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Staged Repair of Pulmonic Stenosis and Hypoplastic Right Ventricle

Staged Repair of Pulmonic Stenosis and Hypoplastic Right Ventricle Abstract ISOLATED pulmonic valvular stenosis can be successfully treated by either closed or open techniques. Good results have been obtained with a transventricular valvulotome,1 under direct vision with hypothermic circulatory arrest,2 or with extracorporeal circulation.3 When severe pulmonic stenosis is associated with a diminutive right ventricular cavity, the prognosis is poor and most of these patients die in infancy.4 This report concerns two infants with this combination of anomalies and who were successfully treated by a two-stage surgical procedure. Initial closed transventricular valvotomy was performed under emergency conditions in each patient. Subsequently a definitive correction involving a more precise valvotomy and prosthetic enlargement of the right ventricular outflow tract was performed with cardiopulmonary bypass. Analysis of previous and present results suggests that prognosis is improved by a two-stage repair, consisting of an initial rapid palliative valvotomy and a later definitive procedure. Case Histories Case 1. —A 1-year-old References 1. Brock, R.C.: Pulmonary Valvulotomy for the Relief of Congenital Pulmonic Stenosis , Brit Med J 1:1121, 1948.Crossref 2. Blount, S.G., Jr., et al: Isolated Valvular Pulmonic Stenosis, Clinical and Physiologic Response to Open Valvuloplasty , Circulation 10:161, 1954.Crossref 3. McGoon, D.C., and Kirklin, J.W.: Pulmonic Stenosis With Intact Ventricular Septum Using Extracorporeal Circulation , Circulation 17:180, 1958.Crossref 4. Benton, J.W., Jr., et al: Pulmonary Atresia and Stenosis With Intact Ventricular Septum , Amer J Dis Child 104:161, 1962. 5. Moss, A.J., et al: Determine Blood Pressure in Infants: Use of the Flush Technique , Calif Med 87:166, 1957. 6. Enthoven, R.; Dunst, M.; and Richman, B.: Congenital Hypoplasia of the Right Ventricle and Tricuspid Valve With Survival Into Adult Life , Amer J Cardiol 11:532, 1963.Crossref 7. Medd, W.E., et al: Isolated Hypoplasia of the Right Ventricle and Tricuspid Valve in Siblings , Brit Heart J 23:25, 1961.Crossref 8. Kessler, A., and Adams, P.: Association of Transposition of the Great Vessels and Rudimentary Right Ventricle, With and Without Tricuspid Atresia , Pediatrics 19:851, 1957. 9. Elliot, L.P.; Adams, P., Jr.; and Edwards, J.E.: Pulmonary Atresia With Intact Ventricular Septum , Brit Heart J 25:489, 1963.Crossref 10. Davignon, A.L., et al: Congenital Pulmonary Atresia With Intact Ventricular Septum: Clinicopathologic Correlation of Two Anatomic Types , Amer Heart J 62:591, 1961.Crossref 11. Williams, J.; Barratt-Boys, B.G.; and Lowe, J.B.: Underdeveloped Right Ventricle and Pulmonary Stenosis , Amer J Cardiol 11:458, 1963.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Surgery American Medical Association

Staged Repair of Pulmonic Stenosis and Hypoplastic Right Ventricle

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Publisher
American Medical Association
Copyright
Copyright © 1965 American Medical Association. All Rights Reserved.
ISSN
0004-0010
eISSN
1538-3644
DOI
10.1001/archsurg.1965.01320160051011
Publisher site
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Abstract

