Takei Kohta, Murakami Tomoaki, Takeda Atsuhito
Department of Pediatrics, Hokkaido University Hospital, Sapporo, Japan.
Department of Cardiology, Chiba Children's Hospital, 579-1 Heta-cho, Midori-ku, Chiba, 266-0007, Japan.
Pediatr Cardiol. 2018 Oct;39(7):1462-1467. doi: 10.1007/s00246-018-1917-5. Epub 2018 Jun 6.
Progressive aortic root dilation and stiffening have been reported in patients with tetralogy of Fallot (TOF), even after repair. However, the implications of such aortic dilation and stiffening have not been fully assessed. A stiff aorta has a negative effect on the coronary circulation. 15 patients with TOF after repair were enrolled in this study. Using a left ventriculogram and pressure waveform recorded by a pressure sensor-mounted catheter, we analyzed the diameter and distensibility of the ascending and descending aorta. We also calculated the subendocardial viability ratio (SEVR), which measures the cardiac blood supply-workload balance. These values were compared with those in one-to-one age-matched controls. Moreover, the correlation between the diameter ratio (aortic diameter of the patient/aortic diameter of the matched control subject) and the distensibility ratio (distensibility of the patient/distensibility of the matched control subject) was analyzed. The mean age of patients was 3.9 ± 1.6 years. In patients with TOF, the indexed diameter was larger (0.20 ± 0.02 vs. 0.15 ± 0.02 mm/cm, p < 0.0001) and the distensibility was lower (5.1 ± 2.2 vs. 7.7 ± 2.0 cm dynes 10, p = 0.0009) in the ascending aorta than in control subjects. There was no difference in the SEVR between patients and control subjects. The distensibility ratio was significantly negatively correlated with the indexed diameter ratio in the ascending aorta (r = 0.35, p = 0.019). Regardless of the aortic stiffening, the cardiac blood supply-workload balance was maintained. We speculate that the aortic root dilation might be a mechanism to functionally complement aortic stiffness in patients with TOF.
法洛四联症（TOF）患者即使在修复术后，也有进展性主动脉根部扩张和僵硬的报道。然而，这种主动脉扩张和僵硬的影响尚未得到充分评估。僵硬的主动脉对冠状动脉循环有负面影响。本研究纳入了15例TOF修复术后患者。使用左心室造影和通过安装压力传感器的导管记录的压力波形，我们分析了升主动脉和降主动脉的直径及扩张性。我们还计算了心内膜下存活比率（SEVR），该比率用于衡量心脏血液供应与工作负荷的平衡。将这些值与年龄匹配的一对一对照组的值进行比较。此外，分析了直径比（患者主动脉直径/匹配对照组主动脉直径）与扩张性比（患者扩张性/匹配对照组扩张性）之间的相关性。患者的平均年龄为3.9±1.6岁。在TOF患者中，升主动脉的指数直径更大（0.20±0.02 vs. 0.15±0.02 mm/cm，p<0.0001），扩张性更低（5.1±2.2 vs. 7.7±2.0 cm dynes 10，p = 0.0009），与对照组相比。患者和对照组之间的SEVR没有差异。升主动脉的扩张性比与指数直径比显著负相关（r = 0.35，p = 0.019）。尽管主动脉僵硬，但心脏血液供应与工作负荷的平衡得以维持。我们推测主动脉根部扩张可能是TOF患者在功能上补偿主动脉僵硬的一种机制。
