Hypertrophic Cardiomyopathy

What a mouthful, huh...well, that is my new diagnosis...conclusion, at this current time "have fun, ride with your buddies, DO NOT RACE, NO ANAROBIC EXTENDED STATES, take it easy enjoy your rides...so says the Doctor...and thats what I'll do...I had these instructions prior to Bear Creek, but kept it to myself just to get the last race of the year done, it was hell...ESPECIALLY when Jennifer found out...I told her AFTER the race, she was pretty upset, understandable...but ya all know me, if it wasn't the race to kill me it'd be her...hahaha...serious though (no laughing matter)besides, I hate all the "fuss"...the DH was easy enough, but as most of you racers may have noticed (especially if you were passing) I spent most of the uphill sections walking...I was keeping my heart rate at a tolerable pace (ie under 160 bpm for me)...but for the next 2 months NO RACING...well have another stress test and more examines in November, then every 2 months after that to see if there has been any change...med's have been up'ed a bit, all is well..no need for panic...just cannot be my "Obsessive Compulsive" self anymore...

What is "Hypertrophic Cardiomyopathy" you ask...here is a definition:
"HCM is a complex but relatively common form of genetic heart muscle diease that occurs in 1 out of 500 people, but often goes undiagnosed in the community, and has caused some confusion to both patients and physicians periodically over the years. HCM is the most common cause of heart-related sudden death in people under 30 years of age, and it can also be responsible for exercise disability at almost any age. Although HCM is a chronic disease without a known cure, a number of treatments are now available to alter its course." It has been referred to as "Athletes Heart" The heart has become enlarged and the left ventracul muscle has weakened...

This is a cut and paste that explains it all.....
The Many Names and Other Sources of Uncertainty

Much of the confusion about HCM, as well as the limited awareness about the condition in the general public, comes from factors such as the vast array of complex names and acronyms given to the disease (over 75 in number), and its infrequent occurrence in cardiology practice. Patient support and advocacy groups have closed the information gap by using the internet, facilitating more effective communication between interested parties independent of geography (Hypertrophic Cardiomyopathy Association [HCMA] web site is available at http://www.4HCM.org; phone (973) 983-7429 or (877) 329-4262).

How Is HCM Diagnosed?

HCM is usually identified by an echocardiogram that produces ultrasound images of the thickened wall of the heart muscle (hypertrophy of the left ventricle). This is usually most prominent in the ventricular septum (the wall separating the left and right ventricles), but is not accompanied by an enlarged cavity. Normal thickness of the left ventricle is 12 mm or less; in HCM, the thickness is usually 15 mm or more, although we know that some people who carry a mutant HCM gene may have normal wall thickness.

In HCM patients, hypertrophy does not usually appear on echocardiogram until early adolescence and then may increase dramatically until the end of the accelerated growth period. However, since hypertrophy may not begin until middle age, we now suggest that some relatives of those with diagnosed HCM should be checked with an echocardiogram periodically well past adolescence. Small differences in wall thickness reported to adult patients from one clinic visit to another are usually not clinically important; technical factors such as the angle of the sound-wave beam can account for such variations. Sometimes modestly increased thickness must be distinguished from the innocent consequences of athletic training (athlete’s heart) or high blood pressure.

Echocardiograms will also show whether (partial) obstruction of blood flow from left ventricle into aorta, caused by forward motion of the mitral valve, is present (and to what degree), and also whether there is abnormal leakage through the mitral valve. Invasive cardiac catheterization or electrophysiological studies are now rarely necessary. The ECG usually shows a wide variety of abnormalities but is of limited value in HCM, with the exception of family screening.

Your physician may suspect HCM by the presence of a heart murmur, new symptoms, abnormal ECG, or family history. Physical examinations alone, including those prior to participation in sports, are not reliable for identifying HCM because about 75% of patients do not have obstruction to the outflow of blood from the left ventricle and a loud heart murmur is therefore absent.

