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balancing your life with

Congenital Heart Disease By Teresa A.By Lyle, Teresa RN, A. MN, Lyle, CPNP APRN, MN, CPNP

60 Product # 588A

Y

ou 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: s THINGSYOUSHOULDKNOWABOUT#($ s WHATYOUCANDOTOSTAYHEALTHY s HOWBESTTOEXERCISEANDSTAYACTIVE s THECHOICESYOUNEEDTOMAKE s REFERENCESFORSPECIlCCONDITIONS 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:

Published and distributed by:

PRITCHETT & HULL ASSOCIATES, INC.

Pritchett & Hull Associates, Inc.

Š 3440 OAKCLIFF RD NE STE 110

ATLANTA GA 30340-3006

Printed in the U.S.A.

#OPYRIGHTĂš  BY0RITCHETT(ULL!SSOCIATES )NC All rights reserved. No part of this book may be photocopied, reprinted or otherwise reproduced without written permission from Pritchett & Hull Associates, Inc. ISBN: 978-1-933638-51-5

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.

T

able 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

1

W

hy 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 ďŹ rst 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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A

bout 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: s 3IMPLE s -ODERATE s #OMPLEX 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 to you. * Adapted 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 ďŹ xed 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 ďŹ nd out what is right for you. These are the defects in the simple category: T an un-repaired small atrial septal defect (ASD) or patent foramen ovale (PFO) T secundum or sinus venosus atrial septal defect (ASD) that was closed with sutures (stitches) or a patch T ventricular septal defect (VSD) that was closed with sutures (stitches) or a patch T patent ductus areteriosus (PDA) that was closed in early childhood (In most cases, you do not have to see a cardiologist long-term.) T pulmonary valve stenosis (mild) 4

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. T aortic stenosis (bicuspid aortic valve) T anomalous pulmonary venous drainage, total (TAPVR) or partial (PAPVR) T secundum or sinus venosus atrial septal defect (ASD) that was not repaired T atrioventricular canal, complete (AVC) or partial (A-V Canal) T coarctation of the aorta T Ebstein anomaly T infundibular right ventricular outflow obstruction (RVOTO) T ostium primum atrial septal defect T patent ductus arteriosus that was not closed (PDA) T pulmonary valve regurgitation or insufficiency (moderate to severe) T pulmonic valve stenosis (PS)—moderate to severe T sinus of valsalva fistula/aneurysm T tetralogy of fallot (TOF) T ventricular septal defect (VSD) when there is any other congenital heart defect T ventricular septal defect (VSD) which has not been closed 5

Complex Your disease category is complex if you have one or more defects that have: s NOTBEENREPAIRED s BEENPARTLYREPAIRED OR s NEEDEDMORETHANONESURGERYTOREPAIR All of your follow-up health care should be in an ACHD program if your heart defect puts you in this category. T conduits, valved or non-valved (homograft or Rastelli-type repair) T congenitally corrected transposition of the great arteries (l-TGA) T cyanotic congenital hearts (all types) T double outlet right ventricle (DORV) T Eisenmenger syndrome T Fontan procedure T mitral atresia

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Complex (continued) T single ventricle—double inlet or outlet, common or primitive (SV or DILV) T pulmonary atresia (PA)—all forms T pulmonary artery hypertension (PAH) T transposition of the great arteries (TGA) —repaired with Mustard/Senning procedure T transposition of the great arteries (TGA)— repaired with arterial switch T tricuspid atresia (TA) T truncus arteriosus or hemi-truncus Tother complex defects (crisscross heart, isomerism and heterotaxy syndromes)

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T

hings 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. s !RRHYTHMIA s )NFECTIVE%NDOCARDITIS s 0ULMONARYHYPERTENSION s Heart failure s (EPATITIS# s 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.

