Fetal health center

Your baby has been diagnosed with an atrioventricular canal (AVC) abnormality, a condition where the heart doesn’t form normally before birth. We understand how frightening it is to hear your baby has a heart problem, so please know that you are in the right place. We have the specialized expertise and technology to take the best care possible of your baby now, and as he or she grows. We encourage you to read the information below, educate yourself, and then talk with your doctor about the best plan for your baby.

What is an atrioventricular canal abnormality (AVC)?

In a normal heart, the right and the left sides of the heart are completely separated and each side has its own job. The right heart pumps low-oxygen “blue” blood to the lungs, while the left heart pumps high-oxygen “red” blood to the body. When an AVC defect is present, the two heart sides are not fully separated. There is a hole between the right and left upper chambers of the heart (atria) and also a hole between the right and left lower chambers of the heart (ventricles). These holes between the right and left sides allow the red and blue blood to mix. In addition, babies with AVC defects have valve abnormalities. The tricuspid valve (right-sided heart valve) and mitral valve (left-sided heart valve) control the flow of blood between the upper and lower chambers of the heart. A CAVC defect usually has a single common valve separating the upper and lower heart chambers instead of one right-sided and one left-sided valve. This common valve often doesn’t work well.

What causes an atrioventricular canal abnormality?

We do not fully understand what causes a baby to have an AVC defect, but we do know it is not caused by anything the mother did during pregnancy. An AVC defect occurs in 1 out of every 2,000-5,000 babies. In some cases, it can be part of a syndrome and the baby can have other problems. An AVC heart defect is often associated with Trisomy 21 (Down Syndrome).

How is an AVC abnormality diagnosed?

Heart abnormalities are most commonly diagnosed by ultrasound during routine prenatal care. In some cases, however, there is no sign of an AVC on prenatal ultrasound, and the diagnosis is made in the first days or weeks after birth.

How will my baby’s heart diagnosis affect my pregnancy and delivery?

An AVC heart abnormality does not usually affect prenatal life. Your baby should grow normally and your pregnancy should not be affected by your baby’s heart abnormality. Labor and delivery are also usually not affected by this fetal heart problem. We want your baby to be full term (delivered near your due date) and have as normal of a delivery as is safe for you and your baby. Most hospitals will be able to care for your baby’s special needs after birth, but you should discuss your planned delivery location with your OB doctor.

What will life be like for my baby after birth?

A newborn with an AVC abnormality is usually stable and healthy during the first week or two after birth. However, the AVC heart abnormalities allow excess blood to flow to the lungs. This causes the heart and the lungs to work harder than normal. Without treatment, a baby with an AVC abnormality may breathe fast and have difficulty growing. Medicines can be used to help the heart and lungs work better, and feeding support may be needed to help your baby grow big and strong for surgery. Heart surgery is usually at six to nine months of age and is very low-risk. The surgeon will fix the holes in your baby’s heart and will also separate the common valve into two separate valves. After the surgery, most babies do not require long-term medications, physical restrictions or additional heart surgeries. Most children after AVC repair will have healthy and active lives.

A cleft lip is a condition seen at birth where a child has a split in the skin and muscle of the upper lip. The split can extend in any length including into the base of the nose. Often, the split also involves the gumline just behind the lip.

A cleft palate is also a birth abnormality where the split occurs in the roof of the baby’s mouth. It can extend in any length, from the soft palate near the back of the throat to the gumline at the front of the mouth. A cleft lip or palate can occur by itself or in combination.

What causes cleft lip and cleft palate?

A combination of environmental and inherited factors can prevent complete formation of the lip or mouth tissues early in pregnancy. Approximately 1 in 750 babies are born with some type of clefting. Sometimes, clefting results when a child inherits their condition from one or both parents, but other factors such as medications, drugs or dietary deficiencies can also increase the risk for clefting. Often, we do not know the exact cause. If a baby is born with a cleft and also has other birth differences, then physicians may recommend genetic testing to see if a specific genetic reason can be determined.

What are the effects of a cleft lip and cleft palate?

The effects of a cleft condition depends on the severity of the split in the tissue. An infant with a cleft lip may have difficulty latching to the breast or bottle for feeding in infancy. Some simple feeding change may be all the adjustment needed to help a baby feed effectively. A cleft lip also creates a visible difference in facial symmetry with the lips and nose. As a child grows, an unrepaired split can also cause some difficulties with speech.

