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Scimitar Syndrome
My daughter is 2 yrs old right now, at 10 months of age she was diagnosed with Scimitar Syndrome. The surgeons decided she could hopefully wait until the age of 5 to have any sort of intervention.
At 13 months of age they did a Heart Cath to do some coiling of her Scimitar veins. They were very shocked at what they found during the cath. Her pressures were extremely high, the right lung of course was quite alot smaller, and they had to put in 9 coils in 3 veins as well as a plug in another. She ended up going into respiratory arrest during the cath and required chest compressions and intubation. They decided they couldn't wait to do her repair.
At 14 months of age she had open-heart surgery. According to her Op Report this is the procedure they did: Autologous pericardial patch tunneling of right pulmonary veins from infradiaphragmatic inferior vena cava through the ASD to the left atrium. Repair of ASD (autologous pericardial patch repair).
A month ago she went in for a Heart Cath again and the surgeons are baffled. They can't seem to locate her repair they did a year ago. Her right lung is very red with blood again and they just can't seem to give us any answers as to what has happened to this repair that they "knew" would work.
Here are my questions:
Can you give me a better explanation of Scimitar? I've heard it called PAPVR as well, is this correct? Is this hereditary? How many children have this condition? What is the long-term prognosis? Our doctors just seem to go in circles when I ask them questions like this, as if they really aren't sure. And the internet is no help!
How can her surgical repair just "disappear" (as they say)?
Is there a better way to do this repair?
Are we doing everything we can for her?
Her lips and tongue turn purple in color quite often (sometimes at the same times, sometimes separately), they told me this was nothing to worry about, it this true? Thank you for your time!
At 13 months of age they did a Heart Cath to do some coiling of her Scimitar veins. They were very shocked at what they found during the cath. Her pressures were extremely high, the right lung of course was quite alot smaller, and they had to put in 9 coils in 3 veins as well as a plug in another. She ended up going into respiratory arrest during the cath and required chest compressions and intubation. They decided they couldn't wait to do her repair.
At 14 months of age she had open-heart surgery. According to her Op Report this is the procedure they did: Autologous pericardial patch tunneling of right pulmonary veins from infradiaphragmatic inferior vena cava through the ASD to the left atrium. Repair of ASD (autologous pericardial patch repair).
A month ago she went in for a Heart Cath again and the surgeons are baffled. They can't seem to locate her repair they did a year ago. Her right lung is very red with blood again and they just can't seem to give us any answers as to what has happened to this repair that they "knew" would work.
Here are my questions:
Can you give me a better explanation of Scimitar? I've heard it called PAPVR as well, is this correct? Is this hereditary? How many children have this condition? What is the long-term prognosis? Our doctors just seem to go in circles when I ask them questions like this, as if they really aren't sure. And the internet is no help!
How can her surgical repair just "disappear" (as they say)?
Is there a better way to do this repair?
Are we doing everything we can for her?
Her lips and tongue turn purple in color quite often (sometimes at the same times, sometimes separately), they told me this was nothing to worry about, it this true? Thank you for your time!
A related discussion, Scimitar was started.
Is it genetic?
There are cases where it does run in the family, but I am under the impression that in a majority of the cases, it just happens. There is nothing that you yourself can do to prevent or cause Scimitar syndrome.
Long term prognosis?
As a general rule, the more symptomatic the child is earlier in life, the more problematic it will be later on. I second the doctor's advice. You know your child best, and if you think that the doctors in your area aren't doing enough, then get a second opinion, perhaps in a center that has more congenital patients than where your taking her now.
There are cases where it does run in the family, but I am under the impression that in a majority of the cases, it just happens. There is nothing that you yourself can do to prevent or cause Scimitar syndrome.
Long term prognosis?
As a general rule, the more symptomatic the child is earlier in life, the more problematic it will be later on. I second the doctor's advice. You know your child best, and if you think that the doctors in your area aren't doing enough, then get a second opinion, perhaps in a center that has more congenital patients than where your taking her now.
MEDICAL PROFESSIONAL
Dear Rach,
Scimitar syndrome is a special type of partial anomalous pulmonary venous return (PAPVR). PAPVR occurs when some of the pulmonary veins, the veins that drain the lungs, return to the right side of the heart instead of to the left atrium. The PAPVR in scimitar syndrome drains some, or all, of the right lung. As well, not only is there PAPVR, but there is also anomalous arterial connection to this specific section of the lung. In scimitar, the arterial supply often arises from the lower portion of the aorta, the main artery that takes blood out to the body; ordinarily, the blood supply should come from the pulmonary arteries. This is also sometimes associated with the heart pointing to the right or being located in the right chest along with hypoplasia, or underdevelopment, of the right lung. There can be other intracardiac structural defects, as well, such as the atrial septal defect (ASD) that your daughter had. The term, “scimitar,” comes from the chest X-ray appearance of the anomalous pulmonary vein looking like a Turkish sword. It is a very uncommon condition in comparison to the overall incidence of congenital heart defects (about 1%) or in comparison to routine PAPVR cases. It is not hereditary, as best as we can tell at this time. The long term prognosis depends very much on the anatomy and physiology, which can be quite variable amongst these kids.
