Arrhythmias – Tachycardia Explained (Part Two)

Last week we discussed the issues that can cause the heart to beat too slow. This week, we look at what happens when the heart beats too fast. Carol Raimondi, completes part two of her resource on arrhythmias, this time covering tachycardia.




There are many types of tachycardias, but here we will focus on the most common tachycardias seen in congenital heart defects. When diagnosing these arrhythmias, the same tests as mentioned in part one are used to determine the arrhythmia. The only additional diagnostic test that is common with tachycardias, is an electrophysiology study (EP study). This test helps to confirm diagnosis and locate where an arrhythmia originates. It is done by a cardiologist who specializes in heart rhythm disorders in the EP lab. This is an invasive test where thin catheters are placed through the arm, leg and/or neck, then threaded through the blood vessels to the heart. The doctor can then use these to map the electrical conduction of the heart and reproduce arrhythmias in a controlled setting. This is very useful in planning for treatment.
Sinus Tachycardia
This refers to a heart rate that is more than 100 beats per minute.  Common reasons this occurs is due to a physiologic response to exercise, fever, dehydration,stress, pain, or stimulants, such as caffeine. When the factor that caused it is resolved, then generally, the heart rate returns to normal.
Atrial Fibrillation
A-Fib, as its often called, is the most common type of tachycardia. It occurs when, instead of the sinus node initiating the electrical conduction, there are many different impulses firing throughout the atrium. This results in a fast and very chaotic rhythm. Since it is so fast and irregular, the atria quiver or “fibrillate” and are unable to effectively squeeze the blood into the ventricles. A-Fib can come and go, or it can be continuous. Symptoms may include palpitations, lightheadedness, shortness of breath, fatigue or fainting, though some patients have no symptoms at all. The main concerns with atrial fibrillation is the increased risk of stroke and heart failure. As the atria are not emptying fully, blood can pool within the chamber and form blood clots.The clot can then travel to the brain and cause a stroke. Also, the irregularity of the rhythm makes the pumping action of the heart less efficient. If this continues for a long period of time, it can weaken the heart muscle, causing heart failure.
Atrial Flutter
Similar to A-Fib, atrial flutter occurs when the electrical conduction originates from an abnormal circuit in the atrium. With atrial flutter, there is still a very fast heart rate, but the rhythm is regular. It is not uncommon have atrial fibrillation in addition to atrial flutter. Like atrial fibrillation, the symptoms of atrial flutter can include palpitations, dizziness, fatigue, shortness of breath or fainting.
Supraventricular Tachycardia(SVT)
Supraventricular, or “above the ventricle”, tachycardia, is also referred to as atrial tachycardia, It occurs when there is an extra conduction pathway in the atria, usually present since birth, that creates a loop of overlapping signals. This arrhythmia is rarely life threatening. It is not uncommon for the atrial heart rate to get up to 300 beats per minute during an episode of SVT.  An episode may last for a few seconds up to a couple of hours. Symptoms of this include palpitations, feeling as though your heart is “racing”, lightheadedness, or shortness of breath. If the arrhythmia lasts for more then 20 minutes, it is recommended to go to the emergency room to receive medication to treat the irregular rhythm.
Ventricular Tachycardia
This refers to a very fast heart rate the comes from abnormal electrical signals in the ventricles, or lower chambers, of the heart. Since the heart rate is so fast, it does not allow adequate time for blood to fill into the ventricle, thus a much lower amount of blood is delivered to the body. These arrhythmias may last only a few seconds and not cause any issues. If it continues longer than that, it can be very serious and requires emergency treatment.  Symptoms are similar to the other tachycardias, but fainting can occur if it is sustained for more than several seconds.
Ventricular Fibrillation
This occurs when very fast, chaotic impulses in the ventricle cause it to quiver, or fibrillate. When this happens, blood can not  be pumped efficiently to the body. This is considered a life-threatening emergency if not treated within minutes and requires a defibrillator to “shock” the heart back into rhythm.


