcardiology.biomedcentral.com/articles/10.1186/s40949-020-00048-6

  After discussion within the multidisciplinary team, we proceeded with escalation to triple therapy and she was commenced on intravenous epoprostenol. […] Moreover, there was a significant subjective and objective improvement in functional and prognostic parameters. […] Early double and triple combination therapy in this patient group are not supported by evidence but can prove to be beneficial in select patients with clear evidence of suboptimal response to treatment or disease progression. […] Aggressive treatment with PAH therapies should be considered, including combination therapy.

• #45 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. […] Vasodilator therapy improves symptoms in patients with Eisenmenger syndrome and should be used routinely in the management of this patient population. […] Long-term prostacyclin therapy has been shown to improve hemodynamics and the quality of life in patients with congenital heart disease and PAH. […] Bosentan therapy resulted in improved oxygen saturation and improved NYHA class. […] A systematic review of the effect of endothelin-receptor antagonists in patients with Eisenmenger syndrome found that bosentan was safe and improved hemodynamics, but results were mixed regarding exercise capacity. […] Anticoagulation is still not routinely recommended for these patients. […] Pregnancy should therefore be avoided by women with Eisenmenger syndrome.

• #46 Eisenmenger’s syndrome medical therapy – wikidoc

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

  If surgical intervention is not available, treatment is mostly palliative. It includes anticoagulants, pulmonary vasodilators such as bosentan, PGE5 inhibitor, prostacyclin, antibiotic prophylaxis to prevent endocarditis, phlebotomy to treat polycythemia, and maintaining proper fluid balance. […] These measures can prolong lifespan and improve quality of life. They include: Anticoagulants as warfarin; however, the evidence for its benefit is mainly based on observational studies. […] Pulmonary vasodilators such as bosentan: The BREATHE-5 trial showed improved exercise capacity and symptomatic improvement. […] PGE5 inhibitor as sildenafil that showed a mortality benefit in clinical trials. […] Prostacyclin may improve pulmonary artery pressure and may improve length of life. […] Antibiotic prophylaxis to prevent endocarditis.

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

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

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

• #48 Eisenmenger’s Syndrome | Doctor

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

  Prevention of infective endocarditis: The National Institute for Health and Care Excellence (NICE) recommends that if a person at risk of infective endocarditis is receiving antimicrobial therapy because they are undergoing a gastrointestinal or genitourinary procedure at a site where there is a suspected infection, the person should receive an antibiotic that covers organisms that cause infective endocarditis. […] Routine therapeutic venesections have no place in treatment, as they cause or exacerbate iron deficiency and reduce oxygen tissue delivery, thus increasing the risk of cerebrovascular events. Venesection should only be considered for patients with significantly elevated haemoglobin concentration and hematocrit. […] Chronic use of oxygen or pulmonary vasodilators is controversial. […] With continued improvements in the diagnosis, pre-operative management, refinement of surgical techniques and postoperative management strategies, patients with Eisenmenger’s syndrome might be amenable to, and benefit from, repair. […] Ultimately, heart-lung transplant may be indicated.

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

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

  1. General measures: Administration of influenza vaccine every year, updated vaccination against coronavirus disease 2019 (COVID-19) as per the national guidelines, and pneumococcal vaccine every 5 years. […] Patients should be followed in specialized centers with expertise in care of adults with congenital heart disease and pulmonary hypertension. […] Treatment of hyperviscosity should only be performed in patients with hematocrit 65% who have moderate to severe symptoms of hyperviscosity and in whom iron deficiency and dehydration have been excluded. […] Treatment of arrhythmias: Sinus rhythm should be maintained with individualization of antiarrhythmic therapy. […] Targeted treatment of pulmonary hypertension: In patients with World Health Organization (WHO) functional class III symptoms, the endothelin-receptor antagonist (ERA) bosentan should be initiated.

