{ "schemaVersion": 2, "eyebrow": "Adult Congenital Cardiology", "title": "Eisenmenger Syndrome", "summary": "Eisenmenger syndrome requires confirmation of fixed pulmonary vascular disease and shunt physiology, exclusion of reversible mimics, avoidance of destabilizing interventions, and individualized PAH-directed therapy in an adult congenital heart disease–pulmonary hypertension center.", "seoDescription": "Point-of-care diagnosis, risk assessment, PAH-directed treatment, complication management, and pregnancy counseling for Eisenmenger syndrome.", "clinicalQuestion": "How should physicians confirm, treat, and longitudinally manage Eisenmenger syndrome while avoiding harmful interventions?", "specialty": "Adult Congenital Heart Disease and Pulmonary Hypertension", "audience": "U.S. physicians and medical trainees", "tags": [ "Eisenmenger syndrome", "pulmonary arterial hypertension", "congenital heart disease", "right-to-left shunt", "cyanotic congenital heart disease", "bosentan", "pulmonary hypertension" ], "keyTakeaways": [ "Confirm Eisenmenger physiology with expert imaging and invasive hemodynamics, including a detailed shunt run, before labeling an unrepaired shunt inoperable or considering closure.[5][6]", "Exclude postcapillary pulmonary hypertension, valvular-regurgitation streaming, and RV outflow obstruction as alternative causes of bidirectional or right-to-left shunting; management differs materially for each.[5][6]", "Do not close an established Eisenmenger shunt; morbidity and mortality are prohibitively high with repair of open shunts once severe pulmonary vascular disease is present.[5]", "For symptomatic adults with LVEF >40% or reduced exercise capacity, initial PAH-directed monotherapy is recommended; drug selection should reflect defect type and be directed by ACHD-PH specialists.[9]", "Avoid routine phlebotomy and routine anticoagulation: secondary erythrocytosis supports oxygen delivery, while simultaneous thrombosis and bleeding risks require indication-specific decisions.[1][24]", "Pregnancy is contraindicated because systemic vasodilation can worsen right-to-left shunting, cyanosis, low cardiac output, and paradoxical embolism.[9][19]" ], "sections": [ { "id": "confirm-physiology", "eyebrow": "Diagnosis", "heading": "Confirm Eisenmenger physiology before treating the shunt", "intro": "The critical diagnostic decision is whether severe pulmonary vascular disease has rendered the communication nonclosable.", "paragraphs": [ "Establish the anatomic lesion, direction of shunting, ventricular function, and pulmonary hypertension phenotype with transthoracic echocardiography plus advanced imaging when anatomy is incompletely defined. CMR can quantify shunt magnitude and avoids ionizing radiation; CTA or CMR is superior to echocardiography for extracardiac vascular anatomy when pulmonary venous or complex vascular connections are relevant.[5]", "Perform cardiac catheterization at an adult congenital heart disease and pulmonary hypertension center when Eisenmenger syndrome is suspected or management will depend on operability. Obtain pulmonary and systemic pressures, pulmonary vascular resistance, ventricular filling pressures, and a detailed shunt run. A single elevated pulmonary pressure does not establish Eisenmenger syndrome without demonstrating the relevant shunt physiology and excluding competing causes.[6][22]", "Specifically investigate alternative drivers of bidirectional or right-to-left flow: postcapillary pulmonary hypertension, flow streaming from a valvular regurgitant lesion, and RV pressure overload from RV outflow tract obstruction. If RVOT obstruction, rather than irreversible pulmonary vascular disease, is the source of right-to-left shunting, relieve the RVOT obstruction rather than treating the patient as Eisenmenger syndrome.[5][6]" ], "bullets": [ "Use pulse oximetry at rest and with exertion to document cyanosis and desaturation; obtain CBC and iron studies to characterize secondary erythrocytosis and iron deficiency.[22]", "Obtain ECG and assess ventricular function because arrhythmias, progressive heart failure, and sudden cardiac death are major Eisenmenger complications.[1][6]", "Consider pulmonary function testing when lung disease or hypoxemia from a noncardiac pulmonary process could contribute to pulmonary hypertension.[22]" ], "subsections": [], "table": { "caption": "Diagnostic branches that alter the next intervention.[5][6]", "columns": [ "Finding", "Interpretation", "Next step" ], "rows": [ [ "Large intracardiac or great-artery communication with high PVR and bidirectional shunting", "Eisenmenger physiology is likely after hemodynamic confirmation.