Critical congenital heart disease (CCHD) refers to lesions of the cardiovascular system, present at birth, which if left untreated in early infancy, will severely compromise the infant’s well-being and survival. Transposed great arteries, hypoplastic left heart, total anomalous pulmonary venous drainage, coarctation of the aorta, and interrupted aortic arch account for more than 70% of CCHD. Until recently, clinical examination followed by blood gas analysis (100% oxygen challenge) and echocardiogram have been the mainstays for diagnosis. However, these methods are unsatisfactory in preventing missed diagnosis before discharge in hospital-born infants. Prenatal diagnosis results in 25% to 50% detection of CCHD in the United States at the present time. In the last 10 to 15 years, noninvasive transcutaneous pulse oximetry has provided the means and impetus for blood oxygen saturation screening as an adjunct to traditional screening methods. It now seems that sufficient evidence exists to embrace universal oxygen saturation screening as one of the newborn screening tests before discharge from the hospital. The optimal method for universal oxygen saturation screening remains debatable, continues to be studied, and is evolving. Nevertheless, the current state of universal oxygen saturation screening should help provide reassurance to the scientific, medical, and health policy communities that this is yet another example of good preventive medicine at work in pediatrics and newborn medicine.

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