Her rare heart condition was “fixed,” but its impact endures

At age 10, Shari Weiss had surgery to correct a vascular ring. At 36, she's learning that her heart condition is still part of her life.

Shari Weiss, 36, has never met anyone else with a vascular ring.

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Shari Weiss thought the heart surgery she had as a child was in her past.

In the late 1990s, when she was 10 years old, Shari started having strange symptoms—shortness of breath, a flushed face, coughing fits—first when exercising and then during her daily life. Eventually, she was diagnosed with a vascular ring, a rare congenital heart defect where the aorta (the artery that carries oxygenated blood from the heart to the body) forms a ring around the trachea and the esophagus. In many cases, symptoms are mild, so people might not know they have it until well into childhood or even adulthood. 

Shari, who grew up in Garden City, N.Y.,  had heart surgery in 1998. Though people with a vascular ring need regular, lifelong medical checkups, according to Mayo Clinic, Shari fell out of care. It’s a common experience for people with congenital heart disease: Less than 10% of U.S. adults with CHD who need specialty care actually get it, per an estimate from the Adult Congenital Heart Association.

Today, the 36-year-old Shari lives in Wilmington, N.C., and edits content for several websites, after a decade as an entertainment journalist. She also writes

TeenDramaWhore

, a Substack for fans of teen soap operas. (You can follow her on X at @sizzlemaker or Instagram at @teendramawhore.) All the while, her heart issues surreptitiously influenced her life—in the way she never returned to her beloved basketball, in the scar on her back, in her desire to freeze her eggs. It wasn’t until her 30s when a few turning points, including a revelation from Jimmy Kimmel, changed her perspective. 

Read on for Shari’s story. And if you have a vascular ring, comment below or email me at theheartdialogues@substack.com. Shari wants to hear from you! 

You were diagnosed at 10 years old. How did you learn you had a congenital heart defect? 

Up until that point, I was just like any other adolescents. I went to school, I went to camp. But that summer of 1997, I went to sleepaway camp for the first time. It was an eight-week program, and I got really into basketball. I would go and try to shoot free throws. I would get extremely out of breath, and my face would turn really red, and it became increasingly difficult. But there was nothing that really required medical attention at the time. 

That fall, I joined a [Police Athletic League] basketball league. My dad was one of the coaches. The same thing was happening, getting very out of breath, my face would get extremely red and it would be very difficult to get back to baseline. I started having coughing fits and chest tightness, and my pediatrician diagnosed me with exercise-induced asthma. Because it seemed that the only time that I was having symptoms is when I exerted myself. However, the treatments for that, like an inhaler, weren't really helping. And then I started having this chest tightness and coughing fits, even in settings where I wasn't exerting myself. The first turning point came in January of ’98. I went to see “Titanic” in the theaters for the umpteenth time because I obsessed with it.

I remember that.

I had this tremendous coughing fit throughout the movie and just could not recover from it. We went home afterwards and tried a steam shower and things like that, and I was just not calming down. And so we were advised to go to the emergency room. We did, and while I was there, I had my first nebulizer treatment, and that did get me back to baseline. They took an X-ray, they sent me home. 

A few days later we got a call, and that was the first time we learned something was wrong. As it turned out, there was something wrong with my X-ray. The X-ray revealed that I was born with a right aortic arch, which we had never known before because I never had imaging done before. My pediatrician sent me for an MRI, which was a little traumatic, being in a closed MRI at 10 years old. And that imaging showed the vascular ring, and it was around my trachea and esophagus. That was followed by a bronchoscopy that showed just how tightly the ring was compressing my airway and affecting the esophagus as well. And from that testing, it became clear that I needed to have surgery sooner than later to repair the issue.

Shari and her mom at the camp where her symptoms first appeared, 1997

Wow. When did you have the surgery?

I had the surgery on March 17th of 1998. I was in the hospital for five days, starting in the pediatric ICU for a few days before going to a room. Then I was out of school for a month. Then even upon returning to school, I was not permitted to participate in gym class. I just had to sit and watch everyone. I was not allowed to participate in recess either, so I just had to stay inside while everyone else went out to play. I wasn't allowed to carry a backpack. I had to have a set of textbooks at home, and then when I was actually in class, I would share with a classmate. That greatly affected my life in fifth grade.

