Task force 1: The changing profile of congenital heart disease in adult life
Task force 1: The changing profile of congenital heart disease in adult life
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DOI：
10.1016/s0735-1097(01)01272-4
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发表时间：
2001-04-01
影响因子：
24
通讯作者：
Webb, GD
中科院分区：
文献类型：
--
作者：
Warnes, CA;Liberthson, R;Webb, GD
在过去的50年里，心脏手术、重症监护和非侵入性诊断的非凡进步导致美国和世界各地患有先天性心脏病（CHD）的成年人数量大幅增长。大约85%的出生时患有心血管异常的婴儿可以预期达到成年，随着手术技术的不断改进，这可能在未来二十年内进一步增加（1）。在加拿大，估计成人先天性心脏病的幸存者人数将从1996年的94 000人增加到2006年底的124 000人。虽然人们普遍认为美国有大量的成年冠心病患者，但缺乏准确的统计数据。报告的新生儿CHD患病率差异很大，在一定程度上取决于病变纳入和排除标准。例如，一些研究包括室间隔缺损（VSD）;然而，这些人中约有三分之二的人在成年时不再患有VSD。许多研究排除了二叶主动脉瓣，这是存在于1%的活产婴儿。此外，不同的确定方法（如体格检查、超声心动图、登记数据）得出的婴儿期CHD患病率也不同。英国最近的一项研究（2）回顾了1985年至1994年期间在一个健康地区（纽卡斯尔）的所有出生情况，并注意到在377，310例活产人口中有1，942例CHD病例（发病率为5.2/1，000）。在这些新生儿中，预计有1，514人可以存活16年。由于额外的诊断有时在儿童期后期进行，预计至少有2，192名儿童可以存活16年。此外，估计有784人需要在成年后进行随访。这些数字预测了成人CHD患者的随访需求，每10万例活产有200例，或英国每年有1,600例（假设人口为5000万）。假设美国人口为2.8亿，这意味着每年增加8，960例成人病例，即本十年增加89，600例。然而，从20世纪80年代中期开始的大多数研究，以及最近的加拿大研究，报告CHD出生人数接近10/1000活产（3）。由于缺乏数据，确定成年后这一人群的确切规模和组成具有挑战性。这次贝塞斯达会议的一个重要任务是估计病人人数，这对项目规划和资源分配至关重要。根据美国人口普查数据，对1940年至1989年记录的出生率进行了平均（表1-3）。复杂、中度和轻度病变对应的诊断见表4至表6，这些诊断由第4工作组使用。根据1.5/1，000例复杂性CHD活产的记录发病率（表1），并通过推断早期至最近几年的可能存活率，得出该组存活者的大致数量。根据新英格兰地区婴儿心脏计划（NERICP）对导管插入术数据、手术结果和尸检诊断的审查，1,000例活产婴儿中有1.5例发生率[4]。使用这种方法，估计2000年有117，000名患有真正复杂CHD的成年人生活在美国。随着手术技术的改进，预计这一数字将在未来十年内增加。使用类似的模型，表2显示了表5中定义的中度CHD患者到2000年的预期生存率。2.5/1，000的患病率来自已发表的儿童数据，以及一些开始时病变较简单但出现并发症的患者（例如，VSD伴瓣膜病变...
The extraordinary advances in cardiac surgery, intensive care, and noninvasive diagnosis over the last 50 years have led to an enormous growth in the US and throughout the world in the number of adults with congenital heart disease (CHD). Approximately 85% of babies born with cardiovascular anomalies can expect to reach adulthood, and with continued improvement in surgical technique, this could increase further in the next two decades (1). In Canada, it is estimated that the number of survivors with adult congenital heart disease (ACHD) will increase from 94,000 in 1996 to 124,000 by the end of 2006. Although there is a general recognition that there are large numbers of adults with CHD in the US, accurate statistics are lacking. Reported prevalence rates of CHD in newborns vary widely and depend, to some extent, on lesion inclusion and exclusion criteria. For example, some studies include ventricular septal defects (VSDs); however, about two thirds of these individuals no longer have a VSD by adult age. Many studies exclude bicuspid aortic valves, which are present in 1% of live births. In addition, different methods of ascertainment (eg, physical examination, echocardiography, registry data) yield varying prevalence rates of CHD in infancy. A recent English study (2) reviewed all births in one health region (Newcastle) between 1985 and 1994, and noted 1,942 cases of CHD in a population of 377,310 live births (incidence of 5.2 per 1,000). Of these newborns, 1,514 were predicted to survive 16 years. Because additional diagnoses are sometimes made later in childhood, at least 2,192 children were expected to survive 16 years. Also, an estimated 784 would require follow-up in adult life. These figures predict the need for follow-up of adults with CHD, for 200 cases per 100,000 live births, or 1,600 cases every year in the UK (assuming a population of 50 million). Assuming a population of 280 million in the US, that would mean an increase of 8,960 adult cases annually, or 89,600 cases in the current decade. Most studies from the mid 1980s onward, however, as well as more recent Canadian studies, report the number of CHD births to be close to 10 in 1,000 live births (3). Defining the exact size and composition of this population in adulthood is challenging, because data are lacking. An important mandate of this Bethesda Conference is to estimate patient numbers, which are essential for program planning and resource allocation. On the basis of the US census data, the documented birth rates from 1940 to 1989 were averaged (Tables 1–3). The diagnoses corresponding to complex, moderate, and mild lesions are shown in Tables 4 through 6, and are those used by Task Force 4. Based on a documented incidence of 1.5 in 1,000 live births for complex CHD (Table 1) and by extrapolating likely survival rates for the early through more recent years, the approximate numbers of survivors in this group were derived. The incidence of 1.5 in 1,000 live births was based on the large New England Regional Infant Cardiac Program (NERICP) review of catheterization data, surgical findings, and postmortem diagnoses (4). Using this approach, 117,000 adults with truly complex CHD are estimated to live in the US in the year 2000. With improved surgical techniques, this number can be anticipated to increase over the next decade. Using a similar model, Table 2 demonstrates the anticipated survival, to the year 2000, of patients with moderate CHD, as defined in Table 5. A prevalence of 2.5 in 1,000 is derived from published data on children, as well as some patients who began with more simple lesions but acquired complications (eg, VSD with valve lesions …