Materiały źródłowe

• #1 Eisenmenger Syndrome – StatPearls – NCBI Bookshelf

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

  Eisenmenger syndrome (ES) is a constellation of symptoms that arise from a congenital heart defect and result in large anatomic shunts. Due to anatomic variations present at birth, hemodynamic forces initially result in a left-right shunt, which develops into severe pulmonary arterial hypertension (PAH) and elevated vascular resistance. Ultimately, due to increased pulmonary vascular resistance, the left-to-right shunt will become a right-to-left shunt, resulting in significant hypoxemia and cyanosis. […] The most common defects leading to Eisenmenger syndrome are ASD, VSD, and PDA defects. The following 3 main processes result in the ultimate reversal of a left-to-right into a right-to-left shunt: Vasoconstriction, which is due to imbalances in pulmonary vascular tone, followed by Vascular remodeling, which is due to the proliferation of pulmonary vascular smooth muscle, and finally Thrombosis, which is caused by the increased resistance of blood flow.

• #2 Eisenmenger Syndrome: Background, Pathophysiology, Etiology

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

  Eisenmenger syndrome refers to any untreated congenital cardiac defect with intracardiac communication that leads to pulmonary hypertension, reversal of flow, and cyanosis. […] The previous left-to-right shunt is converted into a right-to-left shunt secondary to elevated pulmonary artery pressures and associated pulmonary vascular disease. […] Development of the syndrome represents a point at which pulmonary hypertension is irreversible and is an indication that the cardiac lesion is likely inoperable. […] Over time, any communication that allows a left-to-right shunt causes increased pulmonary vascular flow and, eventually, irreversible vascular injury. […] The histologic changes are mediated by increased endothelin 1, elevated thromboxane, platelet activation, and production of intrinsic elastase and vascular endothelial growth factors.

• #3 Eisenmenger’s syndrome pathophysiology – wikidoc

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

  The progression of a heart defect to Eisenmenger’s syndrome depends on the size of left to right shunt, severity of pulmonary vascular disease, and type of defect. The left-to-right shunt at the start increases the pulmonary vascular flow and leads to pulmonary artery hypertension. This causes damage to the delicate pulmonary capillaries, creating scars and fibrous tissue. This leads to hypoxemia, which is compensated by increased RBCs production, leading to polycythemia and hyperviscosity syndrome. Eventually, the building pressure in the pulmonary circulation will cause shunt reversal and development of Eisenmenger’s syndrome. […] Eisenmenger’s syndrome can develop in many types of congenital heart diseases. […] It has been found that among all the congenital heart defects, ventricular septal defect most frequently develops Eisenmenger’s syndrome followed by atrial septal defect and patent ductus arteriosus.

• #4 Eisenmenger Syndrome Causes | Stanford Health Care

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

  Eisenmenger syndrome progresses over time as a result of the effects of high blood pressure in the lungs. This high blood pressure, or pulmonary hypertension, occurs because of congenital heart defects that cause blood flow from the left side of the heart to the right side of the heart (left-to-right shunt). […] Because the pressures within the left side of the heart are normally greater than those within the right side of the heart, an opening between the left and right side of the heart will cause blood to flow from the left side of the heart into the right side. This left-to-right shunting of blood within the heart causes increased blood flow in the blood vessels of the lungs. The increased blood flow in the lungs’ blood vessels causes increased pressure in these vessels (pulmonary hypertension).

• #5 Eisenmenger syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5436914/

  Eisenmenger syndrome (ES) stands for pulmonary hypertension, reversal of the central shunts flow, and cyanosis secondary to any congenital heart defect (CHD) associated with non-repaired intra- or extra-cardiac communication. In this syndrome, the original central left-to-right shunt reverses the direction to right-to-left due to long-standing pulmonary hypertension and high pulmonary vascular resistance turning to systemic or supra-systemic levels, and due to structural obstructive pulmonary vascular disease. […] The chronic pulmonary overflow and the transmission of high systemic pressure to the pulmonary circulation secondary to the central left-to-right shunt induced, over time, the pulmonary vascular change characterized by the decrease of the overall cross-sectional area of the pulmonary arteriolar bed. This pulmonary vascular physical injury may cause mechanical stretch and intimal tears accompanied by histological changes with increased intrinsic elastase production and vascular endothelial growth factors, which will further give rise to intimal hyperplasia and medial hypertrophy of peripheral pulmonary arteries. Moreover, endothelial dysfunction, platelet activation, and increased thromboxane and endothelin levels will cause obstructive pulmonary lesions and pulmonary hypertension.