I could see a kid having lots of different reactions to that, but what was it like for you?

It was really challenging. When you hear that you have asthma, you don't really think—at any age—that's going to end up winding to needing heart surgery down the line. It was very strange to wrap my head around that. Returning to school after being away for a month was very difficult. I feel like a month for a child can be a really long time. It seemed that my closest friends that I had at that age really got a lot closer at that time in my absence. When I came back, I was largely excluded, and that very much affected my trajectory socially for the rest of elementary school. And then my scar—it’s on my back actually. It's really large in terms of length, and I healed very badly. I healed keloid, so it's not flat. It's raised and bumpy and has always been this pink, red, purple color. I had to adjust to being the kid who, if I wore something, other kids would say to me, “What's wrong with your back? What happened to your back? What's that thing on your back?” It just became this lifelong insecurity.

Did you try to hide it?

It was very difficult, due to it being on the back and the fact that it was raised and discolored. It shows if I wear certain tank tops, tube tops, white shirts, see-through shirts, bathing suits, strapless dresses. It's just always there.

I have a scar on my back as well, and then I also have a scar on my front. I would imagine that it would be hard for people to understand that a scar on your back came from heart surgery.

Yes. Yes. That is true. I even see doctors and they'll say to me, “Remind me what that scar on your back is from?” And I have to remind them again that I have this defect, and I had this surgery, and this is what it left me with. Even just a couple of years ago, at my community pool, a woman, a stranger I didn't know, just out of nowhere comments to me, “That's some scar you have!” It's like, no shit, lady. I'm very much aware that it's there and how unsightly it is. Even in romantic relationships, it has always been an insecurity. Will a man be grossed out by this thing on my back? Will he not want to touch my back? For a long time, many years after the surgery, I couldn't be touched there. It was very numb or tingly or to touch it would just be painful. It's kind of always been this reminder that I can't escape from.

How did the surgery and having the heart defect change your life as you got older? 

I never went back to sports. I was too scared, even though it was explained to me, the issue is done, you're going to be fine. I just had this lingering fear in the back of my head that I'm going to exert myself one day and drop dead. Even though I had loved basketball and wanted to keep doing it, I never did it. Never went back to it. In high school, I refused to run the mile that you had to do once or twice a year in gym class. It was just this deep-rooted fear of mine that stayed with me. Even now when I exercise, my face gets extremely red, and it can take over an hour for it to get back to its normal color and pigmentation and for the heat on my cheeks to stop radiating.

Do you work out now? Do you still feel that way? 

Yeah, I do cardio and lots of walks and things like that, but nothing crazy just because I'm not big into exercise anyway.

That's fair! You also had a lapse in care. How did it happen?

For a few years, I remember, I continued seeing my pediatric cardiologist, who went on to be the head of a big program somewhere. I don't remember any reason for stopping. I don't remember being told we didn't have to come back. I honestly don't know what happened. I just know that I just stopped seeing them. The only times it would really come up would be if I went to a new doctor and I had to give my medical history. You have to list past surgeries, and I would list it. Almost every doctor has no idea what it is. I would have to explain to them what it is, and that's kind of it. I just kept going about my life. 

I would think about it a lot because I think about my scar a lot. I see it a lot. I feel it a lot. Even now, 25 years later, there can be this weird twinge or an itch or something like that. So it's never actually been far from my mind, but I never considered myself as someone where this is going to come up again or I still have some kind of defect. Maybe I didn't understand it to its fullest extent, given the age that I was at the time. But it was always in the back of my mind that I have this experience, it makes me different from other kids. Never really dealt with any lingering emotion from it. Then I had a couple of turning points. The first was back in 2017 when Jimmy Kimmel went public with his son's heart issues.

I remember that!

Even though they were significantly different than mine, I had a really emotional reaction to it. The way he explained what happened and how much the nurses meant to him and what have you. It got me thinking about my time as a child in the hospital and things like that. I even wrote an essay about it that I never did anything with, and I just tried to stuff it back down. More recently, I had a couple of wake up calls. The first came in [the spring of] 2021 when, for the first time, a lot of my old symptoms returned.

