Raras
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Síndrome Eisenmenger
ORPHA:97214CID-10 · I27.2CID-11 · BB01.0DOENÇA RARA

A Síndrome de Eisenmenger (SE) é uma forma de hipertensão arterial pulmonar (HAP), que significa ter pressão alta nas artérias dos pulmões. Ela está associada a problemas cardíacos congênitos (defeitos no coração que a pessoa nasce) que não foram corrigidos por cirurgia. A síndrome se caracteriza por malformações no coração que fazem com que o sangue comece a fluir de forma anormal – na direção oposta ou em ambos os sentidos – através de uma comunicação (uma espécie de "atalho" ou "abertura") dentro do coração ou entre os grandes vasos sanguíneos (normalmente entre a aorta e a artéria pulmonar). Essa situação leva ao desenvolvimento da pressão alta nas artérias pulmonares (HAP).

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Introdução

O que você precisa saber de cara

📋

A Síndrome de Eisenmenger (SE) é uma forma de hipertensão arterial pulmonar (HAP), que significa ter pressão alta nas artérias dos pulmões. Ela está associada a problemas cardíacos congênitos (defeitos no coração que a pessoa nasce) que não foram corrigidos por cirurgia. A síndrome se caracteriza por malformações no coração que fazem com que o sangue comece a fluir de forma anormal – na direção oposta ou em ambos os sentidos – através de uma comunicação (uma espécie de "atalho" ou "abertura") dentro do coração ou entre os grandes vasos sanguíneos (normalmente entre a aorta e a artéria pulmonar). Essa situação leva ao desenvolvimento da pressão alta nas artérias pulmonares (HAP).

Pesquisas ativas
3 ensaios
26 total registrados no ClinicalTrials.gov
Publicações científicas
963 artigos
Último publicado: 2026 Jun

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-9 / 1 000 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.2
Belgium
Início
Adolescent
+ adult, childhood
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: I27.2
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

❤️
Coração
15 sintomas
🫃
Digestivo
4 sintomas
🩸
Sangue
3 sintomas
🫁
Pulmão
3 sintomas
🧠
Neurológico
1 sintomas
🫘
Rins
1 sintomas

+ 30 sintomas em outras categorias

Características mais comuns

90%prev.
Morfologia anormal do coração
Muito frequente (99-80%)
90%prev.
Intolerância ao exercício
Muito frequente (99-80%)
90%prev.
Hipertensão arterial pulmonar
Muito frequente (99-80%)
55%prev.
Dispneia de esforço
Frequente (79-30%)
55%prev.
Palpitações
Frequente (79-30%)
55%prev.
Taquicardia supraventricular
Frequente (79-30%)
58sintomas
Muito frequente (3)
Frequente (10)
Ocasional (31)
Muito raro (14)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 58 características clínicas mais associadas, ordenadas por frequência.

Morfologia anormal do coraçãoAbnormal heart morphology
Muito frequente (99-80%)90%
Intolerância ao exercícioExercise intolerance
Muito frequente (99-80%)90%
Hipertensão arterial pulmonarPulmonary arterial hypertension
Muito frequente (99-80%)90%
Dispneia de esforçoExertional dyspnea
Frequente (79-30%)55%
PalpitaçõesPalpitations
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico963PubMed
Últimos 10 anos200publicações
Pico202553 papers
Linha do tempo
2026Hoje · 2026🧪 1996Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

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Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
Aprovado2
3Fase 31
2Fase 22
1Fase 11
·Pré-clínico7
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 13 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Síndrome Eisenmenger

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

2 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

26 ensaios clínicos encontrados, 3 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

🥉Melhor nível de evidência: Relato de caso
Timeline de publicações
500 papers (10 anos)
#1

Protocol for an open-label, randomised, controlled trial to evaluate the efficacy and safety of sotatercept add-on therapy compared with pulmonary vasodilator-based standard of care for pulmonary vasodilator-resistant pulmonary arterial hypertension associated with unrepaired congenital shunts (atrial septal defect, ventricular septal defect or patent ductus arteriosus), including Eisenmenger syndrome: the SuMILE trial.

BMJ open2026 Mar 12

Eisenmenger syndrome and pulmonary arterial hypertension (PAH) due to unrepaired congenital shunts, including atrial septal defect (ASD), ventricular septal defect (VSD) and patent ductus arteriosus (PDA), remain life-threatening conditions despite advances in congenital heart disease (CHD) care. In this population, vasodilator-based therapies effective in other forms of PAH have shown limited benefit, and no disease-modifying treatment has been established. Sotatercept, an activin-signalling inhibitor, improved exercise capacity and haemodynamics in phase 2/3 PAH trials; however, patients with unrepaired CHD, including Eisenmenger syndrome, were excluded. The efficacy and safety of sotatercept in this population remain unknown. The SuMILE trial is a prospective, exploratory, multicentre, open-label, randomised, controlled trial conducted at 11 Japanese tertiary centres. 36 adults with vasodilator-resistant PAH due to unrepaired ASD, VSD or PDA, including Eisenmenger syndrome, will be randomised 2:1 to sotatercept add-on therapy plus vasodilator-based PAH therapy versus vasodilator-based PAH therapy alone. Sotatercept will be administered subcutaneously every 3 weeks in accordance with label-approved dose-modification rules for haemoglobin and platelet changes. The primary endpoint is the change in 6-min walk distance from baseline to week 24. Key clinical events will be independently adjudicated. Secondary endpoints include all-cause mortality or lung transplantation; pulmonary hypertension-related hospitalisation or initiation of parenteral prostacyclin and changes in WHO functional class, N-terminal pro-brain natriuretic peptide and emPHasis-10. Exploratory endpoints include genotype, right heart catheterisation and cardiac MRI parameters. The primary analysis will use ANCOVA, adjusting for baseline 6-min walk distance and randomisation stratum in the intention-to-treat population. The protocol has been reviewed and approved by the certified central review board (Kyushu University Hospital Clinical Ethics Review Board) and participating institutions. Written informed consent will be obtained from all participants. Findings will be disseminated through peer-reviewed journals, scientific conferences and trial registries. Japan Registry of Clinical Trials no. 1071250069; ClinicalTrials.gov NCT07356778. Protocol version and date: V.1.3; 23 October 2025.

