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Functionally Single-Ventricle Complications After Fontan Palliation-A Narrative Review
Małgorzata Kowalczyk1, Mirosław Kowalski1
1Department of Adult Congenital Heart Disease, National Institute of Cardiology in Warsaw, 04-628 Warsaw, Poland.
Functionally single-ventricle (FSV) defects require Fontan palliation, leading to long-term complications like organ dysfunction and psychosocial challenges. Comprehensive, multidisciplinary care is essential for managing these complex pediatric heart conditions.
Area of Science:
- Cardiology
- Pediatric Congenital Heart Disease
Background:
- Functionally single-ventricle (FSV) defects are complex congenital heart anomalies necessitating Fontan palliation.
- Fontan circulation, while improving survival, presents unique physiological challenges predisposing patients to long-term complications.
Purpose of the Study:
- To review the spectrum of cardiovascular and systemic complications associated with Fontan physiology.
- To highlight the impact of these complications on patients' quality of life and the need for comprehensive management.
Main Methods:
- Literature review of studies on Fontan palliation outcomes.
- Analysis of cardiovascular, systemic, and psychosocial complications in FSV patients.
Main Results:
- Key cardiovascular issues include ventricular dysfunction, valve regurgitation, arrhythmias, and thromboembolic events.
- Systemic complications comprise Fontan-associated liver disease (FALD), protein-losing enteropathy (PLE), plastic bronchitis (PB), renal, endocrine, and psychosocial burdens.
- These complications lead to frequent hospitalizations and impaired life opportunities.
Conclusions:
- Fontan physiology poses significant long-term risks requiring vigilant monitoring and management.
- A multidisciplinary approach is crucial to address the complex interplay of hemodynamic, organ-specific, and psychosocial factors in Fontan patients.
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