The effects of a cleft palate include difficulty feeding and normal speech development. An infant with a cleft condition may also have difficulty latching onto or extracting milk from the breast or bottle at feeding. These feeding challenges, if not addressed, could lead to malnutrition and poor weight gain. There are some specialized bottle and feeding interventions that can help a baby feed effectively. As the infant grows, an unrepaired split in the roof of the mouth can also cause difficulties with speech and hearing.

How do you care for a child with a cleft lip or cleft palate?

If your child was born with a cleft condition, the ideal care setting is a clinic with a ‘cleft team’ where a group of surgeons, nurses, genetic counselors, speech and feeding therapists and many other experts come together to provide cleft-related care. By working together, your child’s cleft team can provide the most efficient and coordinated care. The Kansas City Cleft and Craniofacial Care Program housed at Overland Park Regional Medical Center offers this type of comprehensive care for children and adults.

Will my child need surgery?

We typically recommend surgery to repair cleft conditions. Repairing a split in the lip will create a more typical lip appearance and motion. There are rare instances where surgery is not an option for treatment. Surgery most often happens when the infant is three to six months of age.

Our physicians recommend surgery for infants born with a cleft palate to repair the mouth and help the tissue function more normally for speech and hearing. Cleft palate surgery most often happens between nine months and 15 months of age. Timing for surgery depends on the baby’s growth and overall health.

Cleft lip and cleft palate repairs are surgeries performed under general anesthesia. Typically, a baby will stay overnight in the hospital after surgery to make sure they are not in pain and that they are able to resume feeding well. After a cleft lip repair, infants resume their typical feeding routine from the breast or bottle shortly after surgery. After a cleft palate repair, infants may be on an altered diet for several days to ensure proper healing. Overall, babies heal relatively quickly after surgery.

Do you also care for adults with a history of cleft lip or cleft palate?

Living with a cleft condition may be a life-long endeavor. The challenges for a child born with a cleft abnormality may be significant and require different amounts of care over time. Often, cleft care providers focus on managing these challenges during childhood. Once these children become adults, they may still have medical needs. Their medical needs may involve speech, feeding, dental care, breathing or even cosmetic appearance.

If you are an adult with a cleft condition and ongoing medical concerns, we are happy to provide your continued care and help address your concerns. Adults with cleft conditions deserve the same thoughtful, coordinated care that children receive.

Your baby has been diagnosed with a Congenital Diaphragmatic Hernia (CDH), a condition in which the diaphragm doesn’t develop completely, creating a hole that allows the organs to move from the abdomen into the chest. We understand how frightening any non-routine pregnancy or birth experience is, so please know that you are in the right place. We have the specialized expertise and technology to take the best care possible of your baby now, and as he or she grows. We encourage you to read the information below, educate yourself and then talk with your doctor about the best course of action for your baby.

What is a congenital diaphragmatic hernia?

The diaphragm is the muscle under the lungs responsible for breathing. It separates the abdominal cavity from the chest cavity. The diaphragm is formed by several muscles growing together and meeting in the middle to form one whole muscle that separates the chest cavity from the abdominal cavity. When the muscles fail to meet in the middle, a hole in the diaphragm, the CDH, results. Through this hole, the abdominal organs can move into the chest. When the abdominal organs are in the chest, they press on and inhibit the development of the baby’s lungs. Even though most CDH occur on only one side (most commonly the left side), both lungs are affected.

What causes a congenital diaphragmatic hernia?

We do not fully understand what causes a baby to have CDH, but we do know it is not caused by anything the mother did or ate during pregnancy. CDH occurs in 1 out of 2,000-5,000 babies. In some cases, it can be part of a syndrome and the baby can have other problems. In many cases, though, CDH is an isolated condition.

How is a congenital diaphragmatic hernia diagnosed?

CDH is most commonly diagnosed by ultrasound during routine prenatal care. In some cases, however, there is no sign of a CDH on prenatal ultrasound, and the diagnosis is made after birth or even later in life. When a baby is born with CDH, he or she will have problems breathing and the belly is flat. These are signs to the doctor that the baby may have CDH. If an OB suspects CDH before birth, mom will be referred to one of our maternal-fetal medicine specialists. That physician may perform other tests to confirm the diagnosis, or get more information about the condition to devise an appropriate birth plan for the baby’s delivery.

What complications can occur with a congenital diaphragmatic hernia?

