What is Dextrocardia?

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Dextrocardia is an abnormal congenital positioning of the heart. Instead of the heart forming in the fetus on the left side, it flips over and forms on the right side. There are several types of dextrocardia, also called looping defects. Dextrocardia is frequently diagnosed in a routine prenatal sonogram, although not every radiologist will catch it, particularly if there are no cardiac structural abnormalities.

Mirror image dextrocardia is a very rare condition. It may be present in about one in 130,000 people. In this looping defect, not only does the heart flip the wrong way and develop on the wrong side of the chest, but also all the other organs in the middle of the body are reversed. In essence, an x-ray of mirror image dextrocardia looks like a mirror image of the normal heart and organ placement.

Mirror image dextrocardia was first noted in the 1920s, when x-rays revealed this abnormal placement. Fortunately, this type of looping defect does not involve structural abnormalities of the heart or other organs. In some cases, cilia, tiny hair-like structures, in the nose and lungs move in the opposite direction, causing a greater susceptibility to colds or illness. Aside from this susceptibility, a person with this condition does not require any special treatment or surgeries.

Dextrocardia with abnormal heart is a far more serious condition, which usually requires one or more surgeries to address structural abnormalities. It is a more common condition than mirror image dextrocardia, and in most cases, the position of the other organs in the middle of the body is not reversed. The outcome and survival rates for dextrocardia with abnormal heart depends upon the severity of the defects, which generally include a complicated form of transposition of the arteries, called levo-transposition, or both arteries arising from the right ventricle, called double outlet right ventricle.

Large ventricular septal defects are also common in dextrocardia with abnormal heart. The right and left ventricle may be so similar, unlike in the normal heart, that it is difficult to differentiate which ventricle is right or left. In many cases, the ventricular septal defect is so large that the ventricles are termed a single ventricle. One or both ventricles may be smaller than usual.

With complicated transposition and single ventricle, two surgical approaches are possible. If the ventricles are large enough, surgeons may elect to perform a Rastelli, one and a half ventricle repair. This surgery constructs a tunnel through the heart to properly reroute the blood. If possible, the ventricular septal defect may be repaired. However, prior to this treatment, the superior vena cava is directed to the smaller pulmonary veins, and blood from the upper body never passes through the right ventricle. This initial procedure, called the Glenn shunt, reduces the heart’s workload and buys time for the child to grow before performing the Rastelli.

The Rastelli has an excellent survival rate, once past the initial surgery. It is a complicated and often lengthy procedure. The main disadvantage of this surgery is the length of time a child may need to be on heart lung bypass. It is perfectly acceptable, as well as advisable, to ask a surgeon about his or her experience and success rate with this or any other surgery. Further surgical intervention may be necessary in the form of pulmonary or aortic valve replacements several years after the initial Rastelli.

Levo-transposition, small ventricles, and a high degree of pulmonary stenosis in dextrocardia with abnormal heart generally requires the three-staged Fontan operation. In this case, the surgeon performs three surgeries to palliate the defects. Improvement in the Fontan has led to improved survival rates, and many children born with this condition live well into their thirties before ultimately needing a heart transplant, the only true fix for single ventricle.

Dextrocardia may also be present in a condition called heterotaxy, which involves not only abnormal heart placement and structural abnormalities, but also the absence of a spleen, or the presence of a number of small spleens. Treatment depends upon the degree and severity of these structural anomalies. Lack of a spleen, asplenia, can complicate any operations because natural resistance to infection is significantly reduced.

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Discuss this Article

16
Im having a baby girl in Sept.. im 25 weeks and last week the doctors told us that her heart and stomach was on the right side. they said she has Dextrocardia but she also has 2 holes in her heart or sumthing called Atrioventricular septal defect and she has Heterotaxy syndrome and abnormal visceral situs.. They said she might have 2 have surgery her 1st couple of years.. has anybody have sum advice or ever heard of sumthing like this....?
- Babygirl123
15
My daughter was Dextrocardia with Situs Inversus, meaning she is a mirror image of all of her organs including her heart. We didn't find out until she was 18 months old during a routine Dr's visit. She had a cold and when we took her to the doctor they did a chest xray and this is when they discovered it. Sounds odd that they wouldn't know when I was pregnant but there was no reason to suspect anything.

She spent 1 week in the hospital after birth for jaundice and at 14 months old spent 2 days in the hospital for the roto-virus. During those hospital visits they didn't discover it then either. Shortly after finding out she went to Children's Hospital in Atlanta for a bronchial study to see if she had Kartagener's syndrome. Luckily she doesn't! She is a healthy thriving 3 year old little girl that really the only complication we have with this is some breathing issues like Asthma. She takes singular and Qvar just like any other child takes for Asthma. She is having her tonsils and adenoids taken out in July because she snores so loudly. With her lungs being reversed she already has a little bit of a challenge so we are hoping this surgery will help her to breathe better and sleep better.

My husband and I realize that we are one of the lucky ones since all of her organs mirrored. There are times it does get frustrating having to explain her condition to everyone from daycare, to sitters, to just the average person who is curious. She sees her pediatric cardiologist once per year just to check her heart but everything appears perfect although backwards. She is truly a miracle and I wouldn't trade her for anything in the world.

If you have a child born with this condition just know that there may be a few more doctors that are curious and want to "see" for themselves through x-rays. There may be a few more boxes to fill out on a health screen questionnaire, and a few more co-pays and deductibles to pay because of having to see a specialist for even the slightest little thing but embrace your child's difference and know that having a child as unique as this is truly a blessing. If anyone ever needs to chat about dextrocardia I'll be happy to be your friend.

