Words of Support for New Heart Moms

​​​We know the fear, the sadness, the devastation, and the anger you have felt since your child was diagnosed with a congenital heart defect (CHD). We know you are looking for answers, for information, for anything that will help you understand this and get you through what is ahead for you. How do we know this? Because we have been there, too. 

Heart Mom to Heart Mom: You are not alone on this journey!

  • Don’t compare your child to “heart-healthy” children. If you want to see if your child is meeting milestones, ask your child’s doctors what is “normal” for a child with his or her heart defect―not what is normal for a heart-healthy child your child’s age.
  • You choose who to listen to, take advice from, and rely on during your journey. Not everyone will understand what you are going though. The ones who do, who want to understand how to help you, will be integral parts of your support system.
  • Don’t be afraid to ask questions. If you feel like something is wrong, don’t be afraid to ask your child’s healthcare team. Everyone has unique strengths and knowledge. For example, if you are having trouble with insurance coverage, turn to your pediatrician. He or she can gather the right information and advocate on your child’s behalf—a good thing to keep in mind!
  • Don’t be afraid to get a second opinion. Good and humble doctors will welcome a second opinion.
  • Become an expert and an advocate. Knowledge is power. Immerse yourself into learning as much as you can about your child’s diagnosis, medication, and treatment plan. Remember to avoid “internet overload” by sticking to reputable websites like Don’t forget to ask your child’s doctors where he or she recommends going to learn more. You truly are your child’s best advocate. Often, mothers will notice or sense things that others cannot—our maternal instincts are usually right.
  • Ask for help if you need it. It’s often hard for moms to ask for help―we want to feel like we can do it all. But, you don’t need to be superwoman! Make a list of things people can help you with, and the next time someone asks, let them do one of the things on the list.
  • Take some time for yourself. You are still a person and need time to recharge. When your child is in the hospital, we know this is especially tough to do. But, you have the most expensive babysitters your child will ever have at your disposal—take a break! Get rest when you can, drink lots of water, and go for a walk once a day. Getting outside of those hospital walls―even for a couple of minutes―will help you feel better.
  • Stay positive. Don’t let others’ opinions of the situation dictate your emotional well-being. People may say or do offensive things. Learn to let them roll; they just don’t know any better and most of the time it’s innocent ignorance.

YOU CAN DO THIS!  You are stronger than you think!


Screening and Treating Kids for High Blood Pressure: AAP Report Explained

​We usually think of high blood pressure—also known as hypertension—as a problem that affects adults. But, in fact, the condition can be present at any age.

An estimated 3.5% of all children and teens in the United States have high blood pressure, however, the condition often goes undetected and untreated.

Early Detection is Key

If high blood pressure isn’t identified at a young age, it could go undiagnosed for years—eventually leading to organ damage and other health problems, like coronary artery disease, in adulthood.

The American Academy of Pediatrics (AAP) report, Clinical Practice Guidelines for the Screening and Management of High Blood Pressure in Children and Adolescents, gives pediatricians a simpler screening table to follow that identifies blood pressures needing further evaluation.

How blood pressure is measured?

The term blood pressure actually refers to two separate measurements:

  • Systolic blood pressure is the highest pressure reached in the arteries as the heart pumps blood out for circulation through the body.
  • Diastolic blood pressure is the much lower pressure that occurs in the arteries when the heart relaxes to take blood in between beats.

If either or both measurements are above the range found in healthy people of the same age and sex, it’s called hypertension.

Screening Kids for High Blood Pressure 

It’s a difficult diagnosis in kids since blood pressure can be affected by many factors, such as height, age and sex. The AAP and the National Heart, Lung and Blood Institute both recommend that children have yearly screenings for high blood pressure, starting at age 3, at their annual well-child visits.

  • If your child’s blood pressure is high, he or she will typically be asked to come back a week later to see if the high readings persist.
  • If your child has elevated readings in three consecutive visits, he or she should have an evaluation for the cause of hypertension and risk of organ injury.

Infants who were preterm, or of low birth weight, who had a difficult or long hospital stay may need blood pressure screenings sooner than age 3. Children who have congenital heart disease, who are receiving medications that might increase blood pressure, or who have other medical conditions may also be at a greater risk of high blood pressure.

Tip for parents: Remember to ask your pediatrician if he or she measured your child’s blood pressure, and if so, if it was a concerning reading.

What are the Causes?

High blood pressure in children is almost always asymptomatic—that is, without any symptoms or noticeable discomfort.

In many cases, high blood pressure seems to develop with age. As a result, a child may show no signs of high blood pressure as an infant, but may develop the condition as he or she grows.

By age 7, more than 50% of hypertension is due to obesity; this rises to 85-95% by the teenage years. Thus, good eating habits (without overeating and emphasizing foods low in sodium, low in added sugar and high in fruits and vegetables) and plenty of physical activity are important throughout the early years of childhood (and for the rest of life).

