Posts Tagged ‘congenital heart defects’

Emory and Children’s Healthcare of Atlanta Partner to Form Georgia’s First Comprehensive Congenital Heart Center

We are excited to announce the launch of the Congenital Heart Center of Georgia, 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.

Not too long ago, most babies born with serious heart defects died in childhood. Thanks to advances in cardiac care, many patients with congenital heart defects are able to live well into adulthood. However, as adults, they need ongoing, specialized care that a practice like the Congenital Heart Center of Georgia can provide.

Even if a person born with a congenital heart defect is not experiencing symptoms, he or she should continue to receive regular, ongoing medical management. Recent research shows that 40% of people between 13 and 21 with congenital heart defects discontinue care for their heart condition. The newly formed Congenital Heart Center of Georgia will help patients make a seamless transition from pediatric to adult care, as well as make sure they benefit from the latest medical research and receive the most appropriate treatments available.

For more information about the Congenital Heart Center of Georgia, please visit www.congenitalheartgeorgia.org.

Related Resources

Dr. Wendy BookAbout Wendy Book, MD
Dr. Book is a professor of medicine at Emory University School of Medicine and a cardiologist at Emory Healthcare. She specializes in cardiology, internal medicine and transplantation and is the director of the Emory Adult Congenital Heart (EACH) Center. Her areas of clinical interest include congenital heart disease, pregnancy in women with heart defects, heart failure and cardiac transplantation. Dr. Book has been practicing at Emory since 1998 and is highly regarded by her patients and colleagues.

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.

Managing Patients with Fontan Circulation

Dr. Francois Fontan first performed the Fontan procedure in 1971 on children with tricuspid atresia, or lack of a tricuspid valve. Fontan redirects venous blood from the right atrium to the pulmonary arteries, without pumping it from the lungs to the heart.

The Fontan Procedure is a palliative surgery; in other words, it doesn’t focus on curing the disease, rather, it reduces the symptoms and severity of it, resulting in an improved quality of life for the patient. It’s generally performed on children with complex congenital heart defects. Specifically, it’s used when a child only has one effective ventricle.

The Fontan is typically done as a two-stage repair. The first stage is referred to as a Bidirectional Glen procedure, or Hemi-Fontan. In this stage, oxygen-poor blood is redirected from the upper part of the body to the lungs. The pulmonary arteries are disconnected from their blood supply and the superior vena cava is removed from the heart and directed into the pulmonary arteries. The inferior vena cava transports blood from the lower body and remains connected to the heart. This redirection allows the single ventricle of the heart to do much less work. The second stage is known as Fontan completion, and it also redirects blood from the inferior vena cava to the lungs.

Although there are many different types of Fontan operations, they all serve a common purpose: to cause one effective ventricle to pump oxygen-rich blood to the aorta and into the body.

Patients who undergo Fontan surgery require life-long management to address any problems that may occur, such as heart rhythm issues, a weakened ventricle, or blockages and/or narrowing in the Fontan circulation. In many cases, children who undergo these procedures will require some form of Fontan revision surgery later in life.

It’s difficult to predict exact outcomes for Fontan children—in some cases patients will be able to participate in sports and vigorous activity; however, others may be severely limited in terms of exercise. Because this procedure is still relatively new, there are still many questions as to how well single ventricle heart/Fontan patients will fare as they enter their 30s and 40s, which is why ongoing monitoring is so crucial.

Please feel free to post any questions or comments you may have regarding this or other congenital heart defect procedures in the comments section.

About 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.

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.

Pulmonary Valve Replacement

Over the past few decades, we’ve made great strides in congenital heart surgery, which has increased survivorship among long-term sufferers of congenital heart disease. While many options for congenital heart disease and defect repairs are available, patients may require future additional surgeries.

Tetrology of Fallot is one of the most common types of congenital heart defects, and is a prime example of a condition that could require pulmonary valve replacement. Tetraology of Fallot (TOF) actually refers to four heart defects present from the time of birth: a ventricular septal defect (hole between the ventricular chambers which allows blue and red blood to mix), pulmonary stenosis (narrowing that makes it difficult for blue blood to reach the lungs), right ventricular hypertrophy (thickening of the heart muscle from pumping blood past the narrowing) and an overriding aorta. These defects often cause an infant to have cyanosis, or blue-tinged color of the skin, resulting from lack of oxygen-rich blood.