#2

Sotatercept in Patients With Eisenmenger Syndrome.

Pulmonary circulation2026 Jan

Eisenmenger syndrome represents a complex and severe form of pulmonary arterial hypertension (PAH) associated with congenital heart disease. Sotatercept, a first-in-class activin-signaling inhibitor, offers a novel therapeutic approach in the treatment of PAH, and has emerged as a promising therapeutic agent. However, patients with Eisenmenger syndrome were not included in its pivotal trials. Therefore, data regarding its effect in this patient cohort is lacking. We present two cases of patients with Eisenmenger syndrome treated with sotatercept. Sotatercept improved the subjective and objective exercise capacity in both patients without any significant adverse side effects.

#3

A Dissecting Pulmonary Aneurysm Complicating Eisenmenger Syndrome.

European heart journal. Cardiovascular Imaging2026 Mar 23
#4

Unrepaired Truncus Arteriosus Type 1 With Eisenmenger Syndrome and Recurrent Embolic Strokes: An Adult Case Report.

The American journal of case reports2026 Mar 11

BACKGROUND In adults, non-corrected truncus arteriosus type 1 with Eisenmenger syndrome is a rare congenital heart defect characterized by a single large artery from the heart that directs blood flow to the lungs, leading to irreversible pulmonary hypertension and a reversal of blood flow. This report describes the case of a 44-year-old woman with congenital Eisenmenger syndrome and unrepaired truncus arteriosus type 1 presenting with recurrent thromboembolic cerebral infarcts. CASE REPORT A 44-year-old woman presented with a 2-month history of recurrent neurological deficits, including aphasia, right-sided weakness, and vertigo. Magnetic resonance imaging (MRI) of the brain confirmed acute embolic infarcts, while computed tomography (CT) angiography ruled out large-vessel occlusion. Cardiac evaluation revealed a 15-mm perimembranous ventricular septal defect with a bidirectional shunt and a severely calcified truncal valve with moderate regurgitation. Imaging confirmed truncus arteriosus type 1, with the pulmonary arteries originating from the ascending aorta. The right ventricular systolic pressure was estimated at 114 mmHg, and baseline oxygen saturation (SpO₂) was 70-80%. Due to irreversible pulmonary vascular disease, she was not a candidate for surgical repair. A conservative management strategy was adopted, including dual antiplatelet therapy, sildenafil, macitentan, bisoprolol, and oxygen therapy. At the 6-month follow-up, the patient remained neurologically stable with her SpO₂ improved to 85-88%. CONCLUSIONS This case highlights the exceptional natural course of uncorrected truncus arteriosus in adulthood. It underscores that for patients with Eisenmenger physiology where surgical repair is not feasible, a multidisciplinary approach focusing on tailored antithrombotic and pulmonary vasodilator therapies is essential for stroke prevention and clinical stability.

#5

Risk stratification for adult patients with pulmonary arterial hypertension associated with congenital heart disease. A scientific statement of the ESC Working Group on Pulmonary Circulation & Right Ventricular Function, the ESC Working Group on Adult Congenital Heart Disease, and the Association of Cardiovascular Nursing & Allied Professions of the ESC.

European journal of heart failure2026 Mar 09

For patients with pulmonary arterial hypertension (PAH), current guidelines recommend a 3- and 4-strata risk stratification model at baseline and follow-up, respectively. Risk stratification models in PAH are mainly derived from idiopathic PAH cohorts and are not automatically applicable to all patients with PAH associated with congenital heart disease (CHD), especially in those with Eisenmenger syndrome, given the differences in pathophysiology and clinical phenotype. Additional features such as shunt location, complexity of CHD, degree of cyanosis, iron deficiency, syndromic co-morbidity and biomarkers, other than brain natriuretic peptide may play an important role in the prognostication of these patients. This scientific statement aims to discuss in detail individual prognosticators and propose a comprehensive model of risk stratification for patients with PAH-CHD, mainly those with Eisenmenger syndrome.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC528 artigos no totalmostrando 200

2026

A Dissecting Pulmonary Aneurysm Complicating Eisenmenger Syndrome.

European heart journal. Cardiovascular Imaging
2026

Protocol for an open-label, randomised, controlled trial to evaluate the efficacy and safety of sotatercept add-on therapy compared with pulmonary vasodilator-based standard of care for pulmonary vasodilator-resistant pulmonary arterial hypertension associated with unrepaired congenital shunts (atrial septal defect, ventricular septal defect or patent ductus arteriosus), including Eisenmenger syndrome: the SuMILE trial.

BMJ open
2026

Sotatercept in Patients With Eisenmenger Syndrome.

Pulmonary circulation
2026

Unrepaired Truncus Arteriosus Type 1 With Eisenmenger Syndrome and Recurrent Embolic Strokes: An Adult Case Report.

