10:15 〜 11:45
[II-APT3-04] Congenitally Corrected Transposition: Efforts to minimize late development of systemic RV Failure
Congenitally corrected transposition of the great arteries is a rare defect representing approximately 0.5% of all congenital heart disease. ccTGA is characterised by the combination of atrioventricular discordance and ventriculo-arterial discordance. This combination, atrioventricular discordance and ventriculoarterial discordance, produces a unique set of management challenges.
Patients with congenitally corrected transposition of the great arteries have a morphological right ventricle that sustains the systemic circulation. In these patients, regurgitation of the systemic atrioventricular valve, the tricuspid valve, is a common finding. When TV regurgitation becomes severe, it is associated with RV failure and decreased survival. Associated anomalies such as ventricular septal defect, morphologic left ventricular outflow tract obstruction and tricuspid valve abnormalities occur in the majority of these patients. Although the presence of these associated conditions influences the clinical presentation of this condition and the type and timing of surgical intervention, the ability of the morphologic right ventricle and the tricuspid valve to withstand a lifetime of exposure to systemic pressure largely determines the ultimate outcome of these patients.
Progressive dysfunction of the tricuspid valve and right ventricle occurs after prolonged exposure to systemic pressure in a substantial percentage of patients who have undergone an atrial switch operation for transposition of the great arteries with increased risk for patients who have accompanying lesions. If sensitive measures of ventricular function are used, an even larger percentage of cc-TGA patients can be shown to have subclinical evidence of limited cardiovascular reserve. A significant percentage of cc-TGA patients had perfusion defects during exercise stress testing with radionuclide imaging.
Increasing morphologic LV pressure either naturally by PS or banding may maintain RV geometry & prevent progression of TR. Shifting the ventricular septum leftward, reduces left ventricular (LV) end-diastolic volume and causes LV to fall within the Frank-Starling curve and reduces LV filling dynamics and end-diastolic pressure.
Earlier intervention with tricuspid regurgitation even with minimal severity could reverse the future RV failure. Recent data supports the use of tricuspid valve replacement as an alternative to repair, especially in adult patients. The use of the double switch operation is predicated on the assumption that long-term outcomes will be better with the morphologic left ventricle supporting the systemic circulation.
Surgery for congenitally corrected transposition of the great arteries has evolved overtime. Anatomical repair of cc-TGA is now performed with quite satisfactory outcomes in children. The double switch operation has become the preferred surgical procedure in selected cases. However, Systemic A-V valve regurgitation strongly associated with RV dysfunction and CHF whether this is causative or secondary remains speculative.
1.Warnes CA. Transposition of the great arteries. Circulation. 2006; 114:2699–709.
2.Hiramatsu T, Matsumura G, Konuma T, et al. Long-term prognosis of double-switch operation for congenitally corrected transposition of the great arteries. Eur J Cardiothorac Surg. 2012; 42:1004–8.
3.Bogers AJ, Head SJ, de Jong PL, et al. Long term follow up after surgery on congenitally corrected transposition of the great arteries with a right ventricle in the systemic circulation. J Cardiothorac Surg. 2010; 5:74.
4.Graham TP Jr, Bernard YD, Mellen BG, et al. Long-term outcome in congenitally corrected transposition of the great arteries: a multi-institutional study. J Am Coll Cardiol. 2000; 36:255–61.
5.Said S.M., Dearani J.A. (2016) Physiologic Repair of Congenitally Corrected Transposition of the Great Arteries. In: Lacour-Gayet F., Bove E., Hraška V., Morell V., Spray T. (eds) Surgery of Conotruncal Anomalies. Springer.
Patients with congenitally corrected transposition of the great arteries have a morphological right ventricle that sustains the systemic circulation. In these patients, regurgitation of the systemic atrioventricular valve, the tricuspid valve, is a common finding. When TV regurgitation becomes severe, it is associated with RV failure and decreased survival. Associated anomalies such as ventricular septal defect, morphologic left ventricular outflow tract obstruction and tricuspid valve abnormalities occur in the majority of these patients. Although the presence of these associated conditions influences the clinical presentation of this condition and the type and timing of surgical intervention, the ability of the morphologic right ventricle and the tricuspid valve to withstand a lifetime of exposure to systemic pressure largely determines the ultimate outcome of these patients.
Progressive dysfunction of the tricuspid valve and right ventricle occurs after prolonged exposure to systemic pressure in a substantial percentage of patients who have undergone an atrial switch operation for transposition of the great arteries with increased risk for patients who have accompanying lesions. If sensitive measures of ventricular function are used, an even larger percentage of cc-TGA patients can be shown to have subclinical evidence of limited cardiovascular reserve. A significant percentage of cc-TGA patients had perfusion defects during exercise stress testing with radionuclide imaging.
Increasing morphologic LV pressure either naturally by PS or banding may maintain RV geometry & prevent progression of TR. Shifting the ventricular septum leftward, reduces left ventricular (LV) end-diastolic volume and causes LV to fall within the Frank-Starling curve and reduces LV filling dynamics and end-diastolic pressure.
Earlier intervention with tricuspid regurgitation even with minimal severity could reverse the future RV failure. Recent data supports the use of tricuspid valve replacement as an alternative to repair, especially in adult patients. The use of the double switch operation is predicated on the assumption that long-term outcomes will be better with the morphologic left ventricle supporting the systemic circulation.
Surgery for congenitally corrected transposition of the great arteries has evolved overtime. Anatomical repair of cc-TGA is now performed with quite satisfactory outcomes in children. The double switch operation has become the preferred surgical procedure in selected cases. However, Systemic A-V valve regurgitation strongly associated with RV dysfunction and CHF whether this is causative or secondary remains speculative.
1.Warnes CA. Transposition of the great arteries. Circulation. 2006; 114:2699–709.
2.Hiramatsu T, Matsumura G, Konuma T, et al. Long-term prognosis of double-switch operation for congenitally corrected transposition of the great arteries. Eur J Cardiothorac Surg. 2012; 42:1004–8.
3.Bogers AJ, Head SJ, de Jong PL, et al. Long term follow up after surgery on congenitally corrected transposition of the great arteries with a right ventricle in the systemic circulation. J Cardiothorac Surg. 2010; 5:74.
4.Graham TP Jr, Bernard YD, Mellen BG, et al. Long-term outcome in congenitally corrected transposition of the great arteries: a multi-institutional study. J Am Coll Cardiol. 2000; 36:255–61.
5.Said S.M., Dearani J.A. (2016) Physiologic Repair of Congenitally Corrected Transposition of the Great Arteries. In: Lacour-Gayet F., Bove E., Hraška V., Morell V., Spray T. (eds) Surgery of Conotruncal Anomalies. Springer.