Population-based evaluation of a suggested anatomic and clinical classification of congenital heart defects based on the International Paediatric and Congenital Cardiac Code

Abstract

BackgroundClassification 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).MethodsWe 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).ResultsThe 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).ConclusionOur 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.

Associated genetic syndromes and extracardiac malformations strongly influence outcomes of fetuses with congenital heart diseases

“…The anatomy of the cardiac defects was determined from postnatal echocardiography and surgical reports. The cardiac defects of all fetuses and inborn babies were classified in 10 groups, based on the anatomical and clinical ACC-CHD classification [18]; we added five more classes for non-structural cardiac abnormalities.…”

Coding 299 Fetal Hearts Scans Using One Single Item from International Pediatric and Congenital Cardiac Code (IPCCC) and the Anatomic and Clinical Classification (ACC-CHD) Lists: Results, Limits, and Comparison of Discordances Related to Professional Experience

“…Some will have a functional logic (for a therapeutic approach) others will have anatomical, embryological logic, or even physiological sense... This is why the authors of the ACC-CHD recommend to use their tools with "logic and intuition" (10), or that the authors of the IPCCC proposed thousands of codes to be combined with each other. We feel that the clinical truth probably lies somewhere in between.…”

“…It offers a complete and comprehensive coding system (over 10,000 referenced codes). In 2011, a French team, in collaboration with other European centers, published a new CHD coding system, the Anatomical and Clinical Classification of Congenital Heart Defects (ACC-CHD) [10]. Their goal was to achieve a more intuitive and easy to use tool, grouping heart diseases in 10 categories and 23 subcategories.…”

Hereditary syndromes associated with the congenital heart diseases in Azerbaijan

“…последнее десятилетие большинство авторов сходятся во мнении, что частота врожденных по-роков сердца (согласно МКБ-10 Q 20 -Q 28 ) варьирует от 19 до 75 на 1000 родившихся живыми [1][2][3], при этом тяжелые врожденные пороки регистрируются с часто-той 19,1-23,9 на 1000 рождений [4]. Большое значение в диагностике и определении генеза пороков сердца имеют фетальная и постнатальная эхокардиография, клинические и генетические методы исследования [4].…”

“…(1) проводили нейросонографию с последующей кон-сультацией невролога, ультразвуковое исследование внутренних органов, определение инфекционного статуса. Ряд обследований проведен индивидуально, исходя из особенностей клинического статуса боль-ных.…”