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Patent Ductus Arteriosus | Boston Children's Hospital

  • Patent ductus arteriosus is a congenital heart condition in which the ductus, a small opening or pathway between the pulmonary and aortic valves, remains open or patent. In most cases, the ductus closes within a day after birth. However, in patent ductus arteriosus, the ductus remains open, causing blood to mix between the two valves.

    • also known as PDA
    • sixth most common congenital heart defect
    • occurs twice as often in girls as in boys
    • if left untreated, can lead to a condition called pulmonary hypertension
    • surgery or cardiac catheterization common treatments for PDAs that don’t close on their own

  • What is patent ductus arteriosus?

    All babies are born with a small opening between their pulmonary and aortic valves.

    • While a baby develops in the uterus, it’s not necessary for blood to circulate through his lungs because oxygen is provided through the placenta.
    • During pregnancy, this opening is necessary to allow oxygen-rich (red) blood to bypass the baby's lungs and flow into the body.
    • This passageway is called a ductus arteriosus.

    At birth, when the placenta is removed, the baby's lungs must now provide oxygen to his/her body. As the baby takes the first breath, the blood vessels in the lungs open, and blood begins to flow through and pick up oxygen.

    At this point, the ductus arteriosus is not needed to bypass the lungs. Under normal circumstances, within the first few days or weeks after birth, the ductus arteriosus closes and blood no longer passes through it. Most babies have a closed ductus arteriosus within 72 hours of being born.

    In some babies, however, the ductus arteriosus remains open (patent). The opening between the aorta and the pulmonary artery allows oxygen-rich (red) blood to pass back through the blood vessels in the lungs. This is called patent ductus arteriosus.

    How common is patent ductus arteriosis?

    Patent ductus arteriosis is the sixth most common congenital heart defect, occurring in 5 to 10 percent of all children born with congenital heart disease. Patent ductus arteriosus occurs twice as often in girls as in boys.

    What causes patent ductus arteriosus? 

    Almost all children have a PDA present at birth. In some children, the PDA does not close, and remains open and the condition now becomes knows as patent ductus arteriosus (PDA). Although exact reasons why this happens in some patients and not in others are not knows, the most common association for a PDA is prematurity.

    PDA also can occur in combination with other heart defects.

    normal heart

    Why is patent ductus arteriosis a concern? 

    When the ductus arteriosus stays open, oxygen-rich (red) blood passes from the aorta to the pulmonary artery, mixing with the oxygen-poor (blue) blood already flowing to the lungs. The blood vessels in the lungs have to handle a larger amount of blood than normal. How well the lung vessels are able to adapt to the extra blood flow depends on how big the PDA is and how much blood from the aorta is able to pass through it.

    Extra blood causes higher pressure in the blood vessels in the lungs, a condition called pulmonary hypertension. The larger the volume of blood that goes to the lungs, the higher the pressure.

    The lungs are able to cope with this extra blood flow and pressure for a while, depending on exactly how high the pressure is. Without medical treatment, however, the blood vessels in the lungs become diseased by the extra pressure.

    Also, because blood is pumped at high pressure through the PDA, the lining of the pulmonary artery becomes irritated and inflamed. Bacteria in the bloodstream can easily infect this injured area, causing a serious illness known as bacterial endocarditis.

    What are the symptoms of a patent ductus arteriosus? 

    The size of the opening between the aorta and the pulmonary artery will affect the type of symptoms, the severity of symptoms and the age at which they first occur. The larger the opening, the greater the amount of blood that passes through and overloads the lungs. 

    A child with a small patent ductus arteriosus might not have any symptoms, and your child's physician may only have discovered the defect by hearing a heart murmur. Infants with a larger PDA may exhibit different symptoms. The following are the most common symptoms of PDA: 

    • fatigue
    • sweating
    • rapid breathing
    • heavy breathing
    • congested breathing
    • disinterest in feeding, or tiring while feeding
    • poor weight gain

    The symptoms of PDA may resemble other medical conditions or heart problems. Always consult your child's physician for a diagnosis.

  • How is patent ductus arteriosus diagnosed? 

    Patent ductus arteriosis is often first detected when your doctor hears an abnormal heart sound or heart murmur when listening to your baby’s heart.

