Restrictive cardiomyopathy and odd pacemaker behaviour

So, I went for the first stage of a heart transplant assessment on Tuesday -- mostly tests I'd had before (chest X-ray, ECG, ultrasound) but also an exercise test wired up to an ECG and monitoring breathing and blood pressure and oxygen sats.

As expected, my results showed (quoting the tech) 'you're rubbish at excercise'. Not really a surprise as persistent breathlessness and inability to exercise are why they're considering me for a hear transplant.

However, discussion with the consultant at the end of the process was interesting.

1. I have restrictive cardiomyopathy, and have had for 10 years or longer (although this is the first time it's been diagnosed.)  'Longer' is because it was probably the cause of my atrial fibrillation, which started being symptomatic in about 2004 and was what led to my pace and ablate procedure in 2018. (Procedure worked fine to stop me feeling the AF symptoms, but didn't cure anything else, unsurprisingly given the RCM diagnosis.) Next stage of transplant assessment is on hold pending investigation of the cause of the RCM -- no point in giving me a new heart of I'm going to trash that as well.

2. My pacemaker may be contributing to the problems exercising. At the start of the session on a fixed bike, heart rate rose as expected from 60 to 100/105. However, it then fell back immediately to 75/80 and stayed there stubbornly no matter what I did.

The Transplant Centre are writing to my cardiologist (who is also an EP) to suggest he investigates -- he only got involved in my case in February this year so he wasn't involved in any of the previous diagnosis/treatment or the implanation and tuning of the pacemaker, but he's pretty on the ball, so I'm hopeful I'll get an appointment some time in the next century (given Covid-19 restrictions :) )

Just curious however -- has anyone experienced anything similar, or can they suggest what settings might be awry? Boston Scientific L110.






Odd pacemaker behavior

by AgentX86 - 2020-09-28 13:09:34

Since you've had a pace and ablate (AV ablation), you're pacemeker dependent.  Your heart isn't in control.  Your pacemaker is. There are only two reasons your heart rate isn't staying up during exercise.  One is the heart is being paced at the proper rate but for some reason it's not pumping at that rate - pacing signals not causing contractions.  The other is that you're having ectopics that aren't being counted as heartbeats. I guess a  third possibility is that your pacing clinic doesn't know what they're doing and has your rate response all messed up.  In any case, it would seem the reason would be clear.  From what you've said, it sounds like #1.

Perhaps they're not telling you why because it doesn't much matter.  It doesn't change the fact that you're the tin man.  I wish you well.

Restrictive cardiomyopathy and pacing

by Selwyn - 2020-09-29 09:45:29

My hypertrophic cardiomyopathy does not interfere with pacing. I suspect that a restrictive cardiomyopathy does as the muscle itself is diseased  ( not just more of it as in the hypertrophy case). 

Firstly, you need to ensure that your pacemaker is set to respond to exercise ( rate response). I would think if that was working then  the threshold values for getting the electrical signal to your heart muscle to contact may need to change with time, as some diseases causing restrictive cardiomyopathy are progressive.  It would be worth talking to the pacemaker people about your need for this as original settings may be out of date due to your disease.

I play table-tennis with a heart transplanted guy ( he's had his new heart for some years now).   Although we are both having to be very careful at present because of Covid ( I spoke with him a couple of days ago), and altered immunity,  life is still meaningful. The UK has an excellent regional transplant service. Whilst we can hope for best it is nice to know there is a 'get out of jail' card available.

WIth sincere best wishes for some decent health. 



To AgentX86

by atiras - 2020-10-02 04:46:58

The Ultrasound shows my heart contracting in line with the pacemaker signas... and it's pumping efficiently (the problem is filling). My suspicion is the rate response settings aren't right -- probably the minute ventilation.


It's not that 'they're not telling me why' -- it's because the heart transplant consultant doesn't know and is referring me back to my cardiologist for investigations. He clearly said it matters, as it's affecting my exercise tolerance.

To Selwyn

by atiras - 2020-10-02 04:49:54

Thanks for the encouragement -- I am thinking rate response is to blame for the odd behaviour. For the rest, who knows. Scan ina coupld of weeks for wAttr Amyloidoisis to rule it out or in, and we'll see whre we go from there.

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