Posts Tagged ‘congenital heart disease’

What Causes Congenital Heart Disease?

Congenital heartCongenital heart defects (CHDs) are the most common type of birth defect, affecting about 1% of infants born in the United States. While doctors can sometimes pinpoint the likely cause of a particular defect, most of the time the cause is uncertain.

Most CHDs are the isolated type, meaning that they occur alone without other birth defects. In most isolated CHDs, the cause cannot be determined and is generally assumed to be a combination of genetic (inherited) and environmental factors.

There are a number of genetic birth defects that often occur together with CHDs, including Down syndrome, Turner syndrome, Marfan syndrome and Williams syndrome. In these cases, a defect in the infant’s DNA causes the heart to develop improperly. For instance, about half of babies born with Down syndrome also have a CHD, most often a defect in the wall between the left and right sides of the heart (atrioventricular septal defect).

A mother’s exposure to certain substances during pregnancy can increase the risk for CHDs. Some medications increase risk, including certain acne and seizure medications. Environmental exposures can be more difficult to pinpoint but may contribute as well. A mother ingesting too much alcohol during pregnancy can also increase the risk of her infant being born with a heart defect.

In addition to environmental exposures, some health issues in pregnant women can play a role in increasing the risk for CHDs. These include infections such as rubella, as well as chronic conditions that are not under control, such as diabetes and lupus.

The Congenital Heart Center of Georgia was created to bridge the gap between pediatric and adult care for people with CHDs. If you were born with a CHD and haven’t been evaluated regularly by a cardiologist, you were recently diagnosed with a CHD or you have a child who will be transitioning into adult care in the near future, learn more about the Congenital Heart Center of Georgia and make an appointment today.

About Dr. Rodriguez

Fred Rodriguez, MDFred Rodriguez, MD, is a pediatric cardiologist who practices pediatric cardiology at the Children’s Healthcare of Atlanta Sibley Heart Center and adult congenital heart disease at the Emory Clinic and Emory University Hospital. Dr. Rodriguez earned his medical degree from the Louisiana State University at New Orleans School of Medicine, where he also completed his combined residency in both internal medicine and pediatrics. Following his residency, he completed a cardiology fellowship at Texas Children’s Hospital in Houston, with additional training in adult congenital heart disease. He is board certified in pediatrics, pediatric cardiology and internal medicine.

About the Congenital Heart Center of Georgia

The Congenital Heart Center of Georgia is a collaboration between Children’s Healthcare of Atlanta and Emory Healthcare. The Congenital Heart Center of Georgia is a comprehensive program for children and adults with congenital heart disease (CHD) that provides a continuum of lifesaving care from before birth through adulthood. It is the first comprehensive CHD program in the South and one of the largest in the country. The program is led by Emory Healthcare cardiologist Wendy Book, MD, along with Robert Campbell, MD, chief of cardiac services and director of cardiology at Children’s Sibley Heart Center. To schedule an appointment, please call 404-778-7777.

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Congenital Heart Defect Repair in Childhood: Will I Need Another Surgery?

congenital heart repairNot too long ago, most babies born with serious heart defects died in childhood. Thanks to advances in cardiac care, some estimates indicate that today as many as 90% of children with congenital heart disease (CHD) are able to live well into adulthood. In fact, there are now more adults than children living with CHD, and it has become increasingly clear that this growing population requires ongoing, specialized care. For instance, even if their defects are treated surgically in childhood, many patients will require additional surgery as adults to keep their hearts functioning correctly.

When many surgical procedures were first performed to correct congenital heart defects in children, the medical community generally assumed they were curative. But as the first generation of post-operative patients survived into adulthood, some began to develop late complications associated with the procedures they underwent as children.

Unfortunately, many of these late complications develop gradually and are associated with non-specific symptoms. In addition, CHD is so closely associated with infancy and childhood, that many patients assume they no longer need to worry about their condition once they have reached adulthood. Consequently, they may not make the connection between the symptoms they develop as adults and their CHD—especially if it was successfully corrected in childhood.

This relatively recent phenomenon bolsters the argument that patients with CHD—even if their defect was surgically corrected in childhood—need to continue regular follow-up with a congenital heart specialist into adulthood so that he or she can monitor for subtle changes that may indicate a serious problem.