When high blood pressure becomes severe in children, it may be a symptom of another serious problem, such as kidney disease or abnormalities of the heart or of the nervous or endocrine (gland) system.

Diagnosing High Blood Pressure in Children​

If your child’s blood pressure is high, your pediatrician may recommend tests to see if there is an underlying medical problem causing it. These tests include studies of the urine and blood. Sometimes, ultrasounds are used to examine the heart or the kidneys. If no medical problem can be found, your child will be diagnosed with essential hypertension—the word essential refers only to the fact that no cause could be found.

About essential hypertension:

In most children and adolescents, no specific cause is found for hypertension. Doctors take into account the child’s medical history (other diagnoses like kidney disease), family history (heritability of hypertension is about 50%),  risk factors such as low weight at birth, and whether the child is currently overweight.

Management & Treatment 

The first-line treatment for high blood pressure in children remains lifestyle changes.

  • If obesity is a possible cause, the first step will be to have your child lose weight. This will need to be very closely monitored by your pediatrician. Not only will weight loss lower blood pressure, it can provide many other health benefits as well.
  • Limit the salt in your child’s diet. Giving up the use of table salt and restricting salty foods can lower blood pressure in some patients. Common sources of salty foods include bread, deli meats, pizza, and foods prepared outside the home.   
  • Use caution when shopping for packaged foods. Most canned and processed foods contain a great deal of salt, so check labels carefully to make sure the items have little or no salt added.
  • Help your child get more aerobic exercise. Aerobic physical activity can lower blood pressure, because of its effect on blood vessels and the heart—even when patients do not lose weight. The AAP recommends 60 minutes of physical activity each day.

About blood pressure-lowering medications:

If lifestyle changes do not work, or if the blood pressure is very high, a more aggressive approach is needed. The AAP guidelines recommend pediatricians start blood pressure-lowering medications if lifestyle changes fail to reduce your child’s blood pressure, or your child has another condition like diabetes or kidney disease.

There are a variety of anti-hypertensive medications that have been tested and proven to be safe in the short- and long-term. The number of children who will end up with prescriptions for anti-hypertensive medications is small, about 1% or less.

Long-term care:

Once your pediatrician knows your child has high blood pressure, he or she will want to check it frequently to make sure the hypertension is not becoming more severe. Depending on how high the blood pressure is, your pediatrician may refer your child to a specialist—usually a pediatric nephrologist (kidney specialist) or pediatric cardiologist (heart specialist).


Reproductive Health in Young Women with Congenital Heart Defects

​​​A congenital heart defect (CHD) is the most common type of birth defect. Due to advances in medical and surgical therapies, more than 90% of infants born with a CHD will live to see their 18th birthday.

As dreams of a full and rich adult life—including a career and family—frequently become reality, adolescents and adults should receive specialized cardiology care throughout their lifetime. The impact of a CHD and its interaction with typical conditions of adulthood such as pregnancy, diabetes, and hypertension must be considered as women living with CHD age.

​​​”Young women living with a CHD can expect better outcomes if they partner with their health care team when making health decisions, especially when it comes to reproductive health,” said Elyse Foster, M.D., a cardiologist at the University of California, San Francisco, and a member of the Congenital Heart Public Health Consortium (CHP​HC).

Anyone who has a heart murmur or had heart surgery as a child should be evaluated by a congenital cardiologist as an adult.

Women living with a CHD, specifically, can make informed choices and decrease their chances of a high-risk birth when they work with their health care team. While the overall risk of complications during pregnancy is relatively low, women with a CHD are still eight times as likely to experience cardiovascular complications during pregnancy. 

Reproductive health considerations should start even before a young woman is ready to start a family. These topics should include contraception, the decision to carry a pregnancy, and the challenges of parenting with a CHD. See the articles below for more information on each subject.

  • Birth Control for Young Women with a CHD
  • Preconception Counseling for Women with a CHD
  • Planning a Healthy Pregnancy with a CHD
  • Genetics and CHDs
  • Parenting with a CHD: Why Prioritizing Your Own Health Is Important

​”Guidelines and recommendations for care are available for the management of adult patients with congenital heart defects,” said Michelle Gurvitz, M.D., a cardiologist at Boston Children’s Hospital and a member of the CHPHC. “However, even with the availability of this information, too great a number of patients fall through the cracks in the system.” Dr. Gurvitz advocates for a team-based approach to care for her adult patients, including congenital cardiology, primary care and other specialties as needed—including obstetrics, gynecology and psychology.


Pulmonary Hypertension in Infants & Children

Pulmonary Hypertension in Infants and Children

Pulmonary hypertension is high blood pressure in the arteries of the lungs. It is a rare, progressive disease affecting people of all ages.