[6]", "Manage in an ACHD-PH center; do not proceed directly to defect closure.[5][6]" ], [ "Right-to-left shunt with RVOT obstruction", "The obstruction may be the treatable cause of shunting rather than irreversible pulmonary vascular disease.[5]", "Define anatomy and assess for RVOT-directed intervention.[5]" ], [ "Elevated pulmonary pressures with high left-sided filling pressures", "Postcapillary pulmonary hypertension may be contributing to shunt reversal.[6]", "Treat and characterize left-heart disease before assigning Eisenmenger-directed management.[6]" ], [ "Apparent shunt reversal with significant valvular regurgitation", "Regurgitant-flow streaming can create misleading saturation or shunt findings.[6]", "Use imaging and catheterization data to define the flow mechanism before therapy.[6]" ] ] } }, { "id": "stabilize-and-avoid-harm", "eyebrow": "Acute Care", "heading": "Stabilize without disrupting fragile shunt-dependent physiology", "intro": "Acute care should preserve systemic perfusion and avoid interventions that increase right-to-left shunting.", "paragraphs": [ "Treat new hemoptysis, syncope, decompensated heart failure, sustained arrhythmia, or rapidly worsening hypoxemia as indications for urgent evaluation in an ACHD-PH center. Eisenmenger syndrome carries high arrhythmic risk, progressive heart failure, thrombotic and bleeding diatheses, and premature mortality; acute changes should not be attributed to baseline cyanosis without reassessment.[1]", "Avoid nonindicated defect closure and avoid routine volume-depleting or hemodynamically destabilizing interventions. The right-to-left shunt can preserve systemic cardiac output at the cost of oxygen saturation; abrupt systemic vasodilation can increase shunting and reduce pulmonary flow.[20][19]", "Do not perform routine phlebotomy for secondary erythrocytosis. Increased red-cell mass is adaptive for oxygen transport; phlebotomy can worsen iron deficiency and oxygen delivery. Evaluate iron status and use replacement therapy for iron deficiency, which has been associated with improved exercise capacity and quality of life in cyanotic congenital heart disease and Eisenmenger syndrome.[24][13]" ], "bullets": [ "If pulmonary artery thrombosis is documented, balance anticoagulation against hemoptysis and hemorrhage risk rather than anticoagulating solely for Eisenmenger physiology.[24]", "Pulmonary artery thrombosis has been reported in up to 20% of affected patients; older age, pulmonary artery dilatation, and biventricular dysfunction increase risk.[24]", "When paradoxical embolism is a concern, avoid unfiltered intravascular air and ensure meticulous IV-line handling; pregnancy-related systemic vasodilation further increases shunting and paradoxical embolism risk.[19]" ], "subsections": [], "table": { "caption": "Common management pitfalls in Eisenmenger syndrome.[5][19][24]", "columns": [ "Pitfall", "Why it is harmful", "Preferred action" ], "rows": [ [ "Closing an established Eisenmenger shunt", "Surgical repair of open shunts with severe pulmonary vascular disease has prohibitively high morbidity and mortality.[5]", "Confirm physiology invasively and manage as inoperable PAH-CHD unless expert reassessment identifies a different mechanism.[5][6]" ], [ "Routine phlebotomy for elevated hematocrit", "Secondary erythrocytosis supports oxygen delivery; repeated phlebotomy can aggravate iron deficiency.[24]", "Check iron studies and treat iron deficiency rather than phlebotomizing routinely.[13][24]" ], [ "Routine anticoagulation", "Thrombosis risk coexists with hemoptysis and hemorrhage risk.[24]", "Reserve anticoagulation for individualized indications such as documented pulmonary artery thrombosis after bleeding-risk assessment.[24]" ], [ "Pregnancy", "Systemic vasodilation increases shunting, cyanosis, low cardiac output, and paradoxical embolism risk.[19]", "Advise against pregnancy and provide preconception counseling.[9][19]" ] ] } }, { "id": "pah-directed-therapy", "eyebrow": "Disease Modification", "heading": "Select PAH-directed therapy by symptoms, ventricular function, and defect type", "intro": "Treat in collaboration with clinicians experienced in both adult congenital heart disease and pulmonary hypertension.", "paragraphs": [ "For adults with Eisenmenger syndrome, LVEF greater than 40%, and either symptoms or reduced exercise capacity, current ACC/AHA guidance recommends initial monotherapy with PAH-directed therapy to improve symptoms, hemodynamics, and overall survival.[9] Expert-center treatment is important because evidence, safety, and prognostic interpretation differ from idiopathic PAH.