Figure 1. Clinical phenotype in a proband with MFS. The picture shows the ocular, skeletal and cardiovascular features of the proband in family QT753. (A) shows the ectopia lentis. (B) shows the arachnodactyly. (C) show the cubitus valgus and the pectus carinatum. D: The echocardiography of this patient was normal when he was 8 years old. But five years later, when the he was 13 years old, echocardiography showed an aortic diameter of 5.8 cm at the sinuses of Valsalva with severe aortic valve insufficiency.