The American journal of case reports
2026

Risk stratification for adult patients with pulmonary arterial hypertension associated with congenital heart disease. A scientific statement of the ESC Working Group on Pulmonary Circulation & Right Ventricular Function, the ESC Working Group on Adult Congenital Heart Disease, and the Association of Cardiovascular Nursing & Allied Professions of the ESC.

European journal of heart failure
2026

Treatment of Brain Abscess in Patient with Eisenmenger Syndrome.

Journal of Korean Neurosurgical Society
2025

Maternal and Perinatal Outcome in Women with Congenital Heart Disease: An Observational Study.

JNMA; journal of the Nepal Medical Association
2026

Heart-Lung transplantation: Meeting allocation challenges in France.

JHLT open
2026

Lung Retransplantation after Heart-lung Transplantation: A Case Report.

Transplantation proceedings
2026

Management of Acute Cholecystitis in a Patient With Eisenmenger Syndrome and Abdominal Heterotaxy: A Case Report.

The American journal of case reports
2026

Pneumonia or something more? Eisenmenger syndrome in a resource-limited setting.

Tropical doctor
2025

[A Case of Bilateral Breast Mastectomy Performed under Local Anesthesia with Ultrasound Guided Nerve Blocks].

Gan to kagaku ryoho. Cancer &amp; chemotherapy
2026

Extracorporeal membrane oxygenation for pulmonary arterial hypertension complicating pregnancy: case series and literature review.

BMC pregnancy and childbirth
2026

Treat-and-Repair Strategy for Atrial Septal Defect with Eisenmenger Syndrome due to Pulmonary Arterial Hypertension: A Case Report.

Internal medicine (Tokyo, Japan)
2025

Standardized mortality ratio in adults with congenital heart disease.

European heart journal open
2025

Influence of gestational history on neural tube defects and Eisenmenger syndrome: associations with intellectual disability and visual impairment in children and adults - a systematic review.

Frontiers in pediatrics
2025

Dyslipidemia Patterns in Adults with Congenital Heart Disease: Focus on HDL Cholesterol.

Journal of clinical medicine
2025

Case Report: Concurrent atrial and ventricular septal defect in a young Sphynx cat.

Frontiers in veterinary science
2025

Bridge to recovery: A case of V-V ECMO following V-A ECMO in Eisenmenger syndrome.

Perfusion
2025

Acute Decompensated Heart Failure in a Woman With Eisenmenger Syndrome: A Case Report.

Cureus
2025

Long-term outcome of patients with Eisenmenger syndrome receiving pulmonary arterial hypertension-targeted therapy.

Open heart
2025

Predictive Insights From the 6-Minute Walk Test in the Eisenmenger Syndrome.

JACC. Advances
2025

Atrial Septal Defects: From Embryology to Pediatric Pulmonary Hypertension.

Journal of clinical medicine
2025

Prevalence of Pulmonary Arterial Thrombosis in Adults With Eisenmenger Syndrome.

Pulmonary circulation
2025

Secondary hyperparathyroidism in adult congenital heart disease: the CHD-HYPER-study.

European heart journal open
2025

Treatment options for children with pulmonary arterial hypertension associated with congenital heart disease.

Expert review of respiratory medicine
2025

Unrepaired Dextro-Transposition of the Great Arteries: Eisenmenger Syndrome with Giant Pulmonary Artery Aneurysm.

Radiology
2025

[Initial care and stabilization of ACHD patients : Teamwork improves outcomes].

Medizinische Klinik, Intensivmedizin und Notfallmedizin
2025

Specific risk factors for heart-lung transplantation.

JHLT open
2025

Long-term outcomes of Eisenmenger syndrome in the era of targeted pulmonary vasodilator therapy.

The National medical journal of India
2025

Pulmonary arterial compliance as a long-term prognostic indicator in pulmonary arterial hypertension associated with adult congenital heart disease: results from a National multicenter prospective registry.

BMC cardiovascular disorders
2025

A Rare Eisenmenger Syndrome Presentation in the Intensive Care.

Clinical case reports
2025

Cardiopulmonary Exercise Testing in Congenital Heart Disease: A Never-Ending Story from Paediatrics to Adult Life.

Children (Basel, Switzerland)
2025

Continuous spinal anesthesia in a case of Eisenmenger syndrome undergoing TURBT- A case report.

Saudi journal of anaesthesia
2025

A Rare Case of Chronic Thromboembolic Pulmonary Hypertension in an Elderly With Atrial Septal Defect and Eisenmenger Syndrome.

Cureus
2025

Analysis of Clinical Characteristics of 54 Dead Pregnant Patients With Pulmonary Hypertension.

Pulmonary circulation
2025

Critical Care of the Adult With Congenital Heart Disease.

JACC. Advances
2025

Large Patent Ductus Arteriosus in an Adult With Severe Pulmonary Hypertension and Polycythemia: PDA With Severe Pulmonary Hypertension.

JACC. Case reports
2025

Pregnancy in patients with pulmonary hypertension: a systematic review and meta-analysis with meta-regression.

Journal of thoracic disease
2025

Fatal Congenital Heart Disease in a Postpartum Woman.

Diagnostics (Basel, Switzerland)
2025

Refractory hypoxaemia in the presence of two common conditions: a case report.

European heart journal. Case reports
2025

Extended Use of Central Veno-Arterial Extracorporeal Membrane Oxygenation in Lung Transplantation for Patients with Pulmonary Arterial Hypertension.

European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
2025

A Giant Pulmonary Artery Aneurysm in a Young Male With Eisenmenger Syndrome.

