The Effect of Age, Diagnosis, and Previous Surgery in Children and Adults Undergoing Heart Transplantation for Congenital Heart Disease

Jacqueline M. Lamour, Kirk R. Kanter, David C. Naftel, Maryanne R. Chrisant, William R. Morrow, Barry S. Clemson, James K. Kirklin

Research output: Contribution to journalArticle

160 Citations (Scopus)

Abstract

Objectives: We sought to evaluate the outcomes and identify risk factors for mortality after heart transplantation (HT) for congenital heart disease (CHD) in infants, children, and adults. Background: CHD is considered a risk factor for mortality after HT, yet this unique group of patients represents a spectrum of complexity. Methods: There were 488 patients transplanted for CHD from the combined Pediatric Heart Transplant Study (1993 to 2002, n = 367) and the Cardiac Transplant Registry Database (1990 to 2002, n = 121) who were analyzed. Results: The median age at HT was 12.4 years. Primary diagnosis included single ventricle (36%), d-transposition of the great arteries (12%), right ventricular outflow tract lesions (10%), l-transposition of the great arteries (8%), ventricular/atrial septal defects (8%), left ventricular outflow obstruction (8%), and other (18%). Ninety-three percent of patients had at least 1 operation before HT. Survival at 3 months post-HT was significantly worse in CHD patients versus children with cardiomyopathy, but not adults with cardiomyopathy (86%, 94%, and 91%, respectively). There was no difference in conditional 3-month survival among the 3 groups. Five-year survival was 80%. Risk factors for early mortality were older recipient age, older donors with longer ischemic times, and pre-HT Fontan operations. Predicted survival in Fontan patients was lower (77% and 70% at 1 and 5 years) versus non-Fontan patients (88% and 81% at 1 and 5 years). Risk factors for constant phase mortality included younger recipient age, higher transpulmonary gradient, cytomegalovirus mismatch at HT, and earlier classical Glenn operation. Conclusions: Patients undergoing transplantation for CHD have a good late survival if they survive the early post-operative period. Risk factors for reduced survival are older age at transplant and a previous Fontan operation.

Original languageEnglish (US)
Pages (from-to)160-165
Number of pages6
JournalJournal of the American College of Cardiology
Volume54
Issue number2
DOIs
StatePublished - Jul 7 2009
Externally publishedYes

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Heart Transplantation
Heart Diseases
Survival
Fontan Procedure
Transposition of Great Vessels
Mortality
Transplants
Cardiomyopathies
Ventricular Outflow Obstruction
Atrial Heart Septal Defects
Ventricular Heart Septal Defects
Cytomegalovirus
Registries
Transplantation
Tissue Donors
Databases
Pediatrics

Keywords

  • adults
  • congenital heart disease
  • heart transplantation
  • outcomes
  • pediatrics

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

The Effect of Age, Diagnosis, and Previous Surgery in Children and Adults Undergoing Heart Transplantation for Congenital Heart Disease. / Lamour, Jacqueline M.; Kanter, Kirk R.; Naftel, David C.; Chrisant, Maryanne R.; Morrow, William R.; Clemson, Barry S.; Kirklin, James K.

In: Journal of the American College of Cardiology, Vol. 54, No. 2, 07.07.2009, p. 160-165.

Research output: Contribution to journalArticle

Lamour, Jacqueline M. ; Kanter, Kirk R. ; Naftel, David C. ; Chrisant, Maryanne R. ; Morrow, William R. ; Clemson, Barry S. ; Kirklin, James K. / The Effect of Age, Diagnosis, and Previous Surgery in Children and Adults Undergoing Heart Transplantation for Congenital Heart Disease. In: Journal of the American College of Cardiology. 2009 ; Vol. 54, No. 2. pp. 160-165.
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abstract = "Objectives: We sought to evaluate the outcomes and identify risk factors for mortality after heart transplantation (HT) for congenital heart disease (CHD) in infants, children, and adults. Background: CHD is considered a risk factor for mortality after HT, yet this unique group of patients represents a spectrum of complexity. Methods: There were 488 patients transplanted for CHD from the combined Pediatric Heart Transplant Study (1993 to 2002, n = 367) and the Cardiac Transplant Registry Database (1990 to 2002, n = 121) who were analyzed. Results: The median age at HT was 12.4 years. Primary diagnosis included single ventricle (36{\%}), d-transposition of the great arteries (12{\%}), right ventricular outflow tract lesions (10{\%}), l-transposition of the great arteries (8{\%}), ventricular/atrial septal defects (8{\%}), left ventricular outflow obstruction (8{\%}), and other (18{\%}). Ninety-three percent of patients had at least 1 operation before HT. Survival at 3 months post-HT was significantly worse in CHD patients versus children with cardiomyopathy, but not adults with cardiomyopathy (86{\%}, 94{\%}, and 91{\%}, respectively). There was no difference in conditional 3-month survival among the 3 groups. Five-year survival was 80{\%}. Risk factors for early mortality were older recipient age, older donors with longer ischemic times, and pre-HT Fontan operations. Predicted survival in Fontan patients was lower (77{\%} and 70{\%} at 1 and 5 years) versus non-Fontan patients (88{\%} and 81{\%} at 1 and 5 years). Risk factors for constant phase mortality included younger recipient age, higher transpulmonary gradient, cytomegalovirus mismatch at HT, and earlier classical Glenn operation. Conclusions: Patients undergoing transplantation for CHD have a good late survival if they survive the early post-operative period. Risk factors for reduced survival are older age at transplant and a previous Fontan operation.",
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T1 - The Effect of Age, Diagnosis, and Previous Surgery in Children and Adults Undergoing Heart Transplantation for Congenital Heart Disease

