ARVD/C Information Home Page

Most who arrive at this website will have generally had some type of introduction to ARVD or ARVD/C. The acronym is not often heard, nor are the words Arrhythmogenic Right Ventricular Dysplasia (ARVD) or Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) — so, if you are here, chances are you have already learned a little about this Sudden Death heart disease.

If you are looking for an introduction to ARVD/C from a medical standpoint (through the eyes of doctors, scientists and researchers), our "Resources for Patients" page provides many links to that type of information. If you are looking for an introduction to ARVD/C from the viewpoint of someone personally affected by this diagnosis, someone who knows many others who are, this page contains it.

Author: Patricia Miranda
Contributing Author: Micheline Long
Contributing Editors: Patricia O'Neil and Leslie Munson
© Copyright Patricia Miranda and Micheline Long 2006
 

You have been diagnosed with Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy, a rare heart muscle disease. I want you to know that you are not alone. If you are newly diagnosed, you are not alone. If you are struggling with the ups and downs, you are not alone. If you are looking for a little information, help and hope, you have come to the right place.

The "Resources for Patients" page at this site has plenty of links to internet resources for medical and clinical data on ARVD/C. I urge you to visit those sites and take in all that you possibly can.

In this column you will find the words of a fellow patient. While I, myself, still have a lot to learn, I can take what I have seen and heard in a collective sense, and spill it all out here for you.

This is the ARVD/C experience from a patient's perspective. A straight forward approach to help you get past the initial shock of having a "heart condition," through the ups and downs, and on to living the happiest, fullest, most meaningful life that you can.

I was diagnosed with ARVD/C in 2001. I have lived, and witnessed through others, the ARVD/C patient experience. I have been a member of the International ARVD Family Support Network since 2003. I have communicated with other ARVD patients through over 12,464 group email exchanges, as well as seminars, and phone calls.

Receiving this diagnosis can be unsettling and overwhelming. Any heart condition can conjure up scary thoughts and strong emotions, but a heart condition you have never heard of before can be particularly disturbing. At first, many of us are left reeling, blindsided by our diagnosis. Thoughts of your own mortality invariably creep in. A feeling of loss may ensue. You may feel a loss of health, stability and all that you thought was normal.

Speaking of normal, the word normal can become a foreign concept. What could possibly be normal about a life that includes a rare heart disease, crazy unpredictable symptoms, and most likely your own personal jumper cables implanted in your chest?

Well, there is a little something called a "new normal." Your "new normal" is what you arrive at once you figure out how you are going to deal with all of this heart stuff, and still get on with participating in and enjoying life. It may not look like anything anyone without ARVD/C would call normal, but it will work for you. Like your favorite pair of jeans, you just need to break it in, get comfortable with it.

For me, and many ARVD/C patients I know, learning everything we can about ARVD/C, arrhythmias, and ICDs has gone a long way towards bringing about our "new normal." This knowledge has helped bring about acceptance, and has helped us arrive at a place where we can better cope with whatever might come next.

ARVD/C is an illness of varying degrees. No matter where you fall on the ARVD/C scale, there are things you can do, things you can learn that can greatly improve the quality of your life. I would like to talk about some of the things you might have to deal with and give you some tips and coping skills that have helped other ARVD/C patients.

 
DIAGNOSIS

A definite diagnosis of ARVD/C is made when someone meets the Task Force Criteria for diagnosis (ref: "How is a diagnosis of ARVD/C determined? ") However, some of us fall just short of fulfilling the criteria requirements, but to the experts we are deemed most likely to have ARVD/C. Our diagnosis falls in the "probable" ARVD/C category. I am a "probable."

Due to the emergent circumstances that often surround the need to immediately protect a person from Sudden Death; certain non-invasive tests may not be performed first. These tests might have been helpful in proving the existence of a certain criterion for the diagnosis of ARVD/C. Unfortunately, later on, there may be reasons why the test cannot be used to do so. For example, it would be dangerous and unreasonable to cease a medication just to get an accurate reading on the amount of PVCs a person has in 24 hours. An MRI, which might have been helpful in proving a criterion, might not be doable with an implanted ICD. Some people do not fulfill the diagnostic criteria due to the fact that they are in the early stages of ARVD/C. As a result, a person may very well have ARVD/C, but it cannot be proven by the diagnostic criteria, thus they are a "probable" ARVD/C.

