Obstructed Hypertrophic Cardiomyopathy (HOCM)

I asked in the expert forum but sometimes it takes awhile for the doctors to answer and sometimes I get more help from other patients than my own doctors, so I'm asking here also.

What's the "gold" standard of diagnosing HOCM or Obstructed Hypertrophic Cardiomyopathy is? (concentric) If a cardiac catherization and other testing said I have it, is that definitive that I do?

I found out through a new EP on June 3rd and the hospital last week for a head/neck injury, that my original EP back in 2009 diagnosed me with HOCM but never told me.  I knew it was a possibility but he was so focused on ARVD I put it out of my mind and never thought to ask about it again.  The dr at the hospital told me I should follow up with Mayo clinic as the EP suggested, so I put in a call to get an appointment and genetic testing.

I have a lifelong history of fainting & arrhythmia's, 2009 enlarged heart (cardiomegaly), 2004 SoB started that worsened in 2010 to laying down, edema in my hands & ankles/feet. I wasn't aware that HOCM could cause polymorphic VT, maybe that's caused by something else - no cause was found.  HOCM would explain why I reacted to Nitroglycerin the way I did during my tilt table test, yet my bp/hr stayed the same the 20 minutes before being given it.

Other than a patchy family history I don't know much...my mother had epilepsy at age 13 and died of breast cancer @52 and dad died last year of Lymphoma @67; but had an enlarged heart they think may have been due to treatment.  I went to the HCMA registered to ask questions, but can't post or reply; the mods said they were having problems.

Now I'm trying to figure out how to prove that diagnosis right or wrong. I want answers for not only me but my children - my oldest son started fainting recently (25) while at PT and my oldest daughter (24) has had lifelong arrhythmia's and fainted when she was younger, my youngest daughter (8) has tachycardia.

is_something_wrong posted in the HR forums with some info; which was great but I would like to hear from some other HOCM patients about what you went through to get dx, I know I've seen a couple in the HR forum who have it and would like any input from anyone who has it here in the HD forum also =)

[Mom2four85]

I forgot to add something:  

I've had hypotension my entire life until a few months ago...bp normally stayed at 90/60 and I was treated with Midodrine as well as other treatments to try and raise my bp to stop fainting episodes, nothing worked my bp would not go up even w/exercise it wouldn't raise much.

In 2011, my bp started being extremely high 150/100 - 189/140 and my dr suggested I quit taking the Midodrine, so I stopped.  My bp keeps rising though and staying high and the lowest it's been even off the meds is 130/85.

could this be related?

[grendslori]

Based on the things you have written on here, it certainly sounds like you have HCM. I would get and keep the appointment with the Mayo Clinic (or if you are closer, then the Cleveland Clinic). Your children also have to be screened for the disease as it is more times than not, genetic in nature. The Concentric form of this disease is also rarer. The diease can also cause VT and therefore arrhythmia issues need to be addressed. As far as testing goes, the Echo is probably the best tool for getting a diagnoses. The cath helps to show how still the chamber walls are, in other words, who well they contract with each beat. Take care

[grendslori]

Sorry.... I meant to say : "how STIFF the chambers are" not "how still the chambers are".

[Mom2four85]

thanks for the reply, I think I've talked to you about this before but my memory is horrible.

I asked Dr. Borek from CC in the live chat yesterday what could cause malignant arrhythmia's and he did say one of them were HCM.  ARVD was ruled out, Brugada and Long QT were ruled out.

I'm in less shock now and trying to learn everything I can about HOCM so I can add to my list of questions.

I had problems with my exercise stress test they couldn't explain, Concentric LVH on my Echo and wall motion abnormalities on my MRI they said I had to have further tests for so then I had the cardiac cath which then they dx me with HOCM and failed to mention anything about what it was but focused on what caused caused the problems.

I wish I could go back to the original EP to pin him down and get answers but I can't, so I'm going to stick with the new EP at the university here until I see the dr's at Mayo or CC in S. FL.

[grendslori]

Do you have high blood pressure? Sometimes HBP can cause HCM. If you have normal blood pressure, it's probably a genetic form of this muscle disease. In HCM the muscle fibers in the heart walls are in disarray, the fibers don't 'line up with each other' the way they should. The walls become too thick and as a result, too stiff. It's like, for an example, you trying to push your finger through a thick roast or steak....the meat just doesn't 'give'. The fact that your form is 'concentric' means that it is through all of the walls, basically. HCM usually affects the septal wall only, in the concentric forms it not only affects the septal (or dividing wall between the left and right ventricle) but also the 'outside walls' of the heart. My own daughter had concentric hcm, but in her case, all of the walls of her left and right ventricles were affected with the disease.  

[Mom2four85]

I've had Hypotension from age 9 - 44; with fainting spells from my bp being so low 70/40 - 90/60 until the past 4 or 5 months, it spiked w/o meds to 150/115 - 189/140.   They can't figure out why and said it was due to ANS dysfunction but I've questioned that, but it may be both HCM and ANS issues.

Mayo in Jax contacted me today for an appt so I'm waiting to see when I can get in, if they don't have some answers then I'm going to S FL Cleveland Clinic to Dr. Craig Asher which I'm told sees many HCM patients.