Glossary of Native Terms
(or words Microsoft refuses to acknowledge)
Note: This glossary is directly relevant to the text in the narrative book Heart Warriors, A Family Faces Congenital Heart Disease. I do not go into great detail about heart defects Liam does not have, treatments he would not receive, or experiences common to many CHD patients, but not to us.
For instance, Liam’s never crashed or stroked (that I know of), so nothing about that is included. He never had a J or G tube and had an NG tube only briefly, so nothing about feeding issues is in here either. That said stroke and feeding issues are huge complications faced by many but not most CHD families.
Watch my website, http://www.amandaroseadams.com, for more detailed information about congenital heart disease, collaborative care, stories from other families, and additional resources for the Heartland.
Disclaimer: While the information in this glossary may prove a helpful starting point, it IS NOT and is not meant to be comprehensive. No matter what you read in any book or on the Internet ALWAYS consult your pediatric cardiologist with your questions (or your adult congenital heart specialist if you’re a teen or adult). If they don’t answer you (and I’m not saying if they don’t give you the answer you want to hear, but if they ignore you outright) find a new doctor and ask that one. It is your right and your responsibility to own your healthcare information, participate as a team member in decisions about your care, and be respected in that role by your healthcare providers. Sometimes you have to work harder and reach further. I’m sorry, but that’s just how it is.
Atria: The average healthy human heart has two atrial chambers. The left atrium receives oxygenated blood from the lungs through the pulmonary vein. The right atrium receives deoxygenated blood the body has already used from the superior vena cava and inferior vena cava. After his first open-heart surgery, Liam was left with one large atrium that receives blood from the pulmonary vein and both the inferior and superior vena cava. During his second open-heart surgery the superior vena cava was removed and attached to his pulmonary branch artery. This was redone during his third open-heart surgery During his fourth open-heart surgery Liam’s inferior vena cava was removed and a Fontan conduit sewn to the side of his atria to allow blood overflow back into the heart. The conduit was then routed to flow into his pulmonary branch arteries as well. Now there is no systemic venous blood flow to Liam’s heart at all.
Atrial fibrillation (A-Fib): Liam may or may not have been born with A-Fib. Because the area of the heart that causes this issue is close to parts of Liam’s heart that were missing or malformed, A-Fib may be endemic to his other birth defects, but surgery probably made it worse. After Liam’s second major surgery, the Glenn shunt, he had a prolonged episode of A-Fib as his heart’s swelling decreased. This repeated itself for every surgery and cath since. Now Liam is in a persistent state of entering and leaving A-Fib wherein his sinus node fails and his atrium or upper chamber (he only has one –see the previous and subsequent entries) wiggles instead of squeezes. As long as Liam’s ventricle squeezed in response, Liam avoided a pacemaker. Now, the A-fib is persistent enough that medication can’t stop it, and Liam needs a pacemaker in the near future.
Atrial Septal Defect: A hole between the two upper chambers of the heart. Liam had a couple of small ones that became non-issues when his entire atrial septum was surgically removed by Dr. Starnes during the DKS procedure. For some children with CHD, this is the only heart defect they have and it is highly treatable if diagnosed early.
Aorta: In a healthy heart, the aorta ascends from the dominate left ventricle to feed the body oxygen-rich blood. Liam’s aorta not only ascended from the wrong side of his heart, but because the pulmonary valve it was attached too was feeding it a mere trickle of blood, it developed to be about 10% of the size it should have been to sustain his life. Additionally, this hyoplastic aorta failed to provide significant volume to feed his aortic arch, so he had a severe coarctation as well. During his first surgery, Liam’s aorta was dissected from his pulmonary valve and grafted to his pulmonary artery to allow the blood volume from the left side of his heart to feed his body.
Aortic Arch: is fed by the aorta and diverts oxygen rich blood to the brain and arms. Liam had a severe kink (coArctation) in his aortic arch, which could lead to massive stroke if untreated. Dr. Starnes surgically corrected Liam’s coarctaion. Like an ASD, a CoArc is sometimes the only heart defect a child might have and while a dangerous defect if untreated, is frequently treated in a cath lab now. Liam’s condition was too complex and required surgical intervention.
