Coronary Dysfunction in Developing Myocardial Damage in Congenital Heart Diseases

据报道，在成年期，肥厚心脏的心肌缺血是进行性心肌损伤的加重因素。然而，对心肌负荷严重的先天性心脏病的冠状动脉血流动力学研究较少。为了评估先天性心脏病的冠状动脉血流动力学，我们使用冠状动脉内多普勒导丝检测了青紫型先天性心脏病患者静息和三磷酸腺苷（ATP）诱导的充血反应期间的冠状动脉血流动力学。该研究组包括28例日本患者（男性18例，女性10例）（平均年龄：5.12±4.9岁），这些患者正在接受常规心导管插入术以评估术前(16例；10例TOF， 4例DORV， 2例TGA)和术后（12例；9例TOF， 3例DORV）冠心病。正如我们之前报道的那样，冠状动脉流速变量是用一根175厘米长、0.018英寸的柔性、可操纵的血管导管来测量的，导管的尖端集成了一个12 mhz的压电超声传感器（FloWireR, Cardio metrics, Inc., Mountain View, California）。冠状动脉血流储备（CFR）是微血管床效价的一个指标，通过计算冠状动脉内注射ATP后的峰值充血平均峰值速度（APV）与基线APV之商来评估CFR。先前报道的年龄匹配的对照数据被用作比较冠状动脉血流模式的参考值。在术前组，16例LAD患者中有69%的DSVR值显著升高，RCA患者中有81%的DSVR值显著升高。16例LAD患者中11例（75%）和RCA患者中13例（81%）在收缩期也检测到明显的反向血流信号。另一方面，在术后组中，12例LAD患者中只有1例（8%）检测到明显较高的DSVR值，而RCA中没有。只有1例（8%）LAD和2例（17%）RCA出现血流信号反转。在术前组，16例LAD患者中有9例（56%）的CFR值显著降低，RCA患者中所有患者（100%）的CFR值显著降低。另一方面，在术后组中，12例LAD和RCA患者中只有3例（25%）的CFR显著降低。每条血管CFR降低的患者术后时间较短（< 2年），而CFR正常的患者术后时间较长。在本研究中，所有术前患者的冠状动脉血流谱异常和CFR降低表明冠状动脉阻力显著增加，小冠状动脉扩张效力降低或收缩期心肌顺应性降低，发展为心肌缺血或不可逆心肌损伤。在心肌负荷明显的先天性心脏病的治疗和手术中，应认真考虑继发性冠状动脉功能障碍，防止心肌缺血成为不可逆心肌损伤的加重因素。少

It has been reported, in adulthood, that myocardial ischemia in the hypertrophic heart was an exacerbation factor to progressive myocardial damage. However, there have been few studies on the coronary hemodynamics in the congenital heart diseases with significant myocardial overload. To assess the coronary hemodynamics in the congenital heart diseases, we examined the coronary flow-velocity dynamics using an intracoronary Doppler guide wire at rest and during the adenosine triphosphate (ATP) -induced hyperemic responses in patients with cyanotic congenital heart diseasesThis study group consisted of 28 Japanese patients (18 males and 10 females) (mean ages : 5.12 ± 4.9 years) who were undergoing the routine cardiac catheterization for the evaluation of pre- (16 patients ; 10 TOF, 4 DORV, 2 TGA) and post-operative (12 patients ; 9 TOF and 3 DORV) CHDs. As we previously reported, coronary flow-velocity variables were measured using a 175-cm-long, 0.018-inch flexible, steer able angioplas … More tic guide wire with a 12-mHz piezoelectric ultrasound transducer integrated into its tip (FloWireR, Cardio metrics, Inc., Mountain View, California). The coronary flow reserve (CFR), which was an indicator of the potency in the micro vascular bed, was assessed by calculating the quotient of the peak hyperemic average peak velocity (APV) after intracoronary injection of ATP and the baseline APV. Age-matched control data reported previously were used as reference values for comparisons of coronary flow patternsIn the pre-operative group, significantly higher DSVR values were noted in 69 % of 16 patients in the LAD and in 81 % in the RCA. A significant reversed flow signal in the systolic phase were also detected in 11(75 %) of 16 patients in the LAD, and 13(81 %) in the RCA. On the other hand, in the post-operative group, significantly higher DSVR values were detected in only 1(8 %) of 12 patients in the LAD, and none in the RCA. A reversed flow signal was noted in only 1(8 %) in the LAD and 2(17 %) in the RCA. In the pre-operative group, significantly lower CFR values were noted in 9(56 %) of 16 patients in the LAD, and in all patients (100 %) in the RCA. In the Posto-perative group, on the other hand, a significant reduction in CFR was noted in only 3(25 %) of 12 patients in both of the LAD and the RCA. The post-operative periods were shorter (< 2 years) in the patients with a reduced CFR in each vessel, and longer in the Patients with a normal CFR. AIn this study, abnormal coronary flow profile and reduced CFR noted in all of the pre-operative patients indicate a significant increase in coronary resistance with a reduced dilatory potency of the small coronary artery or a decrease in myocardial compliance in the systolic Phase, developinging in myocardial ischemia or irreversible myocardial damage. In management and surgical operation of the congenital heart diseases with significant myocardial overload, it is necessary to take into careful consideration the secondary coronary dysfunction for preventing myocardial ischemia as an exacerbation factor to irreversible myocardial damage. Less