Aortic Valve-Sparing Operation in a 8 Years Old Boy with Loeys-Dietz Syndrome with Annuloaortic Ectasia

Abstract

An 8 year-old boy had a cardiac murmur pointed out on day three after birth and was given a diagnosis of ventricular septal defect (VSD). He underwent VSD patch closure at two months after birth. He was also found the having Loeys-Dietz syndrome on the basis of mutation of TGFBR2 and physical examination at the age of 2 years. He had been followed up at pediatrics clinic of our hospital since then, and was hospitalized for a 46.5-mm extension of valsalva sinus diameter and moderate aortic insufficiency. The aortic valve was three-cusped and had no abnormality. We performed valve-sparing aortic root replacement. He was discharged on day 18 after the operation without any problems in the postoperative course. Use of an artificial heart valve for the surgery of the aortic root lesion in childhood will probably cause reoperation in the future and difficulty in Warfarin anticoagulation control. A careful decision is needed in the choice of an operation method. Valve-sparing aortic root replacement is a useful operation for patients without aortic valve abnormality.