Hostname: page-component-848d4c4894-4hhp2 Total loading time: 0 Render date: 2024-05-11T19:22:09.741Z Has data issue: false hasContentIssue false
  • English
  • Français

Management of hypoplastic left heart syndrome with intact atrial septum: a two-centre experience

Published online by Cambridge University Press:  08 September 2015

Shyam K. Sathanandam ,
Ranjit Philip ,
David Gamboa ,
Andrew Van Bergen ,
Michel N. Ilbawi ,
Christopher Knott-Craig ,
B. Rush Waller ,
Alexander J. Javois  and
Bettina F. Cuneo
Shyam K. Sathanandam
Affiliation:
Advocate Children’s Hospital, Oak Lawn, Illinois, United States of America University of Tennessee Health Science Center, LeBonheur Children’s Hospital Memphis, Tennessee, United States of America
Ranjit Philip*
Affiliation:
University of Tennessee Health Science Center, LeBonheur Children’s Hospital Memphis, Tennessee, United States of America
David Gamboa
Affiliation:
Advocate Children’s Hospital, Oak Lawn, Illinois, United States of America School of Medicine, The University of Utah, Salt Lake City, Utah, United States of America
Andrew Van Bergen
Affiliation:
Advocate Children’s Hospital, Oak Lawn, Illinois, United States of America
Michel N. Ilbawi
Affiliation:
Advocate Children’s Hospital, Oak Lawn, Illinois, United States of America
Christopher Knott-Craig
Affiliation:
University of Tennessee Health Science Center, LeBonheur Children’s Hospital Memphis, Tennessee, United States of America
B. Rush Waller
Affiliation:
University of Tennessee Health Science Center, LeBonheur Children’s Hospital Memphis, Tennessee, United States of America
Alexander J. Javois
Affiliation:
Advocate Children’s Hospital, Oak Lawn, Illinois, United States of America
Bettina F. Cuneo
Affiliation:
Advocate Children’s Hospital, Oak Lawn, Illinois, United States of America Children’s Hospital Colorado, Aurora, Colorado, United States of America
*
Correspondence to: R. Philip, MD, Division of Pediatric Cardiology, Le Bonheur Children’s Hospital, Heart Institute, 3rd Floor, 50 N. Dunlap Street, Memphis, TN 38103, United States of America. Tel: +901- 287-5092; Fax: +901-287-4646; E-mail: rphilip@uthsc.edu
Get access Rights & Permissions [Opens in a new window]

Abstract

Introduction

Hypoplastic left heart syndrome with an intact atrial septum is a poor predictor of outcomes. Prenatal assessment of pulmonary venous Doppler and emergent postnatal cardiac intervention may be associated with better outcomes.

Materials and methods

A retrospective review of all hypoplastic left heart syndrome patients in two centres over a 5-year period was performed. Group 1 included patients with adequate inter-atrial communication. Group 2 included patients with prenatal diagnosis with an intact atrial septum who had immediate transcatheter intervention. Group 3 included patients with intact atrial septum who were not prenatally diagnosed and underwent either delayed intervention or no intervention before stage 1 palliation. Primary outcome was survival up to stage 2 palliation.

Results

The incidence of hypoplastic left heart syndrome with a restrictive atrial communication was 11.2% (n=19 of 170). Overall survival to stage 2 or heart transplantation was 85% and 67% for Groups 1 and 2, respectively (n=129/151, n=8/12; p=0.03), and 0% (n=0/7) for Group 3. Survival benefits were observed between Groups 2 and 3 (p<0.001). Foetal pulmonary vein Doppler reverse/forward velocity time integral ratio of ⩾18% (sensitivity, 0.99, 95% CI, 0.58–1; specificity, 0.99, 95% CI, 0.96–1) was predictive of the need for emergent left atrial decompression.

Conclusion

Using a multidisciplinary approach and foetal pulmonary vein Doppler, time-saving measures can be instituted by delivering prenatally diagnosed neonates with hypoplastic left heart syndrome with intact atrial septum close to the cardiac catheterisation suite where left atrial decompression can be performed quickly and safely that may improve survival.