Another issue with managing CHD in adulthood is that adult cardiologists may have difficulty treating conditions in hearts repaired—often effectively re-configured—by pediatric surgeons years earlier. Conversely, pediatric surgeons may be unfamiliar with the unique complications that can arise years later as “corrected” anatomy ages, and in general may not have the specific training and experience required to address congenital disease in adults.

In response to this growing crisis, Emory and Children’s Healthcare of Atlanta have teamed up to help ensure that patients with CHD don’t get lost to follow-up as they transition into adulthood. The Congenital Heart Center of Georgia combines the expertise of Children’s Sibley Heart Center with that of Emory’s Adult Congenital Heart Center to address this crucial need. It is the first program of its kind in the South and one of the largest in the country.

About Dr. Kogon

Brian E. Kogon, MD , is chief of Pediatric Cardiothoracic Surgery at Children’s Sibley Heart Center and Emory University Hospital , surgical director of the Adult Congenital Heart Disease Program at Emory University Hospital and director of the Congenital Cardiac Surgery Fellowship at the Emory University School of Medicine.

Dr. Kogon received his medical degree from the University of Cincinnati and completed his residency in general surgery and a fellowship in cardiothoracic surgery at Indiana University. He then went on to complete his fellowship in pediatric cardiac surgery at Emory University, joining the staff in 2004.

Dr. Kogon is now a nationally recognized leader in pediatric and adult congenital heart disease. He has numerous publications in peer-reviewed journals and presents nationally at the major cardiothoracic surgery society meetings. He has earned various awards over the years, most recently the Teacher of the Year award for Pediatric Cardiac Surgery from the Sibley Cardiology Fellowship Program and Emory University.

Dr. Kogon’s major areas of interest include pediatric cardiac surgery, cardiac transplantation and adult congenital heart surgery.

About the Congenital Heart Center of Georgia

The Congenital Heart Center of Georgia is a collaboration between Children’s Healthcare of Atlanta and Emory Healthcare. The Congenital Heart Center of Georgia is a comprehensive program for children and adults with congenital heart disease (CHD) that provides a continuum of lifesaving care from before birth through adulthood. The program is led by Emory Healthcare cardiologist Wendy Book, MD, Robert Campbell, MD, chief of cardiac services and director of cardiology at Children’s Sibley Heart Center, and Brian Kogon, MD, chief of Pediatric Cardiothoracic Surgery at Children’s Sibley Heart Center and Emory University Hospital and surgical director of the Adult Congenital Heart Disease Program at Emory University Hospital. To schedule an appointment please call 404-778-7777.

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Signs and Symptoms of Congenital Heart Defects

CDH BabyBecause congenital defects can decrease the heart’s ability to pump blood and deliver oxygen throughout the body, they often produce telltale signs. Below are some of the more common symptoms that indicate a baby may have congenital heart disease (CHD).

  • Heart Murmur

A heart murmur is often the first sign of CHD. In basic terms, a murmur is just an extra heart sound, in addition to the regular sounds of a beating heart. Heart murmurs usually don’t indicate the presence of any heart problem. Sometimes a doctor can use a stethoscope alone to determine whether a particular murmur is a sign of heart disease. In other cases additional tests are necessary to determine the exact nature of a murmur.

  • Breathing Difficulties

Breathing difficulty caused by blood building up in the lungs (lung congestion) is a sign of a serious defect that will likely need medical or surgical intervention in the first year of life. Lung congestion may be the result of excessive blood flow from the left side of the heart to the right side through an abnormal connection, such as a hole in the heart or a connection between major blood vessels that allows blood to bypass the heart. Congestion can also be the result of an obstruction in blood flow on the left side of the heart that causes blood to back up in the vessels returning blood from the lungs.

  • Blue Skin

Some CHDs result in an inadequate amount of oxygen in the blood, which can cause the baby’s skin to have a bluish tint, especially in the lips, tongue, fingernails and toenails—called cyanosis. Cyanosis can result from an obstruction of blood flow to the lungs or a hole within the heart that allows oxygen-poor blood to flow from the right side to the left side and out to the body. It can also be related to other heart issues, including an abnormal positioning (transposition) of the arteries leaving the heart.