Aortic Valve: is the valve that, in a healthy heart, is found on the left ventricle of the heart and passes oxygen rich blood to the aorta. Liam’s aortic valve was attached to his pulmonary artery, so the left side of his heart was pumping blood to his lungs. However, due to his fetal heart structures, this blood was diverted to his aortic arch and allowed him to develop in utero. Had those structures failed at birth, Liam would have flooded his lungs with blood and died rapidly of oxygen deficiency to the brain or a pulmonary embolism. This is why in utero heart screening mid term is so vital.
Cardiac MRI: Also called MRA, this is a magnetic resonance imaging screen of the heart itself. Liam had one before his Fon-tempt. If Liam gets a pacemaker in coming years, MRA will no longer be an option for him. Though it does not measure venous or lung pressure like a cardiac cath, it is less risky and a nice option for patients and cardiologists.
Cardiac Anesthesiologist, Pediatric Variety: This M.D. is usually a pediatrician who does fellowships in both cardiology and anesthesiology in order to administer anesthesia to babies and children with heart defects. Anesthesiology is complicated even in completely healthy patients, for children with deformed hearts it is both an art and a science. Without these folks, surgeries and caths for kids like Liam would be too dangerous to attempt. Kudos to these amazing doctors!
Cardiac Catheterization. Pediatric Variety: During a cardiac cath, the patient (Liam) is taken to a sterile cath lab where he is put under general anesthesia and a catheter is inserted into the femoral artery in his groin to access his heart. Sometimes, due to the DKS reconstruction of his aorta and pulmonary arteries, the doctors also accessed Liam’s carotid artery in his neck. Anyway, once the catheter is in the arteries it’s threaded into the heart to measure pressures and do all kinds of fancy things like add a stent, test closing the hole left open in Liam’s Fontan conduit, and coil off all kinds of peripheral or collateral veins that keep growing even though we don’t want them. Sigh. . . infection risk exists as does the risk of bleeding, stroke, and cardiac arrest. Also, Liam’s sinus node always gets extra persnickety when poked in a cath.
Cardiac Intensive Care Unit, Pediatric Varity: This is just a personal opinion, not backed by any medical certification of any sort, but I would never allow Liam to be treated surgically for any reason by a hospital that did not invest in a dedicated cardiac intensive care unit. Some hospitals have NICUs (Neonatal Intensive Care Unit) and PICUs (Pediatric Intensive Care Units) but no CICUs, and that’s not cutting it for my kid. Now that I know better, only the centers that do high enough volume of surgeries to hire the best staff and maintain a CICU would even be under consideration for my child. A CICU has nurses and attending critical care professionals fully devoted to the care of cardiac patients. Whereas, a hospital with only a PICU might have a trauma patient treated by the same nurse as a cardic surgery patient – not good enough says this author. At least, not good enough for my child. Any nurse or attending touching Liam must know as much or more about cardiology than I do, and that’s not always the case in every PICU where the nurses must be jacks or jills-of-all-trades to their patients. Noble, yes, honorable, absolutely! Wonderful people work in a PICU and help cancer patients and brain injury patients. I’m not disparaging them in the least. But when given the choice, as we are in America, I’ll take the CICU every time for my child with half a heart. Note that some hospitals have a “functional” CICU within their PICU where specialized nurses are assigned to cardiac patients. Ask about this if they don’t have a dedicated CICU space.