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

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

  Cardiac anomalies that involve large intracardiac left-to-right shunts often eventually cause increased pulmonary resistance, which first causes bidirectional shunting and ultimately right-to-left shunting (shunt reversal). […] With shunt reversal, deoxygenated blood enters the systemic circulation, causing hypoxia and its complications (eg, clubbing of fingers and toes, secondary polycythemia); polycythemia may cause hyperviscosity, stroke, or other thromboembolic disorders, and/or hyperuricemia. […] 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.

• #7 :: KCJ :: Korean Circulation Journal

  https://e-kcj.org/DOIx.php?id=10.4070/kcj.2007.37.7.287

  Untreated or even treated Eisenmenger syndrome is characterized by a progressive increase in the pulmonary vascular resistance leading to right ventricular failure and death. […] The main vascular changes of pulmonary arterial hypertension are 1) vasoconstriction, 2) smooth-muscle cell and endothelial-cell proliferation, and 3) thrombus formation in the pulmonary vessels. […] High flow and high pressure of pulmonary artery may induce pulmonary vascular endothelial damage, leading to a loss of the endothelial barrier function. […] Endothelial dysfunction also affects the production of vasoconstrictors and vasodilators, and this shifts the balance in favor of factors that induce vasoconstriction and ultimately pulmonary vascular remodeling. […] The pulmonary vascular resistance increases with endothelial dysfunction and this ultimately causes a decrease in left-to-right shunting.

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

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

  Non-restrictive, post-tricuspid shunts such as a VSD or PDA (high flow/high pressure) induce advanced PAH-remodeling frequently and rapidly, usually within a few years. […] The assessment of reversibility is nowadays primarily based on clinical judgement and measurements of hemodynamic variables, which have limitations as surrogates for the stage of the arteriopathy. […] Early identification of the cardiac defect allows detection of no or mild (reversible) arteriopathy, and timely shunt closure can permanently repair the CHD. […] According to current guidelines, assessment of reversibility is limited to hemodynamic variables: those in favor of reversible PAH-CHD are a left-to-right shunt and a PVR index 4 Woods units. […] In conclusion, PAH in CHD can be reversed by early shunt closure, but this potential is lost beyond a certain point of no return.

• #9 EISENMENGER SYNDROME IN THE ADULT WITH CONGENITAL HEART DISEASE | Thoracic Key

  https://thoracickey.com/eisenmenger-syndrome-in-the-adult-with-congenital-heart-disease/

  The incidence of PAH and development of reversed shunting depends on the specific heart defect as well as any interventions performed. The risk of developing ES is determined by the size of the initial left-to-right shunt as well as the volume of pulmonary blood flow. The volume of pulmonary blood flow is determined by the PVR, which is normally high in the neonatal period, and gradually drops over a period of about 2 months. Larger shunts have an increased risk of progressing to ES. […] The progression to Eisenmenger syndrome is represented by a spectrum of morphologic changes in the capillary bed that progress from reversible lesions to irreversible ones. Endothelial dysfunction and smooth muscle proliferation result from the changes in flow and pressure, increasing the PVR. […] Approximately 50% of infants with a large, nonrestrictive VSD or patent ductus arteriosus (PDA) develop pulmonary hypertension within the first year of life, if the shunt is not surgically closed. […] Iatrogenic ES can occur after placement of a surgical systemic-to-pulmonary artery shunt, such as a Blalock-Taussig-Thomas shunt. The risk of PAH depends on the diameter and length of the shunt as well as the anatomic location.

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

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

  The most common defects leading to Eisenmenger syndrome are ASD, VSD, and PDA defects. The following 3 main processes result in the ultimate reversal of a left-to-right into a right-to-left shunt: Vasoconstriction, which is due to imbalances in pulmonary vascular tone, followed by Vascular remodeling, which is due to the proliferation of pulmonary vascular smooth muscle, and finally Thrombosis, which is caused by the increased resistance of blood flow.