• #50 Eisenmenger’s Syndrome | Doctor

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

  Eisenmenger’s syndrome treatment and management includes heart failure management. […] Management of arrhythmias. […] Therapy for pulmonary artery hypertension: bosentan, a dual endothelin receptor antagonist (ERA), was the first drug to be studied and was recommended as a first-class drug in symptomatic patients. Macitentan, a newer-generation ERA, has also been assessed. Trials on phosphodiesterase type 5 (PDE-5) inhibitors also showed positive haemodynamic and functional effects. Limited data exist on the use of intravenous, subcutaneous, or inhaled prostanoids in symptomatic patients, and usually used as third-line therapy. […] The use of anticoagulation remains a controversial issue and should be carefully considered on a case-by-case basis. Current guidelines state that anticoagulation should be offered for atrial arrhythmias and in the presence of pulmonary artery thrombus or embolism in patients at low bleeding risk. Anticoagulation is therefore not routinely recommended.

• #51 Eisenmenger’s Syndrome | Doctor

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

  Eisenmenger’s syndrome treatment and management includes heart failure management. […] Management of arrhythmias. […] Therapy for pulmonary artery hypertension: bosentan, a dual endothelin receptor antagonist (ERA), was the first drug to be studied and was recommended as a first-class drug in symptomatic patients. Macitentan, a newer-generation ERA, has also been assessed. Trials on phosphodiesterase type 5 (PDE-5) inhibitors also showed positive haemodynamic and functional effects. Limited data exist on the use of intravenous, subcutaneous, or inhaled prostanoids in symptomatic patients, and usually used as third-line therapy. […] The use of anticoagulation remains a controversial issue and should be carefully considered on a case-by-case basis. Current guidelines state that anticoagulation should be offered for atrial arrhythmias and in the presence of pulmonary artery thrombus or embolism in patients at low bleeding risk. Anticoagulation is therefore not routinely recommended.

• #52 Review of Eisenmenger Syndrome

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

  Eisenmenger syndrome (ES) is defined as pulmonary hypertension at a systemic level, caused by a high pulmonary vascular resistance (PVR), with reversed or bidirectional shunt at the aortopulmonary, ventricular, or atrial level. […] Advanced pulmonary arterial hypertension therapies have an important role in the management of patients with ES. The authors propose a staged approach beginning with monotherapy with an endothelin receptor antagonist or phosphodiesterase type 5 inhibitor. The next steps would include combination therapy, then triple therapy, and then assessment for lung or heart-lung transplantation. […] Routine anticoagulation is not indicated for patients with ES. Anticoagulation should be reserved for selected patients with atrial arrhythmias or known pulmonary artery thrombus or embolism.

• #53 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.

  Single-lung or total heart-lung transplant may be used in patients with severe hypoxemia or heart failure and no contraindications to surgery in whom the risk of death within 1 year is 50%. […] Noncardiac surgery: This should only be performed when absolutely necessary and unavoidable due to the high mortality associated with Eisenmenger syndrome. […] Endocarditis prophylaxis is indicated in all patients with cyanotic congenital heart disease. […] Routine anticoagulation: Although patients with Eisenmenger syndrome are at risk of thrombosis (especially in the dilated pulmonary arteries), currently available data do not support the routine use of anticoagulation in these patients due to the inherent increased risk of bleeding as well as difficulty with the routine monitoring of anticoagulation.

• #54 Eisenmenger’s Syndrome | Doctor

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

  Prevention of infective endocarditis: The National Institute for Health and Care Excellence (NICE) recommends that if a person at risk of infective endocarditis is receiving antimicrobial therapy because they are undergoing a gastrointestinal or genitourinary procedure at a site where there is a suspected infection, the person should receive an antibiotic that covers organisms that cause infective endocarditis. […] Routine therapeutic venesections have no place in treatment, as they cause or exacerbate iron deficiency and reduce oxygen tissue delivery, thus increasing the risk of cerebrovascular events. Venesection should only be considered for patients with significantly elevated haemoglobin concentration and hematocrit. […] Chronic use of oxygen or pulmonary vasodilators is controversial. […] With continued improvements in the diagnosis, pre-operative management, refinement of surgical techniques and postoperative management strategies, patients with Eisenmenger’s syndrome might be amenable to, and benefit from, repair. […] Ultimately, heart-lung transplant may be indicated.