A quote from the article Genetics of Right Ventricular Cardiomyopathy (J Cardiovasc Electrophysiol, Vol. 16, pp. 927-935, August 2005; Sen-Chowdhry S, Syrris P, McKenna WJ) explains the difficulty of diagnosing "early" or "concealed stage" ARVD/C.

 
ARRHYTHMIAS

Arrhythmias are a major symptom of ARVD/C and a top concern for ARVD/C patients. Those abnormal heart rhythms are quick to grab your attention. Some ARVD/C patients have only a few incidents of arrhythmia. For some, their arrhythmias occur frequently; others are heart stable and have the occasional Hot Phase or Ventricular Tachycardia (VT) Storm, which are times when their hearts are particularly unstable. Everyone is different when it comes to instability.

An electrophysiologist (EP) is a cardiologist who is trained to deal with the electrical workings of your heart. Since arrhythmias stem from an electrical problem, the EP is your go-to doc for those abnormal heart rhythms.

Abnormal heart rhythms can be as mild as a fluttering feeling in your chest, or as bold as the feeling of a lone tennis shoe knocking about in the dryer with major thu-thumping going on. Some people do not feel their arrhythmias.

One thing is for sure, once you have experienced those crazy heart rhythms you would like to avoid them in the future, if at all possible. Well, it is not always possible. Arrhythmias have a way of showing up when they want. Quite a few patients have reported that certain things can act as a trigger for some of their arrhythmias. By taking note of the possible triggers for their arrhythmias, many patients have learned that they can avoid at least some of their abnormal rhythms.

Possible arrhythmia triggers that have been mentioned by ARVD/C patients include: fatigue, overeating, alcohol, dehydration, extreme temperatures, illness – flu, fever, colds, infections, certain foods/additives, caffeine, MSG (monosodium glutamate), sweets, cold and flu meds containing pseudoephedrine, cigarette smoke filled rooms, epinephrine in the numbing agent used by the dentist, exercise — especially strenuous or competitive, and STRESS — stress instigates arrhythmia — arrhythmia instigates stress — it can be a vicious cycle. You can read more about triggers in this document.

Some of the other symptoms of ARVD/C, such as syncope (fainting/passing out) and dizziness or lightheadedness can be a direct result of the arrhythmias. Not everyone will have every symptom.

 
MEDICATIONS

There are medicines that are given for the management and treatment of arrhythmias. Many of us are on a combination of medicines. While it is safe to say that none of us want to have to take pills, once the right combination and dosage is found, these medicines can provide welcomed relief.

It can really be a challenge to stay on track with your medicines, when to take them, and keeping up with any changes or adjustments your doctor may suggest. Pill boxes, using a timer or watch alarm, sticking to a routine, anything that can help you avoid the confusion of, "Did I or didn't I take my pill?" is going to be helpful. Whatever specific directions come with a medicine, or are given to you by your doctor, make sure you follow them. Stopping some heart medications suddenly can actually lead to arrhythmias and/or an actual heart attack. Never stop taking a medicine without the advice of your doctor.

All medications come with their own risks for side effects. Talk with your doctor about this. Sometimes you must switch to a different medicine, but other times lowering a dose, or slowing down the rate a dose is increased, will help ease the side effects. Some side effects taper off after you have been on the medicine for awhile.

Even when receiving the best of treatment, arrhythmias can "breakthrough." If this happens to you, talk with your doctor. Your doctor may be able to give you helpful suggestions or he might want to adjust your medications.

 
MINERALS – HERBAL MEDICINES – OVER THE COUNTER MEDICINES

Some patients have gotten additional help for their arrhythmias by taking minerals prescribed by their doctor. I am talking about Potassium and Magnesium, in particular. Too much or too little of either in your system is not good. Your doctor can do a simple blood test to check your levels.

