Congenital cardiac defects, like coarctation of the aorta and bicuspid aortic valve, are found in approximately one-third of TS patients, with bicuspid aortic valve being the most prevalent, occurring in approximately 25% of TS patients [15], the link between TS and the aortic bicuspid valve is most likely due to several complex mechanisms, one of which appears to be the action of TIMP1 and TIMP3. Here, TIMP1 is linked to Timothy syndrome.