Cardiology and Cardiac Surgery Conditions

Aortic valve stenosis (also known simply as aortic stenosis) is a condition that affects the aortic valve, a part of the heart that plays an important role in how blood is pumped to the body.

The aortic valve connects the heart’s lower left chamber to the body’s largest artery, the aorta. As it opens and closes, it controls the flow of blood from the heart to the rest of the body.

With aortic valve stenosis, the aortic valve may be too narrow or stiff, and missing one or more of the flaps that allow it to open and close properly. As a result, blood can’t flow through the way it’s supposed to. This puts strain on the heart, because it has to work harder than it should to pump blood out to the body.

Aortic valve stenosis is often part of a condition known as left ventricular outflow tract obstruction (LVOTO). It can also occur as part of other heart disorders, like Shone complex or endocardio fibroelastosis (EFE). So it’s important for a team of heart specialists to evaluate the patient to understand exactly what’s going on, make the correct diagnosis, and determine the right care. Connecticut Children’s Heart Center specializes in this team approach, and uses advanced cardiac imaging for a detailed diagnosis.

What are the signs and symptoms of aortic valve stenosis?

Many children and adults with aortic stenosis only have mild signs or symptoms, or none at all.

More serious cases may have the following symptoms.

Infants:

• Bluish or greyish skin, lips and nails
• Trouble feeding
• Trouble gaining weight
• Trouble breathing
• Sweaty or clammy skin

Older children and adults:

• Heart murmur
• Chest pain
• Short of breath
• Tired or dizzy

At Connecticut Children’s, we can diagnose many congenital heart problems in utero before babies are even born, so we have an action plan in place for a family and baby right from delivery.

What causes aortic valve stenosis?

Aortic valve stenosis usually occurs before birth, when the aortic valve doesn’t develop properly during early pregnancy. Doctors don’t know exactly what causes it.

This condition may also develop later in life as a result of a degenerative heart disease.

How is aortic valve stenosis treated?

If a case of aortic stenosis is mild, it may not need any treatment. But it’s important to check in regularly with a heart doctor to make sure it’s not getting worse.

More serious cases have a number of other treatment options.

NON-SURGICAL

• Medication to treat symptoms
• Balloon valve repair (valvuloplasty): Uses a long, thin tube (catheter) to place a balloon in the aortic valve, and inflate it to open the valve
• Sometimes for adults, transcatheter aortic valve replacement (TAVR): Uses a catheter to place a new aortic valve

SURGICAL

• Aortic valve repair (valvuloplasty): Open-heart surgery to reconstruct or reshape the aortic valve
• Aortic valve replacement: In rare cases, open-heart surgery known as the Ross procedure, which replaces the aortic valve using the baby’s own pulmonary valve

Most patients with a congenital heart defect, including aortic valve stenosis, need lifelong care from congenital heart experts. Connecticut Children’s Heart Center keeps patients connected to cardiologists who understand their unique anatomy, provides follow-up tests like echocardiograms at locations close to home, and continues congenital heart care in adulthood.

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A heart beat that’s irregular or uneven is called an arrhythmia.

Normally, the heart muscle squeezes and relaxes in a steady, predictable pattern, controlled by electrical signals. When something goes wrong with the heart’s electrical system, the heart may feel like it’s beating too fast (sinus tachycardia), too slow (bradycardia), or out of rhythm.

There are lots of different types of arrhythmias, depending on their exact cause. Two common types are atrial fibrillation and atrial flutter.

Many arrhythmias are harmless, and don’t need special care. But some can lead to health problems, and need to be treated. So it’s always important to have them checked out by a heart doctor.

What are the signs and symptoms of an arrhythmia?

Sometimes, an arrhythmia doesn’t have any obvious signs or symptoms. When symptoms do appear, they can include:

• Racing heart beat
• Slow heart beat
• Uneven or irregular heart beat
• Pounding heart beat
• Fluttering feeling in chest (“atrial flutter”)
• Chest pain or discomfort
• Dizziness or feeling lightheaded
• Trouble breathing
• Unusual sweating
• Feeling very tired
• Passing out

What causes an arrhythmia?

Sometimes, a baby is born with an arrhythmia. Sometimes it appears later in life.

The following can cause arrhythmias.

• Congenital heart defect, including problems with the heart’s electrical system
• Changes to the heart’s structure, such as from cardiomyopathy
• Overactive or underactive thyroid gland
• Electrolyte levels that are too high or too low
• Certain infections, including COVID-19
• Unusual reaction to medications
• The healing process after heart surgery

How are arrhythmias treated?

In some cases, arrhythmias go away on their own or don’t require any special treatment.

When arrhythmias do require treatment, the approach depends on the specific type of arrhythmia, how serious it is, and the patient’s weight and anatomy.

NON-SURGICAL

• Vagal maneuvers: Exercises that slow the heart rate
• Medication to control the heart rate or return it to a normal rhythm
• Medication to prevent blood clots
• Cardioversion: Electric shock procedure to restore the heart’s normal rhythm
• Catheter ablation: Procedure using a long, thin tube (catheter) to stop the path of electrical signals that cause arrhythmia
• Pacemaker: Using a catheter, device placed through the vein to control the heart rhythm
• Cardioverter-defibrillator (ICD): Using a catheter, device placed through the vein to watch for and control abnormal heart rhythms

SURGICAL

• Pacemaker: Device placed directly on the heart to control the heart’s rhythm
• Cardioverter-defibrillator (ICD): Device placed in the chest to watch for and control abnormal heart rhythms
• Maze procedure: Incisions in heart’s upper chambers to correct heart rhythm

Some patients with arrhythmias, especially those caused by a congenital heart defect, need lifelong care from heart experts. Connecticut Children’s Heart Center keeps patients connected to cardiologists who understand their unique anatomy, provides follow-up tests like echocardiograms at locations close to home, and continues congenital heart care in adulthood.

An atrial septal defect (ASD) is a hole between the upper chambers (atria) of the heart. The most common is called an ostium secundum ASD, located in the center of the wall separating the atria.

Depending on an ASD’s size and location, it can allow blood to mix between the atria, and cause too much blood to flow to the lungs. As a result, the heart and lungs have to work harder than they should, putting a strain on the body.

(A hole between the heart’s lower chambers is called a ventricular septal defect, or VSD.)

ASDs are among the most common types of congenital heart disease. Some ASDs are minor and go away on their own. Others require treatment. Many occur as part of other heart conditions, like partial anomalous pulmonary venous connection (PAPVC) or Scimitar syndrome – so it’s important to look at the entire heart, and understand or rule out other problems.

Families rely on Connecticut Children’s Heart Center for exactly that. We use advanced imaging technology like echocardiography and cardiac magnetic resonance imaging (MRI), and bring together specialists in cardiac catheterization, congenital cardiac surgery and more to determine the right plan of care.

What are the signs and symptoms of an ASD?

In less severe cases, many children with an ASD have only mild symptoms, or no symptoms at all. They might not even be diagnosed until later in life.

More serious ASDs may have the following signs.

