We all know that hypoplastic left heart syndrome, or HLHS, is a congenital heart defect that can lead to pulmonary hypertension, pulmonary edema, and death.
The problem is that there are no official statistics on the survival rate of HLHS, so we have no way to know. But we’ve seen a few different ways to explain it: the left ventricle doesn’t pump at all, and this causes the lungs to fill with fluid, which then causes the heart to pump around in a funny rhythm until the heart is dead.
It’s called a “fatal septic shock” because the fluids in the right ventricle get too large and start pooling in the lungs, causing a condition that looks and sounds like a “fluid bubble in the lungs.” So the condition is called “hypoplastic left heart syndrome.
Basically, if the heart runs the wrong way, the lungs can’t get enough oxygen and the brain shuts down. That causes the patient’s body to stop beating.
This condition is very rare. In the USA, it’s about 1 in 100,000 births. It’s common in China, India, and Brazil, but even in these places it’s rare. The best way to avoid it is to be sure that if you’re having a baby with a heart defect your baby is treated with surgery immediately.
The good news is that it doesn’t happen very often. Hypoplastic left heart syndrome is caused when a child has a heart abnormality that prevents the muscles from working properly. It can occur in boys or girls but is more common in boys because of the greater development of the child’s heart. Surgery can prevent hypoplastic left heart syndrome and is generally the recommended treatment.
Because of the high rate of surgery, hypoplastic left heart syndrome can become life-threatening in some cases. One in every 1,000 newborns has it and the rest develop it between one and two years of age. Surgery to repair or replace the heart can mean a life-long wait as a newborn with hypoplastic left heart syndrome is not always born with a functioning heart.
The good news is that the rates of surgery are falling. In the U.K., only 40% of infants with this condition are born with a functioning heart. In the U.S., the numbers are much higher, though surgery rates have been declining since the ’90s.
There have been more than 1 million babies born with the condition since 2007, but the numbers are up by about 50% since 2010. Experts are hopeful that the rate of surgery will continue to decrease as a result.
Hypoplastic left heart syndrome is a condition in which the left side of the heart is underdeveloped. That means the right side is functioning. If, however, the right side of the heart is underdeveloped, then only the left side is working, and the baby will be born with a heart. The most common causes of this condition are a heart defect or a congenital heart disease. Congenital heart disease is a congenital malformation of the heart’s structure that occurs before birth.