My two-year-old daughter was born with ToF. She had her full repair at 13 weeks of age.
She had a fairly complication free recovery - although she had a lung collapse and fluid accumulation around her heart post-repair.
Now she lives with severe pulmonary regurgitation and her right ventricle is dilating.
Recently she has also started to have some rhythm issues. She has bradycardia and junctional escape beats (around 65-70 bpm). The junctional rhythm was first noticed a year ago when she wore a holter monitor (I was noticing some irregular behavior, and she seemed "off"). Now, her irregular rhythm is caught on a regular ekg. Her cardiologist calls this sick sinus syndrome, and says she will need a pace maker at some point in her life.
I asked him about a timeline for the pacemaker, and he suggested maybe teens or early 20's. He also suspects that this will be the same timeline for a valve replacement.
It makes me nervous to "wait and see"....I'm wondering what sort of expectations I should have for monitoring, and what sort of progression of symptoms I should expect?
Are there specific tests or monitoring I should request for her?
For our other readers, tetralogy of Fallot (ToF) is a complex congenital cardiac defect in which there is a ventricular septal defect (VSD), or hole in the heart, that is accompanied by pulmonary stenosis (obstruction) and override of the VSD by the aorta. It sounds like when her ToF was repaired, she likely had a transannular patch across the pulmonary valve. This patch augmented the size of the pulmonary valve so that it wouldn’t be stenotic, but it also made the valve incompetent (regurgitant), which is a typical outcome in this kind of intervention. We are finding that patients are requiring pulmonary valve replacement, as this leads to dilation of the right ventricle with exercise intolerance, decreased right ventricular pump function, and arrhythmias into adulthood. Unfortunately, the criteria for timing of pulmonary valve replacement are not clear at this time. There are those who look at certain criteria on the ECG, others who look at the right ventricular volume as indexed to body surface area, and still others who look at induced arrhythmias with exercise.
One of the other things that can happen with any heart surgery is damage to the sinus node, the natural pacemaker of the heart. This happens at the site of the cannula that is put into the superior vena cava (the vein that returns blood from the head and neck to the right side of the heart) to send blood to the heart-lung bypass machine. We do see a number of patients requiring pacemakers after congenital heart surgery, either requiring it early or developing the need later. The good news here is that there are fairly agreed upon and published criteria for the timing of pacemaker placement. These criteria are split out into those patients with normal hearts and those with congenital cardiac defects. They also include a low resting heart rate threshold, presence of prolongation of the QTc (a measurement on the ECG), and several other criteria for which your cardiologist should be evaluating your daughter. As well, exercise intolerance and/or worsening fatigue can be a simple criterion for which you can look. The tests that can demonstrate these other findings include routine ECG and 24-hour Holter monitoring, with consideration of exercise stress testing when she is old enough. Be aware, though, that placement of a pacemaker depends not on the timing of her heart valve repair, but on her heart and how she is doing. If you are not getting the answers that you feel that you should from your cardiologist, you can certainly obtain a second opinion.
Yes, my daughter did have a transanullar patch. She also had a vascular ring repaired (right sided aortic arch, aberrant left subclavian artery), and her PDA ligated. Her cardiologist called her stenosis severe, but her surgeon called it atresia in his surgical notes.
Post repair she had a shunting PFO, and three tiny muscular VSDs. All four defects have closed on their own.
Through circumstances outside of our control (a move, and a change of insurance), she has had three different cardiologist in her short life.
I feel like I am constantly trying to get to know her cardiologist and his/her style. Then, I have the conflicting philosophies to deal with too.
Her first cardiologist told me to expect her to turn blue prior to her repair. As stupid as this sounds, I never pressed her when I said that she was turning blue often...I never pushed to say how blue she was turning. It turns out that she was having severe Tet spells that were NOT okay. When I finally took her to the ER, we found her de-saturating into the teens (17% at the lowest). When she was transferred to the CICU on a Thursday, she was placed on propanolol to try and control her tet spells. Then when that didn't work, they tried morphine. Finally on Monday, the surgeon did her repair. She had several tet spells on the table while they tried to sedate her and start bypass.
I live with a ton of guilt because I feel like I should have pushed harder for her care. I don't feel like I was forceful enough in my description of her "blue spells". Clearly, her cardiologist at the time felt like she would have blue spells, but nothing this severe.
Thankfully, she is a bright, very talkative, and out-going child. I was very worried that she would suffer long-term consequences from my in action.
I really trusted her last cardiologist a lot. He was the one who ordered the holter monitor last summer.
Cardiologist #3 seems wonderful too. He engages my daughter and did not seem huried. He answered all of my questions in depth (at least the questions that I could come up with while there).
The problem is that cardiologist #2 and #3 differ in their opinions on tests.
Cardiologist #2 recommended that she have cardiac MRIs at least every other year to monitor her right ventricle dilation. Cardiologist #3 feels like an annual echo is sufficient, and that information gained from cardiac MRI is not worth the risk of sedation and intubation.
Both doctors agreed and have documented her sinus node issues and both doctors feel like periodic holter monitoring and "other" electrical studies will be in her future. Cardiologist #2 didn't give a time line for a pace maker, and #3 said teens/twenties.
Cardiologist #2 said that her valve surgery would probably be around 10 years of age, #3 says teens/early twenties.
With the memory of her infancy, and what I judge as my lack of action, I sit in a delicate balance. I definitely see changes in her stamina. She is so easy to read. Often she will be walking, and the corners of her lips will turn down and she will hang her head. She has to stop everything, right away. Also, she has periodic episodes (maybe five or so in the last year) where she gets very upset, and will turn blue across her forehead, and then have a long recovery period where she is nearly asleep (lays listlessly).
Yet again, I have a hard time being forceful in my description about these symptoms for two reasons. First, I would really like for her to not be limited (or feel that limits have been placed on her), and second, I worry that I will push her into unnecessary intervention, or that her doctors will label me as an over anxious parent (which quite honestly is an accurate description).
Perhaps I'm the one in need of medical intervention ;)....
After all that, what are your thoughts about cardiac MRI? Should I press for her newest cardiologist to provide this test?
And second, is there a point where I should not be managing her symptoms at home? What warrants a trip to the ER?
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