AU - Lamour, Jacqueline M.

AU - Kanter, Kirk R.

AU - Naftel, David C.

AU - Chrisant, Maryanne R.

AU - Morrow, William R.

AU - Clemson, Barry S.

AU - Kirklin, James K.

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N2 - Objectives: We sought to evaluate the outcomes and identify risk factors for mortality after heart transplantation (HT) for congenital heart disease (CHD) in infants, children, and adults. Background: CHD is considered a risk factor for mortality after HT, yet this unique group of patients represents a spectrum of complexity. Methods: There were 488 patients transplanted for CHD from the combined Pediatric Heart Transplant Study (1993 to 2002, n = 367) and the Cardiac Transplant Registry Database (1990 to 2002, n = 121) who were analyzed. Results: The median age at HT was 12.4 years. Primary diagnosis included single ventricle (36%), d-transposition of the great arteries (12%), right ventricular outflow tract lesions (10%), l-transposition of the great arteries (8%), ventricular/atrial septal defects (8%), left ventricular outflow obstruction (8%), and other (18%). Ninety-three percent of patients had at least 1 operation before HT. Survival at 3 months post-HT was significantly worse in CHD patients versus children with cardiomyopathy, but not adults with cardiomyopathy (86%, 94%, and 91%, respectively). There was no difference in conditional 3-month survival among the 3 groups. Five-year survival was 80%. Risk factors for early mortality were older recipient age, older donors with longer ischemic times, and pre-HT Fontan operations. Predicted survival in Fontan patients was lower (77% and 70% at 1 and 5 years) versus non-Fontan patients (88% and 81% at 1 and 5 years). Risk factors for constant phase mortality included younger recipient age, higher transpulmonary gradient, cytomegalovirus mismatch at HT, and earlier classical Glenn operation. Conclusions: Patients undergoing transplantation for CHD have a good late survival if they survive the early post-operative period. Risk factors for reduced survival are older age at transplant and a previous Fontan operation.

AB - Objectives: We sought to evaluate the outcomes and identify risk factors for mortality after heart transplantation (HT) for congenital heart disease (CHD) in infants, children, and adults. Background: CHD is considered a risk factor for mortality after HT, yet this unique group of patients represents a spectrum of complexity. Methods: There were 488 patients transplanted for CHD from the combined Pediatric Heart Transplant Study (1993 to 2002, n = 367) and the Cardiac Transplant Registry Database (1990 to 2002, n = 121) who were analyzed. Results: The median age at HT was 12.4 years. Primary diagnosis included single ventricle (36%), d-transposition of the great arteries (12%), right ventricular outflow tract lesions (10%), l-transposition of the great arteries (8%), ventricular/atrial septal defects (8%), left ventricular outflow obstruction (8%), and other (18%). Ninety-three percent of patients had at least 1 operation before HT. Survival at 3 months post-HT was significantly worse in CHD patients versus children with cardiomyopathy, but not adults with cardiomyopathy (86%, 94%, and 91%, respectively). There was no difference in conditional 3-month survival among the 3 groups. Five-year survival was 80%. Risk factors for early mortality were older recipient age, older donors with longer ischemic times, and pre-HT Fontan operations. Predicted survival in Fontan patients was lower (77% and 70% at 1 and 5 years) versus non-Fontan patients (88% and 81% at 1 and 5 years). Risk factors for constant phase mortality included younger recipient age, higher transpulmonary gradient, cytomegalovirus mismatch at HT, and earlier classical Glenn operation. Conclusions: Patients undergoing transplantation for CHD have a good late survival if they survive the early post-operative period. Risk factors for reduced survival are older age at transplant and a previous Fontan operation.

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