Posts Tagged ‘congenital heart disease’

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.

Related Resources

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.

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.

Balancing Life with Congenital Heart Disease

Until 1960, many people born with moderate and complex forms of Congenital Heart Disease (CHD) didn’t reach adulthood. As people who suffer from CHD age, they may develop health problems that are unique and require special treatment. Consequently, it’s vital that individuals with CHD strive for a healthy balance in their lives. Adults with moderate or complex CHD benefit from regular evaluation at an Adult Congenital Heart Center, especially if they have been out of cardiac care for many years.

If you have heart disease, realize that above all, you are a success and a survivor. Still, living with chronic illness can take a serious toll on you, emotionally and physically. It’s important that you stay as educated and informed about your heart disease as possible.

Regular check-ups with your ACHD doctor may help recognize any issues or problems early before symptoms develop.  Be sure to accept help when you need it, and share you healthcare plans with your friends and family. They, along with your doctors and nurses, can help you to continue your success.

Bear in mind that there are ways for you to stay healthy and live better. Your risks of developing problems with both the heart and other organs may be higher when you have CHD, so it’s crucial for you to maintain a healthy weight, follow a heart-healthy diet plan, stay as active as you can, and have your blood pressure and any needed blood work checked regularly. Your ACHD doctor can devise a plan specific to your needs.

Here are a few more tips that will help to keep you and your heart healthier:

- Get a flu vaccine each year if your doctor advises

- Consider a pneumonia vaccine if your doctor advises

- Take good care of your teeth, take antibiotics before dental visits if your doctor advises you to;

- Discuss any non-cardiac surgery (unless it’s an emergency) with your doctor or cardiologist prior to scheduling it

- Ask your doctor about over-the-counter drugs and herbal supplements before taking any

- Avoid smoking and smokeless tobacco

- Some young adults with CHD need to limit alcohol and caffeine in their diets; check with your doctor to see how alcohol and caffeine may affect you

- Avoid all illicit drugs

Additionally, I highly recommend The Therapeutic Lifestyle Changes (TLC) diet plan—a low saturated fat, low cholesterol diet that helps to reduce your total blood cholesterol as well as your LDL cholesterol levels. The lower your cholesterol levels, the lower your risk is for coronary heart disease. The TLC plan provides fats, cholesterol, carbohydrates, fiber, and protein throughout your daily calorie intake. If your doctor has recommended a low sodium diet, you should follow those guidelines in addition to heart healthy choices with the TLC diet.

Staying physically active is also a key component to maintaining heart health. It’s quite possible to stay active through leisure activities, such as golf, dancing, swimming, cycling, and walking. If you’d like to adopt a more strenuous exercise routine, be sure to discuss it with your doctor first.

Last, when you have CHD, it’s crucial to consider physical limitations when it comes to your career. Learn what your limitations are by speaking with your doctor, and be sure to understand and keep up with your healthcare insurance coverage.

As medical professionals, we’ve learned from history and experience, and are able to help you to be prepared for issues as well as triumphs. However, no one can be sure of what the future holds—it’s important to be prepared and continue to enjoy each and every success. Be proud of what you’ve accomplished so far, and continue to strive to maintain balance among work and play.

About Teresa Lyle, APRN:

Teresa received her Graduate degree from Emory University (Master of Nursing, Adult Health– Critical Care), and attained her Post-Graduate degree from University of Texas at Arlington (Pediatric Nurse Practitioner). Her clinical interests include ACHD, cardiac surgery, transition from pediatric to adult CHD care, pregnancy and planning in women with complex CHDs, and Eisenmenger syndrome/pulmonary hypertension.


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.