Hypoplastic left heart syndrome (HLHS) is a rare congenital disease that is present at birth. The word hypoplastic means incomplete or development of an organ or a tissue, in this case the left side of the heart is underdeveloped. Most of the structures in the left side of the heart are hypoplastic in a baby with HLHS. In a child born with HLHS, the mitral valve is too small or completely closed which means there is no way for blood to get from the left atrium to the left ventricle.
The left ventricle is very small so it can’t hold much blood. And the aortic valve is too small or completely closed which means the aorta can’t pump enough oxygenated blood into the body. In North America, there is about 0.016 – 0.036% of babies born with this condition and there is a 100% mortality rate when left untreated. It accounts for about 2-3% of all congenital heart diseases and it accounts for 23% of all infants who die from such causes.
Hypoplastic left heart syndrome occurs in up to 4 out of every 10,000 babies born alive. HLHS occurs more often in boys than in girls. About 10% of babies with HLHS also have other birth defects. When looking for HLHS in a newborn baby, the symptoms could show up at birth or even a few days after birth. These symptoms may include blue or purple tint to the lips, nails, and skin which is also referred to as cyanosis, difficulty breathing, difficulty feeding, lethargy, which is sleepy or unresponsive, pale skin, sweaty or clammy skin, cool skin, fast heart rate, heavy and/or rapid breathing. The underdevelopment of the heart usually starts at two months of pregnancy. Scientists and doctors are still unsure why it happens or if there are any particular people at risk for hypoplastic left heart syndrome. With HLHS, there are many dangers and risks that are presented for the child. There are obvious risks such as the child will not be able to pump enough blood to the body, but there are also many unforeseen complications and risks after treatment that we will look at later. When diagnosing a child with HLHS, you can use screen for birth defects using screening tests. The baby could show signs of heart failure such as faster than normal heartbeat, lethargy, liver enlargement and rapid breathing. The tests that health professionals use to diagnose HLHS may include an electrocardiogram, echocardiogram, x-rays of the chest, or a cardiac catheterization. Treatment for HLHS may include medicines to help strengthen the heart muscle, lower their blood pressure, and work with the body to help get rid of extra fluid. Nutrition can also play a factor in helping babies with HLHS. Babies with HLHS should most often be eating a high-calorie formula or may be fed through a feeding tube because the baby may be too tired to eat and won’t have a healthy amount of weight. There is also surgery that will help restore heart function. With hypoplastic left heart syndrome, there are three stages of surgery that the infant will have to go through ” Norwood Procedure, Bi-directional Glenn Shunt Procedure, and Fontan Procedure. The Norwood Procedure will be done within the first two weeks of the baby’s life. During surgery, the surgeons create a new aorta and connect it to the right ventricle to help the heart pump blood to the lungs and the rest of the body. The Bi-directional Glenn Shunt Procedure is done around 4 to 6 months of age and it creates a connection from the pulmonary artery to the superior vena cava to reduce the work the right ventricle has to do by allowing blood to return from the body and go straight to the lungs. The last procedure is the Fontan procedure done anywhere from 18 months of life to 3 years of age. In this procedure, the doctors will connect the pulmonary artery to the inferior vena cava making it so that oxygen-rich blood and oxygen-poor blood will no longer mix. This procedure also returns the infant’s skin back to a normal color. Even with these surgeries, the infant is not cured from HLHS. They still will need to follow up regularly with a cardiologist and will have lifelong complications. A heart transplant is a possibility in the future as well. With these procedures, there are still lots of complications that may arise. Some including long-term diarrhea, blockage of the artificial shunt, heart failure, irregular heart rhythms, strokes, sudden death, blood clots, or neurological impairment. There could also be some other complications with treatment such as the child may get tired easily when participating in physical activity, the nervous system could have developmental problems, or there could be buildup of fluid in the feet, lungs, abdomen, or lungs. When looking at postoperative care for a baby with hypoplastic left heart syndrome, you may be looking at many different tools and machines. These could include a ventilator to help the child breath, IV catheters to help with getting fluid to help the child, an arterial line to measure pulse, a nasogastric tube to keep the stomach drained of acid and gas bubbles that could possibly build up, a urinary catheter to allow the child to urinate, a chest tube to help drain the chest of blood that could build up post surgery, and a heart monitor to keep track of heart rhythm, heart rate, arterial blood pressure and other numbers. As far as prevention for a fetus with HLHS, there is no known preventions just like most congenital diseases. Because we don’t know what cause these types of diseases, we are not sure how to prevent them. After having a baby with hypoplastic left heart, there is a possibility that you can have another child without this congenital heart disease, but it is more likely that your second child will have HLHS. When choosing this topic to research, I chose a disease in babies because I want to be either a labor and delivery nurse or a neonatal nurse, both pertaining to infants. This subject of hypoplastic left heart syndrome is also close to my heart because my cousin was diagnosed with it as an infant and is still dealing with it at the age of 11. Although it is a rare disease, I believe that it is important to know about because it does still affect many infants. This information will help me as a nurse because even though it is a rare disease, I could still deal with it or a disease, condition or birth defect like it and know how to handle it because of its similarity to HLHS.Sources:Children’s Hospital. (2014, April 02). Hypoplastic Left Heart Syndrome (HLHS). 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