Past and current cause-specific mortality in Eisenmenger syndrome
Cristel M Sørensen Hjortshøj1, Aleksander Kempny2, Annette Schophuus Jensen1
1Department of Cardiology, Rigshospitalet, Copenhagen, Denmark.
European Heart Journal
|April 22, 2017
Summary
Eisenmenger syndrome (ES) patients live longer but still face high mortality. Recent trends show deaths from heart failure increasing, while procedural deaths have decreased, indicating a shift in causes of death for this condition.
Area of Science:
- Cardiology
- Pulmonary Hypertension
- Internal Medicine
Background:
- Eisenmenger syndrome (ES) is a severe condition with significant morbidity and mortality.
- Therapeutic strategies and specialist follow-up have evolved since the 2000s.
Purpose of the Study:
- To determine cause-specific mortality in Eisenmenger syndrome.
- To evaluate changes in mortality patterns between 1977 and 2015.
Main Methods:
- Retrospective, descriptive, multicentre study including 1546 patients from 13 countries.
- Analysis of cause-specific mortality before and after July 2006 ('early' vs. 'late' eras).
- Median follow-up of 6.1 years.
Main Results:
- Over 558 deaths recorded; 74% had identified causes.
- Leading causes of death: heart failure (34%), infection (26%), sudden cardiac death (10%).
- Heart failure deaths increased in the 'late' era (P=0.032), while procedural and thromboembolic deaths decreased (P<0.05). Median survival increased significantly (52.3 vs. 35.2 years, P<0.001).
Conclusions:
- Despite improved care, all-cause and cardiac mortality in ES remain high.
- Patients in the 'late' era experience longer survival, with deaths predominantly from chronic heart failure rather than acute events.
- Lifelong specialized care and further research for ES are essential.
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