• #11 KoreaMed Synapse

  https://synapse.koreamed.org/articles/1016231

  Untreated or even treated Eisenmenger syndrome is characterized by a progressive increase in the pulmonary vascular resistance leading to right ventricular failure and death. […] This classification implied irreversibility and inoperability for a subgroup of patients with congenital heart defects. […] The main vascular changes of pulmonary arterial hypertension are 1) vasoconstriction, 2) smooth-muscle cell and endothelial-cell proliferation, and 3) thrombus formation in the pulmonary vessels. […] High flow and high pressure of pulmonary artery may induce pulmonary vascular endothelial damage, leading to a loss of the endothelial barrier function. […] The pulmonary vascular resistance increases with endothelial dysfunction and this ultimately causes a decrease in left-to-right shunting.

• #12 Eisenmenger Syndrome | Calgary Guide

  https://calgaryguide.ucalgary.ca/eisenmenger-syndrome/eisenmenger-syndrome/

  Eisenmenger Syndrome: Pathogenesis and clinical findings […] Long-standing left-to-right shunt with too much pulmonary blood flow […] Structural changes occur in pulmonary arteries and arterioles to adapt to flow and pressure […] Hypertrophy of the smooth muscles (media) of pulmonary arteries and arterioles […] Thickening of the intima (innermost layer) of pulmonary arteries and arterioles […] Pressure within the right ventricle gradually […] Right ventricular pressure is equal to, or exceeds left ventricular pressure […] Shunt changes from left-to-right to right-to-left […] De-oxygenated blood originating from the right ventricle bypasses the lungs and goes into systemic circulation […] Pulmonary hypertension (mean pulmonary artery pressure at rest 25mmHg) […] Right ventricular hypertrophy (enlarging)

• #13 Eisenmenger Syndrome – StatPearls – NCBI Bookshelf

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

  Histological examination of the vasculature in Eisenmenger syndrome would resemble other forms of pulmonary hypertension. Increased proliferation of vascular smooth muscle, evidence of lung parenchymal scarring (pulmonary fibrosis), and decreased luminal caliber due to cellular proliferation in the vascular walls all are indicative of a PAH process.

• #14 Eisenmenger Syndrome: Background, Pathophysiology, Etiology

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

  The progression to Eisenmenger physiology is represented by a spectrum of morphologic changes in the capillary bed that progress from reversible lesions to irreversible ones. […] The cellular and molecular mechanisms remain fully uncharacterized, representing pathways of inflammation, cell proliferation, increase in the extracellular matrix, vasoconstriction, fibrosis, and intravascular thrombosis. […] The mechanism of pulmonary hypertension in congenital heart disease may share characteristics with other mechanisms of pulmonary hypertension, but the pathways remain complex. […] Any increase in flow, as is observed in patients with intracardiac defects and initial left-to-right shunts, results in increased pulmonary artery pressures. […] Additionally, any increase in resistance, as occurs in pulmonary vascular obstructive disease, results in a decrease in effective flow at the same pressure.

• #15 Eisenmenger syndrome: current perspectives | RRCC

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

  ET-1 (Endothelin-1) is a powerful vasoconstrictor, produced in endothelial cells, with elevated concentrations seen in patients with PAH. ET-1 plays a key role in the pathogenesis of PAH by inducing proliferation, fibrosis, and inflammation. […] 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.

• #16 Eisenmenger syndrome – physiology and evaulation of eisenmenger’s syndrome | PPT

  https://www.slideshare.net/slideshow/eisenmenger-syndrome-physiology-and-evaulation-of-eisenmengers-syndrome/266239476

  Increase in PVR Pathophysiology of Eisenmenger syndrome Left to right shunt Increased pulmonary blood flow Endothelial dysfunction and Vascular remodeling Inverted shunt (Right to left) RV hypertrophy Produces the progressive structural histologic changes Mechanical stretching in the pulmonary vasculature Increased expression of numerous mediators and receptors Increased expression of signaling molecules 1. Endothelin-1 2. NO 3. Prostacyclin 4. Thromboxan A2.

