I had a reader* (on my family blog) ask a question the other day that I figured some others may be wondering. I also think answering it could potentially help educate others about single ventricle heart disease, and education is POWER and could mean another child going from orphan to a son or daughter. So here goes.
Way back in March, a team of volunteers from Love Without Boundaries traveled to Guizhou Province and visited several orphanages. In one of those orphanages, they found a little baby girl who was not doing well and who appeared to have heart disease, which was apparent in her blue-tinged lips and other signs I’m assuming involving her breathing and possibly her fingers and toes (clubbing). By April, LWB was given permission to seek evaluation and possible treatment for this now 14-month-old baby girl in Shanghai. They also in April pleaded with supporters for funding for several children including a little girl they had given the name Keely. Within weeks, this little baby girl was receiving a much-needed, open-heart surgery in Shanghai. I wish I could share a face*book post link, but if there is a way I haven’t figured it out! Anyway, LWB posted many updates on their face*book page about the little girl they call Keely when she was in Shanghai receiving surgery for her heart
She received what is more commonly called a glenn shunt. This link is a very layman’s terms description of the Glenn shunt. It has pictures. If you are really adventurous, I have found youtube videos of the operation and I’ll be honest: I have watched them and find them absolutely amazing and fascinating. The Prez thinks I’m a bit off, but I really found watching them helped me understand it a lot better and to see a surgeon working with such a tiny heart—amazing and miraculous.
**If you would like to see the link, I do have it posted on my family blog. I do not have it posted here because it is graphic. I just want to be careful and not offend! I find it fascinating and informational, but I realize others may not. You can also google you*tube and glenn shunt to find it if interested.**
After the Glenn shunt in early May, this baby girl was now receiving more richly oxygenated blood to her head and upper body. Still, the lower half of her body and lower limbs are still receiving very blue (oxygen-poor) blood. She needs another surgery, which is called the fontan in order to COMPLETE the fontan circulation.
To answer Trish’s question, yes, LWB provided her with a much-needed and life-saving surgery, but she needs another surgery. She needs the full fontan circulation completed. This surgery she had in Shanghai though not only gave her a better chance for the full fontan circulation to be completed, it greatly improved her quality of life and her chances of being adopted. If I’m being completely honest, the Prez and I found a huge amount of relief and peace in knowing she was given such an incredible gift and chance. Our first daughter didn’t receive this surgery and time took its toll on her little heart as she waited and FOUGHT to live. I still get totally overwhelmed when I think of what our Li’l Miss ENDURED. We watched her coming home video again today and I have a snippet of video with her labored breathing … every single time I am overcome when I remember seeing her suffering like that. NO CHILD should have to suffer like that.
Our little girl, who is the same little girl known as Keely with Love Without Boundaries and who we will meet in 15 DAYS!, has single-ventricle cardiac disease just like our Li’l Miss has. Well, their defects are somewhat different but ultimately they both were born with one formed ventricle instead of two, and both needed the fontan in order to live the most full life possible with current medical advances. There is no cure or heart repair for single ventricle disease, but the glenn shunt and hopefully following that the fontan will give our waiting daughter the chance to lead a full life. She also has been diagnosed with dextrocardia, which by itself can by a totally non-issue, but with a complex defect such as single ventricle it certainly complicates matters. We are also unsure of possible other defects because we have received some conflicting information. Rather than pursue a lot of clarification, we decided to move forward because clarification was not going to change our decision to pursue her adoption nor was it going to significantly affect the fact that we will not know her surgical options until she is home and can be evaluated by a cardiologist and particularly through cardiac catheterization.
This page from Cincinatti Children’s Hospital is one that I find extremely helpful for a laymen like me to understand our daughter’s heart defects. You can click through and read about treatment options and various considerations.
Trisha also wondered why the urgency if our daughter already received life-saving surgery and if she is “doing well” as was stated recently on a post about her. While she is doing well right now, ideally she needs the fontan circulation to be completed (the lower vena cava to be rerouted to bypass the pumping chambers of the heart and flow directly by pressure into the lungs) and for it to be done sooner than later. While neither of these operations is considered curative, the optimum outcome for her would be a completed fontan circulation.