In regards to your daughter, it is difficult to tell exactly what was going on from the information that you gave me. I would think that they most likely coiled the anomalous arterial connections at age 13 months, not the veins. At that time, it sounds like they may have found evidence of pulmonary hypertension, or high blood pressure across the lungs. Then, they baffled the vein through the ASD into the left atrium. However, it sounds as if the baffled vein may have disappeared. Unfortunately, based on what you are telling me, it sounds as if this pulmonary vein may have occluded. The pulmonary veins are quite sensitive to being manipulated, and the combination of the surgery with the history of pulmonary hypertension may have caused the inner lining of these anomalous pulmonary veins to just abnormally grow and to seal them off. Without knowing everything about your daughter’s anatomy and what the veins looked like prior to the operation, I can’t say whether there may have been a better way to have performed this surgery, as you are asking. In the meantime, despite the coil embolization of the anomalous arterial connections to this section of lung, she may have developed what is called collateral vessels, which are extra vessels that naturally form when the body needs help to bypass an obstruction. I also don’t have enough information about her turning blue, such as what her oxygen saturations are when this happens as well as what the pressure on the right side of her heart is. It is possible that she still has pulmonary hypertension, which could be causing problems for her lungs and heart. I would suggest that, if you are not satisfied with the information or the evaluation that you are receiving from your doctors, you consider a second opinion for evaluating your daughter to see where things need to go from here for her.
Scimitar syndrome is a special type of partial anomalous pulmonary venous return (PAPVR). PAPVR occurs when some of the pulmonary veins, the veins that drain the lungs, return to the right side of the heart instead of to the left atrium. The PAPVR in scimitar syndrome drains some, or all, of the right lung. As well, not only is there PAPVR, but there is also anomalous arterial connection to this specific section of the lung. In scimitar, the arterial supply often arises from the lower portion of the aorta, the main artery that takes blood out to the body; ordinarily, the blood supply should come from the pulmonary arteries. This is also sometimes associated with the heart pointing to the right or being located in the right chest along with hypoplasia, or underdevelopment, of the right lung. There can be other intracardiac structural defects, as well, such as the atrial septal defect (ASD) that your daughter had. The term, “scimitar,” comes from the chest X-ray appearance of the anomalous pulmonary vein looking like a Turkish sword. It is a very uncommon condition in comparison to the overall incidence of congenital heart defects (about 1%) or in comparison to routine PAPVR cases. It is not hereditary, as best as we can tell at this time. The long term prognosis depends very much on the anatomy and physiology, which can be quite variable amongst these kids.
In regards to your daughter, it is difficult to tell exactly what was going on from the information that you gave me. I would think that they most likely coiled the anomalous arterial connections at age 13 months, not the veins. At that time, it sounds like they may have found evidence of pulmonary hypertension, or high blood pressure across the lungs. Then, they baffled the vein through the ASD into the left atrium. However, it sounds as if the baffled vein may have disappeared. Unfortunately, based on what you are telling me, it sounds as if this pulmonary vein may have occluded. The pulmonary veins are quite sensitive to being manipulated, and the combination of the surgery with the history of pulmonary hypertension may have caused the inner lining of these anomalous pulmonary veins to just abnormally grow and to seal them off. Without knowing everything about your daughter’s anatomy and what the veins looked like prior to the operation, I can’t say whether there may have been a better way to have performed this surgery, as you are asking. In the meantime, despite the coil embolization of the anomalous arterial connections to this section of lung, she may have developed what is called collateral vessels, which are extra vessels that naturally form when the body needs help to bypass an obstruction. I also don’t have enough information about her turning blue, such as what her oxygen saturations are when this happens as well as what the pressure on the right side of her heart is. It is possible that she still has pulmonary hypertension, which could be causing problems for her lungs and heart. I would suggest that, if you are not satisfied with the information or the evaluation that you are receiving from your doctors, you consider a second opinion for evaluating your daughter to see where things need to go from here for her.
Thank you so much for clarifying a few things for me! I'm still wondering if someone can answer my questions about:
Is it genetic?
Long-term prognosis?
Thank you so much!
Is it genetic?
Long-term prognosis?
Thank you so much!
I am not a doctor, though I will try to answer some of the questions.
Scimitar syndrome describes the shape that appears on the xray that is not normal. It is shaped like a curved sword. Scimitar happens in 1 to 3 three kids per 100000 births.
In normal kids, the circulation goes like this:
left side of the heart->body->right side of the heart->lungs->left side of the heart
in between the lungs and the left side of the heart are the pulmonary veins. They are what carries the blood to the left side of the heart. There are four of them in total, with each lung getting two veins. In Scimitar, the veins from the right lung connect abnormally(instead of into the the top of the left heart in normal kids) to some vessels in the body (most usually the IVC, the biggest vein in the body). This is also called PAPVR, which stands for Partial Anomolous Pulmonary Vein Return. I can speculate that it sounds like they did the right kind of repair, but from what I know each kid is their own separate masterpiece when it comes to surgical repair.
I don't know enough about your specific case to really help you any further, and it is rather complicated (not to mention beyond my scope) and not a straight forward kind of thing. I hope what I've told you helps some.
Scimitar syndrome describes the shape that appears on the xray that is not normal. It is shaped like a curved sword. Scimitar happens in 1 to 3 three kids per 100000 births.
In normal kids, the circulation goes like this:
left side of the heart->body->right side of the heart->lungs->left side of the heart
in between the lungs and the left side of the heart are the pulmonary veins. They are what carries the blood to the left side of the heart. There are four of them in total, with each lung getting two veins. In Scimitar, the veins from the right lung connect abnormally(instead of into the the top of the left heart in normal kids) to some vessels in the body (most usually the IVC, the biggest vein in the body). This is also called PAPVR, which stands for Partial Anomolous Pulmonary Vein Return. I can speculate that it sounds like they did the right kind of repair, but from what I know each kid is their own separate masterpiece when it comes to surgical repair.
I don't know enough about your specific case to really help you any further, and it is rather complicated (not to mention beyond my scope) and not a straight forward kind of thing. I hope what I've told you helps some.
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