[1]", "Bosentan is a reasonable treatment for symptomatic Eisenmenger syndrome associated with shunts other than ASD, VSD, or great-artery shunts, including patent ductus arteriosus, aortopulmonary window, complex congenital lesions, or Down syndrome.[9] Prior ESC/ERS guidance specified bosentan as initial therapy for symptomatic Eisenmenger syndrome; the BREATHE-5 trial is the pivotal randomized trial supporting its use in this population.[18][24]", "For symptomatic Eisenmenger syndrome associated with ASD, VSD, or great-artery shunts, PDE-5 inhibition with sildenafil or tadalafil is reasonable.[9] In patients who worsen clinically despite bosentan, one cohort added sildenafil 20 mg orally three times daily after right-heart catheterization; clinical status, oxygen saturation, 6-minute walk performance, laboratory testing, and hemodynamics were reassessed at 6 months.[23]" ], "bullets": [ "Use functional status, exercise capacity, oxygen saturation, ventricular function, and hemodynamics to judge treatment response; do not rely on an idiopathic-PAH risk score alone.[1][20]", "A 6-minute walk distance threshold should not be interpreted in isolation, particularly in Down syndrome, where noncardiac factors can substantially reduce distance.[18]", "Escalate persistent clinical worsening to an ACHD-PH team for reassessment of anatomy, hemodynamics, adherence, complications, and combination-therapy candidacy.[1][23]" ], "subsections": [ { "heading": "Risk assessment limitations", "paragraphs": [ "Risk models derived from idiopathic PAH should not be directly extrapolated to Eisenmenger syndrome. Patients may have better exercise performance or lower natriuretic peptide levels than expected for their pulmonary vascular resistance because an adapted hypertrophied RV and right-to-left shunt can preserve output despite severe disease.[1][20]" ], "bullets": [ "Trend WHO functional class, 6-minute walk distance, oxygen saturation, biomarkers, echocardiographic RV and ventricular parameters, and clinical events rather than using a single score as a treatment trigger.[12][20]" ] } ], "table": { "caption": "PAH-directed treatment choices supported by contemporary ACHD guidance.[9][18][23]", "columns": [ "Clinical setting", "Therapy approach", "Key monitoring decision" ], "rows": [ [ "Symptomatic or reduced exercise capacity, LVEF >40%", "Start PAH-directed monotherapy.[9]", "Assess symptoms, functional capacity, oxygenation, ventricular function, and clinical deterioration longitudinally.[9][20]" ], [ "Symptomatic ASD, VSD, or great-artery shunt", "PDE-5 inhibitor therapy, such as sildenafil or tadalafil, is reasonable.[9]", "Reassess clinical response and do not infer operability from symptomatic improvement alone.[6][9]" ], [ "Symptomatic PDA, aortopulmonary window, complex lesion, or Down syndrome", "Bosentan is reasonable.[9]", "Monitor treatment response in an ACHD-PH center.[1][9]" ], [ "Clinical worsening on bosentan", "In one cohort, sildenafil 20 mg orally three times daily was added after RHC, with reassessment at 6 months.[23]", "Use invasive reassessment and individualized bleeding, oxygenation, and hemodynamic review before escalation.[23]" ] ] } }, { "id": "longitudinal-surveillance", "eyebrow": "Follow-up", "heading": "Monitor multisystem complications and reassess when the trajectory changes", "intro": "Stable Eisenmenger physiology is fragile; new symptoms should prompt targeted evaluation rather than empiric escalation alone.", "paragraphs": [ "Follow patients in a multidisciplinary ACHD-PH center because chronic cyanosis, abnormal loading conditions, and altered systemic and pulmonary perfusion can produce multiorgan dysfunction, systolic or diastolic dysfunction, arrhythmias, and sudden cardiac death.[1][6] Serial evaluation should include oxygen saturation, CBC and iron studies, functional capacity, ECG, echocardiography, and clinical assessment for hemoptysis, thrombosis, heart failure, and arrhythmia.[1][22]", "Routine follow-up invasive hemodynamics are generally not needed after Eisenmenger syndrome has been established. Reconsider catheterization when clinical worsening, a potential therapeutic change, uncertain shunt mechanism, or possible alternative contributor to pulmonary hypertension would change management.[6]", "Interpret falling exercise capacity, escalating functional class, worsening cyanosis, new edema, syncope, palpitations, hemoptysis, or thrombotic events as potential evidence of progression or a superimposed complication. Reassess structural anatomy, ventricular function, rhythm, iron status, and pulmonary vascular hemodynamics according to the presenting change.