It is important I think to note here that the fontan operation is a last resort. It is not optimum, but it is what is available to better a child’s life and give them a chance to live a life without being on oxygen hopefully and to experience everyday things that many of us take for granted. But ultimately, the fontan bypasses the pumping chambers of the heart because the defects are so complex that this is BETTER than allowing the blood to flow through the heart. I still have a drawing our amazing Dr. B, the surgeon who performed Li’l Miss’s fontan and who will perform Li’l Bit’s as well God willing she is operable, gave us as he explained in a way we could understand exactly what it was he would be doing to our Li’l Miss’s heart. I saved it for her to have someday. I will never forget sitting in her hospital room that night, the night before he would operate on her heart, and hearing him tell us the risks, him having to ask us to sign the consent and his obvious grace toward us in that moment, and ultimately him saying as he stood, “It is in the hands of her Maker.” Yes it is. Even today, that is it. Our Li’l Bit’s heart is in the hands of her Maker.
On that same page from Cincinatti Children’s Heart Encyclopedia, you can read this:
For a heart with a Fontan reconstruction to work well, there are a few crucial features that must be maintained. These key factors must be kept in mind when planning the medical or surgical management of children with single ventricle defects from the first day of life onward.
The single ventricle must not be overworked for a long period of time, in terms of either having to pump too much blood or pump at too high a pressure.
The pulmonary arteries must grow well without stenosis (narrowing) and must remain low resistance (or be very relaxed). If the pulmonary arteries are narrow or if the resistance in these vessels is high, blood will not be able to flow into them without a pump, so the Fontan will not be successful.
Lastly, leaky or tight valves may adversely affect the function of the ventricle or the flow of blood to the lungs.
I bolded the parts above because this
information is crucial to understanding why the urgency and also why what LWB and its supporters provided is so amazing. Not only did the shunt operation she had done buy time so to speak, but it GREATLY INCREASES her chances for a successful and complete fontan circulation by hopefully protecting her lungs and pulmonary arteries from high pressures that could make a fontan impossible.
We know personally how NOT getting the glenn shunt adversely affects a child. We saw with our own eyes how sick our older daughter was. She was 35 months old and never received the glenn shunt which our Li’l Bit has had done thanks to LWB and its supporters.
As far as the urgency it is mult-faceted. The ideal time for completing the fontan is two to three years of age. Not only that, the longer our daughter waits for the full palliation, the more chance she could become ill and not be able to fight it and also the more chance her heart is being overworked (and could be enlarged), and finally the more chance irreversible damage is being done to her lungs and/or pulmonary arteries.
This is an excerpt from the Cincinatti Children’s page:
The third and final stage in the reconstruction of a single ventricle heart defect is the Fontan completion operation. This operation is usually performed at 2 or 3 years of age, based on the child’s size and clinical status.
On that note, the optimum time for a child in the US to receive the hemi-fontan, or glenn shunt, is by six months of age. Our daughter received it at 14 months of age. We don’t know what happened with her heart and lungs and pulmonary arteries while she waited so long. What we do know is that she survived for all of that time, that she made it through the surgery and that she is doing well right now.
I also want to add that children with single ventricle heart disease are very susceptible to infections, to *regular* childhood illnesses being much more serious for them, to the lack of oxygen causing secondary problems such as poor brain development, learning disabilities, damage to the lungs which could be irreversible. While the surgery LWB provided most likely improved our daughter’s oxygenation from the 40s or 50s to the 70s or 80s, this is still not ideal. A normal heart will function in such a way that the body will receive blood with an oxygen saturation of 99% or better. So, 80% oxygen saturation is certainly better than 40 or 50% saturation, but is not something ideal for the long term. It is very possible our daughter could live with just a hemi-fontan and at 70-80% oxygen saturation, but we are hopeful that she can receive the completed fontan circulation and hopefully 95% or better oxygen saturation. For reference, Li’l Miss had oxygen saturation of 40% when she came home and 70-80% after her complete fontan repair. However, due to her case of having no partial fontan done, her surgeon felt it necessary to leave a fenestration (a type of pop-off valve if you will to allow pressure releases) in her heart. About a year after her fontan operation, the fenestration was closed surgically in the cath lab. Since that time, Li’l Miss has held steady with oxygen saturation of 96-99%.
I want to touch on something I am often asked about: life expectancy. I copied this from that Cincinatti Children’s page as well because it explains what we know so well (and also a lot of what we just don’t know):
The limitations children experience due to their heart defect, though, can vary greatly. At one end of the spectrum there are children with Fontan circulations who have participated in competitive sports such as swimming and gymnastics.
Other children may have significant limitation in their capacity for exercise. Most children fall somewhere in between the extremes.
Most children are on a blood thinner called coumadin after their Fontan to prevent clots from forming in the Fontan circuit. When a child is on a blood thinner, you must take extra careful to avoid falls or head trauma as they are at increased risk for internal bleeding.
How long a heart with a single ventricle reconstruction can function is not known. The first children to have a successful Fontan operation are just now 30 years old and many improvements in surgical technique and medical management have occurred over this time period.