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!RRHYTHMIAABNORMALRHYTHM 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: s PALPITATIONS s CHESTPAINORTIGHTNESS in your neck or shoulder area s DIZZINESSORFAINTING s SHORTNESSOFBREATH 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: s TAKINGMEDICINE s HAVINGANABLATIONANON SURGICAL procedure to correct an abnormal rhythm) s GETTINGAPACEMAKERIMPLANTED s GETTINGAN)#$IMPLANTABLECARDIAC deďŹ brillator) implanted 9

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: s APROSTHETICORARTIlCIALHEARTVALVEEITHERFROM tissue or a mechanical valve) s HADENDOCARDITISBEFORE s ONEOFTHEFOLLOWING – 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 s AHEARTTRANSPLANTANDYOUHAVEHEARTVALVEDISEASE

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*Source: Prevention of Infective Endocarditis Guidelines in 2007

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; s NOTICESWELLINGINYOURLEGS and ankles s BESOREORBLOATEDAROUND your stomach s BETIREDALOT s LOSEYOURAPPETITE If uid backs up into your lungs, you may: s FEELSHORTOFBREATH s HAVEACHRONICCOUGH that does not go away s WAKEUPBREATHLESS during sleep s lNDYOUCANNOT sleep unless you prop up on pillows or sit up in a chair

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Hepatitis C Hepatitis is an inammation 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. 13

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. 0ROTECTIONANDPREVENTION Anyone with ACHD should take these steps to prevent liver problems: s 3EEYOUR!#($PHYSICIANREGULARLYANDMAKESURETHAT visits include liver monitoring. s 4ALKTOYOURDOCTORABOUTWHETHERYOUWILLNEEDA Hepatitis C screening. s -AKESUREYOUR(EPATITISVACCINESAREUPTODATE s !VOID – alcohol – too much Tylenol – estrogen contraceptives

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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: s ENLARGEMENTOFTHEHEART s STRETCHINGORSTRESSONTHEHEART from abnormal heart valves s ANABNORMALHEARTRHYTHM s CORONARYARTERIES s PROBLEMSOUTSIDEOFTHE heart, such as: – sore muscles – inammation – an injured rib – a pinched nerve – esophageal spasm (trouble swallowing) – gastric reux (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. 7HATAREYOURSYMPTOMSOUTOFBREATH DIZZY ETC !LSO 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 ORISITSHORTER

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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! 1.0HLEBOTOMY Check with your ACHD doctor before having any blood removed. 2.!IR&ILTERS If you have cyanotic CHD, and you need an IV, ask about an “air filter” to prevent an air embolism. 3.!IR4RAVEL Check with your doctor before flying. 16

P

lan 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: s MAINTAINAHEALTHYWEIGHT s FOLLOWAHEART HEALTHYDIETPLAN s STAYASACTIVEASYOUCAN s HAVEYOUR blood pressure and cholesterol checked regularly

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Stay healthy These guidelines may help keep you and your heart healthier: s ' ETAmUSHOT each year if your doctor advises. s ' ETAPNEUMONIA vaccine if your doctor advises it. s 4AKEANTIBIOTICS before dental visits if your doctor has advised you to. Take good care of your teeth and get regular dental check-ups and cleanings. s $ISCUSSANYNON CARDIACSURGERYUNLESS 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. s !VOIDOVER THE COUNTERDRUGSANDHERBALSUPPLEMENTS 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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s !VOIDSMOKINGAND smokeless tobacco. s 3OMEYOUNGADULTS with CHD need to limit alcohol and caffeine in their diets. Check with your doctor to see how alcohol and caffeine may affect you. s !VOIDALLILLICITDRUGSSUCHASMARIJUANA cocaine, methamphetamines, etc. 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.

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&OLLOWAHEALTHYDIETPLAN The TLC (4HERAPEUTIC,IFESTYLE#HANGES*) diet is a heart healthy way to eat. It is a low saturated fat, low cholesterol diet for your calorie needs. If you follow it, it can help to reduce your total blood cholesterol as well as your LDL cholesterol levels. Lower cholesterol levels help reduce your risks for coronary heart disease. To follow this plan, ask your doctor or dietitian how many calories you should have each day. Write that amount here: My daily calories = ________________ Eat enough calories to get to and keep a healthy weight. And spread those calories out this way: &ATS s LESSTHANOFTHESECALORIESINSATURATEDFAT s LESSTHANINPOLYUNSATURATEDFAT s LESSTHANINMONOUNSATURATEDFAT  ORLESSOFYOURCALORIESINALLFATSˆ saturated and unsaturated) Cholesterol s ,ESSTHANMGMILLIGRAMS EACHDAY Carbohydrates s "ETWEEN OFYOURDAILYCALORIES &IBER s "ETWEEN GRAMSEACHDAY 0ROTEIN s !BOUTOFYOURDAILYCALORIES 21 *From the Third Adult Treatment Panel (ATPIII), National Institute of Health

&OODLABELS The amount and types of foods you eat are important. Learn to read food labels and keep up with the amount of fat, saturated fat, cholesterol and sodium you eat each day.