- iluvg8tr
14
I wrote about my great-grandson when he was 6 months old having dextrocardia and a mirror image heart. He is now 9 months old and just had his second open heart surgery to re-route an artery and to remove the shunt that was put in when he was 2 weeks old. He is doing really well and if you didn't know it, he looks and acts like any other baby. The only noticeable trait is his little fingernails are a little blue, but other than that he is fine. He plays, he crawls a little, and eats good. He has two teeth and three more are coming in. He will have to have one more surgery to correct the arteries when he is about 4 years of age.

I just want to let anyone out there know that if your child has this condition, or you know somebody who has dextrocardia that it can be treated and you can live a relatively normal life. They did tell us that Cole may not live to be an "old" person without a heart transplant, but with technology these days, who knows what new procedures will be available later on.

- Jill61945
13
What problems may I face in my marriage having dextrocardia?
- anon31347
12
Dear everybody,

My two sons have a case called kartagener syndrome with dextrocardia.Tthey have health problems.

I want to know is there a permanent cure .

- nakhla1964
11
Dear Jill61945

Your grandsons oxygen level doesnt sound right to me, My sons heart doctor says that my sons oxygen level needs to be in the 90s. I suggest that you get a second opinion. No baby gets used to having low oxygen. I hope this helps you. May god be with you and your grandson.

- snickers75
10
My great-grandson who is 6 months old has dextrocardia and two holes in his heart. He has had one surgery and is scheduled for another next month. My biggest worry right now is that his oxygen level is dropping. Right now it is in the low 70s. Otherwise, he is a happy baby, he is growing, teething, and seems normal in every other way. Sometimes his hands and feet look bluish. The cardiologist says he is adjusting to this lower oxygen level but I am wondering what effect that will have on the rest of his body before he has all the surgeries he needs to correct the problems.
- Jill61945
9
Hi, My sister had dextrocardia, but unfortunately she died when she was 6 due to a very sudden and severe chest infection. It was a very long time ago, when information and knowledge were scarce about this condition. My parents were not really aware of the conditions and complications they should look out for. Fortunately, these days, there is plenty of information available. I hope that everyone who is affected by this condition gets all the facts that they need.
- anon18261
8
Thank you wgwriter for your kind words. It helps so much to know that there are more people going through what im going through. Hope all is well on your end too.
- snickers75
7
Snickers, That's terrific that he's doing so well, and as mentioned some holes do repair themselves and never need surgery. Sticking with cardiologist advice is just good sense on this. It's always a good idea to have an exact diagnosis (if its hard to remember have your cardiologist write it down for you), just in case a child needs emergency care. Preemies are much more prone to heart defects that do close, particularly holes in the atrial septum and a continued opening of the patent ductus. I'm sure the book, and continued doctor's advice will really help you out on this, but each kid's case is different. I'm so glad to hear he is healthy and doing well, though I know it can be challenging raising a baby with any problems :)
- WGwriter
6
Wow! Its Crazy but i dont know where the holes are exactly in his heart. All i know is that his heart doctor said that they are fixing themselves and that she can hardly hear them any more. And that she doesnt think that he will need surgery in the future. And when it comes to his weight gain hes doing so good. He is a preemie so of course he's smaller than a normal baby his age but he just keeps on putting on the weight. Im very proud at how well he is doing and so are all of his doctors because he has more then just a heart condition. He also has congenital scoliosis and his ears never developed. For right now hes as healthy as a horse. He sees his heart Doctor in September but i will call the office and find out about the holes and let you know.
- snickers75
5
Snickers, Can you elaborate on the holes in his heart? That could suggest some other conditions. Is your son dextrocardia with mirror image placement of the organs, or another condition related is dextrocardia with abnormal heart.

Generally, unless the holes are located in the patent ductus area, they may require surgical intervention at a later point. Keep me posted on this. I assume you mean a few holes in the septum (the dividing wall between the two sides of the heart)? Sometimes this can even be fixed now by non-invasive methods, but it can cause problems in infants, especially in the areas of gaining weight properly. Many kids can go for years without needing surgery, but I'd like to know specifically, if you don't mind, what the doctors are thinking about your son. Note I am not a doctor (nor do I play one on television), but I did run a support group for families with kids with heart conditions for 5 years. I have a bit more experience in this area given my son's condition.

- WGwriter
4
Thank you anon13937 for posting a comment about dextrocardia. It brings me great hope that my son will be ok. He also has a Bicuspid Aoritic Valve and two small holes in his heart but his heart doctor says that he is doing great. And too WGwriter, I will read the book The Heart of a Child. Thank you.
- snickers75
3
Hi, I'm twenty and I have Dextrocardia.

I love to tell people that my heart is on the other side of my chest! I am a perfectly healthy male. I also have a Bicuspid Aortic Valve. I really hope that doesn't cause problems later in life.

- anon13937
2
Hi Snickers75,

My son has dextrocardia too, though he had a lot of other heart conditions that required several surgeries. From what I know about mirror image dextrocardia, there are a couple of complications you may look for. First, he should have an emergency bracelet. If your son ever needed emergency surgery, you'd want to make sure a surgeon was operating in the right place and not making unnecessary incisions. Second, the cilia, fine little hairs in the nose and lungs, tend to beat in the wrong direction. This may make your son a little more susceptible to colds and flus. You might want to be sure your son gets flu shots each year.

Generally, though, when no other abnormality of the heart's structure exists, your son should do very well. It's a medical curiosity more than anything else, and most people with only the mirror image condition lead very normal lives.

You might want to obtain a copy of "The Heart of a Child" a very good book that explains lots about heart conditions in general.

- WGwriter
1
my son was born with mirror image dextrocardia and everything else seems to be normal. My question is, will he have complications in the future?
- snickers75

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