Then there are herbal medicines. ARVD/C is a serious heart condition and most of us are taking serious heart medicines. Herbal medicines, like heart medicines, contain chemicals. Throwing them into the mix can be very dangerous. How might they affect your heart? How might they interact with your heart medications? Talk with your doctor before trying any herbal medicine.

In fact, it is a good idea to check with your doctor before taking any Over-The-Counter (OTC) medications to make sure they will not aggravate your condition or interact with any prescribed medicine you might be taking. Did you know that certain cold and flu medicines contain pseudoephedrine which can instigate arrhythmias? Keep in mind that your condition may be remaining stable due to a fine tuning of treatments. OTC meds that you took in the past, and that seemed harmless and beneficial, may no longer be good for you. Get professional advice — educate yourself before using OTC medicines.

 
AICD'S OR ICD'S

Many ARVD/C patients will be offered and receive an Automatic Implantable Cardioverter-Defibrillator (AICD or ICD). ICDs are amazing, life-saving devices. If your heart goes into a dangerous arrhythmia the ICD will deliver treatments, such as the painless anti-tachycardia pacing (ATP), or the shock, in order to get your heart back into a normal rhythm.

When people receive an ICD often their first thought is, "What are the things I can not do now that I have an ICD?" They often feel as if the ICD is going to greatly limit their life. The ICD does limit certain things, you will be warned against standing beside a high voltage arc welder, for example, and you can find other such warnings in your ICD booklet or on your ICD manufacturer's website. For many, these limits are not life changing. At the most, they are usually just a small inconvenience. The ICD is there to free you. It is there to free you from the threat of Sudden Death.

Some people never receive a shock, while others receive an occasional shock. Some people might receive more than one shock in a short period of time. Some people may receive inappropriate shocks, which are shocks that should not have occurred for one reason or another. After a shock, doctors typically check your ICD. Depending upon what they learn, some doctors may make a change or adjustment to device settings and/or medications. For some, maintaining stability can be quite the balancing act.

How does the shock from an ICD feel? Well, it has been described in many colorful ways. Being hit on the back by a basketball, a baseball, a soccer ball, a 2x4, being punched in the chest, an explosion in the heart, and my personal favorite — like a garage door spring coming loose or breaking inside your chest.

A shock is not painful to everyone; many only feel the "force" of it. Even for those who do feel pain, the pain is gone as quickly as the shock — in the blink of an eye. It really does come and go that quickly.

You may want to ask your doctor about the protocol that you should follow in the event that you do receive a shock.

I have had one shock. It hurt like blazes, and was over in a split second. But, long after the sensation of the shock had disappeared, well, there was a lingering of strong emotions. There was a fear and dread of the next shock. I needed time to process the event.

After a shock, some people are able to have a "pick yourself up, dust yourself off, and start all over again" type of attitude and that is fantastic. That is definitely what I will aim for next time, if there is a next time. However, I think that many of us fall somewhere in the, "give me some time to deal with it", kind of haze. And still for others, it takes longer to sort it all out.

There are things you can try to help you get through the anxiety. Learning and using relaxation techniques has proven invaluable to those in the ARVD/C patient community. There are times when people recognize a distinct need to 'quickly settle down,' or work their way through and out of anxiety.

In the "Patient Library" there is a document titled "Breathe.htm." This document introduces you to a Diaphragmatic Breathing Technique taught by many counselors. This technique is simple to learn and has helped numerous ARVD/C patients with tension and anxiety related to their ICDs, arrhythmias, and all life stressors. To receive the greatest benefit from this technique, people are advised to practice it well and become an expert at it before it is really needed.

Take advantage of the experience of a large community of people with ARVD/C. For relaxation they suggest changing your thoughts and thinking of a favorite vacation, a happy event, old jokes. Some use meditation to help calm their mind. You can watch a favorite movie — comedies are great picker-uppers, call a friend or family member, start a new home or craft project. Support groups can be the very best places to turn to during this time. There is the International ARVD Family Support Network. The ZAPPER, Implantable.com and "ICD Support Group" are websites which provide message boards for the support of those with ICDs.