Fortunately, the prognosis for children with this condition has greatly improved over the last several decades (assuming proper diagnosis and treatment is administered). During surgical repair of TOF, the hole is closed with a patch, and the narrowing from the diseases pulmonary valve is removed, which can leave patients with a ‘leaky’ pulmonary valve. Although some patients may do well for a decade or more with the leaky valve, the pulmonary valve will eventually need to be replaced in most children born with TOF. Perhaps the most challenging aspect of treatment for patients suffering from this condition is exactly when to time treatment.

Other repairs may be associated with the need for pulmonary valve replacement in the future, including repair for pulmonary atresia with ventricular septal defect (VSD), truncus arteriosus, the Ross procedure for aortic valve disease, and double outlet right ventricle and d-transposition of the great arteries with VSD.

Surgical replacement of the pulmonary valve is the standard treatment for pulmonary valve disease.  Valve replacement surgery involves the replacement of one (or more) of the valves of the heart, typically with an artificial heart valve or a bioprosthesis (a prosthesis consisting of an animal part or animal tissue).

Pulmonary valve replacement is the most common operation performed in the adult congenital heart disease population. This surgery can be performed with extremely low morbidity and mortality. Patients are typically out of the hospital within the first week, and able to perform their normal daily routine. They’re fully recovered within 4 weeks. A surgically placed pulmonary valve is expected to last 10-15 years, or longer.

For very select patients who have already had conduit replacement of the pulmonary valve, transcatheter pulmonary valve replacement may be an option. While this option has a shorter recovery time, the longevity of the valve remains unknown. Early findings show that transcatheter PVR could be used as an alternative to traditional surgical methods in appropriate patients. The percutaneous PVR approach involves the placement of a bovine valve inside a balloon stent.

As physicians, we’re encouraged by the great strides we’re making from both surgical and technological standpoints with pulmonary valve replacement and congenital heart disease in general.

Do you have questions regarding any of these medical advances? If so, please be sure to let me know in the comments section.

About Brian E. Kogon, MD:

Dr. Kogon is an assistant professor of surgery and director of the congenital cardiac surgery fellowship at Emory. Additionally, he’s the surgical director of adult congenital cardiac surgery at Emory University Hospital. His clinical interests include pediatric cardiac surgery, cardiac transplantation, and adult congenital heart surgery. Dr. Kogon has been with Emory as a faculty member since 2004.

Congenital Heart Defects in Children: Is Surgery a Cure?

Approximately 8-10 children out of 100 are affected by congenital heart defects—one of the most common birth defects. Although many children diagnosed with this condition require surgery, some do not.

Generally, we treat congenital heart defects one of two ways: either with surgery or catheter procedures. Depending upon the complexity of the defect(s), some children may require several procedures over time. Exact treatment options depend on several factors, such as overall health, age, and size of the child.

Sometimes simple heart defects can be repaired percutaneously during a heart catheterization. With heart catheterization, we thread thin plastic tubes (catheters) through the blood vessels and into the heart. This type of procedure is less invasive than heart surgery, and allows for faster recovery time. However, most types of congenital heart defects require surgery.

Open-heart surgery can involve several treatments, including repairing or replacing heart valves, closing or patching holes in the heart with stitches, widening arteries and openings to the valves, and a number of other procedures. Fortunately, most defects in babies can be repaired, but the majority of defects cannot be cured. In the rare situation when a defect cannot be repaired, the baby or child may need to undergo a heart transplant.

At times, babies and/or children may need more than one procedure to repair a defect. Repairs of moderate to complex heart defects restore the circulation, allowing the body to receive the blood flow it needs, and the need for additional surgeries and/or medications is not uncommon.

With improvements in technology, operative technique, and medicine, the survival rates of children with congenital heart disease are steadily increasing. Interestingly, for the first time in history, the number of adults with congenital heart disease has reached the number of children with this condition. Adults with congenital heart defects previously repaired in childhood may require additional surgeries as adults. Further, adults diagnosed with a previously unoperated heart defect, and those requiring additional surgery for a previously operated heart defect require a team approach for surgical planning. In fact, these statistics have raised questions as to whether pediatric congenital surgeons with experience in adult congenital heart disease could achieve improved outcomes.

Through our research at Emory, we’ve found that there are improved outcomes with pediatric cardiac surgeons performing the surgeries on adults with congenital heart disease. This is definitely a subject worth further exploration and research.

Do you have questions about congenital heart defects in children or adults? If so, feel free to let me know your thoughts in the comments section.

About Wendy Book, MD:

Dr. Book is an associate professor of medicine and physician at Emory. She specializes in cardiology, internal medicine, and transplantation. Her areas of clinical interest include congenital heart disease, pregnancy in women with heart defects, heart failure and cardiac transplant. Dr. Book has been practicing at Emory since 1998.