Ultimately, severe shock resulting in seizures, renal failure, liver failure, and worsening cardiac function may develop. Whether these problems are reversible depends on both the severity and the duration of shock. This heart defect is one of the most readily diagnosed on fetal echocardiograms and is one of the most common cardiac defects picked up on screening obstetrical ultrasounds. Such early diagnosis of the anomaly allows for prompt intervention for stabilization at the time of birth so that severe shock may be avoided.
Planning to deliver such an infant at a hospital capable of aggressive newborn resuscitation is important in improving the chances for a good outcome.
Echocardiography is the principal method of diagnosing hypoplastic left heart syndrome. It can give detailed information of the anatomy of the various cardiac structures affected in hypoplastic left heart syndrome, as well as important information about the function of the right ventricle, the heart valves, the size of the atrial septal defect important for blood mixing and the size of the patent ductus arteriosus. Cardiac catheterization is rarely used as part of the initial evaluation with this heart defect due to the high risks in an often unstable newborn.
Catheterization, though, does play an important role in the evaluation of the cardiopulmonary function and anatomy in older children with hypoplastic left heart syndrome while planning for later stages in the treatment. If the fetus has been diagnosed before delivery, stabilization measures are started immediately so the newborn does not become unstable.
In newborns that are delivered and then suspected of having hypoplastic left heart syndrome, stabilization begins even while diagnostic tests are going on.
The rapid stabilization of these infants must begin as soon as the diagnosis is suspected. Catheters are placed, usually in the umbilical blood vessels, which allow medications to be given and blood to be obtained for testing. An infusion of prostaglandin, a medication that prevents the patent ductus arteriosus from closing, is begun, thus maintaining the pathway for blood to reach the body from the right ventricle.
Even though the infant may have low oxygen saturations, supplemental oxygen is avoided since it tends to promote more blood flow to the lungs, which may steal blood flow from the body and place excessive demands on the already stressed single right ventricle.
Manipulations of medications and respiratory treatments including possible mechanical ventilation are performed to optimally balance the flow of blood to the body and the flow of blood to the lungs. Close monitoring is essential to detect any organ dysfunction and maintain cardiopulmonary stability because infants with this anomaly may be very unpredictable and undergo quite sudden changes.
There are essentially three treatment options that have been proposed for children with hypoplastic left heart syndrome. In the past, due to poor outcomes with available treatments at that time, no treatment was often recommended. Today it is rare that a family may choose not to treat a child with HLHS, though in cases when the infant is unable to be satisfactorily stabilized, no treatment may be advised.
Cardiac transplantation in the newborn period is performed as primary treatment for hypoplastic left heart syndrome at some centers in this country. While transplantation has the advantage of replacing the very abnormal heart of a child with HLHS with one of normal structure, this treatment is limited by the scarcity of newborn organs available for transplantation and the life-long need for anti-rejection therapy.
Additionally, although outcomes for transplantation continue to improve, and the incidence of rejection is lowest in patients transplanted as newborns, the average life span of the transplanted heart is limited currently less than 15 years.
The most commonly pursued treatment for hypoplastic left heart syndrome is "staged reconstruction" in which a series of operations, usually three, are performed to reconfigure the child's cardiovascular system to be as efficient as possible despite the lack of an adequate left ventricle. These surgeries do not correct the lesion, and are instead considered "palliative.
The first operation in the staged approach is known as the Norwood operation and is typically performed in the first week of life. The Heart of the Matter. Turek has performed the Norwood procedure hundreds of times and is a leader in the field. Share: Facebook Twitter. Ready for an appointment? Featured Doctors Images. Related Doctors. November 03, May 20, At this stage, all deoxygenated blood flows passively through the lungs.
Gateway Medical Building Fourth St. Norwood Procedure Treatment for hypoplastic left heart syndrome requires either a three-step surgical procedure called staged palliation or a heart transplant. Stage 1 This procedure is performed shortly after birth.
Stage 2 Bi-directional Glenn Operation This operation usually is performed about six months after the Norwood to divert half of the blood to the lungs when circulation through the lungs no longer needs as much pressure from the ventricle. Stage 3 Fontan Operation This is the third stage, usually performed about 18 to 36 months after the Glenn. Request an Appointment. Refer a Patient. Pediatric Cardiothoracic Surgery.
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