congenital-heart-disease

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I really debated posting these–they are, after all, ridiculous. But I took them because they made me feel good. I spend a lot of time being exposed and touched as part of my illness, and never on my terms. So I decided to expose and represent myself my way.

There are pretty strict rules about who gets to be sexy. Most of the time, as a conventionally attractive average sized white cis girl, I fall pretty well within the bounds of visual acceptability. But today I’m covered in electrodes. My illness is suddenly extremely visible and my body is no longer acceptable. Medicine objectifies and desexes me and culture ignores me. So, fuck that. I’m representing myself. Sick is femme too.

Also, enjoy my boobs I guess.

Total Anomalous Pulmonary Venous Return (TAPVR)

In an anatomically normal heart, the pulmonary veins connect to the left side of the heart and provide oxygen-rich blood to the body. In TAPVR, the pulmonary veins connect….well, not there. 

TAPVR is known for it’s “snowman sign” on chest xray, as the abnormal veins and heart together look a bit like a snowman. 

There are three types of TAPVR: supracardiac, cardiac and infracardiac. 

In supracardiac TAPVR, the pulmonary veins connect to the superior vena cava (which carries oxygen-poor blood from the body back to the heart). Oxygen-rich blood from the pulmonary veins mixes with oxygen-poor blood from the SVC. 

In cardiac TAPVR, the pulmonary veins connect directly to the right atrium. Oxygen-poor blood is delivered into the right atrium, which mixes with the oxygen-rich blood from the pulmonary veins.

In infracardiac TAPVR, the pulmonary veins connect to the inferior vena cava and veins in the liver. The IVC carries oxygen-poor blood back to the heart, which mixes with the oxygen-rich blood from the pulmonary veins.

(Images from www.obimages.net

There is also mixed TAPVR (rare), where the pulmonary veins connect randomly to the heart and partial TAPVR, where some pulmonary veins still connect normally.

So how does oxygenated blood get to the left side of the heart and out to the body?! An ASD (atrial septal defect, or hole between the atria), allows blood to pass from the right side to the left side of the heart. The blood will be mixed, however, meaning the baby will not be fully saturated and may be cyanotic.

TAVPR is repaired surgically, with the pulmonary veins being moved to attach to the left atrium and the ASD closed. Surgery is usually performed within the first few weeks of life, except in the case of obstructed TAPVR. A medical emergency, obstructed TAPVR occurs when the pulmonary veins are also narrowed and prevent blood from returning to the heart. Surgery will need to be performed imminently.

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This is my undergraduate thesis project on congenital heart defects. It’s in the art gallery at Arcadia through commencement on May 15th. All of the images were painted in Corel Painter with some elements in Photoshop.

The first image is a morphologically normal heart. The others show four complex congenital conditions: Tetralogy of Fallot, Persistent Truncus Arteriosus, Hypoplastic Left Heart Syndrome, and dextro-Transposition of the Great Arteries.

I’m so excited to be studying Medical and Biological Illustration at the Johns Hopkins School of Medicine’s Department of Art as Applied to Medicine in August.

JuliaLerner.com

Story of My Life

While you all know me on here as a huge 1D fan who is all about the positivity and love, I wanted to take today, my birthday, to tell you all something else about me.  I am a Congenital Heart Disease patient and survivor.

When I was born, 27 years ago today, I was born blue, so the doctors instantly knew that something was wrong.  6 hours later, before I even had a name, I was put in an ambulance and rushed to a children’s hospital where I was diagnosed with various heart conditions.  My heart failed to develop properly and I had to have open heart surgery.  A doctor told my parents that it was unlikely that I would survive.

I had my first surgery when I was 2 days old, my second when I was 9 months old, and my third when I was 2 and a half years old.

Although it has been about 25 years since my last surgery, I am not cured.  CHD kids like me are never cured, we are treated.  I will need a valve replacement at some point in the future, as the valve I have now (a cow valve!), won’t last forever.  I visit a cardiologist every year where I go through tests to try to gauge when this surgery will have to happen.  In the photo below, taken a few years ago, I am wearing a Holter Monitor which studies my heart activity for 24 hours. 

