Most of these babies will require surgery during infancy to correct their structural heart defect. Severe methemoglobinemia
The first-line treatment for severe methemoglobinemia is methylene blue, a medication that will reduce methemoglobin in the blood. This is possible because methylene blue oxidizes NADPH, which in turn can convert methemoglobin back to hemoglobin. Epidemiology
Out of all the babies born with congenital heart defects, about 25% have cyanosis as a result. Tetralogy of Fallot is the most common cyanotic cardiac heart defect.Methemoglobinemia is considered to be rare, with acquired methemoglobinemia encountered more than the congenital form. Outcomes
In the case of cyanotic causing heart defects, about 75% of infants survive to 1 year of age and 69% survive to 18 years of age. These individuals have an increased risk of developmental delay, heart failure, or heart rhythm disorders.Methemoglobinemia responds well to treatment, its prognosis is associated with the level of methemoglobinemia and the degree of end organ damage it can cause. Death can occur when levels reach 70%. History
The first successful operation to treat blue baby syndrome caused by Tetralogy of Fallot occurred at Johns Hopkins University in 1944. Through a collaboration between pediatric cardiologist Helen Taussig, surgeon Alfred Blalock, and surgical technician Vivien Thomas, the Blalock-Thomas-Taussig shunt was created. Dr. Taussig had recognized that children with Tetralogy of Fallot who also had a patent ductus arteriosus (PDA) typically lived longer, so the trio tried to create the same effect as a PDA by joining the subclavian artery to the pulmonary artery, relieving the childs cyanosis.
Blue baby syndrome can refer to conditions that cause cyanosis, or blueness of the skin, in babies as a result of low oxygen levels in the blood. This term has traditionally been applied to cyanosis as a result of:
Cyanotic heart disease, which is a category of congenital heart defect that results in low levels of oxygen in the blood. This can be caused by either reduced blood flow to the lungs or mixing of oxygenated and deoxygenated blood. Methemoglobinemia, which is a disease defined by high levels of methemoglobin in the blood. Increased levels of methemoglobin prevent oxygen from being released into the tissues and result in hypoxemia.Although these are the most common causes of cyanosis, there are other potential factors that can cause a blue tint to a babys skin or mucous membranes. These factors include hypoventilation, perfusion or ventilation differences in the lungs, and poor cardiac output of oxygenated blood, among others. The blue baby syndrome or cyanosis occurs when absolute amount of deoxygenated hemoglobin >3g/dL which is typically reflected with an O2 saturation of <85%.Both of these conditions cause cyanosis, or a bluish discoloration of skin or mucous membranes. Normally, oxygenated blood appears red and deoxygenated blood has more of a blue appearance. In babies with low levels of oxygen or mixing of oxygenated and deoxygenated blood, the blood can have a blue or purple color, causing cyanosis. Signs and symptoms
The main sign of blue baby syndrome is cyanosis.