How pulmonary hypertension affects infants and children: 

Children are either born with narrowing in the lung arteries or the arteries become thicker and narrower over time. The narrowing in the arteries causes increased pressure. The right ventricle pumps blood to the lungs to receive oxygen. In pulmonary arterial hypertension, a type of pulmonary hypertension, the right ventricle needs to pump against the increased pressure in the lung arteries. This can lead to heart problems over time, especially with the right ventricle. The walls of the right ventricle can become much thicker than normal, and the function of the right ventricle can decrease over time.

Pulmonary hypertension in newborns:

Persistent pulmonary hypertension of the newborn (PPHN) is the term used when babies are born with the disease. Normal transitions in circulation that should occur after birth do not, and this can lead to symptoms such as fast breathing, difficulty breathing, and/or low oxygen saturation.

Pulmonary hypertension in children:

Pulmonary hypertension can develop in children due to an unknown cause (idiopathic PH) or due to another medical problem including congenital heart or lung disease.

Symptoms of pulmonary hypertension:

Symptoms of pulmonary hypertension depend on the exact cause, and a complete work-up is needed to figure out what that underlying cause is.

In children, there can be a range of symptoms including:

  • Fatigue with activity
  • Shortness of breath with activity
  • Chest pain
  • Fainting, particularly with activity
  • Swelling of the legs or ankles
  • Dizziness

What type of doctor treats children with pulmonary hypertension?

If your pediatrician is concerned about your child’s symptoms, you may be referred to a pediatric pulmonary hypertension specialist for an evaluation―usually a pediatric cardiologist or pulmonologist with extensive training in pulmonary hypertension who treats many children with the condition.

Diagnosing pulmonary hypertension:

To confirm the diagnosis, your child’s doctors will review the results of a physical exam and various tests taken at the appointment.

Examples of tests include:

  • Chest x-ray
  • Electrocardiogram (tracing of the electrical activity in the heart)
  • Echocardiogram (ultrasound of the heart)
  • Six-minute walk test or cardiopulmonary exercise testing (to understand how the body responds to exercise)
  • Ventilation-perfusion lung scintigraphy (looks at the circulation of blood and air in the lungs)

More advanced imaging:

  • Chest/cardiac computed tomography (CT) (uses radiation to look at the lungs and blood vessels)
  • Chest/cardiac magnetic resonance imaging (MRI) (uses magnetic fields to look at the lungs and blood vessels)
  • Cardiac catheterization (procedure to measure the pressure in the blood vessels in the lungs)
  • Lung biopsy (invasive procedure to get a small amount of lung tissue for testing)

Treatments for pulmonary hypertension:

Whenever possible, it is important to identify and treat the underlying problem. If your child has a heart valve problem that is causing the pulmonary hypertension, for example, then treatment would first involve repair to the valve.

There are many medications used to decrease the pressure in the lung blood vessels that improve the quality of life for infants and children with pulmonary hypertension. Warfarin (Coumadin), digoxin, and vasodilators, such as nifedipine, intravenous prostacyclin, or both are examples. These medications can be taken by mouth, inhaled into the lungs, or given through an IV into the blood vessels. Everyone responds differently to treatment, so it is important to talk with your child’s doctors to determine what is right for your child.

Outlook for children with this disease:

There is no cure for pulmonary hypertension, and some children eventually require lung or heart-lung transplants. However, new treatments are available to help prevent the disease from progressing and new research is being done all the time to hopefully result in more options.  


Preconception Counseling for Women with a Congenital Heart Defect

​​Even though pregnancy and childbirth stress the heart, most women with a congenital heart defect (CHD) are able to have healthy pregnancies if they choose to do so.

Women with a CHD interested in getting pregnant should see a health care professional who specializes in CHDs before becoming pregnant to determine any risks for complications during pregnancy.

Preconception counseling—as it is called—can identify any heart problems that may need treatment before conception to assess a baby’s risk of being born with a CHD. 

Consult with a Specialist:

If you have a CHD and are considering becoming pregnant, you should schedule an appointment with a congenital cardiologist. You will need to tell the congenital cardiologist if you have any cardiac symptoms, such as:

  • Shortness of breath
  • Fatigue
  • Chest pain
  • Dizziness
  • Heart palpitations (i.e. a feeling like your heart is fluttering or beating too fast or too hard) 

Bring a list of all of your medications to a preconception counseling appointment for review. Some medications should not be taken during pregnancy. 

Cardiac Testing Prior to Conception:

Your doctor will perform special medical tests​​ to see if your heart can handle the increased demands of pregnancy. At a minimum, you will receive an echocardiogram—a type of ultrasound that allows doctors to see a video of the heart.

