balancing your life with
congenital heart disease By Teresa A. Lyle, APRN, MN, CPNP Product # 588B
You are an important part of medical history! Since the 1960’s, medical advances are helping you and others, born with congenital heart disease (CHD), to live longer and fuller lives. As a result, you need special care for health problems that may come up as you age. This book will help you learn why you are so special and how you can balance your life with your condition. It includes:
• things you should know about CHD • what you can do to stay healthy • how best to exercise and stay active • the choices you need to make • references for specific conditions A young adult with congenital heart disease is much like a patchwork quilt … On the outside, beautiful to see. On the inside, there are many twisted strands, knots and patterns to be interpreted, understood and appreciated to make it beautiful.
Order this book from: PRITCHETT & HULL ASSOCIATES, INC. 3440 OAKCLIFF RD NE STE 126 ATLANTA GA 30340-3006
or call toll free: 800-241- 4925 Copyright© 2020 by Pritchett & Hull Associates, Inc. All rights reserved. No part of this book may be photocopied, reprinted or otherwise reproduced without written permission from Pritchett & Hull Associates, Inc. Published and distributed by: Pritchett & Hull Associates, Inc. Printed in the U.S.A.
This book is dedicated to the doctors and nurses who pioneered in the care of children with congenital heart disease. It is intended to help you learn more about your CHD. It is not a replacement for medical care and should not be used as such.
Table of Contents Why your needs are special................................................................2 About congenital heart disease.......................................................3-7 Simple..................................................................................................4 Moderate.............................................................................................5 Complex............................................................................................6-7 Things to be aware of................................................................... 8-16 Arrhythmia (abnormal rhythm)..........................................................9 Bacterial endocarditis................................................................10-11 Heart failure......................................................................................12 Hepatitis C.........................................................................................13 Liver health........................................................................................14 Chest pain.........................................................................................15 Chronic cyanosis and erythrocytosis...............................................16 Plan your future..................................................................................17 Live better...................................................................................18-21 Stay healthy.................................................................................19-20 Follow a healthy diet plan..........................................................21-23 Food labels........................................................................................22 Salt and fluid balance......................................................................23 Stay active...................................................................................24-25 What to do.........................................................................................24 What to avoid....................................................................................25 When to stop.....................................................................................25 Make good choices.......................................................................26-32 Intimacy and family issues..............................................................26 Thinking about raising a family.......................................................26 Birth control.......................................................................................27 Pregnancy..........................................................................................28 Your career........................................................................................29 Health insurance........................................................................29-30 Disability insurance and benefits....................................................31 Advance directives............................................................................32 End-of-life considerations for implanted defibrillator........................................................................................32 Looking forward............................................................................33-34 Appendix........................................................................................35-57
Why your needs are special You are a mystery Until 1960, many people born with moderate and complex CHD did not live to be adults. So as you age, you may have health problems that are unique and need special treatment. Only time will tell us what these may be because you are living in uncharted territory. You may not be aware that all of the major advances in treating CHD have taken place since the first successful surgery on an infant with cyanotic CHD in 1944. Thanks to advances in diagnostic testing, your life with CHD can be much improved. And, continuing research keeps advancing knowledge about the best way to diagnose and treat CHD.
Your reality Having CHD means that you need to take special care of yourself. Accept that you have a chronic condition and that you may need help from others in the future. With CHD, choosing the right school, the right career, the right places to travel and live are all very important. To stay healthy, you may need to plan for things that others take for granted.
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About congenital heart disease To help in the treatment of your heart disease, healthcare professionals put CHD into categories*. In doing this, they can address the healthcare needs of each young adult. Knowing the category you are in can help you know what care you need. The categories are:
• Simple • Moderate • Complex The heart defect(s) you have helps your heart doctor decide which category your treatment falls into. You may already know what your CHD is, but you may not exactly understand what it is. If you know, read through the defects that make up each category and check those that apply to you. If you do not already know, ask your heart doctor which condition(s) you have and then check those that apply
*A dapted from “Care of the Adult with Congenital Heart Disease.” Presented at the 32nd Bethesda Conference, Bethesda, Maryland, October 2-3, 2000.
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Simple If you mainly have a single defect that was fixed when you were an infant or a child, your disease category is simple. You may need to be seen by a cardiologist or adult congenital heart disease ACHD specialist at times. Some patients do not need long-term follow-up for simple defects that have been repaired. Check with your doctor to find out what is right for you.
