Operations for subaortic stenosis in univentricular hearts

Tom R. Karl, Kevin G. Watterson, Shunji Sano, Roger B B Mee

Research output: Contribution to journalArticle

55 Citations (Scopus)

Abstract

Optimal prevention and treatment of subaortic stenosis (SAS) in the univentricular heart with subaortic outlet chamber and high pulmonary blood flow remains controversial, especially when complicated by aortic arch obstruction. Herein we analyze our surgical results. Group 1 consisted of 11 infants (mean age, 10 days) with univentricular heart and SAS. Ten required repair of interrupted aortic arch (n = 7) or coarctation with hypoplastic arch (n = 7). Four patients had relief of SAS by either Damus-Kaye-Stansel connection (n = 2) or aortopulmonary window (n = 2), with three operative deaths and one late death. Six had one-stage arterial switch and atrial septectomy with arch repair ( 5 6) with one operative death and one late death. Two survivors have progressed to bidirectional cavopulmonary shunt, a third has had a Fontan operation, and a fourth awaits Fontan. In group 2, 11 children required operation for acquired SAS after pulmonary artery banding. Nine have progressed to Fontan operation with either staged (n = 3) or concurrent (n = 6) relief of SAS by Damus-Kaye-Stansel connection or subaortic resection. Fontan mortality was 11% (70% confidence limits, 2% to 32%). Group 3 consisted of 3 patients without pulmonary artery banding who had SAS diagnosed at Fontan evaluation. All 3 survived Fontan operation and relief of SAS by Damus-Kaye-Stansel connection or subaortic resection. Group 4 consisted of 1 patient with previous pulmonary artery banding (no SAS) who underwent Fontan operation but required Damus-Kaye-Stansel connection 30 months later for SAS. We conclude that arterial switch, arch repair, and atrial septectomy effectively palliate newborns with univentricular heart and SAS, although ultimate Fontan suitability is yet to be established. Both Damus-Kaye-Stansel connection and subaortic resection provide good long-term relief of SAS in select patients, and previous or concurrent treatment of SAS does not preclude successful Fontan operation in otherwise suitable patients.

Original languageEnglish
Pages (from-to)420-428
Number of pages9
JournalAnnals of Thoracic Surgery
Volume52
Issue number3
DOIs
Publication statusPublished - 1991
Externally publishedYes

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Pathologic Constriction
Fontan Procedure
Thoracic Aorta
Pulmonary Artery
Survivors
Newborn Infant
Lung
Mortality
Therapeutics

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery

Cite this

Operations for subaortic stenosis in univentricular hearts. / Karl, Tom R.; Watterson, Kevin G.; Sano, Shunji; Mee, Roger B B.

In: Annals of Thoracic Surgery, Vol. 52, No. 3, 1991, p. 420-428.

Research output: Contribution to journalArticle

Karl, Tom R. ; Watterson, Kevin G. ; Sano, Shunji ; Mee, Roger B B. / Operations for subaortic stenosis in univentricular hearts. In: Annals of Thoracic Surgery. 1991 ; Vol. 52, No. 3. pp. 420-428.
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abstract = "Optimal prevention and treatment of subaortic stenosis (SAS) in the univentricular heart with subaortic outlet chamber and high pulmonary blood flow remains controversial, especially when complicated by aortic arch obstruction. Herein we analyze our surgical results. Group 1 consisted of 11 infants (mean age, 10 days) with univentricular heart and SAS. Ten required repair of interrupted aortic arch (n = 7) or coarctation with hypoplastic arch (n = 7). Four patients had relief of SAS by either Damus-Kaye-Stansel connection (n = 2) or aortopulmonary window (n = 2), with three operative deaths and one late death. Six had one-stage arterial switch and atrial septectomy with arch repair ( 5 6) with one operative death and one late death. Two survivors have progressed to bidirectional cavopulmonary shunt, a third has had a Fontan operation, and a fourth awaits Fontan. In group 2, 11 children required operation for acquired SAS after pulmonary artery banding. Nine have progressed to Fontan operation with either staged (n = 3) or concurrent (n = 6) relief of SAS by Damus-Kaye-Stansel connection or subaortic resection. Fontan mortality was 11{\%} (70{\%} confidence limits, 2{\%} to 32{\%}). Group 3 consisted of 3 patients without pulmonary artery banding who had SAS diagnosed at Fontan evaluation. All 3 survived Fontan operation and relief of SAS by Damus-Kaye-Stansel connection or subaortic resection. Group 4 consisted of 1 patient with previous pulmonary artery banding (no SAS) who underwent Fontan operation but required Damus-Kaye-Stansel connection 30 months later for SAS. We conclude that arterial switch, arch repair, and atrial septectomy effectively palliate newborns with univentricular heart and SAS, although ultimate Fontan suitability is yet to be established. Both Damus-Kaye-Stansel connection and subaortic resection provide good long-term relief of SAS in select patients, and previous or concurrent treatment of SAS does not preclude successful Fontan operation in otherwise suitable patients.",
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