Serving Size 1 cup 140g (140g) Servings Per Container 10 Amount Per Serving Calories 221

Calories from Fat 11

% Daily Value*

Most food labels list the SERVINGSIZEANDCONTENTSFOR 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 speciďŹ c nutrient information. Your grocer can also give you the food label information about items that are not pre-packaged. 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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Total Fat 1g

2% 0%

Saturated Fat 0g Trans Fat Cholesterol 0mg

0%

Sodium 1mg

0%

Total Carbohydrate 43g

14% 10%

Dietary Fiber 3g Sugars 1g Protein 8g Vitamin A

0

5

Vitamin C

Calcium

1%

5

Iron

0% 10%

* Percent Daily Values are based on a 2,000 calorie diet. Your daily values may be higher or lower depending on your calorie needs: Calories Total Fat Less than Sat Fat Less than Cholesterol Less than Sodium Less than Total Carbohydrate Dietary Fiber 1g Fat = 9 calories 1g Carbohydrates = 4 calories 1 g Protein = 4 calories

2,000 65g 20g 300mg 2,400mg 300g 25g

2,500 80g 25g 300mg 2,400mg 375g 30g

Salt and uid 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 uid intake daily. Many with heart failure should try to keep their total sodium intake to less than a teaspoon of salt a day (2,000 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 try to drink no more than CUPSOZ OFmUIDSADAY&INDOUTFROMYOURDOCTORIFTHIS applies to you. If it does, keep up with all the uids 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 uid intake to keep from getting dehydrated. The right balance is also needed to help keep their blood from getting “too thickâ€?. 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 &LUIDINTAKE????????OZ per 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: s GOLFWITHACART s DANCINGSLOW s SWIMMINGALWAYSSWIMWITHAFRIEND s 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. 4ALKTOYOURDOCTORBEFORESTARTINGMORESTRENUOUS aerobic exercise or taking part in any competitive sports. 24

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: sHAVEANYCHESTPAIN sHAVEANYCHESTTIGHTNESS sFEELOUTOFBREATH sFEELLIKEYOUCOULDFAINT 25

M

ake good choices

)NTIMACYANDFAMILYISSUES 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.

4HINKINGABOUTRAISINGAFAMILY Bring your ďŹ ancĂŠ, spouse or partner to the clinic with you to listen and learn ďŹ rsthand 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.

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When either you or your spouse has CHD, there is, in GENERAL ATORISKTHATYOURBABYWILLHAVE#($"UT it may not be the same CHD you have. And certain types of CHD may have higher or lower risks. To ďŹ nd 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.

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 speciďŹ c questions about a birth control method.

M O D N C O

OM D N CO

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.

OM D N CO

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.

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0REGNANCY 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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RE

ER CHOIC

ES

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.

CA

Your career

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 health, 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.

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Health insurance and disability beneďŹ ts vary from country to COUNTRY3OMECOUNTRIESPROVIDECOVERAGETOALLCITIZENS EVENWITH pre-existing conditions. In the U.S., the Pre-Existing Condition Insurance Plan (PCIP) was created in 2010 for those unable to get insurance due to a pre-existing condition. s #ONTACTYOURSTATEINSURANCECOMMISSIONERSOFlCEORTHEWEB site: www.pcip.gov for more information. s -EDICAIDISINSURANCEFORTHOSEWHOAREDISABLEDANDHAVEALOW income. Apply for it at your local County Department of Family and Children’s Services. s K eep up with your healthcare follow up, no matter what your insurance status. Many specialty ACHD Centers have resources to help with your healthcare. s &ORSOMEHELPFULRESOURCES SEEPAGES  It is very important you continue your health insurance coverage. s'APSININSURANCECOVERAGEBEYONDDAYSMAYPREVENT continued coverage. s0#)0 MAKESITEASIERFORPEOPLEWITH#($TOGETINSURANCE after six months without coverage. The plan features reduced premiums that would be in line with the policy market rate in your state. Also, you no longer have to wait for a denial letter from the insurance company. A letter from your doctor within the last 12 months stating your medical condition or disability will qualify for PCIP. 30

