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In many cases, blue baby syndrome may be apparent shortly after birth but the condition may not become apparent until later. Because blue baby is often the result of an underlying and serious heart defect, newborns who exhibit the cyanosis must be taken to an appropriate pediatric cardiac facility as quickly as possible. There, physicians will attempt to diagnose the defect and develop a plan of action. This may include medications to manage the symptoms, interventional cardiac catheterization procedures or temporary surgeries (palliative) to give the child time to grow until the defect can be fixed or corrective surgeries to fix the underlying defect.
Medications may be used to ease the symptoms of some congenital heart diseases by reducing the workload of the muscles, vessels or chambers of the heart. A fetus is “protected” from many of the heart problems causing cyanosis because of two temporary communications that permit adequate mixing of blood as the blood is detoured away from the lungs. This is because the lungs are collapsed and get virtually no blood supply as a fetus and all of the oxygen passes through the placenta from the mother. Some medications, like prostoglandins, may permit continued blood flow to the lungs as was the case during pregnancy by keeping one of these channels, the patent ductus arteriosus, open until more lasting intervention is accomplished.
If medications are not helpful, then a more invasive treatment option may be advised, such as interventional cardiac catheterization. During this procedure, the physician can employ various strategies to improve valve, vessel and chamber problems. Working through a thin tube, a catheter, that has been inserted through a large blood vessel (e.g., in the upper thigh) and guided to the heart, physicians can open vessels and valves that have narrowed stenosis, close defects in the wall septum between the heart’s chambers and repair malformed vessels. Catheters are also used during palliative operations to create a right-to-left shunt that allows some oxygen-rich blood to be pumped out to the body. Catheter-based techniques are taking the place of open-heart surgery for repair of many heart defects and offer a less invasive treatment option for many children.
Although an approved in-utero surgery is not yet available, there are surgeries that can treat many heart defects soon after birth. These surgeries include the following:
- Arterial switch operation. A procedure in which the two reversed major arteries (pulmonary arteryand aorta) are switched to their normal positions.
- Shunting procedure. This is usually a temporary way to relieve the symptoms of a defect (palliative surgery), and the defect may be fully repaired after the patient has had some time to grow. In one such procedure, a Blalock-Taussig shunt is used to form a new passageway between the aorta and the pulmonary artery. Thus, additional blood can travel from the aorta to the lungs through the pulmonary artery, increasing the flow and quality of blood flow to the lungs and restoring the patient’s natural pinkish color.
- Damus-Kaye-Stansel procedure. A surgery performed to repair transposition of the great arteries with subaortic obstruction, in which the pulmonary artery is cut into two segments. The first segment is already connected to the lungs at one end, and the surgeon connects the other end to the right ventricle. Thus, oxygen–poor blood can travel from the right ventricle through the first segment of the pulmonary artery and to the lungs. The second segment is already connected to the left ventricle at one end, and the surgeon connects the other end to the aorta. Thus, oxygen–rich blood can travel from the left ventricle through the second segment of the pulmonary artery and out the aorta, to the rest of the body.
- Fontan procedure. This is a multiple-staged procedure that corrects such cyanotic conditions as pulmonary atresia, tricuspid atresia, and hypoplastic left heart syndrome (HLHS). It is performed by connecting the veins returning from the body to the pulmonary artery, thus allowing blood to travel directly from the body to the lungs, via the pulmonary artery, bypassing the right ventricle.
Many of these patients, both before and after surgery, are at risk of developing endocarditis, an inflammation or infection of the lining of the heart. To prevent this, they will need to take antibioticsbefore any minimally invasive or invasive procedures (e.g., dental cleaning) for life.
While the long–term prognosis depends on the nature and severity of the specific condition(s) and on the appropriate treatment options, chances are better than ever before that properly treated blue babies will grow up to live happy, healthy lives. |