CLINICAL STUDY: CARDIAC SURGERY
Survival analysis and risk factors for mortality in transplantation and staged surgery for hypoplastic left heart syndrome
Pamela C. Jenkins, MD, PhD*,
Michael F. Flanagan, MD, FACC*,
Kathy J. Jenkins, MD, MPH ,
James D. Sargent, MD*,
Charles E. Canter, MD, FACC ,
Richard E. Chinnock, MD ,
Robert N. Vincent, MD, FACC||,
Anna N. A. Tosteson, ScD¶ and
Gerald T. O’Connor, PhD, DSc, FACC¶
* Department of Pediatrics, Dartmouth Medical School, Hanover, New Hampshire, USA
Department of Cardiology, Children’s Hospital, Boston, Massachusetts, USA
Department of Cardiology, St. Louis Children’s Hospital, St. Louis, Missouri, USA
Department of Cardiology, Loma Linda University Children’s Hospital, Loma Linda, California, USA
|| Department of Cardiology, Emory University School of Medicine, Atlanta, Georgia, USA
¶ Departments of Medicine and Community and Family Medicine, Dartmouth Medical School, Hanover, New Hampshire, USA
Manuscript received September 9, 1999;
revised manuscript received March 15, 2000,
accepted May 24, 2000.
Reprint requests and correspondence: Dr. Pamela C. Jenkins, Department of Pediatrics, Dartmouth Medical School, Hanover, New Hampshire 03755
pcj{at}hitchcock.org
OBJECTIVES
We compared survival in treatment strategies and determined risk factors for one-year mortality for hypoplastic left heart syndrome (HLHS) using intention-to-treat analysis.
BACKGROUND
Staged revision of the native heart and transplantation as treatments for HLHS have been compared in treatment-received analyses, which can bias results.
METHODS
Data on 231 infants with HLHS, born between 1989 and 1994 and intended for surgery, were collected from four pediatric cardiac surgical centers. Status at last contact for survival analysis and mortality at one year for risk factor analysis were the outcome measures.
RESULTS
Survival curves showed improved survival for patients intended for transplantation over patients intended for staged surgery. One-year survival was 61% for transplantation and 42% for staged surgery (p < 0.01); five-year survival was 55% and 38%, respectively (p < 0.01). Survival curves adjusted for preoperative differences were also significantly different (p < 0.001). Waiting-list mortality accounted for 63% of first-year deaths in the transplantation group. Mortality with stage 1 surgery accounted for 86% of that strategy’s first-year mortality. Birth weight <3 kg (odds ratio [OR] 2.4), highest creatinine  2 mg/dL (OR 4.7), restrictive atrial septal defect (OR 2.7) and, in staged surgery, atresia of one (OR 4.2) or both (OR 11.0) left-sided valves produced a higher risk for one-year mortality.
CONCLUSIONS
Transplantation produced significantly higher survival at all ages up to seven years. Patients with atresia of one or both valves do poorly in staged surgery and have significantly higher survival with transplantation. This information may be useful in directing patients to the better strategy for them.
|
Abbreviations and Acronyms
| | ASD | = atrial septal defect | | HLHS | = hypoplastic left heart syndrome | | OR | = odds ratio | | ROC | = relative operating characteristic | | RV | = right ventricle, right ventricular | | TR | = tricuspid regurgitation |
|
This article has been cited by other articles:
|
|
|
|
 
R. G. Ohye, J. W. Gaynor, N. S. Ghanayem, C. S. Goldberg, P. C. Laussen, P. C. Frommelt, J. W. Newburger, G. D. Pearson, S. Tabbutt, G. Wernovsky, et al.
Design and rationale of a randomized trial comparing the Blalock-Taussig and right ventricle-pulmonary artery shunts in the Norwood procedure.
J. Thorac. Cardiovasc. Surg.,
October 1, 2008;
136(4):
968 - 975.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. A. Glatz, R. T. Fedderly, N. S. Ghanayem, and J. S. Tweddell
Impact of Mitral Stenosis and Aortic Atresia on Survival in Hypoplastic Left Heart Syndrome
Ann. Thorac. Surg.,
June 1, 2008;
85(6):
2057 - 2062.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
F. J. Fricker
Hypoplastic Left Heart Syndrome: Diagnosis and Early Management
NeoReviews,
June 1, 2008;
9(6):
e253 - e259.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
B. Alsoufi, T. Karamlou, B. W. McCrindle, and C. A. Caldarone
Management options in neonates and infants with critical left ventricular outflow tract obstruction
Eur. J. Cardiothorac. Surg.,
June 1, 2007;
31(6):
1013 - 1021.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. E. Canter, R. E. Shaddy, D. Bernstein, D. T. Hsu, M. R.K. Chrisant, J. K. Kirklin, K. R. Kanter, R. S.D. Higgins, E. D. Blume, D. N. Rosenthal, et al.
Indications for Heart Transplantation in Pediatric Heart Disease: A Scientific Statement From the American Heart Association Council on Cardiovascular Disease in the Young; the Councils on Clinical Cardiology, Cardiovascular Nursing, and Cardiovascular Surgery and Anesthesia; and the Quality of Care and Outcomes Research Interdisciplinary Working Group
Circulation,
February 6, 2007;
115(5):
658 - 676.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
B. Alsoufi, J. Bennetts, S. Verma, and C. A. Caldarone
New Developments in the Treatment of Hypoplastic Left Heart Syndrome
Pediatrics,
January 1, 2007;
119(1):
109 - 117.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
K. S. Sundareswaran, K. R. Kanter, H. D. Kitajima, R. Krishnankutty, J. F. Sabatier, W. J. Parks, S. Sharma, A. P. Yoganathan, and M. Fogel
Impaired power output and cardiac index with hypoplastic left heart syndrome: a magnetic resonance imaging study.
