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Hemodynamics in Adults with Systemic Right Ventricles: Differences Between Congenitally Corrected and Complete
William R Miranda1, C Charles Jain2, Alexander C Egbe2
1Department of Cardiovascular Medicine, Mayo Clinic, 200 First St SW, Rochester, MN, 55905, USA. miranda.william@mayo.edu.
Insights
Adults with d-transposition of the great arteries (d-TGA) and congenitally corrected TGA (ccTGA) after atrial switch share a systemic right ventricle (sRV) but exhibit distinct hemodynamics and valve issues. These differences necessitate tailored heart failure treatments for each condition.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Adult Congenital Heart Disease
Background:
- Congenitally corrected transposition of the great arteries (ccTGA) and complete transposition of the great arteries (d-TGA) are often managed similarly post-atrial switch due to their shared systemic right ventricle (sRV).
- However, their distinct anatomical origins may lead to underlying hemodynamic differences that warrant investigation.
Purpose of the Study:
- To compare the hemodynamics and structural/valvular abnormalities between adults with ccTGA and d-TGA following an atrial switch procedure.
- To identify potential differences that could influence clinical management and treatment strategies.
Main Methods:
- Retrospective review of 138 adult patients undergoing cardiac catheterization between 2000 and 2021.
- Patients were categorized into d-TGA (post-Mustard procedure), complex ccTGA (with VSD and/or LVOTO), and isolated ccTGA groups.
- Analysis included hemodynamic parameters, ventricular size and function, and prevalence of valvular regurgitation.
Main Results:
- No significant differences were observed in systemic right ventricle (sRV) or left ventricular size and function across the groups.
- ccTGA patients more frequently presented with moderate or greater tricuspid regurgitation compared to d-TGA patients.
- The ratio of pulmonary artery wedge pressure (PAWP) to sRV end-diastolic pressure (sRVEDP) was higher in d-TGA, and cardiac index was higher in d-TGA, suggesting increased ventricular afterload in ccTGA patients.
Conclusions:
- Despite sharing an sRV, adults with d-TGA and ccTGA post-atrial switch exhibit significant hemodynamic and structural/valvular differences.
- These findings underscore the need for further research into condition-specific responses to heart failure therapies for d-TGA and ccTGA.
Abstract:
Despite their anatomical differences, congenitally corrected (ccTGA) and complete transposition of the great arteries (d-TGA) post-atrial switch are frequently studied together and managed similarly from a medical standpoint due to the shared systemic right ventricle (sRV). The aim was to assess differences in their underlying hemodynamics. The study is a retrospective review of 138 adults with ccTGA or d-TGA post-atrial switch undergoing cardiac catheterization at Mayo Clinic, MN between 2000 and 2021. ccTGA was categorized into isolated or complex ccTGA depending on concomitant ventricular septal defect and/or left ventricular outflow obstruction. There were 53 patients with d-TGA (91% post-Mustard procedure), 51 with complex and 34 with isolated ccTGA. Isolated ccTGA patients were older (51.8 ± 13.1 years) than those with d-TGA (37.5 ± 8.3 years) or complex ccTGA (40.8 ± 13.4 years). There were no differences in sRV or left ventricular size and function across groups. The ccTGA group more commonly had ≥ moderate tricuspid regurgitation than those with d-TGA; ≥ moderate mitral and ≥ moderate pulmonary regurgitation were most prevalent in complex ccTGA. There were no differences in sRV end-diastolic pressure (sRVEDP) or PAWP between groups. However, the ratio of PAWP:sRVEDP was higher in those with d-TGA compared to those with ccTGA. Cardiac index was higher in the d-TGA group than both groups of ccTGA patients with the latter showing higher indices of ventricular afterload. In conclusion, despite sharing a sRV, adults with d-TGA and ccTGA have substantial differences in hemodynamics and structural/valvular abnormalities. Further investigation regarding disease-specific responses to heart failure therapy in those with d-TGA and ccTGA is warranted.
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