Responding to the report of a baby turning blue is never an enjoyable event, but the dispatcher tells you that’s where you’re headed. You’re responding to a 3-month-old child turning blue. You run through some possibilities: airway obstruction, seizure. Or maybe mom just thought he turned blue. Your initial thoughts are all moved aside when you arrive to find the mother holding the child tightly to her chest with the infant’s arms and legs tucked in toward her chest. The child is definitively blue. You ask mom what’s going on as you approach, and she tells you her child was born with a heart defect. She explains that she was told by the child’s doctor that if the child turned blue, she was to hold her tightly to her chest and call 9-1-1.
Assessment of the child reveals an underdeveloped infant crying, being held by his mother. The mother is insistent that she must continue to hold the child to her chest. She does allow you to provide blow-by oxygen and feel for a pulse. The infant’s pulse rate is strong at 140 beats per minute, and his skin feels warm. She requests transport to the local children’s hospital where her child is treated. During transport, the child calms and his skin pinks. The mother tells you the condition her child was born with is called Tetralogy of Fallot.
Tetrology of Fallot is one of the most common congenital heart disorders accounting for 7–10% of all congenital cardiac malformations.1–2 It’s named for the physician who initially identified the structural malformations in 1888: Louis Arthur Etienne Fallot. However, the condition was first documented much earlier by Niels Stenson in the late 1600. The condition is sometimes referred to as Blue Baby Syndrome.
The condition has variations, but there are four primary alterations in cardiac structure. There’s an incomplete intervenricular septum or ventricular septal defect. This hole in the wall of the ventricular septum allows the mixing of blood from the left and right ventricle. The oxygenated blood in the left ventricle mixes with the deoxygenated blood in the right ventricle. There’s an overriding aorta. The aorta sits toward the right side of the heart over the ventricular septum. This placement toward the right side allows deoxygenated blood from the right ventricle to be pushed easily back into systemic circulation. The structural changes in the ventricular septum and the aorta cause a narrowing of the pulmonic opening, making it difficult for blood to move from the right ventricle into the lungs. Finally, there’s right ventricular hypertrophy or enlargement caused by the right ventricle having to push against an increased pressure caused by the narrowed pulmonic valve. When the child exerts itself or is stressed, the flow of blood from the right and left ventricles will flow predominantly through the aorta, decreasing bold flow through the pulmonary trunk and lungs. This results in the child becoming cyanotic.
Exertion in an infant can be as simple as eating or crying. These cyanotic episodes have been referred to as Tet spells. If a child with Tetrology of Fallot becomes cyanotic, parents and caretakers are told to put the child in a knee-to-chest position. By placing pressure on the vascular system of the arms and legs, there’s an increase is systemic resistance. This increase resistance causes a shifting of blood flow through the pulmonary artery and into the lungs. EMS providers should allow the knee-chest position and administer oxygen. Advanced level providers should establish vascular access and consider a fluid bolus to increase venous return and right-ventricular output. Subcutaneous morphine can be considered to calm patients and decrease their sympathetic response.
Surgical repair is required. The timing of the surgery depends on the severity of pulmonary obstruction. Long-term survival rates are good post surgical intervention, and children can expect to experience a normal life. They will, however, require lifelong follow-up with a cardiologist. There’s an unexplained occurrence of ventricular dysrhythmias in adult patients born with Tetrology of Fallot.
This case offers a great example of the rare but potentially alarming calls seen by EMS involving congenital defects. When presented with a child born with a congenital defect, listen to caretakers. Most times caretakers will have a good, at least basic, understanding of the child’s condition. So when squeezing the blue baby seems wrong, it may be right.
1. Orphanet Journal of Rare Diseases. (2009). Tetralogy of Fallot. In OJRD. Retrieved Apr. 18, 2012, from www.ojrd.com/content/4/1/2.
2. Bhimji S. (Apr. 4, 2012). Tretalogy of Fallot. Retrieved Apr. 18, 2012, from http://emedicine.medscape.com/article/2035949-overview.