Int J Cardiol. 2011 Aug 4;150(3):e116-8. doi: 10.1016/j.ijcard.2010.02.067. Epub 2010 Mar 11.
We treated a 6-year-old boy who had polysplenia syndrome and tetralogy of Fallot with a small right ventricle (RV), an atrial septal defect, a hemiazygos connection, and bilateral superior vena cava. Because the RV was too small for a biventricular repair to be performed, the patient underwent a total cavopulmonary shunt operation although his heart was biventricular and a pacemaker (VVI) had been implanted for management of the sick sinus syndrome complicated by polysplenia syndrome. After the operation, marked asynchronous contraction was noted between the morphological right and left ventricles and was probably responsible for the low cardiac output noted in this patient. In order to clarify the significance of the asynchronous contraction, we determined the cause of the low cardiac output by studying the time course of the volume changes in the morphological right and left ventricles during a cardiac cycle by using angiograms. In addition, we studied the interventricular flow dynamics by using pulsed-Doppler echocardiography. After a Fontan-type operation is performed on patients with a biventricular heart, the 2 ventricles may not function in perfect coordination when they have to work as 1 unit. These patients are likely to develop cardiac dysfunction due to interventricular to-and-fro flow dynamics. Asynchronous contraction between the 2 ventricles caused by abnormal interventricular conduction impaired the cardiac performance in the present case.
我们治疗了一名 6 岁男孩，他患有多脾综合征和小右心室（RV）、房间隔缺损、半奇静脉连接和双侧上腔静脉的法洛四联症。由于 RV 太小，无法进行双心室修复，尽管患者的心脏是双心室的，并且已经植入了起搏器（VVI）来治疗多脾综合征并发的病态窦房结综合征，但仍进行了全腔肺动脉分流术。手术后，在形态学上的右心室和左心室之间观察到明显的异步收缩，这可能是导致该患者心输出量低的原因。为了阐明异步收缩的意义，我们通过使用血管造影术研究心脏周期中形态学上的右心室和左心室的容积变化过程，来确定低心输出量的原因。此外，我们通过脉冲多普勒超声心动图研究了室间血流动力学。在对具有双心室心脏的患者进行 Fontan 型手术后，当它们必须作为 1 个单位工作时，2 个心室可能无法完美协调地工作。这些患者由于室间往复血流动力学可能会发展为心功能障碍。本病例中，异常室间传导引起的 2 个心室之间的异步收缩损害了心脏功能。