Depending on your symptoms and echocardiogram results, your doctor may perform other tests such as:

  • Holter monitoring: Provides a constant reading of your heartbeat so your doctor can see if you have an unusual heart rhythm.
  • Exercise test: Shows your doctor how your heart handles stress when it needs to work harder.
  • Cardiac MRI: Another type of imaging test that can very accurately show heart size and how well the heart is working. 

About catheterization:

Occasionally, pre-conception ​​test results show that a woman needs a surgery or a catheterization—a nonsurgical way to treat the heart. Catheterization is done by inserting a thin tube into a blood vessel in the neck, arm or leg and threading it to the heart.

Your doctor may recommend performing surgery or catheterization before pregnancy to treat damage to a heart valve, narrowing of a vessel, or an aneurysm.

Determining Risks for Pregnancy Complications:

Your congenital cardiologist will give you a “risk assessment” on your likelihood to have cardiac complications during pregnancy. In some cases, your doctor may say your risk of having a dangerous pregnancy is very high. In this situation, discussions with your doctors can help you and your partner better understand these risks as well as consider other options. 

Preconception Counseling with Other Specialists:

Ask your congenital cardiologist for help finding other medical specialists who can help you make decisions about pregnancy and delivery.

  • Maternal-Fetal-Medicine Specialist: Also called a “high-risk obstetrician” and has experience caring for women with a CHD. 
  • Genetic Counselor: Reviews your medical history and may perform testing to determine the baby’s risk for having a CHD. In general, a baby whose mother or father has a CHD has a small increased chance (about 5% on average) of being born with a CHD, too.
  • Anesthesiologist: Can talk to you about anesthesia options for labor and delivery. Most women with a CHD can have an epidural during labor and delivery. Vaginal delivery is usually preferred over a cesarean section for most women with a CHD. There may be some special monitoring and accommodations made during your labor and delivery, but all medical care is geared toward the safety of you and your baby.
  • Social Worker: Can help counsel you and your partner on how to handle the stress of managing both your cardiac and pregnancy health.


Parenting can be hard. It is important to talk to your congenital cardiologist about how a CHD will affect your health after delivery, too.


Postural Orthostatic Tachycardia Syndrome (POTS)

Postural Orthostatic Tachycardia Syndrome (POTS) is a condition caused by a problem with the way the nerves regulate blood flow. As a result, enough blood doesn’t go to the right place at the right time.

For example, when a child or teen with POTS stands up (a change in posture), the blood vessels in the legs and feet are overly relaxed and allow pooling of blood in the veins there. This, in turn, decreases the blood flow back to the heart and brain. This can cause dizziness and blurred vision.

To make up for the lack of blood flow, the heart beats much faster (tachycardia). With POTS, there is usually an increase in heart rate by 30-40 points from lying to standing or a standing heart rate of greater than 120 bpm.POTS often starts around the time of puberty but can develop at any age. About 75-85% of cases are in females. POTS may improve or even disappear in 80% in most people by the time they are in their early 20s. Symptoms sometimes begin after a viral illness, major surgery, or trauma. They often are worse in the morning, making it difficult to get ready for school.

What causes POTS?

POTS is thought to be a disorder of the autonomic nervous system, the part of the nervous system that controls heart rate, blood pressure, movement of food through the gut, sweating, and many other functions. Other factors that can contribute to POTS include dehydration, poor fitness, anxiety, and/or hypervigilance (being much more sensitive to feeling changes in the body).

Symptoms of POTS

POTS causes a variety of symptoms with the heart and blood vessels, including:

  • Palpitations (the sensation of rapid changes in heart rate or racing heartbeat)
  • Chest discomfort
  • Dizziness, lightheadedness, or fainting (syncope)
  • Exercise intolerance–feeling too fatigued for reasonable physical activity
  • Extreme tiredness

Because the autonomic nervous system has such a wide impact throughout the body, other symptoms of POTS may include:

  • Gastrointestinal (GI) symptoms like nausea, bloating, cramping, vomiting, diarrhea, or constipation
  • Muscle and/or joint pain
  • Trouble sleeping (insomnia)
  • Headaches, especially migraines
  • Blurred vision
  • Fuzzy thinking, poor concentration or “brain fog”
  • Anxiety and/or depression
  • Cold extremities with reddish/purplish coloration of the feet
  • Heavy, painful and prolonged menstrual periods

Diagnosing POTS

A thorough history and physical exam are the keys to help your child’s doctor make the diagnosis of POTS. They may also recommend some tests, such as:

  • Orthostatic vital sign measurements including the Active Stand Test are very helpful: the child’s heart rate and blood pressure are measured first while lying down, then sitting, and then while standing for 2, 5 and 10 minutes. If the patient is < 19 years old, the test is abnormal if the heart rate rises more than 40 beats above the resting rate by 10 minutes of standing (more than 30 beats is the cut off if older than 19 years). A drop in blood pressure with standing occurs less frequently. The Active Stand Test may bring on the symptoms of POTS.
  • Electrocardiogram (ECG) is used to rule out other heart problems that can cause racing heart beats.

