American Medical Association Family Medical Guide - American Medical Association [175]
Symptoms
The lack of oxygen in the blood caused by transposition of the great arteries makes the baby’s skin turn blue (cyanosis) within the first few hours of life. He or she may breathe rapidly and have difficulty feeding. A doctor can sometimes hear a heart murmur through a stethoscope.
Diagnosis
If a baby’s skin turns blue after birth, the doctor will immediately order tests to look for heart defects and determine the cause. Diagnostic tests will include a chest X-ray, a recording of the electrical activity of the heart (electrocardiogram; see page 559), and an ultrasound examination of the heart (echocardiogram; see page 561. The baby will probably be transferred to a neonatal intensive care unit for evaluation by a pediatric cardiologist (a doctor who specializes in treating heart disorders in children).
Treatment
If a baby has been diagnosed with transposition of the great arteries, the doctor probably will recommend cardiac catheterization (see page 592). In this procedure, a thin, flexible tube (catheter) is threaded through an artery to the heart to enlarge the normal opening between the two upper heart chambers (atria). The opening will allow blood that has traveled to the lungs (and received oxygen) to pass to the right side of the baby’s heart and out to the body. Within the first 2 weeks of life, the baby also will need to have surgery to switch the positions of the aorta and pulmonary artery. After successful surgery, the child’s chances for a full, healthy life are excellent.
Hypoplastic Left Heart Syndrome
Hypoplastic left heart syndrome is a very severe congenital heart defect in which the left side of the heart is underdeveloped. The left ventricle (the pumping chamber), left atrium (the chamber that receives blood from the lungs), aorta (the major blood vessel to the body from the heart), and the valves in the left side of the heart are all absent or are extremely small. As a result, the right side of the heart must do the job of both sides to circulate blood through the body. Within days after birth, the baby’s heart begins to fail. Without immediate treatment, a child usually dies within a week.
Symptoms
A newborn with hypoplastic left heart syndrome may look healthy at first, but soon becomes weak and short of breath, has difficulty feeding, and may turn very pale or blue. His or her pulse is weak and the skin is cold, and he or she may have difficulty breathing. Within hours or days, a newborn will become critically ill and die unless he or she is given immediate care.
Diagnosis
If a newborn develops symptoms in the hospital nursery, a doctor usually will diagnose the problem with a chest X-ray, an ultrasound examination of the heart (echocardiogram; see page 561), or a recording of the electrical activity of the heart (electrocardiogram ; see page 559). If your baby shows any symptoms of hypoplastic left heart syndrome after leaving the hospital, take him or her to the nearest hospital emergency department immediately. He or she will need to be cared for at a medical center that has extensive experience treating babies with this type of heart defect.
At the hospital, the doctor will stabilize the baby with intravenous heart and blood pressure medications. The baby will need to have surgery within days to enable the right side of his or her heart to pump blood to the rest of the body and to insert a shunt to allow blood to flow to his or her lungs from the pulmonary artery. A second operation is done at about 6 to 12 months of age and a third one between ages 2 and 4. Even after these operations, a child’s heart will not be normal; he or she always will need medication to keep the heart functioning well and will need to see a pediatric cardiologist (a doctor who specializes in treating heart disorders in children) regularly. If the three-stage surgery is not recommended in your child’s case, a heart transplant