    Diagnostic testing for congenital heart disease varies by the child's age, clinical condition, and institutional preferences. Depending on the type of murmur your doctor hears, he or she may order further testing such as:

    • chest x-ray
      • takes a picture of the heart and lungs
      • with PDA, the heart may be enlarged due to larger-than-normal amounts of blood-flow through the lungs
    • electrocardiography (ECG or EKG)
      • records the electrical activity of the heart
    • echocardiography (echo)
      • sound waves are used to create a picture of the heart and look for structural defects and other problems
      • can show the pattern of blood flow through the opening and determine how large the opening is, as well as how much blood is passing through it
      • An echo is the most common way that a PDA is diagnosed.
    • cardiac catheterization
      • gives very detailed information about the structures inside the heart
      • thin, flexible tube (catheter) is inserted into your child’s groin and is guided to the inside of the heart
    • cardiac magnetic resonance imaging (MRI)
      • non-invasive test that uses 3-D technology to accurately determine blood flow and functioning of the heart as it is working
  • What are the treatments for patent ductus arteriosus? 

    A small patent ductus arteriosus may close spontaneously as your child grows.

    A PDA that causes symptoms will require medical management, and possibly even surgical repair. Your child's cardiologist will check periodically to see whether the PDA is closing on its own.

    If a PDA does not close on its own, it will be repaired to prevent lung problems that will develop from long-term exposure to extra blood flow.

    Treatment may include: 

    Medical management

    In premature infants, an intravenous (IV) medication called indomethacin may help close a patent ductus arteriosus. Indomethacin is related to aspirin and ibuprofen and works by stimulating the muscles inside the PDA to constrict, thereby closing the connection.

    Your child's physician can answer any questions you may have about this treatment. Some children will have no symptoms and do not require medications. Others may need to take medications to help the heart and lungs work better. Medications that may be prescribed include:

    • digoxin - a medicine that helps strengthen the heart muscle, enabling it to pump more efficiently
    • diuretics – medications that help the kidneys remove excess fluid from the body

    Adequate nutrition

    Most infants with PDA eat and grow normally, but premature infants or those infants with a large PDA may become tired when feeding, and are not able to eat enough to gain weight. Options that can be used to ensure your baby will have adequate nutrition include:

    • high-calorie formula or breast milk
    • supplemental tube feedings - feedings given through a small, flexible tube that passes through the nose, down the esophagus and into the stomach

    Surgery

    The goal is to repair the patent ductus arteriosus before the lungs become diseased from too much blood flow and pressure. Surgical repair is usually recommended for infants younger than 6 months of age who have large defects that are causing symptoms, such as poor weight gain and rapid breathing. For infants who aren't showing any symptoms, the repair may often be delayed until after 6 months of age.

    Your child's cardiologist will recommend when the repair should be performed. Your child's PDA may be repaired surgically in the operating room or by a cardiac catheterization procedure. The surgical repair, also called PDA ligation, is performed under general anesthesia. The procedure involves closing the open PDA with stitches to prevent the surplus blood from entering your child's lungs. In selected cases, robotic surgery equipment is used to correct PDA using minimally-invasive surgery techniques.

    Cardiac catheterization

    During this procedure, the child is sedated and a small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin and guided to the inside of the heart. Once the catheter is in the heart, the cardiologist pass a special device, called a coil or occluder, into the open PDA, preventing blood from flowing through it.  Advancements in cardiovascular surgical repair include video-assisted thoracoscopic surgery. Children need to be at least 5 kg to be considered for transcatheter closure. Thus, premature infants, because of their small size, are not candidates for this procedure, and require surgical closure of the PDA.

    What is the long-term outlook for my child after PDA surgery? 

    In premature infants, the outlook after PDA surgery depends on the following: 

    • your child's gestational age
    • any other illnesses present in your baby

    Most children born full-term with a patent ductus arteriosus repair will live healthy lives after recovering from the hospitalization. Their activity levels, appetite and growth should return to normal.

    In some cases, your child's cardiologist may recommend that antibiotics be given to prevent bacterial endocarditis after you leave the hospital

  • What is the latest research on PDA? 

    Complementing the Cardiovascular Program of Boston Children's Hospital is the nation's most intensive clinical and basic research program focused on pediatric heart disease. Children's is a world leader in opening new avenues of "translational research," bringing laboratory advances to the bedside and doctor's office as quickly as possible. All senior medical staff members of the Cardiovascular Program participate in clinical research activities. 