Inheritance

HCM is caused by mutations in any one of 10 genes and appears in 50% of individuals in each generation. The mutant genes that cause HCM influence certain proteins that are part of the heart muscle. Therefore, when HCM is diagnosed, all close relatives are advised to have an echocardiogram. While analysis of DNA is the most certain method for diagnosing HCM, it is time-consuming, expensive, confined to research-oriented laboratories, and cannot yet play a role in routine day-to-day decisions for patients. Gene therapy would be a daunting task and is not presently a realistic expectation to become a cure for HCM.

General Outlook and What to Expect From HCM

HCM is unique because it may be identified during any phase of life, from infancy to old age (sometimes over 90 years). While its potential adverse consequences have been emphasized for years, particularly the possibility of sudden death, a more appropriately balanced perspective on HCM has emerged.

It is a myth that HCM represents a generally unfavorable disease. Its risks have probably been exaggerated to many patients. In fact, realistic mortality rates for HCM are only about 1% per year and are not dissimilar to the general US adult population for all causes. Therefore, HCM frequently causes no or only mild disability over a lifetime, and many patients achieve normal life expectancy (some without even being aware of their disease). Therefore, many HCM patients deserve reassurance about their prognosis.

Sudden Death

Sudden and unexpected death is the most devastating and unpredictable complication of HCM, but only a minority of patients are actually at risk. Sudden death in HCM may occur without warning signs and is caused by lethal heart rhythm disturbances (called ventricular tachycardia and ventricular fibrillation) that probably originate from the disorganized heart muscle structure or from small scars. Patients are rarely aware of rhythm abnormalities that may precede sudden death; however, fluttering, pounding, or skipped beats (palpitations), as well as dizziness and fainting, should always be reported to the cardiologist.

While sudden death occurs most commonly in children and young adults, the risk extends into mid-life and beyond (although less frequently). Reaching a particular age does not therefore confer immunity from sudden death. Sometimes sudden collapse occurs with vigorous exertion on the athletic field; athletes with HCM should be disqualified from most organized sports to reduce their risk.

A number of risk factors for sudden death have been identified, although most patients will never experience a life-threatening rhythm. HCM patients should have a clinical risk assessment with history and physical examination, echocardiography, 24-hour ECG recording (Holter monitor), and exercise testing, and should be routinely evaluated by a cardiologist about every 12 months.

Who Is at Risk?


Patients with a prior cardiac arrest ("heart stoppage").

Patients with one or more family members with sudden death caused by HCM, particularly when closely related.

Patients who experience fainting (syncope) that is otherwise unexplained, particularly when it is related to physical activity, occurs repeatedly, or appears in young people.

Patients with brief episodes of rapid heart beat (ventricular tachycardia) shown on Holter monitor when present on several recordings.

Patients whose blood pressure fails to rise during exercise testing, particularly in those younger than 50 years of age.

Patients with extreme thickness of left ventricular wall (30 mm or more) on echocardiogram.

Symptoms

Some patients with HCM develop shortness of breath and chest discomfort, as well as fainting, dizziness, palpitations, and fatigue, with physical activity. Symptoms may begin at any age and often do not appear until mid-life (30s or 40s). Symptoms can develop at different rates, with long periods of stability, and often vary from day-to-day; severe exercise limitation is, however, uncommon. Occasionally, patients may be unable to sleep in a flat position or may awaken short of breath.

HCM involves a unique form of heart failure in which the heart muscle is often not dilated and flabby, but rather is stiff and has normal pumping capacity. Shortness of breath results from the high pressures in the heart chambers and can be controlled with ß-blockers, verapa- mil, or disopyramide. Although any patient with HCM can develop symptoms, those with obstruction to the outflow of blood from the left ventricle are most likely to experience severe disability.

Patients with obstruction should take antibiotics before dental procedures to prevent blood-borne infection of the mitral valve. Women with HCM generally experience little difficulty during pregnancy and delivery, with the exception of some of those with advanced disease.

Atrial Fibrillation

Atrial fibrillation occurs frequently in HCM and accounts for many unexpected hospitalizations and unscheduled work loss. Atrial fibrillation can be well tolerated and does not increase the risk of sudden death. In older patients, it may cause heart failure and stroke (clots can form in the enlarged and fibrillating atrium and travel to the brain). Because of the risk for stroke, anticoagulants are usually recommended. It may be necessary to control heart rate with drugs, or restore normal heart rhythm with an electric shock, or with medications.

