Outcome after orthotopic cardiac transplantation in adults with congenital heart disease

Jacqueline M. Lamour, Linda J. Addonizio, Mark E. Galantowicz, Jan M. Quaegebeur, Donna M. Mancini, Maryanne R. Kichuk, Ainat Beniaminovitz, Robert E. Michler, Alan Weinberg, Daphne T. Hsu

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Abstract

Background - Advances in surgical and medical management have greatly improved long-term survival rates in patients with congenital heart disease (CHD). As these patients reach adulthood, myocardial dysfunction can occur, leading to cardiac transplantation. Methods and Results - We reviewed the pretransplantation and posttransplantation courses of 24 patients > 18 years old (mean age, 26 years; range, 18 to 56 years) with CHD who received a transplant between January 1985 and September 1998. The relation between preoperative and perioperative risk factors for complications and death was assessed. Single ventricle was the pretransplantation diagnosis for 12 patients (50%), and d-transposition of the great vessels was the diagnosis for 4 patients (16%). Twenty-two patients had a mean of 2 previous operations. At cardiac transplantation, additional surgical procedures were required to correct extracardiac lesions in 18 patients (75%). Refractory heart failure was present in 22 patients, significant cyanosis was present in 7, and protein-losing enteropathy was present in 4. There were 5 early deaths due to bleeding (n=3) and infection (n=2). The Kaplan-Meier survival rate after cardiac transplantation was 79% at 1 year and 60% at 5 years. No anatomic or surgical risk factor was predictive of death. The outcome of patients with CHD who received a transplant was compared with that for patients without CHD (n=788). Mean bypass and ischemic times were significantly longer in patients with CHD than in patients without CHD. Survival rates after transplantation did not differ significantly between patients with and those without CHD (P=0.83). Conclusions - Successful cardiac transplantation is obtainable in adults with complex CHD, with an outcome similar to that of patients without CHD. A detailed assessment of cardiac anatomy and careful surgical planning are essential to the pretransplantation and posttransplantation management of these patients.

Original languageEnglish (US)
JournalCirculation
Volume100
Issue number19 SUPPL.
StatePublished - Nov 9 1999
Externally publishedYes

Fingerprint

Heart Transplantation
Heart Diseases
Survival Rate
Protein-Losing Enteropathies
Transplants
Transposition of Great Vessels
Cyanosis
Anatomy

Keywords

  • Heart defects, congenital
  • Risk factors
  • Transplantation

ASJC Scopus subject areas

  • Physiology
  • Cardiology and Cardiovascular Medicine

Cite this

Lamour, J. M., Addonizio, L. J., Galantowicz, M. E., Quaegebeur, J. M., Mancini, D. M., Kichuk, M. R., ... Hsu, D. T. (1999). Outcome after orthotopic cardiac transplantation in adults with congenital heart disease. Circulation, 100(19 SUPPL.).

Outcome after orthotopic cardiac transplantation in adults with congenital heart disease. / Lamour, Jacqueline M.; Addonizio, Linda J.; Galantowicz, Mark E.; Quaegebeur, Jan M.; Mancini, Donna M.; Kichuk, Maryanne R.; Beniaminovitz, Ainat; Michler, Robert E.; Weinberg, Alan; Hsu, Daphne T.

In: Circulation, Vol. 100, No. 19 SUPPL., 09.11.1999.

Research output: Contribution to journalArticle

Lamour, JM, Addonizio, LJ, Galantowicz, ME, Quaegebeur, JM, Mancini, DM, Kichuk, MR, Beniaminovitz, A, Michler, RE, Weinberg, A & Hsu, DT 1999, 'Outcome after orthotopic cardiac transplantation in adults with congenital heart disease', Circulation, vol. 100, no. 19 SUPPL..
Lamour JM, Addonizio LJ, Galantowicz ME, Quaegebeur JM, Mancini DM, Kichuk MR et al. Outcome after orthotopic cardiac transplantation in adults with congenital heart disease. Circulation. 1999 Nov 9;100(19 SUPPL.).
Lamour, Jacqueline M. ; Addonizio, Linda J. ; Galantowicz, Mark E. ; Quaegebeur, Jan M. ; Mancini, Donna M. ; Kichuk, Maryanne R. ; Beniaminovitz, Ainat ; Michler, Robert E. ; Weinberg, Alan ; Hsu, Daphne T. / Outcome after orthotopic cardiac transplantation in adults with congenital heart disease. In: Circulation. 1999 ; Vol. 100, No. 19 SUPPL.
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abstract = "Background - Advances in surgical and medical management have greatly improved long-term survival rates in patients with congenital heart disease (CHD). As these patients reach adulthood, myocardial dysfunction can occur, leading to cardiac transplantation. Methods and Results - We reviewed the pretransplantation and posttransplantation courses of 24 patients > 18 years old (mean age, 26 years; range, 18 to 56 years) with CHD who received a transplant between January 1985 and September 1998. The relation between preoperative and perioperative risk factors for complications and death was assessed. Single ventricle was the pretransplantation diagnosis for 12 patients (50{\%}), and d-transposition of the great vessels was the diagnosis for 4 patients (16{\%}). Twenty-two patients had a mean of 2 previous operations. At cardiac transplantation, additional surgical procedures were required to correct extracardiac lesions in 18 patients (75{\%}). Refractory heart failure was present in 22 patients, significant cyanosis was present in 7, and protein-losing enteropathy was present in 4. There were 5 early deaths due to bleeding (n=3) and infection (n=2). The Kaplan-Meier survival rate after cardiac transplantation was 79{\%} at 1 year and 60{\%} at 5 years. No anatomic or surgical risk factor was predictive of death. The outcome of patients with CHD who received a transplant was compared with that for patients without CHD (n=788). Mean bypass and ischemic times were significantly longer in patients with CHD than in patients without CHD. Survival rates after transplantation did not differ significantly between patients with and those without CHD (P=0.83). Conclusions - Successful cardiac transplantation is obtainable in adults with complex CHD, with an outcome similar to that of patients without CHD. A detailed assessment of cardiac anatomy and careful surgical planning are essential to the pretransplantation and posttransplantation management of these patients.",
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AU - Addonizio, Linda J.