• Heart murmur
• Heart beat that’s too fast, too slow or irregular
• Trouble breathing
• Lung problems and infections, such as pneumonia
• Poor appetite
• Poor growth
• Low energy

What causes ASDs?

ASDs develop before birth, when the inner wall (septum) dividing the left and right sides of the heart doesn’t form properly, leaving a hole. Sometimes, this condition is passed down from a parent. But most of the time, ASDs seem to occur by chance, with no clear cause.

How are ASDs treated?

Some smaller ASDs close on their own, or simply need a heart doctor to keep an eye on them. Others need a special procedure.

NON-SURGICAL

• ASD transcatheter repair: Minimally invasive procedure that uses a long, thin tube (catheter) to insert a mesh implant in the ASD, sealing the hole

SURGICAL

• ASD closure: Open-heart surgery to close the hole using stitches or a patch
• If needed, correct any other heart defects – for example, the Warden procedure to address PAPVC, a condition that often occurs in patients with ASDs

Some patients with ASDs need to check in regularly with their doctor throughout life. Connecticut Children’s Heart Center provides expert care close to home, continuing in adulthood with our nationally-accredited Adult Congenital Heart Disease program.

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An atrioventricular canal defect (also known as an atrioventricular septal defect, or an AV canal defect or AV septal defect) is a condition that develops before birth, when the central area of a baby’s heart doesn’t form properly during pregnancy.

With this condition, the wall dividing the left and right side of the heart has one or more holes in it (known as atrial septal defects or ventricular septal defects). Sometimes the two valves that connect the heart’s upper and lower chambers don’t develop correctly either. Instead of forming as separate valves, they may form just one common valve.

This can allow blood to mix between the heart’s chambers and leak from the valves, and cause too much blood to flow to the lungs. As a result, both the heart and the lungs have to work harder than they should, putting a strain on the body.

Many babies with an AV canal defect often have other heart problems too. Connecticut Children’s uses echocardiography and cardiac magnetic resonance imaging (MRI) to take detailed pictures of the heart. Our specialists work as a team to understand each patient’s unique heart conditions, and make a plan for treatment.

What are the signs and symptoms of an AV canal defect?

Many children and adults only have mild signs or symptoms, or none at all. More serious cases may have the following symptoms.

• Bluish or greyish skin, lips and nails
• Heart murmur
• Heart beat that’s too fast, too slow or irregular
• Trouble breathing
• Chest pain
• Low energy
• Swelling in the legs, ankles and feet

At Connecticut Children’s, we can diagnose many congenital heart problems in utero before babies are even born. We work with our award-winning neonatologists, fetal cardiologists, the mother’s labor and delivery doctors, and our other pediatric specialists to plan ahead for the birth and the important moments that follow.

What causes AV canal defects?

AV canal defects develop before birth, when the baby’s heart doesn’t form properly during the first eight weeks of pregnancy.

Doctors don’t fully understand what causes this condition, but have noticed that it often occurs in in children with Down syndrome (trisomy 21). It may also be passed down from a parent, or caused by contact during pregnancy with certain chemicals or illnesses.

How are AV canal defects treated?

Babies born with this condition typically need to have surgery within their first six months of life. The timing and type of surgery depends on their unique condition and health.

Connecticut Children’s cardiac surgeons specialize in cutting-edge techniques to treat atrioventricular canal defects. Our team is known for performing complex surgeries on even the tiniest babies, with outstanding surgical outcomes.

NON-SURGICAL (before surgery)

• Medication to manage the condition until the baby is big enough for surgery

SURGICAL

• Two-ventricle (biventricular) repair: Complex procedure to repair or replace any valves that didn’t form correctly, creating a normal, two-ventricle arrangement
• Less often, staged reconstruction known as “single ventricle palliation”: A series of open-heart surgeries to rebuild the heart and redirect how blood flows through it. This approach may be used when the AV canal is “unbalanced,” meaning it can’t support two ventricles.

• Norwood procedure: Allows the right ventricle to pump blood to the body
• Glenn procedure: Connects a large vein to the pulmonary artery, allowing blood to flow directly to the lungs
• Fontan procedure: Directs blood from the lower half of the body directly into the lungs to receive oxygen

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Cardiomyopathy is a disease that weakens and enlarges the heart muscle, making it harder for the heart to pump blood to the rest of the body. It can disrupt the heart’s rhythm, and cause extra fluid to build up around the lungs and body.

There are several different types of cardiomyopathy, which lead to different symptoms and treatments.

• Dilated cardiomyopathy, which is the most common type for kids, makes the heart muscle thinner
• Hypertrophic cardiomyopathy (HCM) makes the heart muscle thicker
• Restrictive cardiomyopathy makes the heart muscle stiffer
• Other rare types

Whatever its type, cardiomyopathy is a serious condition. While there’s usually no cure, there are ways to treat it. Connecticut Children’s experts use advanced heart imaging, including cardiac magnetic resonance imaging (MRI), to see detailed pictures of how the heart is functioning and make the right plan for each patient’s care.

Since cardiomyopathy can be caused by certain cancer treatments, Connecticut Children’s Heart Center works closely with our Center for Cardio-Oncology, where specialists detect and prevent heart damage in cancer survivors.

What are the signs and symptoms of cardiomyopathy?

At first, cardiomyopathy may not cause any symptoms. As the disease gets worse, so do its symptoms.

• In infants, poor feeding
• In infants, poor weight gain
• Heart murmur
• Heart beat that’s too fast, too slow or irregular
• Chest pain or discomfort
• Fast breathing or trouble breathing
• Feeling very tired
• Dizziness or passing out
• Swelling in the legs, ankles and feet
• Swelling in the belly

What causes cardiomyopathy?

For the most part, doctors don’t know exactly what causes cardiomyopathy. In some patients, there may be a link to the following risk factors.

• Taking certain medications to treat cancer
• Duchenne muscular dystrophy (DMD)
• Family history of cardiomyopathy
• Pregnancy complications
• Infections, ex. COVID-19
• Metabolic disorders, ex. diabetes
• Poor nutrition
• Contact with poisonous chemicals
• Heart valve problems
• Damage from a heart attack

But cardiomyopathy also occurs in patients without any of the above risk factors.

How is cardiomyopathy treated?

Treatment depends on the type and seriousness of each case.

NON-SURGICAL

• Medication to help the heart work better, lower blood pressure and/or get rid of extra fluid in the lungs and body
• Ventilator to help with breathing

Often, treatment is non-surgical, focused on what’s causing the cardiomyopathy and keeping the heart as healthy as possible. But in extreme situations, patients may need surgery.

SURGICAL

• Implantable cardioverter-defibrillator (ICD): Device placed in the chest to watch for and control abnormal heart rhythms
• Pacemaker: Device placed in chest to control the heart beat
• Ventricular assist device (VAD): Open-heart surgery to place a VAD in the heart to help pump blood to the rest of the body
• In serious cases, heart transplant

Most of the conditions in this list are congenital heart defects – a heart problem that a baby is born with. They’re sometimes referred to as congenital heart disorders, or birth defects.

What are the signs and symptoms of a congenital heart defect?