Cardiologist, General variety: If you are a sixty year old with no previous history of heart problems, this is your guy. If you are a heart warrior – stay away, far away! Seriously, regular cardiologists are amazing folks worthy of our respect, but they have NO BUSINESS treating children or adults with CHD unless they’ve done a fellowship in pediatric cardiology OR the emerging field of Adult Congenital Heart Disease. No fellowship or experience with CHD – no touching my kid, not even when he’s fifty. Cardiologist, Pediatric variety: Here’s your man (or woman). This doctor is first and foremost a PEDIATRICIAN who treats the developing bodies of children and then a cardiologist versed in all the possible known permeations of malformed hearts and their associated venous and arterial structures. This guy/gal knows the hemodynamics of an altered heart and the impact on the liver, digestive, and respiratory systems. Your PedCard MUST answer your questions. He/she is not remotely obligated to tell you what you want to hear, but this person should be honest with you and help you understand the situation so you can make informed decisions. If he/she doesn’t, even if you have to go a thousand miles to find one who does, get thyself to a better communicator. Note: There is an emerging field of ADULT CHD doctors. These are not necessarily pediatricians. They may be internists or general cardiologist who go on to purposefully study heart defects and their treatments and how they affect the adult body. We need these people because the pediatric cardiologist is primarily a pediatrician – which is exactly what you need for the first sixteen to eighteen years – then you need the new guy. And with more and more children surviving CHD into adulthood, we need a lot more of these experts.
Cardiothoracic Surgeon, Pediatric/CHD variety: (see also God – just kidding God, please don’t smote me!). This person, when he (I’ve met two she’s – so there are some, but typically it’s still a ‘he’) is at his best saves several lives per week. When he’s at his worst, he should retire. This is a dangerous and difficult job, saving babies many wouldn’t ever touch. When choosing a cardiothoracic surgeon (and yes in MOST cases you really do get to choose and if you don’t feel like you’re getting options – stamp your feet until someone pays attention) always, always, always expect them to explain the surgery in detail and if they don’t offer, you ask:
1) How many of this particular type of procedure have they done in their career?
2) How many at THIS hospital?
3) What’s the morbidity rate for patients with this procedure?
4) Is that rate higher or lower than the national average?
5) Do you perform heart surgery on adults who don’t have CHDs, and how often? (RED FLAG – if this doctor doesn’t have enough business just doing CHD surgeries you should find one who does). A very esteemed surgeon told me that if hospital’s have mortality rates of higher than 3-5% on Norwoods, BT Shunts, Glenns and Fontans, they’re not doing enough of them and they’re not doing them well enough. These are 2010 numbers, not 2003 when Liam was still on the forefront of surgical successes. So if they tell you 10% or 20% in 2012 and it’s not a new procedure for the rarest of the rare defects that I’ve never even heard of – get thyself on the phone with thy insurance company for a second opinion. (If you have to be airlifted to save your child’s life – just think about that for a second – do you really have to think about it?)
Central Line (See Also Central Venus Catheter in more scholarly texts): A central line is like an IV but the catheter for the fluid is threaded from the entry point (in Liam’s case always the neck or groin, but can be placed in the chest) all the way to the heart and can administer medication as well as venous pressures. They also withdraw blood from the line to measure blood gasses, which gives the most accurate oxygenation reading. During postoperative recovery, this is one of the first things to go because it is pretty risky to have this plastic thing hanging around inside your swollen heart – infection and what-not. PICC lines are the same but they’re threaded from the arm instead of neck/groin/chest. Liam had a PICC line for over six weeks and we had to take him home with it to administer his Vancomyacin in 2006.
Chest tubes: Liam has had twenty-one in his life. They are thin plastic tubes that stretch for stripping of fluid which is collected and measured, and sometimes sent to pathology. During surgery small x-shaped incisions are made in the torso, and the tubes are inserted to release fluid or air from around the lungs and prevent wet or collapsed lungs. They are very painful to have pulled, and the removal should always include a sedative and a pain medication (Versed of Chloral Hydrate + Morphine or Fentanyl) beforehand. The holes need to be stitched and leave pucker-shaped scars. According to Leslie McCall, chest tubes were the most painful part of open-heart surgery.
Chloral hydrate: Medicine that makes babies and children very sleepy before surgery or other procedures. Administered in liquid form, Liam hates it. So, I didn’t let them give it to him before his caths in the summer of 2008, which made him traumatized by being gassed. I will regret it for the rest of my life. Wish I had my own Chloral hydrate right now . . . sniff. Don’t skip the chloral, better to fight to get it down than fight to get them unconscious.
Coarctation: a kink or constriction of tissue in the aortic arch.