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AU - Mancini, Donna M.

AU - Kichuk, Maryanne R.

AU - Beniaminovitz, Ainat

AU - Michler, Robert E.

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AU - Hsu, Daphne T.

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N2 - Background - Advances in surgical and medical management have greatly improved long-term survival rates in patients with congenital heart disease (CHD). As these patients reach adulthood, myocardial dysfunction can occur, leading to cardiac transplantation. Methods and Results - We reviewed the pretransplantation and posttransplantation courses of 24 patients > 18 years old (mean age, 26 years; range, 18 to 56 years) with CHD who received a transplant between January 1985 and September 1998. The relation between preoperative and perioperative risk factors for complications and death was assessed. Single ventricle was the pretransplantation diagnosis for 12 patients (50%), and d-transposition of the great vessels was the diagnosis for 4 patients (16%). Twenty-two patients had a mean of 2 previous operations. At cardiac transplantation, additional surgical procedures were required to correct extracardiac lesions in 18 patients (75%). Refractory heart failure was present in 22 patients, significant cyanosis was present in 7, and protein-losing enteropathy was present in 4. There were 5 early deaths due to bleeding (n=3) and infection (n=2). The Kaplan-Meier survival rate after cardiac transplantation was 79% at 1 year and 60% at 5 years. No anatomic or surgical risk factor was predictive of death. The outcome of patients with CHD who received a transplant was compared with that for patients without CHD (n=788). Mean bypass and ischemic times were significantly longer in patients with CHD than in patients without CHD. Survival rates after transplantation did not differ significantly between patients with and those without CHD (P=0.83). Conclusions - Successful cardiac transplantation is obtainable in adults with complex CHD, with an outcome similar to that of patients without CHD. A detailed assessment of cardiac anatomy and careful surgical planning are essential to the pretransplantation and posttransplantation management of these patients.

AB - Background - Advances in surgical and medical management have greatly improved long-term survival rates in patients with congenital heart disease (CHD). As these patients reach adulthood, myocardial dysfunction can occur, leading to cardiac transplantation. Methods and Results - We reviewed the pretransplantation and posttransplantation courses of 24 patients > 18 years old (mean age, 26 years; range, 18 to 56 years) with CHD who received a transplant between January 1985 and September 1998. The relation between preoperative and perioperative risk factors for complications and death was assessed. Single ventricle was the pretransplantation diagnosis for 12 patients (50%), and d-transposition of the great vessels was the diagnosis for 4 patients (16%). Twenty-two patients had a mean of 2 previous operations. At cardiac transplantation, additional surgical procedures were required to correct extracardiac lesions in 18 patients (75%). Refractory heart failure was present in 22 patients, significant cyanosis was present in 7, and protein-losing enteropathy was present in 4. There were 5 early deaths due to bleeding (n=3) and infection (n=2). The Kaplan-Meier survival rate after cardiac transplantation was 79% at 1 year and 60% at 5 years. No anatomic or surgical risk factor was predictive of death. The outcome of patients with CHD who received a transplant was compared with that for patients without CHD (n=788). Mean bypass and ischemic times were significantly longer in patients with CHD than in patients without CHD. Survival rates after transplantation did not differ significantly between patients with and those without CHD (P=0.83). Conclusions - Successful cardiac transplantation is obtainable in adults with complex CHD, with an outcome similar to that of patients without CHD. A detailed assessment of cardiac anatomy and careful surgical planning are essential to the pretransplantation and posttransplantation management of these patients.

KW - Heart defects, congenital

KW - Risk factors

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