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Background: Double Outlet Right Ventricle (DORV) is a cyanotic congenital heart disease in which both the pulmonary artery and the aorta arise from right ventricle; hence, via an often-present ventricular septal defect (VSD), oxygenated blood in left ventricle returns to right. When it has features that mimic Tetralogy of Fallot (TOF), there will also be overriding aorta, pulmonary stenosis, as well as right ventricular hypertrophy. DORV can be associated with neurodevelopmental delays, particularly in children from resource-limited settings.
Case Presentation: We present the case of 11-year-old female, the sixth of seven children born to subsistence farmers of Igbo tribe, who presented with lifelong symptoms of breathlessness on mild exertion, easy fatigability, poor academic performance, poor growth and lack of self-care. Physical examination revealed central cyanosis, reddish conjunctivae, grade 4 digital clubbing, microcephaly (OFC 48cm), and neurodevelopmental delays, including an unsteady gait, hypertonia in the lower limbs, and disorientation. Cardiovascular examination showed a pulse rate of 135 beats per minute and a pansystolic murmur (A pansystolic murmur can occur too due to VSD depending on the pressure gradient) at left lower sternal border. Echocardiography confirmed the presence of TOF-type DORV. Urgent blood test revealed polycythaemia. The patient’s developmental delays were consistent with chronic hypoxemia arising from the congenital heart disease.
Conclusion: This case underscores importance of early diagnosis as well as intervention in children with TOF-type DORV, mainly in resource-limited settings. The neurodevelopmental delays seen in this patient draw attention to the need for multidisciplinary management approach that addresses both cardiac as well as neurological aspects of congenital heart disease in a resource-limited setting.

Keywords: Neurodevelopmental delay; Tetralogy of fallot; Double outlet right ventricle