• #17 Eisenmenger’s syndrome pathophysiology – wikidoc

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

  The progression of a heart defect to Eisenmenger’s syndrome depends on: Size of left to right shunt, Severity of pulmonary vascular disease, Type of defect (it develops more frequently in uncorrected ventricular septal defect compared to atrial septal defect). […] The left-to-right shunting causes an increase in pulmonary vascular flow, which in turn leads to pulmonary artery hypertension. […] This leads to reversal of shunt and development of cyanosis. […] Further, the increased pressure causes damage to delicate capillaries, which then are replaced with scar tissue. […] The scar tissue does not contribute to oxygen transfer, therefore decreasing the useful volume of the pulmonary vasculature. […] The scar tissue also provides less flexibility than normal lung tissue, causing further increases in blood pressure.

• #18 Eisenmenger’s Effects

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

  Increased Pulmonary Vascular Resistance (PVR): Smooth muscle proliferation thickens the pulmonary arteriole walls and narrows the lumen, while extracellular matrix deposition decreases compliance. The combination of narrowed lumen and stiffened vessel walls increases PVR. […] Right Heart Strain and Shunt Reversal: Elevated PVR raises right heart pressures, resulting in right ventricular hypertrophy and ultimately reversing the shunt. […] Hypoxemia and Cyanosis: The now right-to-left shunt reduces systemic arterial oxygen saturation, a defining characteristic of Eisenmenger syndrome.

• #19 KoreaMed Synapse

  https://synapse.koreamed.org/articles/1016231

  Eventually, when the resistance becomes significantly elevated, the shunting becomes bidirectional and then it becomes right-to-left. […] The long-term follow up for those patients with elevated pulmonary vascular resistance and they have undergone closure of their defect is worse than that for the unrepaired, Eisenmenger syndrome patients with the same lesion. […] For the genetic background, 6% of patients with PAH-associated congenital heart disease were found to have bone morphogenetic protein receptor type 2 missense mutations; this frequency was comparable with that reported for patients with anorexigen-associated PAH, but it was considerably lower than that observed for patients with idiopathic or familial PAH. […] The risk factors associated with a failing heart are currently better understood. […] The degree of hypoxemia is also correlated with the NYHA functional class and it is associated with a poorer prognosis, as is an elevated right atrial mean pressure, which probably reflects ventricular failure.

• #20 Eisenmenger Syndrome – Zero To Finals

  https://zerotofinals.com/paediatrics/cardiology/eisenmengersyndrome/

  Eisenmenger syndrome occurs when blood flows from the right to the left side of the heart across a structural heart lesion, bypassing the lungs. Three underlying lesions can result in Eisenmenger syndrome: Atrial septal defect, Ventricular septal defect, Patent ductus arteriosus. Eisenmenger syndrome can develop within a few years with large lesions or in adulthood with small shunts. It can develop more rapidly during pregnancy. […] The systemic pressure (left side) is greater than the pulmonary pressure (right side). This means that blood will flow from the left side to the right side across a defect, described as a left-to-right shunt. Blood still travels to the lungs and gets oxygenated, so the patient does not become cyanotic. […] Over time, the extra blood flowing into the right side of the heart and the lungs increases the pressure in the pulmonary vessels, causing pulmonary hypertension. When the pulmonary pressure exceeds the systemic pressure, blood begins to flow from the right to the left side of the heart across the defect, described as a right-to-left shunt. Deoxygenated blood bypasses the lungs and enters the body, causing cyanosis.

• #21 Eisenmenger Syndrome Causes | Stanford Health Care

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

  If the pulmonary hypertension continues without treatment, the pressure in the right side of the heart may increase to the point that the right side pressure is greater than the left. When this occurs, blood will flow from the right side of the heart to the left (right-to-left shunt), which means that oxygen-poor blood is mixed with the oxygen-rich blood pumped out to the body from the left ventricle. […] A reversal of the shunt resulting in a right-to-left shunt causes insufficient oxygen in the blood. In addition, high pressure in the lungs causes progressive changes in the pulmonary blood vessels, which results in irreversible damage to the lining of these blood vessels. Fibrosis (the growth of scar tissue due to infection, inflammation, injury, or even healing), and thrombus (clotting) may also occur. The changes inside the lung blood vessels may be referred to as pulmonary vascular obstructive disease or secondary pulmonary arterial hypertension (PAH).