It depends. Many cause trouble breathing, extremely low energy, and a greyish or bluish tint to skin, lips and nails. Some cause an irregular heart beat, chest pain, dizziness, or swelling in the legs or belly. Some have very mild symptoms or no symptoms at all. Check the list for specific conditions.

What causes congenital heart defects?

These conditions occur when the baby’s heart doesn’t form properly during pregnancy, often during the first eight weeks. Most seem to occur by chance, without any known cause.

In the U.S., about 1 in 100 babies is born with some type of congenital heart defect.

How are congenital heart defects treated?

Some are minor issues that go away on their own. Others are serious disorders that need surgery or another procedure right away. Connecticut Children’s Heart Center specializes in diagnosing and treating the full range of these conditions, including very rare and complex cases.

Connecticut Children’s uses state-of-the-art heart imaging and cardiac catheterization to fully understand and diagnose each patient’s condition. We tap into experts across our 30-plus pediatric specialties to find the right plan of care. We offer cutting-edge treatments, from minimally invasive catheter procedures to complex surgeries with outstanding outcomes.

This care starts before a baby is born, with our fetal cardiologists, and continues through life with our nationally-accredited Adult Congenital Heart Disease program – one of the nation’s first accredited programs of its kind.

For information about specific congenital heart defects, check out the rest of this list.

Double outlet right ventricle (DORV) affects the heart’s connection to its largest artery, the aorta.

Usually, the aorta is attached to the left side of the heart, and another artery called the pulmonary artery is attached to the right. This allows blood to flow through the lungs to receive oxygen, and then for that “oxygen-rich” blood to flow through the rest of the body.

But in babies with DORV, both arteries are attached to the right side of the heart, so blood can’t take the correct path. Blood that’s too low in oxygen gets pumped out to the body, while oxygen-rich blood flows back to the lungs. This puts a strain on the heart, which has to work harder than it should to make sure the body gets enough oxygen-rich blood.

Babies with DORV are also born with a ventricular septal defect (VSD), a hole in the wall between the lower left and lower right chambers of the heart. Their condition can be more or less serious depending the size and location of the VSD, and the exact position of the aorta and pulmonary artery.

Sometimes babies born with DORV have other congenital heart problems too, including:

• Coarctation of the aorta
• Mitral valve problems
• Pulmonary atresia
• Pulmonary valve stenosis
• Right-sided aortic arch
• Transposition of the great arteries (TGA)

What are the signs and symptoms of double outlet right ventricle?

DORV is often detected before birth, or in the first few days after a baby is born. Symptoms include:

• Heart murmur
• Bluish or grayish lips, skin and nails
• Fast breathing
• Sweating or clammy skin
• Trouble feeding
• Poor weight gain
• Extreme sleepiness

At Connecticut Children’s, we can diagnose many congenital heart problems in utero before babies are even born. We work with our award-winning neonatologists, fetal cardiologists, the mother’s labor and delivery doctors, and our other pediatric specialists to plan ahead for the birth and the important moments that follow.

What causes double outlet right ventricle?

DORV occurs before birth, when the heart doesn’t develop properly during early pregnancy. Doctors don’t know exactly what causes it.

Babies with certain genetic conditions — like trisomy 13, trisomy 18, or DiGeorge syndrome — are more at risk for DORV, but it also occurs in babies without any of these conditions.

How is double outlet right ventricle treated?

Usually, babies with DORV need one or more heart surgeries during their first few years of life. The timing and type of these surgeries depend on the exact arrangement of their aorta and VSD, and any other heart problems or health issues.

Connecticut Children’s cardiac surgeons specialize in cutting-edge techniques to treat DORV. Our team is known for performing complex surgeries on even the tiniest babies, with outstanding surgical outcomes.

SURGICAL

• Intraventricular tunnel repair: Creates a tunnel from the VSD to the aorta and places a patch that controls the direction of blood flow
• Arterial switch: Moves the aorta to the left side of the heart and closes the VSD
• In rare cases, staged reconstruction known as “single ventricle palliation”: A series of open-heart surgeries to rebuild the heart and redirect how blood flows through it

• Norwood procedure: Allows the right ventricle to pump blood to the body
• Glenn procedure: Connects a large vein to the pulmonary artery, allowing blood to flow directly to the lungs
• Fontan procedure: Directs blood from the lower half of the body directly into the lungs to receive oxygen

Most patients with a congenital heart defect, including DORV, need lifelong care from congenital heart experts. Connecticut Children’s Heart Center keeps patients connected to cardiologists who understand their unique anatomy, provides follow-up tests like echocardiograms at locations close to home, and continues congenital heart care in adulthood.

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Ebstein anomaly is a birth defect that affects the tricuspid valve, which connects the heart’s right chambers and helps control how blood moves through the heart.

Normally, blood flows through the tricuspid valve on its way from the heart’s upper right chamber to the lower right chamber. The valve opens and closes to keep blood moving in the correct direction.

But in Ebstein anomaly, the tricuspid valve isn’t formed correctly, so the flaps that make it open and close don’t function properly, and part of the valve is in the wrong place.

Some babies with Ebstein anomaly are born with other heart problems, like an abnormal right ventricle, an atrial septal defect (ASD), pulmonary stenosis or pulmonary atresia. Often, Ebstein anomaly affects the heart’s electrical system too, causing an irregular heartbeat.

What are the signs and symptoms of Ebstein anomaly?

This condition can range from mild to serious. In mild cases, Ebstein anomaly might not cause any symptoms at all, and can go undetected for many years.

More serious cases of Ebstein anomaly can cause the following symptoms.

• Bluish or greyish skin, lips and nails
• Heart murmur
• Heart beat that’s too fast, too slow or irregular
• Trouble breathing, especially with activity
• Coughing
• Poor appetite
• Poor growth
• Low energy
• Swelling in the legs
• Swelling in the belly

At Connecticut Children’s, we can diagnose many congenital heart problems in utero before babies are even born using a fetal echocardiogram, or echo. We work with our award-winning neonatologists, fetal cardiologists, the mother’s labor and delivery doctors, and our other pediatric specialists to plan ahead for the birth and the important moments that follow.

What causes Ebstein anomaly?

Ebstein anomaly occurs before birth, when the fetal heart doesn’t develop properly during early pregnancy. It’s possible that in some cases, it’s passed down genetically from a parent. But most of the time, there’s no known cause.

How is Ebstein anomaly treated?

NON-SURGICAL

• Medical management of heart rhythm problems or heart failure
• Catheter ablation: Procedure using a long, thin tube (catheter) to stop the path of electrical signals that cause arrhythmia

SURGICAL

If needed, Connecticut Children’s cardiac surgeons specialize in cutting-edge techniques to treat Ebstein anomaly. Our team is known for performing complex procedures on even the tiniest babies, with outstanding surgical outcomes.

• Tricuspid valve repair: (based on the patient’s anatomy, ex. Cone procedure) to try to use the patient’s own valve and reconstruct it
• Tricuspid valve replacement: Replaces the valve
• Maze procedure: Creates incisions in the heart’s upper chambers to stop the path of electrical signals that cause arrhythmia
• In severe cases, heart transplant

Most patients with Ebstein anomaly need to follow up with their heart doctor throughout life. Connecticut Children’s Heart Center keeps patients connected to cardiologists who understand their unique anatomy, provides important tests like echocardiograms at locations close to home, and continues congenital heart care in adulthood.