I was having unexplained, uncontrollable coughing fits again. I was having chest tightness. I was getting really fatigued easily from everyday tasks, along with some other things. No one could really figure out what was going on. To this day, we don't know the cause, but it had me pretty much out of capacity for a number of months. Having some of these symptoms that I had last time when I was 10 come back—it was very re-traumatizing. It was very, very upsetting to be experiencing those things again. Your mind wanders, what does it mean? And so I ended up going to see a doctor in an adult congenital heart defect program [nearby]. 

When I went to see him, he informed me that, at the very least, I should have been having CT scans every 10 years. That just shocked me. While I know, relatively speaking, that needing a test every 10 years is absolutely nothing compared to others who require multiple testings every year, my mind was just blown that I was supposed to be having continual care. No one had ever told me that before.

I had already missed two of the follow-up testing periods that I should have had. That was really eye-opening. The second wake up call was when I read your essay in the Wall Street Journal. It resonated with me so much, and it dawned on me that just because my vascular ring was repaired, it doesn't mean I'm actually cured of the issue. I still have the right aortic arch. I had an endoscopy years ago that showed that my trachea is still significantly indented. And it just dawned on me for the first time that this defect was corrected, but still exists to a certain extent.

Maybe it wasn't as in my past as I thought it was. And then the third and final turning point came just a few weeks after your essay last spring. I went for a consultation and testing to begin the process of freezing my eggs. The doctor told me that I would automatically be a high-risk pregnancy because of these issues, and that to even freeze my eggs, I would need to get clearance from a cardiologist. No one had ever told me these things before. Obviously, I've seen gynecologists for years and years and other doctors, and nobody ever told me that there could be pregnancy complications as a result of this. That was really stunning. 

I want to get into that much more in depth, but can you talk about what your care is like today? Are you seeing a cardiologist regularly?

I saw that doctor back in 2021 as I went down this rabbit hole with doctors of all different specialties, trying to figure out what was wrong, never got to the bottom of it. In the fall, I started to recover and I kind of put it behind me. And the cardiologist gave me the name of a doctor here in Wilmington where I live in case I wanted to finally continue managed care. And frankly, I never did anything with it. I have still not seen any cardiologist since then, and another two years have gone by. But everything that I've read from you, and as I'm learning more about the importance of continued care, even if it's just monitoring, I've become more motivated to change that. I just still need to actually do it.

It's really hard. It's hard to get appointments with these doctors. They have so many patients. I wind up taking off a whole afternoon of work. It's really expensive, even though I have great health insurance. That's just the logistical hurdles, let alone the emotional ones. It's very understandable. You mentioned the egg freezing. Where is your head at now, hearing that you’d need a cardiologist’s clearance and that there might be complications? 

It was certainly jarring because it was nothing that I expected to hear, and it was nothing that had even remotely come up as being related to one another before. I do not have a current relationship with a cardiologist, so I would need to find someone to get that clearance to do the egg freezing. And the only way I was going to be able to afford the egg freezing process—because it's extremely expensive—was through a fertility benefit from my employer. However, just a few days after I went for my consultation and testing, my employer paused the benefit. There's no way that I could possibly afford it on my own.

This is a really hard situation no matter what. Even those who get regular cardiac care are also sometimes surprised when they hear about the risks. That seems like such a problem.

Yes, I completely agree. This happened to me at 10 years old, I'm now 36. Think about for how many years I've been going to a gynecologist or a primary doctor, and no one has ever said anything about this. Even as I've talked over the years about my desire to be a mother and to explore my options and things like that. It was really stunning. Even now that the subject has been broached, it's unclear to me, it has not been explained to me what exactly the risks are. What is the connection between my issues and a pregnancy that could be problematic? It never dawned on me before that this abnormal anatomy that I have, would there be a risk of a child having that too? I don't know. It's not something I ever thought of before.

You're right, it's not even just cardiologists. It's primary care doctors and gynecologists. There's such a lack of knowledge about congenital heart defects, even though they affect so many people.

Yes. My defect is extremely rare, it accounts for less than 1% of all childhood heart defects. Part of me wants to give medical providers grace for not knowing what it is. At the same time, it's very jarring to go to a doctor and say, “I have X,” and the medical professional has no idea what you're talking about. And that I, as the patient, need to explain to you, the doctor, what a vascular ring is.