Treatment of tachycardia can be divided into two separate categories, prevention of arrhythmias and treatment during an arrhythmia.
Catheter Ablation- This is an invasive procedure that, like an EP study, involves insertion of small catheters through the arm, leg, and or neck, that are threaded through blood vessels into the heart. Once the abnormal circuit is found, the catheter uses either heat or extreme cold to damage the extra pathway, thus preventing it from allowing the arrhythmia to occur.
There is a class of medication know as anti-arrhythmics that doctors can prescribe to help prevent fast heart rates. These may be used in conjunction with other cardiac meds, such as beta- blockers or calcium channel blockers, to provide optimal coverage in keeping the heart rate and rhythm regular. Another class of medication that is used frequently for atrial fibrillation is anti-coagulants, or blood thinners. Warfarin, or Coumadin, is still the most commonly used type of blood thinner, but there are newer medicines that can be used as well. While blood thinners do not prevent atrial fibrillation, they help prevent blood clots from forming, thus reducing the risk of stroke associated with this arrhythmia.
When a fast heart rhythm, such as A-Fib, is not successfully prevented by medicine or catheter ablation, a pacemaker can be used to help control the arrhythmia. As discussed in part one, a pacemaker will watch the heart for abnormal rhythms, and then initiate the correct conduction when needed.
Implanted Cardioverter Defibrillator(ICD)
Similar to a pacemaker, this small device is surgically implanted under the skin. It has leads that are threaded through a vein into the heart. The ICD then “watches” the heart, and, if a dangerous arrhythmia such as ventricular tachycardia is detected, it will deliver electrical shocks to the heart to restore a normal rhythm. The device is programmed with specific parameters so that the device does not shock inadvertently. It sends data wirelessly to the physician via an in home monitor, and also requires semi-annual visits to the pacemaker/ICD clinic for a full interrogation.
Though rarely done for arrhythmia prevention alone, a surgical procedure can be done to block the abnormal pathways. Known commonly as a MAZE procedure, a number of small incisions are made in the heart tissue in a specific pattern. The scar tissue that forms from these incisions does not conduct electricity, so the abnormal pathway that was causing the arrhythmia is blocked. This procedure is usually done if the patient is having open heart surgery to treat another heart disorder.
Treatment During an Arrhythmia
There are a number of medications that can be administered intravenously to stop an arrhythmia while it is is happening. The type of medication will vary depending on the patient and the arrhythmia. There are also oral medications that can be taken at home to help control fast or irregular heart rhythms.
Vagal Manuevers
The vagus nerve is responsible for heart rate. There are certain maneuvers that affect this nerve, and can be used, with physicians instruction, to help lower a fast heart rate. Some of these include coughing, bearing down (as if having a bowel movement), and applying ice to the face and neck area.
Cardioversion can be done to help “reset” the conduction system of the heart. It may be performed as a scheduled procedure for someone who has had ongoing atrial fibrillation.This is done while the patient is sedated in a controlled setting with a lower amount of electricity. It also is used in emergency situations when ventricular tachycardia or ventricular fibrillation is present. This is done by placing paddles or patches on the chest. Then, a charged current is delivered that affects the electrical system of the heart and intends to restore a regular rhythm.
Carol Raimondi is an adult CHD patient and nurse, living with Congenitally Corrected Transposition of the Great Arteries.  She has had 4 open heart surgeries in her 40 years, as well as a pacemaker since the age of 6. After spending a large part of her childhood in and out of hospitals, she developed a passion for nursing. She went to school to become a cardiac nurse. Carol’s many hospital experiences helped her as a nurse to better understand what her patients were going through and  to care for them with that much more compassion and empathy.
Due to worsening medical issues, Carol had to give up the profession she loved. That did not stop her from being a patient advocate, however. She joined her local hospital’s’ Patient Family Advisory Council, which she now co-chairs, and shortly thereafter she joined Mended Little Hearts Chicago(MLHC) as an adult CHD liaison. She then expanded her work in the CHD community by starting an adult and teen CHD group within MLHC and became an  Ambassador for the Adult Congenital Heart Association. Currently she sits as the Pediatric Congenital Heart Association of IL(PCHA-IL)  President. Her proudest moments are when she is advocating and raising awareness for the CHD community, both on Capitol Hill in D.C. and locally.

The Biggest Gift

After  receiving a heart transplant, Megan Horton,  a Texas Children’s staffer shares how she celebrates the biggest gift she’s ever received . Happy Holidays, Everyone! 



Twelve years ago, a family lost their daughter. Twelve years ago, a 17-year-old lost her best friend. Twelve years ago, friends, family and loved ones had to say goodbye to a girl who passed away too soon.

Twelve years ago, I received the gift of life at Texas Children’s Hospital with a heart transplant. It’s always hard for me to celebrate my “heart birthday” each summer, when I know a family is grieving. The girl who donated her heart would have been 27 this year. I hope her family would find joy in all the things I’ve been able to accomplish by receiving the gift of their daughter’s heart.

In the past 12 years, I’ve accomplished so much. I graduated from high school and college, moved to a new city, landed my dream job, celebrated so many birthdays and anniversaries, and traveled to paradise.

I was only 14 years old when I received the greatest gift of my heart transplant, and while I’ve faced many challenges, I’ve always tried to have a positive outlook on life and remember that my life is a gift.

Each and every Christmas, no matter what presents are under the tree, my greatest gift is always the fact that I am there to celebrate with my family with a strong and joyful heart.

