ArticleEuropean heart journal2016
Current therapy and outcome of Eisenmenger syndrome: data of the German National Register for congenital heart defects.
Article in European heart journal, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 45 papers.
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Who cites it
45 citing papers in PubMed, 108 citations in OpenAlex.
- Evaluation of Macitentan in Patients With Eisenmenger Syndrome.Circulation · 2019Trial
- Eisenmenger Syndrome After Delayed Atrial Septal Defect Closure at High Altitude: A Case Report.The American journal of case reports · 2026Article
- Registry-based estimation of cardiac event-free survival in congenital heart disease complicated by pulmonary hypertension: A nationwide registry study from Japan.International journal of cardiology. Congenital heart disease · 2026Article
- Article
- Congenital Heart Disease and Pulmonary Arterial Hypertension: Current Perspectives.Reviews in cardiovascular medicine · 2026Review
- Emergency cesarean section in pregnant women with severe pulmonary hypertension: the potential role of extracorporeal life support.Ulusal travma ve acil cerrahi dergisi = Turkish journal of trauma & emergency surgery : TJTES · 2026Article
- Sotatercept in Patients With Eisenmenger Syndrome.Pulmonary circulation · 2026Article
- Long-term outcome of patients with Eisenmenger syndrome receiving pulmonary arterial hypertension-targeted therapy.Open heart · 2025Article
- Atrial Septal Defects: From Embryology to Pediatric Pulmonary Hypertension.Journal of clinical medicine · 2025Review
- Prevalence of Pulmonary Arterial Thrombosis in Adults With Eisenmenger Syndrome.Pulmonary circulation · 2025Article
- Update on Eisenmenger syndrome - Review of pathophysiology and recent progress in risk assessment and management.International journal of cardiology. Congenital heart disease · 2024Review
- Medical Therapy for Heart Failure in Adult Congenital Heart Disease Patients.Structural heart : the journal of the Heart Team · 2024Review
- The Right Heart in Congenital Heart Disease.Current heart failure reports · 2023Review
- Integrated cardiothoracic team approach for complex lung transplantation procedures in Japan-current status and future direction.Journal of thoracic disease · 2023Review
- Are Guideline-recommended Risk Classification Schemes in Pulmonary Hypertension Adequately Robust to Guide the Real-world Setting?Balkan medical journal · 2023Article
- The Consistent Effectiveness and Safety of Macitentan Therapies Across Idiopathic and Congenital Heart Disease-Associated PulmonaryArterial Hypertension: A Single-Center Experience.Anatolian journal of cardiology · 2022Article
- Long-Term Study on Therapeutic Strategy for Treatment of Eisenmenger Syndrome Patients: A Case Series Study.Children (Basel, Switzerland) · 2022Article
- Cerebral abscess as a neurosurgical emergency in Eisenmenger syndrome: illustrative case.Journal of neurosurgery. Case lessons · 2022Article
- Successful bronchial artery embolization in a patient with Eisenmenger syndrome and recurrent haemoptysis.International journal of cardiology. Congenital heart disease · 2022Article
- A novelExperimental and therapeutic medicine · 2022Article
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Authors and funding
9 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
aimsWe aimed to assess the contemporary outcome of Eisenmenger syndrome (ES), delineate the use of disease targeting therapies (DTT) in these patients and to investigate the effect of treatment on outcome in the community. METHODS AND
resultsPatients with ES were systematically identified from the German National Register for Congenital Heart Defects. Data on underlying diagnosis, medical therapy, and survival were collected. The impact of DTT on survival was assessed using time-dependant Cox analysis. Overall, 153 ES patients were included (mean age 34.0 ± 13.3 years, 46% females). Of these, 88 (57.5%) were treated with at least one DTT (76.1% Bosentan, 20.5% Sildenafil) while 17.6% were on dual DTT. In addition, 24.8% of patients received digoxin, 10.5% angiotensin-converting enzyme-inhibitors/angiotensin receptor blockers, and 17.6% β-blockers. Moreover, 17.6% of patients were treated with oral anticoagulants, while 23.5% of patients received Aspirin. The survival rate at 1, 5, and 10 years of follow-up was only 92, 75, and 57% in the entire cohort, and was even worse in treatment naive ES patients (survival rate 86, 60, and 34% at 1, 5, and 10 years). Use of DTT was independently associated with a better survival (hazard ratio 0.42, P= 0.015).
conclusionThis study illustrates the alarmingly poor survival prospects of Eisenmenger patients by community-based data even in the current era with advanced DTT and in a country with a wealthy health system. Treatment naive ES patients had especially high mortality rates approaching 60-70% at 10 years of follow-up. Treatment with DTT was associated with better survival.
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