  • Failure to Thrive

Another result of inadequate oxygen in the blood is that an infant may lose weight or not gain enough, or may take longer to reach developmental milestones. These symptoms can result directly from the body not receiving enough oxygen to thrive, or they may be an indirect consequence of the infant tiring during feeding because of a lack of oxygen and, as a result, not receiving enough nutrients.

  • Excessive Sweating

Many CHDs can cause excess blood flow through the lungs, which makes breathing more difficult. The increase in exertion required to breathe can, in turn, result in excess sweating. Because feeding is a common form of activity in babies, this excess sweating is often closely associated with feeding, though any activity that causes an increase in the infant’s breathing rate can also cause increased sweat production. Excess blood flow to the lungs can also accelerate the infant’s metabolism, a side effect of which is increased sweating.

If you notice any of these signs in your baby or child, call your doctor right away. If your doctor notices these signs, you may be referred to a pediatric cardiologist.

About Dr. Rodriguez

Fred Rodriguez, MDFred Rodriguez, MD, is a pediatric cardiologist who practices pediatric cardiology at the Children’s Healthcare of Atlanta Sibley Heart Center and adult congenital heart disease at the Emory Clinic and Emory University Hospital. Dr. Rodriguez earned his medical degree from the Louisiana State University at New Orleans School of Medicine, where he also completed his combined residency in both internal medicine and pediatrics. Following his residency, he completed a cardiology fellowship at Texas Children’s Hospital in Houston, with additional training in adult congenital heart disease. He is board certified in pediatrics, pediatric cardiology and internal medicine.

About the Congenital Heart Center of Georgia

The Congenital Heart Center of Georgia is a collaboration between Children’s Healthcare of Atlanta and Emory Healthcare. The Congenital Heart Center of Georgia is a comprehensive program for children and adults with congenital heart disease that provides a continuum of lifesaving care from before birth through adulthood. It is the first comprehensive congenital heart disease program in the South and one of the largest in the country. The program is led by Emory Healthcare cardiologist Wendy Book, MD, Robert Campbell, MD, chief of cardiac services and director of cardiology at Children’s Sibley Heart Center, and Brian Kogon, MD, chief of pediatric cardiothoracic surgery. To schedule an appointment, please call 404-778-7777.

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Congenital Heart Defects in Newborns

newbornCongenital heart defects (CHDs) are abnormalities present at birth that can affect the structure and function of the heart. Approximately 1% of infants born in the United States have CHDs. A baby’s heart begins to develop at conception, but is completely formed by eight weeks into the pregnancy. CHDs occur during this crucial first eight weeks of the baby’s development. Specific steps must take place in order for the heart to form correctly. Often, CHDs are a result of one of these crucial steps not happening at the right time, leaving a hole where a dividing wall should have formed or a single blood vessel where two ought to be, for example.

Some CHDs are known to be associated with genetic disorders, such as Down syndrome, but the cause of most CHDs is unknown. In these cases, doctors generally assume the cause is some mixture of environmental and inherited (genetic) factors.

Common types of congenital heart defects, which can affect any part of the heart or its surrounding structures, include:

While CHDs sometimes go undiagnosed for years — even into adulthood — others cause serious symptoms at birth, requiring the infant to be placed in the hospital’s neonatal intensive care unit (NICU) for immediate evaluation by a cardiologist.

Today there are more treatment options for CHDs than ever before, and most defects are treated successfully. If you suspect that your child has a heart defect, the sooner you get medical attention, the better chance your child will have of making the fullest recovery possible.

About Dr. Campbell

Robert Campbell, MD, is chief of cardiac services and director of cardiology at Children’s Sibley Heart Center. Dr. Campbell earned his medical degree from Emory University, where he also completed a residency in pediatrics. He completed a pediatric cardiology fellowship at the University of Michigan C.S. Mott Children’s Hospital.

About the Congenital Heart Center of Georgia

The Congenital Heart Center of Georgia is a collaboration between Children’s Healthcare of Atlanta and Emory Healthcare. The Congenital Heart Center of Georgia is a comprehensive program for children and adults with congenital heart disease that provides a continuum of lifesaving care from before birth through adulthood. It is the first comprehensive congenital heart disease program in the South and one of the largest in the country. The program is led by Emory Healthcare cardiologist Wendy Book, MD, Robert Campbell, MD, chief of cardiac services and director of cardiology at Children’s Sibley Heart Center, and Brian Kogon, MD, chief of pediatric cardiothoracic surgery. To schedule an appointment, please call 404-778-7777.