Clinical case reports
2025

Successful interventional closure of patent ductus arteriosus in three pediatric cases with congenital heart disease and severe pulmonary hypertension: a case series and literature review.

Frontiers in cardiovascular medicine
2025

Active Surveillance of Papillary Thyroid Cancer-A Feasibility Experience from a Tertiary Care Centre.

Indian journal of surgical oncology
2025

Is transplantation an option for adult Eisenmenger syndrome?Is the grass on the other side of fence greener?

Indian heart journal
2025

Maternal outcomes among pregnant women with shunt-related congenital heart disease-associated pulmonary hypertension: a retrospective study.

BMC anesthesiology
2025

Perioperative Risk in Adults with Congenital Heart Disease Undergoing Non-Cardiac Surgery: Challenges and Tailored Strategies.

Journal of clinical medicine
2025

Common shunt lesions with pulmonary hypertension-who will benefit from surgery?

Indian journal of thoracic and cardiovascular surgery
2025

Group D of pulmonary arterial hypertension and its relationship to congenital heart disease: Is there a non-invasive way to predict the unpredictable?

Global cardiology science &amp; practice
2025

Novel Use of MitraClip for Treatment of Severe Aortic Regurgitation.

Journal of the Society for Cardiovascular Angiography &amp; Interventions
2025

Meta-analysis of targeted drugs for pulmonary hypertension to improve exercise tolerance and associated factors in eisenmenger syndrome.

Journal of cardiothoracic surgery
2025

Utilization of the flow ratio measured by echocardiography (FRecho) compared to the flow ratio by right heart catheterization (FRrhc) for detecting Eisenmenger syndrome in uncorrected acyanotic adult congenital heart disease (ACHD).

BMC medical imaging
2024

Interventional Management of a Rare Case of Complex Congenital Heart Disease in an Adult Patient: A Case Report.

The journal of Tehran Heart Center
2025

Isolated Pulmonary Arterial Thrombosis in Patient with Eisenmenger Syndrome Treated with Catheter-directed Thrombolysis: A Case Report and Literature Review.

Acta medica Indonesiana
2025

A 19-year-old male with truncus arteriosus type I: a rare case report from Syria.

Annals of medicine and surgery (2012)
2025

Risk stratification in Eisenmenger syndrome.

Archives of cardiovascular diseases
2025

High Altitude May Protect Against the Early Development of Irreversible Pulmonary Hypertension in Patients With Congenital Heart Disease.

Pulmonary circulation
2025

Challenging Perioperative Management of a MEN2A Syndrome Patient Complicated by Eisenmenger Syndrome.

Turkish journal of anaesthesiology and reanimation
2025

Massive Hemoptysis in a Patient With Eisenmenger Syndrome, Polysplenia and Transverse Liver.

Clinical case reports
2025

Advances in Atrial Septal Defect Closure: Managing Pulmonary Hypertension and Expanding Treatment Criteria Applicability.

Cardiology in review
2025

Replacement of the systemic tricuspid valve in a patient with corrected transposition of the great arteries, ventricular septal defect, and Eisenmenger syndrome.

Indian journal of thoracic and cardiovascular surgery
2025

[Adult congenital heart disease misdiagnosed as Eisenmenger syndrome: a case report].

Zhonghua xin xue guan bing za zhi
2025

Author Correction: Contemporary survival outcomes of congenital systemic-to-pulmonary shunt in children with borderline pulmonary vascular resistant index compared to Eisenmenger syndrome.

Scientific reports
2024

Aortopulmonary Window in Adult Patient Operated on With Echocardiography Assessment.

Annals of thoracic surgery short reports
2024

Safety, tolerability, and efficacy of an in-class combination therapy switch from bosentan plus sildenafil to ambrisentan plus tadalafil in children with pulmonary arterial hypertension.

Pulmonary circulation
2024

Eisenmenger syndrome during pregnancy: A true anesthetic challenge.

Medical journal, Armed Forces India
2024

Intraoperative Hemodynamic Instability in a Patient With Ebstein's Anomaly Complicated With Eisenmenger Syndrome.

Case reports in cardiology
2024

Pulmonary arterial hypertension with left to right shunts: When to treat and/or close?

International journal of cardiology. Congenital heart disease
2024

Update on Eisenmenger syndrome - Review of pathophysiology and recent progress in risk assessment and management.

International journal of cardiology. Congenital heart disease
2024

Multimodality Evaluation of Turner Syndrome With Right Ventricular Dilation and Partial Anomalous Pulmonary Venous Return.

Cureus
2025

Survival prospects of pulmonary arterial hypertension associated with congenital heart disease: Insights from the HOPE registry.

International journal of cardiology
2024

Survival comparison in adults with congenital systemic to pulmonary shunt and borderline elevated pulmonary vascular resistance versus Eisenmenger syndrome.

Scientific reports
2024

Macitentan in Pulmonary Arterial Hypertension Due to Congenital Heart Disease (CHD-PAH): Real-World Evidence from the OPUS/OrPHeUS Studies.

Cardiology and therapy
2024

Operative strategies and outcomes for patients with severe pulmonary artery hypertension and intestinal obstruction: case series from single institution-sharing lessons to improve surgical outcomes.

Journal of surgical case reports
2024

[Implantation of leadless pacemaker in complex congenital heart disease with Eisenmenger syndrome and high-degree atrioventricular block: a case report].

Zhonghua xin xue guan bing za zhi
2024

Case report: Eisenmenger syndrome in a dog with ventricular septal defect: long term management and complications.