I’m very blessed that I received my transplant when I did. Every day, 22 people will pass away because they didn’t receive an organ in time. Please sign-up to be an organ donor and make your wishes known to your family. If you’d like to learn more about organ donation, please visit



Megan Horton is a heart recipient and the blog content manager for Texas Children’s Hospital.

Wellness – The Most Wonderful Time Of Year

The Holiday Season is meant to be filled with family and joy. It can also be a time of great stress, especially for families with chronically ill loved ones. In today’s post, Becky Hunt shares her experience with losing a child to CHD as well as managing her own illness, and explains how to de-stress from it all around the holidays.




Ah, December! It’s the most wonderful time of the year! Time for enjoying the festivities of the season! Exciting, right?! Well, for many of us, the next few weeks bring along added stress that can dampen our spirits and make the season a little less bright. Making travel plans, buying gifts, driving the kids to this party and that program, it’s non stop! Most “wonderful” time? Talk about most STRESSFUL time of the year!


Christmas was always my most favorite time of year! That was until my world came crashing down on me many times over.

My story starts with a little girl named Gracie. My baby girl. Gracie was born August 2nd, 2012 with Hypoplastic Left Heart Syndrome, or “half a heart”. She lit up my life for 82 days. The girl who changed MY heart inspired me to start a non-profit organization that creates dream cakes for kids with Congenital Heart Defects to brighten their lives. 5 years later that non-profit, Cakes From Grace, is thriving and growing and reaching more and more heart families by the day.

3 years after we lost Gracie I heard the 3 words that no one ever wants to hear, “YOU HAVE CANCER”.


Me? Haven’t I been through enough? Why me? Why now? I have CAKES to bake!

But the truth is, Cancer seemed to be nothing compared to losing your child or even seeing THEM suffer the way she did in the hospital for 82 days.

But I am here 2 years later, gone through several surgeries, 2 years of treatment and no Cancer in sight.

After Gracie passed away I dove right back into work. Started 5 different businesses (like one wasn’t enough). And just buried myself in it. At first work was my place to hide, to escape, it was a distraction, a place I could numb the feelings and avoid the hurt.

My obsession with work grew and soon it turned into an obsession with stress. I couldn’t escape. I felt like if I stopped then the world would crumble beneath me. Like I was letting people down. Constantly giving of myself, my services, my time, never saying no.

Christmas was always my most favorite time of the year. That was until we lost Gracie. I found myself angry that there wasn’t a spot at the table for her. No gifts under the tree for her. She wasn’t there to decorate the tree with me or sing our favorite Christmas songs I always sang to her.

The Holidays for me started to turn into a chore. The lists, the gifts I needed to find. My loved ones started to become a checklist. Ok, that person is done, 3 more to go, and 1 week to get it done before Christmas!

The endless amount of things on the Calendar. Christmas concerts, parties we were invited to, family get-togethers, gift shopping, UGH and the standing in lines! When am I gonna have the time to put up a Christmas tree?! 23 days of December just are NOT enough to fit it all in!

Everything constant, work, grief, the to-do lists all piling up!


Then how in the world DO you fit it all in? How in the world can you DE-stress?


1. Master The Art of Saying “No”

You don’t HAVE to attend every party you are invited to. Make a list of all the parties you and your kids were invited to, have them pick 1 or 2 of them to attend, not all of them.

2. Skip The Lines

You don’t have to go out and stand in those lines waiting to purchase the ONE thing you came here for! Shop online if that stresses you out! Put up your feet and cuddle up at home! Let the USPS guy bring it to you!

3.Take Time for You

Instead of giving your spouse a list of things you would like for Christmas, as a gift, ask him to take the kids out for a few hours while you read a book or watch a Christmas Movie and drink a glass of wine in a QUIET house. Ah, doesn’t THAT sound nice?!​​​​​​​​​​​​​​​​​​​

4. Write a priority list, NOT a to-do list!

Instead of freaking out over the things you need to get done, try to organize your list in order from what the top priority/needs to be done right NOW to what can wait for a bit.

5. Put the work DOWN

For some of us we can make our own hours, like myself. For others, you don’t have that luxury. But if you DO have the option, carve out at least 1 hour during your day to do something for you. Something that makes you happy. You’ll go back to work feeling refreshed.

6. Acknowledge Your Feelings

The holidays can bring up a whole bunch of emotions from sadness & loss to anger & frustration. It is OKAY. Just because it’s the ‘happiest time of the year’, does not exclude you from feeling those emotions. Forcing that “happy” on yourself can weigh you down even more.

​What I’ve learned is you can’t take care of your house, your kids, your to-dos if you don’t take care of YOU first.

Life is so darn short, kids grow fast and days grow faster.