Late complications including irregular rhythms and heart failure may occur. Some speculate that most single ventricle hearts will not function efficiently beyond 30 to 40 years, but improvements in surgical technique and medical care may increase this age significantly. In some cases, if the ventricular function deteriorates significantly, heart transplantation may be considered. Because of the possibility of late complications in patients with single ventricle anamoly, continued regular follow-up with a cardiologist for the life of a patient is essential.
So in all honesty, we don’t know how long our little girls born with single ventricle disease will live. But who of us does know? What we do know is that they are amazing people and they deserve every good thing life has to offer, including a forever family. We also know our Li’l Miss has brought great joy and love to our family and occupies a place in our hearts that we never knew needed filing, and we don’t doubt our Li’l Bit will do the same.
We hope she is able to have the completed fontan circulation. We hope in the Lord ultimately for her future. He is the only Hope we have.
*I am cross-posting this from my blog. There are so many children who wait with single ventricle heart disease. I hope by sharing more, others will come to a place of feeling led to take that leap and bring one of these amazing children home. In that regard, please email me (cljjs (at) yahoo (dot) com) or leave a comment if you know of children who wait with single ventricle heart disease. I’d love to advocate for them.
Greetings: Thank you for your article. I have been presented with a child from China with a single ventricle, he is currently at Morning Star foundation in Beijing – Noah. I spoke wiht a cardiologist this afternoon at length about the risks and lifestyle related to this. I cannot recall the last time I teared up and now cannot seem to cease; I am uncertain whether to engage in the adoption of this beautiful child, I am a single parent. Advocacy may assist Noah in that apparently no surgeries have been authorized for him out of Morning Star but clearly are needed. Thanks, Samantha
We adopted our son 4 years ago from Ukraine. He also had a complex congenital heart defect. He has Glenn surgery at the age of 3 in Ukraine, but had the fontan completion done at the University of Iowa at age 7 (we adopted him at age 5). He has done very well, but does have to have regular check ups with the cardiologist.
If you are really interested in adopting a kid, then i think you should move forward with adopting this kid whom you are presented with. I believe that these children are gifts of god. Coz even am one among them. I had underwent a complex heart surgery for single ventricle at the age of 16 and 17. And today am very happy. My parents are proud of me. I believe that every single ventricle kid will have a power inside him/her which just drives them. It had been since 8 yrs am living after the procedure. And am completely healthy and am able to workout for nearly an hour everyday without any stress.
If you want clarifications for any other queries, feel free to mail me at email@example.com.
My daughter is 17 1/2 years old and was born with single ventricle heart defects as well as situs inversus. Before the age of 3 she went through a pulmonary artery banding, the Glenn shunt procedure and the Fontan procedure. Until Christmas time 2011 she appeared very normal and healthy to people who were not aware of her situation. December 23, 2011 my daughter had a significant stroke. Apparently she had smaller unnoticable strokes prior the that one and a few more since. We were told that it was a possible side effect of the Fontan procedure but a blood clot had also passed through to her brain. I just wanted to share this information because when I rushed her to the ER they assumed she had overdosed on drugs and refused to believe she was having a stroke. Finally, three hours later she was medically transported to a better and more competant hospital.
My son 6 years old now and waiting for fonton surgery but delayed due to high PA pressure. I want to share the experience with you to educate our selves for the benifit of my son. please write me on firstname.lastname@example.org
I was born with single vent, plus other heart defects, was very limited with exertion growing up, I never had surgery as a child. But at 34yrs I had 2 strokes and a seizure, 11 months after that I had to make the decision to have the Glenn procedure done, it changed my sats from 86/88% to 90/91% and no longer blue. I am 5 months post op and am now faced with the decision to have fontan surgery to get lower extermities good oxygen as well, as this decision is a very hard one to make, I have lived a good life [exhausting but good :)] and not sure I want to challenge my body any further, but just not sure yet.
After reading all of the blogs and responses of everyone, I am still at a loss of words as to the question, how do you cope with a 9 year old boy, who went three 3 open heart surgeries by 18months old….. I must first say that my son Alexander is one of the most unique boys I have ever met.
Alexander was born with a sinle left ventricle, on the right side, and his rt ventricle, on his left side never formed. With that being said, he doesn’t have any physical or mental challenges to date. However, 3 years ago he had a catherization and his stats were ok. He O2 was a bit low in the seventies, but since then, up to now, his stats seemed well, in the 80s/90s… That being said, in the past 8 months he seemed to be getting more out of breath and was noticeably more tired more frequently and less active at times. He pushes himself to keep up with his friends and older brother so I in short had a catherization done today to see if there was any blockage, which I new in my heart, something was changing, and drastically.
