A rare cause of metabolic acidosis; Hypoplastic left heart syndrome

Metabolic acidosis is a common problem especially in newborns. Metabolic acidosis develops due to acid deposition rather than HCO3 loss in body fluids. It separates into two groups as anion gap normal and anion gap increased depending on anion gap level. Metabolic diseases, neonatal sepsis, congenital adrenal hyperplasia and congenital heart diseases should be thought with the observation of metabolic acidosis in a previously healthy infants. NaHCO3 infusion, renal replacement treatment and tamps like karbicarb, dichlora acetate, tromethamine are used for treatment of congenital heart diseases or lactic acidosis secondary to hypoxia. In this article we want to emphasize there are not only congenital metabolic illnesses under this non-response acidosis table but also there are congenital heart diseases which are in substantial rate.