Less than 2 weeks ago my son and his wife were told that their 9 1/2 month old daughter had more than just a hole in her heart. This was all they expected to hear at first. A Pediatrician seen her and sent her off for tests, due to a very strange and somewhat loud noise that could be heard in her back as well. Her echocardiogram showed a narrowing of the mitral valve caused by a membrane. Today when I went with the kids to speak to the Cardiologist about the pending open heart surgery, I asked how blocked her mitral valve was. We were informed that there was only about 3 mm left of the opening and that it was blocked otherwise. Is this as serious as it sounds? When I started asking about this tissue the term non cancerous like tumor came up as an explanation. The doctor continued by saying that she had not seen something like this in over 10 years and she expressed how rare it was. The Doctor also said that her pressure on the one side of heart is 130. Apparently this is high, correct? She had said something about 200 when she was giving systollic and dystollic but I missed the entire numbers. Over whelmed with information of course. My main question for you is this, does this sound like Myxoma? I was doing on line research to better understand what my grand daughter has and what the risks are when having this surgery. Her surgery is in 12 days from now. From the time of discovery to the date of surgery is exactly 3 weeks, so you can well imagine what these young parents and the rest of our family is going through.
If this indeed sounds as though its Myxoma, what if any are the risks during surgery and will she have a full and complete recovery. Can it return? Will Mom have a higher risk of having another baby with the same thing? I did read there may be a genetic factor invovled?
Any information will help, as we are trying to understand everything in a very short period of time
This does sound scary for your granddaughter and her parents. It is very difficult to tell from this information what kind of tumor this is, and without seeing her imaging studies, there is little way to tell. I can tell you that it is probably not a rhabdomyoma, the most common cardiac tumor of childhood. At this point, though, there are several different kinds of tumors that this could be, so it doesn’t make much sense to speculate. Myxomas are actually quite rare in children, though. The diagnosis of the type of tumor can often be made with a combination of echocardiography (cardiac ultrasound) and MRI. The various tumors demonstrate different characteristics to their appearances in the multiple imaging modalities within MRI, thus helping to tell them apart. Once a specific diagnosis of the tumor is made, further information regarding planning, prognosis, subsequent children, etc., can be addressed.
Overall, it sounds like the pressure in her left atrium, and potentially on the right side of her heart, is quite high. This should be able to come down after removal of the tumor. Hopefully, it will be able to salvage the valve so that it will function normally. However, depending on whether this tumor is on the inside of the left atrial wall or actually inside the wall will lead, in part, to how the intervention goes. The surgeon may need to reconstruct part of the left atrium after resecting the tumor. As you can imagine, this is much more difficult in a 9 month old baby as compared to a 9 year old child, with smaller structures throughout. I would make sure that they are at a pediatric cardiac center that has experience with this and can anticipate some of the other problems that can come with this.
Just to clarify, the Cardiologist we talked to today is not the one that will be performaning the surgery. We will meet the surgeon the day before the surgery at childrens (BC). Is there any questions we should be asking him to help us better understand all this?
Thank you for taking the time to respond to my questions. Your information reconfirms the importance of trying to ask more questions when we get the opportunity the day before the surgery. There has been very limited opportunities to ask anything due to the short amount of time the family has had to absorb everything and also limited contact with a specialist.
I have to ask one more question. As the mitral valve is narrowed with only a 3mm opening, what is the chance of regurgitation at this point? My granddaughter has always had a cough since she was born. Nothing too serious up till recently but a cough non the less. Currently she is coughing a fair bit and being able to determine if she has a cold virus or not is hard. She has been on antibiotics since last Sunday and I would say the cough since then has increased, ruling out a bacterial component in my way of thinking. She has 6 teeth so far and so the runny nose is not always indictive of a cold since she is teething once again. I worry about her cough and I worry it will interfere with the surgery pending.
Any thoughts/ best educated guess on the cough and possible regurgitation?
It's a wonderful thing you do here, giving families an opportunity to ask questions. Clearly being able to give out information is a challenge for you as you are not the doctor directly involved, but your willingness to provide direction and support is to be commended. Thank You !
Regarding the mitral regurgitation, without knowing who your surgeon is, what the anatomy of the tumor is, and where exactly it is in relation to the valve doesn't give me much ability to answer this. Hopefully, since the tumor is above the valve, the valve isn't directly involved but has flow obstructed by the tumor itself. If the valve otherwise is anatomically normal and functions normally, the risk of regurgitation may be low assuming that the tumor can be removed and the left atrium can be reconstructed without damaging the valve. However, if the tumor involves the valve leaflets themselves, it's a different story, and all bets are off.
As far as her cough, if she's had a cough since she was born, that's not a normal thing. Newborns are allowed to sneeze all they want, but a cough indicates something wrong. It can be almost anything, so I'm can't diagnose at all without the information that I need to be able to do that in this forum. I will say, though, that one of the potential etiologies could also be related to the increased left atrial pressures and backup of pressure/fluid across the lungs.
My granddaughters surgery was cancelled today for this coming Tuesday. We are still going in on Monday for the pre-op day and hopefully to meet with the Surgeon who will be doing the operation.
My question for you is this, how long can a 10 month old's heart sustain high blood pressure such as her's as described to you earlier in my post. What we have lacked so far was any comparison testing to see if the growth is rapid or not. They decided on priority for her during a case conference without knowing the speed of growth but based on current status. They told the kids today that unfortunately they had a few very critical babies brought from other areas within the province for urgent care and the ICU is over loaded. The hospital stated that Nov 3 was as far as they would take it but hope to get her in Oct 26th.
I am hoping we can at least hear information on Monday after redoing all her tests including an Echo whether the growth is closing in fast or not. But my concern at present is how long a small heart in a baby can sustain without anything going wrong and her beconing one of those critical babies.
Without evaluating her directly or in the interim since we have discussed her, I do not know how she will do. If nothing else has changed, though, she should be able to maintain these pressures for one more week, though it isn't particularly good for her heart or lungs. As well, I don't know if the tumor could spread by direct extension, metastasis, or embolization into the blood stream.
We had our pre op day at childrens and I must say as a family we are a little more comfortable now that we understand more about the heart and what is going on. The initial cariodologist appt lead us to believe certain information(lack of understanding on our part I am sure) and the appointment at childrens cleared up a great deal for us.
First to explain. The pressure I had mentioned earlier I thought was blood pressure was actually the gradient of the LVOFT which is 130. We were told typcially the average gradient for a 10 month old is around 20, so clearly this is high. We also got to view the newest echocardiogram. They sedated her to get a really good picture of the problem. They also used a hot heart model to explain to us the problem. The uniquiness of this case is the location of the growth. It is right between the mitral valve and the aortic valve. The growth is measuring 1cm from the mitral valve and it crosses what appears to be almost the entire space between the two walls (lots of thickness of the wall of course). What they have told us is they do not know for sure what it is, the location is very rare and until they go in and remove the tissue and do the pathology its anyones guess. It may sound odd but its comforting to hear at least they do not know for sure about anything other than it needs to be removed.
We feel better to know that the heart centre at Childrens although not large in scale does on average 180 open heart surgery's a year and they have some excellent surgeons to handle this operation. They also indicated she could probably go on for a fair bit longer without intervention but would not want to risk serious complications and have her become critical hence the urgent desire to remove the growth now.
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