When a baby is born with CDH, he or she can have significant breathing problems. This is because the organs normally in the abdomen are in the chest and pressing on the lungs, inhibiting their development. The condition may also affect the baby’s circulation due to the pressure on the heart as well as the underdevelopment of the lungs and blood vessels. The underdeveloped lungs do a poor job of getting oxygen into the blood and eliminating carbon dioxide from the blood – a process called gas exchange that is necessary to survive. In addition, because the lungs don’t work properly, the heart has to work very hard to pump blood through the lungs (this is called pulmonary hypertension) and can result in heart failure.

The survival rate for babies with CDH is high – around 80 percent. Your doctor with talk with you about making sure baby is monitored as he or she grows. Babies with CDH often suffer from other complications, such as an inability to eat well and significant problems with gastroesophageal reflux. Babies in more severe cases can have ongoing problems with heart and lung function and need long-term support with their breathing, or medications to help with high blood pressure in the lungs.

Less common, babies with CDH can also have issues with hearing loss, chest wall problems while growing, and developmental delays. The hernia can come back in as many as 35 percent of cases.

For this reason, once your child leaves the NICU, it’s important that he or she continues to receive close follow-up from our multi-disciplinary group of pediatric specialists as well as your pediatrician to make sure your baby gets the best care possible and thrives as he or she grows.

How is a congenital diaphragmatic hernia treated?

Babies with CDH require surgery to fix the hole in the diaphragm and return the abdominal organs into the abdominal cavity. However, the biggest problem for babies born with a CDH is that their lungs are not fully developed and don’t function properly.

Therefore, stabilizing the baby’s heart and lungs is the first priority after birth. The baby may be placed on a ventilator or breathing machine to help him or her breathe. A tube is also placed through the nose or mouth into the stomach to keep the stomach and intestines empty and small in size so they don’t cause more compression of the lungs. During this period, the doctor will also perform blood work, heart ultrasounds and other tests to better understand how the heart and lungs are working, and how much support the baby needs from the ventilator and the medications.

Once the heart and lungs are stabilized, your baby’s doctors will start planning the timing of the surgery. Surgery may be performed within a few days of birth or delayed longer depending on how your baby is doing. In cases where a baby’s heart and lungs cannot be stabilized, we may transfer the baby to a facility that offers Extra Corporeal Membrane Oxygenation (ECMO), a type of heart-lung bypass that allows the baby’s heart and lungs time to rest and stabilize.

What does the surgical repair entail?

There are several ways to surgically fix CDH and the method used depends on the size of the hole in the diaphragm and how stable the baby’s heart and lungs are. In all cases, the pediatric surgeon initially puts all of the organs back into the abdomen, then assesses how big or small the hole in the diaphragm is. When the hole is small, the surgeon can use stitches to close it. When the hole is larger, this is not possible. In those cases, a mesh screen is used to repair the diaphragm. When babies with CDH have very stable heart and lungs, a minimally invasive approach can be used in which the surgeon is able to use small incisions and a camera to perform the surgery.

Are there complications after surgery?

Because the stress of surgery can worsen any lung or heart problems, CDH repair is often delayed. However, even after delay, the baby may still have worsening heart and lung problems after surgery. Therefore, your baby’s heart and lung function will be monitored closely after surgery, and we gradually wean him or her off of medications and breathing support until the baby is doing everything on his or her own. Some babies with CDH require breathing support and medications for a prolonged period of time and may even need to go home with medical devices to help them.

Once your baby passes a stool, small feedings can be started. This is usually done with a small tube passed through the baby’s nose or mouth into the stomach. Uncomfortable as it looks to us, babies tolerate this very well, and it allows the doctors to assess how well the baby’s intestines are working. If the baby is doing well on the feedings, the amount can be increased and eventually the baby can take a bottle by mouth.

Please keep in mind that babies with CDH can struggle with feeding, and this part of the hospitalization can feel like a roller-coaster and be very frustrating for families. While the babies are working on feeding, they can receive all of their nutrition through a specialized IV, and they grow very well on this. In some cases, the babies require a surgically placed feeding tube in the stomach to help them get all of their nutrition. Each baby is different and it’s hard to predict how long your baby will be in the NICU, but in general, a surgery to repair CDH means a hospital stay of six to 12 weeks.

Your baby has been diagnosed with gastroschisis, a condition in which the intestines develop outside the baby’s body. We know this can be very frightening and you are concerned for your baby. Please know you are in good hands with Overland Park Regional Medical Center’s fetal care team. Our specialists are trained and prepared to take care of your baby. We encourage you to read the information below, and talk with your doctor about the best care plan and options for your baby.

What causes gastroschisis?