Keywords

Hypoplastic left heart syndromeintact atrial septumcongenital heart disease
Type
Original Articles
Information
Cardiology in the Young , Volume 26 , Issue 6 , August 2016 , pp. 1072 - 1081
Check for updates
Copyright
© Cambridge University Press 2015 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1. Rychik, J, Rome, JJ, Collins, MH, DeCampli, WM, Spray, TL. The hypoplastic left heart syndrome with intact atrial septum: atrial morphology, pulmonary vascular histopathology and outcome. J Am Coll Cardiol 1999; 34: 554–560.CrossRefGoogle ScholarPubMed
2. Atz, AM, Feinstein, JA, Jonas, RA, Perry, SB, Wessel, DL. Preoperative management of pulmonary venous hypertension in hypoplastic left heart syndrome with restrictive atrial septal defect. Am J Cardiol 1999; 83: 1224–1228.CrossRefGoogle ScholarPubMed
3. Boucek, MM, Mathis, CM, Kanakriyeh, MS, McCormack, J, Gundry, SR, Bailey, LL. Management of the critically restrictive atrial septal defect awaiting infant cardiac transplantation. Am J Cardiol 1992; 70: 559.Google Scholar
4. Vlahos, AP, Lock, JE, McElhinney, DB, van der Velde, ME. Hypoplastic left heart syndrome with intact or highly restrictive atrial septum: outcome after neonatal transcatheter atrial septostomy. Circulation 2004; 109: 2326–2330.CrossRefGoogle ScholarPubMed
5. Kahn Ali, MA, Bricker, JT, Mullins, CE, Al Yousef, S, Nihill, MR, Vargo, TA. Blade atrial septostomy: experience with the first 50 procedures. Catheter Cardiovasc Diagn 1991; 23: 257–262.Google Scholar
6. Seib, PM, Faulkner, SC, Erickson, CC, et al. Blade and balloon atrial septostomy for left heart decompression in patients with severe ventricular dysfunction on extracorporeal membrane oxygenation. Catheter Cardiovasc Interv 1999; 46: 179–186.3.0.CO;2-W>CrossRefGoogle ScholarPubMed
7. Park, SC, Neches, WH, Mullins, CE, et al. Blade atrial septostomy: collaborative study. Circulation 1982; 66: 258–266.CrossRefGoogle ScholarPubMed
8. Coe, JY, Timisky, J, Villnave, DJ, Trevenen, C. Transcatheter puncture of the atrial septum using a new radio frequency catheter. Catheter Cardiovasc Interv 1998; 44: 116.Google Scholar
9. Justino, H, Benson, LN, Nykanen, DG. Transcatheter creation of an atrial septal defect using radio frequency perforation. Catheter Cardiovasc Interv 2001; 54: 83–87.CrossRefGoogle Scholar
10. Du Marchie Sarvaas, GJ, Trivedi, KR, Hornberger, LK, Jin Lee, K, Kirsch, JA, Benson, LN. Radio frequency – assisted atrial septoplasty for an intact atrial septum in complex congenital heart disease. Catheter Cardiovasc Interv 2002; 56: 412–415.CrossRefGoogle Scholar
11. Michelfelder, E, Gomez, C, Border, W, Gottliebson, W, Franklin, C. Predictive value of fetal pulmonary venous flow patterns in identifying the need for atrial septoplasty in the newborn with hypoplastic left ventricle. Circulation 2005; 112: 2974–2979.CrossRefGoogle ScholarPubMed
12. Divanovic, A, Hor, K, Cnota, J, Hirsch, R, Kinsel-Ziter, M, Michelfelder, E. Prediction and perinatal management of severely restrictive atrial septum in fetuses with critical left heart obstruction: clinical experience using pulmonary venous Doppler analysis. J Thorac Cardiovasc Surg 2011; 141: 988–994.CrossRefGoogle ScholarPubMed
13. Javois, AJ, Van Bergen, AH, Cuneo, BF, Husayni, TS. Novel approach to the newborn with hypoplastic left heart syndrome and intact atrial septum. Catheter Cardiovasc Interv 2005; 66: 268–272.CrossRefGoogle Scholar
14. Litwin, SB, Plauth, WH Jr, Jones, JE, Bernard, WF. Appraisal of surgical atrial septectomy for transposition of great arteries. Circulation 1971; 43: 17–110.CrossRefGoogle Scholar
