In addition to aneurysm and dissection, early osteoarthritis, and other systemic findings, congenital heart disease including persistent ductus arteriosus, atrial septal defect, pulmonary valve stenosis, atrial fibrillation, and bicuspid aortic valve have also been observed in patients with defects in SMAD3 [11]. This evidence concerns the gene SMAD3 and pulmonary valve stenosis.