The Dr at CHOP today said his device which is supposed to cover his closed dentist ration was now partially sticking out the other way, in terms, blocking the flow of blood to the arterial skeptical defect….preventing him from sustaining his heart rate due to the inability of lack of blood flow to the heart…
So, their un prognosis and still undecided course of action to fix or put a pace maker in is questionable.
I am at a complete loss of words and was under the impression that with all of the “re plumbing” work the did the 1st 18months of his life, is just that. Fixed and in long term, due to an irregular heart beat and needling a pace maker in his late teens would fix that issue…. Today, I was not given that impression. The only options are to 1. Do nothing and see how he does with the slowing down of his activity level…at 9, which I wasn’t expecting, 2. Have his go thru another open heart surgery, fix the device covering the fenistration, and risk, a possible stroke, at 9yrs old, infection, worsening the chances of making his arythmia even worse, and possible death if something else goes u expected lay wron during surgery…
So what is one to think about? Despite the fact that the Dr stated, that remember he is a fontan baby, and it will give out, no one can predict how when or why, but eventually, as a single ventricle baby, he will basically have a limited and u determined life expentency.
When I heard the last part, all I could do is think of how to take my ventricle and have them use it in him and just fix him totally. Why can’t this be done.
I have all od my plumbing where it is supposed to be and the loss of one of my ventricles is nothing to me when it would mean giving my son a more promising and longer life expectancy.
The doctor was pretty speechless, but I was dead on serious. If you can transplant someone’s heart from one body to another, why can’t they do this for a ventricle, which they can transplant the same way and re route all of his plumbing back to an original state of how it should have been in the firsts,ace.
Why am I now the only know case with a child whose device failed and broke off of how it was originally supposed to be… When I asked how many other cases he has know about with this situation, he said he has never had a device break, nonetheless cause such blockage, in all the years he has been doing catherization.
Well that wasn’t the right answer for me. So nowhere do i go with the situation of my son. Chance his life with another open heart surgery, possible stroke during surgery, or even total irrythmatic heart beat suddenly leading to possible death.
Where does comfort lie when your faced with a child who had so much zest for life that of his 9 years of existence, hasn’t had a bad day EVER?
What does one do?
Wait it out and chance the “what if I hadn’t waited” or chance the ” unknown of furthering the risk of taking away a great life now, with low activity level and wait the course of his future.
Does anyone have any guidance?
There has to be a way to save and preserve the meaning of “LIVING”. Single ventricles and how to prolong ones existence for their child has a price, but if I, his mother am willing to bare the change in my body and chance non existence , for my son to have a future, more promising than what a doctor thinks is enough for him right now, and the un foreseen future, why can’t they figure that out transplant and give my son the life I want him to have, which is a life of a future so long, that it all fall into the hand of fate.
Can anyone give me any comfort or hope of doing something, rather than just jumping into another surgery with unexpected answers????
Doing a little research before I go to the cardiologist and I stumbled on this article, I am a Fontan surgery survivor, 20 years old currently, growing up I was always told about the limitations I would have to endure as a patient, and there certainly are some I pay attention to about getting exercise and physical activity, but, I would never say I was limited in my life.
Im facing the same issues that Alexander is and I was born with the same condition as him.
The highest price you really could pay is taking your life away for his, to grow up without a mother would be too high a price for him to pay, its a gesture above all others to offer your heart to someone (quite literally) but thats awfully rash.
Take him to the right doctors, to the best hospital you can find and he will thrive, I was given little chance to live past three, but I had the right care 20 years ago, I can only imagine the type they can give him now.
p.s. the girls will go crazy over the scars
Hello, thank you for this article. I have a two year old son, and we are waiting for the second part od fontan surgery. I would like to ask for a contac of the author of this article, because it is so simmilar to our ”story”. Thank you so much! ( And I applogise for my english, but I’m from Slovenia 🙂 Nina
I have a single ventricle, but I am a fontan failure. Due to some surgical glitches I was unable to have the surgery. When I was told that, initially my family and I were devastated…that was 31 years ago. I ended up w a Glenn and a BT shunt and am doing great. BTW, I am 47 years old. Good luck and Godspeed!
May I ask if you have hetertaxy syndrome with single ventricle ?