I'm curious about your parents' role in all of this. How did they deal both with the diagnosis and surgery? How were they involved? 

Certainly in the beginning for appointments, my mom was just taking me. When there started to be a couple of things that my dad came along for, that very much drove home, Oh, something serious is going on if my dad is skipping work to come with us. 

My strongest memory of their reactions is the morning of my surgery, I am in the pre-op and they've given me a pill to make me woozy, to start getting me ready for sedation. At 10 years old, I certainly don't know what it means to be drunk, I'm basically drunk. From this pill. I'm being taken away from my parents, wheeled into the operating room, and I'm as loopy as can be. I have my teddy bear. I'm throwing it in the air. I'm just being silly beyond silly. But my mom is standing there just hysterically crying. As they take me away. That has just stayed with me because it shows the gravity of the situation and what it must've been like for her as a parent, to watch her baby being taken away for heart surgery. 

The first night or two, I insisted that she stay in the hospital with me, which she did. I needed really hands-on care when I was at home. I didn't even return to my bedroom. I slept in the living room for a number of nights. The couch was just on a better setup for me, especially with this being on my back. 

Shari and her mom, earlier this year

Once we got past the immediate crisis of it, it just became this thing in our past. It would only come up over the years in relation to my scar. Like every other person, I can't put sunscreen on my back by myself. I need someone else to do it. As a kid, my parents would do it, and they would just have to be super gentle because of how delicate that spot was for me. As a teen, you're going to sweet sixteens and things like that, picking out dresses with my mom, [saying,] “How much does my scar show?” 

Even in recent years, as bad as it still looks, and it still looks really bad, it's actually improved. Part of it is not nearly as raised as it once was, and not nearly as red and purple as it once was. There are times my mom and I marvel over that. I did see [in my teens] a plastic surgeon to discuss the possibility of fixing the scar to make it more aesthetically palatable, as vain as that may sound. My mom and I went to that appointment. I credit the plastic surgeon very much for his integrity. He admitted to me that there was no guarantee that he could actually make it any better than it was. I decided it was not worth going to the trouble. 

How does your heart stuff show up at work?

I'm very fortunate to be in such a position where this does not affect my day-to-day life. I'm not in a position where I'm someone that needs to go for testing every year or multiple times a year. I've been very blessed in that I'm able to live a life where this does not affect my career in any way. Certainly when I was having some of these health issues in 2021, I definitely missed work for doctor's appointments. I spoke with my leader at the time about some of the emotions that were being triggered, things like that. Otherwise, it's this secret that only I know about. I started my current job in March of 2022. I’m full-time remote, so no one sees me, there would never be an opportunity for someone to see the scar based on what I'm wearing. And generally, “Oh, I had a heart surgery as a 10 year old”—it's not something that comes up in everyday conversation. 

You don't say!

However, this year marks 25 years since the surgery, and it made me emotional and reflective. I did share it with my boss. It's just like, “Well, here's an interesting fact about me! Today marks 25 years since blah, blah, blah.” Certainly when you say something like that, people are pretty surprised.

It's interesting how much it can impact your life and how little people know that you have something like this. It's very easy to disappear if you want. Switching gears, are there any benefits that have come from having a congenital heart condition? Any way that it has improved your life or changed it for the better?

That's a really interesting question. I'm not sure. I know in the grand scheme of things, I am very, very lucky and blessed. There are many people with congenital heart defects who not only have really rough roads, but continue to, and that their condition is a factor in their everyday life, and it's not in mine. I'm very lucky. At the same time, it's been very lonely because I've never known a single other person with a right aortic arch and a vascular ring. I've never in my life spoken to another person with this defect. I'm only recently realizing to begin with, Oh, this is a congenital heart defect. Forget about my specific condition, I've never spoken to anyone in general with a heart defect.

You have now! At least one.

I’m grateful for that because I want to learn more. I want to know more. I want to compare stories, not in a “who's worst” kind of way, but just to further my understanding, to find comfort through the community. Part of speaking to you and sharing my story is that maybe somebody's going to read it and be like, “Oh, I have the same thing. Let's connect and learn from each other.” That's my hope.

Have you had a lapse in care? Has your heart condition become a bigger part of your life more recently? Tell us about it!