I challenge you this season. Take more time for you and your loved ones. Maybe this is the year you start new traditions of making home made gifts and cookies and treats instead of spending the time to shop, spending that time away from laughter and joy. Or in my case, just BAKE cake!

Laugh more, live more this season. I DARE you!



Hi Friends! I’m Becky, Mama of the funnest (yes that’s a word in my world) 4 year old and the bravest Heart warrior who lives in Heaven.

I consider myself a lifestyle blogger with a focus on all the things I love and am most passionate about!

I’ve gone, done and experienced a lot in my adult life and I have a passion for sharing and serving. From losing my first daughter, Gracia, to a Congenital Heart Defect at just 82 days old, going through Cancer treatments and surgeries over the past 3 years to running 5 separate businesses as well as a non-profit organization just within the last 5 years.

And now closing a toxic chapter in my life and choosing to start over. Starting fresh and following my BIG SCARY dreams of becoming an author, writing a blog, being a life coach, videographer, continuing to grow my non-profit and putting myself out there in hope that I may inspire and help someone going through their own struggles, triumphs and hurts.

I’m a work-aholic and a stress-aholic on the recovery train to freedom! My mission is to be the best me I can be and to help other women find out what that means for them too.

Learn more about Becky:





Recap – Teen Topics

The 2017 Teen Topic Series posts are all here in one place. Take a look back on the important issues discussed!


Medical I.D.’s and Taking Ownership of Your Care

PCHA began its Teen Topics Series with a post about employing medical IDs as a useful tool. American Medical I.D.s, introduces IDs as one step toward  families helping their teens take ownership of their own care. Read further on the importance of involving teens in self -care and for a special offer .


Going Off to College

Going off to college is a major milestone in a young adult’s life. For many, it is the first time living away from home, from their parents, and from everything familiar to them. A chronic illness can complicate the transition. In this week’s blog, Abby Hack shares what it was like for her to gain her independence while managing atrioventricular block.


Preparing Your Child for Independence

We heard from Abby Hack on heading off to college. Now, we will hear from her mom, Janice. Watching your child leave home for the first time can be worrisome, especially with cardiac issues to consider. Janice shares with us how she has helped prepare Abby to take greater ownership of her own care.


Q&A: Your Questions Answered

in September 2017, patients and families submitted the questions they most wanted answered. We caught up with members of PCHA’s Medical Advisory Board, at the Transparency Summit, to ask those questions. Check out the videos, featuring Dr. Marino, Dr. Madsen, Dr. Gurvitz, and Dr. Sood’s answers on teen and young adult topics.


The Top 10 Things to Remember

As the seasons change, new milestones come and go. These can be especially trying times in the life of an adolescent with congenital heart disease, especially as they are undergoing the major transition of leaving the nest and going off to college, joining the workforce, or just moving far from home.  Dr. Aaron Kay, Director of the Adult Congenital Heart Disease Program at Indiana University Health, has the following Top Ten list to help ease the transition and cap off, for now, PCHA’s Teen Topic Series.


Arrhythmias – Bradycardia Explained (Part One)

This week former Cardiac Nurse, Carol Raimondi, provides us with the first of a two-part resource on arrhythmia and the various diagnosis and treatment. Up this week: Bradycardia.





