成人先天性心脏病的现状与未来  

Current status and future of adult congenital heart disease

作　　者：田苗 张勇[2] 陈寄梅[2] TIAN Miao;ZHANG Yong;CHEN Jimei(Department of Cardiac Surgery, Guangdong General Hospital, School of Medicine, South China University of Technology, Guangzhou, 510030, P.R.China;Department of Cardiac Surgery, Guangdong Provincial Cardiovascular Institute, Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, 510100, P.R.China)

机构地区：[1]华南理工大学附属广东省人民医院心外科,广州510100 [2]广东省心血管病研究所广东省人民医院心外科广东省华南结构性心脏病重点实验室,广州510100

出　　处：《中国胸心血管外科临床杂志》2019年第6期590-600,共11页Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

基　　金：国家重点研发计划(2018YFC1002600);广东省科技计划项目(2017A070701013;2017B090904034;2017030314109;2019B020230003);广东省登峰计划项目(DFJH201802)

摘　　要：先天性心脏病是指胚胎发育时期由于心脏及大血管的形成障碍或发育异常或出生后本应自动关闭的通道未能闭合,导致心脏或胸腔内大血管实体性结构或者功能意义上的异常。近几年来逐步提出了新的更加广泛的定义即结构性心脏病。结构性心脏病狭义上指的是心脏内解剖结构异常所致的心脏病理、生理变化,其中就包括先天性心脏病。几十年前,先天性心脏病被认为是一种儿科疾病,因为大多数严重病变的患者很少存活到成年。由于近几十年来超声心动图、麻醉、重症监护,经皮介入尤其是心脏手术方面的重大进展,儿童先天性心脏病的治疗和干预策略有了很大改善,曾经在儿童时期致命的缺陷现在可以被成功修复或减轻。由于这些成功,90%以上的先天性心脏病患者有望生存至成年,这就导致了一个新的人群,即患有先天性心脏病的成年患者出现了。成人先天性心脏病患者不同于儿童,肺动脉高压导致的右心衰竭最终进展为全心衰竭,艾森曼格综合征的出现导致严重的紫绀和病情的恶化。目前机械辅助循环支持装置和心或心肺联合移植技术的不断进步,提高了终末期成人先天性心脏病心力衰竭患者的生存率。妊娠患者心血管事件的高发需要综合多学科的团队护理,并对分娩进行早期协调规划包括早期妊娠相关风险咨询、密切监测患者心脏功能和定期对胎儿扫描评估。产前产后一体化诊疗模式以及胎儿宫内治疗技术的开展通过胎儿时期的干预从源头上降低了成人先天性心脏病的发病率。其它如心律失常、感染性心内膜炎、脑血管意外事件、其它内科基础代谢性疾病等并发症也对未来的诊治提出了挑战。现对成人先天性心脏病患者的发病率及流行病学、肺动脉高压和终末期心力衰竭等并发症以及产前产后一体化诊疗模式和胎儿宫内治疗进行论述总Congenital heart disease refers to the structural or functional abnormality of the macrovascular in the heart or thoracic cavity caused by the failure of the formation of the heart and large blood vessels during the embryonic development or the abnormal closure of the heart or the closure of the channel after birth. In the past few years, a new and broader definition of structural heart disease has been gradually proposed. Structural heart disease narrowly refers to the pathological and physiological changes of the heart caused by abnormal anatomical structures in the heart, including congenital heart disease. A few decades ago, congenital heart disease was considered as a pediatric disease, because most patients with severe lesions rarely survive to adulthood. Due to recent advances in echocardiography, anesthesia, intensive care, percutaneous intervention, especially cardiac surgery in recent decades, the treatment and intervention strategies for congenital heart disease in children have been greatly improved, a fatal defect in childhood can now be successfully repaired or alleviated. Because of these successes, more than 90% of congenital heart disease patients are expected to survive to adulthood, which has led to emerge a new population: adult patients with congenital heart disease. Adult congenital heart disease patients are different from children. Pulmonary hypertension leads to right heart failure and eventually progresses to whole heart failure. The appearance of Eisenmenger syndrome leads to severe cyanosis and worsening of the disease. At present, the continuous development of mechanical assisted circulation support devices and heart or cardiopulmonary transplantation technology has increased the survival rate of end-stage adult congenital heart disease patients with heart failure. The high incidence of cardiovascular events in pregnant patients requires comprehensive multidisciplinary team care and early coordination planning for delivery, including early counseling for pregnancy-related risks, close

关 键 词：成人 结构性心脏病 肺动脉高压 心力衰竭 移植 妊娠 产前诊断 宫内 

分 类 号：R541.1[医药卫生—心血管疾病]