CARDIOVASCULAR JOURNAL OF AFRICA • Volume 33, No 3, May/June 2022 122 AFRICA Tetralogy of Fallot in the nascent open-heart surgical era in a tertiary hospital in south-west Nigeria: lessons learnt Olukemi T Bamigboye-Taiwo, Babajide Adeyefa, Uvie U Onakpoya, Olugbenga O Ojo, Joel O Eyekpegha, Abayomi Oguns, John AO Okeniyi Abstract Background: Tetralogy of Fallot (TOF) is the leading cyanotic congenital heart disease. We commenced open-heart surgery at the Obafemi Awolowo University Teaching Hospitals Complex (OAUTHC), Ile-Ife, Nigeria in 2016. Objectives: To review the incidence, pattern, management and treatment outcomes of TOF at the OAUTHC. Methods: A retrospective audit was undertaken of hospital records, including echocardiograms of patients with TOF seen from January 2016 to February 2020 at the Paediatric Cardiology Unit, OAUTHC. Results: Seventy-two patients (37 boys and 35 girls) aged 0.17–22 years had TOF. Thirty-three (45.8%) had surgery; 31 (93.9%) corrective surgery and two (6.1%) a modified Blalock–Taussig shunt. Complications following surgery included cardiac dysfunction, post-transfusion malaria, pulmonary regurgitation, pericardial effusion and death (15%). Thirty-nine (54.2%) patients had conservative medical management. Complications included polycythaemia and thrombotic stroke, and 14 (35.9%) patients died. Conclusions: TOF is associated with significant morbidity and mortality in developing countries. Early and safe corrective surgery is desirable. Keywords: congenital heart disease, tetralogy of Fallot, echocardiography, surgical repair, surgical outcome, conservative approach outcome, follow-up results Submitted 29/9/20, accepted 9/10/21 Published online 20/11/21 Cardiovasc J Afr 2022; 33: 122–126 www.cvja.co.za DOI: 10.5830/CVJA-2021-048 Congenital heart defects are themost common formof congenital abnormalities identified in humans, occurring in approximately one in every 125 live births.1 Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease (CHD) beyond the neonatal age group.2 The earliest account of TOF was first given in 1871 by Dane Niels Stensen, a Danish physician.3 However, it was EtienneLouis Arthur Fallot in 1888 who first described in precise detail the four cardinal features that characterise this condition.2,3 He identifiedTOFbasically as a single abnormality affecting themain pulmonary artery (PA) and the sub-pulmonary infundibulum, resulting in pulmonary stenosis/right ventricular outflow tract obstruction (RVOTO), ventricular septal defect (VSD), overriding of the aorta, and right ventricular hypertrophy.2,3 The degree of the RVOTO is highly variable and progressive. The specific terminologies used by Fallot were ‘la maladie bleue’ (the blue disease) or ‘cyanose cardiaque’ (cardiac cyanosis).3,4 Maude Abbott, a paediatric cardiologist of Montreal, Canada, coined the term ‘tetralogy of Fallot’ in 1924.3 TOF occurs in approximately one in 3 000 live births and constitutes seven to 10% of all CHD.5,6 It occurs with equal frequency in boys and girls, as well as in all races and ethnic groups.7 The aetiology is unknown, however, it has been associated with conditions such as untreated maternal diabetes mellitus, ingestion of retinoic acid in the first trimester of pregnancy, uncontrolled maternal phenylketonuria and trimethadione use in pregnancy.7,8 It has also been linked with chromosomal anomalies such as trisomies 21, 18 and 13, microdeletions of chromosome 22 and Alagille syndrome.6,8,9 However, approximately 80% of children with TOF are non-syndromic.8,10 TOF patients develop RVOTO and this determines the clinical severity of the disease.6,7 The severity of symptoms depends on the degree of branch and peripheral pulmonary artery stenosis, which may be a determinant factor for survival after surgical repair and subsequent quality of life and longevity.6,7 Cyanosis is often not present at birth, but with increasing hypertrophy of the right ventricular outflow tract and growth, cyanosis becomes recognisable within the first year of life.7,9,11 Hypercyanotic spells are particularly a problem in children under the age of two years. Severe spells may result in unconsciousness, convulsions or death.7,12 Older children have dusky skin, grey sclera, engorged veins, marked clubbing of the fingers and toes and easy fatigue.13-15 Paediatric Cardiology Unit, Department of Paediatrics, Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Nigeria Olukemi T Bamigboye-Taiwo, MD Babajide Adeyefa, MD John AO Okeniyi, MD Department of Paediatrics and Child Health, Obafemi Awolowo University, Ile-Ife, Nigeria Olukemi T Bamigboye-Taiwo, MD John AO Okeniyi, MD, jaookeniyi@gmail.com Cardiothoracic Surgical Unit, Department of Surgery, Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Nigeria Uvie U Onakpoya, MD Olugbenga O Ojo, MD Joel O Eyekpegha, MD Abayomi Oguns, MD Department of Surgery, Obafemi Awolowo University, Ile-Ife, Nigeria Uvie U Onakpoya, MD Olugbenga O Ojo, MD
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