Gastroschisis occurs when a hole forms in the baby’s abdominal wall and the baby’s bowel pushed through this hole. The bowel then develops outside the baby’s body in the amniotic fluid. When the baby is born, the intestines are exposed, and this places the baby at risk for hypothermia, dehydration and injury to the bowels.

The exact cause of gastroschisis is not known. When intestines develop normally, they start developing outside the baby’s abdomen. However, as the intestines develop, they normally fall back into the abdomen. In gastroschisis, the intestines fail to fall back into the abdomen and remain protruding out through a hole that is usually to the right side of the baby’s belly button. Some believe gastroschisis is caused by a problem with the blood supply to the bowel, while others believe it is due to scar tissue holding the bowel outside the abdomen.

Gastroschisis is not uncommon, and occurs in as many as one in 1,800 babies born. More recently, we've seen more babies with this condition. In most cases, gastroschisis is isolated and the baby has no other issues or conditions.

How is gastroschisis diagnosed?

As your doctor has likely told you, gastroschisis is commonly diagnosed by ultrasound during routine prenatal care. During pregnancy, babies with gastroschisis tend to have a lower than expected weight. When an OB suspects gastroschisis, he or she will begin to monitor the pregnancy very closely, given that later in pregnancy, babies with gastroschisis can have a higher rate of stillbirth. Babies who have this condition are usually delivered early, between 36 and 37 weeks.

How is gastroschisis treated?

When the intestines are exposed, it can cause several dangerous issues. The baby loses heat and can become too cold. The baby also sweats from the bowel and can become dehydrated, and lastly, the exposed intestines are at risk for being injured. Therefore, delivery should take place at a center such as Overland Park Regional Medical Center where a board-certified pediatric surgeon and experienced neonatal team are available to start the appropriate care for your baby right after birth to get their intestine back into the abdomen. This often requires a surgical procedure to put the intestines into the abdomen and close it.

What happens after surgery?

Once the intestines are in the abdomen, the baby is allowed to wake up and have the breathing tube removed. However, baby’s intestines will not work right away, and he or she will not be able to eat or stool immediately. A small tube is placed through the nose or mouth to keep the stomach empty. Babies tolerate this procedure very well and it helps keep them comfortable. During this period, a baby is fed through a specialized IV and can grow very well. During this time, you will be able to hold your baby and bond, although you won’t be able to feed him or her. Your nurse team will work with you, and if you are planning to breastfeed, you will be able to pump your milk, and it will be stored until your baby is ready to eat.

It can take as long as six weeks for the baby’s intestines to start working. The first sign is usually when the baby starts having stools. At the same time, we see the color of baby’s stomach tube turn from green to clear and it can be removed. At this point, the baby is ready to start eating small amounts of food. Feedings are started slowly, through a small tube passed through the baby’s nose or mouth into the stomach. If the baby is feeding well, the feeds are gradually increased until the baby is eating the right amount of feeds and gaining weight. At this point, your baby is ready to go home.

Please know this part of the recovery can be a roller-coaster ride, as all babies with gastroschisis are different and able to tolerate feedings at different rates.

What complications can occur with gastroschisis?

In 10 percent of babies with gastroschisis, there can be significant complications. The most common one is atresia, a condition where the intestine isn’t completely open. This results in a blockage of the bowels. This often isn’t diagnosed right away and can take weeks to diagnose-adding to the frustration and worry of parents. If an atresia is present, additional surgery is required.

There are other, more rare, complications, such as infection or blood flow issues which your doctor can discuss with you as appropriate.

What are the long-term effects for children with gastroschisis?

Over 90 percent of babies with gastroschisis have no long-term effects, and grow to lead normal, active lives. Usually, by the age of two, they have caught up with their peers in terms of growth and nutrition. Babies who have had gastroschisis have a higher rate of developing a hernia – a bulge or protrusion – in their belly button, which can be repaired with outpatient surgery.

Your baby has been diagnosed with a lung condition called pulmonary malformation, an abnormality of the lungs. This is sometimes also called congenital lung malformation. As with any non-routine occurrence during pregnancy, this can sound frightening, but there is no need to panic. Your doctor has detected the condition, which is the first step in closely monitoring, and if necessary, treating it. Pulmonary malformations often resolve themselves, which means your baby may or may not require treatment. If your baby does require treatment, rest assured you’re in the right hands. The specialists at Overland Park Regional Medical Center have successfully treated hundreds of babies with pulmonary malformations. We encourage you to take a few moments to educate yourself about the condition by reading the information below and then talking with your doctor. Above all, please know we are trained and ready to take very, very good care of your baby...and you.

