Congenital Heart Disease: a Pictorial Illustration of Putting Segmental Approach into Practice

Abstract

The human heart is a complex organ in which many complicated congenital defectsmay happen and some of them require surgical intervention. Due to the vastcomplexity of varied anatomical presentations, establishing an accurate and consistentnomenclature system is utmost important to facilitate effective communication amongpediatric cardiologists, cardiothoracic surgeons and radiologists. The Van Praaghsegmental approach to the complex congenital heart disease (CHD) was developed inthe 1960s and has been used widely as the language for describing complex anatomyof CHD over the decades. It utilizes a systematic and sequential method to describe thecardiac segments and connections which in turn allows accurate, comprehensive andunambiguous description of CHD. It can also be applied to multiple imaging modalitiessuch as echocardiogram, cardiac CT and MRI. The Van Praagh notation demonstrates agroup of three letters, with each letter representative for a key embryologic region ofcardiac anatomy: the atria, ventricles and great vessels. By using a 3-steps approach,we can evaluate complex CHD precisely and have no difficulties in communicatingwith other medial colleague. This pictorial essay revisits the logical steps of segmentalapproach, followed by a pictorial illustration of its application.