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What Is Congenital Heart Disease?

Congenital Heart GraphicCongenital heart disease, or CHD, is a broad term that covers a range of conditions present at the time of birth that can affect the structure and function of the heart. CHD is the most common type of birth defect, but thanks to a number of advances in medical and surgical treatment, more and more children with CHD are surviving into adulthood. In fact, according to the Adult Congenital Heart Association (ACHA), there are about one million adults living with CHDs in the U.S.

Some of the most common conditions that cause congenital heart disease include:

As children with CHDs grow into adults, they need ongoing specialty cardiac care. Yet, this high-risk group often experiences lapses in cardiac care due to the perception that they are “fixed” or because they aren’t experiencing symptoms. Moreover, CHDs are so closely associated with infancy and childhood that people often think the conditions just don’t affect adults.

The Congenital Heart Center of Georgia was created to bridge the gap between pediatric and adult care for people with CHDs. If you were born with a CHD and haven’t been evaluated regularly by a cardiologist, you were recently diagnosed with a CHD or you have a child who will be transitioning into adult care in the near future, learn more about the Congenital Heart Center of Georgia and make an appointment today.

About Dr. Book

Wendy Book, MDWendy Book, MD , is the director of the Emory Adult Congenital Heart Center. She has 15 years of experience in adult congenital heart disease, including clinical and research experience. She has a background in heart failure, transplantation and pulmonary hypertension, which complement skills of other Emory Adult Congenital Heart Center physicians. She is board certified in internal medicine, cardiovascular disease, advanced heart failure and transplant cardiology.

About the Congenital Heart Center of Georgia

The Congenital Heart Center of Georgia is a collaboration between Children’s Healthcare of Atlanta and Emory Healthcare. The Congenital Heart Center of Georgia is a comprehensive program for children and adults with congenital heart disease (CHD) that provides a continuum of lifesaving care from before birth through adulthood. It is the first comprehensive CHD program in the South and one of the largest in the country. The program is led by Emory Healthcare cardiologist Wendy Book, MD, along with Robert Campbell, MD, chief of cardiac services and director of cardiology at Children’s Sibley Heart Center. To schedule an appointment please call 404-778-7777.

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Treating Congenital Heart Disease in Adults

Congenital Heart DiseaseDid you know that congenital heart defects affect approximately 40,000 babies each year? And now, due to advances in medicine, many of these patients are living to adulthood and there are estimated to be more than 1 million adults in the United States with congenital heart defects, according to the Centers for Disease Control (CDC).

We strongly recommend that all adults born with a congenital heart defect should have routine follow – up care with a congenital heart specialist in order to ensure the heart is healthy. Conditions can develop later in life that a patient could benefit from additional treatment later. Luckily, not all congenital heart patients have to have surgical or medical treatments – some patients may just need routine monitoring.

Congenital Heart Disease Treatments

For those congenital heart patients who need more advanced treatments your physician will work with you to determine if your condition warrants medical treatment only or if you need more advanced surgical treatment.

Medical Management of Congenital Heart Disease

If a patient has an of the following conditions, preventive care and medical management of the congenital heart disease may be sufficient to keep the heart healthy:

If the congenital heart defect is more serious, surgical or interventional treatment may be required such as:

  • Heart Transplant
  • Pulmonary Valve Replacement
  • Valve Repair and Replacement
  • Septal Defects
  • Congenital Structural Heart Interventions

If you were born with a congenital heart defect it is important to find a physician who has specialized training in congenital heart disease. These physicians have specialized training and experience to deal with the complexities of congenital heart disease. Emory recently partnered with Children’s Healthcare of Atlanta to form the Congenital Heart Center of Georgia that offers congenital heart care from birth until late life. Physicians at the Congenital Heart Center of Georgia have years of dedicated experience on patients with CHDs and will work with the team of physicians across CHOA and Emory to develop a treatment plan that best meets your needs.

