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Long-term survival in aged patients with corrected transposition of the great arteries
Insights
Corrected transposition of the great arteries (C-TGA) is rare, but this study shows patients can live a normal lifespan. Advanced noninvasive techniques aid diagnosis and highlight right ventricle adaptation to systemic pressure.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Medical Imaging
Background:
- Corrected transposition of the great arteries (C-TGA) is a rare congenital heart defect.
- Survival beyond 50 years is limited due to associated defects or complications like valvular insufficiency or heart block.
Observation:
- The study describes four men with uncomplicated C-TGA.
- Two cases were diagnosed using advanced noninvasive imaging techniques, a shift from traditional invasive cardiac catheterization.
- These patients served as a model to observe long-term right ventricle adaptation to systemic pressure load.
Findings:
- The right ventricle demonstrated the capacity for increased muscle mass as a long-term adaptation to a high systemic pressure load.
- Noninvasive diagnostic techniques are becoming crucial for diagnosing C-TGA.
- Patients with C-TGA can achieve a normal lifespan with appropriate medical management.
Implications:
- Highlights the potential for a normal lifespan in C-TGA patients with proper care.
- Emphasizes the growing role of noninvasive diagnostics in complex congenital heart disease.
- Provides insights into the long-term functional adaptation of the right ventricle under systemic pressure.
Abstract:
Corrected transposition of the great arteries is a rare condition, and few patients with this abnormality survive past 50 years of age because of associated congenital defects or the subsequent development of atrioventricular valvular insufficiency or heart block or both. We describe four men with uncomplicated C-TGA. Our patients are of interest for the following reasons: (a) their condition is very rare; (b) the diagnosis of C-TGA traditionally has been verified through invasive cardiac catheterization procedures; however, in our latest two patients, recently developed noninvasive diagnostic techniques played the decisive role in the diagnosis of C-TGA; (c) in these modalities, they presented as a "natural experimental model" that the right ventricle submitted to a high systemic pressure load is capable of increasing muscle mass over long-term adaptation. Our four patients illustrate that patients with C-TGA, even with the associated cardiac anomalies, may live a normal life span with proper management.