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Outcomes of patients who underwent staged repair for pulmonary atresia with ventricular septal defects and major aorto-pulmonary collateral arteries at Southampton University Hospital, United Kingdom

Authors:

M P B Goonetilleke ,

Teaching Hospital, Karapitiya, LK
About M P B
Consultant Paediatric Cardiologist, Teaching Hospital, Karapitiya
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G Veldman

Southampton University Hospital, United Kingdom, GB
About G

Consultant Paediatric Cardiologist, Southampton University Hospital, United Kingdom

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Abstract

Objective: To assess the long-term outcomes of multistage surgical approach in 18 surviving patients in areas of  mortality, requirement for postoperative interventional cardiac catheterization, saturations on air, right ventricular (RV) pressures in relation to the systemic pressures, RV and left ventricular (LV) functions and the adequacy of pulmonary arborisation.

Method: Data of all surviving patients who underwent complete repair at Southampton University Hospital were reviewed. Data were obtained using case notes and computer based patient records. No data were gathered by direct patient contacts. LV and RV ejection fractions were measured by cardiac magnetic resonance imaging. Pulmonary arborisation patterns were assessed using cardiac catheter data.

Results: A total of 26 patients had undergone complete repair. There were 8 (30.8%) deaths and the 18 survivors constituted the study group. Eleven (61.1%) had undergone three or more surgeries to attain complete repair. Fifteen (83.3%) maintained resting saturations above 80% after surgery. Nine (50%) had undergone three or more catheter interventions after complete repair. Eleven (61.1%) had undergone one or more pulmonary ballooning. Nine (50%) had undergone pulmonary artery (PA) stenting. Four (22.2%) had a RV ejection fraction (EF) <45% and 5 (27.7%) had a LV EF <45%. Seven (38.8%) had RV pressures >50% of LV pressures. Certain zones of the lungs were significantly under-filled on complete repair.

Conclusions: Children with pulmonary atresia, ventricular septal defect and major aorto-pulmonary collaterals can be treated using a staged repair. There is a high mortality (31%) with average functional results.

Sri Lanka Journal of Child Health, 2014; 43(2): 84-87

(Key words: Pulmonary atresia; ventricular septal defect; major aorto-pulmonary collaterals)

DOI: http://dx.doi.org/10.4038/sljch.v43i2.7008

How to Cite: Goonetilleke, M.P.B. and Veldman, G., 2014. Outcomes of patients who underwent staged repair for pulmonary atresia with ventricular septal defects and major aorto-pulmonary collateral arteries at Southampton University Hospital, United Kingdom. Sri Lanka Journal of Child Health, 43(2), pp.84–87. DOI: http://doi.org/10.4038/sljch.v43i2.7008
Published on 11 Jun 2014.
Peer Reviewed

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