• #55 Eisenmenger syndrome: current perspectives | RRCC

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

  However, evidence in this is limited and ambiguous. […] The only definitive treatment for ES is lung transplantation (LT) with shunt closure or heart and lung transplantation (HLT). […] The mainstay of PAH treatment is, nowadays, specific disease-targeting pulmonary therapies, which have only been in use since the 1990s. […] So far, only three pathomechanistic pathways in PAH have been translated into clinical practice: prostacyclin, nitric oxide, and endothelin pathways. […] Despite the associated functional limitations and multitude of comorbidities, survival prospects in adults with ES are generally considered better than in other forms of PAH, but mortality remains high. […] Bosentan is currently endorsed as the first-line choice of treatment in patients with ES in the WHO functional class IIIIV.

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

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

  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). […] Patients with right-sided congestive heart failure should follow a no-salt-added or salt-restricted diet. […] Intense athletic activities carry the risk of sudden death in patients with Eisenmenger syndrome. […] Prevention of Eisenmenger syndrome is critical. […] In the course of therapy for patients with Eisenmenger syndrome, the following consultations may be appropriate:

• #57 2020 ESC Guidelines for the Management of ACHD: Focus on PH

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

  Advanced therapies for PAH have been shown to benefit patients with Eisenmenger syndrome and likely will benefit other patients with PAH in CHD. Endothelin-receptor antagonist (ERA) therapy, specifically with bosentan, has been shown to improve objective measures as well as quality of life in patients with Eisenmenger syndrome, but its effect on mortality is less well known. […] Most treatment centers use a symptom-oriented treatment strategy, starting with an oral ERA or phosphodiesterase type 5 inhibitor in low- to intermediate-risk patients and then escalating therapy if symptoms persist or there is clinical deterioration. […] Heart-lung transplantation or lung transplantation with heart surgery is an option in rare patients who are unresponsive to medical treatment, but it must be evaluated on a case-by-case basis because surgical complexity and organ availability are both limiting.

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

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

  Medications to treat pulmonary arterial hypertension (eg, prostacyclin analogs, endothelin antagonists, phosphodiesterase-5 inhibitors) […] There is no specific treatment once the syndrome develops, other than heart and lung transplantation, but drugs that may lower pulmonary vascular resistance (eg, prostacyclin analogs, endothelin antagonists, phosphodiesterase-5 inhibitors) are useful. […] Supportive treatment includes avoidance of conditions that may exacerbate the syndrome (eg, pregnancy, volume depletion, isometric exercise, high altitudes, smoking). Supplemental oxygen may provide some benefit. […] Heart and lung transplantation or lung transplantation with cardiac repair may be an option, but transplantation is reserved for patients with severe symptoms and unacceptable quality of life. Long-term prognosis after transplantation is guarded. […] All patients should be given endocarditis prophylaxis before dental or surgical procedures that are likely to cause bacteremia.

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

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

  Medications to treat pulmonary arterial hypertension (eg, prostacyclin analogs, endothelin antagonists, phosphodiesterase-5 inhibitors) […] There is no specific treatment once the syndrome develops, other than heart and lung transplantation, but drugs that may lower pulmonary vascular resistance (eg, prostacyclin analogs, endothelin antagonists, phosphodiesterase-5 inhibitors) are useful. […] Supportive treatment includes avoidance of conditions that may exacerbate the syndrome (eg, pregnancy, volume depletion, isometric exercise, high altitudes, smoking). Supplemental oxygen may provide some benefit. […] Heart and lung transplantation or lung transplantation with cardiac repair may be an option, but transplantation is reserved for patients with severe symptoms and unacceptable quality of life. Long-term prognosis after transplantation is guarded. […] All patients should be given endocarditis prophylaxis before dental or surgical procedures that are likely to cause bacteremia.

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

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

  The goals of Eisenmenger syndrome treatment are to: […] 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. […] Healthcare professionals don’t recommend surgery to repair the hole in the heart once Eisenmenger syndrome has developed. […] If you need treatment for Eisenmenger syndrome, get care at a medical center with healthcare professionals who have experience in congenital heart diseases.