[1][6][22]" ], "bullets": [ "Assess women of childbearing potential before conception; pregnancy should be actively discouraged because guideline recommendations identify excess maternal morbidity and mortality.[9]", "Provide contraception and coordinate any unavoidable pregnancy-related care through a high-risk cardio-obstetric and PH team; PAH-CHD recognized during pregnancy may otherwise be missed until gestation.[19]", "Do not equate preserved 6-minute walk distance or a relatively low NT-proBNP level with low risk in Eisenmenger physiology.[20]" ], "subsections": [], "table": { "caption": "Triggered reassessment during longitudinal Eisenmenger care.[1][6][22]", "columns": [ "Clinical change", "Targeted assessment", "Management implication" ], "rows": [ [ "New syncope, palpitations, or heart-failure symptoms", "ECG and ventricular assessment; evaluate for arrhythmia and systolic or diastolic dysfunction.[1][6]", "Urgent ACHD-PH review because arrhythmia and progressive heart failure carry substantial risk.[1]" ], [ "Hemoptysis or suspected thrombosis", "Evaluate bleeding source and pulmonary artery thrombosis; reassess anticoagulation risk-benefit.[24]", "Avoid routine anticoagulation; individualize treatment when thrombosis is documented.[24]" ], [ "Worsening cyanosis or exercise limitation", "Rest and exertional pulse oximetry, 6-minute walk assessment, CBC and iron studies, and cardiac imaging.[22]", "Correct iron deficiency and assess for PAH progression, ventricular deterioration, or alternative hemodynamic drivers.[13][22]" ], [ "Unexplained deterioration or planned major treatment change", "Repeat catheterization with detailed shunt evaluation when results will alter management.[6]", "Exclude postcapillary PH, valvular streaming, and RVOT obstruction before changing the Eisenmenger treatment plan.[6]" ] ] } }, { "id": "reproductive-counseling", "eyebrow": "Prevention", "heading": "Make pregnancy avoidance a routine treatment decision", "intro": "Pregnancy risk counseling is a core component of longitudinal Eisenmenger management.", "paragraphs": [ "Advise adults with Eisenmenger syndrome against pregnancy. ACC/AHA guidance classifies this as harm prevention because pregnancy carries excess maternal morbidity and mortality.[9]", "Explain the hemodynamic mechanism during preconception counseling: pregnancy-related systemic vasodilation increases right-to-left shunting and decreases pulmonary blood flow, leading to worsening cyanosis, reduced cardiac output, and paradoxical embolism risk.[19] Involve congenital cardiology, pulmonary hypertension, and high-risk obstetric specialists early if pregnancy occurs.[19]" ], "bullets": [ "Do not defer risk counseling until pregnancy is established; PAH-CHD may first be recognized during pregnancy, complicating assessment and timing of invasive testing.[19]" ], "subsections": [], "table": { "caption": "", "columns": [], "rows": [] } } ], "faq": [], "references": [ { "number": 1, "title": "Eisenmenger Syndrome: JACC State-of-the-Art Review", "detail": "www.jacc.org", "url": "https://www.jacc.org/doi/10.1016/j.jacc.2022.01.022", "authors": "www.jacc.org", "host": "www.jacc.org" }, { "number": 2, "title": "Eisenmenger Syndrome: A Clinical Perspective in a New Therapeutic Era of Pulmonary Arterial Hypertension", "detail": "www.jacc.org", "url": "https://www.jacc.org/doi/10.1016/j.jacc.2008.11.025", "authors": "www.jacc.org", "host": "www.jacc.org" }, { "number": 3, "title": "Sotatercept in Congenital Heart Disease–Associated ...", "detail": "www.jacc.org", "url": "https://www.jacc.org/doi/10.1016/j.jaccas.2026.108814", "authors": "www.jacc.org", "host": "www.jacc.org" }, { "number": 4, "title": "Clinical Evaluation and Management of Pulmonary ...", "detail": "www.ahajournals.org", "url": "https://www.ahajournals.org/doi/full/10.1161/CIRCULATIONAHA.114.006976?doi=10.1161%2FCIRCULATIONAHA.114.006976", "authors": "www.ahajournals.org", "host": "www.ahajournals.org" }, { "number": 5, "title": "2018 AHA/ACC Guideline for the Management of Adults ...", "detail": "www.ahajournals.org", "url": "https://www.ahajournals.org/doi/10.1161/CIR.0000000000000603", "authors": "www.ahajournals.org", "host": "www.ahajournals.org" }, { "number": 6, "title": "2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the ...", "detail": "www.ahajournals.org", "url": "https://www.ahajournals.org/doi/10.1161/CIR.0000000000001402", "authors": "www.ahajournals.org", "host": "www.ahajournals.org" }, { "number": 7, "title": "The Eisenmenger Syndrome in