A heart tumor, also known as a cardiac tumor, is an abnormal growth in the heart. It can develop almost anywhere in the heart: inside one of the heart’s chambers, in the outside wall, or in the heart muscle itself.

Heart tumors are rare in children. Most are noncancerous (benign) and will not spread. However, depending on their location and size, some benign tumors can still cause problems with how the heart works by blocking the flow of blood and causing an abnormal heartbeat.

A smaller number of heart tumors are cancerous (malignant).

What are the most common benign heart tumors in children?

• Rhabdomyomas: Rhabdomyomas typically form in the inside walls of the heart. Usually, there is more than one. These tumors mostly occur in children. Up to 75% are in children with a rare genetic disease called tuberous sclerosis, which causes tumors to form throughout the body. Rhabdomyomas often shrink and disappear on their own during childhood, and do not require special treatment.
• Fibromas: Fibromas are typically found on the inner wall of the heart’s lower chambers. They usually occur in infants and children. Depending on their size and location, fibromas can cause problems with the heart valves, disrupting how blood moves through the heart and to the rest of the body. They can also affect the heart’s electrical system, causing an abnormal heartbeat. Fibromas may require surgery.
• Myxomas typically form in the upper chambers of the heart. In young children, they’re very rare. They occur more frequently in teenagers, and are the most common heart tumor in adults.
• Teratomas are most often found in the heart sac (pericardium), the tight covering that surrounds the heart muscle. Usually, they occur at the base of the heart, where the aorta and pulmonary artery are attached. Depending on their size and location, they can block the flow of blood into these arteries and cause other problems.
• Lipomas are tumors made up of fat cells. In the heart, these types of tumors usually form in the membrane that lines the inside of the heart’s chambers.

What are the most common malignant heart tumors in children?

Malignant, or cancerous, tumors are extremely rare in children. When they do occur, these are the most common types.

• Sarcomas such as rhabdomyosarcomas, which form in muscle tissue
• Angiosarcomas, which can form in the heart chambers, most often in the right upper chamber
• Fibrosarcomas, which form in connective tissues
• Liposarcomas, which form in fat cells
• Primary lymphomas, which affect immune cells

What are the signs and symptoms of a heart tumor?

Symptoms usually depend on how big a tumor is and where it’s located in the heart. Many children and adults with heart tumors only have mild symptoms, or none at all.

When symptoms do appear, they can include:

• Heart murmur
• Heart beat that’s too fast, too slow or irregular
• Low blood pressure
• Trouble breathing
• Chest pain
• Fever
• Dizziness or fainting
• Weight loss
• Feeling very tired
• Muscle pain
• Swelling of legs, ankles and feet

What causes heart tumors?

A tumor forms when cells in the body reproduce uncontrollably.

If this abnormal cell growth begins in the heart, the heart tumor is known as a primary tumor. Primary heart tumors are rare, and usually noncancerous. In most cases, they’re caused by abnormal growth of heart tissue cells. In rare cases, heart tumors are passed down genetically from a parent.

If abnormal cell growth begins as a tumor somewhere else in the body and spreads to the heart, the heart tumor is known as a secondary tumor. They can have a variety of causes, including cancers like lung cancer, breast cancer, melanoma, renal cell cancer, or lymphoma.

How are heart tumors treated?

Doctors begin by carefully diagnosing the type, size and location of a heart tumor, as well as a patient’s overall health.

Some tumors, such as rhabdomyomas, go away on their own, and don’t require anything except regular check-ins with a doctor.

Other tumors need to be removed in surgery. If the tumor is cancerous, the patient may need to follow up with chemotherapy or radiation therapy. In extreme cases, they may need a heart transplant.

Connecticut Children’s Heart Center partners with our Center for Cancer & Blood Disorders to care for patients with cancerous heart tumors. As an integrated health system, we bring together the right experts and cutting-edge treatments, including access to novel therapies and clinical trials.

Heterotaxy syndrome, also known as isomerism, is a rare disorder that affects where organs are located in the body. During pregnancy, a baby’s heart may develop in the right side of the chest instead of the left. Other organs like the lungs, stomach, liver and spleen may form in the wrong place, not form properly, or be missing altogether. In some cases, multiple spleens form but none function correctly. Sometimes this condition affects only one or two organs; sometimes it affects many. It can lead to serious or life-threatening complications.

No two cases of heterotaxy syndrome are exactly alike, but most involve the heart – in particular, changes in how the heart’s upper chambers (atria) develop. There are two main categories.

• Right atrial isomerism: Instead of having a left atria and a right atria, the heart has two right atria. Often, this condition also involves problems with the heart’s valves, holes in the heart’s dividing walls, or other heart defects.
• Left atrial isomerism: Instead of having a left atria and a right atria, the heart has two left atria. This condition may also include valve problems, holes in the dividing walls, and problems with the heart’s electrical system. 

Often, patients with heterotaxy syndrome have other heart defects too, including problems with the heart’s ventricles, connections, veins and muscle.

These heart problems can range from mild to very serious and complex.

What are the signs and symptoms of heterotaxy syndrome (isomerism)?

Heterotaxy syndrome is often diagnosed before birth through fetal echocardiography, allowing Connecticut Children’s to put an action plan in place for a family and baby right from delivery. Or it’s detected very soon after birth.

The most common signs of heterotaxy syndrome are similar to many other heart problems.

• Bluish or grayish lips, skin and lips
• Fast breathing
• Trouble feeding
• Poor weight gain
• Low energy and activity

Sometimes in less severe cases, children with this condition only have mild symptoms, or no symptoms at all. They might not even be diagnosed with heterotaxy syndrome until later in life.

What causes heterotaxy syndrome (isomerism)?

This condition occurs before birth, when a baby’s organs don’t develop properly during pregnancy.

Sometimes, heterotaxy syndrome is passed down directly from a parent’s genes. Other times, something happens during pregnancy, like contact with a certain chemical, that suddenly changes the baby’s genes. Sometimes, there’s no known cause.

How is heterotaxy syndrome (isomerism) treated?

Heterotaxy syndrome affects each patient differently, so treatment depends on which organs are involved and how serious each problem is. (For example, many patients only have one fully working ventricle in their heart instead of two, which affects their surgical options.)

Connecticut Children’s Heart Center uses echocardiography and cardiac magnetic resonance imaging (MRI) to get a detailed understanding of the heart, and plan treatment that’s unique for each patient. Because heterotaxy syndrome can involve so many parts of the body, a patient’s care team also often includes experts in urology, nephrology and many more of our 30-plus pediatric specialties.

Here are some of the possible treatments for heterotaxy syndrome.

