Congenitally Corrected Transposition of the Great Arteries.
I have been trying for a while to write this. First because I am not good with technical writing and second because I don't always feel like I understand everything. When I do try to read up and understand it better I get overwhelmed, but I will do my best to put it here.
Bentley has a unique heart. He has what is called Congenitally corrected Transposition of the Great Arteries. Only .5% of people with a heart defect have this. Which is about 5000 people in the United States. And along with that each case is different.
In the heart you have two ventricles that pump blood. The left ventricle, which is the stronger ventricle, pumps blood to the entire body. While the right ventricle pumps blood to the lungs. The two ventricles pump the blood through two main arteries. The aorta and the pulmonary artery. CCTGA occurs in the womb while the heart is still developing and what happens is the left and right Ventricles twist and form on the opposite sides. Luckily though the great arteries also switch making sure that the blood is flowing to the correct part of the body. However, the ventricle that is supposed to be pumping blood to the lungs is pumping blood to the body. Because this ventricle was made to pump blood the short distance to the lungs it is not equipped or built to pump blood to the entire body. This will cause it to wear down quicker, fail or cause other heart complications.
When one has CCTGA it is almost always followed by other issues in the heart. For example blockages or holes. In Bentleys case it caused him to have a heart block which causes the top and bottom of the heart to beat out of sync.
There are different outcomes to those who have CCTGA. Those who have more serious complications require surgery right after birth. Then there are those who don't require immediate surgery. In those cases they either go into heart failure requiring a heart transplant, or they can go years without medical intervention. Bentley falls into the second category. His body was tolerating his slow heart rate and he didn't have any major holes so immediate surgery wasn't necessary. Our cardiologist at the time decided to just take the "wait and see" approach. Where we just monitored him close with regular visits to the cardiologist and see how he did. He never mentioned to us that surgery was an option. We did know that as he grew, because of his heart block, his heart would slow more and more and would need a pacemaker, that was a necessary thing for him to need and we knew that from before he was born. With Bentleys heart block his heart was beating at an average of 50-60 bpm at birth (and average newborns heart rate will be between 80-160).
For the first couple years of his life Bentley was our miracle, his slow heart didn't slow him down one bit and he had so much energy! You wouldn't even know that he had a heart condition unless you listened to his heart or saw his ekgs or echos. He did so well! When we moved to Arizona we found him a new cardiologist. His second cardiologist had the same opinion as the first that we should take the 'wait and see' approach and monitor him closely. However he did mention the double switch surgery but he gave us the opinion that since he was doing so well and that there was so little information about it that it was safer to just 'wait and see.'
Fast forward to our move to northern California. We found a new cardiologist close to home and things took a turn. After doing all his routine tests, we decided that it was time for a pacemaker because his heart rate was dropping into the 40's while he was sleeping and we didn't want it to get any lower. He also was quite surprised that we didn't have any knowledge about the double switch surgery. He said that because CCTGA is so uncommon many cardiologists don't know much about it and even they don't know all the options. That's when we learned about our amazing surgeon Dr. Frank Hanley. Our cardiologist set up a meeting with him to discuss our options.
We were nervous about our meeting with him. Up to that point we hadn't really thought about any surgery for Bentley beside the pacemaker. We also didn't realize at the time how well known or how much he has contributed to kids with CCTGA. He has specialized in Bentleys condition and the double switch surgery. At the time he was one of two surgeons we found that do the double switch surgery, the other being in Boston ( Since then we have learned about a couple others that perform the surgery but they haven't done near as many as the main two that we researched.) In our meeting he explained that his right ventricle was getting weaker and starting to show some leakage. He recommended to us that we do an arterial banding. This is where they put a small band around the artery that makes the ventricle pump harder therefore strengthening it. After six months they check the artery to see if the band worked then they will tighten it. The goal with the banding is to try to get the ventricle blood pressure the same as the left ventricle so it can support the higher flow of blood that goes into his body. Dr. Hanley recommended that we do the banding to help prolong his heart. He said after that there was also the option of doing what's called the double switch surgery. This is where they physically switch the arteries for them to connect to the correct ventricle. Now because Bentley was doing so well tolerating his heart his surgeon said that he has seen some cases similar to Bentleys where they will live into their 70's before their heart fails. But on the other hand he has seen cases that were also similar and they went into heart failure in their mid 20's. There just aren't enough cases or information to know why some people with CCTGA can live long lives and others don't. He said it was up to us on whether we wanted to do the banding, double switch, or continue to wait and see how he handled the pacemaker. After that meeting we had a tough decision to make, however we did feel that we would do the arterial banding. That way we had a year to decide if we wanted to pursue the double switch or continue to wait and see and not put a 4 year old through major heart surgery.