Adults", "detail": "annals.org", "url": "http://annals.org/aim/article/711368/eisenmenger-syndrome-adults", "authors": "annals.org", "host": "annals.org" }, { "number": 8, "title": "Echocardiographic Predictors of Outcome in Eisenmenger ...", "detail": "www.ahajournals.org", "url": "https://www.ahajournals.org/doi/10.1161/circulationaha.112.091421", "authors": "www.ahajournals.org", "host": "www.ahajournals.org" }, { "number": 9, "title": "2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines", "detail": "www.jacc.org", "url": "https://www.jacc.org/doi/10.1016/j.jacc.2025.09.006", "authors": "www.jacc.org", "host": "www.jacc.org" }, { "number": 10, "title": "Risk stratification for adult patients with pulmonary arterial ...", "detail": "academic.oup.com", "url": "https://academic.oup.com/eurjhf/advance-article/doi/10.1093/ejhf/xuag059/8512130", "authors": "academic.oup.com", "host": "academic.oup.com" }, { "number": 11, "title": "Biventricular longitudinal strain analysis using ...", "detail": "www.sciencedirect.com", "url": "https://www.sciencedirect.com/science/article/pii/S1097664724011438", "authors": "www.sciencedirect.com", "host": "www.sciencedirect.com" }, { "number": 12, "title": "Review Risk stratification in Eisenmenger syndrome", "detail": "www.sciencedirect.com", "url": "https://www.sciencedirect.com/science/article/pii/S1875213625000944", "authors": "www.sciencedirect.com", "host": "www.sciencedirect.com" }, { "number": 13, "title": "Eisenmenger Syndrome: A Multisystem Disorder—Do Not Destabilize the Balanced but Fragile Physiology - ScienceDirect", "detail": "www.sciencedirect.com", "url": "https://www.sciencedirect.com/science/article/abs/pii/S0828282X19312991", "authors": "www.sciencedirect.com", "host": "www.sciencedirect.com" }, { "number": 14, "title": "Sildenafil therapy for pulmonary arterial hypertension associated with atrial septal defects - ScienceDirect", "detail": "www.sciencedirect.com", "url": "https://www.sciencedirect.com/science/article/abs/pii/S0167527306007844", "authors": "www.sciencedirect.com", "host": "www.sciencedirect.com" }, { "number": 15, "title": "Guidelines on diagnosis and treatment of pulmonary ...", "detail": "academic.oup.com", "url": "https://academic.oup.com/eurheartj/article-pdf/25/24/2243/17887941/2243.pdf", "authors": "academic.oup.com", "host": "academic.oup.com" }, { "number": 16, "title": "2015 ESC/ERS Guidelines for the diagnosis and treatment of ...", "detail": "academic.oup.com", "url": "https://academic.oup.com/eurheartj/article-pdf/37/1/67/23492708/ehv317.pdf", "authors": "academic.oup.com", "host": "academic.oup.com" }, { "number": 17, "title": "portopulmonary hypertension and pulmonary arterial ...", "detail": "academic.oup.com", "url": "https://academic.oup.com/eurheartjsupp/article/21/Supplement_K/K37/5678710", "authors": "academic.oup.com", "host": "academic.oup.com" }, { "number": 18, "title": "Update on Eisenmenger syndrome – Review of pathophysiology and recent progress in risk assessment and management", "detail": "pmc.ncbi.nlm.nih.gov", "url": "https://pmc.ncbi.nlm.nih.gov/articles/PMC11658362", "authors": "pmc.ncbi.nlm.nih.gov", "host": "pmc.ncbi.nlm.nih.gov" }, { "number": 19, "title": "Pregnancy in Congenital Heart Disease, Complicated by Pulmonary Arterial Hypertension—A Challenging Issue for the Pregnant Woman, the Foetus, and Healthcare Professionals - PMC", "detail": "pmc.ncbi.nlm.nih.gov", "url": "https://pmc.ncbi.nlm.nih.gov/articles/PMC9033133", "authors": "pmc.ncbi.nlm.nih.gov", "host": "pmc.ncbi.nlm.nih.gov" }, { "number": 20, "title": "Congenital Heart Disease and Pulmonary Arterial Hypertension: Current Perspectives - PMC", "detail": "pmc.ncbi.nlm.nih.gov", "url": "https://pmc.ncbi.nlm.nih.gov/articles/PMC13036531", "authors": "pmc.ncbi.nlm.nih.gov", "host": "pmc.ncbi.nlm.nih.gov" }, { "number": 21, "title": "The Adult Patient with Eisenmenger Syndrome: A Medical Update After Dana Point Part I: Epidemiology, Clinical Aspects and Diagnostic Options", "detail": "pmc.ncbi.nlm.nih.gov", "url": "https://pmc.ncbi.nlm.nih.gov/articles/PMC3083816", "authors": "pmc.ncbi.nlm.nih.gov", "host": "pmc.ncbi.nlm.nih.gov" }, { "number": 22, "title": "Eisenmenger Syndrome - StatPearls - NCBI Bookshelf", "detail": "www.ncbi.nlm.nih.gov", "url": "https://www.ncbi.nlm.nih.gov/books/NBK507800", "authors": "www.ncbi.nlm.nih.gov", "host": "www.ncbi.nlm.nih.gov" }, { "number": 23, "title": "Bosentan-sildenafil association in patients with congenital heart disease-related pulmonary arterial hypertension and Eisenmenger physiology - PubMed", "detail": "www.ncbi.nlm.nih.gov", "url": "https://www.ncbi.nlm.nih.gov/pubmed/21081251", "authors": "www.ncbi.nlm.nih.gov", "host": "www.ncbi.nlm.nih.gov" }, { "number": 