Adjusting to having an ICD can be a bit like a baby adjusting to having a belly button. One day you will realize that you went the whole day without once thinking about your ICD. Each day from then on will be better.

If the aftermath of an ICD shock prevents you from living your life to the fullest, interferes with your daily routine and/or has greatly affected your mood, speak with your doctor. Some ICD patients have been helped by taking prescribed medications for anxiety, though they try to steer clear of addictive medications or those which might interfere with their heart medications.

If you think you are in need of professional help, it might be a good idea to look for a mental health professional who deals with other ICD patients and/or one who deals with Post Traumatic Stress Disorder (PTSD).

This is not about being tough. Forget about that. Allow yourself the time to deal with this in whatever way is best for you. There is nothing about coping with ICDs or ARVD/C that is "one size fits all."

 
ABLATIONS

For most patients, their arrhythmias respond to treatment with medicines and an ICD, however, for those with arrhythmias that do not respond, there is a procedure called a Cardiac Ablation. The Heart Rhythm Society (HRS) has a great page that describes this procedure.

I have never had an ablation. I have communicated with many who have had one, and even some who have had many ablations. Ablation is typically used to treat areas of the heart which are responsible for starting arrhythmias, such as VT. Apparently, in the ARVD/C patient, there can be many of these areas. Additionally, more can occur over time due to the progressive nature of the disease.

I know some patients whose VT was not relieved by meds and who subsequently received ablations. These patients were very thankful for the relief they received through their ablation. On the other hand, I know some patients who were given ablations and feel that ablations may have exacerbated their problems.

An ablation is an irreversible treatment. If the topic of ablations comes up between you and your doctor, you might want to ask questions and voice any concerns you may have. The more information and answers you have, the better prepared you will be to make whatever decision is best for you. Some questions I might ask my doctor are: "How long has the doctor who will be performing my ablations been doing them?" "How many patients have they ablated?" "Were the ablations considered to be successful?" "What is the definition of successful?" "Does this doctor use the most up-to-date cardiac mapping equipment and catheters?"

 
HEART FAILURE

It appears that there are a percentage of ARVD/C patients who will progress toward Congestive Heart Failure (CHF) over the course of living with their disease. This information is coming to light as ARVD/C receives more research. Fortunately, people with ARVD/C are being spared Sudden Death and are living longer with their disease. As more people live through the course of this disease, its evolution is better understood. As it becomes understood, people can be protected from some of its possible outcomes.

CHF occurs when the heart cannot pump efficiently enough to meet all of the body's needs. Possible heart failure symptoms include swelling of the legs, hands, feet and abdomen, extreme fatigue, and shortness of breath, especially with exertion or when lying down. A sudden weight gain can be a sign of CHF.

Please take my advice on this one, if you begin experiencing any of the above mentioned symptoms, alert your doctor. The sooner heart failure can be managed, the better. Heart failure treatment may consist of ACE inhibitors and diuretics. Other treatments might be right for you.

If you have symptoms of heart failure, you might want to strongly consider seeing a heart failure specialist. Heart failure specialists are cardiologists who specialize in the treatment and management of heart failure. They are specialists who are familiar with new advances in heart failure therapies and the latest discoveries in heart failure treatments.

With well managed heart failure your life does not have to stop. You might need to change a few things or slow down a bit to accommodate the changing needs of your body, but your life definitely does not have to stop.

Some ARVD/C patients, who are not in heart failure, are reporting that their doctors are starting them on ACE inhibitors in an attempt to avoid or delay the onset of heart failure. Talk with your doctor about this to see if it would be appropriate for you.

CHFpatients.com is a website dedicated to heart failure information, (however, not specifically heart failure with ARVD/C). It is an excellent resource. They have a message board where you can connect with other heart failure patients.

Despite the best of care, a very small number of ARVD/C patients may not respond to treatments to calm their arrhythmias or arrest their CHF. A heart transplant may be an option for these patients. The success and survival rates for heart transplants overall have improved tremendously in recent years.

 
EXERCISE

While on the subject of heart failure, my thoughts turn to the hot button issue of exercise and ARVD/C. It is no big mystery why the issue of exercise and ARVD/C has spurred some of the most passionate discussions within the ARVD/C patient community. Many ARVD/C patients were athletes or, at the very least, many were extremely active before their diagnosis.

