Division of Cardiovascular-Thoracic Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois.
Congenital Heart Center, University of Florida, Gainesville, Florida.
Semin Thorac Cardiovasc Surg. 2020;32(3):531-538. doi: 10.1053/j.semtcvs.2020.02.007. Epub 2020 Feb 12.
Palliation of patients with hypoplastic left heart syndrome remains challenging. Although coronary ischemia can be catastrophic, the prevalence and pathologies of anomalies of the coronary arteries remains unknown. We reviewed 119 specimens with the features of hypoplastic left heart syndrome, focusing our attention on the aortic root and the coronary arteries. We found 36 (30%) specimens with the combination of mitral and aortic atresia, 26 (22%) with mitral and aortic stenosis, and 57 (48%) with mitral stenosis combined with aortic atresia. In 29 specimens (24%), the coronary arteries were not located in the center of any sinuses, while in 24 specimens (21%) at least 1 coronary artery was located very proximal to a raphe or commissure, with potential for obstruction. The specimens with combined stenosis were more likely to have eccentric positions of the coronary arteries (11 specimens, 42%), compared to the 3 specimens with combined atresia (9%, P = 0.009). The specimens with combined stenosis were also more likely to have positioning at risk for obstruction (9 specimens, 35%), compared to those with combined atresia (3 specimens, 9%, P = 0.05). Coronary arterial fistulous communications were found in 11 specimens (9%), significantly more frequently in specimens with mitral stenosis and aortic atresia (9 specimens, 16%, P = 0.041). The origins of the coronary arteries in patients with hypoplastic left heart syndrome place them at potential risk for ischemia, with fistulous communications being a particular risk in those with mitral stenosis combined with aortic atresia.
左心发育不全综合征患者的姑息治疗仍然具有挑战性。尽管冠状动脉缺血可能是灾难性的，但冠状动脉异常的患病率和病理学仍不清楚。我们回顾了 119 例具有左心发育不全综合征特征的标本，重点关注主动脉根部和冠状动脉。我们发现 36 例（30%）标本同时存在二尖瓣和主动脉闭锁，26 例（22%）标本同时存在二尖瓣和主动脉狭窄，57 例（48%）标本同时存在二尖瓣狭窄和主动脉闭锁。在 29 例标本（24%）中，冠状动脉不在任何窦的中心，而在 24 例标本（21%）中，至少有 1 条冠状动脉非常靠近嵴或交界，有阻塞的潜在风险。与联合闭锁的标本相比，联合狭窄的标本更有可能出现冠状动脉偏心位置（11 例，42%），而联合闭锁的标本只有 3 例（9%），P=0.009。与联合闭锁的标本相比，联合狭窄的标本更有可能出现阻塞风险的定位（9 例，35%），而联合闭锁的标本只有 3 例（9%），P=0.05。在 11 例标本（9%）中发现冠状动脉瘘管交通，在二尖瓣狭窄伴主动脉闭锁的标本中更为常见（9 例，16%），P=0.041。左心发育不全综合征患者的冠状动脉起源使他们存在缺血的潜在风险，而瘘管交通在二尖瓣狭窄伴主动脉闭锁的患者中是一个特殊的风险。
