Mechanisms for cardiac output augmentation in patients with a systemic right ventricle

Conflicting data have been published on the differences in cardiac response to exercise between patients with an atrially switched transposition of the great arteries (TGA), and patients with a congenitally corrected TGA (ccTGA). The purpose of our study was to evaluate the differences in cardiac response to exercise in these 2 patient groups with a systemic right ventricle (RV). Thirty four patients (62% male; mean 35, range 21-69 years) with a systemic RV (20 with an atrially switched TGA and 14 with a ccTGA) were included. Exercise tests with Portapres measurements were performed to assess maximal exercise capacity (V'O(2peak)), cardiac index, stroke volume index, and heart rate augmentation. Cardiac function was assessed by Cardiovascular Magnetic Resonance or Computed Tomography, and serum NT-proBNP levels. We found that both groups were able to significantly augment cardiac index during exercise. Cardiac index augmentation during exercise was positively related to V'O(2peak) (r=0.37, p <0.05), and independent of resting cardiac function. Although the increase in cardiac index during exercise was similar in both patients with TGA and with ccTGA, mechanisms to achieve this increase differed between groups. ccTGA patients increased both stroke volume and heart rate during exercise. Atrially switched TGA patients augmented heart rate during exercise, but failed to increase stroke volume. Mechanisms to achieve cardiac output augmentation differ between ccTGA patients and TGA patients. We suggest that therapeutic approaches should be tailored to the specific patient group to avoid counterproductive effects