Other tests that are sometimes needed include:

  • Blood tests: thyroid function tests, complete blood counts
  • 24 Hour Holter monitor: this device records all the heart beats for 24 hours so the heart rate and rhythm can be examined
  • Echocardiogram: an ultrasound of the heart to look at the heart’s structure and function

A team of specialists may be involved in managing this multi-system disorder. It may include cardiologists, neurologists, gastroenterologists, therapists, psychiatrists, rheumatologists, or endocrinologists.

Treatment for POTS

There is no perfect treatment for POTS. Treatment is individualized to each child’s specific symptoms, and recommendations may include:

  • Increasing water intake to at least 60-80 ounces per day (depending upon the child’s size) and an increase in the amount of salt in the diet. Together, these help to retain fluid and fill up the overly relaxed blood vessels.
  • Getting lots of sleep- up to 10 hours a night.
  • Sleeping with the head of the bed mildly raised. Over time, this decreases morning symptoms of dizziness as the nervous system is retrained.
  • Exercising. Gentle workouts at first and gradually increasing to moderate exercise daily. To start, exercises need to be those where the child is not upright, like swimming, recumbent biking, or rowing. Improving leg and core strength improves blood return from the legs. Exercise also improves fitness and energy, and helps to retrain the nervous system over time.
  • Eating a healthy diet and avoiding skipping meals. Frequent, small meals can be helpful.
  • Wearing compression stockings.
  • Avoiding caffeine.

Are there medications for POTS?

Medications are typically considered only for those patients who still have significant symptoms even after trying the above changes in hydration, sleep and exercise. Referral to a pediatric cardiologist or neurologist with expertise in POTS is recommended if medications are needed. A variety of different medications are possible, and must be tailored to the individual child’s symptoms. Medications are used to together with, not as a substitute for, the treatments above.

Other types of care for POTS

Because POTS may last for years and can include flare-ups of symptoms, it is easy to become frustrated, anxious, stressed and even depressed. Many families benefit from counseling to help deal with this chronic disorder. Children may require special care plans at school.


Planning a Healthy Pregnancy with a Congenital Heart Defect

​​Women with a congenital heart defect (CHD) who, after preconception counseling, are able to have a safe or low-risk pregnancy, can take additional steps to plan for a healthy pregnancy.   

Your Medical Care Team:

Be sure your medical care team has knowledge and experience in treating pregnant women with a CHD. The specific members of this team may differ depending on where you live and what kind of heart condition you have.

  • Congenital Cardiologist: In particular, one who specializes in adults with CHDs.
  • Maternal-Fetal-Medicine Specialist: Also called a “high-risk obstetrician” and has experience caring for women with a CHD. 
  • Genetic Counselor: Reviews your medical history and may perform testing to discuss the baby’s risk for having a CHD. In general, a baby whose mother or father has a CHD has a small increased chance (about 5%) of being born with a CHD, too.
  • Anesthesiologist: Can talk to you about anesthesia options for labor and delivery. Most women with a CHD can have an epidural during labor and delivery. Vaginal delivery is usually preferred over a caesarean section for most women with a CHD. There may be some special monitoring and accommodations made during your labor and delivery, but all medical care is geared toward the safety of you and your baby.
  • Social Worker: Can help counsel you and your partner on how to handle the stress of managing both your cardiac and pregnancy health.

During Your Pregnancy:

It is very important to maintain regular care with your obstetrician and congenital cardiologist throughout your pregnancy. Although most women will do well and have healthy pregnancies, women with a CHD do have a higher risk for pregnancy complications, including arrhythmias or irregular heartbeats and premature birth.

Your congenital cardiologist ​will determine how often you will require cardiac care during pregnancy. Most women with a CHD see their congenital cardiologist once each trimester, but sometimes more frequent appointments are needed. Your doctor will also determine whether you need cardiac testing during your pregnancy. Most women with a CHD receive an echocardiogram (ultrasound of the heart) early in pregnancy and in the last trimester.

As with any pregnant woman, there are ways you can help reduce health risks to your baby before and during pregnancy:

  • Take a daily prenatal vitamin that includes folate.
  • Avoid smoking, alcohol and other drugs (other than medications prescribed by your doctor).
  • Eat a healthy diet. 
  • Exercise.
  • Get enough rest.

Labor & Delivery:

At some point, you will discuss the type of delivery—vaginal or cesarean section—with your obstetrician. Typically, a vaginal delivery is preferred over a cesarean section for most women with a CHD. There may be some special monitoring and accommodations made during your labor and delivery, but all medical care is geared toward the safety of you and your baby.