About Dr. Sahu

Anurag Sahu, MDAnurag Sahu, MD is an Assistant Professor of Medicine at Emory University School of Medicine and is the Director of the Cardiac Intensive Care Unit at Emory University Hospital. He also specializes in cardiac MRI and cardiac CT imaging with specific training imaging of adults with congenital heart disease. He is Board Certified in Internal Medicine, Cardiovascular Medicine, Nuclear Medicine, Cardiovascular CT and Echocardiography. He has 4 years of adult congenital heart clinical and research experience.

About the Congenital Heart Center of Georgia

The Congenital Heart Center of Georgia, a collaboration between Children’s Healthcare of Atlanta and Emory Healthcare, is one of the largest programs in the U.S.—and the only one in Georgia—specializing in the treatment of children and adults with congenital heart disease (CHD). The team, led by Wendy Book, M.D., Robert Campbell, M.D., and Brian Kogon, M.D., provides individuals with congenital heart disease appropriate lifelong care from before birth through adulthood. To schedule an appointment please call 404-778-7777. Find more information about this unique partnership by visiting congenitalheartgeorgia.org.

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Emory Adult Congenital Heart Center Treatments

What are the Most Common Types of Congenital Heart Defects?

Types of Heart DefectsCongenital Heart Defects (CHDs) occur in approximately 8 out of 1,000 newborns and are the most common kind of birth defect, according to the National Institutes of Health. This equals more than 35,000 babies each year in the U.S.

Congenital heart defects occur when the heart does not develop normally before the baby is born. Some congenital heart defects are simple and do not need treatment or can be easily fixed. Others are more complex, but due to improvements in diagnosis and treatment, most children who have complex congenital heart defects survive into adulthood and are able to lead active lives.

Many adults with ACHD need continued special heart care throughout their lives, and also need to learn how ACHD can affect employment, pregnancy and family planning.

According to the American Heart Association, there are 18 major types of congenital heart defects. The most common types are:

Aortic Valve Stenosis (AVS):

  • AVS occurs when a valve from the heart to the body does not properly open and close. This causes the left ventricle to pump harder (stenosis), or can leak blood backwards (regurgitation).
  • This increased pressure in the left ventricle may cause the heart muscle to thicken, get larger and be damaged.
  • If severe, it can cause chest pain, weakness, dizziness and fainting.

Aortic Valve Stenosis can be treated to reduce the pressure, or surgery may be needed. If the aortic valve has become too weak, then it may need to be replaced.

Atrial Septal Defect (ASD):

  • This is similar to VSD, but is an opening between the upper chambers of the heart
  • If it is small, it may not cause any problems and may not need treatment.
  • If it is larger, it may cause a murmur, or can cause permanent damage to the blood vessels in the lungs.
  • It can be fixed via open-heart surgery or cardiac catheterization (using a device to plug the hole)

Coarctation of the Aorta (CoA)

  • CoA is caused by the narrowing of the aorta (a major artery) and can reduce blood flow to the upper and lower parts of the body.
  • This causes increased blood pressure in the left ventricle and an overworked heart that is trying to pump past the blockage. This causes a thickening of the muscles and long-term damage.
  • A serious obstruction may be treated with a balloon and catheter, or by surgery to repair the narrowing.

Complete Atrioventricular Canal Defect (CAVC):

  • CAVC is when a large hole in the center of the heart affects all the four chambers. The blood mixes together and is not properly directed to each stage of circulation.
  • It is common among children with Down Syndrome.
  • Extra blood is pumped into the lung arteries and causes both the heart and lungs to work harder, causing the lung to be congested.
  • This can cause children to breathe faster and harder than normal. They can have issues feeding and growing at a normal rate. Permanent damage can occur to the lung vessels.
  • Open-heart surgery is needed to fix the defect since it cannot close on its own. Additional surgeries or medication may be needed.

Patent Ductus Arteriosis (PDA)

  • This describes an unclosed hole in the aorta
  • The ductus arteriosus is an artery found in the fetus that moves the blood away from the lungs. It is no longer needed after birth, and usually closes up in the first few days.
  • If it is small, it will not cause any symptoms or cause the heart and lungs to work harder. It may close up in the first few months.
  • If the PDA is large, it can cause the infant to breathe faster than normal and can prevent them from feeding or growing normally. It can also cause permanent damage to the lung’s blood vessels. This may require a plug to be inserted, surgery, or medication to help it close up.

