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Preventing disease progression in Eisenmenger syndrome
Ana Barradas-Pires1, Andrew Constantine1,2, Konstantinos Dimopoulos1,2
1Department of Cardiology, Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK.
Expert Review of Cardiovascular Therapy
|April 15, 2021
Summary
Eisenmenger syndrome, a severe complication of congenital heart defects, involves pulmonary vascular disease and cyanosis. Early screening and management, including pulmonary arterial hypertension therapies, are crucial for improving patient outcomes.
Area of Science:
- Cardiology
- Pulmonology
- Medical Genetics
Background:
- Eisenmenger syndrome is an extreme manifestation of pulmonary arterial hypertension in patients with congenital heart disease (CHD).
- It results from untreated congenital heart defects leading to severe pulmonary vascular disease, right-to-left shunting, and chronic cyanosis.
- Regular assessment for pulmonary hypertension in all CHD patients is recommended.
Purpose of the Study:
- To review the progression of congenital heart defects to Eisenmenger syndrome.
- To discuss screening, diagnosis, and management strategies for Eisenmenger syndrome.
- To highlight the role of pulmonary arterial hypertension therapies in managing this condition.
Main Methods:
- Literature review of congenital heart defects, Eisenmenger syndrome progression, and management strategies.
- Discussion of disease mechanisms and clinical manifestations.
- Analysis of current expert opinions on treatment and outcomes.
Main Results:
- Eisenmenger syndrome necessitates expert care to prevent disease progression and manage significant morbidity and mortality.
- Shunt closure is contraindicated once Eisenmenger syndrome develops.
- Pulmonary arterial hypertension therapies, alongside supportive care, can improve quality of life and exercise tolerance.
Conclusions:
- Early identification and management of pulmonary hypertension in CHD patients are vital.
- While pulmonary arterial hypertension therapies offer benefits, optimal timing for initiation and escalation requires further research.
- Comprehensive, expert-led care is essential for patients with Eisenmenger syndrome.
Keywords:
Eisenmenger syndromePulmonary arterial hypertensioncongenital heart defectscyanosispulmonary vascular diseasetargeted therapiesMore Related Videos
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