Regardless of the type of delivery, an anesthesiologist is usually involved to assist with pain control. Most women with a CHD can have an epidural during labor and delivery, but some types of medicine for pain control can affect the heart rate and blood pressure—why meeting with an anesthesiologist before delivery is important!

Your Baby’s Health:

Your baby is at increased risk of being born with a CHD if you or your baby’s father have a CHD. Preconception counseling can also assess your baby’s CHD risk.

Once you’re expecting, your doctor will do a special ultrasound of your baby’s heart, called a fetal echocardiogram, to see if your baby has any major structural issues to the heart.  


Parenting with a Congenital Heart Defect: Why Prioritizing Your Own Health is Important

​​​If you are a parent who lives with a congenital heart defect (CHD), it can be challenging to balance the day-to-day demands of being a mom or dad while living with and caring for your own health.

Who to Put First?

It may be difficult some days to figure out who to put first – your children or yourself. When it is hard to find the energy to even get out of bed, it is important to take care of your own physical and emotional needs first. As a parent, this may not sound “right,” but the best gift you can give your child is to also care for yourself.

A CHD can be unpredictable, as can your child’s needs. Plan ahead.

  • Ask for help. When a CHD overrules parenthood, call friends, family members or a sitter to help. Parenting changes with older children, but you still need to be okay with reaching out to others.
  • Find time to relax. Whether visiting with friends, seeing a movie, meditating, or just reading, it is essential to make relaxation a priority.
  • Lower your expectations. Your parenting style may be different from your sister’s or your friend’s, but that does not mean you are not a good parent. Lowering your expectations will help make meeting the challenges of parenting with a CHD seem easier.

Planning for the Future

Every person with a CHD should have a living will. It not only notifies your family members about your wishes, but it also guides them in decision making if you are unable to speak for yourself. 

Once you have a living will, be sure to:

  • Store it in a safe place in your home and tell your family members where it is located. This is not a document to keep in the bank safety deposit box. It should be located in a secure but easily accessible spot in your home.
  • Talk to your family about the instructions included in your living will. Family members are more accepting of a loved one’s wishes if they are aware of what those are ahead of time.  
  • Bring it to the hospital if you are admitted. Living wills are now required by hospitals so be sure to bring it with you if you are being admitted to the hospital.

Kawasaki Disease in Infants and Young Children

​​​​​​​​Kawasaki disease is the leading cause of acquired heart disease in infants and young children in the United States. More than 4,200 U.S. children are diagnosed with Kawasaki disease each year.

The condition causes inflammation in the blood vessels, and the symptoms can be severe. In addition to several days of fever, children with Kawasaki disease may develop symptoms such as rash, swollen neck glands,  swollen hands and feet, and red eyes, lips and tongue. 

Early on, Kawasaki disease can affect the function of the heart muscle or the heart valves. If it is recognized and treated early, children can begin to feel better in a few days with a low likelihood of long-term heart issues.

Fast Facts:

  • 80% to 90% of Kawasaki disease cases occur in children under age 5 and older than 6 months. It is less common for older children and adolescents to get the disease, but some do. 
  • Kawasaki disease is not contagious. It does not spread among family members or children in child care centers.
  • Kawasaki disease occurs more frequently in children of Asian or Pacific Island ancestry. However, it can affect people in all racial and ethnic groups.
  • The cause of Kawasaki disease is not known, but it is thought to be a reaction by the body’s immune system.

Signs & Symptoms:  

Kawasaki Disease begins with a fever above 102 degrees F that lasts for at least five days. Other signs and symptoms may include:

  • Rash anywhere on the body but more severe in the diaper area.
  • Red, bloodshot eyes without pus, drainage, or crusting.
  • Swelling and tenderness of a gland (lymph node) on one side of the neck.
  • Swollen hands and feet with redness on the palms of the hands and the soles of the feet.
  • Very red, swollen, and cracked lips; strawberry-like tongue with rough, red spots.
  • Significant irritability and fussiness.
  • Peeling fingers and toes (typically 2 to 3 weeks after the beginning of fever).

Note: The following key signs and symptoms may not be present at the same time. In some very young infants, only a few of these actually develop. Other non-specific symptoms may also be present, such as vomiting, diarrhea, stomachache, cough, runny nose, headache, or pain or swelling of the joints. 

Signs & Symptoms: Kawasaki Disease

When to call the doctor:

If your child has a fever for 4 to 5 days with any of the key signs and symptoms above, ask your doctor whether he or she could have Kawasaki disease. It can be challenging to diagnose, so your child may have to be examined several times.  

There is no specific, single test to diagnose Kawasaki disease. If Kawasaki disease is suspected, however, your doctor may order tests to monitor heart function (an echocardiogram) and may take blood and urine samples. In addition, your doctor may refer you to a pediatric specialist in infectious disease, rheumatology or cardiology for more guidance in diagnosis and treatment. 

