Congenitally corrected transposition of the great arteries: Current treatment options

Abstract

Congenitally corrected transposition of the great arteries is a relatively rare cardiac malformation characterized by atrioventricular and ventriculoarterial discordance. This double discordance results in a physiologically corrected circulation with the morphologic right ventricle (RV) serving as the systemic pump. Associated anomalies are present in approximately 98% of cases and include most commonly ventricular septal defect, pulmonary stenosis, and anomalies of the systemic atrioventricular valve (SAVV). Conduction abnormalities are common as well, predisposing these patients to the development of complete heart block. The management of these patients is primarily determined by the presence and severity of the associated anomalies. Conventional repair, which leaves the morphologic RV as the systemic ventricle, has resulted in high incidence of tricuspid regurgitation (TR) and progressive dysfunction of the RV. In the majority of patients, congestive heart failure secondary to RV dysfunction occurs by the fifth or sixth decade. The cause and effect relationship between TR and RV dysfunction remains to be determined. The advent of the Double Switch operation, which restores the morphologic left ventricle (LV) as the systemic ventricle, has yielded favorable outcomes at early follow-up. This procedure should be applied in young patients to achieve optimal results. Retraining the LV by pulmonary artery banding is associated with high morbidity and mortality when attempted after infancy or early childhood. Long-term follow-up is needed to evaluate the potential superiority of this procedure and the incidence of rhythm, baffle, and conduit complications for this management option.

PKC translocation and ERK1/2 activation in compensated right ventricular hypertrophy secondary to chronic emphysema.

“…Most patients with L-transposition of the great arteries, a condition in which the anatomic right ventricle serves as the systemic ventricle from birth, eventually develop severe RV failure. This usually occurs before the age of 30 [13]. Thus, clinical evidence suggests that the genetic program of the right ventricle is not designed to face an increased work load even when the adaptation is very gradual.…”

Rheumatic “Taussig-Bing Heart”: A Case Report

“…Medical management with diuretics, afterload reduction with angiotensin converting enzyme inhibitors such as captopril and digoxin (inotropic) is adapted for the failing systemic right ventricle [40], depending on the severity of associated malformations [41]. The role of angiotensin receptor blocker with losartan was evaluated and found to have no improvement in exercise capacity and no reduction in neurohormonal levels in patients with systemic right ventricle [42].…”

Remote Control of Pulmonary Blood Flow

“…In patients where left ventricular retraining is required because of late referral in the presence of transposition of the great arteries [21] and in congenitally corrected transposition of the great arteries (= double discordance) [22][23][24][25][26], the FloWatch® is the only technique allowing to modulate the distal pulmonary artery pressure in a fashion suitable with the continuously variable clinical needs of these conditions, generally requiring repeated adjustments and prolonged stay in intensive care unit [21].…”

“…Progressively increasing interest in pulmonary artery banding has been recently dictated by the indication for left ventricular retraining in transposition of the great arteries with late referral [21] and in congenitally corrected transposition of the great arteries (= double discordance) [22][23][24][25][26].…”