[II-AEPCJS-03] Right ventricular function in patients with congenitally corrected transposition of the great arteries
Keywords:Congenitally corrected transposition of the great arteries, Right ventricular function, Systemic right ventricle
Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart disease. Right ventricular (RV) function has a great impact on clinical outcome in patients with ccTGA. Patients without significant associated intracardiac lesions usually do not have symptoms of heart failure in childhood and do well until adult life. However, the gradual dysfunction of the RV, which supports systemic circulation, will occur after decades in life. RV dysfunction usually remains subclinical for decades. By 45 years of age, more than 30% of patients with isolated ccTGA and more than two thirds of patients with associated lesions develop clinical congestive heart failure.
As mechanisms of RV dysfunction, high pressure inside of the RV causes increased ventricular wall stress and dysfunction of the trabecular component and valvular apparatus of the RV. Another mechanism of RV dysfunction is thought to be inadequate perfusion of the coronary artery, as only one coronary artery supplies the hypertrophied RV mass. Tricuspid regurgitation (TR) has also been found to be a major contributor to the development of progressive RV dysfunction. In ccTGA, the tricuspid annulus dilates, the papillary muscles move away from the inflow tract, and the tethered cords do not allow the leaflets to occlude the orifice. These mechanisms predispose TR to a steady increase over time.
In this presentation, the mechanisms of RV dysfunction are summarized on the basis of reviewed articles. This will be helpful to the audience in understanding these mechanisms and in their clinical practice.
As mechanisms of RV dysfunction, high pressure inside of the RV causes increased ventricular wall stress and dysfunction of the trabecular component and valvular apparatus of the RV. Another mechanism of RV dysfunction is thought to be inadequate perfusion of the coronary artery, as only one coronary artery supplies the hypertrophied RV mass. Tricuspid regurgitation (TR) has also been found to be a major contributor to the development of progressive RV dysfunction. In ccTGA, the tricuspid annulus dilates, the papillary muscles move away from the inflow tract, and the tethered cords do not allow the leaflets to occlude the orifice. These mechanisms predispose TR to a steady increase over time.
In this presentation, the mechanisms of RV dysfunction are summarized on the basis of reviewed articles. This will be helpful to the audience in understanding these mechanisms and in their clinical practice.