• #22 Eisenmenger syndrome | Pathology Student

  https://www.pathologystudent.com/eisenmenger-syndrome/

  So lets say you have a big ASD. In utero, blood flows through the hole from right to left (since pressure is higher on the right). When youre born, the pressure on the right drops dramatically, and flow is now from left to right. […] Over time, though, the right side of the heart gets exposed to a higher amount of pressure, since its got all this flow going through the hole from left to right. And if the right side of the heart is exposed to high pressure, then by extension, so are the lungs. […] In response to this abnormally high pressure, the vessels in the lungs constrict. If theres enough constriction, it can become very difficult to pump blood through those lungs even harder than it is to pump blood through the body. In other words, the pressure on the right can become higher than the pressure on the left! And when that happens, the flow through the ASD will be from right to left. This is called Eisenmenger syndrome, by the way, and its a dangerous situation because its hard to get enough oxygen-rich blood to your organs and tissues.

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

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

  Eisenmenger syndrome is a complication of uncorrected large intracardiac or aortic to pulmonary artery left-to-right shunts. Increased pulmonary resistance may develop over time, eventually leading to severe pulmonary hypertension, bidirectional shunting with progressively increasing right-to-left shunting. […] Once Eisenmenger syndrome occurs, the increased pulmonary vascular resistance and pulmonary hypertension are irreversible, precluding primary correction of the original defect. […] Right-to-left shunting due to Eisenmenger syndrome results in cyanosis and its complications. Systemic oxygen desaturation leads to clubbing of fingers and toes, secondary polycythemia, hyperviscosity, hemoptysis, central nervous system events (eg, brain abscess or stroke), pulmonary artery thrombosis, and sequelae of increased red blood cell turnover (eg, hyperuricemia causing gout, hyperbilirubinemia causing cholelithiasis, iron deficiency with or without anemia).

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

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

  Eisenmenger syndrome (ES) represents the most severe phenotype of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) and occurs in patients with large unrepaired shunts. […] Central cyanosis is the primary clinical manifestation leading to secondary erythrocytosis and various multiorgan complications that increase morbidity and affect quality of life. […] Although the recent use of advanced PAH therapies has substantially improved functional capacity and increased life expectancy, long-term survival remains poor. […] Impaired exercise tolerance, decreased arterial oxygen saturation, iron deficiency, pre-tricuspid shunts, arrhythmias, increased brain natriuretic peptide, echocardiographic indices of right ventricular dysfunction and hospitalisation for heart failure predict mortality.

• #25 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

  The reason for the superior survival of Eisenmenger patients compared to idiopathic pulmonary arterial hypertension patients is likely due to a better adaptation of the right ventricle to increased afterload given the slower disease progression and potentially the higher plasticity of cardiac myocytes earlier in life. […] As such, progressive dilatation or worsening of right ventricular function in Eisenmenger syndrome should be considered an ominous sign as they may herald failure of the balanced but fragile physiology. […] Risk factors of mortality in Eisenmenger patients include age, lower oxygen saturation, complexity of the underlying heart defect, signs and symptoms of heart failure, levels of brain natriuretic peptides, low six minute walk test distance, NYHA functional class, presence of Down syndrome, absence of sinus rhythm or arrhythmias, the presence of a pre-tricuspid shunt, worse right ventricular function [especially lower tricuspid annular plane systolic excursion (TAPSE)], low albumin and increased C-reactive protein.

• #26 Eisenmenger Syndrome – Zero To Finals

  https://zerotofinals.com/paediatrics/cardiology/eisenmengersyndrome/

  Cyanosis refers to the blue discolouration of skin caused by a lack of oxygen. The bone marrow responds to low oxygen saturation by producing more red blood cells and haemoglobin to increase the oxygen-carrying capacity of the blood. This results in polycythaemia, which is a high concentration of haemoglobin in the blood. Polycythaemia gives patients a plethoric complexion (reddish skin) and increases the plasma viscosity and risk of thrombus formation. […] […] […] Early management of the underlying lesion can prevent Eisenmenger syndrome. A heart-lung transplant is the only definitive treatment once Eisenmenger syndrome develops.

