Health Issue: Born with a Heart Defect and Lost to Care

Health Issue: Born with Heart Defect and Lost to Care

achd patient with doctor

There’s a very real health issue happening right now. Well, it’s been going on for the last 3-4 decades. And we haven’t been able to break through the “noise” to spread awareness for it. I need your help.

I’m referring to adults who were born with a heart defect as a baby and have grown into teenagers and adults.  This condition is known as adult congenital heart disease, or ACHD. For reference, about 1 in 100 babies are born with a congenital heart defect (CHD) each year.

What Being Lost to Care Means

In the United States, less than 20% of adults born with a heart defect are estimated to still be receiving the specialist care they need. The medical community refers to this as being “lost to care.”

In other words, a very large number of patients with congenital heart disease are lost to care.

And a large proportion of those lost to care – if not the majority – need to see a cardiologist who has training in congenital heart disease at least once in their lifetime (and maybe more, depending on their condition). This is because CHD-related issues are very different than typical adult heart problems. More importantly, patients are nearly three times as likely to live longer under the care of congenital heart specialists and surgeons. 

Frequently ACHD patients need to see two cardiologists: one who can provide for the congenital heart disease needs and the other for their more general cardiology needs. This is so important that national patient organizations and national medical institutions have begun recognizing specific centers and cardiologists  through a nationwide accreditation process.

You might be wondering how these folks have become lost to care in the first place. It typically happens between ages 15-30. We have found that by age 12, one third of patients are lost to follow-up; by age 17 half are lost to follow-up; and by age 22 more than two thirds are not being followed by a cardiologist trained in congenital heart disease.

There are a few reasons why we think patients become lost to care:

  • They think they were “cured” in early childhood and have felt fine since

    In early childhood, when these patients had their congenital heart defect surgically repaired, the surgeon told them their child was “repaired”, “corrected” or even “fixed.” When their parents heard this, they might have thought that their child was “cured.” Over time, as their child continued to feel fine, it stopped being top of mind.

  • They got older and lost their medical follow-up

    This problem is not unique to patients with congenital heart disease. As patients with well-managed childhood conditions grow into their teenage years, and especially when they go off to college, their health takes a back seat to the new changes in their life. They move away and they might not see a doctor for their childhood condition for four or more years.

  • They got older and don’t want to think about their heart condition

    Some young people, especially teenagers, don’t want to be different from their peers. They don’t want to take their medications and don’t want to do anything – like go to appointments and have diagnostic tests – that remind them of their congenital heart defect.

  • They see a doctor, but not the right one

    I think there is sometimes an expectation that all doctors should know and recognize what conditions people have. But the reality is that not all physicians or cardiologists are trained in congenital heart disease. It’s imperative to find one who has been.

  • They know they have a scar, but that’s it

    From my point of view, this is the scariest scenario of them all, and it happens frequently. These folks know they have a scar on either their chest, under the armpit, or under their shoulder blade on their back. They don’t know any more, other than they had a surgery when they were very young. And for any of the reasons above, they stopped seeing a doctor for it.

The importance of seeing a cardiologist trained in congenital heart disease

We can loosely compare heart specialists to house repairs. While both roofers and plumbers know how to work on houses, you wouldn’t hire a roofer to fix a leaky pipe. In the same way, while two doctors are trained to look after hearts, one may be more specifically trained to care for individuals who were born with heart disease. The other is trained to care for patients who develop heart disease later in life.

Regular follow-up appointments – once a year or every other year, depending on the condition – are imperative because as a repaired heart from early childhood grows and adapts through life, negative consequences can take place, such as heart rhythm disturbances, leaky valves, strokes, obstruction, or even heart failure.

Heart Deterioration Can be Prevented if caught early

This type of heart deterioration does not happen overnight. The changes happen in small, subtle ways, and they can be prevented if caught early with physical evaluations, diagnostic testing and imaging and addressing the problems early.

The problem is that people don’t notice the small subtle changes in their heart – they only notice the catastrophic changes like when they’re short of breath, their heart races uncontrollably, or they swell up with fluid accumulation in their legs or abdomen. At this point the heart disease, while still treatable, has often advanced significantly.

However, if deterioration in heart function is caught early, some of the therapies to prevent the catastrophic side effects from happening can be instituted such as specific medicines, pacemakers, cardiac catheter-based treatments and sometimes surgery.

This issue of patients with congenital heart disease being “lost to care” is a world-wide problem, but it’s especially prevalent in the United States.

But the good news is that no one has to be lost to care. With good ole fashioned awareness spreading and word of mouth, we can treat these patients sooner and prevent them from having irreversible and potentially catastrophic damage to their hearts.

If you are reading this and you had a heart defect as a child, or know someone who did, go here to request more information or click on the social media icons above to share and help spread the word.


This is a two-part series. In my next blog post, I share some questions and a chart to help identify if someone has congenital heart disease and is lost to follow-up care.

Subscribe today for more stories, tips and updates.

Andrew Redington, MD

About the Author: Andrew Redington, MD

Andrew Redington, MD, is the executive co-director of the Heart Institute and chief of Pediatric Cardiology at Cincinnati Children’s. He specializes in both pediatric and adult congenital heart disease and has written more than 300 peer-reviewed publications, 30 book chapters and has co-edited seven books. Dr. Redington lives in Cincinnati and in his spare time enjoys the amazing arts the city has to offer.