Complications if left untreated:


If Kawasaki disease is left untreated, it can lead to serious complications such as inflammation of the blood vessels. This can be particularly dangerous because it can affect the coronary arteries–the blood vessels that supply blood to the heart muscle–causing coronary artery aneurysms to develop. An aneurysm is a ballooning out of a damaged and weakened blood vessel wall. 

Fortunately, treatment within the first 10 days of illness significantly decreases the risk of aneurysms. That’s why it is very important to diagnose Kawasaki disease by the 10th day of the illness. Treatment should begin as soon as possible. 

Treatment for Kawasaki Disease:

Children diagnosed with Kawasaki disease are admitted to the hospital. The medication used to treat Kawasaki disease in the hospital is called intravenous gamma globulin (IVIG). IVIG is given through a vein over 8 to 12 hours. Children stay in the hospital for at least 24 hours after completing the IVIG dose to make sure the fever does not return and other symptoms are improving.

Children may also be given aspirin to lower the risk of heart problems. (Aspirin should only be given to young children under doctors’ supervision, as it can cause a serious liver condition called Reye Syndrome.)​

Additional treatments may be needed if a child does not respond well to the single dose of IVIG, the fever returns, or there are abnormal findings on the first echocardiogram. Another dose of IVIG or other medications that fight inflammation such as steroids, infliximab, or etanercept may be recommended. Pediatric specialists in rheumatology, infectious disease, or cardiology may be consulted in this situation.

What to expect after hospital discharge:

Children treated for Kawasaki Disease are sent home from the hospital on a low dose of aspirin to take by mouth every day for 6 to 8 weeks. As they recover, it is not uncommon for these children to be extra tired or seem “off” for several weeks. Rest is very important. Peeling of skin the hands and feet is expected and is not cause for alarm.

Call your doctor right away if your child develops a fever or any of the other symptoms of Kawasaki Disease return. Further evaluation will be needed to determine if your child needs to be go back to the hospital.

Follow-up Care:

It is very important to closely monitor children who have had Kawasaki Disease to make sure they are improving and to check for the development of coronary aneurysms. Aneurysms most often form after the first couple of weeks of illness, so children should be scheduled for an echocardiogram and a check-up at 2 weeks and again at 6 to 8 weeks after their fever first started. More frequent follow up and echocardiograms will be necessary if there are abnormal findings on any of the echocardiograms.

Note: Live viral vaccines should be postponed at least 11 months after IVIG, because IVIG can cause the vaccines to be ineffective. These include the MMR (measles, mumps, rubella) and Varicella (chicken pox) vaccines. Children over 6 months of age should receive the inactivated influenza (flu) vaccine injection.

Long-term follow-up:

Children treated for Kawasaki disease who do not develop aneurysms have an excellent long-term outcome. However, it is important for them to follow a heart healthy diet and lifestyle. Cholesterol levels should be checked every 5 years.

Children who do have coronary aneurysms should be under the care of a pediatric cardiologist as they grow and will require special long-term care and follow-up.


Helping Children With Congenital Heart Disease Stay Healthy, Active and Fit

Congenital heart disease is the term for defects that affect the heart that are present at birth (congenital). If your child has congenital heart disease, this means there’s a problem with their heart’s structure or the way it works.

There are many different types of congenital heart disease. Some are simple and don’t need to be treated. Others are more complex and may need medication or surgery.

Why congenital heart disease puts kids at increased risk for obesity

Children who have congenital heart disease are at a higher risk of having overweight and obesity. There are several reasons for this, including:

  1. Needing more calories in early life: Babies with congenital heart disease tend to have a hard time gaining weight, since they burn more energy breathing faster and trying to eat. To help them grow, they’re given high-calorie formula or supplements. But after surgery or over time as their child’s symptoms get better, parents often keep using the same higher-calorie feedings.
  2. Having common risk factors: Studies show that certain groups of kids are more at risk for obesity, including those who:
  • have a body mass index (BMI) between the 85th and 95th percentiles
  • have a family history of obesity in one or both parents
  • gain weight faster than they grow in height at a young age OR whose BMI increased quickly before age 7 years old
  • have excessive weight gain in adolescence
  • were very active and have become inactive
  • are inactive in general, especially adolescents

Many kids with congenital heart disease fall into one or more of these groups.

  1. Exercise restrictions: In the past, doctors used to limit physical activity for kids and teens with congenital heart disease. Unfortunately, kids who begin an inactive lifestyle are more likely to stay that way. Now experts know that exercise and active play has many benefits and it’s safe for most children with congenital heart disease. Even when it’s doctor-approved, parents may worry about how exercise will affect their child. It can also be hard for kids who were told at first to limit their activity to get moving.

