An institutional approach to, and results for, patient with tetralogy with pulmonary atresia and major systemic-to-pulmonary collateral arteries

• Published in: Cardiology in the young Vol. 20; no. S3; pp. 128 - 134
• Main Authors: DeCampli, William M., Argueta-Morales, I. Ricardo, Zabinsky, Jennifer, Hannan, Robert L., Burke, Redmond P.
• Format: Journal Article
• Language: English
• Published: Cambridge, UK Cambridge University Press 01.12.2010
• Subjects: 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; tetralogy of Fallot with pulmonary atresia; unifocalisation; Pulmonary atresia with ventricular septal defect; tetralogy of Fallot; outcomes
• ISSN: 1047-9511; 1467-1107
• DOI: 10.1017/S1047951110001186

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.