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

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

  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). […] Patients with right-sided congestive heart failure should follow a no-salt-added or salt-restricted diet. […] Intense athletic activities carry the risk of sudden death in patients with Eisenmenger syndrome. […] Prevention of Eisenmenger syndrome is critical. […] In the course of therapy for patients with Eisenmenger syndrome, the following consultations may be appropriate:

• #62 Eisenmenger Syndrome | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/20936

  Cardiopulmonary transplantation is curative for Eisenmenger syndrome. However, this is impractical in most settings. Pharmacology has improved symptoms but not mortality. Potential pharmaceuticals for the treatment of Eisenmenger syndrome include diuretics, antiarrhythmics, and anticoagulation in some patients. Supplemental oxygen has not definitively shown to have a mortality benefit. Vasodilator therapies may provide an opportunity for clinical research, and studies have shown some symptomatic improvement. The Bosentan Randomized Trial of Endothelin Antagonist Therapy-5 trial showed improved exercise capacity and symptomatic improvement with endothelin antagonists in patients with ASD, VSD, and PDA with Eisenmenger syndrome […] Clinical follow-up should focus on checking annual CBC, iron studies, kidney function, and uric acid, with a focus on correcting any abnormalities. Patients also should be assessed with pulse oximetry, both with and without supplemental O2. Any abnormalities suggesting hypoxemia warrant further evaluation. Surgical correction of the causative heart defect in adult patients is generally contraindicated. In patients who have developed PAH as a consequence of unrepaired CHD, the defect itself may be acting as a protective measure, preventing worsening of the pulmonary vascular resistance in the face of increasing right ventricular pressure. In children, the development of pulmonary vascular disease (greater than 6 Woods units/m2) with poor vasodilator response is likely to face post-surgical complications, including right heart failure, increased pulmonary hypertension, and hypertensive crises. Classically, it is thought to be of little benefit to undergo such risky procedures with a plethora of potential consequences. Additionally, even with clinical response to vasodilator therapies for the pulmonary arterial hypertension component, there are only a select few case reports that show benefit for the closure of the anatomical defect. However, there is promising evidence that cardiac surgery and close peri- and post-operational management of pulmonary vascular resistance with advanced PAH therapies may improve safety and mitigate downstream problems.

• #63 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. […] Vasodilator therapy improves symptoms in patients with Eisenmenger syndrome and should be used routinely in the management of this patient population. […] Long-term prostacyclin therapy has been shown to improve hemodynamics and the quality of life in patients with congenital heart disease and PAH. […] Bosentan therapy resulted in improved oxygen saturation and improved NYHA class. […] A systematic review of the effect of endothelin-receptor antagonists in patients with Eisenmenger syndrome found that bosentan was safe and improved hemodynamics, but results were mixed regarding exercise capacity. […] Anticoagulation is still not routinely recommended for these patients. […] Pregnancy should therefore be avoided by women with Eisenmenger syndrome.

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

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

  Medications to treat pulmonary arterial hypertension (eg, prostacyclin analogs, endothelin antagonists, phosphodiesterase-5 inhibitors) […] There is no specific treatment once the syndrome develops, other than heart and lung transplantation, but drugs that may lower pulmonary vascular resistance (eg, prostacyclin analogs, endothelin antagonists, phosphodiesterase-5 inhibitors) are useful. […] Supportive treatment includes avoidance of conditions that may exacerbate the syndrome (eg, pregnancy, volume depletion, isometric exercise, high altitudes, smoking). Supplemental oxygen may provide some benefit. […] Heart and lung transplantation or lung transplantation with cardiac repair may be an option, but transplantation is reserved for patients with severe symptoms and unacceptable quality of life. Long-term prognosis after transplantation is guarded. […] All patients should be given endocarditis prophylaxis before dental or surgical procedures that are likely to cause bacteremia.

• #65 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.

  Single-lung or total heart-lung transplant may be used in patients with severe hypoxemia or heart failure and no contraindications to surgery in whom the risk of death within 1 year is 50%. […] Noncardiac surgery: This should only be performed when absolutely necessary and unavoidable due to the high mortality associated with Eisenmenger syndrome. […] Endocarditis prophylaxis is indicated in all patients with cyanotic congenital heart disease. […] Routine anticoagulation: Although patients with Eisenmenger syndrome are at risk of thrombosis (especially in the dilated pulmonary arteries), currently available data do not support the routine use of anticoagulation in these patients due to the inherent increased risk of bleeding as well as difficulty with the routine monitoring of anticoagulation.

