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Solitary indeterminate single ventricle with aortic atresia

A newborn baby weighing 2.3 kg presented to the children’s emergency department with signs of respiratory distress. Initial assessment demonstrated poor peripheral perfusion, weak peripheral pulses and an elevated blood lactate level. Transcutaneous saturations were 85% on air. There was tachycardia, which was out of proportion to tachypnoea. A chest radiograph showed an abnormal cardiac silhouette. After consulting with the regional paediatric cardiology team, the child was commenced on a prostaglandin infusion and a transfer was arranged. Transthoracic echocardiography demonstrated a functionally single ventricle of indeterminate origin with aortic atresia. A decision was made to proceed towards single ventricle palliation. Due to the child’s small size, banding of the branch pulmonary arteries was performed and the child remained on prostin infusion as an interim procedure. A Norwood operation was performed at 2 months of age. A 5-mm Sano conduit was inserted. The child was discharged from hospital at 3.5 months of age. Without an antenatal diagnosis, infants with aortic atresia or severe aortic stenosis and single ventricle physiology present early in the postnatal period with features of cardiogenic shock. Fluid resuscitation and administration of prostaglandin are essential. Unless echocardiography is available locally, the diagnosis may not be confirmed until transfer has been made to a cardiology centre. Usually echocardiography is sufficient to make the diagnosis, although additional imaging modalities, such as computed tomography, cardiac magnetic resonance, or cardiac catheterization, are occasionally required. The Norwood procedure is used as a palliative stepwise strategy. Mortality has improved dramatically in the last 30 years; however, there remains a burden of morbidity. This case explores the diagnosis, strategy, and common pitfalls often encountered in managing children with this new diagnosis.