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Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 18, 2014
A 6-year follow-up study of adult patients with congenitally corrected transposition
Mirta Koželj1, Marta Cvijić1, Pavel Berden2
11Department of Cardiology,University Medical Centre Ljubljana,Zaloška 7,1525 Ljubljana,Slovenia.
Insights
Congenitally corrected transposition of the great arteries (ccTGA) leads to heart failure primarily due to systemic right ventricular dysfunction. Biomarkers like NT-proBNP and tissue Doppler imaging help monitor disease progression.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Cardiac Imaging
Background:
- Congenitally corrected transposition of the great arteries (ccTGA) presents unique challenges in adult congenital heart disease management.
- Long-term outcomes, particularly the development of heart failure, require further elucidation.
- The role of tricuspid regurgitation (TR) and reliable monitoring markers in ccTGA are not fully understood.
Purpose of the Study:
- To assess medium-term heart failure development in adult ccTGA patients.
- To identify the impact of tricuspid regurgitation on heart failure progression.
- To determine sensitive markers for monitoring ccTGA-related cardiac dysfunction.
Main Methods:
- Prospective 6-year follow-up of 19 adult ccTGA patients.
- Clinical evaluation, NT-proBNP levels, exercise stress testing.
- Echocardiography, magnetic resonance, CT, and Tissue Doppler Imaging (TDI).
Main Results:
- Two deaths due to heart failure occurred.
- Significant decline in exercise capacity and systemic ventricular ejection fraction (p=0.001).
- TDI revealed decreased peak tricuspid annular velocities (systolic p=0.032, diastolic p=0.048); NT-proBNP levels increased (p=0.022).
Conclusions:
- Progressive exercise intolerance in ccTGA is primarily driven by systemic right ventricular dysfunction.
- Tricuspid regurgitation may contribute to heart failure, especially with Ebstein anomaly.
- NT-proBNP and TDI parameters are sensitive markers for detecting changes and guiding management in ccTGA.
Abstract:
The aims of this study were to assess the development of heart failure in patients with congenitally corrected transposition of the great arteries in a medium-term follow-up, to identify the impact of tricuspid regurgitation on the development of heart failure, and to determine the most reliable marker for its identification. The prospective 6-year follow-up study included 19 adult patients. All patients were evaluated clinically by the determination of N-terminal pro-hormone brain natriuretic peptide levels, exercise stress testing, echocardiography magnetic resonance, or CT. Among them, two patients died of heart failure. There was a decline in exercise capacity and systolic systemic ventricular function (p=0.011). Systemic ventricular ejection fraction decreased (48.3±13.7 versus 42.7±12.7%, p=0.001). Tissue Doppler imaging showed a decline in peak tricuspid systolic annular velocity (10.3±2.0 versus 8.3±2.5 cm/second, p=0.032) and peak tricuspid early diastolic annular velocity (14.6±4.3 versus 12.0±4.5 cm/second, p=0.048). The tricuspid regurgitation did not increase significantly. N-terminal pro-hormone brain natriuretic peptide levels increased (127.0 ng/L(82.3-305.8) versus 226.0 ng/L(112.5-753.0), p=0.022). Progressive exercise intolerance in congenitally corrected transposition of the great arteries appears to be driven mainly by a progression in systemic right ventricular dysfunction. Tricuspid regurgitation is likely to play a role, especially in patients with structural abnormalities of the tricuspid valve - Ebstein anomaly. The N-terminal pro-hormone brain natriuretic peptide levels and tissue Doppler parameters appear sensitive in detecting changes over time and may guide management.