Treatment

The implantable cardioverter-defibrillator (ICD) is the most reliable and effective treatment for HCM patients at high-risk. It has the potential to alter the disease course by automatically sensing and terminating lethal disturbances of heart rhythm, often in young people with little or no symptoms. ICDs are clearly warranted for those who survive a cardiac arrest, but should also be considered as a preventive measure for other high-risk HCM patients after taking into consideration the strength of their risk factors(s), the level of risk acceptable to patient and family, and the access to ICDs.

Should symptoms worsen despite medications and the patient’s lifestyle become unacceptable, major decisions about treatment depend on whether blood flow obstruction is also present. For those patients, the most standard option is the septal myectomy operation, in which the surgeon removes a small amount of muscle from the upper part of the septum. At experienced centers, myectomy has low operative mortality, and most patients experience long-lasting improvement in their capacity for physical activity. This is due to the return of pressures within the heart to the normal range and the elimination of mitral valve leakage.

For those patients who do not have ready access to major centers experienced with this operation, or who have unacceptable risk because of other medical conditions, advanced age, or previous heart surgery (or are insufficiently motivated for operation), 2 other treatment options are potential alternatives to a septal myectomy. Pacemakers may improve symptoms and reduce obstruction in some HCM patients, particularly those of advanced age. The data from several controlled trials suggest, however, that improvement with pacing is often largely a placebo effect.

The septal ablation technique has been developed, in which a small amount of absolute alcohol is introduced into a small coronary artery branch for the purpose of destroying heart muscle in the septum, leading to reduced obstruction and symptoms. Alcohol ablation and septal myectomy have similar risks. However, ablation is a new technique, follow-up of patients is relatively brief, and there is some concern that the permanent scar produced within the sep-tum could eventually generate serious rhythm disturbances and actually increase risk for sudden death.

Treatment options are more limited for patients having severe symptoms without obstruction, such as those reaching the "end-stage" phase in which pumping capacity becomes impaired. Such patients may become candidates for a heart transplant.

Common Misconceptions About HCM


With HCM will my life be shortened? Probably not, although the disease can have important implications for some patients. HCM is compatible with normal life expectancy, often with few if any complications.

I am afraid my heart will continue to enlarge until something bad happens. Usually the thickening process in HCM ceases by the time full growth and maturity is achieved (at about age 17 to 18). There are extraordinary exceptions, but this rule covers about 90% to 95% of the relevant clinical situations.

Will injection of alcohol into my septum be a cure? Alcohol septal ablation is a promising alternative to surgery that can decrease obstruction and symptoms. However, HCM is a chronic disease, and none of the available treatments can be regarded as a "cure."

My cardiologist says I have obstruction and need a major procedure, but I feel fine. Obstruction can have consequences over long periods of time and may need to be relieved. However, major interventions such as surgery and alcohol ablation are not justified unless patients also have symptoms that significantly limit their lifestyle.

I am afraid I am getting worse; my obstruction went from 20 (mm Hg) to 30 (mm Hg); or, I am getting better; my gradient went from 30 (mm Hg) to 20 (mm Hg). Patients should be aware that obstruction in HCM can change under a variety of circumstances from day-to-day (even hour-to-hour); therefore, small variations in either direction should not be taken as evidence of worsening or improvement in their disease.

I have been told that I may need surgery, but that it is dangerous. I think I would like to try something safer. Actually, in experienced hands, surgery has a risk of only 1% to 2% (and even less in recent years), which is similar to that for alcohol septal ablation.

I have heard that HCM is more common in men. HCM is transmitted as a dominant trait and precisely one-half of those who inherit the gene are men and one half are women. However, HCM often goes unrecognized in women, who represent only about 40% to 45% of those patients in published clinical studies.

WHEW...thats a lot of sh** isn't it...but that helps explain it for those that might be "freakin" out, it's all good...I've got good doctors and am pretty stubborn...

