Hôpital Marie-Lannelongue, CMR-M3C, Université Paris-Sud, Le Plessis-Robinson, France.
J Anat. 2013 Mar;222(3):349-57. doi: 10.1111/joa.12023. Epub 2013 Jan 15.
Outflow tract defects, including cardiac neural crest defects (so-called conotruncal defects) and transposition of the great arteries, are due to an abnormal rotation of the outflow tract during cardiac development. Coronary orifices are often abnormal in outflow tract defects, particularly in common arterial trunk (CAT). A recent study indicates that abnormal coronary artery pattern in a mouse model with common arterial outlet (Tbx1-/- mouse mutant) could be due to a reduced and malpositioned subpulmonary coronary-refractory myocardial domain. The aim of our study was to demonstrate the relation between coronary orifices pattern in outflow tract defects in human and the abnormal embryonic rotation of the outflow tract. We analyzed 101 heart specimens with outflow tract defects: 46 CAT, 15 tetralogy of Fallot (TOF), 29 TOF with pulmonary atresia (TOF-PA), 11 double-outlet right ventricle with subaortic ventricular septal defect (DORV) and 17 controls. The position of left and right coronary orifices (LCO, RCO) was measured in degrees on the aortic/truncal circumference. The anterior angle between LCO and RCO (α) was calculated. The LCO was more posterior in TOF (31 °), TOF-PA (47 °), DORV (44 °), CAT (63 °), compared with controls (0 °, P < 0.05), and more posterior in CAT than in other outflow tract defects (P < 0.05). The RCO was more anterior in TOF (242 °), TOF-PA (245 °) and DORV (271 °) than in controls (213 °, P < 0.05), but not in CAT (195 °). The α angle was similar in TOF, TOF-PA, DORV and controls (149 °, 162 °, 133 °, 147 °), but significantly larger in CAT (229 °, P < 0.0001). In all outflow tract defects but CAT, the displacement of LCO (anterior) and RCO (posterior), while the α angle remains constant, might be due to incomplete rotation of the myocardium at the base of the outflow tract, leading to an abnormally positioned subpulmonary coronary-refractory myocardial domain. The larger α angle in CAT could reflect its dual identity, aortic and pulmonary.
流出道缺陷，包括心脏神经嵴缺陷（所谓的圆锥动脉干缺陷）和大动脉转位，是由于心脏发育过程中流出道的异常旋转所致。流出道缺陷常伴有冠状动脉口异常，尤其是共同动脉干（CAT）。最近的一项研究表明，在具有共同动脉出口（Tbx1-/- 小鼠突变体）的小鼠模型中，异常的冠状动脉模式可能是由于肺下冠状-refractory 心肌域减少和位置异常所致。我们的研究目的是证明人类流出道缺陷的冠状动脉口模式与流出道胚胎旋转异常之间的关系。我们分析了 101 例流出道缺陷心脏标本：46 例 CAT，15 例法洛四联症（TOF），29 例法洛四联症伴肺动脉闭锁（TOF-PA），11 例右心室双出口伴主动脉下室间隔缺损（DORV）和 17 例对照。在主动脉/干周长上以度测量左、右冠状动脉口（LCO、RCO）的位置。计算 LCO 和 RCO 之间的前角（α）。与对照组（0°，P<0.05）相比，TOF（31°）、TOF-PA（47°）、DORV（44°）和 CAT（63°）中的 LCO 更靠后，而 CAT 中的 LCO 比其他流出道缺陷更靠后（P<0.05）。与对照组（213°，P<0.05）相比，TOF（242°）、TOF-PA（245°）和 DORV（271°）中的 RCO 更靠前，但 CAT 中则不然（195°）。TOF、TOF-PA、DORV 和对照组的α角相似（149°、162°、133°、147°），但 CAT 中的α角明显较大（229°，P<0.0001）。在除 CAT 以外的所有流出道缺陷中，LCO（前部）和 RCO（后部）的位移，而α角保持不变，可能是由于流出道底部心肌的不完全旋转，导致肺下冠状-refractory 心肌域位置异常。在 CAT 中，较大的α角可能反映了其双重身份，即主动脉和肺动脉。
