Hypoplastic Left Heart Syndrome

1. Front Matter

   Pages i-xiii

   PDF

2. The Epidemiology and Genetics of Hypoplastic Left Heart Syndrome

   • Elizabeth Goldmuntz

   Pages 1-8

3. Fetal Development and Prenatal Evaluation of Hypoplastic Left Heart Syndrome

   • Meryl S. Cohen

   Pages 9-28

4. Delivery and Obstetrical Issues

   • Samuel Parry, Sara J. Marder

   Pages 29-37

5. Diagnostic Assessment of Hypoplastic Left Heart Syndrome

   • Jack Rychik

   Pages 39-67

6. Pre-Operative Management of Hypoplastic Left Heart Syndrome

   • Sarah Tabbutt, Gil Wernovsky

   Pages 69-87

7. Staged Reconstruction for HLHS

   • Thomas L. Spray

   Pages 89-103

8. Postoperative Management after First-Stage Palliation

   • Gil Wernovsky, Doff B. McElhinney, Sarah Tabbutt

   Pages 105-128

9. Staged Reconstruction For HLHS

   • Tom R. Karl

   Pages 129-148

10. Staged Reconstruction for HLHS

    • J. William Gaynor

    Pages 149-166

11. Anesthetic Management for HLHS

    • James M. Steven, Susan C. Nicolson

    Pages 167-191

12. The Role Of Cardiac Catheterization During Staged Reconstruction

    • Jacqueline Kreutzer, Jonathan J. Rome

    Pages 193-228

13. Nursing Care During Staged Reconstruction

    • Rhonda Foltz, Kathryn M. Dodds

    Pages 229-240

14. Infant Heart Transplantation

    • Timothy M. Hoffman, Thomas L. Spray

    Pages 241-250

15. The Neurology of Hypoplastic Left Heart Syndrome

    • Robert Ryan Clancy

    Pages 251-272

16. Feeding Issues and Somatic Growth

    • Mitchell I. Cohen

    Pages 273-288

17. Cardiac Structure And Function After Staged Reconstruction

    • William Mahle

    Pages 289-306

18. Arrhythmias After Surgery

    • Ronn E. Tanel, Larry A. Rhodes

    Pages 307-328

19. Exercise Physiology and Capacity

    • Steven Paridon

    Pages 329-346

20. Complications After the Fontan Operation

    • Jack Rychik

    Pages 347-359

Despite approximately 50 years of progress in the management of com­ plex congenital heart disease, hypoplastic left heart syndrome (HLHS) has been one of the last cardiac malformations to yield to effective surgical treat­ ment. The surgical therapies for hypoplastic left heart syndrome have evolved by two quite disparate routes. One approach has been the staged reconstruc­ tion operations utilizing an initial operation pioneered by many but developed by Dr. William Norwood and subsequently modified by many other investigators. The first stage reconstruction operation has been refined and standardized such that the majority of infants now proceed to second and third stage reconstruc­ tive operations culminating in a Pontan Kreutzer single ventricle physiology. An alternative surgical approach has been the development of neonatal and infant heart transplantation for HLHS. This approach also has had a signifi­ cant impact on the treatment of HLHS and other cardiac malformations and has been associated with good intermediate term results. Both of the thera­ peutic approaches devised for treatment of hypoplastic left heart syndrome have been effective and the outcomes are continuing to improve with each.

• cardiovascular
• congenital heart disease
• diagnostics
• genetics
• growth
• heart
• heart transplantation
• transplantation

• Division of Cardiology, Children’s Hospital of Philadelphia, Philadelphia, USA

  Jack Rychik, Gil Wernovsky

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