As we were preparing for his pacemaker and PA banding we had to get a Dental clearance. What goes on in your mouth is very closely related to your heart. (If there is any infection in your mouth it could travel to the heart while undergoing heart surgery). We found out that Bentley had a couple cavities that needed to be filled before the surgery. Easy right? Well he had to have it done at a hospital under anesthesia with an anesthesiologist who specializes in cardiac cases. We called just about everywhere until we found a hospital that would take him before his surgery. We were cutting it close but we were able to get into UCSF a week before his surgery was scheduled. On the day of his dental appointment his routine filling of cavities ended up being much more. Towards the end of the procedure Bentley went into three short episodes of V-fib. Which is where the heart goes into erratic pulses causing the heart to stop pumping blood. Bentley was ok, they finished up his dental work, but they admitted him to the cardiac ICU because he needed to be kept under observation to make sure he didn't have any more episodes.
We were blessed that we have such great friends. Hadley was able to stay the night at their place while Alex came straight from work and stayed with Bentley during the night and I was able to go home and get some sleep. The next day we talked with the drs. they decided that we could either stay at UCSF and redo all his tests and their team could make a diagnosis and set a surgery plan or they could transfer us to Stanford where his surgery was taking place the next week. We decided it would be best to take him to stanford where they were familiar with his case. That's when we got to ride in an ambulance to Stanford.
At Stanford they discovered that Bentley's V fib was related to Torsades. Which is a type of tachycardia found in patients who have long QT syndrome. However Bentley tested negative for Long Qt. The Drs. decided to keep him under observation but they believed that after putting the pacemaker in it would correct the Torsades. (Which it did!)
For a whole week we just got to hang out in the ICU at Stanford. They tried to move up his surgery but there weren't' any openings. (His surgeon is always scheduled months out). A week later Bentley had his pacemaker put in and a PA band placed. We were in the hospital for a total of 14 days, one of which was Bentleys 4th birthday and we were so happy to come home.
Six months later we went back to the hospital for a series of tests to see if the band was working. Bentley's heart was doing really well and was ready to have his PA band tightened. Before surgery the hospital wants to make sure that you are completely healthy before going under anesthesia to avoid further complications. This means that we need to stay away from people and germs leaving us homebound for 4 weeks. This time around the surgery got pushed back 6 weeks because Bentley caught a cold.😩(This meant we were home for almost 10 weeks.) Then after the six weeks we got a call saying his new surgery date would most likely get bumped as well because there was supposed to be a baby born on the day of Bentleys surgery that needed our surgeon. They called on a Tuesday and offered for us surgery slot on Thursday, which meant pre-op would be the next day. Or we could wait and see if the baby came on our original surgery date. After being home so long we took the Thursday slot.
Bentley did great this time we only stayed in the hospital for 9 days, this was over Thanksgiving week and we were so happy to come home.
I'm going to end this one here. Its long and honestly I don't expect anyone to really read it. Its more for me.
The next entry will be about Bentleys last surgery and our experience with it.
Love always
the Richards family
I have been trying for a while to write this. First because I am not good with technical writing and second because I don't always feel like I understand everything. When I do try to read up and understand it better I get overwhelmed, but I will do my best to put it here.
Bentley has a unique heart. He has what is called Congenitally corrected Transposition of the Great Arteries. Only .5% of people with a heart defect have this. Which is about 5000 people in the United States. And along with that each case is different.
In the heart you have two ventricles that pump blood. The left ventricle, which is the stronger ventricle, pumps blood to the entire body. While the right ventricle pumps blood to the lungs. The two ventricles pump the blood through two main arteries. The aorta and the pulmonary artery. CCTGA occurs in the womb while the heart is still developing and what happens is the left and right Ventricles twist and form on the opposite sides. Luckily though the great arteries also switch making sure that the blood is flowing to the correct part of the body. However, the ventricle that is supposed to be pumping blood to the lungs is pumping blood to the body. Because this ventricle was made to pump blood the short distance to the lungs it is not equipped or built to pump blood to the entire body. This will cause it to wear down quicker, fail or cause other heart complications.
When one has CCTGA it is almost always followed by other issues in the heart. For example blockages or holes. In Bentleys case it caused him to have a heart block which causes the top and bottom of the heart to beat out of sync.
There are different outcomes to those who have CCTGA. Those who have more serious complications require surgery right after birth. Then there are those who don't require immediate surgery. In those cases they either go into heart failure requiring a heart transplant, or they can go years without medical intervention. Bentley falls into the second category. His body was tolerating his slow heart rate and he didn't have any major holes so immediate surgery wasn't necessary. Our cardiologist at the time decided to just take the "wait and see" approach. Where we just monitored him close with regular visits to the cardiologist and see how he did. He never mentioned to us that surgery was an option. We did know that as he grew, because of his heart block, his heart would slow more and more and would need a pacemaker, that was a necessary thing for him to need and we knew that from before he was born. With Bentleys heart block his heart was beating at an average of 50-60 bpm at birth (and average newborns heart rate will be between 80-160).
For the first couple years of his life Bentley was our miracle, his slow heart didn't slow him down one bit and he had so much energy! You wouldn't even know that he had a heart condition unless you listened to his heart or saw his ekgs or echos. He did so well! When we moved to Arizona we found him a new cardiologist. His second cardiologist had the same opinion as the first that we should take the 'wait and see' approach and monitor him closely. However he did mention the double switch surgery but he gave us the opinion that since he was doing so well and that there was so little information about it that it was safer to just 'wait and see.'