Damus, Kaye, Stansel Procedure (DKS) or Modified Norwood: In Liam’s case, Dr. Starnes grafted/patched Liam’s aorta and pulmonary artery together. He also removed his atrial septum, creating only one upper chamber in the heart. Before surgery, Liam’s pulmonary blood flow from his heart to lungs came from his pulmonary artery which came off the left side of his heart. His blood flow crossed his ventricular spetal defect and drained, rather than was pumped out of an opening where his pulmonary valve would be if his heart formed properly into his aorta which was also on the wrong side of the heart. This would have been purely depleted oxygen if not for it mixing together in his heart. He could not live like this and his organs were suffering from oxygen depletion including his kidneys, liver, and the heart muscle itself. During the surgery, Dr. Starnes added a Gore-Tex® tube to replace pulmonary blood flow. As the aorta was grafted to the pulmonary artery to supply blood to the body, the tube was grafted to another artery (I honestly don’t know which one and it stymies cardiologists to this day who see his heart on an echo) to supply blood to the lungs. This artificial tube was a modified Blalock-Taussig Shunt and was replaced during Liam’s Glenn procedure three months and one week later. Finally, during this surgery, Dr. Starnes removed a section of Liam’s aortic arch (coarctation) and reconnected the ascending aorta. In all, you could say, Liam had four different open-heart surgeries in one, and all in under an hour on an organ the size of a small egg or golf ball. At the time the mortality rate for this procedure was 20%.
Digoxin: In Liam’s case this is used to stop his atrial flutter. His atrial flutter is caused because Liam’s heart’s all scarred up and doesn’t contract properly. This can cause clotting issues because the heart is not pumping regularly. It’s possible, without treatment (Digoxin) he’ll need a pacemaker to control his atrial flutter. Digoxin has not prevented his atrial fibulation though.
Ductus arteriosus: is a connection between an unborn baby’s pulmonary artery and his aortic arch. Because an unborn baby doesn’t use his lungs, the ductus arteriosus diverts the blood that would normally go to the lungs back to the body for use. Since the mother’s body oxygenates her unborn baby’s blood, this flow is sustained until birth. See also: Foramen ovale and Ductus venosus. After Liam was born, this connection was kept open by prostaglandin for two weeks until his DKS surgery.
Ductus venosus: is a connection between an unborn baby’s inferior vena cava and his umbilical cord. This allows his mother’s oxygen to enter his body, travel to his heart, and be pumped to his aorta through the pulmonary artery’s ductus arterious connection with the aortic arch.
Echocardiogram (Echo): This is a sonogram or ultrasound of the heart. Liam’s first echo was with Dr. Doom, and he has had at least seventy of these procedures including transesophageal echos through his throat and esophagus during surgery to make sure he had no internal bleeding before they closed his chest. I can now tell what structures we’re looking at during an echo and have explained them to Liam at his last echo so he understood. As a baby/toddler Liam had to be sedated with hydrochloral or Versed to be subdued for an echo, now he asks questions. This is one of the least invasive and most useful diagnostic tools.
Enalapril (Captopril): This is a blood pressure medicine that Liam takes and has taken since he was two weeks old to ease the pressure of altered blood flow on his narrowed and constricted vascular system.
Fentanyl: A very strong drug that works fast to stop intense pain. Liam had double the recommended dose before his sternal reset in 2006, and he was still in extreme pain. Wikipedia says it’s 100 times more powerful than morphine, and yet he screamed.
Flat as a Pancake: After a cath, patients must lay absolutely still to prevent their femoral arteries from “popping a clot,” and bleeding out. This was particularly difficult when Liam threw up bile and then dry heaved after his last two heart caths. . . sigh. Popping a clot when Liam was on blood thinners to get that cath into his heart led to sandbags being placed on his groin, even as he continued to dry heave . . . sigh . . . so much blood – not a nice memory. “Flat as a pancake, sweetheart, flat as a pancake,” says I.
