Autoimmune Vasculitis in a Child Following Tetralogy of Fallot Repair in Uganda

Abstract

Background: Tetralogy of Fallot is one of the conotruncal abnormalities associated with 22.q.11 deletion syndrome which presents with thymic hypoplasia, hypocalcaemia and have an increased risk of autoimmune disorders. We report about a child post TOF repair who presented to the Uganda Heart Institute diagnosed to have autoimmune vasculitis. Clinical Case: An 11 year girl who had undergone TOF repair 8 years prior to the illness presented with persistent high grade fevers for 2 weeks, abdominal pain, petichae with no arthralgia. Clinical examination revealed a sick child febrile 39 °C, with petichae on palms and sole of the feet. Oral thrush and bleeds in the mouth and no dysmorphism. She also had blisters on the lower limbs with secondary infection. She was hypoxic with oxygen saturation of 86% on room air. Blood pressure 88/56mmHg with grade 3/6 ejection systolic murmur in the left upper sternal edge. Baseline laboratory results upon admission showed leukocytosis, WBC 17.9 × 103, with a neutrophilia, 16 ×103 (89%), Haemoglobin 12.3 g/dl, thrombocytopenia 93× 103 /μl and elevated C- reactive protein of 80 mg/l(NR <6 mg/l). The peripheral film revealed macro platelets, left shift and no schistocytes. The electrolytes were deranged; hypocalcaemia 1.6 (2.1-2.5), hypokalemia 2.6 mmol/L and hypomagnesaemia 0.6 mEq/L. Thyroid function tests and PT/APTT were normal. No organisms were grown on blood culture. urine analysis revealed yeast cells and cultured >105 candida albicans. Despite antibiotics, she worsened with headache, seizures and loss of consciousness. Cardiac echo showed intact VSD patch, moderate PA conduit stenosis and no vegetations. Brain CT was normal. Autoimmune panel C-ANCA was elevated (18U/ml). Intravenous methyl prednisolone was initiated and the child regained consciousness in 24 hours. Conclusion: Tetralogy of Fallot patients may manifest with hypocalcaemia, severe infections and have a tendency of autoimmune disorders. A multidisciplinary model is necessary in the management of these patients.