Increasing Mortality Burden among Adults with Complex Congenital Heart Disease
Increasing Mortality Burden among Adults with Complex Congenital Heart Disease
复制标题
DOI：
10.1111/chd.12201
复制
发表时间：
2015-03-01
影响因子：
0.3
通讯作者：
Colman, Jack M.
中科院分区：
文献类型：
--
作者：
Greutmann, Matthias;Tobler, Daniel;Colman, Jack M.
背景先天性心脏病治疗的进展使死亡率主要转移到成年期。然而，患有复杂先天性心脏病的成年幸存者无法治愈，并且仍然存在年轻人过早死亡的风险。因此，我们的目的是描述复杂先天性心脏病成人患者队列的演变和死亡风险，从1980年到2009年，在一个中心随访的12644例成人先天性心脏病患者中，176例有艾森曼格综合征，76例有未修复的紫绀缺陷，221例有大动脉转位的心房调转手术，先天性矫正型大动脉转位158例，Fontan姑息术227例，法洛四联症修补术789例。我们描述了这6个患者队列的30年演变，分析了成年后的生存概率，并预测了未来的死亡人数，直到2029年。结果自1980年以来，有一个稳定的增加，患者人数如下，除了在艾森曼格综合征和未修复的紫绀缺陷的队列。在1980年至2009年期间，研究队列中有308名患者（19%）死亡。截至2009年底，85%的幸存者年龄在50岁以下。所有队列的生存率估计值明显低于一般人群，队列之间存在重要差异。在即将到来的二十年中，我们预测的年轻人主动脉下右心室，Fontan姑息，修复法洛四联症的死亡人数大幅增加。ConclusionsAnticipatory action是必要的，以准备临床服务越来越多的年轻人死于复杂的先天性心脏病的风险。
BackgroundProgress in management of congenital heart disease has shifted mortality largely to adulthood. However, adult survivors with complex congenital heart disease are not cured and remain at risk of premature death as young adults. Thus, our aim was to describe the evolution and mortality risk of adult patient cohorts with complex congenital heart disease.MethodsAmong 12644 adults with congenital heart disease followed at a single center from 1980 to 2009, 176 had Eisenmenger syndrome, 76 had unrepaired cyanotic defects, 221 had atrial switch operations for transposition of the great arteries, 158 had congenitally corrected transposition of the great arteries, 227 had Fontan palliation, and 789 had repaired tetralogy of Fallot. We depict the 30-year evolution of these 6 patient cohorts, analyze survival probabilities in adulthood, and predict future number of deaths through 2029.ResultsSince 1980, there has been a steady increase in numbers of patients followed, except in cohorts with Eisenmenger syndrome and unrepaired cyanotic defects. Between 1980 and 2009, 308 patients in the study cohorts (19%) died. At the end of 2009, 85% of survivors were younger than 50 years. Survival estimates for all cohorts were markedly lower than for the general population, with important differences between cohorts. Over the upcoming two decades, we predict a substantial increase in numbers of deaths among young adults with subaortic right ventricles, Fontan palliation, and repaired tetralogy of Fallot.ConclusionsAnticipatory action is needed to prepare clinical services for increasing numbers of young adults at risk of dying from complex congenital heart disease.