Frontiers in veterinary science
2024

Pulmonary hypertension in adults with congenital heart defects (ACHDs) in light of the 2022 ESC PAH guidelines-part II: supportive therapy, special situations (pregnancy, contraception, non-cardiac surgery), targeted pharmacotherapy, organ transplantation, special management (shunt lesion, left ventricular disease, univentricular hearts), interventions, intensive care, ACHD follow-up, future perspective.

Cardiovascular diagnosis and therapy
2024

Pulmonary hypertension in adults with congenital heart defects (ACHDs)-in light of the 2022 ESC PAH guidelines-part I: definition, epidemiology, classification, diagnostics, genetics, risk stratification and follow-up, gender aspects.

Cardiovascular diagnosis and therapy
2024

Biventricular longitudinal strain analysis using cardiovascular magnetic resonance feature-tracking: Prognostic value in Eisenmenger syndrome.

Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance
2024

Evaluation of Outcomes of Pregnancy in Women with Eisenmenger Syndrome: Is There Any Prognostic Criterion?

Turk Kardiyoloji Dernegi arsivi : Turk Kardiyoloji Derneginin yayin organidir
2024

Bronchial Atresia with Eisenmenger Syndrome in a Complex Congenital Heart Disease-A Rare Coexistence: A Case Report.

The Journal of the Association of Physicians of India
2024

Unexpected Origins: A Case Report of Escherichia coli-Linked Cerebral Abscess in an Adult.

Cureus
2024

Swyer-James-MacLeod Syndrome in a Patient with Eisenmenger Syndrome.

Turk Kardiyoloji Dernegi arsivi : Turk Kardiyoloji Derneginin yayin organidir
2024

Cutting the Gordian knot of diuretic resistance using continuous ultrafiltration in a Holt-Oram patient with decompensated heart failure and Eisenmenger syndrome: a case report.

European heart journal. Case reports
2024

Eisenmenger Syndrome Combined with Pulmonary Artery Aneurysm and Dissection.

Radiology
2024

Erythrocytosis in congenital heart defects: hints for diagnosis and therapy from a clinical case.

Frontiers in medicine
2025

Giant right pulmonary artery aneurysm in Eisenmenger syndrome.

Revista espanola de cardiologia (English ed.)
2024

Septal Defects: Unveiling Sex-Based Disparities and Screening Challenges for Timely Intervention Through a Case Report and Systematic Literature Review.

Cureus
2024

Pseudo-subarachnoid haemorrhage: An unusual presentation of hyperviscosity syndrome.

The National medical journal of India
2024

Contemporary survival outcomes of congenital systemic-to-pulmonary shunt in children with borderline pulmonary vascular resistant index compared to Eisenmenger syndrome.

Scientific reports
2024

Eisenmenger syndrome complicated by the presence of an aneurysm of the pulmonary trunk and compression of the left main coronary artery.

Postepy w kardiologii interwencyjnej = Advances in interventional cardiology
2024

Atrial Septal Defect (ASD) Repair Unveiling an Unusual Conduction Conundrum: A Wenckebach Case Report.

Cureus
2024

Perioperative Management of an Obstetric Patient with Eisenmenger Syndrome and Pre-Eclampsia Using Milrinone.

Annals of cardiac anaesthesia
2024

Right ventricular fibrosis in adults with uncorrected secundum atrial septal defect and pulmonary hypertension: a cardiovascular magnetic resonance study with late gadolinium enhancement, native T1 and extracellular volume.

Frontiers in cardiovascular medicine
2024

Successful Cardiac Surgical Management in a Trisomy 21 Child After Long-Term Hospitalization Associated With Bronchopneumonia and Hepatitis C Virus Seropositivity: A Case Report.

Cureus
2024

How severity and classification of pulmonary hypertension affect pregnancy outcomes: a systematic review and timeline.

International journal of obstetric anesthesia
2023

Survival and outcomes of isolated neonatal ventricular septal defects: A population-based study from a middle-income country.

Annals of pediatric cardiology
2024

Complex Cardiovascular Morbidities in Prader-Willi Syndrome: A Multidisciplinary Approach.

Cureus
2024

Development and Validation of a Nomogram of Persistent Pulmonary Hypertension in Adult Pretricuspid Shunts After Correction.

Journal of the American Heart Association
2024

The Anesthetic Complexity of Eisenmenger Syndrome: A Clinical Case.

Cureus
2024

Medical Therapy and Monitoring in Adult Congenital Heart Disease Heart Failure.

Heart failure clinics
2024

Cyanotic Nephropathy in an Adult Patient with Eisenmenger Syndrome: A Case Report and Literature Review.

Kidney &amp; blood pressure research
2024

Successful intraoperative management of laparoscopic hysterectomy in a patient with Eisenmenger syndrome: a case report.

JA clinical reports
2024

Management of Unrepaired Tetralogy of Fallot in an 86-Year-Old Patient Undergoing Transcatheter Aortic Valve Replacement.

Cureus
2024

Acquired Cardiovascular Diseases in Patients with Pulmonary Hypertension Due to Congenital Heart Disease: A Case Report.

Medicina (Kaunas, Lithuania)
2024

The Unrepaired Tetralogy of Fallot: A Tale of Delayed Presentation and Limited Access to Care.

Cureus
2024

Application of a modified clinical classification for pulmonary arterial hypertension associated with congenital heart disease in children: emphasis on atrial septal defects and transposition of the great arteries. An analysis from the TOPP registry.

Frontiers in cardiovascular medicine
2024

Remimazolam in perioperative management of Eisenmenger syndrome: a case report.

JA clinical reports
2024

Noncardiac surgery in Eisenmenger syndrome.