SURGICAL

• Pacemaker: Device placed in the chest to control the heart rhythm
• Repair holes in the heart’s wall
• If only a single working ventricle, staged reconstruction known as “single ventricle palliation”: A series of open-heart surgeries to rebuild the heart and redirect how blood flows through it
  • Norwood procedure: Allows the right ventricle to pump blood to the body
  • Glenn procedure: Connects a large vein to the pulmonary artery, allowing blood to flow directly to the lungs
  • Fontan procedure: Directs blood from the lower half of the body directly into the lungs to receive oxygen
• Other procedures to reconstruct the heart
• Reconstruct other organs

NON-SURGICAL

• Medication to help the heart or kidneys work better and protect against infections
• Cardiac catheterization (a minimally invasive procedure using a long, thin tube) to correct some heart problems

Patients with heterotaxy syndrome need lifelong follow-up care by congenital heart experts. Connecticut Children’s Heart Center specializes in this kind of care. We understand the unique anatomy of individuals who have had surgery or a procedure to correct this condition. We also provide advanced heart imaging close to home, and a seamless transition to congenital heart care for adults.

Hypoplastic left heart syndrome (HLHS) is a rare birth condition that affects the left side of the heart. It’s one of the most complex heart problems seen in newborns.

With HLHS, the heart’s left-sided valves (the aortic valve and the mitral valve) are either too small or didn’t form properly. In addition, the heart’s lower left chamber (ventricle), which is responsible for pumping blood out to the rest of the body, is often too small. So is the aorta, the main artery through which that blood normally flows. As a result of all of this, the heart can’t pump enough oxygenated blood to the body.

The baby’s heart often develops differently in a few other ways too.

• Babies with HLHS are often born with a hole between the left and right chambers of the heart, known as an atrial septal defect (ASD).
• The blood vessel known as the ductus arteriosus, which connects the aorta and the pulmonary artery, usually remains connected after birth instead of going away. This condition is called patent ductus arteriosus (PDA).

These changes in the heart’s design allow at least some oxygen-rich blood to flow out to the body. However, the heart has to work much harder than it should.

What are the signs and symptoms of hypoplastic left heart syndrome?

HLHS is almost always detected before birth, or soon after a baby is born. Babies may have the following symptoms.

• Bluish or grayish lips, skins and nails
• Fast breathing
• Trouble feeding
• Poor weight gain
• Low energy and activity
• Fewer than normal wet diapers

At Connecticut Children’s, we can diagnose many congenital heart problems in utero before babies are even born. We work with our award-winning neonatologists, fetal cardiologists, the mother’s labor and delivery doctors, and our other pediatric specialists to plan ahead for the birth and the important moments that follow.

What causes hypoplastic left heart syndrome?

This condition occurs before birth, when a baby’s heart doesn’t develop properly during early pregnancy. There’s no one cause for this heart condition. For many babies, it’s passed down through a parent’s genes, and is linked to other genetic syndromes. For others, it may be caused by coming into contact with a certain chemical during pregnancy. Or it may be some combination of these causes.

How is hypoplastic left heart syndrome treated?

Connecticut Children’s congenital cardiac surgeons specialize in complex techniques to correct HLHS, and are known for outstanding surgical outcomes.

SURGICAL

• Staged reconstruction known as “single ventricle palliation”: Between birth and age 3, a series of three surgeries to rebuild the heart and redirect how blood flows through it
  • Norwood procedure: Allows the right ventricle to pump blood to the body
  • Glenn procedure: Connects a large vein to the pulmonary artery, allowing blood to flow directly to the lungs
  • Fontan procedure: Directs blood from the lower half of the body directly into the lungs to receive oxygen
• Sometimes, surgery to enlarge the hole between the heart’s upper chambers, allowing more oxygenated blood to flow from the left to the right side of the heart
• In extreme cases, heart transplant

NON-SURGICAL (temporary measures before surgery)

• Medication or a stent to keep the PDA open, so the right side of the heart can continue pumping some blood out to the body
• Sometimes, cardiac catheterization to enlarge the hole between the heart’s upper chambers, allowing more oxygenated blood to flow from the left to the right side of the heart

Sometimes, patients take medication after surgery to help control how much blood goes to the lungs and body.

After surgery, patients with HLHS should follow up with congenital heart experts who understand their unique anatomy. Connecticut Children’s provides this lifelong care, including advanced heart imaging close to home, and our nationally-accredited Adult Congenital Heart Disease program.

Myocarditis is a condition that causes inflammation in the muscles of the heart, the myocardium. As a result of illness or a bad reaction to a chemical, the myocardium becomes swollen, or inflamed, which makes it harder for the heart to pump blood. This can also disrupt the heart’s electrical system, causing an irregular heart beat.

Usually, myocarditis is mild and goes away on its own. But serious cases, if not treated, can damage the heart.

What are the signs and symptoms of myocarditis?

Mild cases of myocarditis may not have any symptoms. More serious cases may cause the following:

• Chest pain
• Rapid, pounding or uneven heartbeat
• Trouble breathing
• Feeling very tired
• Passing out

What causes myocarditis?

For most kids, myocarditis is caused by a viral infection, including a common summer infection called coxsackie virus.

For all ages, possible causes include:

• Viral infection, including COVID-19
• Bacterial infection
• Fungal infection
• Immune conditions and autoimmune disorders
• Allergic reactions
• Unusual reaction to a medication
• Medicines used to treat cancer
• Eating or drinking a poisonous chemical

How is myocarditis treated?

Most cases go away on their own, without long-term effects.

If a case is more serious, it may require medicine to treat both the cause, like the original viral infection, and any symptoms. Doctors usually recommend avoiding major exercise for several months until the heart has completely recovered.

To know exactly what’s happening in the heart, doctors may use advanced cardiac imaging like cardiac magnetic resonance imaging (MRI). Connecticut Children’s 3T scanner shows 3D images of the heart, its tissues and how blood is flowing through it. This state-of-the-art technology is also fast – part of our comfortable and kid-friendly experience.

Patent ductus arteriosus (PDA) is a rare birth condition that affects the flow of blood around the heart and lungs.

During pregnancy, while a baby is still in the mother’s womb, a blood vessel called the ductus arteriosus connects the heart’s two main arteries. Once the baby is born, this connection is no longer needed. Normally, it shrinks and closes on its own within a few days.

But for babies with PDA, the ductus stays open. As a result, blood with and without oxygen mixes in the heart in a way that it shouldn’t, and too much blood flows to the lungs. This strains the heart, and over time, can damage the lungs.

What are the signs and symptoms of patent ductus arteriosus?

This condition is often found in the first few days or weeks of a baby’s life.

• Bluish or grayish lips, skin and nails

• Heart murmur
• Trouble breathing
• Trouble feeding, or not enough energy to feed
• Trouble gaining weight
• Infections
• Low energy and activity
• Poor growth

If PDA isn’t detected early, it can go undetected until later in life, and the symptoms – like feeling tired or short of breath – can seem like other conditions.

What causes patent ductus arteriosus?

Doctors don’t know exactly what causes this heart condition.

PDA is more common in premature babies, and can occur with other heart problems. It’s also more common among babies with neonatal respiratory distress syndrome, babies with genetic disorders such as Down syndrome, and babies whose mothers were infected with rubella (German measles) during pregnancy.

How is patent ductus arteriosus treated?

Connecticut Children’s Heart Center includes not only congenital cardiac surgeons, but experts in cardiac catheterization, a minimally invasive way to evaluate and treat heart problems.