24, "title": "An Approach to Pulmonary Arterial Hypertension in the Adult Patient With Congenital Heart Disease - American College of Cardiology", "detail": "www.acc.org", "url": "https://www.acc.org/latest-in-cardiology/articles/2014/07/18/12/56/an-approach-to-pulmonary-arterial-hypertension-in-the-adult-patient-with-congenital-heart-disease", "authors": "www.acc.org", "host": "www.acc.org" } ], "editorialNote": "Prepared from cited clinical literature using Astra's research workflow. Verify recommendations against current guidance and patient-specific factors.", "citations": [ { "number": 1, "title": "Eisenmenger Syndrome: JACC State-of-the-Art Review", "detail": "www.jacc.org", "url": "https://www.jacc.org/doi/10.1016/j.jacc.2022.01.022", "authors": "www.jacc.org", "host": "www.jacc.org", "snippet": "ES may be considered a paradigm for CHD, where clinical practice has been transformed as a result of recent evidence delineating pathophysiological and adaptive mechanisms, safety, and efficacy of advanced PAH therapies, some derived from randomized clinical trials (RCTs).9-13 However, there are sti", "score": 0.7515939 }, { "number": 2, "title": "Eisenmenger Syndrome: A Clinical Perspective in a New Therapeutic Era of Pulmonary Arterial Hypertension", "detail": "www.jacc.org", "url": "https://www.jacc.org/doi/10.1016/j.jacc.2008.11.025", "authors": "www.jacc.org", "host": "www.jacc.org", "snippet": "Eisenmenger syndrome (ES), the most advanced form of pulmonary arterial hypertension (PAH) associated with congenital heart disease, is a devastating condition that has a considerable impact on patients' lives. Patients who develop ES typically exhibit 1 or more of a range of cardiac defects, includ", "score": 0.6847074 }, { "number": 3, "title": "Sotatercept in Congenital Heart Disease–Associated ...", "detail": "www.jacc.org", "url": "https://www.jacc.org/doi/10.1016/j.jaccas.2026.108814", "authors": "www.jacc.org", "host": "www.jacc.org", "snippet": "by GP Leung · Cited by 1 — Update on Eisenmenger syndrome - review of pathophysiology and recent progress in risk assessment and management.", "score": 0.4302197 }, { "number": 4, "title": "Clinical Evaluation and Management of Pulmonary ...", "detail": "www.ahajournals.org", "url": "https://www.ahajournals.org/doi/full/10.1161/CIRCULATIONAHA.114.006976?doi=10.1161%2FCIRCULATIONAHA.114.006976", "authors": "www.ahajournals.org", "host": "www.ahajournals.org", "snippet": "Catheterization revealed mildly elevated PVR. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. adult patients", "score": 0.5073975 }, { "number": 5, "title": "2018 AHA/ACC Guideline for the Management of Adults ...", "detail": "www.ahajournals.org", "url": "https://www.ahajournals.org/doi/10.1161/CIR.0000000000000603", "authors": "www.ahajournals.org", "host": "www.ahajournals.org", "snippet": "will not be beneficial if the source of right-to-left shunting is RVOT obstruction; rather, alleviation of the RVOT obstruction is the necessary treatment. Accurate diagnosis of Eisenmenger syndrome and exclusion of other potential contributors to right-to-left shunting or pulmonary hypertension by ", "score": 0.49626526 }, { "number": 6, "title": "2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the ...", "detail": "www.ahajournals.org", "url": "https://www.ahajournals.org/doi/10.1161/CIR.0000000000001402", "authors": "www.ahajournals.org", "host": "www.ahajournals.org", "snippet": "Bidirectional shunting through large septal defects or large communications between the great arteries associated with severely elevated PVR may be diagnosed as Eisenmenger syndrome. Accurate diagnosis of PAH, along with a detailed shunt run, should be obtained in all patients with Eisenmenger syndr", "score": 0.48294178 }, { "number": 7, "title": "The Eisenmenger Syndrome in Adults", "detail": "annals.org", "url": "http://annals.org/aim/article/711368/eisenmenger-syndrome-adults", "authors": "annals.org", "host": "annals.org", "snippet": "by W Vongpatanasin · 1998 · Cited by 424 — The Eisenmenger syndrome is characterized by elevated pulmonary vascular resistance and right-to-left shunting of blood through a systemic-to-", "score": 0.40967003 }, { "number": 8, "title": "Echocardiographic Predictors of Outcome in Eisenmenger ...", "detail": "www.ahajournals.org", "url": "https://www.ahajournals.org/doi/10.1161/circulationaha.112.091421", "authors": "www.ahajournals.org", "host": "www.ahajournals.org", "snippet": "by P Moceri · 2012 · Cited by 153 — Echocardiography is widely used in the assessment of patients with various types of PAH because it provides accurate information