Singh T P, Di Carli M F, Sullivan N M, Leonen M F, Morrow W R
Department of Pediatrics, Wayne State University School of Medicine, Detroit, Michigan, USA.
J Am Coll Cardiol. 1998 Feb;31(2):437-43. doi: 10.1016/s0735-1097(97)00488-9.
This study sought to evaluate regional myocardial flow reserve in long-term survivors of repair of anomalous left coronary artery from pulmonary artery (ALCAPA) and to relate the flow abnormalities to the patients' exercise performance.
Patients with ALCAPA usually present during infancy with severe ischemic cardiomyopathy. The left ventricular function recovers after surgical repair. However, the extent of recovery of myocardial blood flow (MBF) and its potential physiologic significance in long-term survivors are unknown.
We evaluated MBF (ml/g per min) at baseline and during maximal coronary vasodilation by adenosine in 11 patients after ALCAPA repair (median age 17 years, range 7 to 22) using nitrogen-13 ammonia and dynamic positron emission tomographic imaging. Patients also underwent an incremental exercise test with metabolic monitoring. In each patient, MBF was quantified in the three major vascular territories: the left anterior descending and left circumflex coronary artery territories and the right coronary artery (control region) territory.
Basal MBF was mildly reduced in the left coronary territories versus the control region (0.79 +/- 0.14 vs. 0.85 +/- 0.19, p = 0.05). During hyperemia, flow in the left coronary territories was significantly lower than that in the control region (2.1 +/- 0.5 vs. 2.6 +/- 0.5, p < 0.001). As a result, myocardial flow reserve was lower in the left coronary territories than in the control region (2.6 +/- 0.7 vs. 3.2 +/- 0.7, p < 0.001). Exercise performance was impaired in patients when compared with age-matched control subjects. Maximal oxygen consumption correlated linearly with maximal hyperemic flows in the left coronary artery territories (r = 0.73, p = 0.03).
Long-term survivors of ALCAPA repair demonstrate regional impairment of myocardial flow reserve. This may contribute to impaired exercise performance by limiting cardiac output reserve.
本研究旨在评估肺动脉起源异常左冠状动脉（ALCAPA）修复术后长期存活者的局部心肌血流储备，并将血流异常与患者的运动能力相关联。
ALCAPA患者通常在婴儿期表现为严重的缺血性心肌病。手术修复后左心室功能可恢复。然而，长期存活者中心肌血流量（MBF）的恢复程度及其潜在的生理意义尚不清楚。
我们使用氮-13氨和动态正电子发射断层扫描成像技术，对11例ALCAPA修复术后患者（中位年龄17岁，范围7至22岁）在基线和腺苷介导的最大冠脉血管扩张期间的MBF（毫升/克每分钟）进行了评估。患者还进行了递增运动试验并进行代谢监测。在每位患者中，对三个主要血管区域的MBF进行了定量：左前降支和左旋支冠状动脉区域以及右冠状动脉（对照区域）区域。
与对照区域相比，左冠状动脉区域的基础MBF轻度降低（0.79±0.14 vs. 0.85±0.19，p = 0.05）。充血期间，左冠状动脉区域的血流明显低于对照区域（2.1±0.5 vs. 2.6±0.5，p < 0.001）。因此，左冠状动脉区域的心肌血流储备低于对照区域（2.6±0.7 vs. 3.2±0.7，p < 0.001）。与年龄匹配的对照受试者相比，患者的运动能力受损。最大耗氧量与左冠状动脉区域的最大充血血流呈线性相关（r = 0.73，p = 0.03）。
ALCAPA修复术后的长期存活者表现出局部心肌血流储备受损。这可能通过限制心输出量储备导致运动能力受损。
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