Radiology Department, Hôpital Clocheville, Université François Rabelais de Tours, France
Pediatric Cardiology Department, Hôpital Clocheville, Université François Rabelais de Tours, France.
J Am Heart Assoc. 2017 Dec 29;7(1):e006921. doi: 10.1161/JAHA.117.006921.
In our practice, we noticed an increased frequency of tracheobronchial branching abnormalities (TBAs) in patients with tetralogy of Fallot (ToF). This study aimed to determine whether an association exists between congenital TBAs and ToF with or without pulmonary atresia.
The frequency of TBAs on chest computed tomography was assessed in 55 patients with ToF without pulmonary atresia, 34 patients with ToF with pulmonary arteria, and 100 control patients. We then looked for a possible association between TBAs and pulmonary artery branch hypoplasia, the presence of major aortopulmonary collateral arteries, and the presence of the chromosome 22q11 deletion. TBAs were significantly more frequent in patients with ToF with or without pulmonary atresia than in the control group (any TBAs, 21% versus 2% [<0.001]; bronchial situs anomalies, 6% versus 0% [=0.002]; right tracheal bronchus, 4% versus 0% [=0.04]; left eparterial bronchus, 8% versus 0% [=0.005]); and tended to be more frequent in those with ToF without pulmonary atresia than in those with ToF with pulmonary atresia (any TBAs, 27% versus 12% [=0.11]; left eparterial bronchus, 13% versus 0% [=0.04]). TBAs were readily multiple (8 patients of 19 with TBA) and concerned essentially the upper lobes. TBAs were not associated with pulmonary branch hypoplasia, major aortopulmonary collateral arteries, or the chromosome 22q11 deletion.
We demonstrated a significantly increased frequency of tracheobronchial abnormalities in patients with ToF with or without pulmonary atresia compared with a control group. These results suggest an interaction between abnormalities in conotruncal septation and tracheobronchial branching and may provide a new clue to the pathogenesis of conotruncal heart diseases.
在我们的实践中，注意到法洛四联症（ToF）患者的气管支气管分支异常（TBA）频率增加。本研究旨在确定先天性 TBA 与伴有或不伴有肺动脉闭锁的 ToF 之间是否存在关联。
在 55 例无肺动脉闭锁的 ToF 患者、34 例有肺动脉闭锁的 ToF 患者和 100 例对照患者中，评估了胸部 CT 上 TBA 的频率。然后，我们寻找 TBA 与肺动脉分支发育不良、主肺动脉侧支动脉的存在以及 22q11 缺失之间的可能关联。有或无肺动脉闭锁的 ToF 患者的 TBA 明显多于对照组（任何 TBA，21%比 2%[<0.001]；支气管位异常，6%比 0%[=0.002]；右主支气管，4%比 0%[=0.04]；左叶间支气管，8%比 0%[=0.005]），且无肺动脉闭锁的 ToF 患者的 TBA 发生率高于有肺动脉闭锁的 ToF 患者（任何 TBA，27%比 12%[=0.11]；左叶间支气管，13%比 0%[=0.04]）。TBA 容易多发（19 例中有 8 例），主要涉及上叶。TBA 与肺分支发育不良、主肺动脉侧支动脉或 22q11 缺失无关。
与对照组相比，我们在有或无肺动脉闭锁的 ToF 患者中发现气管支气管异常的频率显著增加。这些结果表明圆锥动脉干间隔异常与气管支气管分支异常之间存在相互作用，可能为圆锥动脉干心脏疾病的发病机制提供新线索。