on cardiac", "score": 0.3373132 }, { "number": 9, "title": "2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines", "detail": "www.jacc.org", "url": "https://www.jacc.org/doi/10.1016/j.jacc.2025.09.006", "authors": "www.jacc.org", "host": "www.jacc.org", "snippet": "| New | Section 4.4.5.1. HLHS/Norwood Repair is new. |\n| New | 4.4.6. Eisenmenger Syndrome | N/A | COR 3 Harm: Adults with Eisenmenger syndrome should be advised against pregnancy to decrease the risk associated with excess maternal morbidity and mortality. |\n| Revised | 4.4.6. Eisenmenger Syndrome ", "score": 0.52802294 }, { "number": 10, "title": "Risk stratification for adult patients with pulmonary arterial ...", "detail": "academic.oup.com", "url": "https://academic.oup.com/eurjhf/advance-article/doi/10.1093/ejhf/xuag059/8512130", "authors": "academic.oup.com", "host": "academic.oup.com", "snippet": "Current guideline-based risk stratification models have limitations when applied to patients with PAH-CHD, particularly those with Eisenmenger", "score": 0.77261686 }, { "number": 11, "title": "Biventricular longitudinal strain analysis using ...", "detail": "www.sciencedirect.com", "url": "https://www.sciencedirect.com/science/article/pii/S1097664724011438", "authors": "www.sciencedirect.com", "host": "www.sciencedirect.com", "snippet": "Eisenmenger syndrome (ES) is a severe complication of group 1 pulmonary arterial hypertension (PAH) secondary to uncorrected congenital heart disease (CHD),", "score": 0.30613327 }, { "number": 12, "title": "Review Risk stratification in Eisenmenger syndrome", "detail": "www.sciencedirect.com", "url": "https://www.sciencedirect.com/science/article/pii/S1875213625000944", "authors": "www.sciencedirect.com", "host": "www.sciencedirect.com", "snippet": "by E Valdeolmillos · 2025 · Cited by 1 — Several risk factors have been identified as independent predictors in Eisenmenger syndrome, including the 6-minute walk distance, echocardiographic markers", "score": 0.26092392 }, { "number": 13, "title": "Eisenmenger Syndrome: A Multisystem Disorder—Do Not Destabilize the Balanced but Fragile Physiology - ScienceDirect", "detail": "www.sciencedirect.com", "url": "https://www.sciencedirect.com/science/article/abs/pii/S0828282X19312991", "authors": "www.sciencedirect.com", "host": "www.sciencedirect.com", "snippet": "Title: Eisenmenger Syndrome: A Multisystem Disorder—Do Not Destabilize the Balanced but Fragile Physiology - ScienceDirect\nEisenmenger syndrome is the most severe and extreme phenotype of pulmonary arterial hypertension associated with congenital heart disease. Advanced pulmonary arterial hypertensi", "score": 0.6219319 }, { "number": 14, "title": "Sildenafil therapy for pulmonary arterial hypertension associated with atrial septal defects - ScienceDirect", "detail": "www.sciencedirect.com", "url": "https://www.sciencedirect.com/science/article/abs/pii/S0167527306007844", "authors": "www.sciencedirect.com", "host": "www.sciencedirect.com", "snippet": "Title: Sildenafil therapy for pulmonary arterial hypertension associated with atrial septal defects - ScienceDirect\n# Sildenafil therapy for pulmonary arterial hypertension associated with atrial septal defects. This extended case series reviews our experience with sildenafil treatment in three pati", "score": 0.49685127 }, { "number": 15, "title": "Guidelines on diagnosis and treatment of pulmonary ...", "detail": "academic.oup.com", "url": "https://academic.oup.com/eurheartj/article-pdf/25/24/2243/17887941/2243.pdf", "authors": "academic.oup.com", "host": "academic.oup.com", "snippet": "by N Galiè · 2004 · Cited by 1584 — pregnancy be avoided or termi- nated in women with cyanotic congenital heart disease, PH, and Eisenmenger syndrome. controlled randomised trials.213", "score": 0.559411 }, { "number": 16, "title": "2015 ESC/ERS Guidelines for the diagnosis and treatment of ...", "detail": "academic.oup.com", "url": "https://academic.oup.com/eurheartj/article-pdf/37/1/67/23492708/ehv317.pdf", "authors": "academic.oup.com", "host": "academic.oup.com", "snippet": "by N Galiè · 2016 · Cited by 8055 — Long-term outcome of double-lung and heart2lung functional capacity in Eisenmenger syndrome: pulmonary arterial hypertension due to congenital", "score": 0.49509352 }, { "number": 17, "title": "portopulmonary hypertension and pulmonary arterial ...", "detail": "academic.oup.com", "url": "https://academic.oup.com/eurheartjsupp/article/21/Supplement_K/K37/5678710", "authors": "academic.oup.com", "host": "academic.oup.com", "snippet": "by L Savale · 2019 · Cited by 27 — Improved survival among patients with Eisenmenger syndrome receiving