Many children with congenital heart disease have increased pulmonary artery pressure, particularly those with large VSDs. With painful procedures, these patients can experience pulmonary vasospasm. In such conditions, cyanosis and lethargy can develop and can mimic the hypercyanotic episodes of tetralogy of Fallot. Treatment is directed toward alkalinization with intravenous sodium bicarbonate and 100 percent oxygen to facilitate pulmonary vasodilation. Anxiolysis and analgesia are useful.
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