An institutional approach to, and results for, patient with tetralogy with pulmonary atresia and major systemic-to-pulmonary collateral arteries
Tetralogy of Fallot with pulmonary atresia and diminutive or absent intrapericardial pulmonary arteries is a rare congenital abnormality, with high morbidity and mortality. Despite great advances in surgical- and catheter-based therapies, management remains challenging and controversial. We describe...
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Published in | Cardiology in the young Vol. 20; no. S3; pp. 128 - 134 |
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Main Authors | , , , , |
Format | Journal Article |
Language | English |
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Cambridge, UK
Cambridge University Press
01.12.2010
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Abstract | Tetralogy of Fallot with pulmonary atresia and diminutive or absent intrapericardial pulmonary arteries is a rare congenital abnormality, with high morbidity and mortality. Despite great advances in surgical- and catheter-based therapies, management remains challenging and controversial. We describe the surgical methods and the results from our institution.
We performed a retrospective study of the medical records of patients included in our institutional database with tetralogy and pulmonary atresia, concentrating on those predominantly managed by our programme over their lifetime. We obtained demographics and records of all catheterisations and operations, and established mortality. We assessed the current state of those surviving in terms of clinical function at their most recent clinical evaluation and right ventricular function by echocardiography.
We assessed 38 patients, with 89% follow-up. The mean number of catheterisations for each patients was 5, with a range from 1 to 15. The mean number of operations was 2.2, with a range from 1 to 6. Unifocalisation had been performed in 26 patients, with 12 undergoing procedures to recruit the native pulmonary vasculature. Of the overall cohort, eight patients died. The ventricular septal defect had been closed in all but two patients. Most patients have no or mild exercise intolerance. Right ventricle dysfunction has been a continuing hazard for 15 years.
An individualised approach, using unifocalisation as well as aggressive attempts to recruit the available native pulmonary vasculature, achieves outcomes in the intermediate term superior to the natural history of the lesions, and comparable with those of other studies. |
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AbstractList | Abstract
Background
Tetralogy of Fallot with pulmonary atresia and diminutive or absent intrapericardial pulmonary arteries is a rare congenital abnormality, with high morbidity and mortality. Despite great advances in surgical- and catheter-based therapies, management remains challenging and controversial. We describe the surgical methods and the results from our institution.
Methods
We performed a retrospective study of the medical records of patients included in our institutional database with tetralogy and pulmonary atresia, concentrating on those predominantly managed by our programme over their lifetime. We obtained demographics and records of all catheterisations and operations, and established mortality. We assessed the current state of those surviving in terms of clinical function at their most recent clinical evaluation and right ventricular function by echocardiography.
Results
We assessed 38 patients, with 89% follow-up. The mean number of catheterisations for each patients was 5, with a range from 1 to 15. The mean number of operations was 2.2, with a range from 1 to 6. Unifocalisation had been performed in 26 patients, with 12 undergoing procedures to recruit the native pulmonary vasculature. Of the overall cohort, eight patients died. The ventricular septal defect had been closed in all but two patients. Most patients have no or mild exercise intolerance. Right ventricle dysfunction has been a continuing hazard for 15 years.
Conclusions
An individualised approach, using unifocalisation as well as aggressive attempts to recruit the available native pulmonary vasculature, achieves outcomes in the intermediate term superior to the natural history of the lesions, and comparable with those of other studies. BACKGROUNDTetralogy of Fallot with pulmonary atresia and diminutive or absent intrapericardial pulmonary arteries is a rare congenital abnormality, with high morbidity and mortality. Despite great advances in surgical- and catheter-based therapies, management remains challenging and controversial. We describe the surgical methods and the results from our institution.METHODSWe performed a retrospective study of the medical records of patients included in our institutional database with tetralogy and pulmonary atresia, concentrating on those predominantly managed by our programme over their lifetime. We obtained demographics and records of all catheterisations and operations, and established mortality. We assessed the current state of those surviving in terms of clinical function at their most recent clinical evaluation and right ventricular function by echocardiography.RESULTSWe assessed 38 patients, with 89% follow-up. The mean number of catheterisations for each patients was 5, with a range from 1 to 15. The mean number of operations was 2.2, with a range from 1 to 6. Unifocalisation had been performed in 26 patients, with 12 undergoing procedures to recruit the native pulmonary vasculature. Of the overall cohort, eight patients died. The ventricular septal defect had been closed in all but two patients. Most patients have no or mild exercise intolerance. Right ventricle dysfunction has been a continuing hazard for 