Pulmonary Valve Stenosis

  • This is when a thick or fused heart valve doesn’t open fully. This increases the pressure in the right pumping chamber (ventricle) and can cause damage due to the overworked heart muscle in that area.
  • If it is serious, it can cause cyanosis (blueness). Otherwise there may be no symptoms.
  • It can be treated to relieve pressure in the right ventricle, but the valve cannot be made normal.

Tetralogy of Fallot (fa-loh)

  • This defect includes four problems:
    • A hole between the lower chambers of the heart
    • An obstruction between the heart and lungs
    • Overly thickened muscle around the lower right chamber of the heart
    • The aorta rests over the hole in the lower chambers
  • This is commonly found in children with Down Syndrome or DiGeorge Syndrome.
  • Blood travels across the hole from the bottom right chamber to the left (VSD) and out into the artery that is for the body. The block in the pulmonary valve can cause less blood to be pumped to the lungs.
  • Children who do not have a repaired tetralogy of Fallot are blue.
  • This is treated surgically, usually with a temporary operation coming first, before complete repair.

Transposition of Great Arteries

  • This can occur when either the arteries carrying blood away from the body are reversed (d-transposition) or when the bottom chambers are reversed along with the arteries (I-transposition).]
  • The d-transposition is more serious because the arteries are connected to the wrong chambers of the heart. This requires surgery early in life that enlarges a connection between the right and left upper chambers so that some oxygen-rich and oxygen-poor blood can go to the correct side of the heart.
  • Depending on the surgical procedure, more surgery may be needed in the future to help the heart pump better, repair valves or correct heart rhythms.
  • The I-transposition may cause long-term damage to the right ventricle, though surgery may not be needed.

Ventricular Septal Defect (VSD)

  • VSDs are one of the most common types of congenital heart defects
  • VSD is a hole in the wall separating the two lower chambers of the heart
  • Usually, the wall closes up before the fetus is born, but if it doesn’t, it can cause higher pressure inside the heart or reduced oxygen to the body
  • If it is a small hole, it won’t cause symptoms apart from a loud murmur through a stethoscope. It may even close on its own.
  • If it is a large hole, open-heart surgery may be needed to close it so that severe symptoms and permanent damage do not occur.

Though most of these conditions may not affect the types of activities you or your child may be able to do, it is important to schedule regular check ups with your physician. The doctor may then suggest which exercises and activities should be avoided.

If you are one of the more than one million adults with a CHD and are not in the care of a physician trained specifically to deal with congenital heart disease, we highly recommend you schedule a visit with one of the Emory Adult Congenital heart specialists for follow up care.

Related Resources:

 

Dr. Michael McConnellAbout Michael McConnell, M.D.
Dr. McConnell specializes in Pediatric Cardiology and general Pediatrics. His area of clinical interest is in cyanotic congenital heart disease management, syncope, and single ventricle patient management. Dr. McConnell completed his residency at the Children’s Hospital of Alabama, and his fellowship was held at the Children’s Hospital Medical Center in Cincinnati, OH. He’s been practicing at Emory since 2000.

About the Congenital Heart Center of Georgia
The Congenital Heart Center of Georgia is collaboration between Children’s Healthcare of Atlanta and Emory Healthcare. The Congenital Heart Center of Georgia is a comprehensive program for children and adults with congenital heart disease (CHD) that provides a continuum of lifesaving care from before birth through adulthood. It is the first comprehensive CHD program in the South and one of the largest in the country. The program is led by Emory Healthcare cardiologist Wendy Book, MD, along with Robert Campbell, MD, chief of cardiac services and director of cardiology at Children’s Sibley Heart Center.

Welcome Aboard : Dr. Woodhouse and Dr. Shonkoff

Emory Heart & Vascular Center is excited to welcome Dr. Sheila Woodhouse and Dr. David Shonkoff to the Emory team! Doctors, Woodhouse and Shonkoff will practice at 5 locations around Gwinnett County in Duluth (2 locations), Johns Creek, Snellville and Lawrenceville.*

The Emory Heart & Vascular Center – Gwinnett offers a comprehensive spectrum of in-office cardiac and vascular diagnostic testing and treatments. Some of the services the practice will provide cardiology patients are echocardiography, stress echocardiography, nuclear stress testing, treadmill stress testing, carotid duplex ultrasound imaging, ankle-brachial index (ABI) testing and holter and event monitoring.