Polish archives of internal medicine
2024

Thirty years of surgical management of pediatric pulmonary hypertension: Mid-term outcomes following reverse Potts shunt and transplantation.

The Journal of thoracic and cardiovascular surgery
2023

A case of atrial septal defect with right-to-left shunting without pulmonary hypertension.

Journal of cardiology cases
2024

Prognosis factors in heart-lung transplantation: 30 years experience in a reference center.

Cirugia espanola
2024

Triple vasodilator therapy in pulmonary arterial hypertension associated with congenital heart disease.

Heart (British Cardiac Society)
2023

Clinical features and pregnancy outcomes in women with aortopulmonary window defect: case series.

European heart journal. Case reports
2023

Intravascular Ultrasound-Guided Stent Implantation in the Left Main Coronary Artery Extrinsic Compression by Pulmonary Artery Aneurysm Due to Eisenmenger Syndrome.

Turk Kardiyoloji Dernegi arsivi : Turk Kardiyoloji Derneginin yayin organidir
2023

Eisenmenger Syndrome: A Revisit of a Hidden but Catastrophic Disease.

West African journal of medicine
2023

Evaluating the role of serum uric acid in the risk stratification and therapeutic response of patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD).

Frontiers in pharmacology
2023

Metabolomics in Pulmonary Hypertension-A Useful Tool to Provide Insights into the Dark Side of a Tricky Pathology.

International journal of molecular sciences
2023

Outcomes of pregnancy in women with different types of pulmonary hypertension.

BMC cardiovascular disorders
2023

Pseudoaneurysm after aortopulmonary window repair and bilateral lung transplantation for eisenmenger syndrome: a case report.

Journal of cardiothoracic surgery
2024

One-minute sit-to-stand test in patients with pulmonary arterial hypertension associated with congenital heart disease: A single-center prospective study.

Hellenic journal of cardiology : HJC = Hellenike kardiologike epitheorese
2023

Pulmonary vascular disease as a complication of pediatric congenital heart diseases.

Translational pediatrics
2023

Heart-lung transplantation in Eisenmenger syndrome with pulmonary embolisms.

The American journal of the medical sciences
2023

Risk Assessment Tool Implementation in Congenital Heart Disease-Associated Pulmonary Arterial Hypertension.

Anatolian journal of cardiology
2023

Fatal Association of Eisenmenger Syndrome and Severe Preeclampsia.

Cureus
2023

De novo progressive dural arteriovenous fistula with putamen hemorrhage associated with long-term Down syndrome: A case report and literature review.

Radiology case reports
2023

Eisenmenger syndrome combined with pulmonary artery dissecting aneurysm.

European heart journal
2023

Management and outcomes of pulmonary artery hypertension and Eisenmenger syndrome during pregnancy: a prospective observational cohort study.

BJOG : an international journal of obstetrics and gynaecology
2023

Atrial fibrillation ablation in a single atrium with inferior vena cava interruption.

Annals of noninvasive electrocardiology : the official journal of the International Society for Holter and Noninvasive Electrocardiology, Inc
2023

Characteristics, treatments and survival of pulmonary arterial hypertension associated with congenital heart disease in China: Insights from a national multicenter prospective registry.

The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation
2023

Common congenital heart problems in acute and intensive care.

European heart journal. Acute cardiovascular care
2023

Advanced heart failure in adult congenital heart disease: the role of renal dysfunction in management and outcomes.

European journal of preventive cardiology
2023

Outcome of Down patients with repaired versus unrepaired atrioventricular septal defect.

International journal of cardiology. Congenital heart disease
2023

Impact of current targeted drug therapy on the prognosis of Eisenmenger syndrome: A large-scale retrospective analysis.

Hellenic journal of cardiology : HJC = Hellenike kardiologike epitheorese
2022

Successful delivery in a woman with ventricular septal defect complicated with Eisenmenger syndrome: case report.

The Pan African medical journal
2023

An Unusual Presentation of Eisenmenger Syndrome in a Middle-Aged Woman Without Known Cardiac History.

Cureus
2024

Univentricular hearts not undergoing Fontan: the type of palliation matters.

Revista espanola de cardiologia (English ed.)
2023

A Caseating Tricuspid Mass in an Adult With Eisenmenger Syndrome Secondary to Aorto-Pulmonary Window.

JACC. Case reports
2023

Congenital unilateral absence of right pulmonary artery with VSD and wide aortopulmonary window in an adult patient: a case report.

Annals of medicine and surgery (2012)
2023

Successful Eisenmenger syndrome-targeted drug therapy in pregnant women: a case series and literature review.

BJOG : an international journal of obstetrics and gynaecology
2023

[Nacimiento anómalo de arteria pulmonar derecha en un paciente joven con síndrome de eisenmenger].

Archivos de cardiologia de Mexico
2023

Case report: Thoughts on two cases of total anomalous pulmonary venous connection complicated with pulmonary artery hypertension.

Frontiers in cardiovascular medicine
2024

Cyanotic Congenital Heart Disease in Pregnancy: A Review of Pathophysiology and Management.

Cardiology in review
2023

Outcomes of heart-lung transplantation in Eisenmenger syndrome compared to primary pulmonary hypertension.

Asian cardiovascular &amp; thoracic annals
2023

Multidisciplinary approach for the management of term pregnancy complicated by Eisenmenger syndrome.

Journal of Zhejiang University. Science. B
2023

Pregnancy in Eisenmenger syndrome: a case series from a tertiary care hospital of Northern India.

Cardiology in the young
2023

Characteristics of exercise intolerance in different subgroups of pulmonary arterial hypertension associated with congenital heart disease.

