Arrythmogenic cardiomyopathy

[Chinkoflight]

Does anyone on this support group have this as a diagnosed condition and what was your pathway to getting the diagnosis? I have experienced completely out of the blue heart events over the last two and a half years and NHS treatment in less than ideal circumstances, post pandemic etc.

Severe stroke - March 2022, thrombolysed and for the most part a full recovery. Brilliant emergency care. Poor, none existent follow-up. So on advice of my GP private echocardiogram, Holter monitor and cardiologist review diagnosed cryptogenic stroke, no obvious cause. Antiplatelet and statins prescribed.

GP routine blood tests for Clopidogrel medication review showed some issues which he wanted to check the liver. Ordered an echocardiogram. Liver clear but gall bladder (happen chance) was full of stones (asymptomatic). Referred to specialist (NHS) who advised a quick key hole procedure to remove. Viewed as urgent because of the number of risky stones. Apparently there were complications on the operating table with signs of severe inflammation (asymptomatic) causing the key hole operation to be abandoned for a full surgical operation to remove the gallbladder and extended stay in hospital.

February 2023 after no interim NHS follow up for the stroke a letter came to advise me to have a Implanted loop recorder (ECG 24/7 monitor) fitted and three months later a singular short Afib was detected. Medication was changed to anticoagulant Edoxaban.

January 2024 while on a Parkrun I collapsed (syncope) with no warning and no symptoms. 4 days in ED, eventually my ILR was interrogated and showed a 'textbook' 23 seconds left ventricular tachycardia . A further 6 weeks in general hospital coronary care unit for tests before an ICD was implanted. Echocardiogram clear, angiogram clear. I think the latter result was almost a disappointment for the Drs as they had advised me I might (would) have stents fitted at the same time. Because they couldn't find a problem my diagnosis was Idiopathic Sudden Cardiac arrest. (No diagnosis in my book) A cardiologist in the team suggested I'd had a silent heart attack! I'm clearly just a person who has no symptoms!

However one cardiologist decided I should have more tests that weren't available in the GH. So I have to wait until I can get a very expensive day out to Oxford for a specialist MRI. This shows I have severe thinning of an area of the left ventricle with dilation as well as scar tissue. Clearly the likely cause of the LVT and of course a permanent future risk. The recommendation is to pursue investigations for a non-ischaemic cause. Although some time on from the event a further specialist PET scan is arranged back at Oxford for the day to look for evidence of sarcoidosis. Non found on the heart but inflammation found around the lower spine and pelvis! (No symptoms) Left for another day!

No further tests were done, the ICD was fitted and discharged. No ischaemic or non-ischaemic diagnosis was ever offered including in my notes to my GP. No ongoing support has been offered and my recent 6 month follow-up was a 'desk based review' of my remote ICD data. It was described as a hospital appointment but I didn't have to attend and wouldn't have any follow up if no problems were detected.

Do I have grounds for thinking I may have an arrythmogenic cardiomyopathy?

From my private cardiology GP referral post stroke,until my admission to cardiology after my SCA I didn't have any contact with a cardiologist, and since my discharge no follow up contact other than the desk based ICD check. Is this usual?

I am normally calm and quite stoical but the level of uncertainty about 'the next event', imposed lifestyle changes ,eg no driving, but no advice or support on lifestyle , exercise etc I'm feeling a bit at sea.

I know I'm looking at a rare set of circumstances but clearly the diagnostics point in the direction of a less common non-ischaemic cause.

[Ppiman]

That is an amazing set of events. Stoical you must be! You really have been through the mill. Phew. Is your EF and things like BP and weight in the deemed-to-be healthy range? Have you been checked for sleep apnoea, too?

I suspect I would be asking what might be done to protect the ventricles over time. With the heart I get the feeling, however, that it can be really hard for doctors to know as much as we like to think they do.

Steve

[Chinkoflight in reply to Ppiman]

Hi, thanks for the reply. EF okay, overweight but gradually coming down but overall fairly fit and active. However, I was okay and comfortable with exercise but have recently felt a level of anxiety that I'm just not used to. The Drs have a high dose of pragmatism. From the initial spot of the LVT there was only one treatment option to fit an ICD whatever the cause. There was no desire to find a cause, they were happy to say heart attack because of raised troponin levels. But they did the echo and angiogram and were surprised. They were never going to fully investigate non ischaemic causes. I was just hoping to find out whether that's typical of most general hospitals at the moment or just the one I was in, which is in need of improvement.

If I were right the think arrythmogenic cardiomyopathy was a possible diagnosis then I am right to feel anxious about exercise and possible prognosis.

[Ppiman in reply to Chinkoflight]

I wish you the best. You seem to be doing all the right things.

Steve