These are the defects in the simple category: an un-repaired small atrial septal defect (ASD) or patent foramen ovale (PFO) secundum or sinus venosus atrial septal defect (ASD) that was closed with sutures (stitches) or a patch ventricular septal defect (VSD) that was closed with sutures (stitches) or a patch patent ductus areteriosus (PDA) that was closed in early childhood (In most cases, you do not have to see a cardiologist long-term.) pulmonary valve stenosis (mild)
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Moderate If you had one or more defects that needed complex repairs as an infant or as a young child or have a partly repaired defect, your disease category is moderate. These defects may require ongoing treatment or more surgery as an adult. If your heart defect falls into this category, your doctor should work closely with ACHD specialists to care for you. aortic stenosis (bicuspid aortic valve) anomalous pulmonary venous drainage, total (TAPVR) or partial (PAPVR) secundum or sinus venosus atrial septal defect (ASD) that was not repaired atrioventricular canal, complete (AVC) or partial (A-V Canal) coarctation of the aorta Ebstein’s anomaly infundibular right ventricular outflow obstruction (RVOTO) ostium primum atrial septal defect patent ductus arteriosus that was not closed (PDA) pulmonary valve regurgitation or insufficiency (moderate to severe) pulmonic valve stenosis (PS)—moderate to severe sinus of valsalva fistula/aneurysm tetralogy of fallot (TOF) ventricular septal defect (VSD) when there is any other congenital heart defect ventricular septal defect (VSD) which has not been closed
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Complex Your disease category is complex if you have one or more defects that have:
• not been repaired • been partly repaired, or • needed more than one surgery to repair All of your follow-up health care should be in an ACHD program if your heart defect puts you in this category. conduits, valved or non-valved (homograft or Rastelli-type repair) congenitally corrected transposition of the great arteries (l-TGA) cyanotic congenital hearts (all types) double outlet right ventricle (DORV) Eisenmenger syndrome Fontan procedure mitral atresia
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Complex (continued) single ventricle—double inlet or outlet, common or primitive (SV or DILV) pulmonary atresia (PA)—all forms pulmonary artery hypertension (PAH) transposition of the great arteries (TGA)— repaired with Mustard/Senning procedure transposition of the great arteries (TGA)— repaired with arterial switch tricuspid atresia (TA) truncus arteriosus or hemi-truncus other complex defects (crisscross heart, isomerism and heterotaxy syndromes)
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Things to be aware of Your main goal in living with your heart disease is to learn as much as you can about it, what to expect and what to look out for.
Long-term problems Some young adults with CHD may be at higher risk for heart disease, coronary artery disease or high blood pressure. Some surgeries that were called “repairs” were short-term fixes. These repairs were a way to sustain you at that time. You may have associated health problems that others without CHD do not have, and you may have them much earlier. This may be related to your past surgery and your underlying heart problem.
• Arrhythmia • Infective Endocarditis • Pulmonary hypertension • Heart failure • Hepatitis C • Chest pain Learn more about these problems and find out if you are at risk and if so, what to do to lower your risks. 8
Arrhythmia (abnormal rhythm) An abnormal rhythm is one of the most common problems in young adults with CHD. Changes in your heart rhythm are often caused by scars inside your heart from surgery or the heart problem you were born with. Or they may be due to stress or stretching of the heart muscle or valves over time.
Symptoms with a change in heart rhythm include: • palpitations • chest pain or tightness in your neck or shoulder area • dizziness or fainting • shortness of breath Palpitations make you aware of a change in your heart rhythm. They may be just skipped beats, or they may be fast or slow rhythms. Heart rhythm problems may be warning signs that something is changing in your heart condition. You should be checked as soon as possible. You may need to be watched for awhile. Or you may need treatment that might include:
• taking medicine • having an ablation
(a non-surgical procedure to correct an abnormal rhythm)
• getting a
pacemaker implanted
• getting an ICD
(implantable cardiac defibrillator)
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Bacterial endocarditis Bacterial endocarditis is an infection of your heart’s inner lining (the endocardium) and/or your heart valves. It is a rare disease caused by bacteria (germs). If the germs get into your bloodstream, they can settle on one of your heart valves, leading to infection. For those at high risk, antibiotics are prescribed before having any dental procedure. This is to prevent germs getting into the blood stream. You are at higher risk if you have a chronic cyanotic heart condition, a valve replacement or a hole remaining near a VSD patch. Current guidelines* say you should take antibiotics before a dental procedure if you have:
• a prosthetic or artificial heart valve (either from tissue or a mechanical valve)
• had endocarditis before • one of the following:
– unrepaired cyanotic heart disease, even if you have palliative shunts or conduits
– heart disease that was completely repaired with man-made material or device (for the first six months after surgery or catheter intervention)
– repaired heart disease but there are still defects at or near the site of a man-made patch or device
• a heart transplant and you have heart valve disease
* Source: Management of Patients with Valvular Disease Guidelines in 2017 ACC/AHA
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Endocarditis can be treated. The key is early diagnosis. Talk with your doctor to see if you should take antibiotics before any planned procedure (dental work). Keep in mind that this may change if you have more surgery or your heart condition changes. If your doctor prescribes antibiotics for you, be sure that your dentist, and any other doctor you go to, knows about it too.