On another note, one of my riding buddies from the west coast is coming out this week and will be going out riding this weekend...I will be taking him to see the local "stomping" grounds at Patapsco, then going up to Gambrills Water Shed for some more "narly" east coast riding...anyone is welcome to come along, let me know...

C-ya all on the trails, but NO RACING!!! :)
Ed

2008 Race Season Completed!

This past weekend was the Bear Creek Summer Showdown, last Race of the MASS series for 2008. I started the year out in California with my first race on February 10th 2008...since then this past weekend completed 30 total races for the season..way too many! I started getting burned out approx a month ago...tried to revive my spirt by doing group fun rides during the week so as not to think of it as "training", but I guess I just needed to take the time off, would have been better all around...Oh well I did survive...

Finished up the weekend in 10th out of 10 in the Single Speed class, and 4th overall for the season...I am very proud of myself for the acomplishments gained this year...Competing in the SS class in the beginning was somewhat humbling never having the opportunity to stand on the podium (once this year)going against the "young guns" did become rather fun "ribbing" some of the youngins that this old man just about beat ya..:) For you non-racers, the Single Speed class is "open", it does not have an age range like the other classes...which I will compete in next year, the 50+ guys (if my Doctor permits).

Bear Creek is a very suitable race course for a finale...it has climbing that will sap your lungs...rocks like you've never seen...lots and lots of them, strangley very few log hops...funny I remember last year "blasting" through this course, granted I was on a full suspension geared bike and we only had one lap to do...I think this year with "burnout" and SS it got the best of me...I was determined to finish even if I had to walk the whole course, which it felt like on the last lap...the rocks actually felt good today, I went through most all the rock gardens riding them and not "dabbing". But the hills really took their toll...I had to pay close attention to my heart rate this race so I spent alot of time recovering on the hills by walking and then "bombing" on the DH's.


I will really miss the weekend races for the group of friends vs. the races themselves, that is the biggest fun about racing...hanging out with all my new buddies...hopefully we can all continue to get together throughtout the fall and winter doing "fun" rides...I know there is a Jamboree in Nov. so I'm already counting down the days...I really enjoy my group rides on Tuesday night and Wednesday night, their all a super bunch of guys to ride with...now if I can include maybe some additional nights...and for you that know, don't forget we still have to complete on "adventure" at Patapsco real soon...

After my upcoming Doctor appointments I'll keep you all abreast as to my riding abilities, may need to slow down a bit for a while, we'll see...until then, keep pedaling, rubber side down, and all those other cliches, c-ya on the trails...

Oh yeah, and BIG thanks to everyone for making this past year so great!!! Love ya all for your support and encouragement, but most especially just for your friendship, it means alot to me to hear you all acknowlege me at the races and I apologize to you if I had not remembered your name....I do remember your faces though, and it makes me very happy to have done something for you too. Again THANKS!!!

Stans Blowing Out

This was during the Shasta Lemurian Race in California this past May...the "smoke" coming from the front tire is actually Stans burping out!!!

Back To Back Weekend

Went up to Randy & Gunnars cabin this weekend for a couple races...the first race was on Saturday at Wellsboro...the 15th Annual Laurel Mountain Classic...Gunnar kept teasing me about ALL the STEEP climbing that was invloved...he wasn't teasing, there was..whew!!! Rather tough on SS and just 10 days after surgery, 21 miles too...the location was perfect, central northern PA...just on the edge of the Hurricane, so we only received a slight drizzle AFTER the race...This race featured the Single Speed class as OPEN for sport/expert, yikes, felt strange looking back at the Sport pack, and being up front with all those expert riders for the MASS start...heck I was right behind Ray from Visit PA team, didn't last when the "horn" went off, hahaha...

More to follow, I'm tired right now, just had to get something up for now, will write some more tomorrow...BTW thanks, Randy & Gunnar, your great friends!!! Todays race made my 29th of the season!!

Well I'm back...I've been to the doctor to check out why my thigh swelling hasn't gone down (actually grew a little)..well it seems to be full of blood, so he may want to go back in and "suck" it out...find out more on Thursday...