Dr. Woodhouse specializes in women with heart disease, valve disease and arrhythmias, congestive heart failure, atherosclerotic heart disease and preventive cardiology, risk factor modification management and cardiac related high risk pregnancies and post pardum cardiac care. Impressively, she is triple boarded in cardiovascular imaging modalities and has particular interest in cardiac and vascular imaging.

Dr. Shonkoff specializes in congestive heart failure, heart disease prevention, vavular heart disease, congenital heart disease, refractory hypertension, and cardiac imaging.

Locations
Emory Heart & Vascular Center – Duluth
1845 Satellite Boulevard, Suite 500
Duluth, Georgia 30097

Emory Heart & Vascular Center – Johns Creek
6335 Hospital Parkway, Suite 110
Johns Creek, Georgia, 30097

Emory Heart & Vascular Center – Eastside
1608 Tree Lane, Suite 101
Snellville, Georgia 30078

Saint Joseph’s Medical Group
4855 RiverGreen Parkway
Duluth, GA 30096

Emory Heart & Vascular Center – Lawrenceville
771 Old Norcross Road
Suite 105
Lawrenceville, GA 30046

For hours of operation and to schedule an appointment please call 404-778-6670 or 404-778-6590.

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Managing Patients with Cyanotic Heart Disease

Some congenital heart defects cause cyanosis, or low oxygen levels in the blood, which can give children a bluish appearance. In many cases, the cyanotic heart defect is repaired in childhood, and oxygen levels return to normal. Sometimes, a complete repair isn’t possible and the cyanosis is present for life.

Cyanotic Heart Disease is a heart defect, or group of heart defects that are present at birth. Under normal circumstances, an infant’s blood contains ample oxygen that flows throughout the body. When cyanosis is present, blood flows abnormally (called right-to-left shunt), resulting in too little oxygen in the blood flow and causing the child’s skin to take on a bluish appearance. This bluish discoloration is most often seen on the fingers, lips, and toes. Several types of congenital heart disease may cause cyanosis, including:

- Pulmonary Valve Atresia

- Tetralogy of Fallot

- Tricuspid Atresia

- Hypoplatic Left Heart Syndrome

- Truncus Arteriosus

- Total Anomalous Pulmonary Venous Return

Treatment and management of Cyanotic Heart Disease depends largely on the specific type of congenital heart defect that is present. After birth, infants may receive medication to assist the heart in pumping, keep the blood vessels open, and treat any heartbeat abnormalities. Typically, one or more surgeries are performed during childhood to redirect the blood flow and alleviate the cyanosis. If these surgeries are successful, the child is no longer cyanotic.

However, some older adults were born in a time period when these cyanotic heart defects could not be repaired. They may have had a shunt placed—communication between the aorta or its branches and the pulmonary artery to increase blood flow to the lungs—or they may have had no surgery at all. Some older adults were born with a hole in their heart (this is known as a ventricular septal defect) but were born before surgery was available to close the hole. Generally, if the hole was large enough, they didn’t have cyanosis as a young child, but would go on to develop it later in their childhood as the pulmonary pressures increased, causing a reversal of the shunt. This is known as Eisenmenger syndrome. Adults with Eisenmenger syndrome may benefit from medical therapy with pulmonary vasodilators, and should discuss these options with an adult congenital heart specialist.

Adults who have cyanotic heart disease require special care. Phlebotomy, or removal of blood should be avoided in general, as this leads to iron deficiency and worsening symptoms. High blood counts are the body’s way of compensating for the low oxygen levels.

Individuals with cyanotic heart must exercise caution when traveling to high altitudes, and most should avoid pregnancy. If a patient is hospitalized, an air filter should be placed in the IV lines to prevent small bubbles from reaching the brain—otherwise, the mixing of venous blood and arterial blood can occur.

All patients born with cyanotic heart disease have moderate to complex heart defects. These patients benefit from a multi-disciplinary approach at a Center specializing in the care of adult with congenital heart defects

Regardless of the chosen course of treatment, we believe that when managing this disease, it’s important to adopt a team approach in which primary care physicians, surgeons, cardiologists, nurses, OB/GYNs, hematologists, and anesthesiologists all communicate and work in conjunction to achieve the best results for the patient.