Caution! Any procedure which breaks the skin carries a risk of infection. This includes body art or body piercing. Piercing which involves the nose, lips, tongue, genitals or navel carries more risk than having your ears, eyebrows or nipples pierced. If you want to get a tattoo or have a body part pierced, discuss this with your ACHD doctor before you do.
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Heart failure The symptoms of heart failure (reduced heart function) vary widely, and may be mild and rare, but may also impact your daily quality of life. For you, heart failure may be caused by the nature of your defect and changes in how your heart performs over time. If blood backs up into your veins, you may;
• notice swelling in your legs and ankles • be sore or bloated around your stomach • be tired a lot • lose your appetite If fluid backs up into your lungs, you may:
• feel short of breath • have a chronic cough that does not go away • wake up breathless during sleep • find you cannot sleep unless you
prop up on pillows or sit up in a chair
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Hepatitis C Hepatitis is an inflammation of the liver most often caused by a virus. While there are several types of hepatitis, hepatitis C is the most common. In fact, it is the most common blood-borne infection in the United States. Many young adults received blood during childhood surgery. Prior to 1992, the blood supply was not always screened for hepatitis C. However, it is important to know that most people who had a transfusion before 1992 do not develop hepatitis C as a result of the transfusion. If you are not sure if you are at risk, get tested. If you have hepatitis C, a liver doctor (called a Hepatologist) can check you and offer treatment options. Hepatitis C affects everyone differently. Some people have serious problems within a few years of getting the virus. But some have no problems after 10 years or more.
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Liver health At some point in your life, you might develop liver problems. When these may happen and how they may affect you are not fully understood. For some, having the Fontan type repair causes high venous pressure and added stress on the liver.
Protection and prevention Anyone with ACHD should take these steps to prevent liver problems:
• See your ACHD physician regularly and make sure that visits include liver monitoring.
• Talk to your doctor about whether you will need a Hepatitis C screening.
• Make sure your Hepatitis vaccines are up to date. • Avoid
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– alcohol
– too much Tylenol
– estrogen contraceptives
Chest pain Chest pain, known as angina, is very rare in CHD patients.
If you have any chest pain, see your doctor. It may be caused by:
• enlargement of the heart • stretching or stress on the heart from abnormal heart valves • an abnormal heart rhythm • coronary arteries • problems outside of the heart, such as:
– sore muscles
– inflammation
– an injured rib
– a pinched nerve
– esophageal spasm (trouble swallowing)
– gastric reflux (GERD or heartburn)
Some activities may cause chest pain or make it worse. Take note of what you are doing if you have any chest pain. What are your symptoms (out of breath, dizzy, etc.)? Also note how intense the pain is and how long it lasts. Is the pain very brief – does it last longer than a TV commercial (about 30 seconds) or is it shorter?
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Chronic cyanosis and erythrocytosis Some people were born with heart defects before surgeries were done to repair them. Some people have heart defects that cannot be completely repaired. Defects such as ASD, VSD, and PDA allowed extra blood flow to the lungs through the defect or extra vessel. This damages tiny blood vessels in the lungs over time. Some people develop high blood pressure in the lungs, called pulmonary hypertension (or Eisenmenger Syndrome). This high pressure causes the blood to flow back through the defect, away from the lungs. This is called chronic cyanosis. There is a bluish tint to your skin due to low oxygen levels in your blood. As your body gets less oxygen, it may make extra red blood cells to try to make up for it. When your body does this it is called erythrocytosis. (Some people call this “thick blood.”) These extra red blood cells are often needed to help your tissues get oxygen.
Caution! 1. Phlebotomy
Check with your ACHD doctor before having any blood removed.
2. Air Filters
If you have cyanotic CHD, and you need an IV, ask about an “air filter” to prevent an air embolism.
3. Air Travel
Check with your doctor before flying.
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Plan your future As a child, you may have felt over protected or not protected enough by your parents and family. As a young adult, it is up to you to live your life in balance. You have heart disease. But don’t dwell on it. Follow your doctor’s advice and take your prescribed medicines. Find a healthy balance and avoid extremes. You are a success and a survivor. But living with a chronic illness can take a toll on you. Learn as much as you can about your heart disease. Accept help when you need it. Share your healthcare plans with your friends and family. They, along with your doctors and nurses, can help you continue your success.
