Hôpital Marie-Lannelongue, CMR-M3C, Université Paris-Sud, 133 avenue de la Résistance, 92350 Le Plessis-Robinson, France.
Orphanet J Rare Dis. 2011 Oct 3;6:64. doi: 10.1186/1750-1172-6-64.
Classification of the overall spectrum of congenital heart defects (CHD) has always been challenging, in part because of the diversity of the cardiac phenotypes, but also because of the oft-complex associations. The purpose of our study was to establish a comprehensive and easy-to-use classification of CHD for clinical and epidemiological studies based on the long list of the International Paediatric and Congenital Cardiac Code (IPCCC).
We coded each individual malformation using six-digit codes from the long list of IPCCC. We then regrouped all lesions into 10 categories and 23 subcategories according to a multi-dimensional approach encompassing anatomic, diagnostic and therapeutic criteria. This anatomic and clinical classification of congenital heart disease (ACC-CHD) was then applied to data acquired from a population-based cohort of patients with CHD in France, made up of 2867 cases (82% live births, 1.8% stillbirths and 16.2% pregnancy terminations).
The majority of cases (79.5%) could be identified with a single IPCCC code. The category "Heterotaxy, including isomerism and mirror-imagery" was the only one that typically required more than one code for identification of cases. The two largest categories were "ventricular septal defects" (52%) and "anomalies of the outflow tracts and arterial valves" (20% of cases).
Our proposed classification is not new, but rather a regrouping of the known spectrum of CHD into a manageable number of categories based on anatomic and clinical criteria. The classification is designed to use the code numbers of the long list of IPCCC but can accommodate ICD-10 codes. Its exhaustiveness, simplicity, and anatomic basis make it useful for clinical and epidemiologic studies, including those aimed at assessment of risk factors and outcomes.
先天性心脏病（CHD）整体谱系的分类一直具有挑战性，部分原因是心脏表型的多样性，还因为其常常复杂的关联性。我们研究的目的是基于国际儿科和先天性心脏病代码（IPCCC）的长列表，为临床和流行病学研究建立一种全面且易于使用的 CHD 分类。
我们使用 IPCCC 长列表中的六位数字代码对每个单独的畸形进行编码。然后，我们根据包含解剖、诊断和治疗标准的多维方法，将所有病变分为 10 个类别和 23 个亚类。然后，我们将这种先天性心脏病的解剖和临床分类（ACC-CHD）应用于法国一项基于人群的 CHD 患者队列中获得的数据，该队列包括 2867 例患者（82%为活产，1.8%为死产，16.2%为妊娠终止）。
大多数病例（79.5%）可以用单个 IPCCC 代码识别。“异位症，包括异构和镜像”是唯一一种通常需要多个代码才能识别病例的类别。最大的两个类别是“室间隔缺损”（52%）和“流出道和动脉瓣异常”（20%的病例）。
我们提出的分类并不新颖，而是根据解剖和临床标准将已知的 CHD 谱重新组合成可管理的类别数量。该分类旨在使用 IPCCC 长列表的代码编号，但也可以容纳 ICD-10 代码。其详尽性、简单性和解剖基础使其在临床和流行病学研究中非常有用，包括那些旨在评估风险因素和结果的研究。