Do you have questions or comments regarding Cyanotic Heart Disease? If so, be sure to let me know your thoughts in the comments section.

Pulmonary Valve Replacement: Andrew Sawyer’s Story

My name is Andrew Sawyer, and I’m 25 years old. Believe it or not, before I was 2 years old, I had 4 open-heart surgeries. I spent the first day of my life being transported by helicopter from Douglas, GA to Children’s Healthcare of Atlanta at Egleston for emergency surgery. It was there that Dr. Willis Williams performed 3 open-heart surgeries on me as he attempted to fit the right sized shunt into my heart. I was born without a pulmonary valve (a condition called pulmonary atresia), which prevented the normal flow of blood from my heart to my lungs from being replenished with oxygen.

I spent my first birthday in the hospital, where I underwent my 4th surgery. The surgeons had to place a patch over the area of my missing valve, allowing blood to flow through.

Surgery number five took place when I was in the sixth grade. The existing patch was beginning to fail, so this procedure involved the placement of a pulmonary cadaver valve, restructured my tricuspid valve, and repaired the lining of my right ventricle. Memory is a funny thing—I’ll never forget waking up from the surgery and learning that the Braves were losing to the Yankees in the World Series.

The surgeons predicted that my new pulmonary valve would last for 8-10 years, but remarkably, it lasted for 13. Once a year, I’d go in for my yearly check-up appointment and wonder if this would be the visit that the doctors told me that it was time for another surgery. Every year I heard the welcome words, “see you next year”—until the fall of October of 2009.

That fall, Dr. Book learned through my echocardiogram results that my pulmonary valve was damaged, and it was time for another surgery. I was amazed that the doctors were able to discover this through a simple echo, but technology had advanced since my last surgery, and a catheterization process was unnecessary this time around. Dr. Book and Dr. McConnell recommended that I see Dr. Kogon, a cardiothoracic surgeon specializing in adults with congenital heart defects.

From the start, I knew that I was in great hands with Dr. Kogon—he immediately made me feel at ease, and he was very clear in how he presented my options. I’m not sure I can accurately describe how surreal it was to have a conversation with Dr. Kogon about whether to go with a pig or a cow valve for my surgery. According to Dr. Kogon, there had been great advancements with animal tissue valves. He explained that this would be a better option than a human or mechanical valve—animal valves, for whatever reason, seem to last longer and yield better results. Dr. Kogon estimated that my new bovine valve would last 20-30 years.

Many people ask me if I was discouraged, or scared in reaction to the news of another surgery, but I can honestly say that I wasn’t. I’ve always had an extremely positive attitude throughout my life—this, coupled with my religious conviction carries me through tough times. Strange as it may sound, I compared the pain of my recovery period to one particularly tough summer job I had as a door-to-door salesman. That was one of the harshest, most emotionally taxing periods of my life, and it changed me somewhat. Being told “no” time and time again, and having to get up and hit the road again the next day requires strength and resilience. I realize I’m talking about two completely different types of pain here—but when I was lying in bed in pain post-surgery, that’s exactly what I thought about. I figured, “if I made it through that gut-wrenching summer of door-to-door sales, I can make it through this.”

When I think back to my 6th and most recent surgery, a few things come to mind: first, I couldn’t believe the level of service I experienced at Emory. The nurse technicians were incredibly kind and knowledgeable. I always had baths and a clean bed, and the overall level of care was just phenomenal. Even months after the operation, Dr. Kogon would stop by to visit me during my check-ups with Dr. Book and Dr. McConnell. Knowing I was in such good and capable hands was a comfort in itself.

My recovery experience as a 25-year-old was much different from my experience as a 12-year-old. The doctors explained the difference to me, saying that a 12-year-old body is made up of quite a bit of cartilage, as opposed to a 25-year-old, whose body is made up primarily of bone, causing recovery to be more painful. Even so, I was only in the hospital for 6 days, and I was able to get back to school (medication-free) within 30 days of the surgery.

Aside from being a student, I’m a musician, and over the years, my experiences have inspired me to write several songs, one of which is called “South Georgia Pine”—this video shows footage of me leaving Emory a few months after my last surgery.

I’m incredibly grateful to my family, and to all of the Emory doctors and nurse technicians who have supported me and helped me along on this journey to recovery.