Fast forward to our move to northern California. We found a new cardiologist close to home and things took a turn. After doing all his routine tests, we decided that it was time for a pacemaker because his heart rate was dropping into the 40's while he was sleeping and we didn't want it to get any lower. He also was quite surprised that we didn't have any knowledge about the double switch surgery. He said that because CCTGA is so uncommon many cardiologists don't know much about it and even they don't know all the options. That's when we learned about our amazing surgeon Dr. Frank Hanley. Our cardiologist set up a meeting with him to discuss our options.
We were nervous about our meeting with him. Up to that point we hadn't really thought about any surgery for Bentley beside the pacemaker. We also didn't realize at the time how well known or how much he has contributed to kids with CCTGA. He has specialized in Bentleys condition and the double switch surgery. At the time he was one of two surgeons we found that do the double switch surgery, the other being in Boston ( Since then we have learned about a couple others that perform the surgery but they haven't done near as many as the main two that we researched.) In our meeting he explained that his right ventricle was getting weaker and starting to show some leakage. He recommended to us that we do an arterial banding. This is where they put a small band around the artery that makes the ventricle pump harder therefore strengthening it. After six months they check the artery to see if the band worked then they will tighten it. The goal with the banding is to try to get the ventricle blood pressure the same as the left ventricle so it can support the higher flow of blood that goes into his body. Dr. Hanley recommended that we do the banding to help prolong his heart. He said after that there was also the option of doing what's called the double switch surgery. This is where they physically switch the arteries for them to connect to the correct ventricle. Now because Bentley was doing so well tolerating his heart his surgeon said that he has seen some cases similar to Bentleys where they will live into their 70's before their heart fails. But on the other hand he has seen cases that were also similar and they went into heart failure in their mid 20's. There just aren't enough cases or information to know why some people with CCTGA can live long lives and others don't. He said it was up to us on whether we wanted to do the banding, double switch, or continue to wait and see how he handled the pacemaker. After that meeting we had a tough decision to make, however we did feel that we would do the arterial banding. That way we had a year to decide if we wanted to pursue the double switch or continue to wait and see and not put a 4 year old through major heart surgery.
As we were preparing for his pacemaker and PA banding we had to get a Dental clearance. What goes on in your mouth is very closely related to your heart. (If there is any infection in your mouth it could travel to the heart while undergoing heart surgery). We found out that Bentley had a couple cavities that needed to be filled before the surgery. Easy right? Well he had to have it done at a hospital under anesthesia with an anesthesiologist who specializes in cardiac cases. We called just about everywhere until we found a hospital that would take him before his surgery. We were cutting it close but we were able to get into UCSF a week before his surgery was scheduled. On the day of his dental appointment his routine filling of cavities ended up being much more. Towards the end of the procedure Bentley went into three short episodes of V-fib. Which is where the heart goes into erratic pulses causing the heart to stop pumping blood. Bentley was ok, they finished up his dental work, but they admitted him to the cardiac ICU because he needed to be kept under observation to make sure he didn't have any more episodes.
We were blessed that we have such great friends. Hadley was able to stay the night at their place while Alex came straight from work and stayed with Bentley during the night and I was able to go home and get some sleep. The next day we talked with the drs. they decided that we could either stay at UCSF and redo all his tests and their team could make a diagnosis and set a surgery plan or they could transfer us to Stanford where his surgery was taking place the next week. We decided it would be best to take him to stanford where they were familiar with his case. That's when we got to ride in an ambulance to Stanford.
At Stanford they discovered that Bentley's V fib was related to Torsades. Which is a type of tachycardia found in patients who have long QT syndrome. However Bentley tested negative for Long Qt. The Drs. decided to keep him under observation but they believed that after putting the pacemaker in it would correct the Torsades. (Which it did!)
For a whole week we just got to hang out in the ICU at Stanford. They tried to move up his surgery but there weren't' any openings. (His surgeon is always scheduled months out). A week later Bentley had his pacemaker put in and a PA band placed. We were in the hospital for a total of 14 days, one of which was Bentleys 4th birthday and we were so happy to come home.
Six months later we went back to the hospital for a series of tests to see if the band was working. Bentley's heart was doing really well and was ready to have his PA band tightened. Before surgery the hospital wants to make sure that you are completely healthy before going under anesthesia to avoid further complications. This means that we need to stay away from people and germs leaving us homebound for 4 weeks. This time around the surgery got pushed back 6 weeks because Bentley caught a cold.😩(This meant we were home for almost 10 weeks.) Then after the six weeks we got a call saying his new surgery date would most likely get bumped as well because there was supposed to be a baby born on the day of Bentleys surgery that needed our surgeon. They called on a Tuesday and offered for us surgery slot on Thursday, which meant pre-op would be the next day. Or we could wait and see if the baby came on our original surgery date. After being home so long we took the Thursday slot.
Bentley did great this time we only stayed in the hospital for 9 days, this was over Thanksgiving week and we were so happy to come home.
I'm going to end this one here. Its long and honestly I don't expect anyone to really read it. Its more for me.
The next entry will be about Bentleys last surgery and our experience with it.
Love always
the Richards family
Comments
Post a Comment