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  1. David July 22, 05:06
    Hello my name is David my diagnosis is (this is from my doc at UofM) situs inversus totalis, atrioventricular discordance, ventriculoarterial discordance, (D-TGA), dextrocardia, and ASD that was repaired at the age of 17 he also wrote this "this is a variation of congenitally corrected transposition with the morphologic right ventricle serving the systemic ventricle role. After I had my ASD repaired and I went for check ups my doctors would always say "why are you here you are fine". In Feb of 2014 I developed Dbl Pneumonia and things went down hill from there and no one could figure out why. Then I went to see a ACHD doctor at the university of Michigan and he discovered my Tricuspid valve was leaking at a moderate to severe state and needed to be replaced. Well on June 6TH of this year I had my second OHS and I now feel Great.
    • Andrew Redington, MD July 25, 15:28
      David – thank you for sharing your experience. I believe your story demonstrates so well the importance of timely specialist care. You clearly have a very complex heart condition, such that imaging of your heart, and assessment of your cardiovascular system is indeed very tricky. Thankfully you were able to find a good specialist team who were able to help you preserve your heart muscle function by surgical rescue of valve function. Thank you for sharing your story.
  2. TGA Mom July 22, 08:05
    This is a very relevant article. As the mother of a young adult living with TGA, we always followed up with pediatric cardiologists his entire life. He had a mustard procedure at 7 months old in 1986 and thereafter visited the doctor very 3 months, 6 months or one year, whatever was asked of us. At age 10 we need to switch cardiologist and took our time choosing a well known group of pediatric cardiologists associated with an amazing hospital to care for our son. For the next 11 years I was told that he was "remarkably boring" as his heart was working well and all was good after exam and echo. There were some bumps along the way and stress testing was ordered and other procedures and monitors as well. Nothing was ever found and tweaks in his medication usually were prescribed. Around age 20 we asked for a new round of testing as my son felt (and we saw) many subtle symptoms. We were told by his doctor that he was fine and he didn't recommend testing. Echo, EKG looked good and exam went well. Twice he passed out, even broke his nose, and still no testing. It just didn't sit well with us. He was not well and we knew it. It took some time to figure it out as I thought we were with the best doctors for him. Around age 24, thanks to a FB page for mustard survivors, I found information about other doctors and decided to make a call. After his first appointment the cardiologist told us he thinks there may be a blockage in his baffle and more extensive testing and work up was done at the hospital. A cardiac catherization was set up to see more and possibly place stents. Unfortunately, his blockage was so severe a stent could not be placed. His new doctor asked me, "So, they never told you there was a blockage?" I cried, because for years I had trusted them with the life of my son. He could have died with the severity of the blockage. Three months later, at age 27, it took a while to find a surgeon for him, my son had his mustard re-done. He did very well after an extremely long surgery. He is currently enjoying good health once again. I do feel there were lasting effects from going undiagnosed with this blockage for so long, such as severe anxiety, depression, inability to work due to many of there mental health issues. It also concerns me that a heart that pumps out to the body from the RV was taxed unnecessarily for a long time. We continue with making sure that our now 30 year old ACHD son is seen regularly by his cardiologist, whom we can not thank enough for knowing his stuff. Moral of this story is not all pediatric cardiologist are well trained in CHD and I know we become comfortable where we are but sometimes you NEED to make a change and always follow your could be the best thing you ever do.
    • Rachel Camper
      Rachel Camper July 25, 15:30
      TGA Mom, Thank you for sharing your story and experience with us. Sounds like you were able to make a very wise and timely decision about your son's follow-up care.
  3. Taze June 10, 19:47
    Hi, I am a 53 yr old woman with cctga that was only diagnosed in my late 30's. I am presently seen 12 monthly for echocardiography and ecg at a specialist hospital for ACHD. I had been very stable for years with just a mildly leaky tricuspid valve. However in 2013, severe enlargement of my left atrium was first noted. Then in 2014 my pulmonary valve also began to leak, my RV was thickening, I had trivial pericardial effusion for the first time and in 2015, my left ventricular systolic function was reported as "vigorous" instead of Normal for the first time. My EF dropped from 50 in 2013 to 40-45 in 2017. Despite these signs of deterioration, I was continued to be told that I am doing well. I am looking for alternative care now due to this lack of communication from the ACHD team. I had many stresses in my life that began in 2013, and I started post graduate study in 2014. I stopped exercising due to study, I began to overeat as a response to stress. If I had been told of the deterioration, I would have looked after myself better. Because all the indicators are still "mild" they didn't bother. I feel like they have just decided that deterioration at my age is usual and therefore unremarkable and as nothing can be done medically I have been patted on the head and dismissed! But I can and would have adjusted my lifestyle 3 years ago had I known. I have now lost 10 kg and am trying to get my BP down. I am trying to find a specialist that will talk TO me. Not down to me and keep me informed. I think that my heart is more vulnerable to physical and emotional stress than a normal heart. I intend to maximise my physical health and try to keep my emotional state calm ( bit of a type A here) . I am wondering if this lack of communication is a common problem with ACHD cardiologists?
    • Andrew Redington, MD June 12, 15:22
      Dear Taze, Thank you for your note. Over the years we as physicians learn painfully that we are not always the best communicators, and sometimes also not the best listeners. I am sure this happens to most of us from time to time, but hopefully not too frequently. It would appear to me that you highlight a couple of concerns: 1. The partnership you have with your physician and this includes communication, trust, belief etc. 2. Concern regarding possible deterioration in heart function over time. With regard point 1: Compassionate care in the context of excellent medicine and strong systems resources means fostering good relationships and partnership with our patients. We all get it wrong occasionally however. So my personal advice is to try your best to restore the partnership with your local ACHD unit, and if not possible, then we would of course be very willing to help in managing your care. 2: You are right to be concerned regarding your right ventricle and tricuspid valve particularly. These are well recognized issues that become more important over time. The challenging part of your management is determining when is the right time to intervene however. This takes knowledge and wisdom, and early on as the RV or tricuspid valve becomes worse, this may be particularly difficult to make medical decisions.