This multidisciplinary team works with other pediatric heart experts to evaluate each patient, weigh all the treatment options, and find the best plan of care.

NON-SURGICAL

• Medication to help close the ductus
• Catheter closure: A long, thin tube (catheter) is used to place a special device to seal the ductus

SURGICAL

• Surgical closure: Closes the ductus, usually through an incision in the left chest

Pulmonary atresia affects the pulmonary valve, a part of the heart that controls how blood is pumped to the lungs.

Normally, blood flows through the pulmonary valve on its way from the heart to the lungs, where it receives oxygen before it’s pumped to the rest of the body.

But in pulmonary atresia, the pulmonary valve does not form at all. Blood can’t flow from the heart to the lungs, and the body can’t get the oxygen-rich blood it needs.

This condition occurs as part of a group of heart defects called tetralogy of Fallot, including a ventricular septal defect (VSD) and, often, an abnormal pulmonary artery.

As with all congenital heart defects, it’s important for a patient with pulmonary atresia to have lots of different specialists working together to guide their care. Connecticut Children’s Heart Center is known for this multidisciplinary approach, including experts in advanced cardiac imaging, cardiac catheterization and congenital cardiac surgery.

What are the signs and symptoms of pulmonary atresia?

Pulmonary atresia is usually detected before birth, or very soon after a child is born. Here are signs that a newborn may have pulmonary atresia.

• Bluish or greyish lips, skin and nails
• Heart murmur
• Trouble breathing
• Sweaty or clammy skin
• Feeding problems
• Low energy and activity

At Connecticut Children’s, we can diagnose many congenital heart problems in utero before babies are even born. We work with our award-winning neonatologists, fetal cardiologists, the mother’s labor and delivery doctors, and our other pediatric specialists to plan ahead for the birth and the important moments that follow.

What causes pulmonary atresia?

Pulmonary atresia occurs before birth, when the fetal heart doesn’t develop properly during pregnancy.

In some babies, it may be linked to one of these risks.

• Genetic condition like DiGeorge syndrome
• Family history of congenital heart problems
• Mother had an infection like rubella (German measles) during pregnancy
• Mother used certain harmful medications during pregnancy

But pulmonary atresia also occurs in babies without any of the above risk factors, and there may not be any known cause.

How is pulmonary atresia treated?

Pulmonary atresia must be addressed immediately when a child is born. The treatment plan depends on how severe the child’s case is, and if they have other health risks. Doctors may use medication and minimally invasive procedures at first, delaying surgery until the child is older and strong enough.

NON-SURGICAL

• Medication to help a newborn’s blood flow to their lungs
• Balloon atrial septostomy: Uses a long, thin tube (catheter) to place a balloon in a natural hole in between the upper chambers of the heart. This enlarges the hole and allows blood from both sides of the heart to mix, which results in more oxygen-rich blood going to the body
• Stent placement: Uses a catheter to place a rigid tube called a stent into the ductus arteriosus, an opening between the aorta and pulmonary artery. The stent keeps the ductus open, allowing more blood to travel to the lungs
• RFA (radio frequency ablation): Uses a catheter to create a hole between the right ventricle and the pulmonary artery

SURGICAL

• Shunting: Open-heart surgery to place a tube, or shunt, between the aorta and pulmonary artery, which allows more blood to flow to the lungs. Babies usually outgrow this within a few years
• Pulmonary valve and artery replacement: Open-heart surgery to put in a donor pulmonary valve and artery
• Staged reconstruction: Between birth and age 3, a series of surgeries or catheter interventions to rehabilitate the pulmonary artery

Patients born with pulmonary atresia need care that continues throughout their life, often including additional surgeries or catheter procedures to replace the donor valve and artery. Connecticut Children’s Heart Center is there every step of the way, with care that transitions into adulthood with our nationally-accredited Adult Congenital Heart Disease program.

Pulmonary hypertension is a type of high blood pressure that affects the lungs and heart. There are a few different kinds of pulmonary hypertension: For example, pulmonary arterial hypertension (PAH) involves problems in the lung’s blood vessels. Pulmonary hypertension caused by left-sided heart disease involves the heart’s left valves or pumping chamber.

In every type of pulmonary hypertension, the heart has to work harder than it should to pump blood into the lungs. This strains the heart, and causes it to enlarge and thicken over time.

Pulmonary hypertension is a serious condition that involves the whole body. Although there are a lot of kids with pulmonary hypertension, not a lot of pediatric health systems specialize in treating it. Connecticut Children’s is one of the few.

What are the signs and symptoms of pulmonary hypertension?

Pulmonary hypertension is often mistaken for other health issues, like asthma or being out of shape. The most common symptoms are related to breathing. They include:

• Trouble breathing, especially during activity
• Tiring easily
• Fainting spells
• Lightheadedness or dizziness
• Chest pain
• Bluish or greyish lips, skin and nails
• Swelling in the lower legs and ankles

What causes pulmonary hypertension?

Sometimes, pulmonary hypertension doesn’t have an obvious cause. Other times, it’s the result of another health issue, including:

• Congenital heart disease
• Severe forms of lung disease
• Connective tissue disease
• Sickle cell disease
• Treatment for cancer, liver or kidney diseases
• In rare cases, passed down genetically from parents

How is pulmonary hypertension treated?

Research is changing what we know about pulmonary hypertension every day. Right now, there’s no cure for it. But there are ways to prevent it from getting worse, and to manage its effects on the body.

Each patient’s treatment plan depends on which type of pulmonary hypertension they have, and any other health issues. Connecticut Children’s specialists in cardiac catheterization and advanced heart imaging use cutting-edge techniques to show exactly what’s going on in the heart.

Our experts in pediatric pulmonary hypertension lead multidisciplinary teams – including experts from rheumatology, pulmonary medicine, nephrology, intensive care, and lots of other specialties – to arrive at the right plan for each patient.

NON-SURGICAL

• Medication to lower blood pressure in the lungs
• Medication to help the heart work better
• Medication to improve blood flow to the lungs
• Medication to get rid of extra fluid in the body
• Medication to reduce blood clots in the lung’s blood vessels
• Oxygen to help with breathing

SURGICAL

• Sometimes, transplant evaluation (heart and lung).

Patients with pulmonary hypertension need regular, lifelong care from doctors who specialize in their condition – like the team at Connecticut Children’s Heart Center.

> Related: A New Normal: Lauren’s Story

Pulmonary valve stenosis (also known simply as pulmonary stenosis) is a condition that affects the pulmonary valve, which connects the heart’s lower right chamber to the lungs.

Blood flows through the pulmonary valve on its way to the lungs, where it receives oxygen before it’s pumped to the rest of the body. But in pulmonary stenosis, the pulmonary valve may be too small, narrow or thick, and the flaps that make it open and close may not be formed properly. As a result, it can’t open all the way, and blood can’t flow through the way it’s supposed to. This strains the heart, which has to work harder than it should to pump blood to the lungs.

This condition occurs as part of a group of heart defects called tetralogy of Fallot, including a ventricular septal defect and, often, an abnormal pulmonary artery.