Abstract ISOLATED pulmonic valvular stenosis can be successfully treated by either closed or open techniques. Good results have been obtained with a transventricular valvulotome,1 under direct vision with hypothermic circulatory arrest,2 or with extracorporeal circulation.3 When severe pulmonic stenosis is associated with a diminutive right ventricular cavity, the prognosis is poor and most of these patients die in infancy.4 This report concerns two infants with this combination of anomalies and who were successfully treated by a two-stage surgical procedure. Initial closed transventricular valvotomy was performed under emergency conditions in each patient. Subsequently a definitive correction involving a more precise valvotomy and prosthetic enlargement of the right ventricular outflow tract was performed with cardiopulmonary bypass. Analysis of previous and present results suggests that prognosis is improved by a two-stage repair, consisting of an initial rapid palliative valvotomy and a later definitive procedure. Case Histories Case 1. —A 1-year-old References 1. Brock, R.C.: Pulmonary Valvulotomy for the Relief of Congenital Pulmonic Stenosis , Brit Med J 1:1121, 1948.Crossref 2. Blount, S.G., Jr., et al: Isolated Valvular Pulmonic Stenosis, Clinical and Physiologic Response to Open Valvuloplasty , Circulation 10:161, 1954.Crossref 3. McGoon, D.C., and Kirklin, J.W.: Pulmonic Stenosis With Intact Ventricular Septum Using Extracorporeal Circulation , Circulation 17:180, 1958.Crossref 4. Benton, J.W., Jr., et al: Pulmonary Atresia and Stenosis With Intact Ventricular Septum , Amer J Dis Child 104:161, 1962. 5. Moss, A.J., et al: Determine Blood Pressure in Infants: Use of the Flush Technique , Calif Med 87:166, 1957. 6. Enthoven, R.; Dunst, M.; and Richman, B.: Congenital Hypoplasia of the Right Ventricle and Tricuspid Valve With Survival Into Adult Life , Amer J Cardiol 11:532, 1963.Crossref 7. Medd, W.E., et al: Isolated Hypoplasia of the Right Ventricle and Tricuspid Valve in Siblings , Brit Heart J 23:25, 1961.Crossref 8. Kessler, A., and Adams, P.: Association of Transposition of the Great Vessels and Rudimentary Right Ventricle, With and Without Tricuspid Atresia , Pediatrics 19:851, 1957. 9. Elliot, L.P.; Adams, P., Jr.; and Edwards, J.E.: Pulmonary Atresia With Intact Ventricular Septum , Brit Heart J 25:489, 1963.Crossref 10. Davignon, A.L., et al: Congenital Pulmonary Atresia With Intact Ventricular Septum: Clinicopathologic Correlation of Two Anatomic Types , Amer Heart J 62:591, 1961.Crossref 11. Williams, J.; Barratt-Boys, B.G.; and Lowe, J.B.: Underdeveloped Right Ventricle and Pulmonary Stenosis , Amer J Cardiol 11:458, 1963.Crossref

Journal

Archives of Surgery – American Medical Association

Published: Oct 1, 1965

References

  • Pulmonary Valvulotomy for the Relief of Congenital Pulmonic Stenosis
    Brock , R.C.
  • Isolated Valvular Pulmonic Stenosis, Clinical and Physiologic Response to Open Valvuloplasty
    Blount , S.G., Jr., et al
  • Pulmonic Stenosis With Intact Ventricular Septum Using Extracorporeal Circulation
    McGoon , D.C., and Kirklin, J.W.
  • Pulmonary Atresia and Stenosis With Intact Ventricular Septum
    Benton , J.W., Jr., et al
  • Determine Blood Pressure in Infants: Use of the Flush Technique
    Moss , A.J., et al
  • Congenital Hypoplasia of the Right Ventricle and Tricuspid Valve With Survival Into Adult Life
    Enthoven , R.; Dunst, M.; and Richman, B.
  • Isolated Hypoplasia of the Right Ventricle and Tricuspid Valve in Siblings
    Medd , W.E., et al
  • Association of Transposition of the Great Vessels and Rudimentary Right Ventricle, With and Without Tricuspid Atresia
    Kessler , A., and Adams, P.
  • Pulmonary Atresia With Intact Ventricular Septum
    Elliot , L.P.; Adams, P., Jr.; and Edwards, J.E.
  • Congenital Pulmonary Atresia With Intact Ventricular Septum: Clinicopathologic Correlation of Two Anatomic Types
    Davignon , A.L., et al
  • Underdeveloped Right Ventricle and Pulmonary Stenosis
    Williams , J.; Barratt-Boys, B.G.; and Lowe, J.B.