Congenital heart defects are frequently accompanied by issues with the electrical conduction of the heart. These issues can cause a slow heart rate( bradycardia), a fast heart rate (tachycardia), or an irregular heart rhythm, also referred to as an arrhythmia. There may be one of these conduction defects present, or several at the same time.This can be due to the structural defects that were present at birth, or related to scar tissue that develops with each surgical intervention.  Fortunately, there are options to treat these irregularities.
Here we will be discussing the most common types of issues related to the electrical system of the heart. In order to discuss these abnormalities, first you should understand how things are supposed to work.
In normal conduction of the heart, the impulse is initiated in a group of specialized cells, called the sinus or sinoatrial (SA) node. This is located in the right atrium, or upper chamber, of the heart. Once initiated, is causes the both the right and left atria to contract. The signal then continues to the Atria-Ventricular (AV) Node. This is found between the upper and lower chambers. The signal slows slightly through here, allowing the blood to empty from the atria and enter the ventricles, or lower chambers. From there, the signal continues down through the lower chambers via the left and right Bundle of His, causing the ventricles to contract. Each time this cycle occurs, it results in one complete heart beat. When working properly, it should repeat this cycle without interruption 60-100 times per minute.
Sinus Bradycardia:
This describes a regular rhythm with a rate of less then 60 beats per minute. Sinus bradycardia is usually a benign finding. It is frequently seen in athletes or during sleep. There is generally no treatment for this unless the patient has symptoms, such as lightheadedness or fatigue. In such cases, further evaluation is done to determine an underlying cause, such as medication side effects or metabolic disorders.
First degree AV block:
This refers to a prolonged delay in conduction between the atria and ventricles. Generally, there are no symptoms associated with this, as the ventricle still does contract with every beat. No treatment is required for this type of heart block.
Second degree AV block:
Type 1- This is the less critical of the two types of second degree blocks and rarely requires treatment. In type 1, there is a progressive lengthening of the time it takes for the signal to travel from the sinus node to the AV node. With each contraction, it takes longer and longer until the ventricle “skips” a beat and resets the cycle.
Type 2-  In type 2 AV block, there is no specific pattern to when the signal is blocked from the atria to the ventricles. When this signal is blocked, the ventricles do not get the message to contract, and thus the blood is not pumped to the body. This often leads to third degree heart block. A pacemaker is implanted to treat type 2 AV block.
Third degree AV block:
Third degree, or complete heart block, refers to when the signal between the top and bottom chambers of the heart is absent. The ventricles use a “back up” electrical system, which produces a much slower heart rate then normal. The atria continue to contract as well, but there is complete dissociation between the top and bottom chamber, so the amount of blood being sent to the body is lessthen usual. Symptoms associated with complete heart block include lightheadedness and fainting. If left untreated, this block can cause death. This type of heart block  is critical and requires emergent treatment with a pacemaker. Often a temporary, external pacemaker is placed until the patient receives the permanent pacemaker.
Bundle Branch Blocks (BBB):
The Bundle branches are the fibers that carry the electrical signal from the AV node down through each ventricle. They are described as either the right or left bundle branch. When there is an interruption in this pathway, it is referred to as a bundle branch block(BBB). Generally, bundle branch blocks themselves do not cause any symptoms or require treatment. Further cardiac assessment should be done, however, to determine if there is an underlying cardiac condition. Right BBB is often seen in Congenital Heart Defects, especially in atrial or ventricular septal defects. Left BBB can frequently be seen in patients with coronary heart disease, or with weakening of the heart muscle.
The cardiac conduction irregularities listed above can be diagnosed in several ways. The most common is by performing a 12 lead electrocardiogram (ECG) This is a non-invasive test where patches, connected to leads, are placed on the skin. The technician ensures that the patient lays still for 15-30 seconds while a reading of the electrical signals through the heart is done. A printout of these readings can immeadiately show the heart rate, rhythm, and any damage to the muscle of the heart.
A holter monitor, similar to an ECG, involves applying electrodes to the skin in specific locations. Generally, there are 5 leads or less. These leads are connected to a small device that has recording capabilities. The patient can wear this device for 24-48 hours, depending on the physicians preference.  The device continuously records during the time that it is worn. Once returned, the ECG recordings are downloaded to a computer for a physician to review. The advantage to this test is that  with a prolonged recording period, there may be abnormalities detected that an ECG, which gives a “snapshot” can not detect.
An event monitor is similar to a holter monitor, in that it it is a small portable device that allows for prolonged recording of heart rate and rhythm. This device is usually worn for up to 30 days, and usually the patient can push a button on the device when he/she is experiencing any symptoms. Then they can call in to the device monitoring company and transmit the reading, which is reviewed and forwarded to the patients physician.
Treatment for Bradycardia and Heart Block
Of the above conduction issues, treatment is generally only required for second degree type 2 and third degree heart block. A small, electronic  device, known as a pacemaker, is used to restore normal conduction. This device is surgically implanted in the upper chest or abdomen, just under the skin, in a “pocket” the physician creates. Leads connected to the pacemaker are then threaded through a vein into the heart, where it is attached. The pacemaker “watches” the heart rhythm, and when it detects abnormal conduction, it sends an electrical signal to the heart. The pacemaker is programmed to not let the heart drop beneath a certain rate. Newer pacemakers transmit data wirelessly to their doctor or pacemaker clinic via a small device that can be kept near the patients bedside at home, This allows the physician office to monitor the functioning of the device without requiring the patient to call in, as was done in the past . Usually, every 6-12 months, an in-office pacemaker check is required to do full testing on the device, but this is quick and non-invasive. Pacemakers can last 7-10 years on average, depending on what percentage of time the pacemaker is in use.
Please join us next week for Part Two – Tachycardia.

Carol Raimondi is an adult CHD patient and nurse, living with Congenitally Corrected Transposition of the Great Arteries.  She has had 4 open heart surgeries in her 40 years, as well as a pacemaker since the age of 6. After spending a large part of her childhood in and out of hospitals, she developed a passion for nursing. She went to school to become a cardiac nurse. Carol’s many hospital experiences helped her as a nurse to better understand what her patients were going through and  to care for them with that much more compassion and empathy.