So because today is my birthday, I wanted to share my story with you all.  I wanted to tell you all just a little more about me.  I’m so proud to be a CHD patient.  I wear my scar, which runs from the top of my chest to the top of my stomach, with pride.  

1 in 100 babies in the United States are born with Congenital Heart Disease, but people don’t really know much about it.  So here I am, 27 years later, telling the story of my life. 

Thank you to everyone who reads this, likes this, and/or reblogs this.  Sharing my story and spreading awareness means so much to me and I couldn’t be more grateful for your support.

All the love,

Lauren x

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testing. i’ve not been feeling well for a while now, so my cardiologist decided to run a holter. i hate holters: they’re itchy and uncomfortable and impossible to sleep in. but more than that, they’re strange, exposing, highly visible. both a target for other people’s narratives while being simultaneously erased within them. 

this photoseries on my instagram started as #hospitalglam but it continued as the hospital followed me home. the last time i did something like this, it was an attempt to reclaim my body/identity/femmeness. those images are bold and confident, an attempt to perform myself into the bravest, brashest version of myself, to make things true by saying them. those are images of me not letting the medical establishment get me down. this series started out the same way–you can see it in how easy and confident I am in front of the camera. 

but as time wears on it becomes something different: a portrait of vulnerability and intimacy, the way the enforced vulnerability of the holter wears on me. this series became an attempt to document my experience of exposure, to find myself in the narrative. I wasn’t confident: I was tired, and hurting, and exposed. since I couldn’t do anything about any of that, I decided at least to represent it, to fix it down and document it as proof that it is real. 

above all else, i insist on the reality of my own experience. though these images are less immediately captivating, i think they’re ultimately more interesting and complex. here is my pain, here is my exhaustion, here is my survival.

Hypoplastic Left Heart Syndrome (HLHS)

HLHS is exactly what it sounds like: the left sided structures of the heart are hypoplastic or underdeveloped. While the exact features can vary between patients (HLHS variants), typically HLHS describes 6 defects. 

  1. Mitral valve atresia
  2. Severe aortic valve stenosis
  3. Hypoplastic left ventricle 
  4. Atrial septal defect (ASD)
  5. Hypoplastic ascending aorta
  6. Patent ductus arteriosus (PDA)

Mitral valve atresia. The mitral valve allows blood between the left atrium and the left ventricle. In atresia, the mitral valve does not open. Blood cannot enter the left ventricle, causing the left ventricle to become severely underdeveloped. 

Severe aortic valve stenosis. The aortic valve allows blood into the aorta from the left ventricle. Stenosis means the valve does not open completely. This (combined with mitral valve atresia and left ventricular hypoplasia), means blood cannot enter the aorta where it normally does. Instead, it bypasses the ascending aorta and enters near the aortic arch through the PDA. This decreased blood flow causes the ascending aorta to become severely underdeveloped. 

Hypoplastic left ventricle. Normally, the left ventricle is the strongest in the heart. It pumps blood out through the aorta into the body (systemic circulation). When the ventricle become hypoplastic, it cannot adequately supply the body with oxygenated blood. This is why HLHS is referred to as a single ventricle defect: the right ventricle is the only effective pumping chamber in the heart.

Atrial septal defect. An atrial septal defect is a hole between the atria of the heart. This allows blood to be shunted between them, bypassing the left ventricle and entering the right ventricle. The right ventricle is now responsible for pumping blood to both the body and the lungs. As you might expect, this causes oxygenated and deoxygenated blood to become mixed together. 

Hypoplastic ascending aorta. Because the ascending aorta is underdeveloped, the surgery for HLHS involves constructing a new aorta from the base of the pulmonary artery (one step of the Norwood procedure, the initial surgery for HLHS). 

Patent ductus arteriosus. This isn’t technically a defect, as all babies normally have a PDA that closes shortly after birth. I feel it’s worth noting, though, as the PDA will need to be kept open in babies with HLHS. The PDA is a connection between the pulmonary artery and aorta. When blood is pumped from the right ventricle into the pulmonary artery, the PDA allows some of it to flow into the aorta as well. This is the only way blood is able to enter systemic circulation. Life is dependent upon the PDA in this defect, and prostaglandin must be infused to keep it open until surgery can be performed.