Foley Catheter: If you’ve ever had surgery you know what this is. If not – it’s a tube that goes into the urethra to collect urine, which is then weighed and compared to the amount of saline administered to determine if a patient is retaining too much fluid or is too dehydrated. It’s one of the last things to go during the postoperative phase and is a sure sign that recovery is imminent.
Fontan Procedure: This is the surgery where the inferior vena cava is dissected from the heart and attached to the artificial conduit that then connects to the pulmonary branch arteries. In Liam’s case, the conduit was sewn to his atria and a hole placed in his atria between the conduit and his heart so that if too much blood backed up in his lungs, it could be pushed back through the hole in his heart. That hole is called a fenstration, and because the pressure in Liam’s hypoplastic arteries was so high in 2006, it was larger than average, as was the conduit (they run 16-23 mm and Liam’s is 22 mm). Smaller conduits are used on children who can tolerate higher pressures. Note: There is an older type of Fontan procedure called the lateral tunnel or LT Fontan, where a baffle is run through the atria instead of outside of it (extra cardiac or EC Fontan is the type Liam had). In my observation of hundreds of families, LT Fontans are increasingly less common, but are still done. I’m certain any surgeon who still does LT Fontans has a very good reason based on the patient’s anatomy to do it that way. If that approach or a human or animal homograft instead of a Gortex conduit is used in an EC Fontan you should talk to your surgeon about why those differences are necessary for your child. I’m not remotely suggesting that you question their wisdom, but having it explained to you will teach you more about your own child, and that is always a good thing. You’re simply asking for education not justification.
Fon-tempt/Glenn-Again: Liam’s left and right pulmonary arteries had grown hypoplastic from the diminished blood flow of both the BT shunt and the Glenn flow over time. His arteries were only two millimeters and needed to be eight to support the increased blood flow of the Fontan. This was untenable, so while Liam’s heart was stopped his arteries were splayed and Gortex patches were applied to make the arteries bigger. The junction of the superior vena cava to the pulmonary branch artery was also moved to a new position and the artery repaired where it was removed.
Foramen ovale: this is a natural, not defective, hole between the right and left atria in an unborn baby’s heart. This hole allows more oxygenated blood passed by the ductus venosus to the right atria through the inferior vena cava to make its way to the baby’s brain by pumping out the left side to the aorta. This hole was kept open with prostaglandin in Liam’s body to allow continued mixing. His other atrial septal defects and the Foramen ovale were cut away during the DKS surgery when the entire septum was removed. In some babies the hole won’t close naturally and is then considered a birth defect and may be surgically corrected like an ASD later in life.
Glenn Shunt Procedure, Bidirectional: Liam’s superior vena cava was dissected from his heart and attached to his pulmonary branch arteries to drain blood returning from his arms, head, and upper torso directly to his lungs. This is a common procedure and done in most children with single ventricle hearts, even if a Fontan is avoided in some cases. At one point postoperatively, his oxygen saturation was 44% and he was the color of blueberries. He improved, but his sinus rhythm was never fully restored and the Glenn had to be redone seventeen months later.
Hypoplastic: means, essentially, too small to function properly. Liam’s right ventricle was hypoplastic, meaning it was too small to function. His aorta was hypoplastic, meaning too small to function as an aorta. His pulmonary branch arteries were also hypoplastic.
Mitral Valve: the valve between the left atria and the left ventricle. Because Liam only has one atria and one functioning ventricle, this is his only internal heart valve. It currently regurgitates (throws blood back up into the atria when the left ventricle squeezes instead of passing all of the blood through from the atria to the ventricle and keeping it there). This regurgitation is worrisome and may lead to future procedures for Liam.
PICC Line: See Central Line
Prostaglandin (PGE): are human messenger molecules that control smooth muscle tissue and prevent the fetal heart structures from closing on themselves until the diminished amount of PGE in the neonate’s body after birth signals those structures to close. Since Liam needed his fetal heart structures to remain open for him to survive until his first surgery, he was on a continuous PGE drip for his first fourteen days of life.