Due to worsening medical issues, Carol had to give up the profession she loved. That did not stop her from being a patient advocate, however. She joined her local hospital’s’ Patient Family Advisory Council, which she now co-chairs, and shortly thereafter she joined Mended Little Hearts Chicago(MLHC) as an adult CHD liaison. She then expanded her work in the CHD community by starting an adult and teen CHD group within MLHC and became an  Ambassador for the Adult Congenital Heart Association. Currently she sits as the Pediatric Congenital Heart Association of IL(PCHA-IL)  President. Her proudest moments are when she is advocating and raising awareness for the CHD community, both on Capitol Hill in D.C. and locally.

The Promise of Research for CHD, and Our Responsibility to Advocate

Advancement in standards of care and best practices can only happen with research.  This week, Margaret King discusses the implications increased CHD research has on not only the community but society as a whole, as well as how important it is for each one of us to contact our representatives to increase research funding. 



The Promise of Research for CHD, and Our Responsibility to Advocate


This past month, I and a group of several other local heart families were treated to an astonishing behind-the-scenes tour of the Mitchells’ research lab at Children’s Hospital of Wisconsin, where we learned about the vital work they are doing to identify the genetic processes, risks, and factors in CHD. Just a few weeks prior, I also had the opportunity to attend Mayo Clinic’s Feel the Beat event, where the HLHS Program shares research updates with heart families. Both of these research programs are committed to improving treatment of CHDs across the lifespan, with the further goal of exploring targeted treatments based on individual risk factors.


Many leading pediatric cardiology centers are working tirelessly behind the scenes to make game-changing breakthroughs for current and future CHD patients. From stem cells to genetics, new medical devices and drug therapies, and of course, developing best practices for everyday care and management, research underlies almost all aspects of CHD care. It has enormous implications for the quality of life and outcomes CHD patients will experience.


As members of the CHD community, we can advocate for lifesaving research funding to our representatives, as well as urge our friends, families, neighbors, and colleagues to do the same.


Research Breakthroughs: A ripple effect


With 1 in 100 babies being born with a heart defect, there is an urgent need for research breakthroughs in preventing and optimally treating CHD of all kinds. However, studying individual types of CHDs can have tremendous implications that extend far beyond CHDs themselves. For example, understanding the possible cascade of genetic events that causes hypoplastic left heart syndrome (HLHS) sheds light on the broader process of cascades that cause a number of complex diseases, many of which have stumped researchers for decades.


If we can offer anything from targeted stem cell therapy to genetically-tailored drugs for one disease, it is just a matter of time until these technologies can be used to treat a wider and wider range of illnesses.


These cutting-edge research discoveries have great potential to alleviate suffering, not just in the CHD community, but across entire our society. They offer hope that we truly can “conquer CHD” and many other conditions that have proved to be extremely challenging to treat and manage using the life-saving advancements of the twentieth century. While the breakthroughs of the past were revolutionary, we now know that we can do so much more if we put resources into the proper channels.


Advocating for Research: Our responsibility


Researchers and doctors cannot shoulder the burden of advocating for research alone. The more we help advocate, the more time they can spend on research and collaboration. As it is, researchers often spend a lot of time identifying avenues of funding and writing grant applications for scarce funds, which takes valuable time from their work in the lab.


Many of us understand the importance of advocating to our political representatives, but do we talk to our friends, families, neighbors, and colleagues about how important it is to fund public research through institutions like the National Institute of Health (NIH)?


The NIH is the largest source of biomedical research funding in the entire world, but the process is fiercely competitive, with less than 20% of applications being approved at any level of funding. The number of projects the NIH can fund, as well as what level of funding projects receive, fluctuates with the national budget.


Advocating not only for increased funding, but stability in the NIH budget from year to year, is of utmost importance to make sure the lights stay on in some of our most promising, dedicated labs. After all, when the lights stay on at the lab, researchers can shed light on life-and-death health problems that affect many of us personally, and all of us as a society.


Research takes an enormous amount of time, especially when dealing with pediatric populations and small pools of patients. Simply gaining approval for a clinical study is a complicated process, because researchers have to demonstrate their studies will not cause foreseeable harm to their subjects. With today’s advanced technologies, the studies we need in key areas like genetics and stem cell research are expensive, and can even face ethical and political hurdles. Many of them have several phases, each taking years to complete.


When scientific and medical studies of repute are finally completed, they must undergo peer review to withstand scrutiny from professional colleagues in their field. After that, usually further studies are needed, and even the most promising results need to be duplicated elsewhere before becoming mainstream practice. Each promising finding is simply a building block for further findings, hopefully leading to an eventual “big picture.”


Sharing Research with CHD Families: An institutional necessity


Touring the lab at Children’s Hospital of Wisconsin, and seeing firsthand how dedicated the researchers there are to helping CHD patients gave me new hope as a heart parent, as well as an inspiration to keep advocating for all the lives that will be touched by CHD. It gave me hope that there is either a cure or a radical shift in how we understand and treat CHD on the horizon.