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Live better As you learn about your CHD, and the risks of developing problems as you get older, it is important to set goals to reduce those risks. When your risks are higher, it is important to:
• maintain a healthy weight • follow a heart-healthy diet plan • stay as active as you can • have your blood pressure and cholesterol checked regularly
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Stay healthy These guidelines may help keep you and your heart healthier:
• Get a flu shot each year if your doctor advises. • Get a pneumonia vaccine if your doctor advises it. • Take antibiotics before dental visits if your doctor has advised you to. Take good care of your teeth and get regular dental check-ups and cleanings.
• Discuss any non-cardiac surgery (unless an emergency) with your heart doctor (cardiologist) before you schedule it. This
is very important, even for simple outpatient procedures. Your anesthesia needs may differ with the type of CHD you have.
• Avoid over-the-counter drugs and herbal supplements unless your heart doctor says they are OK. These may:
– interact badly with your prescribed heart drugs
– cause heart rhythm problems
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• Avoid smoking and smokeless tobacco. • Avoid vaping and all forms of e-cigarettes • 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 such as marijuana, cocaine, methamphetamines, etc.
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— Stimulants (like amphetamines or amyl nitrate “poppers”) may be dangerous.
— Marijuana raises the heart rate and oxygen needs of the heart muscle.
— Cocaine can cause fatal heart attacks.
Follow a healthy diet plan Start by knowing how many calories you should be eating. How
many calories you need depends on many factors (your age, sex and activity level). Another factor is whether you want to gain, lose or maintain your weight. If you want to lose weight, you need to use up more calories than you take in. (Remember that everyone should strive for 150 minutes of moderate activity each week.)
Each day, try to include: • Fruits and vegetables of every color • Whole grains • Low-fat dairy • Poultry (without skin) and fish • Nuts and beans • Healthy oils and fats (olive oil, avocado)
Limit: • saturated and trans fats • high salt foods • red meat (including processed and deli meat)
• added sugar (in beverages and processed sweets)
• alcohol (one drink a day for women and two drinks a day for men)
• fast food and boxed or frozen “convenience foods”
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Food labels The amount and types of foods you eat are important. Learn to read food labels and keep up with the amount of saturated fat, added sugars and sodium you eat each day. Most food labels list the serving size and contents (for 1 serving) of that product. The percent of daily value is based on eating 2,000 calories a day. If you need more than 2,000 calories, learn how to adjust the label for your calorie needs. Foods are labeled with nutrition facts based upon daily values. The government requires almost all packaged foods carry specific nutrient information. Your grocer can also give you the food label information about items that are not prepackaged. Such as, fresh vegetables, fruits, meats, poultry and seafood items. Although not all nutrients are required to be listed, most are. And the ones you need to watch out for are.
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Salt and fluid balance Those with reduced heart function If you have heart failure, or are at risk for it, you may need to set limits on how much salt or sodium you eat each day. You also may need to watch your total fluid intake daily. Many with heart failure should try to keep theirtotal sodium intake to less than a teaspoon of salt a day (2,300 mg). Some people should try to have even less. Ask your doctor what is right for you and your diet. As a rule, those with heart failure need to drink no more than 8 cups (64 oz) of fluids a day. Find out from your doctor if this applies to you. If it does, keep up with all the fluids you drink, not just water.
Those with cyanotic heart disease Young adults with CHD, who have cyanosis and erythrocytosis (too many red blood cells causing thick blood), must have the right salt and fluid intake to keep from getting dehydrated. The right balance is also needed to help keep their blood from getting “too thick�. This is another reason why it is important that you talk with your ACHD doctor about what is best for you. What is best for you depends on your heart condition and your
health, not some general rule.
Ask your doctor what you need: Sodium: _________ mg per day is right Fluid intake: _________ ozper day is best
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Stay active Staying active helps improve your stamina and heart health. Use common sense when you exercise. Pick a time of day when it isn’t too hot or cold. Avoid high altitudes. Before you try a new exercise
or activity, discuss it with your doctor.
What to do Leisure activities don’t put much strain on your heart and are often good exercises. These are things like:
• golf (with a cart) • dancing (slow) • swimming (always swim with a friend) • cycling Outside chores like gardening, raking leaves and mowing the grass and inside chores like vacuuming and dusting are most often heart friendly too. Walking is usually safe and a fun workout, if kept at an easy pace. (You should be able to talk freely without being short of breath as you walk.) If you have problems, choose a path that is mostly flat.
Talk to your doctor before starting more strenuous aerobic exercise or taking part in any competitive sports.