What are the signs and symptoms of pulmonary stenosis?

Many children and adults with pulmonary stenosis only have mild signs or symptoms, or none at all. More serious symptoms include:

• Bluish or greyish lips, skin and nails
• Heart murmur
• Heart beat that’s too fast, too slow or irregular
• Trouble breathing
• Chest pain
• Feeling dizzy or lightheaded
• Feeling very tired
• Passing out
• Swelling in belly

What causes pulmonary stenosis?

Pulmonary stenosis usually occurs before birth, when the baby’s heart doesn’t develop properly during pregnancy.

In some babies, it may be linked to one of these risks.

• Genetic condition like DiGeorge syndrome or Noonan syndrome
• Family history of congenital heart problems
• Mother had an infection like rubella (German measles) during pregnancy
• Mother used certain harmful medications during pregnancy

But pulmonary atresia also occurs in babies without any of the above risk factors. For these patients, there’s often no known cause.

How is pulmonary stenosis treated?

If a case of pulmonary stenosis is mild, it may not need any treatment. But it’s important to check in regularly with a heart doctor (cardiologist) to make sure it’s not getting worse.

In more serious cases, doctors may fix or replace the pulmonary valve. Connecticut Children’s Heart Center specializes in a range of techniques, from minimally invasive cardiac catheterization to congenital heart surgery. Our experts work together to determine which approach is best for each patient.

NON-SURGICAL

• Treat symptoms with medicine
• Balloon valvuloplasty: Uses a long, thin tube (catheter) to place a balloon in the pulmonary valve, and inflate it to open the valve
• Transcatheter pulmonary valve replacement: Replaces the pulmonary valve using a catheter

SURGICAL

• Pulmonary valve repair: Open-heart surgery to reconstruct or reshape the pulmonary valve
• Pulmonary valve replacement surgery: Open-heart surgery to replace the pulmonary valve

Patients born with pulmonary stenosis often need to follow up with congenital heart experts throughout their life, including with regular tests like echocardiograms or cardiac magnetic resonance images (MRIs). If their valve was replaced when they were young, they may need additional surgeries or catheter procedures as they grow.

Connecticut Children’s Heart Center is there every step of the way. Our experts understand the unique anatomy of the heart after congenital surgeries or procedures. We’re proud to continue caring for patients in adulthood with our nationally-accredited Adult Congenital Heart Disease program.

> Related: Heart of a Champion: Jordyn’s Story

The heart has two pumping chambers, called ventricles. When a baby is born with only one working ventricle, it’s called a single ventricle defect. This creates serious and complex problems for the heart – but with state-of-the-art care, kids born with these kinds of congenital heart defects can thrive.

Single ventricle defects include the following:

• Atrioventricular Canal Defect (Atrioventricular Septal Defect)
• Double Outlet Right Ventricle (DORV)
• Ebstein Anomaly
• Hypoplastic Left Heart Syndrome (HLHS)
• Pulmonary Atresia
• Tricuspid Atresia

Transposition of the great arteries (TGA) is a rare birth condition that affects how the heart is connected to the lungs and body.

Normally, the right side of the heart pumps blood into the lungs, where it receives oxygen. The left side of the heart then pumps this oxygen-rich blood to the rest of the body. The blood follows this important path thanks to two arteries or “great vessels,” the pulmonary artery and the aorta. The pulmonary artery is connected to the right side of the heart, and the aorta is connected to the left side.

But in TGA, the arteries’ connections are reversed: The pulmonary artery is connected to the left side of the heart, and the aorta is connected to the right side. This causes blood to flow through the heart and lungs in the wrong direction, resulting in something called “parallel circulation.” Instead of sending blood that’s low in oxygen to the lungs, the right side of the heart sends it directly out to the body. Meanwhile, the left side of the heart sends blood that already has enough oxygen back to the lungs.

As a result, the body never gets the oxygen-rich blood that it needs.

Patients with TGA may also have a hole in the heart known as a ventricular septal defect (VSD), or other heart problems. In extreme cases, patients may have a single ventricle anatomy, meaning that only one side of their heart is functioning properly.

What are the signs and symptoms of transposition of the great arteries?

TGA is often detected before birth, or in the first days of a baby’s life. Here are common signs of TGA in infants:

• Bluish or grayish lips, skin and nails
• Trouble breathing
• Trouble feeding
• Poor weight gain
• Low energy and activity

At Connecticut Children’s, we can diagnose many congenital heart problems in utero before babies are even born. We work with our award-winning neonatologists, fetal cardiologists, the mother’s labor and delivery doctors, and our other pediatric specialists to plan ahead for the birth and the important moments that follow.

What causes transposition of the great arteries?

TGA is a condition that occurs before birth, when the great vessels (the aorta and pulmonary artery) don’t attach in the correct position to the heart during pregnancy.

For some babies, TGA may be linked to genetic condition like DiGeorge syndrome. For others, there is no known cause.

How is transposition of the great arteries treated?

Usually, babies with TGA need surgery within a few days of being born.

NON-SURGICAL (temporary, to prepare for surgery)

• Before surgery, supplemental oxygen or a machine to help the baby breathe
• Before surgery, medication to help the baby’s heart and lungs work better
• Before surgery, balloon atrial septostomy: Uses a long, thin tube (catheter) to place a balloon in a natural hole in between the upper chambers of the heart. This enlarges the hole and allows blood from both sides of the heart to mix, which results in more oxygen-rich blood going to the body

SURGICAL

• Arterial switch operation: Surgery to connect the aorta and pulmonary artery to the correct sides of the heart

Like many patients with congenital heart defects, patients born with TGA have a unique anatomy. So it’s important that, throughout their life, they continue to get care from congenital heart experts, understand how their heart was reconstructed in surgery, and how it functions today.

Connecticut Children’s Heart Center provides this expertise at locations close to home, including access routine care like echocardiograms. We’re proud to stay with patients as they grow into adult care, too, with our nationally-accredited Adult Congenital Heart Disease program.

> Related: GROW Hopeful: Joseph’s Story

In this rare disorder, the tricuspid valve – which is supposed to connect the heart’s right chambers – does not form in a baby during pregnancy.

Normally, blood flows from the heart’s upper right chamber, through the tricuspid valve, and into the heart’s lower right chamber. Then it’s pumped to the lungs, where it picks up oxygen. But in babies with tricuspid atresia, the tricuspid valve hasn’t formed. Blood can’t flow through the right side of the heart the way it’s supposed to, and the body doesn’t get the oxygen-rich blood it needs.

Usually, children with tricuspid atresia are also born with an atrial septal defect (ASD) and a ventricular septal defect (VSD), holes in the wall between the left and right sides of the heart. Sometimes they also have other heart problems like transposition of the great arteries (TGA) or patent ductus arteriosus (PDA).

What are the signs and symptoms of tricuspid atresia?

Because this condition is so serious, it’s usually detected during pregnancy or within the first few days after a baby is born.

Here are signs that a newborn may have tricuspid atresia.

