PMID- 11788256
OWN - NLM
STAT- MEDLINE
DCOM- 20020221
LR  - 20190513
IS  - 1010-7940 (Print)
IS  - 1010-7940 (Linking)
VI  - 21
IP  - 1
DP  - 2002 Jan
TI  - Surgical repair of congenital supravalvular aortic stenosis in children.
PG  - 50-6
AB  - OBJECTIVE: Supravalvular aortic stenosis (SVAS) is an uncommon congenital cardiac 
      anomaly characterized by varying degrees of left ventricular outflow tract 
      obstruction beginning distal to the aortic valve. METHODS: Between March 1962 and 
      December 2000, 101 consecutive patients underwent surgical correction for 
      congenital SVAS at Riley Children's Hospital. There were 61 male (60%) and 40 
      female (40%) ranging in age from 3 month to 17 years (medium age, 6.1 years). 
      Fourteen patients (14%) had Williams syndrome. Preoperatively, 11 patients were 
      in New York Heart Association (NYHA) functional class I, 55 in class II, 28 in 
      class III, and seven in class IV. Of the 101 patients, 73 (72%) had localized 
      type SVAS and 28 (28%) diffuse type SVAS. RESULTS: Those with localized SVAS were 
      successfully treated with patch aortoplasty, whereas those with diffuse SVAS 
      required either an apical aortic conduit or extensive endarterectomy with patch 
      aortoplasty. The overall mean pressure gradient was reduced to 21 mmHg (P<0.001) 
      in the early postoperative period. There were one early death (<30 days 
      postoperatively) (1%), two (2%) late deaths, and 14 patients (14%) underwent one 
      or two additional operation (n=17) in a follow-up period ranging from 6 months to 
      30 years (medium 9.4 years). Postoperatively, there were 72 patients (73%) in 
      NYHA functional class I and 26 (27%) in class II. Overall survival including 
      operative mortality was 98% at 10 years, 97% at 20 and at 30 years. CONCLUSION: 
      Good surgical outcome of congenital SVAS can be achieved with the appropriate 
      method of treatment in patients with both localized and diffuse SVAS.
FAU - Brown, John W
AU  - Brown JW
AD  - Section of Cardiothoracic Surgery, James W. Riley Hospital for Children, Indiana 
      University School of Medicine, Indianapolis, IN 46202-5123, USA. 
      jobrown@iupui.edu
FAU - Ruzmetov, Mark
AU  - Ruzmetov M
FAU - Vijay, Palaniswamy
AU  - Vijay P
FAU - Turrentine, Mark W
AU  - Turrentine MW
LA  - eng
PT  - Journal Article
PL  - Germany
TA  - Eur J Cardiothorac Surg
JT  - European journal of cardio-thoracic surgery : official journal of the European 
      Association for Cardio-thoracic Surgery
JID - 8804069
SB  - IM
MH  - Adolescent
MH  - Aortic Stenosis, Supravalvular/complications/*surgery
MH  - Child
MH  - Child, Preschool
MH  - Female
MH  - Heart Defects, Congenital/complications
MH  - Humans
MH  - Infant
MH  - Male
MH  - Reoperation
MH  - Treatment Outcome
EDAT- 2002/01/15 10:00
MHDA- 2002/02/22 10:01
CRDT- 2002/01/15 10:00
PHST- 2002/01/15 10:00 [pubmed]
PHST- 2002/02/22 10:01 [medline]
PHST- 2002/01/15 10:00 [entrez]
AID - S1010794001010879 [pii]
AID - 10.1016/s1010-7940(01)01087-9 [doi]
PST - ppublish
SO  - Eur J Cardiothorac Surg. 2002 Jan;21(1):50-6. doi: 10.1016/s1010-7940(01)01087-9.