• #66 Postoperative Management of Eisenmenger’s Syndrome

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

  The fundamental principles in perioperative management of patients with ES involve preventing systemic hypotension and avoiding elevation of pulmonary vascular resistance. […] Therefore, measures should be in place to detect and manage alteration in hemodynamics during induction of anesthesia and surgery. […] It is common for anesthetic induction to cause hypotension regardless of the induction agent used in ES patients. The use of vasopressor before and during anesthetic induction has been reported to reduce the incidence of hypotension. […] Therefore, it is generally recommended to use vasopressors during induction to maintain SVR and cardiac output. […] 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.

• #67 Postoperative Management of Eisenmenger’s Syndrome

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

  The fundamental principles in perioperative management of patients with ES involve preventing systemic hypotension and avoiding elevation of pulmonary vascular resistance. […] Therefore, measures should be in place to detect and manage alteration in hemodynamics during induction of anesthesia and surgery. […] It is common for anesthetic induction to cause hypotension regardless of the induction agent used in ES patients. The use of vasopressor before and during anesthetic induction has been reported to reduce the incidence of hypotension. […] Therefore, it is generally recommended to use vasopressors during induction to maintain SVR and cardiac output. […] 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.

• #68 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. […] Surgical palliation or repair should be performed early in patients with congenital heart disease to prevent progression to Eisenmenger syndrome. […] Heart-lung transplantation and single or bilateral, sequential lung transplantation, with or without repair of relatively simple congenital cardiovascular anomalies, are viable transplant procedures, and they are the only surgical options for a patient with Eisenmenger syndrome. […] Initiation of vasodilator therapy may require inpatient hospitalization and observation. […] Patients should follow up at a specialized cardiology clinic within 2 weeks of discharge from inpatient care and every 3 months when stable.

• #69 Eisenmenger Syndrome – StatPearls – NCBI Bookshelf

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

  Cardiopulmonary transplantation is curative for Eisenmenger syndrome. However, this is impractical in most settings. Pharmacology has improved symptoms but not mortality. Potential pharmaceuticals for the treatment of Eisenmenger syndrome include diuretics, antiarrhythmics, and anticoagulation in some patients. Supplemental oxygen has not definitively shown to have a mortality benefit. Vasodilator therapies may provide an opportunity for clinical research, and studies have shown some symptomatic improvement. The Bosentan Randomized Trial of Endothelin Antagonist Therapy-5 trial showed improved exercise capacity and symptomatic improvement with endothelin antagonists in patients with ASD, VSD, and PDA with Eisenmenger syndrome. […] Clinical follow-up should focus on checking annual CBC, iron studies, kidney function, and uric acid, with a focus on correcting any abnormalities. Patients also should be assessed with pulse oximetry, both with and without supplemental O2. Any abnormalities suggesting hypoxemia warrant further evaluation. Surgical correction of the causative heart defect in adult patients is generally contraindicated.

• #70 Eisenmenger Syndrome – StatPearls – NCBI Bookshelf

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

  Cardiopulmonary transplantation is curative for Eisenmenger syndrome. However, this is impractical in most settings. Pharmacology has improved symptoms but not mortality. Potential pharmaceuticals for the treatment of Eisenmenger syndrome include diuretics, antiarrhythmics, and anticoagulation in some patients. Supplemental oxygen has not definitively shown to have a mortality benefit. Vasodilator therapies may provide an opportunity for clinical research, and studies have shown some symptomatic improvement. The Bosentan Randomized Trial of Endothelin Antagonist Therapy-5 trial showed improved exercise capacity and symptomatic improvement with endothelin antagonists in patients with ASD, VSD, and PDA with Eisenmenger syndrome. […] Clinical follow-up should focus on checking annual CBC, iron studies, kidney function, and uric acid, with a focus on correcting any abnormalities. Patients also should be assessed with pulse oximetry, both with and without supplemental O2. Any abnormalities suggesting hypoxemia warrant further evaluation. Surgical correction of the causative heart defect in adult patients is generally contraindicated.

