Tetralogy of Fallot coexisting with sickle cell anaemia in a Nigerian child: course, complications, and review of the literature.
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Haematology unit and Sickle Cell Unit, Department of Paediatrics, Usmanu Danfodiyo University, Teaching Hospital, Sokoto , NigeriaAbstract
Tetralogy of Fallot (TOF) is a cyanotic congenital heart disease (CCHD) characterized by hypoxemia, cyanosis, and compensatory polycythaemia. Sickle cell anaemia (SCA) is a genetic disorder of the red cell haemoglobin characterised by recurrent erythrocyte sickling, haemolytic anaemia, and vaso-occlusive crises. Both are associated with significant morbidity and mortality and when they co-exist, complications are additive with poor outcomes. We report a fatal case of a 9-year-old boy diagnosed with SCA at seven months and TOF at age six despite having features suggestive of cardiac disease since infancy. He remained clinically stable (without hypoxic spells or sickle cell crisis) until age 8 when he had severe sepsis with malaria and anaemia warranting Intensive Care Unit (ICU) management. He presented a year later at the emergency unit with acute fever and altered level of consciousness and died within a few minutes of presentation. This case has brought to the fore that cyanotic heart disease such as TOF could coexist with SCA, and the outcome is fatal. A high index of suspicion is thus crucial to early diagnosis and improved outcome.
