title: Aortic valve reconstruction in Rubinstein-Taybi-syndrome: the valuable aid of transesophageal echocardiography.
abstract: A fourteen year old boy with Rubinstein-Taybi-syndrome presented with a severe congenital subvalvular fibrous aortic stenosis with associated aortic regurgitation. Transthoracic echocardiographic imaging and left heart angiography showed a transvalvular peak systolic pressure gradient of 90 mmHg and a regurgitant fraction of 30%. The surgical treatment consisted of resection of the subvalvular fibrous tissue and subsequent aortic valvuloplasty. Intraoperative transesophageal echocardiography revealed a wide systolic opening of the aortic valve and good coaptation of the aortic valve leaflets in diastole. Two-dimensional color-coded and contrast echocardiography were successfully used to confirm a satisfactory reconstruction. Transesophageal echocardiography therefore represents a valuable tool in the assessment of the morphologic and haemodynamic status, especially in rare cases of congenital heart disease in older children.
authors.affiliation: Department of Cardiovascular Surgery, University of Kiel, Germany.
authors.forename: I
authors.initials: I
authors.lastname: Maurer
authors.forename: D
authors.initials: D
authors.lastname: Regensburger
authors.forename: A
authors.initials: A
authors.lastname: Bernhard
mesh_terms: D000293:Adolescent; D001020:Aortic Stenosis, Subvalvular / Q000150:complications / Q000151:congenital / Q000601:surgery*; D001022:Aortic Valve Insufficiency / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery*; D004452:Echocardiography / Q000379:methods*; D004947:Esophagus; D006801:Humans; D007430:Intraoperative Care / Q000379:methods*; D008297:Male; D012415:Rubinstein-Taybi Syndrome / Q000150:complications*