Bhatt Ami B, Lantin-Hermoso Maria R, Daniels Curt J, Jaquiss Robert, Landis Benjamin John, Marino Bradley S, Rathod Rahul H, Vincent Robert N, Keller Bradley B, Villafane Juan
Departments of Internal Medicine and Pediatrics and Division of Cardiology, Harvard Medical School, Boston, MA, United States.
Section of Cardiology, Department of Pediatrics, Baylor College of Medicine, Houston, TX, United States.
Front Cardiovasc Med. 2022 May 25;9:817866. doi: 10.3389/fcvm.2022.817866. eCollection 2022.
Current management of isolated CoA, localized narrowing of the aortic arch in the absence of other congenital heart disease, is a success story with improved prenatal diagnosis, high survival and improved understanding of long-term complication. Isolated CoA has heterogenous presentations, complex etiologic mechanisms, and progressive pathophysiologic changes that influence outcome. End-to-end or extended end-to-end anastomosis are the favored surgical approaches for isolated CoA in infants and transcatheter intervention is favored for children and adults. Primary stent placement is the procedure of choice in larger children and adults. Most adults with treated isolated CoA thrive, have normal daily activities, and undergo successful childbirth. Fetal echocardiography is the cornerstone of prenatal counseling and genetic testing is recommended. Advanced 3D imaging identifies aortic complications and myocardial dysfunction and guides individualized therapies including re-intervention. Adult CHD program enrollment is recommended. Longer follow-up data are needed to determine the frequency and severity of aneurysm formation, myocardial dysfunction, and whether childhood lifestyle modifications reduce late-onset complications.
孤立性主动脉缩窄（即主动脉弓局限性狭窄且无其他先天性心脏病）的当前治疗是一个成功案例，其产前诊断得到改善、生存率高且对长期并发症的认识有所提高。孤立性主动脉缩窄有多种表现形式、复杂的病因机制以及影响预后的进行性病理生理变化。端端吻合术或扩大端端吻合术是婴儿孤立性主动脉缩窄首选的手术方法，而经导管介入治疗则更适用于儿童和成人。在较大儿童和成人中，首选的治疗方法是初次支架置入术。大多数接受治疗的孤立性主动脉缩窄成年患者情况良好，日常活动正常，且能成功分娩。胎儿超声心动图是产前咨询的基石，建议进行基因检测。先进的三维成像可识别主动脉并发症和心肌功能障碍，并指导包括再次干预在内的个体化治疗。建议成年先天性心脏病患者入组研究。需要更长时间的随访数据来确定动脉瘤形成的频率和严重程度、心肌功能障碍，以及儿童时期的生活方式改变是否能减少迟发性并发症。
