Gittenberger-de Groot A C, Sauer U, Bindl L, Babic R, Essed C E, Bühlmeyer K
Department of Anatomy and Embryology, Rijksuniversiteit, Leiden, The Netherlands.
Int J Cardiol. 1988 Feb;18(2):243-58. doi: 10.1016/0167-5273(88)90169-6.
Pulmonary atresia with intact ventricular septum can be complicated by the presence of large ventriculo-coronary arterial communications which disturb normal myocardial perfusion. In the selection of patients for surgery the presence of these communications provides an additional problem. On the basis of previous cineangiocardiographic study, we performed a histopathological study of 16 cases. Twelve presented with ventriculo-coronary arterial communications and four only showed myocardial sinusoids. A tripartite right ventricle was seen in the latter group but not exclusively. It is shown that subepicardial coronary arterial pathology is exclusively present in cases with ventriculo-coronary arterial communications although not solely at a connection site between a communication and an artery. The affected coronary artery is itself focally abnormal and hypoplastic and can be blocked or even absent. The interruption of such an artery can also occur after birth and not necessarily at a site of connection with a communication. This implies that infants with communications either already have a coronary circulation partly or completely dependent on ventriculo-coronary arterial communications at birth or can develop such a condition in time. This hazard to myocardial perfusion, often compromised by a large "steal" from the aorta to the right ventricle, limits the choice of surgical procedures.
室间隔完整的肺动脉闭锁可能会因存在大的心室 - 冠状动脉交通支而复杂化，这会干扰正常的心肌灌注。在选择手术患者时，这些交通支的存在带来了额外的问题。基于之前的心血管造影研究，我们对16例病例进行了组织病理学研究。其中12例存在心室 - 冠状动脉交通支，4例仅显示心肌窦状隙。在后一组中可见三尖瓣型右心室，但并非全部如此。结果表明，心外膜下冠状动脉病变仅存在于心室 - 冠状动脉交通支的病例中，尽管并不局限于交通支与动脉的连接部位。受影响的冠状动脉本身局部异常且发育不全，可能会阻塞甚至缺失。这种动脉的中断也可能在出生后发生，且不一定发生在与交通支的连接部位。这意味着有交通支的婴儿要么在出生时就已经有部分或完全依赖于心室 - 冠状动脉交通支的冠状动脉循环，要么可能会及时发展成这种情况。这种对心肌灌注的危害，常常因从主动脉到右心室的大量“窃血”而受损，限制了手术方式的选择。
