Progressive discrete subaortic stenosis developed after successful primary repair of the supravalvular aortic stenosis in Williams syndrome.
Discrete subaortic stenosis is a rare, late complication of the surgical repair of congenital heart defects. Secondary subaortic stenosis late after surgical repair of supravalvular aortic stenosis in Williams syndrome has not previously been described. A 20 year-old female patient with Williams syndrome became symptomatic eight years after subaortic membrane resection. A discrete subaortic stenosis was identified by echocardiography. She was indicated for a Konno operation with a 19-mm Sorin SL valve inserted in the aortic position, along with ventricular septal defect closure with a pericardial patch. She produced a transient slight hypocalcemia after the operation. She was discharged home in a good condition on the fourth post-operative day. Intracardiac morphological and hemodynamic factors have been suggested as responsible for such a late complication. Our case study suggests the need for regular follow-up in patients with Williams syndrome, even after primary surgical repair.
Yuan
Shi-Min
SM
Departments of Cardiac and Thoracic Surgery, The Chaim Sheba Medical Center, Tel Hashomer, Israel. shi_min_yuan@yahoo.com
Kuperstein
Rafael
R
Lavce
Jacob
J
D021921:Aortic Stenosis, Supravalvular / Q000209:etiology / Q000601:surgery*; D021922:Discrete Subaortic Stenosis / Q000000981:diagnostic imaging / Q000209:etiology*; D018450:Disease Progression; D004452:Echocardiography; D005260:Female; D006801:Humans; D011183:Postoperative Complications; D018980:Williams Syndrome / Q000150:complications*; D055815:Young Adult