15. Park, SC, Neches, WH, Zuberbuhler, JR, et al. Clinical use of blade atrial septostomy. Circulation 1978; 58: 600–606.CrossRefGoogle ScholarPubMed
16. Mani, S, Venkitachalam, CG, Subramaniam, R, et al. Blade and balloon atrial septostomy for creation of an adequate interatrial communication. Indian Heart J 1994; 46: 161–164.Google ScholarPubMed
17. Rao, PS. Transcatheter blade atrial septostomy. Catheter Cardiovasc Diagn 1984; 10: 335–342.CrossRefGoogle ScholarPubMed
18. Cope, C. Technique for transseptal catheterization of the left atrium: preliminary report. J Thorac Surg 1959; 37: 482–486.CrossRefGoogle ScholarPubMed
19. Ross, J Jr, Braunwald, E, Morrow, AG. Transseptal left atrial puncture: new technique for the measurement of left atrial pressure in men. Am J Cardiol 1959; 1059: 653–655.CrossRefGoogle Scholar
20. Nykanen, DG, Pihkala, J, Taylor, GP, Benson, LN. Radio frequency assisted perforation of the atrial septum in a swine model: feasibility, biophysical and histological characteristics. Circulation 1999; 100: 1804.Google Scholar
21. Graziano, JN, Heidelberger, KP, Ensing, GJ, Gomez, CA, Ludomirsky, A. The influence of a restrictive atrial septal defect on pulmonary vascular morphology in patients with hypoplastic left heart syndrome. Pediatr Cardiol 2002; 23: 145–151.CrossRefGoogle ScholarPubMed
22. Marshall, AC, Van der Velde, ME, Tworetzky, W, et al. Creation of an atrial septal defect in utero for fetuses with hypoplastic left heart syndrome and intact or highly restrictive atrial septum. Circulation 2004; 110: 253–258.CrossRefGoogle ScholarPubMed
23. Vida, VL, Bacha, EA, Larrazabal, A, et al. Hypoplastic left heart syndrome with intact or highly restrictive atrial septum: surgical experience from a single center. Ann Thorac Surg 2007; 84: 581–585.CrossRefGoogle ScholarPubMed
24. Tworetzky, W, Wilkins-Haug, L, Jennings, RW, et al. Balloon dilation of severe aortic stenosis in the fetus: potential for prevention of hypoplastic left heart syndrome: candidate selection, technique, and results of successful intervention. Circulation 2004; 110: 2125–2131.CrossRefGoogle ScholarPubMed
25. Walayat, M, Cooper, SG, Sholler, GF. Transesophageal echocardiographic guidance of blade atrial septostomy in children. Catheter Cardiovasc Interv 2001; 52: 200–202.3.0.CO;2-V>CrossRefGoogle ScholarPubMed
26. Taketazu, M, Barrea, C, Smallhorn, JF, Wilson, GJ, Hornberger, LK. Intrauterine pulmonary venous flow and restrictive foramen ovale in fetal hypoplastic left heart syndrome. J Am Coll Cardiol 2004; 43: 1902–1907.CrossRefGoogle ScholarPubMed
27. Cheatham, JP. Intervention in the critically ill neonate and infant with hypoplastic left heart syndrome and intact atrial septum. J Interv Cardiol 2001; 14: 357–366.CrossRefGoogle ScholarPubMed
28. Perry, SB, Lang, P, Keane, JF, Jonas, RA, Sanders, SP, Lock, JE. Creation and maintenance of an adequate interatrial communication in left atrioventricular valve atresia or stenosis. Am J Cardiol 1986; 58: 622–626.CrossRefGoogle ScholarPubMed
29. Akinturk, H, Michel-Behnke, I, Valeske, K, et al. Hybrid transcatheter-surgical palliation: basis for univentricular or biventricular repair: the Giessen experience. Pediatr Cardiol 2007; 28: 79–87.CrossRefGoogle ScholarPubMed
30. Venugopal, PS, Luna, KP, Anderson, DR, et al. Hybrid procedure as an alternative to surgical palliation of high-risk infants with hypoplastic left heart syndrome and its variants. J Thorac Cardiovasc Surg 2010; 139: 1211–1215.CrossRefGoogle ScholarPubMed