Many heart parents rely on social media posts from other parents in order to learn about important research findings and the results of the latest studies. Many of us only hear about this vital work after the fact, and have little means of learning about the latest, cutting-edge discussions and studies that are happening at our own centers.


I commend Mayo Clinic’s Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome (HLHS) for their dedication to sharing their current research with families. Their blog and Facebook posts, as well, as their annual Feel the Beat gathering, which includes a science fair and demonstrations of their current projects for all ages, is refreshingly accessible. The biennial Heart Parent Education Day at the Medical College of Wisconsin/Herma Heart Institute also strives to inform heart parents about standards of care in pediatric cardiology, as well as their latest programs for patients.


I urge all pediatric cardiology institutions to get the word out about the great work they are doing–whether through a newsletter, blog, social media page, or in-person events–to the CHD community. Making in-person events family-friendly helps heart parents attend these events without the stress and expense of finding childcare. When heart parents are empowered not only with knowledge, but also the hope of such inspiring research, they are even more motivated to spread the word to their social and advocacy circles–which is a win/win for everyone.


*** Update***

Upon reading this article, one of our legislative champions reached out to us to share that this piece  “really helps underscore the Bilirakis-Schiff CHD approps letter for FY18 (attached)!  Give yourselves (and our CHD friends in Congress) some credit. These lawmakers are after all, #CHDWise 😉


This reminds us of the work our legislators are doing because of our advocacy efforts. Advocacy works!!!!

We’d like to thank the lawmakers who are already supporting increased CHD research funding, as highlighted in the letter, but we can’t stop!  We need to develop more legislative champions!! You can help by contacting your reps and letting them know you want them to support increased research funding. 

To learn more about CHD related legislation and how to contact Members of Congress from your state, check out PCHA’s Post on the Congenital Heart Futures Act




Margaret King is the mom to the 9- year old mighty K-man, a spirited boy with half a heart who is determined to live fully, and is married to the awesome heart dad, Shawn. A content marketer and writer in Wisconsin, her other interests include hiking and being outdoors, reading, and avoiding going down the thrill water slides her son is passionate about. She hopes to have a small goat farm someday

Alee Faith

Alee Faith was born on June 16th, 2016 .After some poor feeding issues and a sweating episode we ended up at UK on June 27th and we were given her diagnosis: Truncus Arteriosis with vsd. We were transferred to Cincinnati Children’s Hospital and Alee had open heart surgery on July 5th, at 19 days old. The surgeons placed her conduit and corrected the VSD during that particular procedure.
Alee then had a heart cath where 5 stents were placed in December of 2016. She will continue to have surgeries/procedures throughout her life. Life changed drastically for our family when our precious Alee came along. We have follow up visits with our cardiologist at UK every couple of months and we never really know what we are going to be told. The conduit that they placed doesn’t grow with Alee as she grows and gets bigger and in her particular case she has had moderate leaking with that conduit/valve pretty much ever since it was placed. We don’t know if/when her cardiologist is going to come in and inform us it is time to have ohs again, another heart cath, or that we just need to schedule another follow up visit in 3-6 months and everything is still looking the same for now.
We have to hope and pray for the best but at the same expect the worst with her Diagnosis. Organizations like PCHA provided our family with so much hope & support. Although it very well seemed that our world stopped spinning the night of June 27th, 2016,  we didn’t feel so alone with this having the support that we had. PCHA is by our side as an advocate for our heart warrior and a voice alongside our own that she so desperately needs. This Heart Journey continues for our daughter and all other heart warriors. They can never be fixed, only mended time and time again.

Cardiac Devices – The Elephant in the Room

With each CHD, there is no certain path, no one course looks like the next, and complications vary widely. Though a patient may have a specific structural diagnosis, different arrhythmias may develop over time, however patients and families are not always made aware of this possibility immediately. That’s why the diagnosis of an arrhythmia may surprise parents and send them for a loop, just when they think they’ve seen it all.  This also makes it difficult for parents to know just how much to share with teacher and other adults in their children’s lives, just as Alison Connors shares with us this week.

I’m often wondering what to tell people when they ask me about our children. It’s something most parents think about I’m sure. What details will help this teacher, this coach, this babysitter take care of my child? What can I share with them that will give them a view of who they are while they are in their care? For me there is always this looming question of what to say when discussing my children. On one hand I want them to treat my children as the unique individuals they are, discover new things about them, but on the other hand we do have this elephant in the room which is CHD.