Ann. Thorac. Surg.,
October 1, 2006;
82(4):
1267 - 1277.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
W. T. Mahle, K. J. Visconti, M. C. Freier, S. M. Kanne, W. G. Hamilton, A. M. Sharkey, R. E. Chinnock, K. J. Jenkins, P. K. Isquith, T. G. Burns, et al.
Relationship of Surgical Approach to Neurodevelopmental Outcomes in Hypoplastic Left Heart Syndrome
Pediatrics,
January 1, 2006;
117(1):
e90 - e97.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
U Theilen and L Shekerdemian
The intensive care of infants with hypoplastic left heart syndrome
Arch. Dis. Child. Fetal Neonatal Ed.,
March 1, 2005;
90(2):
F97 - F102.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. J. Lodge, J. Rychik, S. C. Nicolson, R. F. Ittenbach, T. L. Spray, and J. W. Gaynor
Improving Outcomes in Functional Single Ventricle and Total Anomalous Pulmonary Venous Connection
Ann. Thorac. Surg.,
November 1, 2004;
78(5):
1688 - 1695.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. A. Connor, R. R. Arons, M. Figueroa, and K. M. Gebbie
Clinical Outcomes and Secondary Diagnoses for Infants Born With Hypoplastic Left Heart Syndrome
Pediatrics,
August 1, 2004;
114(2):
e160 - e165.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. C. Marshall, M. E. van der Velde, W. Tworetzky, C. A. Gomez, L. Wilkins-Haug, C. B. Benson, R. W. Jennings, and J. E. Lock
Creation of an Atrial Septal Defect In Utero for Fetuses With Hypoplastic Left Heart Syndrome and Intact or Highly Restrictive Atrial Septum
Circulation,
July 20, 2004;
110(3):
253 - 258.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. Taketazu, C. Barrea, J. F. Smallhorn, G. J. Wilson, and L. K. Hornberger
Intrauterine pulmonary venous flow and restrictive foramen ovale in fetal hypoplastic left heart syndrome
J. Am. Coll. Cardiol.,
May 19, 2004;
43(10):
1902 - 1907.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. P. Vlahos, J. E. Lock, D. B. McElhinney, and M. E. van der Velde
Hypoplastic Left Heart Syndrome With Intact or Highly Restrictive Atrial Septum: Outcome After Neonatal Transcatheter Atrial Septostomy
Circulation,
May 18, 2004;
109(19):
2326 - 2330.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. A. Kon, L. Ackerson, and B. Lo
How Pediatricians Counsel Parents When No "Best-Choice" Management Exists: Lessons to Be Learned From Hypoplastic Left Heart Syndrome
Arch Pediatr Adolesc Med,
May 1, 2004;
158(5):
436 - 441.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
K. Kuhlthau, T. G. G. Ferris, and L. I. Iezzoni
Risk Adjustment for Pediatric Quality Indicators
Pediatrics,
January 1, 2004;
113(1/S1):
210 - 216.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. B. Mitchell, D. N. Campbell, M. M. Boucek, H. M. Sondheimer, K. C. Chan, D. D. Ivy, B. Pietra, and T. Mackenzie
Mechanical limitation of pulmonary blood flow facilitates heart transplantation in older infants with hypoplastic left heart syndrome
Eur. J. Cardiothorac. Surg.,
May 1, 2003;
23(5):
735 - 742.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
D. A. Ashburn, B. W. McCrindle, C. I. Tchervenkov, M. L. Jacobs, G. K. Lofland, E. L. Bove, T. L. Spray, W. G. Williams, and E. H. Blackstone
Outcomes after the Norwood operation in neonates with critical aortic stenosis or aortic valve atresia
J. Thorac. Cardiovasc. Surg.,
May 1, 2003;
125(5):
1070 - 1082.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R.-K. R. Chang, A. Y. Chen, and T. S. Klitzner
Clinical Management of Infants With Hypoplastic Left Heart Syndrome in the United States, 1988-1997
Pediatrics,
August 1, 2002;
110(2):
292 - 298.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
B. W. Duncan, G. L. Rosenthal, T. K. Jones, and F. M. Lupinetti
First-stage palliation of complex univentricular cardiac anomalies in older infants
Ann. Thorac. Surg.,
December 1, 2001;
72(6):
2077 - 2080.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
D. C. Drinkwater Jr, A. S. Aharon, S. V. Quisling, D. Dodd, V. S. Reddy, A. Kavanaugh-McHugh, T. Doyle, N. R. Patel, F. E. Barr, J. K. Kambam, et al.
Modified Norwood operation for hypoplastic left heart syndrome
Ann. Thorac. Surg.,
December 1, 2001;
72(6):
2081 - 2087.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
P. C. Jenkins, M. F. Flanagan, J. D. Sargent, C. E. Canter, R. E. Chinnock, K. J. Jenkins, R. N. Vincent, G. T. O'Connor, and A. N. A. Tosteson
A comparison of treatment strategies for hypoplastic left heart syndrome using decision analysis
J. Am. Coll. Cardiol.,
October 1, 2001;
38(4):
1181 - 1187.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
