Pulmonary valve stenosis is a type of congenital heart defect. Congenital heart defects are heart problems a baby is born with. These heart problems are usually diagnosed at or before birth.
"Pulmonary" means "of the lungs."The pulmonary artery takes the blood from the heart to the lungs to pick up new oxygen. The pulmonary valve is the gate through which the heart pumps blood into the pulmonary artery.
"Stenosis" means "narrowed." In pulmonary valve stenosis, that gate is too narrow. The heart has to work harder to push blood through it. It also means less blood gets to the lungs. So the blood going out to the body doesn't have enough oxygen.
Even though the gate is too narrow, the baby's body is still able to get some oxygen. That's because there's a small blood vessel that connects the left and right sides of the heart. This blood vessel is called the ductus arteriosus. It is part of the circulation system before birth. It usually closes shortly after birth. But medicine can keep it open until the valve can be fixed. As long as it stays open, red blood mixes with blue blood so that at least some oxygen goes out to the baby's body.
A baby with pulmonary valve stenosis may also have other heart defects.
Your baby may need special care, such as being in the neonatal intensive care unit (NICU). This may be scary for you. But the hospital staff understands this. They will explain what happens and will answer your questions.
Your doctor may hear abnormal heart sounds, such as a heart murmur, when he or she examines your newborn.
Your doctor will order tests to find the cause of abnormal heart sounds or symptoms. The most common test is an echocardiogram, or "echo" for short. It uses sound waves to make an image of your baby's heart.
Other tests, such as an EKG (electrocardiogram), chest X-ray, and checking the amount of oxygen in the blood, also help identify the problem.
A fetal ultrasound, which looks at the baby's heart, may find this defect before birth.
When the narrowing of the valve is severe, the baby may have a blue tint to the skin. This is because not enough oxygen is getting to the baby's body.
Your doctor will help you understand your treatment choices and what to expect from each choice.
Your baby may get medicine to keep the ductus arteriosus open. The medicine may be given through a blood vessel in the belly button.
Your doctor may use a procedure called a balloon valvuloplasty (say "VAL-vyoo-loh-plas-tee") to stretch the valve so that it is more open. Your baby will be asleep during this procedure. The doctor puts a thin tube into a blood vessel in your baby's groin or umbilical cord. This tube is called a catheter. It contains a tiny uninflated balloon. The doctor moves the catheter through the blood vessel to the heart. The doctor feeds the balloon into the valve and then inflates it for a short time to stretch open the valve.
Surgery may be needed to repair or replace the valve.
Follow-up care is a key part of your child's treatment and safety. Be sure to make and go to all appointments, and call your doctor or nurse call line if your child is having problems. It's also a good idea to know your child's test results and keep a list of the medicines your child takes.
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Current as of: December 6, 2017
Adam Husney, MD - Family Medicine
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