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

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

  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). […] Patients with right-sided congestive heart failure should follow a no-salt-added or salt-restricted diet. […] Intense athletic activities carry the risk of sudden death in patients with Eisenmenger syndrome. […] Prevention of Eisenmenger syndrome is critical. […] In the course of therapy for patients with Eisenmenger syndrome, the following consultations may be appropriate:

• #72 Eisenmenger Syndrome: Causes, Symptoms and Treatment

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

  Treatment for Eisenmenger syndrome is aimed at easing symptoms and preventing severe damage to the heart or lungs. Therapies may include: […] By the time Eisenmenger syndrome develops, its usually too late to perform a surgical repair of the underlying heart defect. However, people with severe symptoms and organ damage could need a heart or lung transplant. […] The only way to prevent Eisenmenger syndrome is to have congenital heart defects repaired as early in life as possible. […] Treatment is nonsurgical and focuses on easing symptoms and preventing severe heart and lung damage.

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

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

  1. General measures: Administration of influenza vaccine every year, updated vaccination against coronavirus disease 2019 (COVID-19) as per the national guidelines, and pneumococcal vaccine every 5 years. […] Patients should be followed in specialized centers with expertise in care of adults with congenital heart disease and pulmonary hypertension. […] Treatment of hyperviscosity should only be performed in patients with hematocrit 65% who have moderate to severe symptoms of hyperviscosity and in whom iron deficiency and dehydration have been excluded. […] Treatment of arrhythmias: Sinus rhythm should be maintained with individualization of antiarrhythmic therapy. […] Targeted treatment of pulmonary hypertension: In patients with World Health Organization (WHO) functional class III symptoms, the endothelin-receptor antagonist (ERA) bosentan should be initiated.

• #74 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). […] Much of the therapy being used for Eisenmenger syndrome has been studied in the treatment of idiopathic pulmonary arterial hypertension (IPAH). […] Patients should avoid sudden fluid shifts or dehydration, which may increase right-to-left shunting.

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

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

  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). […] Patients with right-sided congestive heart failure should follow a no-salt-added or salt-restricted diet. […] Intense athletic activities carry the risk of sudden death in patients with Eisenmenger syndrome. […] Prevention of Eisenmenger syndrome is critical. […] In the course of therapy for patients with Eisenmenger syndrome, the following consultations may be appropriate:

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

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

  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). […] Patients with right-sided congestive heart failure should follow a no-salt-added or salt-restricted diet. […] Intense athletic activities carry the risk of sudden death in patients with Eisenmenger syndrome. […] Prevention of Eisenmenger syndrome is critical. […] In the course of therapy for patients with Eisenmenger syndrome, the following consultations may be appropriate:

• #77 Survival Rates in Eisenmenger Syndrome Treated With Pulmonary Vasodilator Therapy – Pulmonology Advisor

  https://www.pulmonologyadvisor.com/news/survival-rates-in-eisenmenger-syndrome-treated-with-pulmonary-vasodilator-therapy/

  Mortality may be independently decreased with the use of advanced pulmonary vasodilator therapy in patients with Eisenmenger syndrome, according to a study published in Heart. […] Study results demonstrated that treatment with pulmonary vasodilator therapy was independently associated with a greater survival rate (hazard ratio [HR]: 2.27, 95% CI 1.49-3.45; P .001). […] The investigators concluded that pulmonary vasodilator therapy may be independently associated with greater survival and decreased mortality in patients with Eisenmenger syndrome.

• #78 Current therapy of Eisenmenger syndrome – Kim – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/10757/html

  Eisenmenger syndrome (ES) is a complex and disastrous medical problem with profound cyanosis and clinical deterioration by significant right to left shunting. […] In the past, the management of patients with ES was limited to conventional therapy with an emphasis on regular informed cardiovascular follow-up. Subsequent clinical studies have made it possible to improve to patient survival and functional capacity. There are major therapeutic targets in PAH treatment using endothelin-receptor antagonists, phosphodiesterase type-5 inhibitors, and prostacyclin derivatives. […] Nevertheless, many studies suggest advanced PAH therapies should be needed to improve the survival of PAH patients. The BREATHE-5 trial, first placebo-controlled trial in patients with ES, demonstrated a significant improvement of hemodynamics and exercise capacity without adversely affecting systemic arterial oxygen saturation on bosentan-treated patients.

