Outcomes after listing for primary transplantation for infants with unoperated-on non-hypoplastic left heart syndrome congenital heart disease: A multi-institutional study

Kristine J. Guleserian, Kenneth B. Schechtman, Jie Zheng, R. Erik Edens, Jeffrey P. Jacobs, William T. Mahle, Stephanie L. Emerson, David C. Naftel, James K. Kirklin, Elizabeth D. Blume, Charles E. Canter

Research output: Contribution to journalArticle

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Abstract

Background: Although heart transplantation has been used as the primary therapy for congenital heart lesions in infants other than hypoplastic left heart syndrome (HLHS), the outcomes in this group of patients have not been determined. Methods: We used the Pediatric Heart Transplant Study database (1993 to 2006) to compare outcomes of 388 infants aged < 6 months listed for HLHS, 161 with other congenital heart diseases (non-HLHS), and 145 with cardiomyopathy in early (1993 to 1999) and recent (2000 to 2006) eras. Results: The cardiomyopathy group was significantly (p < 0.001) different from the HLHS and non-HLHS groups at listing: more girls, older age, and a greater need for high-dose inotropes, mechanical ventilation, and/or mechanical circulatory support. Survival after listing was similar among the groups in the early era. Although outcomes after listing in HLHS and cardiomyopathy patients improved in the recent era, outcomes in non-HLHS patients did not. Survival at 1 and 5 years after listing was significantly worse (p < 0.001) for non-HLHS patients (51%, 48%) vs HLHS (71%, 61%), with age- and sex-adjusted hazard ratio (HR) of 1.79 (95% confidence interval, 1.152.77, p = 0.009) and CM (80%, 74%; HR, 2.72; 95% confidence interval, 1.594.67, p < 0.001) in the recent era. Post-transplant survival in both eras was not significantly different among the groups. Conclusion: Use of heart transplantation as primary therapy for non-HLHS infants has not improved over time and currently is associated with significantly poorer results vs HLHS and cardiomyopathy due to a higher risk for death before transplant.

Original languageEnglish (US)
Pages (from-to)1023-1032
Number of pages10
JournalJournal of Heart and Lung Transplantation
Volume30
Issue number9
DOIs
StatePublished - Sep 2011

Fingerprint

Hypoplastic Left Heart Syndrome
Heart Diseases
Transplantation
Cardiomyopathies
Heart Transplantation
Transplants
Confidence Intervals
Survival
Artificial Respiration
Databases
Pediatrics
Therapeutics

Keywords

  • congenital heart disease
  • heart transplantation
  • infants

ASJC Scopus subject areas

  • Transplantation
  • Cardiology and Cardiovascular Medicine
  • Pulmonary and Respiratory Medicine
  • Surgery

Cite this

Outcomes after listing for primary transplantation for infants with unoperated-on non-hypoplastic left heart syndrome congenital heart disease : A multi-institutional study. / Guleserian, Kristine J.; Schechtman, Kenneth B.; Zheng, Jie; Edens, R. Erik; Jacobs, Jeffrey P.; Mahle, William T.; Emerson, Stephanie L.; Naftel, David C.; Kirklin, James K.; Blume, Elizabeth D.; Canter, Charles E.

In: Journal of Heart and Lung Transplantation, Vol. 30, No. 9, 09.2011, p. 1023-1032.

Research output: Contribution to journalArticle

Guleserian, Kristine J. ; Schechtman, Kenneth B. ; Zheng, Jie ; Edens, R. Erik ; Jacobs, Jeffrey P. ; Mahle, William T. ; Emerson, Stephanie L. ; Naftel, David C. ; Kirklin, James K. ; Blume, Elizabeth D. ; Canter, Charles E. / Outcomes after listing for primary transplantation for infants with unoperated-on non-hypoplastic left heart syndrome congenital heart disease : A multi-institutional study. In: Journal of Heart and Lung Transplantation. 2011 ; Vol. 30, No. 9. pp. 1023-1032.
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abstract = "Background: Although heart transplantation has been used as the primary therapy for congenital heart lesions in infants other than hypoplastic left heart syndrome (HLHS), the outcomes in this group of patients have not been determined. Methods: We used the Pediatric Heart Transplant Study database (1993 to 2006) to compare outcomes of 388 infants aged < 6 months listed for HLHS, 161 with other congenital heart diseases (non-HLHS), and 145 with cardiomyopathy in early (1993 to 1999) and recent (2000 to 2006) eras. Results: The cardiomyopathy group was significantly (p < 0.001) different from the HLHS and non-HLHS groups at listing: more girls, older age, and a greater need for high-dose inotropes, mechanical ventilation, and/or mechanical circulatory support. Survival after listing was similar among the groups in the early era. Although outcomes after listing in HLHS and cardiomyopathy patients improved in the recent era, outcomes in non-HLHS patients did not. Survival at 1 and 5 years after listing was significantly worse (p < 0.001) for non-HLHS patients (51{\%}, 48{\%}) vs HLHS (71{\%}, 61{\%}), with age- and sex-adjusted hazard ratio (HR) of 1.79 (95{\%} confidence interval, 1.152.77, p = 0.009) and CM (80{\%}, 74{\%}; HR, 2.72; 95{\%} confidence interval, 1.594.67, p < 0.001) in the recent era. Post-transplant survival in both eras was not significantly different among the groups. Conclusion: Use of heart transplantation as primary therapy for non-HLHS infants has not improved over time and currently is associated with significantly poorer results vs HLHS and cardiomyopathy due to a higher risk for death before transplant.",
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T1 - Outcomes after listing for primary transplantation for infants with unoperated-on non-hypoplastic left heart syndrome congenital heart disease

