Update on heart tests

I had cardiac biopsies, tested my RV lead to make sure it is functioning properly and will administer a shock (lead my have a fracture as my voltage is low and variable), did right heart cath to check pressures in chambers, and inspected valves.  

12 cardiac biopsies have been sent to 3 labs.  One specific lab works on genetics.  One lab will corraborate with the biopsies taken a few years ago through a German study.  My EP is excited and hopefuly they will find out what is causing my heart to progressively thicken and stiffen.  He said there is no coronary artery disease and my vessels are very healthy.  The biopsy areas included my left and right ventricles to find out why I have so many areas that produces arrhythmias, and the septum, which has areas with no movement.  

My aortic valve is of a concern and he will discuss with me when I see him in the office.  He said some of my symptoms are most likely from the valve's moderate leaking.  I had my mitral valve repaired a couple of years ago due to damage from the changing size of my heart.

My right atrium had high pressure and he said he was not sure why.  I'm sure he will discuss with me when I see him.  Anybody with ideas let me know.  

He put me on a new drug to help with my almost constant chest pain, which he feels is caused by the thickening of the muscle.  

He spent 4 hours and I am really having a slow recovery.  I am having low blood pressure problems.  I am taking the same meds I took before, so I'm not sure why the change.  The new drug does not affect my bp.  My legs feel like I've run a marathon just climbing my stairs, breathless and having to sit down.  My swelling is not caused from volume overload (seen on ICD report), and I wake up with swelling, particularly my feet and lower legs.  

Thanks for listening to me.....  it's been a long road since 1999 that I have been dealing with many different arrhythmias.  I am grateful for every day, surviving a SCA in 2016 (pre ICD) and another one in 2019.  The EP seems very concerned and I feel an urgency for him to try and find out what is causing my cardiac changes.  He said there has to be a reason for the cause of the thickening and stiffening, it just doesn't happen on its own.  I've been under his care since 1999 and he is an amazing person and brilliant EP.  

Happy Thanksgiving to all!  Stay well and stay positive.  We are all lucky to have the technology available to us today.


Hope things improve for you

by Gemita - 2021-11-25 17:12:13

Thank you for the update Islandgirl.  You are an inspiration and you can be proud of what you have achieved since 1999 and it sounds as though you are not yet finished.  I wish you lots of luck on the biopsy front and hope that these will give you and your EP the answers you both so deserve. 

I know it has been a long road but try to go just a little further and you may well find your answer.  Your EP is clearly hopeful they might find the reason for the stiffness and thickening of your heart.  If so, this could help improve future treatment.  At least you have no CAD which must be reassuring.  What new med did your EP give you for the chest pain Islandgirl?  Perhaps he can add a safe med to help increase your blood pressure?

I note they found high right atrial pressure and that you received a right side heart catheterisation.  Have you been diagnosed with right sided heart failure or Pulmonary Arterial Hypertension by chance?

I send my best wishes Islandgirl and hope you recover some strength.

It would be great to find something

by crustyg - 2021-11-26 05:06:09

Having a recognised cause for your many challenges might well help, and if nothing else it would give you a mental target to 'blame'.  And diagnosis often helps with prognosis - although that's not always welcome news.

Odd that you mention cardiac biopsies and genetics in the same breath: it's much easier to send a blood sample, and the days of looking for ragged red fibres are long gone.  But mitochondrial heteroplasmy might be what's being sought and, if found, would explain a lot.

The genetic diagnosis 'success' rate isn't very high - apparently for dilated cardiomyopathy it's in the mid-20% - but the best labs are adding more genes to their panels every year, and refining their techniques to miss less.  It's not you, I know, but at least one contributor here had three attempts to find a genetic diagnosis before finally a lab proved Brugada syndrome, and the genetics industry abounds with stories of well-respected labs (ACP etc. accredited) that missed defective genes (even something as 'simple' as cystic fibrosis is actually quite challenging).  If it weren't for the costs involved, I'd have all genetics samples sent to two different labs.

But it's great that your heart docs recognise that you need a more comprehensive work-up.

Best wishes.


Genetic testing

by Julros - 2021-11-26 16:26:55

My eldest son pressed for answers after having a flutter and a fib onset at age 40, along with an abnormal MRI. He had genetic testing done at a through a major teaching university, and they did it via blood sampling. They found an LMNA defect, and he was fitted with a pacer/ICD shortly after. I have since had genetic testing but it was done on saliva, and perhaps because they knew what gene to look for, I had results in 2 weeks and I have it too.

It doesn't really change treatment, but knowing what it is and knowing there is research being done does offer some comfort. I do wonder if I really benefited from the a flutter ablation I had done, because I seem to in flutter most of the time now, and I think the occurance of arrhythmias will increase with this condition. 