advanced therapy for pulmonary arterial hypertension . Circulation 2010", "score": 0.4600226 }, { "number": 18, "title": "Update on Eisenmenger syndrome – Review of pathophysiology and recent progress in risk assessment and management", "detail": "pmc.ncbi.nlm.nih.gov", "url": "https://pmc.ncbi.nlm.nih.gov/articles/PMC11658362", "authors": "pmc.ncbi.nlm.nih.gov", "host": "pmc.ncbi.nlm.nih.gov", "snippet": "Prior ESC/ERS guidelines recommended starting ERA monotherapy in patients with a 6MWD <450 m, and then adding a second agent if there is no improvement . This recommendation, presumably, would have been intended to be interpreted in the larger clinical context since 6MWD is impacted by musculoskelet", "score": 0.81097376 }, { "number": 19, "title": "Pregnancy in Congenital Heart Disease, Complicated by Pulmonary Arterial Hypertension—A Challenging Issue for the Pregnant Woman, the Foetus, and Healthcare Professionals - PMC", "detail": "pmc.ncbi.nlm.nih.gov", "url": "https://pmc.ncbi.nlm.nih.gov/articles/PMC9033133", "authors": "pmc.ncbi.nlm.nih.gov", "host": "pmc.ncbi.nlm.nih.gov", "snippet": "Patients with Eisenmenger’s syndrome have a higher risk than those with repaired CHD or PAH associated with left-to-right shunts [24][30][31][32][33][34][35]. In pregnant ES patients, systemic vasodilation leads to an increase in right and left shunts and a decrease in pulmonary flow, resulting in higher ", "score": 0.76427686 }, { "number": 20, "title": "Congenital Heart Disease and Pulmonary Arterial Hypertension: Current Perspectives - PMC", "detail": "pmc.ncbi.nlm.nih.gov", "url": "https://pmc.ncbi.nlm.nih.gov/articles/PMC13036531", "authors": "pmc.ncbi.nlm.nih.gov", "host": "pmc.ncbi.nlm.nih.gov", "snippet": "(3). Acute vasoreactivity test (AVT): This test is ideally performed\nwith inhaled nitric oxide (iNO) to determine whether high PVR is fixed or\nreversible.\n\n### 4.3 Risk Stratification: A Unique Challenge in PAH-CHD\n\nOnce a diagnosis is established and the disease is considered inoperable (ES) or\nhig", "score": 0.7409441 }, { "number": 21, "title": "The Adult Patient with Eisenmenger Syndrome: A Medical Update After Dana Point Part I: Epidemiology, Clinical Aspects and Diagnostic Options", "detail": "pmc.ncbi.nlm.nih.gov", "url": "https://pmc.ncbi.nlm.nih.gov/articles/PMC3083816", "authors": "pmc.ncbi.nlm.nih.gov", "host": "pmc.ncbi.nlm.nih.gov", "snippet": "## , also being reflected in the ESC-Guidelines for diagnosis and treatment of pulmonary hypertension , which have significant impact on the management strategies of PAH in congenital heart disease (Table 1).\n\nOverall, the classification system of pulmonary hypertension can be summarized as:\n\n○ WHO ", "score": 0.7368747 }, { "number": 22, "title": "Eisenmenger Syndrome - StatPearls - NCBI Bookshelf", "detail": "www.ncbi.nlm.nih.gov", "url": "https://www.ncbi.nlm.nih.gov/books/NBK507800", "authors": "www.ncbi.nlm.nih.gov", "host": "www.ncbi.nlm.nih.gov", "snippet": "Eisenmenger syndrome 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) ", "score": 0.57322514 }, { "number": 23, "title": "Bosentan-sildenafil association in patients with congenital heart disease-related pulmonary arterial hypertension and Eisenmenger physiology - PubMed", "detail": "www.ncbi.nlm.nih.gov", "url": "https://www.ncbi.nlm.nih.gov/pubmed/21081251", "authors": "www.ncbi.nlm.nih.gov", "host": "www.ncbi.nlm.nih.gov", "snippet": "Title: Bosentan-sildenafil association in patients with congenital heart disease-related pulmonary arterial hypertension and Eisenmenger physiology - PubMed\nAn official website of the United States government. **The .gov means it’s official.**. Federal government websites often end in .gov or .mil. ", "score": 0.36500442 }, { "number": 24, "title": "An Approach to Pulmonary Arterial Hypertension in the Adult Patient With Congenital Heart Disease - American College of Cardiology", "detail": "www.acc.org", "url": "https://www.acc.org/latest-in-cardiology/articles/2014/07/18/12/56/an-approach-to-pulmonary-arterial-hypertension-in-the-adult-patient-with-congenital-heart-disease", "authors": "www.acc.org", "host": "www.acc.org", "snippet": "Reflecting available data, current European Society of Cardiology guidelines focus on patients with Eisenmenger's syndrome and recommend that treatment with the endothelin receptor antagonist Bosentan is initiated in Eisenmenger's syndrome patients in functional class III (class I, level of evidence", "score": 0.65940565 } ], "publishedAt": "2026-08-24T17:13:34.122119+00:00", "updatedAt": "2026-08-24T17:13:34.122119+00:00", "readingMinutes": 5, "slug": "eisenmenger-syndrome" }