Many who have been diagnosed with ARVD/C would like to continue their former level of competitive sports or strenuous exercise and activity. For a number of people, their sport or physical activity was a part of their identity. For others, it was a part of their future plans or even their livelihood. Some believe that their inability to participate at their previous level of activity will lead to a decline in their quality of life. Understandably, this issue is a very painful and emotional one for many ARVD/C patients.

The "ExpertsOnExercise.html" document in this site's "Patient Library" contains the thoughts of several ARVD experts concerning ARVD/C patients and exercise. Those who are looking for an understanding of why it might be important for them to reconsider quality of life issues, career decisions or gym time might find an explanation within this document.

The above mentioned document has an April 14, 2006 update from Dr. Frank Marcus, The Principal Investigator of the United States NIH Multidisciplinary Study of Right Ventricular Dysplasia. Dr. Marcus wrote:

The opinion of ARVD/C experts seems to be that when we participate in strenuous and/or competitive exercise, we are not only putting ourselves at risk for instigating arrhythmias, but we are also positioning ourselves for a possible progression of ARVD/C. When we hear of heart failure in others, it is an excellent reminder of how we might want to do all in our ability to avoid that which threatens to progress our ARVD/C.

Heart failure is not fun. Once in heart failure, running marathons is no longer the issue. There are days when just climbing one set of stairs can be daunting. I know this because I live with it.

Heart failure can be treated and managed, but knowing that there is a way that you might be able to prevent it altogether or, at the very least, to delay its arrival gives you some power over your ARVD/C. Keeping the full scope of ARVD/C in our minds can help us make careful choices. Protecting our hearts, in every way possible, is something we can do. Some things we have no control over. Exercise is something we can control.

There are many former athletes who have coped quite well with slowing things down. After a while, some even enjoy the slower pace. It gives them time to catch up on things they may have missed while rushing by. They have found pleasure in picking up a lower intensity activity such as walking, bowling, yoga, tai chi, Pilates and golfing.

One patient wrote of what so many of us have learned:

 
SUPPORT

A great number of ARVD/C patients have received a lot of comfort, and have become better informed by joining a support group. The International ARVD Family Support Network is an online ARVD/C support group. It is so nice to be able to drop in and know that just about anything you are going through or feeling, well, one of us has been there and we can share our thoughts and coping skills with you.

I have already mentioned The ZAPPER, Implantable.com and "ICD Support Group" as good support mechanisms for those with ICDs. Support groups are great places to be, not only for support, but also for access to sharing about medical news and information regarding many things of concern to the heart patient community.

 
HELPFUL TIPS

Fevers, the flu and bacterial infections have been known to instigate arrhythmias in some of those who are prone to them. In fact, some researchers believe that infectious agents may play a role in cases of ARVD/C. Applying reasonable health care practices can spare you some sickness and infection, and it does not make you a germaphobe.

Do yourself a favor; whenever possible, avoid exposure to the flu and other illnesses. Ask your doctor if they think a yearly flu shot might benefit you. Also, remember that many illnesses are passed by hand and/or mouth and eye contact, so keeping hands washed or sanitized can help you avoid getting sick. Consider keeping some hand sanitizer/anti-bacterial gel or lotion in your purse, briefcase and car. Try to get into the habit of cleansing the hands soon after exposure to the public at large, public places, and being around those who are ill. Always wear a spacesuit and breathing equipment when in close proximity to a Preschooler or Kindergartner. Okay, I was kidding on that last one.

Many with ARVD/C have learned that an ounce of prevention can be worth far more than a pound of cure. Aside from that, dodging the need for another doctor's visit has its benefits.

Write up a list of your doctors, diagnosis, medications including dosages, and the name and phone number of who should be contacted in case of an emergency. Post the list openly or in a fitly labeled envelope on your refrigerator. Keep a mini copy in your purse or wallet. Keep a copy in your car. Update it as needed. Look into getting a Medic Alert bracelet or similar product.