    Boston Children's has pioneered interventional catheterization repair of many types of congenital heart defects, including patent ductus arteriosus. In many cases, this eliminates the need for open-heart surgery, allowing less pain, reduced chance of infection and shorter recovery time. Research also continues on the design and creation of heart valves using a patient's own tissue.

  • AustinAustin's story

    It was 3:08 a.m. on March 3, 2005, when the most beautiful miracle came into this world. Austin Thomas weighed 1 lb., 14 oz. and was 13 inches long. He made his debut 15 weeks earlier than his June 14 due date had predicted. Although he was tiny and had a lot of developing to do, he was absolutely perfect. It was amazing that a person so small could have such perfect fingers and perfect toes and be so beautiful. In those first few hours it would be hard to comprehend the torturous road this precious baby would have to travel and the battles he would have to fight to stay here with us.

    Less than 24 hours after his birth, Austin was transported to Boston Children's Hospital where he would live for the next seven months and for what would be the first of seven surgeries he would have to undergo in the next five months. Austin had two heart surgeries; one to fix a puncture made around the lining of his heart during the insertion of one of his many tubes; the second to close his patent ductus arteriosus (PDA) — an opening between the aorta and the pulmonary artery which usually closes on its own after birth.)

    Before his PDA surgery, doctors tried to medically close the opening, which caused a “blowout” (a hole) in his bowels, so that had to be surgically repaired as well.

    Austin also had another surgery — leaving a large scar across his back — to remove fluid that had been building up in his lungs. I always marveled at how much strength this tiny baby had to have to make it through all these surgeries, and I thanked God every day for the skill of the surgeons who could do these surgeries and save these babies.

    As bad as things seemed, my world came crashing down when I received a call from the nurse letting me know Austin was bleeding rectally and they did not yet know the cause. Although I spent every day and night with Austin, I was advised to go home to sleep, to try to keep up my strength. It was moments like this when I would regret the decision to leave. I spent the day with him and was shocked to see how badly he was bleeding, but was still hoping it was something that would pass — a side effect of being a preemie possibly.

    On April 20, 2005, Austin underwent his scariest surgery yet.  I knew something was wrong when the surgeon came out to talk to us only 20 minutes after beginning surgery. Austin had a condition called necrotizing enterocolitis (NEC). As it was explained to us, it appeared as if cement was poured into Austin’s belly. All of his intestines had fused together in one lump. Trying to separate the bowels at this stage would only cause severe damage and tear the bowels apart. The surgeon said it was the worst case she had ever seen. As a mom I hoped that just meant she hadn’t seen many. Then the words a mother never wants to hear: I was informed we would have to wait for 48 hours before even discussing options, because the surgeon did not see much reason for this discussion. After all Austin had been through I was going to lose my baby.

    I prayed through the night, but not the prayers you would think. I prayed that if he was not going to make it through this, or anything else that was yet to come, that I wanted God to take him now. It was selfish of me to keep this baby fighting so hard for me. If the end results were not going to be good, I did not want him to suffer any longer.      

    mark puderI’m glad he didn’t listen, because the next day I met the man who I consider to be my own special angel. Dr. Mark Puder approached me while I was standing next to Austin’s bed and asked to speak to me. A few of the nurses that watched Austin (and did it amazingly, too) had approached him asking if there was anything he could do to help us. The truth was, even if Austin survived NEC, he was facing a scary future. Because NEC is a bowel disease, Austin was not allowed, nor could he, take food by mouth. He would be fed through an IV. This type of feeding is called total parenteral nutrition (TPN). Although these feedings keep babies alive and provide the nutrients they need, they also cause some severe side effects. The most disturbing was the likelihood of Austin needing a liver transplant due to the damage TPN causes to the liver. So basically the liquid they were giving him to keep him alive could also kill him.

    I knew all of this when I sat down with Dr. Puder. He started off by saying, “I hope you say no.” He explained to me that TPN is made up of two parts. One part that was loaded with the vitamins that Austin needed and another part were essential fats (lipids). These lipids were made from plants. Dr. Puder explained that based on some research he had done, he believed that these fats are what do the actual damage to the liver. Dr. Puder had a replacement to offer me. This replacement was not approved by the FDA yet and would take some special permission to administer to my baby.