I am a mother of three with two children who have faced Congenital Heart Defects. Like most Heart Parents it’s not something you plan, but it’s our life and we do a pretty good job at navigating C.H.D. It is always on my mind what and how much to share especially since two of my children have irregularities in their heart rhythms or arrhythmia.

Arrhythmia isn’t something I thought about when my children had their open heart surgeries. Actually, it was never mentioned when they told me about their hearts and never mentioned until few years ago. I think in the medical profession they are more of the approach of “we will get to that hurdle when and if we get to it.” Even the vague definition for Congenital Heart Defects is ‘problems with the heart’s structure present at birth’ that doesn’t really lead you to believe you may also have “electrical” issues as well or it didn’t in my non-doctor mind. CHD has so many forms and complexities that there isn’t a one size fits all explanation or treatment, so I see their hesitation to lay it all out there. Unfortunately that makes CHD affecting your family a situation of having to roll with the punches of what is going to happen, or at least that is how I have felt.

About two years ago, we were told our daughter had 1st degree heart block. After 4 years of seemingly good appointments, it flung me back into that intense worrying state of mind that we were in for her first year of her life.1st degree heart block, once again something was affecting my child that I had never even heard of before. I remember thinking, “ok, block doesn’t sound good, especially when describing my daughter’s unique heart.” “Why wasn’t this mentioned as a possibility? Or was it, is that the same thing as a murmur? Maybe they mentioned it? No, I would have remembered that.” We were told not to worry, but of course one of my favorite quotes is ‘telling a mother not to worry is like telling water not to get wet.’ I remember crying in the bathroom after we got home from the appointment. Although I knew CHD was lifelong I felt like we were ahead of it or at least had it under control. Arrhythmia seemed like the scariest effect of her surgery. I may or may not have googled how much an AED costs.

With this new information we did what we could and I quickly learned that we did not in fact need our own AED. We did our research and we asked questions. It turns out that arrhythmia is pretty common among some patients who are affected by CHD. That was reassuring for some reason, at least she wasn’t the only one and her pediatric cardiologist was very familiar with this. We followed up about Holter monitor results and asked what to symptoms to look for in the future. We were told that pacemakers would most likely be in her future, but time would tell on when. We were met with some ifs and buts since, unfortunately, our cardiologist is not a fortune teller. Wouldn’t that be nice though? Note to self: google physic pediatric cardiologist. Just kidding. Fast forward just 6 months later and our youngest and second heart warrior was too experiencing irregular heart rhythms. This time, I was more prepared and less shocked. Of course his arrhythmia is not the same as our daughters, but arrhythmia was now on our radar so to speak. Isn’t it weird how things like this become your new normal? Now we had a new discussion point when it came to our kids and how to approach this new element of their health concerns added a new layer to who our kids are.


Thinking about my children at school and how to explain CHD and arrhythmia to their teachers worried us. I would go back and forth on how much to disclose at their schools. I wanted to keep them safe of course, but I didn’t want them to be known for just one thing, that being just their special hearts. We decided to air on the side of caution and give specific details about our children’s hearts to their teachers and school nurse. I am so glad we did because our daughter’s school nurse kept referring to it as congestive heart failure. Yikes, right? While we were worried what to share, our kids were busy telling everyone about their hearts and showing off their Holter monitors. They decided to take the lead and give full disclosure to anyone they met. We were surprised, proud and kind of felt silly. We worried what to say about the “elephant in the room” and how to say it, but when it came down to it, it didn’t matter. To their teachers, they were still just two happy kids in their school and their friends think it’s cool they had heart surgery. At this moment, we have things figured out, but of course life has a way of happening while we’re busy pretending we know what we’re doing and planning.


Two of our children are affected by CHD and have irregular heart rhythms. So where does this lead us? Like most Heart Parents we just don’t know what our children will experience next. This CHD journey isn’t written in stone and we will always have looming questions on what will happen and how to handle social situations well into adulthood. Will I one day have a heart warrior grandchild? Whoa, ok, slow down, but it’s already on my radar. We just have to stay tuned because with CHD we just don’t know exactly what path we will take. At least we no longer see CHD as an elephant in the room.





Alison Connors is a mother of three children: McKenzie, Jackson and Archer. Her oldest McKenzie and youngest Archer both had open heart surgery for congenital heart defects. McKenzie and Archer have been in the care of the PSHU team at Advocate Children’s Hospital in Oak Lawn, Illinois since birth. Alison has been married since 2009 to her best friend Christopher, and she recently went back to work as a 1:1 teacher’s aide for children with special needs. She and her family have a busy life, but she has a passion for volunteering and believes that there is healing power in taking part in something that is bigger than yourself. Having two children with CHD threw Alison onto a path she never expected to be on, but a path her family is very grateful to be on. It’s an honor for Alison and her family to share resources, give hope, and support to others who face the same situations that they have.