Strategies For Treating Hypoplastic Left Heart Syndrome

Hypoplastic left heart syndrome is a type of congenital heart defect that affects the left side of the heart. In patients with this condition, structures on the left side of the heart either fail to form or form improperly. For example, both the mitral and aortic valves may be absent or abnormally small. Similarly, the left ventricle and ascending part of the aorta are often underdeveloped. Hypoplastic left heart syndrome can be diagnosed through an ultrasound while the baby is in the womb, and surgery is needed soon after birth to treat it.

If the condition is not detected before birth, it can be diagnosed in newborns through an echocardiogram. Symptoms associated with hypoplastic left heart syndrome include a weak pulse, pounding heartbeat, breathing difficulties, and a blue tinge to the skin. Individuals with this condition frequently have another heart condition called an atrial septal defect (a hole between the heart's upper chambers). In the United States, an estimated 960 newborns are diagnosed with hypoplastic left heart syndrome each year.

Breathing Machine Assistance

The breathing difficulties associated with hypoplastic left heart syndrome are the result of low oxygen levels in the newborn's body. To ensure the baby gets sufficient levels of oxygen, doctors may use breathing machine assistance. This involves placing the newborn on a ventilator, a device that delivers oxygen. Patients may need to be sedated while a ventilator is in place.

During ventilation, doctors will monitor the baby's heart, lungs, and oxygen levels frequently. Using a stethoscope, they will listen to the child's chest, and a pulse oximeter is used to measure oxygen saturation. Medical staff will also regularly check the ventilation device for any signs of infection. Routine checks will also be done to make sure the device stays in the proper location, and the amount of oxygen delivered through the ventilator will be continually adjusted according to the patient's needs.

Atrial Septostomy

An atrial septostomy is a surgical procedure needed for patients who have an atrial septal defect, one of the heart defects associated with hypoplastic left heart syndrome. The procedure expands the atrial septal defect so the blood from both of the heart's upper chambers can mix together, improving the overall level of oxygen in the blood. Normally, this procedure can be performed in a cardiac catheterization lab or at the child's bedside.

Guided by ultrasound, the procedure typically uses a small balloon to expand the atrial defect. Babies are sedated with general anesthesia for this procedure, and pain relievers are given. After the procedure, patients will be closely monitored in the cardiac critical care section of the hospital. Overall, the procedure carries a low risk, and possible side effects include bruising, bleeding, infections, blood clots, and heart rhythm issues.