    The other thing I needed to think about was that there was only one other child receiving this product. This child was getting better. His liver function had improved, but he was also allowed to have some food by mouth, which is a natural way to heal the liver. So Austin would be the first baby receiving no other form of nutrition. Because he had little in the way of “proof” to show me, Dr. Puder had hoped I would say “no.” No good doctor wants to give a desperate mother hope and not deliver. I could tell by the way he spoke, that his hesitations were for me, but that he truly believed in what he was doing. He wanted to give me time to think it over, but I said “I don’t need any time. Tell me where to sign.” I was told my baby might not make it 48 hours, so I would have done anything to give him a chance to survive.

    This miracle is called Omegaven. Austin would be given it intravenously once a day and it would replace the lipid portion of the TPN. Unlike the lipids, Omegaven was made with fish fats rather than plant fats. Dr. Puder said it would take two weeks to start to show any results and they would test his liver functions daily to check. At the beginning of treatment, Austin looked like any other preemie going through all he was going through. The whites of his eyes were grey, his skin was yellow, and he was so thin you could make out all the ribs in his back. Dr. Puder visited every day and would let me know what the tests were showing.

    As if it were the magic number, two weeks almost to the day after Austin received the first dose of Omegaven, his bilirubin levels began to drop. These levels are associated with liver function and the higher they go the worse for your baby. It’s what makes them look yellow and their eyes grey. From that day forward, it was almost a daily occurrence. His once off-the-chart-levels were slowly decreasing, which is just the opposite of what usually happens to anyone who cannot eat and is only being fed by TPN.

    Austin’s skin started to turn pink, his beautiful blue eyes were bright and he was gaining weight! All of these alone were reason to celebrate. NEC causes some irreversible damage and there is always a reconstructive surgery planned to remove the damaged part of the bowel. In most cases surgeons wait at least three months to give the bowels enough time to heal, but they can perform these reconstructions as soon as six weeks if the health of babies is failing. Because there is less healing time, there’s more risk of the bowels still being stuck together in too many spots and causing further damage.

    As much I would have liked it all be over with, I am happy to announce that Austin was able to have a full three-month recovery time due to his improving, not failing, health. Because he had the time to heal, Austin only lost 12.6 inches of his 26.7-inch bowels. His reconstructive surgery was done by Dr. Tom Jaksic, an amazing surgeon. He informed me that the part of his bowels that Austin lost should not inhibit him at all and he will adapt to the smaller bowel and live a normal life filled with McDonald’s and junk food. Not only had Austin survived the “worst” case of NEC one surgeon had ever seen, but he was a chubby faced, pink-cheeked baby who had defied all odds.

    Austin had one small setback after his surgery and we had to delay the start of mouth feeding. But he finally came home on October 7, 2005, (7 months and 4 days old). We still attend the small bowel clinic with Dr. Jaksic and his team of incredible doctors. We often stop and visit some of his old nurses who always let me know that they had feared I would one day leave that hospital, but would not be taking a baby home with me.

    Austin is just about to celebrate his fifth birthday. He has none of the telltale signs of being a preemie (sunken cheeks, long face, smaller size). He is one of the tallest kids in his class, has the round face of a five-year-old and people are amazed to hear he could have been so small. Besides the many scars that adorn Austin’s belly and back, there is no way of knowing what this child has gone through. He has no delays in school, makes friends easily and has a memory that anyone would be jealous of.

    I spent seven months — day and night — at Boston Children’s, and I am humbled by what I have seen. Even with all that I went through to bring my baby home, I know I am one of the lucky ones. I am thankful for all of Austin’s great nurses, for all the many doctors who gave him care, and for Dr. Jaksic who handled such a delicate surgery and “fixed” my baby’s belly.

    I do, however, hold a special place in my heart for one doctor. Dr. Mark Puder will forever be an angel sent to us. I know in my heart, no matter what else had happened, the reason Austin is here today is because of him. The reason Austin is a healthy boy about to attend kindergarten and thriving in everything he does is because Dr. Puder saw a sad and desperate mother who would not give up on her son, and took a chance with a product he believed in and that saved my baby’s life.

    So to Dr. Puder, Dr. Jaksic and every other person at Boston Children's Hospital who made it possible for me to be a mother, as I had always dreamed to be, I thank you from the bottom of my heart. You will never fully understand the magnitude of what you do each day and the dreams you help come true.

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