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Ventricular Septal Defect (VSD)

  • Ventricular septal defect (VSD) is a hole in the wall between the right and left ventricles of the heart.

    How Boston Children's Hospital treats VSD

    Doctors at Boston Children’s have revolutionized a way to treat VSD, and other congenital heart defects, without open heart surgery. In addition, Boston Children's has begun using angioplasty valve enlargement in order to preserve the pulmonary valve in treatment of VSD, thereby reducing the need for surgery at all.

  • What is ventricular septal defect?

    Ventricular septal defect is a congenital defect in which there is a hole in the wall between the two lower chambers of the heart, known as the right and left ventricles.

    In patients with VSD, oxygen-rich (red) blood passes from the left ventricle through the opening in the septum, and mixes with oxygen-poor (blue) blood in the right ventricle. This can place a great deal of pressure on the lungs.

    What causes VSD?

    Most VSD occurs by chance, with no clear reason for its development.

    During the first 8 weeks of fetal development, your baby’s heart begins as a hollow tube. It then develop partitions that become the septa (or walls) dividing the right side of the heart from the left. Ventricular septal defects occur when the partitioning process does not occur completely, leaving an opening in the ventricular septum.

    What are some symptoms of a ventricular septal defect?

    The size of the ventricular septal opening will affect the type of symptoms, severity of symptoms and the age at which they first occur.

    Symptoms often occur in infancy and may include:

    • fatigue
    • sweating
    • rapid breathing
    • heavy breathing
    • congested breathing
    • disinterest in feeding, or tiring while feeding
    • poor weight gain
  • How will my doctor diagnose ventricular septal defect?

    Your child’s doctor will perform a complete exam and look for signs of VSD, which may include heart murmurs. The location within the chest that the murmur is heard best, as well as the loudness and quality of the murmur will give your child’s cardiologist an idea of which, if any, heart problem your child may have.

    Tests your doctor may perform include:

  • What are the treatments for ventricular septal defect?

    Small ventricular septal defects may close spontaneously as your child grows. A larger VSD usually requires surgical repair.

    Your child's treatment may include:

    • medication to help the heart work better
      • digoxin –  helps strengthen the heart muscle, enabling it to pump more efficiently
      • diuretics –  help the kidneys remove excess fluid from the body
    • antibiotics to prevent bacterial endocarditis (an infection of the inner surfaces of the heart)
    • nutrition to help your child gain weight (infants with a VSD may become tired when feeding)
      • high-calorie formula or breast milk with nutritional supplements
      • supplemental tube feedings
    • cardiac catheterization to patch the hole
      • umbrella-shaped patch is closed and inserted into the damaged area through a small tube, called a catheter
      • umbrella patch is then opened to cover the hole
      • requires a small incision, avoiding the need for open-heart surgery
    • open heart surgery to patch the hole
      • surgeons stitch or patch the hole in the wall
      • This is an option when your child is not eligible for cardiac catheterization
  • The Problem

    Children with a ventricular septal defect often have small pulmonary valves that need to be repaired. The standard treatment is to put a patch across the valve, but that can often leave a child with a leaky valve.

    Innovative Solutions

    To tackle the problem of having to repeatedly fix leaky valves in children with VSD, doctors at Boston Children’s began a program aimed at trying to preserve the pulmonary valve using angioplasty valve enlargement in the operating room. This method helps avoid use of the transanular patch, which often breaks down as a child gets olds. So far, Children’s has performed more than 60 angioplasty valve enlargement procedures, and the incidences of failing patches has decreased from 70 percent to 30 percent.

  • AJAJ's story

    I was 18 weeks pregnant when we went for a routine ultrasound to check to see what gender our baby would be. It was a boy and my husband and I were thrilled to become first time parents. A few days later we got a call to go to a level 2 ultrasound because they saw a possible hole in our baby's heart. We were quickly whisked to a genetic counselor and she made an appointment to get an echocardiogram with the Advanced Fetal Center at Boston Children's. We soon found out that our baby had a complex congenital heart defect. We had no idea what a heart defect was until Dr. Wayne Tworetzky explained in detail what would happen. His diagnosis was transposition of the great vessels, ventricular septal defect, and pulmonary atresia. This was heartbreaking! We went through the rest of the pregnancy not really knowing what to expect, but I received amazing care from Brigham and Woman’s Hospital and Boston Children’s. They prepared us with tours of the Cardiac Intensive Care Unit and talked us through the steps that would be taken when he was born.