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What to avoid Discuss an exercise program with your ACHD doctor – especially if you haven’t exercised regularly. Your doctor may order tests, like an exercise stress test, to find out what activities and amount of exercise are best for you. You may need to limit demanding exercises like pushups, sit-ups, chin-ups, and some contact sports (like football). It you want to do any weight-lifting, talk with your ACHD doctor about it. Weight-lifting may be harmful for some types of CHD. If weight-lifting is OK for you, small (less than 10 lbs) free weights are generally safe. Try to do more repetitions instead of adding more weight.
When to stop Stop exercising and talk to your doctor, if you: • have any chest pain • have any chest tightness • feel out of breath • feel like you could faint
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Make good choices Intimacy and family issues Because you have CHD, you may wonder how this will affect your love life. Since there isn’t a lot of information out there, you may think the worst. It’s important to talk about this with your heart doctor and to make good choices about sex. Most heart conditions do not mean you cannot be intimate. Even though the level of exercise and activity you can handle may be different from another young adult with CHD, you should be able to have sex. If you are a man, talk with your doctor about erectile dysfunction (ED) and potential treatments. Some cardiac drugs have side effects that may lead to ED. But there may be other causes for ED too. It is important you not skip or stop taking key heart medicines to try to improve ED on your own. Talk about ED medicines with your cardiologist before you start taking them.
Thinking about raising a family Bring your fiancé, spouse or partner to the clinic with you to listen and learn firsthand about your heart condition. Talk to your doctor, nurse practitioner or nurse about any fears or issues. It is important for both you and your partner to know about your heart condition.
When either you or your spouse has CHD, there is, in general, a 5 to 10% risk that your baby will have CHD. But it may not be the same CHD you have. And certain types of CHD may have higher or lower risks. To find out more, ask your doctor how to get in touch with a genetic specialist. To help you make the right choices, talk with your heart doctor about your risks.
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Birth control Women should talk about birth control options and the risks of pregnancy before having sex. Preventing an unwanted pregnancy is easier than making life or death choices for both you and your baby. For many CHD women, routine birth control options are OK. Talk with your gynecologist and your ACHD doctor if you have specific questions about a birth control method. Some women with complex heart disease, cyanosis or pulmonary hypertension should not use hormonal contraceptives with estrogen, because these may increase the risk of blood clots. Progesterone-only contraceptives may have other long-term side effects. A condom should always be used with another type of birth control, such as a diaphragm. In the past, intrauterine devices (IUDs) were not used because of the concern for endocarditis. But now, some newer IUDs and cervical rings are much safer. And these may be good options to think about as an alternative to more permanent treatment, such as tubal ligation.
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Pregnancy If you are a young woman with a moderate or complex CHD, you should have a complete physical exam with your heart doctor before
you get pregnant.
Some young women with CHD can expect a normal pregnancy and delivery without worry. For others, there is a high risk to the baby and the mother. Heart disease may also get worse after pregnancy. And, some with complex forms of CHD should never get pregnant. Keep in mind that giving birth is just one way to become a mother. Ask your doctor about other ways, like adoption or surrogacy. There is more to being a mother than being pregnant.
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Your career Make good choices about your career. Finding a job that meets your heart needs and your personal goals is very important. You may need to avoid a job where you have to stand, lift, push or pull a lot. Learn what your limits are by talking with your doctors. Some young adults choose to go to college to get a degree. But college isn’t for everyone. If you are one of them, vocational programs can help train and certify you for a career in programming, maintenance or support. One of these areas may be a good choice if you have physical limitations.
Health insurance Getting and keeping healthcare insurance is very important when you have a pre-existing condition such as CHD. A pre-existing condition is a chronic medical condition or illness that has been treated in the past and is expected to need additional care. When thinking about a job, understand how your CHD will affect your life and disability insurance benefits. Many changes from legislation impact health insurance plans and choices. It is important to consider benefit options carefully when making employment decisions. 29
Health insurance and disability benefits vary from country to country. Some countries provide coverage to all citizens, even with pre-existing conditions. In the U.S., the ACA (Affordable Care Act) was passed, in part, to help those who were unable to get insurance due to a pre-existing condition.
• Contact healthcare.gov for more information. • Medicaid is insurance for those who are disabled and have a low income. Apply for it at your local County Department of Family and Children’s Services.
• Keep up with your healthcare follow up, no matter what your
insurance status. Many specialty ACHD Centers have resources to help with your healthcare.
• For some helpful resources, see pages 54-55.
It is very important you continue your health insurance coverage. •T he ACA gives you the ability to choose a plan from the Marketplace rather than having to go with the plan your employer offers. •T he ACA gives you “portability” in that you can take your healthcare with you if you change jobs.