I literally stumbled upon this website tonight while doing a little research and as I read I just wanted to give the parents (especially those with children with single ventricle hearts) a word of encouragement. I am believed to be the oldest surviving single ventricle patient right now, I am told I was the 4th person to have the original Fontan surgery (I have also had a Glenn) In 3 months I will be 45 yrs old and when I asked my Cardiology team several weeks ago at a annual check up “what is my life expectancy?” They just all kind of looked around until one of them laughingly said we don’t have any idea, we have no reason to think you are not going to be around for a while.
I assure you children today who are single ventricle can have a normal life expectancy, they may have some bumps in the road but modern medicine has made incredible strides in the last 40 years and it will continue to do so as your child gets older. That said I know I am unique and blessed in the fact that I have survived as long as I have, the truth is the Lord has had his hand on me my entire life, and without my faith and trust in him I would not be alive today. If there is one bit of advice I could give a parent of a child with a heart problem (or any child for that matter) it would be to encourage your child’s faith in the Lord, as well as your own. (Make no mistake, I assure you Jesus Christ is very real, I know that may not be a popular viewpoint today but it is the truth)
Also and this may be difficult for some parents who have a child with special needs, but it is very important for their development, treat them as if they were just as normal as any other kid. Trust me they know their limits, they do not need anyone to remind them. And finally be there for them, I know there is no doubt that I would not be around today if I would not have had loving parents. There were times when I knew my parents could not do anything to help my situation, except be there, but they were there, and that is the best thing you can ever do for your child too.
I didn’t mean for this to turn into an essay but I would be happy to answer any questions a parent may have about what they and their child can expect if they are single ventricle. My email is kylehosner at yahoo .com I look forward to hearing from you.
Thank you, for sharing. I have a beautiful baby girl who just turned a year old August 19th she has had 2 open heart surgeries and complications with other surgeries she will be having her next open heart surgery next month, glenn shunt and repairs to the pulmonary arteries so once again on the heart/lung by pass machine, she will be a single ventricle child, provided all goes well the Fontan to follow once she is 3 years old the surgeon has said. Surgeon says there is nothing normal about her heart, so happy she has this skilled surgeon put she does have prayer and the Good Lord on her side,,,,,, it does my heart and soul good being able to hear such a positive story being her Mom and just sitting by her side with positive thoughts she is the happiest baby, if you saw her you would not have a clue anything was wrong and I am blessed beyond measure to be her Mommy. God Bless. Thank you, for this blog and Kyle, Wishing you 45 more years. So happy she has a skilled surgeon at Texas Childrens Hospital in Houston Texas.
Thank you, Averi’s Mommy…….
Thanks everyone for sharing. I came across this blog while searching for answers regarding life expectancy of children born with single ventricle. My son had PA banding 3days after birth then a glenn shunt after 18months and Fontan after 5 years. He’s a wonderful child, so precious that there is not enough word to describe the joy he brings to the family. A boy we believe was created from God’s own heart!
I had another child that’s battling schizophrenia. While God may have given me a very challenging life seeing both of them suffer with a bleak hope of life getting any better, the joy of seeing them compliment another strengthens my faith, The love shared when the strong carries the weak and when the sane hugs the Challenged to ease depression and hallucination, I have witness brotherly love at its best! How long will I have them and who’ll look after them when my wife and I are gone … surely God has a plan.
I am a 34 year old woman with single ventricle who. I had the fontan at 6 years old and it is still going strong ????
Thank you for sharing your article is so similar to my Lil Leia who was born Single Ventricle, dextrocardia, pulmonary stenosis and to top it off she’s heterotaxy syndrome.. I look forward to fully write a brief passage of her first 6 years of miraculously living life after her 5 open heart surgeries which last Fontan was not very beneficial and brought her a step down from her Glenn procedure having low sats and turning blue with little activity and becoming stressed with just a couple of steps.. At this point she is awaiting a catherization home on standby oxygen surgery will be at Texas Children’s Hospital we wait in with great faith in God that her catherization will be beneficial and gather other information needed to help our Lil Princess Leia regain her daily childhood activities as close as a normal child’s day is.
Thank you for this wonderful blog I came across in my endless search for someone similar to my daughter whom I adopted at age 3 and now is 181/2!! My child was born with hetertaxy syndrome consisting of single ventricle dextracardia pulmonary stenosis etc… has survived two failed Fontans and has been living with s Glenn shunt – no options left but seems to be thriving well with SATS of 78 ! Issues with Supra
ventricular tachycardia controlled with meds so far . Will graduate high school in June ????It has been an honor and the joy of our lives raising this amazing , courageous sweet spirited girl.We take one day at a time and try to live each moment as tomorrow is never promised to any of us.
My son is born just 15 days before with a single ventricle..I don’t know the limitations of Fontan procedure..hope for the best and pray the deepest.nice article..thanks to the creator