    The day finally arrived and AJ came into this world two and a half weeks early, weighing 7lbs 2oz and 19 1/2 inches long. Since we were prepared for him to come out not crying and having trouble breathing, we were quickly relieved when he came out pink and screaming. I actually got to hold him a few seconds before he was taken to the NICU, and then over to Boston Children's. Our journey was beginning.AJ

    While we awaited his surgery, we met some of the most amazing surgeons, doctors, and nurses.  AJ was 4 days old when he got his repair. The morning of his surgery was nerve racking. We just wanted it to be over and for him to heal so we could take our baby home. When they took him down it was the hardest moment for us, but we knew everyone that was with him was going to take the best care of our little guy. The surgery took four hours and then his surgeon, Dr. Bacha, came to tell us how it went. He told us AJ did great and went over the logistics and what to expect once we saw him.

    During the next three weeks, we spent ever waking minute between the CICU and 8 east. It seemed as though time was going by so slowly, but each day he was making progress. We had a few bumps, but minor ones, and before we knew it, we took our baby home to begin our lives together. Over the next few weeks, we had a few more unexpected returns and overnight stays on the 8th floor.  He ended up having to get a pacemaker because the closure of his VSD was causing his top chamber to be unable to communicate with the bottom chamber. We knew this may have been a possibility. We were finally able to make our way officially home when AJ was 6 weeks old.

    Since we have been home, we’ve lived a relatively normal life. AJ is developmentally on track, which was a topic of conversation before he was born. We have to go to more appointments to see his cardiologist than a typical child, we take a few extra precautions due to his pacemaker, and he will have more heart surgeries throughout his life, but normalcy surpasses it. By looking at AJ you would never know that he had such a traumatic entrance into the world, until you lift up his shirt to see his scars.

    Without Boston Children's Hospital, AJ would not be as vibrate and amazing as he is. He is truly is miracle and each day we are reminded how thankful and blessed we are because of this remarkable hospital.

    --- Christine Sorrentino

    An omphalocele and ventricular septal defect

    I’m sure that I’m one of many parents who credit Boston Children’s Hospital with having saved their child’s life. But I don’t think many parents have had an experience quite like ours. Aidan was born in April of 2007, with a giant omphalocele, a defect in which the abdominal organs develop within the umbilical cord, outside of the abdomen. This only occurs in an estimated 1 in 2,500 pregnancies. The defect was detected during a routine ultrasound at 20 weeks gestation. It’s pretty hard news to take when you are told your unborn child has a life threatening defect, and that the defect may be associated to chromosomal abnormalities or that other anomalies may be present. We thought ourselves lucky; the omphalocele appeared to be isolated, and his chances of survival seemed pretty good.

    Aidan Aidan was born at UMass Memorial Medical Center in Worcester; it was immediately obvious that he was in respiratory distress and he was placed on a ventilator. We decided to do non-operative management of the omphalocele, a decision that was not supported by the surgeons, but they respected our wishes anyway. After a few weeks, Aidan remained on the ventilator, and without any answers as to why he was still having respiratory difficulties, we made the choice to have Aidan transferred to Children’s Hospital Boston. He was 5 weeks old when he became a patient on 7 North, in the NICU. It was then that we met the wonderful team who would work so hard to save his life, which included Dr. Mary Mullen from Cardiology, Dr. Lawrence Rhein from Pulmonology, and Dr. Russell Jennings from Surgery. With the team in place, we began to get some answers.