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Disability insurance and benefits Work if you can. National, federal or state funded healthcare disability programs should only be used by those that cannot work. To draw disability benefits as an adult in the U.S., you will need to show medical proof of not being able to work at any job. Any disability benefits you had through your parents most often expire at age 18 or 21. It is important to apply for disability insurance early. Processing a disability application can take months. Often, a claim will be denied and you will have to appeal it. An employer that offers short-and long-term disability benefits may help you deal with short-term health issues. In order to get this disability insurance, you will need to answer several questions about your health. The insurance company will then contact your doctors to verify your health condition. Having coverage through your employer may make it easier to get full disability in the future, if you need to. This coverage is worth the cost if your company offers it. And when you have to use it, it is not taxable income.
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Advance directives An advance directive is a document that lets you make specific healthcare decisions now for those times you may not be able to. The two that are most common are a Living Will and a Durable Healthcare Power of Attorney (also called Durable Power of Attorney for Healthcare).
• A Living Will is a way to let your doctor, family and friends know your wishes about being kept alive by machines (like a respirator or ventilator). It also spares your loved ones from having to make those hard choices.
• Durable Healthcare Power of Attorney is a legal document that names someone to make medical decisions for you if you are not able to.
Talk with your lawyer. If you don’t have a lawyer, contact your state bar association for a reference. Every state’s laws vary. It is important that your document works for the state in which you live. And that it has been signed (in good health), witnessed and, if possible, notarized.
End-of-life considerations for implanted defibrillator If you have an ICD, your device cannot stop your heart from wearing out. If your doctor tells you that your heart disease is at the “end stage”, talk to your family. Choose whether you want the defibrillator’s “shock” function turned off. This information should also be part of your advance directives and any hospice and/or palliative care plan.
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Looking forward We have learned from history and recent experience to help you be prepared for problems as well as successes. Medical care gets better every day. But, no one knows what the future holds. Be prepared for setbacks. Enjoy each and every success. Embrace difficult decisions about treatment options with support of your family, friends and your healthcare team. Discussing your congenital heart disease and expectations for future healthcare problems with your ACHD doctor is very important, and should begin in adolescence. The key issues should be reviewed and an ongoing relationship with your ACHD doctor will help guide you through each phase of life. Look at all you’ve accomplished so far. You have learned to balance your “special” heart with family, work and play. Living a long, fulfilling life with CHD is still possible. If you and your family are ready, you’ll be able to get over the bumps in the road and move on with life’s journey.
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Keep in mind — it’s not how long the road is that matters. It’s what you make of it. With all you’ve learned from this book and from your health care team, enjoy your journey.
• Learn all you can about your health condition. • Follow a heart-healthy diet. • Take your medicines. • Keep all your follow-up appointments and work closely with your ACHD heart clinic.
• Stay as active as you can. • Make good choices about your career, your insurance needs, your hobbies and your family.
• Take part in local and national CHD organizations like the ACHA. What is important? Balancing your career, heart condition, family and all other parts of your life!
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appx.
Appendix This appendix is divided into three parts. Part One gives you a diagram of a normal heart, labeled to show how blood flows through it. Part One also contains information on some of the more common complex congenital heart defects in young adults, and checklists that your doctor or nurse can help you with.
• Normal Heart and Blood Flow • Modifiable heart diagram (used to show simple CHDs) • Aortic Stenosis • Congenitally Corrected Transposition of the Great Arteries (CCTGA)
• Ebstein’s Anomaly • Single Left Ventricle (Tricuspid Atresia & Double Inlet LV) • Single Right Ventricle (Hypoplastic Left Heart Syndrome) • Tetralogy of Fallot (TOF) • Transposition of the Great Arteries (TGA) Part Two contains references on where you can get more information about many of the issues addressed in this book. Part Three is for questions and answers and notes you may want to make.
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appx.
Normal Heart and Blood Flow The normal heart is divided into 4 chambers:
Four valves act as one-way doors to direct blood flow:
•
right atrium
•
aortic valve
•
left atrium
•
mitral valve
•
right ventricle
•
pulmonary valve
•
left ventricle
•
tricuspid valve
The chambers on the right side of the heart receive blood from the veins (“blue” blood) and pump the “blue” blood out to the lungs. The chambers on the left side receive the “red” (oxygen-rich) blood from the lungs and pump it out of the heart to the rest of the body.
A wall (septum) divides the right and left side of the heart.
blood from upper body aorta to body superior vena cava to lungs main pulmonary artery
pulmonary veins from lungs left atrium
pulmonary veins from lungs
atrial septum mitral valve
pulmonary valve right atrium
left ventricle
aortic valve
tricuspid valve inferior vena cava
blood from lower body
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right ventricle
ventricular septum
appx.