International journal of cardiology
2022

Novel Hybrid Treatment for Pulmonary Arterial Hypertension with or without Eisenmenger Syndrome: Double Lung Transplantation with Simultaneous Endovascular or Classic Surgical Closure of the Patent Ductus Arteriosus (PDA).

Journal of cardiovascular development and disease
2023

Pregnancy outcomes of women with Eisenmenger syndrome: A single-center study.

International journal of cardiology
2023

Undiagnosed ventricular septal defect with resultant Eisenmenger syndrome presenting with diplopia.

The American journal of emergency medicine
2023

Survival of patients with congenital ventricular septal defect.

European heart journal
2023

Partial anomalous pulmonary venous return in adults: Insight into pulmonary hypertension.

International journal of cardiology. Congenital heart disease
2022

Detection and evaluation of myocardial fibrosis in Eisenmenger syndrome using cardiovascular magnetic resonance late gadolinium enhancement and T1 mapping.

Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance
2022

Long-term course of pulmonary arterial hypertension in adults with congenital heart disease under targeted therapy: a retrospective analysis of a single tertiary center.

Cardiovascular diagnosis and therapy
2022

Evolving Indications for Heart-Lung Transplant in Spain.

Transplantation proceedings
2021

[Evidence base for specific pulmonary vasodilators in adults with congenital heart disease].

Terapevticheskii arkhiv
2023

Leadless pacemaker implantation in Eisenmenger syndrome.

Revista espanola de cardiologia (English ed.)
2023

Risk of pulmonary artery dissection in adults with congenital heart disease.

International journal of cardiology
2022

Monitored anesthesia care for craniotomy in a patient with Eisenmenger syndrome: A case report.

World journal of clinical cases
2022

Targeted Therapies in Patients with Pulmonary Arterial Hypertension Due to Congenital Heart Disease.

Current vascular pharmacology
2022

Cerebral abscess as a neurosurgical emergency in Eisenmenger syndrome: illustrative case.

Journal of neurosurgery. Case lessons
2022

Successful Control of Heavy Menstrual Bleeding Using High-Intensity Focused Ultrasound Ablation of Fibroid Uterus in a Woman With Eisenmenger Syndrome.

Cureus
2022

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

Children (Basel, Switzerland)
2022

Status of adult outpatients with congenital heart disease in Japan: The Japanese Network of Cardiovascular Departments for Adult Congenital Heart Disease Registry.

Journal of cardiology
2022

Successful Bronchial Artery Embolization after Stabilization with Nitric Oxide for the Treatment of Haemoptysis in a Patient with Eisenmenger Syndrome.

Turk Kardiyoloji Dernegi arsivi : Turk Kardiyoloji Derneginin yayin organidir
2022

Case Report: Artifactual Hypoglycemia: A Condition That Should Not Be Forgotten.

Frontiers in endocrinology
2022

Transcatheter closure of secundum atrial septal defect using Cocoon septal occluder: immediate and long-term results.

The Egyptian heart journal : (EHJ) : official bulletin of the Egyptian Society of Cardiology
2022

Thrombosis of aneurysmal pulmonary arteries in patent ductus arteriosus with Eisenmenger syndrome.

Annals of pediatric cardiology
2022

Late presentation of aortopulmonary window: a contemporary series.

Indian journal of thoracic and cardiovascular surgery
2022

Eisenmenger Syndrome in Case of Extended Goldenhar Complex Treated With Heart-Lung Transplantation.

JACC. Case reports
2023

A case series describing the multidisciplinary management of pulmonary arterial hypertension in pregnancy: Time for optimism.

The Australian &amp; New Zealand journal of obstetrics &amp; gynaecology
2022

Challenges in managing a critically ill patient with decompensated Eisenmenger syndrome.

BMJ case reports
2022

Long-Term Survival of Adult Patients With Atrial Septal Defect With Regards to Defect Closure and Pulmonary Hypertension.

Frontiers in cardiovascular medicine
2022

Successful bronchial artery embolization in a patient with Eisenmenger syndrome and recurrent haemoptysis.

International journal of cardiology. Congenital heart disease
2022

Eisenmenger Syndrome Among Children with Unrepaired Congenital Heart Defects in Yunnan, China.

Pediatric cardiology
2022

Maternal and fetal outcomes of pregnant women with pulmonary arterial hypertension associated with congenital heart disease in Beijing, China: A retrospective study.

Pulmonary circulation
2022

Spontaneous hyphema from iris microhemangioma in Eisenmenger syndrome.

American journal of ophthalmology case reports
2023

Inferior sinus venosus defect and anomalous hepatic venous return to the coronary sinus leading to an Eisenmenger syndrome.

Cardiology in the young
2022

Pregnancy in Congenital Heart Disease, Complicated by Pulmonary Arterial Hypertension-A Challenging Issue for the Pregnant Woman, the Foetus, and Healthcare Professionals.

Medicina (Kaunas, Lithuania)
2022

Evaluation of Liver Stiffness After Atrial Septal Defect Closure.

Ultrasound quarterly
2022

Pheochromocytoma and multiple paragangliomas associated with Eisenmenger syndrome: An autopsy case.

Pathology international
2022

Transcatheter leadless permanent pacemaker in complex congenital heart disease with interrupted inferior vena cava: A challenging implantation.

Indian pacing and electrophysiology journal
2022

Role of high-flow nasal oxygen therapy in COVID-19 pneumonia with Eisenmenger syndrome: A case report.

