Management of the failing systemic right ventricle

Conditions in which the right ventricle serves as the systemic pumping chamber are frequently complicated by the development of right ventricular failure and tricuspid valve regurgitation. The right ventricle is the systemic ventricle in conditions of ventriculoarterial discordance with atrioventricular concordance (transposition of the great arteries) or with atrioventricular discordance (congenitally corrected transposition of the great arteries). Concerns regarding actual or potential systemic right ventricular failure in these cases may lead to surgical evaluation and treatment designed to reestablish the left ventricle as the systemic pump. In cases where the left ventricle has prolonged exposure to low pressures in the pulmonary circulation, the left ventricle must be "retrained" to assume a systemic pressure load. Anatomic repair, with or without a preparatory period of left ventricular retraining, is a consideration for three clinically relevant scenarios: (1) patients with transposition of the great arteries after an atrial level switch (Senning or Mustard procedure), (2) patients with congenitally corrected transposition who are unoperated or who have undergone physiologic ("classic") repair, and (3) unoperated patients with transposition who present after the neonatal period.