Other CHD: Your doctor can use this picture of a heart to explain your defect.
Comments:
Potential Long-term Problems:
Future Surgical Procedures:
Pregnancy Recommendations:
37
appx.
Aortic Stenosis Problem valve too narrow
Surgical Procedure (Aortic valve replacement)
mechanical valve
Surgical Procedure (Ross procedure) from donor from pulmonary artery
coronary arteries moved
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tissue valves
appx.
Aortic Stenosis
Comments:
Potential Long-term Problems (Check those that apply to you.)
Aortic root enlargement:
Valve problems:
Ventricular function:
Hypertension:
Coronary disease:
Future Surgical Procedures:
Pregnancy Recommendations:
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appx.
Congenitally Corrected Transposition of the Great Arteries Comments: Malposed Great Arteries
Potential Long-term Problems:
left ventricle
Arrhythmias:
Systematic right ventricle failure:
right ventricle
Ventricular Inversion
Pregnancy Recommendations:
40
appx.
Ebstein’s Anomaly Problem
hole between chambers
PDA
Comments:
enlarged right atrium
Potential Long-term Problems: (check those that apply to you.) malformed tricuspid valve
Arrhythmias:
Valve insufficiency:
RV size/function:
thin right ventricle
fenestration
Surgical Procedure (Extracardiac Fontan)
Future Surgical Procedures:
Pregnancy Recommendations:
conduit (bypass tube)
inferior vena cava
41
appx.
Single Left Ventricle— Tricuspid Atresia tricuspid valve missing
Problem
conduit (bypass tube)
Surgical Procedure (Classic Fontan)
artery too narrow
patch
hole between chambers
right chamber too small fenestration
Surgical Procedure
fenestration
(Intracardiac Fontan)
42
artificial wall
conduit (bypass tube)
Surgical Procedure (Extracardiac Fontan)
appx.
Single Left Ventricle— Tricuspid Atresia Comments:
Potential Long-term Problems (Check those that apply to you.)
Arrhythmias:
Ventricular failure:
Elevated venous pressure:
Liver and/or kidney failure:
Varicose veins:
Protein losing enteropathy:
Pregnancy Recommendations:
43
appx.
Single Left Ventricle — Double Inlet Left Ventricle Problem
fenestration
pulmonary artery over developed
Surgical Procedure (Extracardiac Fontan)
hole between chambers
underdeveloped chamber
stitches
conduit (bypass tube)
Surgical Procedure (Intracardiac Fontan)
Temporary Surgical Procedure pulmonary artery
(modified Blalock-Taussig Shunt) subclavian artery
stitches
temporary shunt
artificial wall 44
fenestration
appx.
Single Left Ventricle — Double Inlet Left Ventricle Comments:
Potential Long-term Problems (Check those that apply to you.)
Arrhythmias:
Ventricular failure:
Elevated venous pressure:
Liver and/or kidney failure:
Varicose veins:
Protein losing enteropathy:
Pregnancy Recommendations:
45
appx.
Single Right Ventricle— Hypoplastic Left Heart Syndrome Problem artery too narrow
modified Blalock-Taussig Shunt (temporary)
(Stage I–Norwood Procedure)
connecting blood vessel artery underdeveloped
hole between chambers
superior vena cava
chamber too small
Surgical Procedure
new aorta
stitches
atrial septum removed fenestration
(Stage II– Bidirectional Glenn Shunt) stitches
conduit (bypass tube) 46
Surgical Procedure
Surgical Procedure (Stage III– Extracardiac Fontan Completion)
appx.
Single Right Ventricle— Hypoplastic Left Heart Syndrome Comments:
Surgical Procedure (Sano modification)
Potential Long-term Problems: (check those that apply to you.)
new aorta
tube (conduit)
Arrhythmias:
Ventricular failure:
Elevated venous pressure:
Liver and/or kidney failure:
Varicose veins:
Protein losing enteropathy:
Pregnancy Recommendations:
47
appx.
Tetralogy of Fallot with or without Pulmonary Atresia Problem
Surgical Procedure
underdeveloped artery
(Complete repair of TOF with Transannular patch)
aorta just above hole hole between chambers
patch
enlarged
narrow opening
cut
Surgical Procedure
stitches
Surgical Procedure
(Pulmonary valve
(Complete repair of TOF with Homograft)
replacement) tissue valves
tube
patch
conduit (bypass tube) 48
cut
mechanical valves
appx.
Tetralogy of Fallot with or without Pulmonary Atresia Comments:
Potential Long-term Problems (Check those that apply to you.)