International journal of cardiology. Congenital heart disease
2022

A Case of Multifocal Extramedullary and Non-Hepatosplenic Extramedullary Hematopoiesis in a 43-Year-Old Man with a History of Congenital Eisenmenger Syndrome.

The American journal of case reports
2022

Eisenmenger Syndrome: JACC State-of-the-Art Review.

Journal of the American College of Cardiology
2022

Pulmonary Hypertension in Adult Congenital Heart Disease in Asia: A Distinctive Feature of Complex Congenital Heart Disease.

Journal of the American Heart Association
2022

Eisenmenger syndrome with left main compression syndrome: a case report.

BMC cardiovascular disorders
2022

Central retinal artery occlusion with cilioretinal sparing in a patient with Eisenmenger syndrome.

BMJ case reports
2022

Anaesthetic management in neurosurgery in a patient with Eisenmenger syndrome and Down syndrome. A case report.

Revista espanola de anestesiologia y reanimacion
2022

Haemoptysis in Pulmonary Arterial Hypertension Associated with Congenital Heart Disease: Insights on Pathophysiology, Diagnosis and Management.

Journal of clinical medicine
2022

Fruitful Pregnancy Outcome in a Case of Eisenmenger Syndrome With Severe Pulmonary Hypertension: A Rare Case Report.

Cureus
2022

Catheter Ablation of Atrioventricular Nodal Reentrant Tachycardia in Patients With Congenital Heart Disease.

Circulation. Arrhythmia and electrophysiology
2021

Medical treatment of pulmonary hypertension in adults with congenital heart disease: updated and extended results from the International COMPERA-CHD Registry.

Cardiovascular diagnosis and therapy
2022

Exercise ventilatory reserve predicts survival in adult congenital heart disease associated pulmonary arterial hypertension with Eisenmenger physiology.

International journal of cardiology. Congenital heart disease
2022

Current Situation and Prognostic Evolution of Combined Heart-lung Transplantation in a European Union Country.

International journal of organ transplantation medicine
2021

The use of pulmonary arterial hypertension therapies in Eisenmenger syndrome.

Expert review of cardiovascular therapy
2022

Venovenous Extracorporeal Membrane Oxygenation During Bilateral Lung Transplantation for a Patient With Eisenmenger Syndrome Complicated With Giant Pulmonary Artery Aneurysm, Azygos Continuation, and Central Airway Obstruction.

Journal of cardiothoracic and vascular anesthesia
2021

Angiography-guided mid/high septal implantation of ventricular leads in patients with congenital heart disease.

Journal of arrhythmia
2022

Effect of oxygen therapy on exercise performance in patients with cyanotic congenital heart disease: Randomized-controlled trial.

International journal of cardiology
Ver todos os 528 no EuropePMC

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Doenças relacionadas

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Protocol for an open-label, randomised, controlled trial to evaluate the efficacy and safety of sotatercept add-on therapy compared with pulmonary vasodilator-based standard of care for pulmonary vasodilator-resistant pulmonary arterial hypertension associated with unrepaired congenital shunts (atrial septal defect, ventricular septal defect or patent ductus arteriosus), including Eisenmenger syndrome: the SuMILE trial.
    BMJ open· 2026· PMID 41819579mais citado
  2. Sotatercept in Patients With Eisenmenger Syndrome.
    Pulmonary circulation· 2026· PMID 41815805mais citado
  3. A Dissecting Pulmonary Aneurysm Complicating Eisenmenger Syndrome.
    European heart journal. Cardiovascular Imaging· 2026· PMID 41871419mais citado
  4. Unrepaired Truncus Arteriosus Type 1 With Eisenmenger Syndrome and Recurrent Embolic Strokes: An Adult Case Report.
    The American journal of case reports· 2026· PMID 41808274mais citado
  5. Risk stratification for adult patients with pulmonary arterial hypertension associated with congenital heart disease. A scientific statement of the ESC Working Group on Pulmonary Circulation &amp; Right Ventricular Function, the ESC Working Group on Adult Congenital Heart Disease, and the Association of Cardiovascular Nursing &amp; Allied Professions of the ESC.
    European journal of heart failure· 2026· PMID 41800797mais citado
  6. Early Results of Sotatercept Treatment in Patients With Severe Pulmonary Hypertension and a Potts Shunt.
    Pulm Circ· 2026· PMID 41971413recente
  7. Congenital Heart Disease and Pulmonary Arterial Hypertension: Current Perspectives.
    Rev Cardiovasc Med· 2026· PMID 41923748recente
  8. Multimodality imaging of patent ductus arteriosus with severe pulmonary hypertension following 3 pregnancies.
    Radiol Case Rep· 2026· PMID 41909114recente
  9. From Cyanosis to Extensive Systemic-to-Pulmonary Collaterals Formation: A Case With an Iatrogenic Connection of the Inferior Vena Cava to the Left Atrium.
    Clin Case Rep· 2025· PMID 41909097recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:97214(Orphanet)
  2. MONDO:0019944(MONDO)
  3. GARD:6323(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Artigo Wikipedia(Wikipedia)
  6. Q572695(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Síndrome Eisenmenger
Compêndio · Raras BR

Síndrome Eisenmenger

ORPHA:97214 · MONDO:0019944
Prevalência
1-9 / 1 000 000
Herança
Not applicable
CID-10
I27.2 · Outra hipertensão pulmonar secundária
CID-11
Ensaios
3 ativos
Início
Adolescent, Adult, Childhood
Prevalência
0.2 (Belgium)
MedGen
UMLS
C0013743
EuropePMC
Wikidata
Wikipedia
Papers 10a
Evidência
🥉 Relato de caso
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