When going out alone — grab your cell phone. If you do not have a cell phone, when going out try to make sure someone knows where you are headed and how long you think you might be gone. Unfortunately, you cannot always rely on passersby to lend a helping hand in case of an emergency.

Keep copies of all of your medical records and test results, including films, tapes or CDs, like those from Echos and MRIs. Make your own personal medical file. Hospitals and doctors offices purge records after a certain amount of time. Your medical records could be the key to helping your medical care at a later point in time. Additionally, having access to your medical records and test results allows you to quickly copy and send them for 2nd opinions and/or research purposes — both of which could be important to you. Make sure to always hang on to your only copies!

 
SECOND OPINIONS

It is always a good idea to get a second opinion, especially when the diagnosis involves such a serious condition. Not to mention the real possibility of family members being affected.

You should try to get your second opinion from doctors who really know ARVD/C.

For more information regarding who to contact for second opinions, you can visit this site's "Resources for Patients" page.

 
RESEARCH – THE FUTURE IS BRIGHT

Through the dedication and perseverance of the researchers involved, along with donations and grants, there has been an explosion of discoveries concerning ARVD/C. A lot has been learned about diagnosing ARVD/C. The understanding of the genetics playing a part in ARVD/C is growing by leaps and bounds. There is a constant flow of news on new discoveries, and you can bet there are greater things waiting just around the corner. This is life-saving information for all ARVD/C patients and their families.

The study of the progression of ARVD/C is one of the things at the top of the list for upcoming research. However, without the participation of ARVD/C patients and their family members, the funding for research could disappear.

You can help by being a part of this invaluable work. If you live in North America (United States or Canada), contact those of the ARVD Patient Registry at John Hopkins and the NIH Multidisciplinary Study of Right Ventricular Dysplasia. They will be more than happy to answer any questions you may have. It is very important to urge family members to join research as well.

When it comes to ARVD/C, given the fact that it is a rare illness, the old adage "one person can make a difference" truly applies here. One person, one family, can make a world of difference for all of us, our futures, and our children's futures.

 
JOHNS HOPKINS HOSPITAL'S YEARLY ARVD FAMILY SEMINAR

Every year, around April or May, Johns Hopkins Arrhythmogenic Right Ventricular Dysplasia (ARVD) program hosts the ARVD Family Seminar in Baltimore, Maryland. The seminar is a chance to catch up on the latest ARVD/C news, as well as to mingle with fellow ARVD/C patients. An informal reception and lunch is provided following the seminar. During or after lunchtime, there is generally an opportunity to participate in some of the research testing or discussion groups.

If you have the means and opportunity to travel to the Johns Hopkins seminar, I recommend it. You can check this website's "Upcoming Events" page, or refer to the NEWS at ARVD.com for information on the next seminar.

 
WRAPPING IT UP

ARVD/C was first identified and named in the late 70's. Although there has not been a long enough period of time to fully research ARVD/C and its evolution, there is encouraging evidence that patients who are properly managed have the hope of living into old age.

ARVD/C patients help run corporations, dance at their children's weddings, and spoil their grandchildren. They go on fantastic vacations, visit exotic locations, and feel the warm sand squish between their toes. They laugh — oh, how they laugh. They live and love just as before their diagnosis, some even more so. Life is a little more precious. Life is to be savored. Perhaps they have picked up a little more resilience, and a little more flexibility along the way. They have eased into their "new normal."

ARVD/C does not have to define you. Through the challenges, anxious moments, and ups and downs, you will find a new strength. You will find your way to your own "new normal." Your normal may not be the same as it was before. But, who knows? In some ways it just might be better.

 
MY HOPE

Well, if you have made it all the way through to here, my hope is that you have received that bit of information, help and hope I mentioned way back in the beginning. This page has discussed serious issues that most of us who have ARVD/C have experienced or considered. I think you will find that the more you learn about your heart, your illness, and all that goes along with it, the better choices you will make for your health and your life. Many people believe that the more knowledge they have gained, the more peace they have found.

Ask questions. Stay informed. Seek support and guidance when you need it. Know that you are not alone.

You are not alone.