Cardiac pacemaker in adult humans is about 70 beats per minute. Because the sinoatrial node is responsible for the rest of the heart's electrical activity, it is sometimes called the "primary pacemaker". If the SA node does not function properly and is unable to control the heart rate, a group of cells further down the heart will become the ectopic pacemaker of the heart. These cells form the Atrioventricular node (or AV node), which is an area between the left atrium and the right ventricle within the atrial septum, will take over the pacemaker responsibility. The cells of the AV node normally
Cardiac neural crest cells the precursor to the pulmonary artery. In humans, mutations in Notch most often result in bicuspid aortic valve disease and calcification of the aortic valve. Bone morphogenetic proteins (BMPs) are required for neural crest cell migration into the cardiac cushions (precursors to heart valves and septa) and for differentiation of neural crest cells to smooth muscle cells of the aortic arch arteries. In neural crest–specific Alk2-deficient embryos, the cardiac cushions of the outflow tract are deficient in cells because of defects in neural crest cell migration. Fibroblast growth factor 8 (FGF8) transcription factors are essential for regulating the addition of