Gupta Saurabh Kumar, Mukherjee Aprateem, Pandey Niraj Nirmal, Ramakrishnan Sivasubramanian, Kothari Shyam Sunder, Saxena Anita, Anderson Robert H
Department of Cardiology, Cardiothoracic Sciences Centre, All India Institute of Medical Sciences (AIIMS), New Delhi, India.
Department of Cardiovascular Imaging and Endovascular Interventions, Cardiothoracic Sciences Centre, All India Institute of Medical Sciences (AIIMS), New Delhi, India.
Pediatr Cardiol. 2025 Mar;46(3):704-712. doi: 10.1007/s00246-024-03490-0. Epub 2024 Apr 22.
We recently encountered several cases of tetralogy of Fallot with an abnormally oriented S-shaped ascending aorta. In this retrospective study, we sought to clarify morphology of this unusual under-recognized variant. Databases were reviewed to identify all patients with tetralogy of Fallot having an S-shaped ascending aorta. Computed tomographic angiography was used for the assessment of cardiac morphology. Out of the 21 patients, 18 (86%) had a right aortic arch, 2 (9%) had a left aortic arch, and the remaining patient (5%) had a double aortic arch. Patients with a right aortic arch, compared to age and sex-matched patients with a right aortic arch but normally oriented ascending aorta, had lesser aortic override (29.3 ± 14% vs 54.8 ± 13.2%; p = 0.0001) and a wider ascending aorta (25.2 ± 6.9 vs 18.0 ± 3.2 mm; p = 0.0003). The S-shaped ascending aorta was located posteriorly, with a higher sterno-aortic distance (25.5 ± 7.7 vs 9.9 ± 4.5 mm; p = 0.0001). The ascending aorta among patients with tortuosity was longer (4.12 ± 1.7 vs 3.07 ± 0.82, p = 0.03) but with similar tortuosity index (1.22 ± 0.19 vs 1.15 ± 0.17, p = 0.23). Of the cases with right aortic arch and S-shaped ascending aorta, 16 (89%) had extrinsic compression of the right pulmonary artery (p = 0.0001), while 7 (39%) had crossed pulmonary arteries (p = 0.008), with no such findings among those with normally oriented ascending aorta. Tetralogy of Fallot with an S-shaped ascending aorta is a variant with lesser aortic override and a more posteriorly located ascending aorta. Compression of the right pulmonary artery and crossed pulmonary arteries is frequent in the presence of a right-sided aortic arch. These findings have important implications for optimal diagnosis and surgical repair.
我们最近遇到了几例法洛四联症合并异常走向的 S 形升主动脉的病例。在这项回顾性研究中，我们试图阐明这种未被充分认识的异常变体的形态。我们查阅了数据库，以确定所有患有法洛四联症且升主动脉呈 S 形的患者。使用计算机断层血管造影来评估心脏形态。在 21 例患者中，18 例（86%）有右位主动脉弓，2 例（9%）有左位主动脉弓，其余 1 例患者（5%）有双主动脉弓。与年龄和性别匹配但升主动脉走向正常的右位主动脉弓患者相比，右位主动脉弓患者的主动脉骑跨程度较小（29.3±14% 对 54.8±13.2%；p = 0.0001），升主动脉更宽（25.2±6.9 对 18.0±3.2mm；p = 0.0003）。S 形升主动脉位于后方，胸骨-主动脉距离更高（25.5±7.7 对 9.9±4.5mm；p = 0.0001）。有迂曲的患者的升主动脉更长（4.12±1.7 对 3.07±0.82，p = 0.03），但迂曲指数相似（1.22±0.19 对 1.15±0.17，p = 0.23）。在有右位主动脉弓和 S 形升主动脉的病例中，16 例（89%）有右肺动脉的外部压迫（p = 0.0001），而 7 例（39%）有交叉肺动脉（p = 0.008），在升主动脉走向正常的患者中未发现此类情况。法洛四联症合并 S 形升主动脉是一种主动脉骑跨程度较小且升主动脉位置更靠后的变体。在存在右位主动脉弓的情况下，右肺动脉受压和交叉肺动脉很常见。这些发现对最佳诊断和手术修复具有重要意义。