What is a Pulmonary Malformation?

Pulmonary malformations are irregularities or abnormalities of the lung that occur when some of the baby’s lung tissue grows more than it should. There are multiple types of pulmonary malformations, however they tend to behave similarly during pregnancy and after a baby is born. Regardless of the type, we generally manage these conditions the same way during a pregnancy.

You may hear these malformations referred to as Congenital Pulmonary Airway Malformations (CPAM). Among more common types are Congenital Cystic Adenomatoid Malformation (CCAM), in which the excess tissue growth causes benign sac-filled cysts to form, and Bronchopulmonary Sequestration (BPS) in which more solid masses of lung tissue develop. Some babies have a combination of cysts and masses. Pulmonary malformations can be inside or outside of the normal lung, and in some cases, the malformation can even be in the abdomen. The effects of pulmonary malformations depend on where they are and how large they are. Some have no effects at all, while others become large enough to cause breathing problems for the baby by compressing the normal, healthy lung. Your doctor will closely monitor the malformation and take action if necessary.

What Causes Pulmonary Malformations?

We don’t know the exact cause of pulmonary malformations, but suspect that parts of the developing lung tissue get confused early on and grow abnormally. It is nothing that you have or haven’t done during pregnancy and there doesn’t appear to be a clear genetic cause. Consequently, we don’t see multiple family members being affected by pulmonary malformations, and the risk of it occurring in a sibling is very low.

How are Pulmonary Malformations Diagnosed?

As your doctor has likely told you, pulmonary malformations are most commonly detected by ultrasound during routine prenatal care. Once diagnosed, the lung spot or lesion should be evaluated by an experienced Fetal Care Team like the team at Overland Park Regional Medical Center. These specialists will evaluate and monitor the lesion and determine whether treatment is needed. How are Pulmonary Malformations Treated? It is very rare to have to treat pulmonary malformations during pregnancy. In fact, during the later stages of pregnancy, we often see the malformation start to shrink in size. We can sometimes facilitate this by administering steroids to the mom. In rare cases, the malformation can cause heart failure for the baby which manifests itself as hydrops, a large fluid build-up in the baby’s tissues and organs causing extreme swelling. However, because we are closely monitoring baby’s condition, our Fetal Care Team can quickly identify those cases at high risk, and treat with steroids to reverse the heart failure. This is successful in nearly 90% of cases.

After you deliver your baby, treatment depends on whether the baby shows signs of breathing issues. If we see no signs of that, your baby doesn’t need immediate treatment and can go home as normal. In around 10 percent of cases, the malformation will completely disappear by the time your baby is six months old. We can determine that by a CT scan of baby’s chest at that time. If the lesion is still present, we recommend removing it to prevent recurrent lung infections, or in rare cases, the development of a malignancy in the abnormal tissue. One of the reasons for delaying surgery until after the baby is six months of age is to allow the baby to grow enough so that we can use a minimally invasive procedure called thoracoscopic surgery to remove the tissue. With this procedure, the pediatric surgeon makes tiny incisions smaller than one-fourth of an inch in size and removes the lesion. This minimally invasive approach results in less pain, quicker recovery and smaller scars. Often, the baby can go home the next day.

If a baby shows symptoms at birth due to a pulmonary malformation, then, depending on the severity of the symptoms, we may need to do surgery right away. In many cases the respiratory symptoms at birth are mild and surgery can still be delayed. We will only recommend/perform surgery if the respiratory symptoms are severe. In the rare event that your baby needs surgery soon after birth, he or she may need to be placed on a ventilator and have the abnormal lung tissue removed through an open, surgical procedure called a thoracotomy.

There are three lobes on the right side of the lungs and two on the left. Pulmonary malformations are often confined to a single lobe of the lung, and the surgery to correct them usually involves removing an entire lobe. Babies tolerate this well and do not have long-term problems related to removal of a single lobe. The surgery will be performed by a board-certified pediatric surgeon and the general anesthesia that will be required, administered by one of our pediatric anesthesiologists.

As with any open surgical procedure, recovery will be a bit longer and the baby will need to stay in our NICU until he or she is breathing normally, feeding well, and gaining weight.

What Happens After Surgery?

Once the baby has fully recovered from the surgery, we will turn him or her over to the care of your pediatrician for routine well-child checks. Most children who have had this surgery do not suffer from any long-term effects and are able to participate in normal activities. Very few require any specialty care afterward.