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In essence cardiac development shows basic similarities of the major processes involved in between species; therefore, mechanisms unraveled in animal models can be reliably used in understanding normal human cardiac development and congenital heart disease (CHD). This chapter provides an update on recent advances in heart development in which it is important to distinguish a first heart field (FHF) and a second heart field (SHF). It provides a general introduction into embryology and then talks about the most common heart malformations in a developmental context. This is followed by a discussion on each specific malformation. The chapter explains the separation of the SHF in an anterior/secondary (arterial pole) and posterior (venous pole) population. It then introduces a number of processes that are essential in the formation of the four-chambered heart with a proper alignment of the atria, ventricles, and great arteries.
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