Marked disparity in mechanical wall properties between ascending and descending aorta in patients with tetralogy of Fallot

Recent studies have linked abnormal aortic medial pathology to progressive aortic root dilatation in patients with tetralogy of Fallot (TOF). To explore whether the aortic medial pathology in TOF is linked to aortic mechanical property, the present study tested the hypothesis that the distribution of impaired aortic elasticity corresponds to the known distribution of abnormal medial pathology (confined to the ascending aorta) in TOF. Pulse wave velocity (PWV) of the proximal and distal aortas was measured with a high-fidelity micromanometer in 98 TOF patients (64 with repaired TOF and 34 with unrepaired TOF) and 63 control subjects. PWV of the proximal aorta was significantly higher in TOF than in the control, but similar in repaired and unrepaired TOF (repaired: 588 +/- 205 cm/s, unrepaired: 680 +/- 288 cm/s, control: 439 +/- 101 cm/s, P < 0.001 for each TOF group vs. control, P = 0.07 for repaired vs. unrepaired TOF). In contrast, PWV of the distal aorta was almost identical among the three groups (repaired: 441 +/- 189 cm/s, unrepaired: 430 +/- 114 cm/s, control: 461 +/- 164 cm/s, P = 0.73, analysis of variance), indicating that abnormal aortic mechanical property is confined to the proximal aorta regardless of the operative status of TOF. This was also confirmed by comparison within the group; PWV of the proximal aorta was significantly higher than that of the distal aorta in both TOF groups (P < 0.001, each), whereas there was no difference in PWV between the proximal and distal aortas in the control subjects (P = 0.61). Consistent with the known histopathological disparity between the media of the ascending and descending aortas, aortic stiffness is markedly increased in the proximal but not in the distal aorta of TOF. These results suggest that aortic wall stiffness is a potentially useful clinical marker of aortic dilation in patients with TOF.