Arrhythmias:
Pulmonary insufficiency:
Right ventricle size/function:
Aorto-pulmonary collaterals:
Future surgery:
Pregnancy Recommendations:
49
appx.
Transposition of the Great Arteries Arterial Switch Repair Problem connecting blood vessel arteries go to wrong chambers
hole between chambers
Surgical Procedure (Arterial Switch) stitches arteries moved
patch
coronary arteries moved
50
appx.
Transposition of the Great Arteries Arterial Switch Repair Comments:
Potential Long-term Problems (Check those that apply to you.)
Coronary artery issues:
Aortic Root stenosis or enlargement:
Pulmonary artery/valve issues::
Liver and/or kidney failure:
Future surgery:
Pregnancy Recommendations:
51
appx.
Transposition of the Great Arteries Mustard/Senning Repair Problem connecting blood vessel arteries go to wrong chambers tied or cut
Surgical Procedure (Red blood path)
baffle hole between chambers tied or cut
Surgical Procedure (Blue blood path)
baffle
52
appx.
Transposition of the Great Arteries Mustard/Senning Repair Comments:
Potential Long-term Problems (Check those that apply to you.)
Arrhythmias:
Sick sinus syndrome:
Systemic right ventricle failure:
Baffle stenosis/leaks:
Pregnancy Recommendations:
53
appx.
References and resources • Adult Congenital Heart Association (ACHA) achaheart.org
Phone: (888) 921-ACHA • Fax: (215) 849-1261
ACHA is a nonprofit organization which seeks to improve the quality of life and extend the lives of adults with congenital heart defects. Website offers many resources and an extended set of links for additional information and contacts for ACHD.
• Americans with Disabilities Act ada.gov
Phone: 800-514-0301 • 800-514-0383 (TTY)
ADA Specialists are available to provide ADA information and answers to technical questions on Monday, Tuesday, Wednesday, and Friday from 9:30 a.m. until 5:00 p.m. or on Thursday from 12:30 p.m. until 5:30 p.m. (Eastern Time).
• Disability Rights Section Mailing Address
U.S. Department of Justice, 950 Pennsylvania Avenue, NW Civil Rights Division, Disability Rights Section - NYA Washington, D.C. 20530
54
appx.
• American Heart Association heart.org 1-800-242-8721
Website offers information and links for Adults with Congenital Heart Disease.
• Affordable Care Act (ACA) healthcare.gov
• U.S. Department of Labor dol.gov 1-866-4-USA-DOL
A lot of useful information on health plans & benefits.
• The Health Insurance Portability and Accountability Act (HIPAA) Fact Sheet cms.gov
• The International 22q11.2 Foundation Inc.
22q.org (A chromosome disorder that can lead to other health problems)
• Social Security Administration ssa.gov
• National Down Syndrome Congress ndsccenter.org
• International Society for Adult Congenital Heart Disease isachd.org
55
appx.
Questions you may have Use this space to write down any questions you have. Ask your healthcare team about them.
Q. A. Q. A. Q. A. Q. A. Q. A. Q. A. Q. A. Q. A. 56
Notes
Author
Reviewers
Teresa A. Lyle, APRN, MN, CPNP Clinical Instructor/Lecturer University of Tennessee, Chattanooga Chattanooga, TN
Wendy M. Book, MD Associate Professor of Medicine (Cardiology) Emory University Medical Director Emory Adult Congenital Heart (EACH) Center Atlanta, GA
Nurse Practitioner Emory Adult Congenital Heart (EACH) Center Atlanta, GA
Special thanks to other reviewers: Amy C. Burk (ACHD patient) Tanya Dutton (CHD parent)
Michael E. McConnell, MD Associate Clinical Professor of Pediatrics (Cardiology) Emory University Emory Adult Congenital Heart (EACH) Center and Sibley Heart Center Atlanta, GA Kathy Murphy, RN, MSN, APRN Clinical Nurse Specialist and Pediatric Nurse Practitioner Cardiovascular Services Children’s Healthcare of Atlanta at Egleston Atlanta, GA
Estella Moore (LCSW) Brian Kogon, MD Assistant Professor of Cardiothoracic Surgery Emory University Surgical Director Emory Adult Congenital Heart (EACH) Center Pediatric Cardiothoracic Surgery Children’s Healthcare of Atlanta at Egleston Atlanta, GA Pamela D. Miner, RN, MN, NP Nurse Practitioner Ahmanson Adult Congenital Heart Disease Center University of California Los Angeles (UCLA) Los Angeles, CA
3440 Oakcliff Road, NE, Suite 126 Atlanta, GA 30340-3006 1-800-241-4925 • www.p-h.com