Pulmonary Artery: is the artery that, in a healthy heart, ascends from the right ventricle to feed the two pulmonary branch arteries that provide deoxygenated blood to the lungs for oxygenation. That blood is then passed back to the left atria by the pulmonary veins. Liam’s pulmonary artery ascended from his aortic valve on the left side of his heart. It was feeding his aortic arch through the ductus arteriosus. If this connection had been closed before surgery, Liam would have lost blood to his brain and either stroked or simply died. Because the right side of his heart was malformed due to the missing tricuspid valve, his pulmonary valve was not functional, and his pulmonary artery could not be moved to the pulmonary valve. Therefore, the aorta was removed from the pulmonary valve and grafted to his pulmonary artery. Now his pulmonary artery serves as his aorta.
Pulmonary Valve: is the valve that, in a health heart, feeds the deoxygenated blood from the right ventricle to the pulmonary artery for delivery to the lungs.
Second Opinions: I’ve never sought one, but I would in a second if I questioned decisions or the options presented to me. We’ve been very blessed with good cardiologists and a strong team approach at both hospitals where Liam received heart surgery. That said, if he needed another one tomorrow, it would not be a planned experience and it would not be typical, so in that case I would be likely to seek a second opinion. No parent or patient should feel awkward or outside of their rights to seek a second opinion. If the other doctor/institution confirms what you’re being told at the first, it just elevates your confidence. If you’re given better options, then it was entirely worth doing.
Stent: A stent is a wire mesh tube that is inserted into a vein or artery to open in wider to allow adequate blood flow either toward or away from the heart. Liam has a stent placed in his left pulmonary branch artery (LPA) because that stretch of artery, despite being augmented twice surgically, was still too constricted to allow adequate blood flow to his lungs. Liam’s stent covered part of his lung tissue and had to be pried open in two or three places to keep blood flow moving to his lungs. The stent helped, but the placement during a cath procedure is somewhat risky.
Tricuspid Atresia: is the absence of a tricuspid valve which results in the absence of a functional right ventricle.
Tricuspid Valve: is, in a healthy heart, the valve that allows blood from the right atria to pass to the right ventricle on its way to the lungs. Liam had absolutely no right ventricle. Rather, he has what might best be described as a knot, like in a piece of wood, but no opening at all on the wall of what was his right atria. Oxygen-rich blood that entered the right side of his heart through the inferior vena cava (fed by the ductus venosus and his umbilical cord) sloshed across his foramen ovale and other holes between his two atrium, ending up in the left atrium and feeding his left ventricle, where it sloshed back to the right ventricle and fed his aorta. Because the blood volume to the right ventricle was so limited the muscle structure never grew large enough to be a functioning chamber of his heart, and Liam only survived gestation because of the foramen ovale, ductus venosus, and ductus arteriosus feeding his oxygenated blood to his body.
Vancomycin: In 2006 this was the strongest antibiotic on the market and used (I know it was at least six and it may have been eight) to treat Liam’s near-fatal staph and strep infection. We had to administer it ourselves three times a day once Liam was home.
Ventricular Septal Defect (VSD): This is a hole between the left and right ventricles. In some children it’s a serious heart defect that can be treated either in a cath lab with a patch that cells grow over and close or in open-heart surgery where it is sewn shut. It’s highly treatable when it is detected early and the only CHD present. However, in Liam’s case it was the one defect that kept him alive the first two weeks so any blood at all could get to his body from his malformed (practically nonexistent) right ventricle that was mis-attached to his aorta. A few months ago during an Echo, I could see Liam’s right ventricle as a little spatter of blood crossed his VSD. It looked like a tiny little closet or alcove under the stairs against a larger room – his left ventricle. Liam saw it too and it was his first inkling about what his heart looks like and why it’s “special.”
Versed: Happy juice that made Liam fail to remember having chest tubes pulled, PICC lines placed and a number of other indignities over the years.
Viagra: Invented for heart disease – its side effect was a marketing gold mine. Several of our very young friends have been on this drug following the Fontan to reduce pulmonary artery pressure that might have caused Fontan failure. It was suggested for Liam if the arterial stent were to fail to bring his oxygen saturation up, but we’ve avoided it to date.