Surgery for HLHS is initiated within the first few weeks after birth, as this defect is incompatible with life without it. Surgeries typically occur in stages as the heart matures. The first being the Norwood procedure, followed by the Glenn procedure and the Fontan. It should be noted that none of these surgeries can create a “normal” heart, but can redirect blood flow so that the body is perfused much more effectively. 

Half A Heart

While this Sunday is more widely known as Super Bowl Sunday (or Puppy Bowl Sunday), it is also the start of Congential Heart Defect (CHD) Awareness Week.

I am a CHD patient.  When I was born, half of my heart had not developed properly.  I was born blue and it was 6 hours later that I was diagnosed with what is called Hypoplastic Right Heart Syndrome, as well as other various defects including a hole in my heart and a murmer.  I was rushed to a different hospital to be treated by specialists; I didn’t even have a name yet.  I had my first open heart surgery when I was 2 days old, my second when I was 9 months old, and my third when I was 2 and a half years old.

(That’s me not long after my second surgery!)

CHD kids like me are never cured.  While it has been 25 years since my last surgery, I see a Cardiologist every year, I go through a number of tests, and I most likely will need a valve replacement because the bovine (yup, I’ve got part of a cow heart) valve I was given will not last forever.

(That’s me wearing a Holter Monitor a few years ago)

Congenital Heart Defects are America’s #1 birth defect, affecting about 1 out of every 100 births.  CHDs are the most common cause of infant death due to birth defects.

I am making this post simply to spread awareness, because that is what next week is all about: educating people about something that is really important to me.  I am SO proud to be a CHD kid.  I am so grateful that I have never felt ashamed of my scar or of my condition.  Not many people know about CHD and I am always happy to share my story. 

Thank you for reading and helping me spread awareness.  My heart may be missing some pieces and may be a little different than everyone else’s, but it is full of love and gratitude.

All the love,

Lauren xx

Once you've had your Glenn, O2 is your friend!

So everyone knows patients with congenital heart disease have low sats. The first thing people joke about cardiac units is how no one freaks out over a kid with sats in the 70s. But is that entirely true? And why do we allow sats to be so low?!

Sats in the 70s are generally only ok for single ventricle patients. We like them to sat between 74 and 85 percent. Where does that number come from? Blood coming from the lungs is 100% saturated with oxygen, blood from the body is about 50% saturated. (50 + 100) / 2 = 75, which represents perfect mixing of blood (single ventricle patients will have mixed blood until surgically corrected).

Why don’t we stick them on a nasal cannula and shoot their sats up to the 90s!? That would be great, right? Aren’t higher sats better? Not for these babies! Oxygen decreases pulmonary vascular resistance, allowing more blood to flow into the lungs. For a baby with mixed blood to get a sat of 95 means that they have almost 3x the amount of blood going to their lungs ….which means much less blood is going out to their bodies. Uh oh! That’s how you put a baby into shock! While low sats are obviously not an ideal way to live, there’s not much you can do about it until surgically palliating the defect or transplanting a new heart. Being blue is better than being grey! 

It’s for this reason that it’s very dangerous to put a baby with single ventricle physiology on 100% oxygen and all of these patients should have a blender in their rooms. The Glenn procedure is the second surgery in correcting single ventricle anomalies and the first to begin the separation of systemic and pulmonary circulations…hence the phrase, “once you’ve had your Glenn, O2 is your friend!” 

Double Outlet Right Ventricle

Double Outlet Right Ventricle (DORV) is a congenital cardiac anomaly in which both the pulmonary artery and aorta arise from the right ventricle. In a normal heart, only the pulmonary artery connects here while the aorta connects to the left ventricle.

DORV is typically associated with a ventricular septal defect (VSD, or a hole between the ventricles). Transposition of the Great Arteries (TGA, or when the pulmonary artery and aorta are in reverse locations), pulmonary stenosis/atresia (abnormalities with the pulmonary valve that prevent or decrease the flow of blood into the pulmonary artery), and corarctation of the aorta (a narrowing of the aorta) can also frequently occur with this defect.