    Aidan Tests were ordered, and a CT scan revealed that Aidan’s ribcage had not grown to proper proportions due to the omphalocele. His lungs were long and skinny, as was his ribcage. He also had a cleft sternum. Cardiologists performed a heart catheterization, which revealed two small holes, a ventricular septal defect (VSD) and patent foramen ovale (PFO), but more concerning than that was their finding that Aidan had pulmonary hypertension. We knew then that Aidan would have a long road to recovery, and that the ventilator would be necessary long term. He had a tracheotomy performed when he was 7 weeks old. One of the ideas that was brought up was to create a prone bed with a hole in it, which would allow Aidan to lie on his belly without compressing the omphalocele. The NOPCO brace shop was brought in, and Rusty Miller made a bed that accommodated Aidan’s needs. The doctors also decided on a new course of treatment for Aidan’s pulmonary hypertension (PH): Viagra. Although the effects of the drug have not been fully studied, it does appear to help improve PH in infants. We decided it was worth the risks.

    As the weeks and then months passed, Aidan seemed to become more stable. In August of 2007, Aidan appeared to be stable enough to transfer to Franciscan Children’s Hospital. Just before being moved there, nurses noted that you could see a pulse high up on Aidan’s omphalocele, which had grown quite large at that point. The doctors didn’t feel that this finding should prevent Aidan’s transfer, and so he was moved to the rehab hospital. He spent a total of 16 days there before needing an emergency transfer back to Children’s, this time as a patient of the M/SICU on 7 South. Aidan had become extremely sick with a respiratory infection and was very dehydrated. But most concerning was that we could now fully see his heart beating in his abdomen. This was something Dr. Mullen had not seen before, and she was uncertain how dangerous this might be. What Dr. Jennings realized was that Aidan had a congenital diaphragmatic hernia (CDH) that had gone undiagnosed up until this point. Aidan’s heart had migrated through that hernia, and was now in his abdomen, a condition known as ectopia cordis. At 5 months old, Aidan was finally diagnosed with Pentalogy of Cantrell. This is an extremely rare condition, and one that exceptionally few babies survive with.

    Aidan We waited for months for Aidan to be stable enough to endure surgery. That day finally came on December 3, 2007. Dr. Jennings took Aidan into surgery along with a heart surgeon. That as well as the CDH repair went without complication, but when Dr. Jennings went to repair the omphalocele, there was too much swelling, and much of the skin that had covered the omphalocele had become useless. Dr. Jennings had to resort to unconventional methods to save Aidan’s life. For weeks, Aidan was placed on heavy sedation and paralytics, and a traction device was devised which attached to the muscle and fascia of Aidan’s abdomen, and stretched it out. Twice a week, Aidan went back to the OR to have the organs reduced further into the omphalocele. After three weeks, the combination of stretching muscle and fascia and reducing the omphalocele resulted in a partial repair of the defect, requiring the use of Gore-Tex to complete the closure of Aidan’s abdomen. That final surgery happened on Christmas Eve, and our hearts were filled with joy as we witnessed a true miracle in the beginning of a new life for our son.

    The recovery following those surgeries was not easy. Aidan spent the next two months struggling to be weaned off the sedation he had become used to. My husband and I worked with Lauren Perlman of the Respiratory Department, and she trained us on how to use the ventilator which would accompany Aidan home. Finally, on March 4, 2008, Aidan left Boston Children’s to come home for the first time ever. We had waited 328 days for this. There were many hugs and tears of joy, and even songs for Aidan as the entire staff said farewell. What a bittersweet moment as we took our baby home, but left behind the doctors, nurses and staff that had become like family to us.

    Aidan Since coming home, so much has changed. Aidan was able to come off the ventilator and oxygen in July of 2008, and a month later he welcomed the arrival of a baby sister. For his second birthday this past April, Aidan had his final closure surgery on his abdomen, and Dr. Jennings created a beautiful little belly button for him. There are still struggles; Aidan has oral aversions and will not eat by mouth. He is 100% dependent on tube feedings. He is also about a year behind in his development. But his days are happy, and for the most part he is healthy.

    There isn’t a day that goes by that I don’t think about the challenges we faced in that first year of Aidan’s life, or of the wonderful doctors and nurses who not only cared for my son, but for my husband and myself.  I will never be able to thank them enough for their compassion, their talent, and their commitment to Aidan. My son is now a proud survivor of Pentalogy of Cantrell, and there are few who can say that. That could not have happened without Boston Children's Hospital.

    -Sarah Doyle

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