15 years.CONCLUSIONSAn individualised approach, using unifocalisation as well as aggressive attempts to recruit the available native pulmonary vasculature, achieves outcomes in the intermediate term superior to the natural history of the lesions, and comparable with those of other studies. Tetralogy of Fallot with pulmonary atresia and diminutive or absent intrapericardial pulmonary arteries is a rare congenital abnormality, with high morbidity and mortality. Despite great advances in surgical- and catheter-based therapies, management remains challenging and controversial. We describe the surgical methods and the results from our institution. We performed a retrospective study of the medical records of patients included in our institutional database with tetralogy and pulmonary atresia, concentrating on those predominantly managed by our programme over their lifetime. We obtained demographics and records of all catheterisations and operations, and established mortality. We assessed the current state of those surviving in terms of clinical function at their most recent clinical evaluation and right ventricular function by echocardiography. We assessed 38 patients, with 89% follow-up. The mean number of catheterisations for each patients was 5, with a range from 1 to 15. The mean number of operations was 2.2, with a range from 1 to 6. Unifocalisation had been performed in 26 patients, with 12 undergoing procedures to recruit the native pulmonary vasculature. Of the overall cohort, eight patients died. The ventricular septal defect had been closed in all but two patients. Most patients have no or mild exercise intolerance. Right ventricle dysfunction has been a continuing hazard for 15 years. An individualised approach, using unifocalisation as well as aggressive attempts to recruit the available native pulmonary vasculature, achieves outcomes in the intermediate term superior to the natural history of the lesions, and comparable with those of other studies. Abstract Background Tetralogy of Fallot with pulmonary atresia and diminutive or absent intrapericardial pulmonary arteries is a rare congenital abnormality, with high morbidity and mortality. Despite great advances in surgical- and catheter-based therapies, management remains challenging and controversial. We describe the surgical methods and the results from our institution. Methods We performed a retrospective study of the medical records of patients included in our institutional database with tetralogy and pulmonary atresia, concentrating on those predominantly managed by our programme over their lifetime. We obtained demographics and records of all catheterisations and operations, and established mortality. We assessed the current state of those surviving in terms of clinical function at their most recent clinical evaluation and right ventricular function by echocardiography. Results We assessed 38 patients, with 89% follow-up. The mean number of catheterisations for each patients was 5, with a range from 1 to 15. The mean number of operations was 2.2, with a range from 1 to 6. Unifocalisation had been performed in 26 patients, with 12 undergoing procedures to recruit the native pulmonary vasculature. Of the overall cohort, eight patients died. The ventricular septal defect had been closed in all but two patients. Most patients have no or mild exercise intolerance. Right ventricle dysfunction has been a continuing hazard for 15 years. Conclusions An individualised approach, using unifocalisation as well as aggressive attempts to recruit the available native pulmonary vasculature, achieves outcomes in the intermediate term superior to the natural history of the lesions, and comparable with those of other studies. |
Author | Zabinsky, Jennifer Argueta-Morales, I. Ricardo Hannan, Robert L. DeCampli, William M. Burke, Redmond P. |
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CitedBy_id | crossref_primary_10_1016_j_athoracsur_2013_01_007 crossref_primary_10_1093_ejcts_ezx043 crossref_primary_10_1053_j_optechstcvs_2019_07_003 crossref_primary_10_1016_j_athoracsur_2015_03_110 crossref_primary_10_1053_j_optechstcvs_2019_07_002 crossref_primary_10_1177_1084822315588519 crossref_primary_10_1016_j_athoracsur_2016_03_011 crossref_primary_10_1017_S1047951120004047 crossref_primary_10_1053_j_semtcvs_2019_05_014 crossref_primary_10_1093_ejcts_ezw069 crossref_primary_10_1093_ejcts_ezt622 |
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Snippet | Tetralogy of Fallot with pulmonary atresia and diminutive or absent intrapericardial pulmonary arteries is a rare congenital abnormality, with high morbidity... Abstract Background Tetralogy of Fallot with pulmonary atresia and diminutive or absent intrapericardial pulmonary arteries is a rare congenital abnormality,... Abstract Background Tetralogy of Fallot with pulmonary atresia and diminutive or absent intrapericardial pulmonary arteries is a rare congenital abnormality,... BACKGROUNDTetralogy of Fallot with pulmonary atresia and diminutive or absent intrapericardial pulmonary arteries is a rare congenital abnormality, with high... |
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SubjectTerms | Abnormalities, Multiple - surgery Cardiac Surgical Procedures - methods Female Humans Infant Male outcomes Pulmonary Artery - abnormalities Pulmonary Atresia - complications Pulmonary Atresia - surgery Pulmonary atresia with ventricular septal defect Retrospective Studies tetralogy of Fallot Tetralogy of Fallot - complications Tetralogy of Fallot - surgery tetralogy of Fallot with pulmonary atresia unifocalisation |
Title | An institutional approach to, and results for, patient with tetralogy with pulmonary atresia and major systemic-to-pulmonary collateral arteries |
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