The VSD allows oxygenated and de-oxygenated blood to mix. This mixed blood is what is carried out to the body. Because of this, you can expect a patient with this condition to have decreased oxygen saturations (usually in the mid-70s to 80s). 

Surgical repair is necessary, but the exact procedure can vary greatly between patients. The approach will depend on the location of the VSD, location of the great arteries, and how developed the ventricles are. Some patients will be able to have a two ventricle repair, while others will require a single ventricle repair.

Hearts like Ours.

Hearts like ours,
they were meant to be swollen
with lust, with passion,
anger; whilst fury is
curled tightly and its
bruised knuckles are neatly
tucked into our ventricles,
ready to punch out pain,
to pump our fists
and fight another day,
even if Death isn’t kissing
our face with It’s feather boa.

Hearts like ours continue to live,
despite the expectations.
Be grateful that our hearts
don’t conform to a self fufilling propechy
like we do.
Physical affliction doesn’t mean
visible affliction.
Our illness is transparent. To us.
We are a window,
but we are curtained by our bodies.
We need to forgive those
who are healthy and judge us.
Just as we judge those
who are healthy
and abuse this privelledge.

Medicine gave us life,
be we gave our life meaning.
We were handed bad luck
yet we turned it into a phenomena of being.
Hearts like ours weren’t born to be pretty,
but that doesn’t stop us from being beautiful.

Tetralogy of Fallot

  1. Pulmonary Stenosis
  2. Right Ventricular Hypertrophy
  3. Ventricular Septal Defects
  4. Overriding Aorta (overrides a VSD)
  • most common cause of cyanotic congenital heart disease
  • signs - clubbing, loud ejection systolic murmur (radiates to back), cyanosis
  • surgery at 6 months, may give temporary shunt to increase pulmonary blood flow (subclavian artery–>pulmonary artery)
Tricuspid Atresia

Tricuspid Atresia occurs when the tricuspid valve (located between the right ventricle and atrium) fails to develop. Since blood has no way of entering the right ventricle, it is very underdeveloped (hypoplastic). This makes TA a single ventricle heart defect, with only the left ventricle able to pump effectively. 

Blood is normally pumped to the lungs by the right ventricle, so how does it make it there in the setting of tricuspid atresia? A VSD (hole beween the ventricles) may exist, allowing blood from the left ventricle to shunt through it into the right ventricle and then to the lungs. If no VSD is present, the baby will rely on the PDA (a connection between the aorta and pulmonary artery normally present in all newborns that closes in the first few days of life) for blood to reach the lungs. A prostaglandin drip will be started to keep the PDA open. 

Ultimately, tricuspid atresia will require at least three open heart surgeries used to repair single ventricle defects. The Norwood operation within the few days of life (where a BT shunt is placed between the aorta and pulmonary artery), the Glenn several months later (where the SVC is connected to the pulmonary artery), and the Fontan within the next few years (where the IVC is connected to the pulmonary artery). Blood flow will ultimately bypass the right side of the heart entirely, passively entering the lungs through the new connections made to the pulmonary artery. 

needed to vent about stuff, soo i decided to write thiss.

 

Hey, I’m Jenniffer and this is my story(:

   When I was born, I was born with a rare/ severe form of Congenital Heart Disease, Congenital heart disease is a malformation of your heart involving your heart chambers, heart valves or major blood vessels that is present at birth. When I was born I was technically born dead, yeah i know it sounds stupid but its true, I wasent breathing and I was bright blue, doctors brought me back to life. I have bicuspid aortic valve stenosis, wich means that I was only born with with 2 flaps on my aortic valve instead of 3, it makes it hard to get enough blood and oxygen through out my body. & I had 4 holes in my heart && My heart is way to big for my body.

     I was told my whole like that as long as I took care of my body, the next oporation i would need could wait till i was about 17… December 19 2010, the day my depression started, that day I went for a cardiologist appointment like usual, but this time was far from normal, I went for a EKG, and I could tell something wasent right from the look on her face, I then went to meet with the doctors and the first words out of his mouth were “I’m sorry to say that things are getting really bad really fast, sugery is needed as soon as possable” I thought I was going to die, right there just.. die. I couldnt beleive it, all my life i was promised this could wait, and now im being told that im getting emergency sugery.

