Repair of congenital heart lesions combined with lung transplantation for the treatment of severe pulmonary hypertension: A 13-year experience

Cliff K. Choong, Stuart C. Sweet, Tracey J. Guthrie, Eric N. Mendeloff, Fabio J. Haddad, Pam Schuler, Maite De La Morena, Charles B. Huddleston

Research output: Contribution to journalArticle

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Abstract

Objective: In patients with severe pulmonary hypertension associated with congenital heart disease, we prefer to perform repair of the congenital heart disease and lung transplantation whenever feasible so as to augment the donor pool and avoid the cardiac complications associated with heart transplantation. We report our experience with repair of congenital heart disease and lung transplantation and compare the results with those of patients who underwent heart-lung transplantation during the same period. Methods: The records of patients who had repair of congenital heart disease and lung transplantation (n = 35) and heart-lung transplantation (n = 16) between 1990 and 2003 were reviewed. Results: The underlying congenital heart disease in the repair of congenital heart disease and lung transplantation group included transposition of great vessels (n = 2), atrioventricular canal defect (n = 2), ventricular septal defect (n = 9), pulmonary venous obstruction (n = 7), scimitar syndrome (n = 2), pulmonary arterial atresia or stenosis (n = 5), and others (n = 8). Thirteen of the patients undergoing repair of congenital heart disease and lung transplantation (37.1%) had the congenital heart disease repaired before lung transplantation; the remaining congenital heart disease repairs were performed concurrently with transplantation. Sixteen patients underwent heart-lung transplantation because of poor left ventricular function or single-ventricle anatomy. Freedoms from bronchiolitis obliterans at 1, 3, and 5 years were 72.9%, 54.7%, and 54.7% for the repair of congenital heart disease and lung transplantation group and 77.8%, 51.9%, and 38.9% for the heart-lung transplantation group, respectively. Survivals at 1, 3, and 5 years were 62.9%, 51.4%, and 51.4% for the repair of congenital heart disease and lung transplantation group and 66.5%, 66.5%, and 60% for the heart-lung transplantation group, respectively. Conclusion: Repair of congenital heart disease and lung transplantation is a feasible treatment option. Long-term outcome is determined by associated complications related to lung transplantation. Despite the complexity of combined congenital heart disease repair with lung transplantation and the resulting perioperative morbidity, the patients had similar outcomes to those of patients who underwent heart-lung transplantation.

Original languageEnglish (US)
Pages (from-to)661-669
Number of pages9
JournalJournal of Thoracic and Cardiovascular Surgery
Volume129
Issue number3
DOIs
StatePublished - Mar 2005

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Heart-Lung Transplantation
Lung Transplantation
Pulmonary Hypertension
Heart Diseases
Therapeutics
Scimitar Syndrome
Bronchiolitis Obliterans
Pulmonary Atresia
Transposition of Great Vessels
Pulmonary Valve Stenosis
Ventricular Heart Septal Defects
Heart Transplantation
Left Ventricular Function

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery

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Repair of congenital heart lesions combined with lung transplantation for the treatment of severe pulmonary hypertension : A 13-year experience. / Choong, Cliff K.; Sweet, Stuart C.; Guthrie, Tracey J.; Mendeloff, Eric N.; Haddad, Fabio J.; Schuler, Pam; De La Morena, Maite; Huddleston, Charles B.

In: Journal of Thoracic and Cardiovascular Surgery, Vol. 129, No. 3, 03.2005, p. 661-669.

Research output: Contribution to journalArticle

Choong, Cliff K. ; Sweet, Stuart C. ; Guthrie, Tracey J. ; Mendeloff, Eric N. ; Haddad, Fabio J. ; Schuler, Pam ; De La Morena, Maite ; Huddleston, Charles B. / Repair of congenital heart lesions combined with lung transplantation for the treatment of severe pulmonary hypertension : A 13-year experience. In: Journal of Thoracic and Cardiovascular Surgery. 2005 ; Vol. 129, No. 3. pp. 661-669.
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abstract = "Objective: In patients with severe pulmonary hypertension associated with congenital heart disease, we prefer to perform repair of the congenital heart disease and lung transplantation whenever feasible so as to augment the donor pool and avoid the cardiac complications associated with heart transplantation. We report our experience with repair of congenital heart disease and lung transplantation and compare the results with those of patients who underwent heart-lung transplantation during the same period. Methods: The records of patients who had repair of congenital heart disease and lung transplantation (n = 35) and heart-lung transplantation (n = 16) between 1990 and 2003 were reviewed. Results: The underlying congenital heart disease in the repair of congenital heart disease and lung transplantation group included transposition of great vessels (n = 2), atrioventricular canal defect (n = 2), ventricular septal defect (n = 9), pulmonary venous obstruction (n = 7), scimitar syndrome (n = 2), pulmonary arterial atresia or stenosis (n = 5), and others (n = 8). Thirteen of the patients undergoing repair of congenital heart disease and lung transplantation (37.1{\%}) had the congenital heart disease repaired before lung transplantation; the remaining congenital heart disease repairs were performed concurrently with transplantation. Sixteen patients underwent heart-lung transplantation because of poor left ventricular function or single-ventricle anatomy. Freedoms from bronchiolitis obliterans at 1, 3, and 5 years were 72.9{\%}, 54.7{\%}, and 54.7{\%} for the repair of congenital heart disease and lung transplantation group and 77.8{\%}, 51.9{\%}, and 38.9{\%} for the heart-lung transplantation group, respectively. Survivals at 1, 3, and 5 years were 62.9{\%}, 51.4{\%}, and 51.4{\%} for the repair of congenital heart disease and lung transplantation group and 66.5{\%}, 66.5{\%}, and 60{\%} for the heart-lung transplantation group, respectively. Conclusion: Repair of congenital heart disease and lung transplantation is a feasible treatment option. Long-term outcome is determined by associated complications related to lung transplantation. Despite the complexity of combined congenital heart disease repair with lung transplantation and the resulting perioperative morbidity, the patients had similar outcomes to those of patients who underwent heart-lung transplantation.",
author = "Choong, {Cliff K.} and Sweet, {Stuart C.} and Guthrie, {Tracey J.} and Mendeloff, {Eric N.} and Haddad, {Fabio J.} and Pam Schuler and {De La Morena}, Maite and Huddleston, {Charles B.}",
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T2 - A 13-year experience