• #79 Long-term results of treatment with bosentan in adult Eisenmenger’s syndrome patients with Down’s syndrome related to congenital heart disease | BMC Cardiovascular Disorders | Full Text

  https://bmccardiovascdisord.biomedcentral.com/articles/10.1186/1471-2261-13-74

  The aim of the present study was to evaluate the long-term efficacy of bosentan in adult ES patients with Downs syndrome. […] In this long-term analysis, treatment of adult ES patients with Downs syndrome with bosentan for a mean period of more than 4 years resulted in significant initial improvements in SaO2 at rest and 6MWD that were sustained during follow-up. […] The long-term results from this study provide confirmation of the significant improvement in exercise capacity and SpO2 in ES patients with Downs syndrome seen in previous studies. […] Long-term treatment with bosentan in adult ES patients with Downs syndrome was well tolerated and significantly improved oxygen saturation and 6MWD.

• #80 Long-term results of treatment with bosentan in adult Eisenmenger’s syndrome patients with Down’s syndrome related to congenital heart disease | BMC Cardiovascular Disorders | Full Text

  https://bmccardiovascdisord.biomedcentral.com/articles/10.1186/1471-2261-13-74

  The aim of the present study was to evaluate the long-term efficacy of bosentan in adult ES patients with Downs syndrome. […] In this long-term analysis, treatment of adult ES patients with Downs syndrome with bosentan for a mean period of more than 4 years resulted in significant initial improvements in SaO2 at rest and 6MWD that were sustained during follow-up. […] The long-term results from this study provide confirmation of the significant improvement in exercise capacity and SpO2 in ES patients with Downs syndrome seen in previous studies. […] Long-term treatment with bosentan in adult ES patients with Downs syndrome was well tolerated and significantly improved oxygen saturation and 6MWD.

• #81 Eisenmenger syndrome: current perspectives | RRCC

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

  However, evidence in this is limited and ambiguous. […] The only definitive treatment for ES is lung transplantation (LT) with shunt closure or heart and lung transplantation (HLT). […] The mainstay of PAH treatment is, nowadays, specific disease-targeting pulmonary therapies, which have only been in use since the 1990s. […] So far, only three pathomechanistic pathways in PAH have been translated into clinical practice: prostacyclin, nitric oxide, and endothelin pathways. […] Despite the associated functional limitations and multitude of comorbidities, survival prospects in adults with ES are generally considered better than in other forms of PAH, but mortality remains high. […] Bosentan is currently endorsed as the first-line choice of treatment in patients with ES in the WHO functional class IIIIV.

• #82 Current therapy of Eisenmenger syndrome – Kim – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/10757/html

  In summary, the GNR-CHD demonstrated better survival of advanced targeted therapy based on the real world as well as tertiary referrals in the Germany. This modern real world registry data reinforce the reason why specialized medical therapies in PAH expert center should be considered in ES patients.

• #83

  https://link.springer.com/article/10.1007/s11936-999-0031-7

  In general, most authors advocate nonintervention in Eisenmengers syndrome, but an awareness of potential palliative measures to avoid destabilizing a delicately balanced physiology is needed as well. This approach has failed to alter long-term outcome, however. […] The approach to the patient with pulmonary vascular obstructive disease (PVOD) should begin with maximization of palliative therapy and should, as compliance and teaching are ensured, proceed to the use of therapies designed to reverse the underlying proliferative changes in the pulmonary vasculature. […] Selective pulmonary vasodilators and antiproliferative agents hold significant promise in altering the natural history of PVOD associated with intracardiac shunting. […] In the future, the management of Eisenmengers syndrome will probably include a multipharmacologic approach that targets several factors in the inflammatory cascade leading to vascular proliferation, perhaps offered in concert with novel surgical or transcatheter strategies designed to limit intracardiac shunting and, if desired, provide complete repair of intracardiac defects.

• #84 Update on Medical Treatment of Patients With Eisenmenger Syndrome

  https://www.jstage.jst.go.jp/article/ihj/56/Supplement/56_14-347/_article

  Eisenmenger syndrome (ES) is the most advanced form of pulmonary arterial hypertension (PAH) associated with congenital heart defects. […] Clinical management of patients with ES has been dramatically changed and developed in a new therapeutic era of PAH, approximately a century after the first ES case described by Dr. Eisenmenger. Targeted drugs for PAH improve functional capacity and quality of life of patients with ES. In addition, these drugs may also provide a benefit for patients survival. […] To achieve further development in medical treatment of patients with ES, they will need to be individualized according to functional classes, underlying cardiac defects, and underlying systemic diseases.

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