     That day the doctors told me that sugery would be set for April 20th.  And I was going to get my Aortic valve replaced with a pig valve.  The next couple months were pure hell, I’ve never felt this scared/ depressed inn my life, but I kept on smiling and pretending like everythings okay, thats what I do best.  April 20th came, I woke up at 4 in the morning to get to the hospital by 6. I got there and they put me in this room with a gown on, and i had to take this nastyy medicine, but i kept on smiling.

     The time came, doctors pushed me down stairs to say my good byes to my family, I dont think I’ve ever cried that hard in my life, Then, they took my back into the oporation room, i was balling my eyes out, they started putting IV’s in me, and putting the mask with the sleeping medicine on, i was still crying because i was soo scared, the last thing i remember was the nurse giving me a teddy bear and telling me that everything was going to end up all right, She lied.

     Nothing ended up right, I came out of sugery more fucked up then before.  During sugery they had to break apart all of my ribs to get to my heart Never have i been in more pain in my life when I woke up. The pain started to get impossable to deal with, so we went to the doctors to see what was wrong, all of the doctorrs told me that i was imaging the pain and that it was alll just in my head. They thought I was crazy. They sent me to two different theripist. You know how fucking much it hurts to have all of them think your fucking crazy?  I killed me on the inside, Never have I thought that seriously about suicide. 

     Summer came, it was the worst summer of my life.  I couldnt do anything because everyone was scared was guna hurt something.  I started haveing way more pain then normal, I was rushed to the Emergency room atlease 4 times a week, there would be these times where my shoulders and my ribs would start to hurt sooo bad, and when that started getting painful I couldnt breath. Doctors didnt know what was wrong, all they knew is that there was an air bubbly in part of my heart.  So they just gave me more and more painkillers, untill one emergency room trip when the doctors told me I had a small heart attack. Me. Fourteen. Haveing a heart attack? As if my life didnt suck enough I got that news.

     Summer  passed, school started. My freshman year, and I’m spending it like this.  So far I'v missed about 75% of school because I’m in sooo much pain that I can’t get out of bed. In October I started Physical Therapy, my theripist thought t was weird that I couldnt let anything touch the skin around all three of my scars, she also though it was weird that I couldnt move my arm.  She started to realized that my shoulder has slipped and is completely out of place, right after she found this out i got sick, real sick. I was fighting a virus that was eating away at my muscles and if we didnt stop it it would start to eat my organs. I stayed strong and i wasent about to give up, I pulled through and fought it. I won but pretty much all of my muscles were gone in my upper body.  I was slowly dieing.

     Time went on, pain got worse, but I kept on smiling.  I was seeing soo many different doctors, i had a cardiologist, pain management doctor, orthipedic sergeon, my sergeon, pediatrician, ER doctors and lots more.  My shoulder has now come completely out of place, and is half way down my back.  No doctors knew what was wrong, all they could tell me is I have two broken ribs, an air bubble in my heart and severe nerve damage.  About a month after of dealing with that pain I went in for a electromyogram test, An electromyogram (EMG) is a test that is used to record the electrical activity of muscles.  The test didnt show anything, all we found out was that i have RSS, Reflex Sympathetic Dystrophy syndrom.  That means that during sugery something got messed up and now the ends of my nerves are messed up and are extremely sensitive.

     The only problem is, theres only on person in the country that knows how to cure it, but the cure isnt FDA approved, and theres a high chance that after getting treated for that I wouldnt make it out alive. So theres a small chance that I’ll get to get this syndrome cured.  They still don’t know whats wrong with my shoulder and ribs Doctors said theyve only seen one recovery from this kind of sugery this bad within the past hundred years.  I’m set to go get an MRI in a couple days then I’ll go meet with a specialist to hopefully figure out whats wrong.  So I can finally get a chance to experence what a normal life is like, like i was promised when i went if for the sugery in the first place and so  no longer have to live in constant fear of dieing.