AU - Choong, Cliff K.

AU - Sweet, Stuart C.

AU - Guthrie, Tracey J.

AU - Mendeloff, Eric N.

AU - Haddad, Fabio J.

AU - Schuler, Pam

AU - De La Morena, Maite

AU - Huddleston, Charles B.

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N2 - Objective: In patients with severe pulmonary hypertension associated with congenital heart disease, we prefer to perform repair of the congenital heart disease and lung transplantation whenever feasible so as to augment the donor pool and avoid the cardiac complications associated with heart transplantation. We report our experience with repair of congenital heart disease and lung transplantation and compare the results with those of patients who underwent heart-lung transplantation during the same period. Methods: The records of patients who had repair of congenital heart disease and lung transplantation (n = 35) and heart-lung transplantation (n = 16) between 1990 and 2003 were reviewed. Results: The underlying congenital heart disease in the repair of congenital heart disease and lung transplantation group included transposition of great vessels (n = 2), atrioventricular canal defect (n = 2), ventricular septal defect (n = 9), pulmonary venous obstruction (n = 7), scimitar syndrome (n = 2), pulmonary arterial atresia or stenosis (n = 5), and others (n = 8). Thirteen of the patients undergoing repair of congenital heart disease and lung transplantation (37.1%) had the congenital heart disease repaired before lung transplantation; the remaining congenital heart disease repairs were performed concurrently with transplantation. Sixteen patients underwent heart-lung transplantation because of poor left ventricular function or single-ventricle anatomy. Freedoms from bronchiolitis obliterans at 1, 3, and 5 years were 72.9%, 54.7%, and 54.7% for the repair of congenital heart disease and lung transplantation group and 77.8%, 51.9%, and 38.9% for the heart-lung transplantation group, respectively. Survivals at 1, 3, and 5 years were 62.9%, 51.4%, and 51.4% for the repair of congenital heart disease and lung transplantation group and 66.5%, 66.5%, and 60% for the heart-lung transplantation group, respectively. Conclusion: Repair of congenital heart disease and lung transplantation is a feasible treatment option. Long-term outcome is determined by associated complications related to lung transplantation. Despite the complexity of combined congenital heart disease repair with lung transplantation and the resulting perioperative morbidity, the patients had similar outcomes to those of patients who underwent heart-lung transplantation.

AB - Objective: In patients with severe pulmonary hypertension associated with congenital heart disease, we prefer to perform repair of the congenital heart disease and lung transplantation whenever feasible so as to augment the donor pool and avoid the cardiac complications associated with heart transplantation. We report our experience with repair of congenital heart disease and lung transplantation and compare the results with those of patients who underwent heart-lung transplantation during the same period. Methods: The records of patients who had repair of congenital heart disease and lung transplantation (n = 35) and heart-lung transplantation (n = 16) between 1990 and 2003 were reviewed. Results: The underlying congenital heart disease in the repair of congenital heart disease and lung transplantation group included transposition of great vessels (n = 2), atrioventricular canal defect (n = 2), ventricular septal defect (n = 9), pulmonary venous obstruction (n = 7), scimitar syndrome (n = 2), pulmonary arterial atresia or stenosis (n = 5), and others (n = 8). Thirteen of the patients undergoing repair of congenital heart disease and lung transplantation (37.1%) had the congenital heart disease repaired before lung transplantation; the remaining congenital heart disease repairs were performed concurrently with transplantation. Sixteen patients underwent heart-lung transplantation because of poor left ventricular function or single-ventricle anatomy. Freedoms from bronchiolitis obliterans at 1, 3, and 5 years were 72.9%, 54.7%, and 54.7% for the repair of congenital heart disease and lung transplantation group and 77.8%, 51.9%, and 38.9% for the heart-lung transplantation group, respectively. Survivals at 1, 3, and 5 years were 62.9%, 51.4%, and 51.4% for the repair of congenital heart disease and lung transplantation group and 66.5%, 66.5%, and 60% for the heart-lung transplantation group, respectively. Conclusion: Repair of congenital heart disease and lung transplantation is a feasible treatment option. Long-term outcome is determined by associated complications related to lung transplantation. Despite the complexity of combined congenital heart disease repair with lung transplantation and the resulting perioperative morbidity, the patients had similar outcomes to those of patients who underwent heart-lung transplantation.

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