• #27 Eisenmenger’s syndrome pathophysiology – wikidoc

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

  The reduction in oxygen transfer reduces oxygen saturation in the blood, leading to increased production of red blood cells in an attempt to bring the oxygen saturation up. […] The excess of red blood cells is called polycythemia. […] Desperate for enough circulating oxygen, the body begins to dump immature red cells into the blood stream. […] Immature red cells are not as efficient at carrying oxygen as mature red cells, and they are less flexible, less able to easily squeeze through tiny capillaries in the lungs, and so contribute to death of pulmonary capillary beds. […] The increase in red blood cells also causes hyperviscosity syndrome. […] Eventually, due to increased resistance, pulmonary pressures may increase sufficiently to cause a reversal of blood flow, so blood begins to travel from the right side of the heart to the left side, and the body is supplied with deoxygenated blood, leading to cyanosis and resultant organ damage.

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

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

  A number of studies reported that 21–71% of ES patients had intrapulmonary thrombosis. […] The possible etiologies of PA thrombosis in patients with ES included local vascular injury related to high PA pressure, hypercoagulation secondary to cyanosis-related factor deficiencies or platelet dysfunction, red blood cell aggregation caused by sluggish flow in PA, or other embolic sources not related to the formation of thrombus in situ. […] Previous studies suggested that progressive RV failure was closely related to mortality in ES patients, and it was associated with RV remodeling and metabolic alterations that could augment glucose uptake. […] The manifestation of metabolism changes disclosed by FDG PET may be a promising target for drug development, especially on pressure-overloaded right ventricles, in the future.

• #29 Nocturnal hypoxaemia in patients with Eisenmenger syndrome: a cohort study | BMJ Open

  https://bmjopen.bmj.com/content/3/3/e002039

  The predominant obstructive pattern of respiratory abnormality affecting the smaller airways may also result in a ventilation perfusion mismatch and nocturnal hypoxaemia. […] The significance of nocturnal desaturation in Eisenmenger syndrome is not clear. […] Nocturnal hypoxaemia can also lead to cardiac arrhythmias and sudden death, which is one of the commonest modes of death in Eisenmenger syndrome. […] In clinical practice, management of Eisenmenger syndrome patients with very low resting saturations is challenging as they often have iron deficiency, which is a marker of poor prognosis. […] The utility of nocturnal oxygen therapy in selected subgroup of Eisenmenger syndrome patients, especially those with higher haematocrit values needs to be further studied in randomised controlled trials.

• #30 Eisenmenger syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5436914/

  Factors influencing the development of advanced pulmonary vasculopathy in subjects with atrial septal defect have not been completely explored. In adults, the general belief (though not proved) is that long exposure to altered flow conditions, which is largely dependent on right ventricular compliance, could alter pulmonary vascular cell biology via shear and stretch forces. However, ES features may be present in young adults with pre-tricuspid defects. […] Thus, the development of ES depends on the heart defect, its size and location (higher prevalence in post-tricuspid defects), and to factors that remain to be determined.

• #31 Eisenmenger syndrome | PPT

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

  Eisenmenger syndrome is pulmonary hypertension with a reversed central shunt. An uncorrected large left-to-right shunt causes irreversible rise in PVR leading to reversal of or bidirectional shunt flow with resultant hypoxemia. Eisenmenger syndrome is not a congenital defect, but a pathophysiologic condition. Around 12 different congenital intracardiac or extracardiac defects can cause Eisenmenger syndrome: Following 3 account for 70-80% of cases: VSD, Atrioventricular septal defect, PDA. With large shunts, the PVR develops relatively quickly, usually within the first two years of life. In patients with ASD, Eisenmenger syndrome may develop in adulthood. The course in adulthood shows that many individuals with Eisenmenger syndrome survive into adulthood with 80% survival at 10 years, 77% survival at 15 years, and 42% at 25 years after diagnosis. The causes of death in Eisenmenger patients include sudden death (30%), congestive heart failure (25%), and hemoptysis (15%). While individuals with Eisenmenger syndrome may remain relatively stable for long periods of time, it is essential to appreciate that their hemodynamic state is very delicately balanced. This balance is easily upset, often with disastrous results. It is important to be certain that the diagnosis of Eisenmenger syndrome is correct. One does not want to miss the opportunity to identify individuals who have reversibility of their pulmonary vascular disease that may enable a surgical repair of the defect. Once Eisenmenger physiology has developed, catheter or surgical interventions have a limited role in management. Surgery to repair the underlying congenital anomaly is not recommended for two reasons: the risk of surgery is exceedingly high, and those who survive the surgery have increased mortality. Pulmonary vasodilator agents such as prostacyclin analogs, endothelin antagonists, and phosphodiesterase inhibitors have been found to reduce PVR and improve functional capacity. Recommendations for medical therapy of Eisenmenger physiology include avoiding activities that carry increased risks, such as pregnancy and dehydration.