T2 - A multi-institutional study

AU - Guleserian, Kristine J.

AU - Schechtman, Kenneth B.

AU - Zheng, Jie

AU - Edens, R. Erik

AU - Jacobs, Jeffrey P.

AU - Mahle, William T.

AU - Emerson, Stephanie L.

AU - Naftel, David C.

AU - Kirklin, James K.

AU - Blume, Elizabeth D.

AU - Canter, Charles E.

PY - 2011/9

Y1 - 2011/9

N2 - Background: Although heart transplantation has been used as the primary therapy for congenital heart lesions in infants other than hypoplastic left heart syndrome (HLHS), the outcomes in this group of patients have not been determined. Methods: We used the Pediatric Heart Transplant Study database (1993 to 2006) to compare outcomes of 388 infants aged < 6 months listed for HLHS, 161 with other congenital heart diseases (non-HLHS), and 145 with cardiomyopathy in early (1993 to 1999) and recent (2000 to 2006) eras. Results: The cardiomyopathy group was significantly (p < 0.001) different from the HLHS and non-HLHS groups at listing: more girls, older age, and a greater need for high-dose inotropes, mechanical ventilation, and/or mechanical circulatory support. Survival after listing was similar among the groups in the early era. Although outcomes after listing in HLHS and cardiomyopathy patients improved in the recent era, outcomes in non-HLHS patients did not. Survival at 1 and 5 years after listing was significantly worse (p < 0.001) for non-HLHS patients (51%, 48%) vs HLHS (71%, 61%), with age- and sex-adjusted hazard ratio (HR) of 1.79 (95% confidence interval, 1.152.77, p = 0.009) and CM (80%, 74%; HR, 2.72; 95% confidence interval, 1.594.67, p < 0.001) in the recent era. Post-transplant survival in both eras was not significantly different among the groups. Conclusion: Use of heart transplantation as primary therapy for non-HLHS infants has not improved over time and currently is associated with significantly poorer results vs HLHS and cardiomyopathy due to a higher risk for death before transplant.

AB - Background: Although heart transplantation has been used as the primary therapy for congenital heart lesions in infants other than hypoplastic left heart syndrome (HLHS), the outcomes in this group of patients have not been determined. Methods: We used the Pediatric Heart Transplant Study database (1993 to 2006) to compare outcomes of 388 infants aged < 6 months listed for HLHS, 161 with other congenital heart diseases (non-HLHS), and 145 with cardiomyopathy in early (1993 to 1999) and recent (2000 to 2006) eras. Results: The cardiomyopathy group was significantly (p < 0.001) different from the HLHS and non-HLHS groups at listing: more girls, older age, and a greater need for high-dose inotropes, mechanical ventilation, and/or mechanical circulatory support. Survival after listing was similar among the groups in the early era. Although outcomes after listing in HLHS and cardiomyopathy patients improved in the recent era, outcomes in non-HLHS patients did not. Survival at 1 and 5 years after listing was significantly worse (p < 0.001) for non-HLHS patients (51%, 48%) vs HLHS (71%, 61%), with age- and sex-adjusted hazard ratio (HR) of 1.79 (95% confidence interval, 1.152.77, p = 0.009) and CM (80%, 74%; HR, 2.72; 95% confidence interval, 1.594.67, p < 0.001) in the recent era. Post-transplant survival in both eras was not significantly different among the groups. Conclusion: Use of heart transplantation as primary therapy for non-HLHS infants has not improved over time and currently is associated with significantly poorer results vs HLHS and cardiomyopathy due to a higher risk for death before transplant.

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