Maintaining a healthy weight

Your pediatrician will monitor your child’s weight to make sure they’re staying in a healthy range. If your child is gaining weight too fast, your doctor will talk to you about making some lifestyle changes. This includes changes in diet, exercise, and reducing inactivity.

Eating a healthy diet

Dietary recommendations for kids and teens with CHD are the same as for any child. Keep these guidelines in mind when you plan meals.

  • Increase your child’s dietary fiber to at least 14 g for every 1000 calories eaten. Foods that contain fiber include:
    • Fresh fruits and vegetables
    • Whole-grain foods such as brown rice, whole-grain pasta, corn, peas, and breads
  • Decrease your child’s intake of dietary fat to less than 30% of their daily calorie intake. There are some simple ways to cut fat such as:
    • Choose lower-fat or fat-free toppings like grated low-fat Parmesan cheese, salsa, herbed cottage cheese, nonfat/low-fat gravy, low-fat sour cream, low-fat salad dressing, or yogurt.
    • Select lean meats such as skinless chicken and turkey, fish, lean beef cuts (round, sirloin, chuck, loin, and lean ground beef with no more than 15% fat content) and lean pork cuts (tenderloin, chops, ham). Buy “choice” or “select” grades of beef rather than “prime.” Trim off all visible fat. Remove skin from cooked poultry before eating.
    • Include healthy oils such as canola or olive oil in your child’s diet. Choose vegetable oils without trans fats made from canola, corn, sunflower, soybean, or olive oils.
    • Use nonstick vegetable sprays when cooking.
    • Stick to fat-free cooking methods such as baking, broiling, grilling, poaching, or steaming when cooking meat, poultry, or fish.
  • Limit cholesterol to less than 300 mg daily. That means limiting full fat dairy, red and processed meat, fried foods, and baked goods.
  • Limit sodium to less than 2500 mg daily.
  • Eliminate or limit sugar-sweetened beverages to less than 4 ounces per day. Encourage your child to drink water instead.
  • Get familiar with actual serving sizes. For example, one piece of bread is one serving, not two (even though we use two for sandwiches).
  • Use the Nutrition Facts label on food packages to find foods with less saturated fat per serving. Pay attention to the serving size as you make choices. Remember that the percent daily values on food labels are based on portion sizes and calorie levels for adults. You’ll need to adjust for your child.

Staying active

All kids and teens, including those with congenital heart disease, should regularly participate in physical activity or sports. Medical professionals recommend that children do 60 minutes of physically active play or sports per day, every day.

Regular physical activity helps prevent weight gain and early-onset adult heart disease such as heart attacks and strokes. Many studies show that exercise can also help prevent diabetes, high blood pressure and high cholesterol. Most children with congenital heart disease live well into adult life. But when you have congenital heart disease, your heart may be more easily damaged by obesity, high blood pressure and diabetes. This is why regular exercise is so important for everyone with congenital heart disease.The majority of children with congenital heart disease don’t need physical activity restrictions. However, some do. If your child is taking blood thinners or they have fragile implantable devices, abnormal heart rhythms, depressed heart function or decreased blood flow to the heart during exercise, their doctor may recommend restricting certain activities.

Your pediatric cardiologist can advise you on the best physical activities for your child at your visits, even if activity restrictions are needed. Be sure to check with your cardiologist before your child starts a new sport or exercise routine.

How to make activity a priority

Daily physical activity is essential for everyone, including kids and teens with congenital heart disease. Here’s how you can improve your child’s activity levels:

  • Make sure your child gets at least an hour of moderate to vigorous activity every day. They should engage in vigorous activity at least 3 days a week.
  • Limit total screen time (computer, video game, TV, tablet, smart phone, etc.) to no more than 2 hours of quality programming per day.
  • Keep TVs, electronics and screens out of your child’s bedroom.
  • Support the recommendations for daily physical education at your child’s school.

Create an active lifestyle

In today’s busy world, it’s harder and harder for children to play and run outside on a daily basis. That makes organized sports and exercise activities good options. Let your child participate in choosing an activity or sport to try. If they enjoy it, they’ll want to keep doing it.

Planning activities with friends can also encourage your child to stay active. Team sports are a great way to get exercise with friends. Getting family members involved can go a long way too. Take family walks, hikes or bike rides. Do a workout video or stretches together. Put on a fun dance video game. Try family bowling, roller skating, or laser tag.

Wearable step counters or heart rate monitors are another way to make exercise fun. Most devices allow you to set goals. This encourages more physical activity. Your child may be inspired to see how many steps they’ve walked in a day or how long their heart was elevated during a workout.

Remember that sports and exercise should be fun. Kids will be more likely to get exercise or participate in sports that they enjoy. The goal is to build a life-long habit of regular exercise. Forcing exercise that your child doesn’t like or doesn’t find rewarding won’t last. Be creative and find ways to get out there and have fun with exercise.