An institutional approach to, and results for, patient with tetralogy with pulmonary atresia and major systemic-to-pulmonary collateral arteries
| Autor: | Jennifer A. Zabinsky, Redmond P. Burke, William M. DeCampli, I. Ricardo Argueta-Morales, Robert L. Hannan |
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| Rok vydání: | 2010 |
| Předmět: |
Male
medicine.medical_specialty Pulmonary Artery Internal medicine medicine Humans Abnormalities Multiple Tetralogy Cardiac Surgical Procedures Retrospective Studies Tetralogy of Fallot business.industry Medical record Infant Retrospective cohort study General Medicine medicine.disease Natural history medicine.anatomical_structure Pulmonary Atresia Ventricle Pediatrics Perinatology and Child Health Cohort Cardiology Female Cardiology and Cardiovascular Medicine Pulmonary atresia business |
| Zdroj: | Cardiology in the Young. 20:128-134 |
| ISSN: | 1467-1107 1047-9511 |
| DOI: | 10.1017/s1047951110001186 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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