• Bluish or grayish lips, skin and nails
• Heart murmur
• Trouble breathing
• Trouble feeding
• Low energy and activity

At Connecticut Children’s, we can diagnose many congenital heart problems in utero before babies are even born. We work with our award-winning neonatologists, fetal cardiologists, the mother’s labor and delivery doctors, and our other pediatric specialists to plan ahead for the birth and the important moments that follow.

What causes tricuspid atresia?

Tricuspid atresia occurs before birth, when the fetal heart doesn’t develop properly during the first eight weeks of pregnancy. Doctors don’t know exactly why this happens.­­

While tricuspid atresia can occur just by chance, it’s a bit more common in babies with:

• Genetic disorders like Down syndrome
• Family history of congenital heart problems
• Mother infected with rubella (German measles) during pregnancy
• Mother with poorly controlled diabetes or lupus (an autoimmune disease)
• Mother who used certain anti-acne or anti-seizure medicines during pregnancy

How is tricuspid atresia treated?

Babies born with tricuspid atresia usually need “staged reconstruction,” a series of up to three surgeries to redirect how blood flows through the heart and lungs. These surgeries are spaced out over the first years of their life.

Connecticut Children’s cardiac surgeons specialize in these complex surgeries, with outstanding outcomes. Our cardiac catheterization experts play an important role before and between surgeries, using minimally invasive techniques to help the baby’s heart stay as healthy as possible.

SURGICAL

• Blalock-Taussig (BT) shunt: Closed-heart surgery to place a tube, or shunt, between the aorta and pulmonary artery, which allows more blood to flow to the lungs. Babies usually outgrow this within a few years
• Pulmonary artery band placement: If blood flow to the lungs is too high, places a band around the pulmonary artery to lessen blood flow
• Glenn procedure: Connects a large vein to the pulmonary artery, allowing blood to flow directly to the lungs
• Fontan procedure: Directs blood from the lower half of the body directly into the lungs to receive oxygen

NON-SURGICAL (Before and between surgery)

• Medication to open the ductus arteriosus, the blood vessel connecting the heart’s two major arteries, improving blood flow to the lungs
• Medication to strengthen the heart muscle, lower blood pressure, and get rid of extra fluid
• Supplemental oxygen or a machine to help the baby breathe
• Balloon atrial septostomy: Uses a long, thin tube (catheter) to make or enlarge a hole between the upper chambers of the heart. This allows blood from both sides of the heart to mix, which results in more oxygen-rich blood going to the body
• Cardiac catheterization: Uses a catheter to place a mesh insert in the ductus arteriosus to keep it open

Patients born with tricuspid atresia need lifelong care from congenital heart experts who understand their heart’s unique anatomy. Connecticut Children’s Heart Center is there every step of the way, with care that transitions into adulthood with our nationally-accredited Adult Congenital Heart Disease program.

This rare birth condition occurs when two of the heart’s most important arteries and their respective valves don’t form separately.

Normally, the aorta carries blood from the heart to the body, and the pulmonary artery carries blood to the lungs. But in truncus arteriosus, these arteries don’t form separately. A baby is born with just one large, common artery (“truncus artery”) to carry blood to both the body and lungs.

There are four different types of truncus arteriosus, depending on exactly how the arteries form. Most babies with truncus arteriosus are also born with a ventricular septal defect (VSD), a hole in the wall between the lower left and lower right chambers of the heart.

As a result of all of this, blood mixes in the heart in a way that it shouldn’t, and too much blood can flow to the lungs and not enough to the body. This damages the lungs and strains the heart.

What are the signs and symptoms of truncus arteriosus?

Truncus arteriosus is often detected before birth, or in the first few days of a baby’s life. The most common signs are similar to many other heart problems.

• Heart murmur
• Bluish or grayish lips, skin and nails
• Trouble breathing
• Trouble feeding
• Sweating, especially during feeding
• Poor weight gain
• Low energy and activity

At Connecticut Children’s, we can diagnose many congenital heart problems in utero before babies are even born. We work with our award-winning neonatologists, fetal cardiologists, the mother’s labor and delivery doctors, and our other pediatric specialists to plan ahead for the birth and the important moments that follow.

What causes truncus arteriosus?

Truncus arteriosus occurs before birth, when a baby’s heart doesn’t develop properly during the first eight weeks of pregnancy.

In some babies, it may be linked to one of these risks.

• Genetic condition like DiGeorge syndrome
• Family history of congenital heart problems
• Mother had an infection like rubella (German measles) during pregnancy
• Mother used certain harmful medications during pregnancy

But truncus arteriosus also occurs in babies without any of the above risk factors, without any known cause.

How is truncus arteriosus treated?

Most babies with truncus arteriosus have heart surgery within their first few weeks of life. Connecticut Children’s congenital cardiac surgeons specialize in this procedure, and are known for outstanding surgical outcomes.

SURGICAL

• Repair of truncus arteriosus: Open-heart surgery to separate and reconstruct the large artery into the correct arteries; place a right ventricle to pulmonary artery (RV-PA) conduit; and repair the VSD, valve problems, and any other heart problems

Patients born with truncus arteriosus need congenital heart care that continues into adulthood. Along with routine follow-up care and tests, they often need another surgery or catheter procedure to swap in a larger RV-PA conduit.

Connecticut Children’s Heart Center provides this lifelong care and expertise with our nationally-accredited Adult Congenital Heart Disease program.

An ventricular septal defect (VSD) is a hole between the lower chambers (ventricles) of the heart. Depending on its size and location, it can allow blood to mix between the ventricles, and cause too much blood to flow to the lungs. As a result, the heart and lungs have to work harder than they should, putting a strain on the body.

(A hole between the heart’s upper chambers is called an atrial septal defect, or ASD.)

VSDs are among the most common types of congenital heart defects. Some VSDs are minor and go away on their own. Others require treatment. Often, they occur as part of other heart problems – so it’s important to look at the entire heart, and understand or rule out other problems.

Families rely on Connecticut Children’s Heart Center for exactly that. We use advanced imaging technology like echocardiography and cardiac magnetic resonance imaging (MRI), and bring together specialists in cardiac catheterization, congenital cardiac surgery and more to determine the right plan of care.

What are the signs and symptoms of a VSD?

Small VSDs may only cause mild symptoms, or none at all. More serious VSDs may have the following signs, and are usually detected in a baby’s first few weeks of life.

• Heart murmur
• Heart beat that’s too fast, too slow or irregular
• Bluish or greyish skin, lips and nails
• Trouble breathing
• Poor appetite
• Poor growth
• Low energy

What causes VSDs?

VSDs develop before birth, when the inner wall (septum) dividing the left and right sides of the heart doesn’t form properly, leaving a hole. Sometimes, this condition is passed down from a parent. But most of the time, VSDs seem to occur by chance, with no clear cause.

How are VSDs treated?

Some smaller VSDs close on their own, or simply need a heart doctor to keep an eye on them. Others need a special procedure.

NON-SURGICAL

• Medication to help the heart work better and improve blood flow

SURGICAL

• VSD closure: Open-heart surgery to close the hole using a patch

Some patients with VSDs need to check in regularly with their doctor throughout life. Connecticut Children’s Heart Center provides expert care close to home, continuing in adulthood with our nationally-accredited Adult Congenital Heart Disease program.