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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!DVANCEDIRECTIVES An advance directive is a document that lets you make speciďŹ c 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). s !,IVING7ILL 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. s $URABLE(EALTHCARE0OWEROF!TTORNEY 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), WITNESSEDAND IFPOSSIBLE NOTARIZED

%ND OF LIFECONSIDERATIONS for implanted deďŹ brillator 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 stageâ€?, talk to your family. Choose whether you want the deďŹ brillator’s “shockâ€? function turned off. This information should also be part of your advance directives and any hospice and/or palliative care plan. 32

L

ooking 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. s ,EARNALLYOUCANABOUTYOURHEALTHCONDITION s &OLLOWAHEART HEALTHYDIET s 4AKEYOURMEDICINES s +EEPALLYOURFOLLOW UPAPPOINTMENTSANDWORK closely with your ACHD heart clinic. s 3TAYASACTIVEASYOUCAN s -AKEGOODCHOICESABOUTYOURCAREER YOUR insurance needs, your hobbies and your family. s 4AKEPARTINLOCALANDNATIONAL#($ORGANIZATIONS like the ACHA. 7HATISIMPORTANT"ALANCINGYOURCAREER heart condition, family and all other parts of your life!

34

A

ppendix

This appendix is divided into three parts. Part One gives you a diagram of a normal heart, labeled to show how blood ows 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. s .ORMAL(EARTAND"LOOD&LOW s -ODIlABLEHEARTDIAGRAM (used to show simple CHDs) s !ORTIC3TENOSIS s #ONGENITALLY#ORRECTED Transposition of the Great Arteries (CCTGA) s %BSTEIN!NOMALY s 3INGLE,EFT6ENTRICLE4RICUSPID Atresia & Double Inlet LV) s 3INGLE2IGHT6ENTRICLE(YPOPLASTIC Left Heart Syndrome) s 4ETRALOGYOF&ALLOT4/& s 4RANSPOSITIONOFTHE'REAT 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.

35

Normal Heart and Blood Flow The normal heart is divided into 4 chambers: s RIGHTATRIUM

Four valves act as one-way doors to direct blood flow:

s LEFTATRIUM

s AORTICVALVE

s RIGHTVENTRICLE

s MITRALVALVE

s LEFTVENTRICLE

s PULMONARYVALVE

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” (oxygenrich) blood from the lungs and pump it out of the heart to the rest of the body.

s TRICUSPIDVALVE 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

pulmonary veins from lungs

left atrium atrial septum

mitral valve

pulmonary valve right atrium left ventricle

tricuspid valve right ventricle

aortic valve

inferior vena cava

blood from lower body 36

ventricular septum

Other CHD: Your doctor can use this picture of a heart to explain your defect.

Comments:

0OTENTIAL,ONG TERM0ROBLEMS 0OTENTIAL ,ONG TERM 0ROBLEMS

&UTURE3URGICAL0ROCEDURES

0REGNANCY2ECOMMENDATIONS

37

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 38

tissue valves

Aortic Stenosis Comments:

0OTENTIAL,ONG TERM0ROBLEMS(Check those that apply to you.) 0OTENTIAL,ONG TERM0ROBLEMS RAortic root enlargement:

RValve problems:

RVentricular function:

RHypertension:

RCoronary disease:

&UTURE3URGICAL0ROCEDURES

0REGNANCY2ECOMMENDATIONS

39

Congenitally Corrected Transposition of the Great Arteries Comments: Malposed Great Arteries

0OTENTIAL,ONG TERM0ROBLEMS RArrhythmias:

Ventricular Inversion

RSystemic right ventricle failure:

0REGNANCY2ECOMMENDATIONS

40

Ebstein Anomaly Comments: Problem

hole between chambers

PDA

enlarged right atrium

0OTENTIAL,ONG TERM0ROBLEMS (Check those that apply to you.) RArrhythmias:

RValve insufďŹ ciency: malformed tricuspid valve

R26SIZEFUNCTION

thin right ventricle fenestration

Surgical Procedure (Extracardiac Fontan)