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

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

  Early identification of congenital heart disease (CHD) allows detection of the pulmonary arteriopathy in an early stage, and timely shunt closure can permanently reverse pulmonary arterial hypertension (PAH). […] In these patients, the arteriopathy is triggered by increased pulmonary blood flow resulting from a left-to-right shunt due to intracardiac or extracardiac defect. […] However, the beneficial effects of shunt closure seem lost after a certain point of no return, after which even accelerated PAH progression may occur months to years after surgery. […] Blood flow and pressure are essential triggers for pulmonary vascular remodeling in CHD. […] Long-standing overload of the pulmonary vasculature can result in the development of pulmonary vascular disease and an increase in pulmonary pressures.

• #33 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. […] Eisenmenger syndrome is high blood pressure in the blood vessels of the lungs and the right side of the heart. An untreated congenital heart defect causes high blood pressure. Symptoms tend to develop during puberty or early adulthood and may include cyanosis, exercise intolerance and heart rhythm abnormalities. Treatment is nonsurgical and focuses on easing symptoms and preventing severe heart and lung damage.

• #34 Eisenmenger Syndrome – ACHA

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

  The diagnosis of ES is based on the presence of three symptoms: A cardiac shunt or opening between the two chambers on opposite sides of the heart, pulmonary hypertension that cannot be reversed with medication, cyanosis or blueness. […] The prognosis for patients with Eisenmenger Syndrome is improving with better diagnosis and care.

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

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

  Endothelin receptor antagonists are used as first-line treatment in symptomatic patients, while phosphodiesterase-5 inhibitors may be added. […] Due to the lack of evidence, current guidelines do not provide a clear therapeutic strategy regarding treatment escalation. […] Finally, the development of a risk score is of utmost importance to guide clinical therapy.

• #36 Eisenmenger Syndrome | Norton Children’s

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

  Eisenmenger syndrome treatment focuses on: Decreasing pressure in the pulmonary artery, Improving oxygen levels in the blood, Lowering high levels of red blood cells. Treatments may include: Blood removal (phlebotomy): This may be done when high red blood cell counts cause the blood to be too thick. Saline solution is added to thin the blood. This is done when symptoms are severe and/or the red blood cell levels are too high. 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.

• #37 Eisenmenger’s Syndrome | Doctor

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

  Eisenmenger’s syndrome is defined as obstructive pulmonary vascular disease that develops as a consequence of a large pre-existing left-to-right shunt causing pulmonary artery pressures to increase and approach systemic levels, such that the direction of blood flow then becomes bi-directional or right-to-left. […] The high pulmonary vascular resistance is usually established by early childhood and can sometimes be present even from birth. […] 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. […] Various factors have been shown to be associated with mortality, such as decreased arterial oxygen saturation, functional class, impaired exercise tolerance, syncopal events, iron deficiency, presence of pre-tricuspid shunts, arrhythmias, increased (NT-pro) brain natriuretic peptide, echocardiographic variables of right ventricular dysfunction, and hospitalisation for heart failure.

• #38 KoreaMed Synapse

  https://synapse.koreamed.org/articles/1016231

  Eisenmenger syndrome is on the extreme end of the spectrum of PAH in the setting of congenital heart disease. […] Eisenmenger syndrome is a condition that involves elevation of the pulmonary arterial pressure to the systemic level, and this is caused by increased pulmonary vascular resistance with reversal or bidirectional shunting through a large intracardiac or extracardiac congenital heart defect. […] The concept of Eisenmenger syndrome is well understood, with its progressive pulmonary vascular disease and reversal of a shunt, and this represents a final pathway for several morphological cardiac defects. […] Eisenmenger syndrome can be prevented in most pediatric patients. […] Although there have been many advances in understanding of the pathophysiology of Eisenmenger syndrome and also advances in treating this disease, there is still no cure for this progressive condition once it is established, aside from lung or heart-lung transplantation, which is associated with significant morbidity and mortality.

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