Thank you for your comments

by islandgirl - 2021-11-26 21:51:26

You are all awesome and give me information to expand my knowledge and questions.

I do not have pulmonary hypertension, and he was thinking before the tests I may have mild PH, from the last echo. I didn't ask him what my EF was, but it's been around 40-45%.  I have dyssnychrony and he adjusts it without success about every 6 months with optimization. When he changes my device this year due to battery depletion, he will work with the insurance company to approve a 3rd lead. My ventricular pacing has increased to 99.8% over the past year (no heart block), and I pace 100% in the RA due to SSS.  I have diastolic dysfunction, heart failure and cardiomyopathy.  My meds include sotalol, sprionolactone, ramipril, cardizem, xarelto, and now gabapenten.  I actually think the gabapenten is helping my chest pain? The sotalol has really helped me with my ventricular arrhythmias.  My diastolic pressure runs high and is managed by the ramipril.  I had atrial flutter just over a year ago and ablation has taken care of it, with my EP sending me a couple of hundred miles away to another EP to perform the complex ablation he had found I needed.  As some of you know, I've had multiple ablations for numerous atrial arrhythmias--no AFib.  My ventricular arrhythmias can't be ablated due to the numerous areas they originate from, in both ventricles.  

I have had cardiac genetic testing in the past, and the most recent cheek swabs revealed 4 genetic markers that included SCA, cardiomyopathy, HF, arrhythmias.  It was with a new company out of San Francisco that he found at a recent EP conference.

Afer the worsening echo in September, there was an urgency for the biopsies.  He said I have many of the signs of amyloidosis, and my problems started in my 40s.  

He is having his medical students and residents work on my case as a project. 

Does anybody know anything about high pressure in the atrium?

Thank you all again for you knowledge and help!

High right atrial pressure

by Gemita - 2021-11-27 02:32:11

Hello Islandgirl, Yes Gabapentin can be used for chronic non-ischaemic chest pain I believe.  Gabapentin is a neuropathic, anti-epileptic medication.  It can be very effective in the treatment of chronic pain in general, but it may cause increased appetite and peripheral edema so you might like to ask your doctors about this and the safety of staying on Gabapentin long term.  

?Causes of high right atrial pressure (RAP) - I won’t pretend to fully understand this subject.  Have a look at the following links (the second link is quite a study).  With your past history it would perhaps not be too difficult to understand why you might have high RAP - ?from fluid overload, heart failure to decreased cardiac output, which won’t be helped by your arrhythmias.  



The reason why I asked about pulmonary hypertension (PH) is because my husband has been diagnosed with this condition as well as right side heart failure, both difficult conditions to manage.  PH has caused his increased right atrial pressure.  We are starting a 12 week course of pulmonary rehabilitation next week (online - not sure how that is going to work).   The cause of his PH is believed to have been inflammatory and autoimmune in nature.  He has COPD and CAD and arrhythmias, both atrial and ventricular, although his ventricular tachycardia thankfully was effectively treated by opening up his LAD artery with two stents in 2018.  He was lucky to have survived.  With PH progression, the increased pulmonary vascular resistance to blood flow leads to increased right atrial pressure and right heart failure.  This is where we are but I note you do not have this condition nor CAD which must be such a relief for both you and your EP.  You have more than enough to cope with

Thank you Gemita

by islandgirl - 2021-11-29 00:21:06

Thank you very much for your references and information.

He had mentioned that the atrial pressure my be an anomalie and he wants to check into it further.  When my echo was done in September, he originally felt I had mild PH.  I sure hope not.....

I am having incapacitating low BP periods, and I believe it is gabapentin, and spent about 3 hours back and forth texting with my EP late yesterday afternoon/early evening going over my med dose log and BP/heart rates, having me take my bp lying down and standing up multiple times.  He is fiddling around with my med doses and says I must increase the gabapentin to 600 mg 3x/day, and he is increasing the dose adding 100 mg doses weekly.  He is also concerned about my aortic valve regurgitation and feels an urgency to work on remodeling my heart to improve my valve function.  


by Gemita - 2021-11-29 12:28:48


Islandgirl, I used to take Pregabalin for neuropathic pain control (a similar med to Gabapentin).  Have a look at the above link.  It mainly refers to the potential dangers of Pregabalin, not Gabapentin, but I think it might be worth discussing with your EP whether Gabapentin is really the best med for you?  I would want to be on the lowest dose possible of Gabapentin to help with any chest pain if that is what it is being used for.  They wanted to give me Gabapentin but the dose needed to control my nerve pain was extremely high compared with Pregabalin, so they switched me to Pregabalin which worked well at lower doses.  

I hope you stay safe. 

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