&UTURE3URGICAL0ROCEDURES

0REGNANCY2ECOMMENDATIONS

conduit (bypass tube)

inferior vena cava 41

Single Left Ventricle—Tricuspid Atresia tricuspid valve missing

Problem

Surgical Procedure

artery too narrow

(Classic Fontan)

hole between chambers

right chamber too small fenestration

Surgical Procedure

fenestration

(Extracardiac Fontan)

(Intracardiac Fontan)

artiďŹ cial wall 42

Surgical Procedure

conduit (bypass tube)

Single Left Ventricle—Tricuspid Atresia Comments:

0OTENTIAL,ONG TERM0ROBLEMS(Check those that apply to you.) 0OTENTIAL,ONG TERM0ROBLEMS RArrhythmias:

RVentricular failure:

RElevated venous pressure:

RLiver and/or kidney failure:

RVaricose veins:

RProtein losing enteropathy:

0REGNANCY2ECOMMENDATIONS

43

Single Left Ventricle — Double Inlet Left Ventricle Problem

Surgical Procedure (Extracardiac Fontan)

Surgical Procedure (Intracardiac Fontan)

Temporary Surgical Procedure pulmonary artery

(modiďŹ ed Blalock-Taussig Shunt)

subclavian artery

temporary shunt

44

Single Left Ventricle — Double Inlet Left Ventricle Comments:

0OTENTIAL,ONG TERM0ROBLEMS(Check those that apply to you.) 0OTENTIAL,ONG TERM0ROBLEMS RArrhythmias:

RVentricular failure:

RElevated venous pressure:

RLiver and/or kidney failure:

RVaricose veins:

RProtein losing enteropathy:

0REGNANCY2ECOMMENDATIONS

45

Single Right Ventricle— Hypoplastic Left Heart Syndrome Problem artery too narrow connecting blood vessel artery underdeveloped

hole between chambers superior vena cava

chamber too small

Surgical Procedure (Stage II–Bidirectional Glenn Shunt)

stitches

46

modified Blalock-Taussig Shunt (temporary)

Surgical Procedure (Stage I–Norwood Procedure)

new aorta

stitches

atrial septum removed

Surgical Procedure (Sano modification) tube (conduit)

Single Right Ventricle— Hypoplastic Left Heart Syndrome Comments: fenestration

Surgical Procedure (Stage III– Extracardiac Fontan Completion)

0OTENTIAL,ONG TERM0ROBLEMS RArrhythmias:

RVentricular failure:

RElevated venous pressure: conduit (bypass tube)

RLiver and/or kidney failure:

RVaricose veins:

RProtein losing enteropathy:

0REGNANCY2ECOMMENDATIONS

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

narrow opening stitches

enlarged

cut

Surgical Procedure

Surgical Procedure

(Complete repair of TOF with Homograft)

(Pulmonary valve replacement) tissue valves

tube

patch

conduit (bypass tube) 48

cut

mechanical valves

Tetralogy of Fallot with or without Pulmonary Atresia Comments:

0OTENTIAL,ONG TERM0ROBLEMS(Check those that apply to you.) 0OTENTIAL,ONG TERM0ROBLEMS RArrhythmias:

RPulmonary insufямБciency:

R2IGHTVENTRICLESIZEFUNCTION

RAorto-pulmonary collaterals:

&UTURESURGERY

0REGNANCY2ECOMMENDATIONS

49

Transposition of the Great Arteries Arterial Switch Repair Problem connecting blood vessel arteries go to wrong chambers

hole between chambers fenestration

Surgical Procedure (Arterial Switch) stitches arteries moved

patch coronary arteries moved

50

Transposition of the Great Arteries Arterial Switch Repair Comments:

0OTENTIAL,ONG TERM0ROBLEMS(Check those that apply to you.) 0OTENTIAL,ONG TERM0ROBLEMS RCoronary artery issues:

RAortic root stenosis or enlargement:

RPulmonary artery/valve issues:

RLiver and/or kidney failure:

0REGNANCY2ECOMMENDATIONS

51

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)

hole between chambers tied or cut

Surgical Procedure (Blue blood path)

bafe

52

bafe

Transposition of the Great Arteries Mustard/Senning Repair Comments:

0OTENTIAL,ONG TERM0ROBLEMS 0OTENTIAL,ONG TERM0ROBLEMS (Check those that apply to you.) RArrhythmias:

RSick sinus syndrome:

RSystemic right ventricle failure:

RBafe stenosis/leaks:

0REGNANCY2ECOMMENDATIONS

53

R

eferences and resources

s !DULT#ONGENITAL(EART!SSOCIATION!#(! http://www.achaheart.org/ 6757 Greene Street, Suite 335, Philadelphia, PA, 19119 0HONE  !#(!s&AX   The Adult Congenital Heart Association (ACHA) is a NONPROlTORGANIZATIONWHICHSEEKSTOIMPROVETHEQUALITY 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. s #ONGENITAL(EART)NFORMATION.ETWORK#(). http://tchin.org 101 North Washington Ave., Suite 1A, Margate City, NJ, 08402 0HONE  s&AX   Website has a page dedicated to ACHD cardiology services listed by state and some international links. An excellent question and answer transcript from a chat between parents and family members of those with ACHD. s !MERICANSWITH$ISABILITIES!CT http://www.ada.gov 0HONE  s  449 ADA Specialists are available to provide ADA information and answers to technical questions on Monday, Tuesday, Wednesday, and Friday from 10:30 a.m. until 4:30 p.m. or on Thursday from 12:30 p.m. until 4:30 p.m. (Eastern Time). s $ISABILITY2IGHTS3ECTION-AILING!DDRESS U.S. Department of Justice, 950 Pennsylvania Avenue, NW Civil Rights Division, Disability Rights Section - NYA Washington, D.C. 20530

54

s !MERICAN(EART!SSOCIATION www.heart.org 7272 Greenville Avenue Dallas, TX 75231 1-800-242-8721 Website offers information and links for Adults with Congenital Heart Disease. s 0RE %XISTING#ONDITION)NSURANCE0LAN www.pcip.gov and ACHA resource web page www.achaheart.org s 53$EPARTMENTOF,ABOR http://www.dol.gov Frances Perkins Building, 200 Constitution Avenue, NW, Washington, DC 20210 1-866-4-USA-DOL A lot of useful information on health plans & beneямБts. s 4HE(EALTH)NSURANCE0ORTABILITYAND!CCOUNTABILITY !CT()0!! &ACT3HEET http://www.dol.gov/ebsa/newsroom/fshipaa.html U.S. Department of Labor s 4HE)NTERNATIONALQ&OUNDATION)NC www.22q.org (A chromosome disorder that can lead to other health problems) s 3OCIAL3ECURITY!DMINISTRATION www.ssa.gov s .ATIONAL$OWN3YNDROME#ONGRESS http://www.ndsccenter.org s )NTERNATIONAL3OCIETYFOR!DULT#ONGENITAL Heart Disease http://www.isachd.org 55

Q

uestions 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

N

otes

57

A

uthor

4ERESA!,YLE !02. -. #0.0 Clinical Instructor/Lecturer University of Tennessee, Chattanooga Chattanooga, TN Nurse Practitioner Emory Adult Congenital Heart (EACH)Center Atlanta, GA

Special thanks to other reviewers: !MY#"URK!#($PATIENT 4ANYA$UTTON#($PARENT %STELLA-OORE,#37

Š 0RITCHETT(ULL!SSOCIATES )NC 3440 Oakcliff Road, NE, Suite 110 Atlanta, GA 30340-3006 1-800-241-4925

R

eviewers

Wendy M. Book, MD Associate Professor of Medicine (Cardiology) Emory University Medical Director Emory Adult Congenital Heart (EACH)Center Atlanta, GA -ICHAEL%-C#ONNELL -$ Associate Clinical Professor of Pediatrics (Cardiology) Emory University Emory Adult Congenital Heart (EACH)Center and Sibley Heart Center Atlanta, GA +ATHY-URPHY 2. -3. !02. Clinical Nurse Specialist and Pediatric Nurse Practitioner Cardiovascular Services Children’s Healthcare of Atlanta at Egleston Atlanta, GA 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 0AMELA$-INER 2. -. .0 Nurse Practitioner Ahmanson Adult Congenital Heart Disease Center University of California Los Angeles (UCLA) Los Angeles, CA


Balancing Your Life with a Mended Heart (588A)