Characteristics of tetralogy of Fallot in children seen at Chris Hani Baragwanath Hospital over last two decades

Background: Tetralogy of Fallot (TOF) is one of the most common cyanotic congenital heart defects seen in children. There is limited data on the characteristics of TOF in African children. Objective: To determine the clinical features, investigation findings, surgical interventions and outcomes of children with TOF in a South African tertiary care setting over a period of 20 years. Methods: A retrospective, descriptive analysis was done on patients with TOF at the Chris Hani Baragwanath Academic Hospital (CHBAH), who had surgery between June 1998 and June 2018. Results: One hundred, seventy-nine patients were included in the analysis. The median age of diagnosis was 13 months (IQR, 2.7 - 44.8 months). Hypercyanotic spells were documented in 90/179 (50.3%) patients. The most common associated genetic syndrome was 22q11 microdeletion (16/45; 8.9%). Associated cardiac anomalies included patent ductus arteriosus (4.5%), patent foramen ovale (11.2%), true atrial septal defect (5.6%) and atrioventricular septal defects (1.1%). Normal coronary artery variations were comprised of conus or infundibular arteries arising from the RCA which were mostly small (42/179; 23.4%), with fewer large vessels (6/179; 3.6%). Anomalous coronary arteries included a single coronary artery origin (7/179; 3.9%) and large LAD arising from the RCA and crossing the RVOT (2/179; 1.1%). A left-sided aortic arch (LAA) was diagnosed in 135/179 (75.4%) patients and a right-sided aortic arch (RAA) in 44/179 (24.6%) patients. Systemic-to-pulmonary shunts were performed in 19/179 (10.6%) patients, while 160/179 (89%) patients had corrective surgery. Severe pulmonary regurgitation was seen in 27/51 (52.9%) patients who had a transannular patch repair, with 18/27 (66.7%) subsequently having a pulmonary valve replacement. Conclusion:This study shows that the characteristics of TOF in children in our centre are similar to those in other centres inside and outside of Africa.
A research report submitted in partial fulfilment of the requirement for the degree of Master of Medicine (MMed) in Paediatrics to the Faculty of Health Sciences, University of the Witwatersrand, School of Clinical Medicine, Johannesburg, 2023
Tetralogy of Fallot (TOF), Heart defects, Chris Hani Baragwanath Academic Hospital