The Laurel Mt Classic was everything everyone said it was...very grass roots, steep, variety, (ie water, logs, rocks)...but you know in a good way, it seemed to flow, never got to the point where it was to much for too long, just the right amount...the weather was okay...it didn't rain during the race, and the sun wasn't out, but it made it very humid, your sweat couldn't evaporate (consequently, wouldn't allow your body to cool)...but brother the descents on this ride would certainly cool ya off with the speed you could get...
The "Stinger" was something else...VERY STEEP DH...sat way back and just pretty much "slid" through...tapping off the brake just in time to make corrections in the direction...
then came the "GREEN MONSTER" everyone was talking about...a grassy hill with a single track in the middle and it just kept rising, just when you thought it was going to top out you came over another little rise to see it going further...must have lasted 15 plus min.s... you know though every time there is a lot of climbing there is always the reward of some kind of DH...one little short burst of DH under the power lines was a grassy descent which had a hidin little "bump"...it had a very bad angle to it...saw it at the last second, yanked my front end up tied to bunny hop the ass end at the last second and "whew" cleared it...could have been a very nasty endo...I heard someone did get hurt there...

The end of the race was FANTASTIC...it was a "Super D" all the way..at least 2+ miles of DH Single Track, mostly smooth, a few rock sections (but going so damn fast just skimmed right over them, passed approx 3-4 riders by yelling out "hot rider" and they'd pull over, thank god cause I had some great 30+mph speed going here...it was actually so long my arms were starting to tire and I was wondering if you really could get tired of DH...hahahaha...NEVER, but whew tough on the arms...there was one little dip which I saw at the last second braked HARD, then let off and jumped it the best I could and got back on the pedals as fast as I could to get the speed back up...then boom...right out onto the dirt road where we started, round the corner and a "squirt" to the finish...no one behind me and no one in front...but I still pedalled it out like a hammster...always finish in a sprint even if by your self!!!

We then headed back to the cabin....more again in a day or so....

Spent another great night at the cabin...very cool place way up near the top of a mountain.

BTW on the drive north from Baltimore I discovered that the front tire holders on a Thule bike rack don't hold up well over 100+MPH...they want to come off, the tire holder flopped back some and one tire actually came a little loose, so in the car they went...the bike holds firmly though...

Sunday we got up early for a long drive over to Ringwood New Jersey for another race..yep a glutten for punishment. Looking back I really shouldn't have done this race, or for that matter either, simply because I thought my leg would be fine well....it may be the unerlying reason for the "GIANT" blood blister now...but oh well, I am "hard" headed....the day turned out perfect htough and the location was awesome, right next to a lake. You would never know that we're only 30 miles outside New York City in "gods" country. The race was 2 laps of (they said) 7 miles, my computer read 8+ miles...the course started out on a fire road relatively flat, then a gradual climb at te end of the road, slowly turning into double then single track...and it kept getting rockier and rockier as we went...so much so that I stopped enjoying myself and so did my leg when I endod and hit it...ouch...so by the time I finished the first lap I had it!!! I pulled out and waited for Gunnar...he did really good, Gunnar got 2nd place, way to go...

We saw some familar faces and spent some time talking to them...Aaron Synder, Rob L. and Matt...Great guys...Aarons a very good ambassador for the sport...he goes out of his way to approach, encourage and talk to the young racers, nice to see...thanks Aaron...

A few things I took out of this weekend (and even at this age we all still continue to learn)...1. trust you doctor and your gut feelings 2. Pre-ride, not knowing the course and selecting the wrong gear on a SS will "kill" ya :) 3. If it's not fun, DON'T DO IT...save yourself for another day :)

Have fun all!!! Keep the rubber side down!!! And help others along the trails, who knows it may be you sometime that needs the help...KARMA!!!

Going to Try and Ride Again

Broke down and ordered a Chris King hub for my "play" rim...since I bent the hell out of my rear I9, decided to get that fixed, but in the meantime have to ride on my stock Rig wheel and it sucks...Have gotten spoiled with the instant engagement of the I9...and your right guys, I am "spoiled", but why not do it the best way...so I will relpace it with the Chris King and have instant engagement and yet have a stronger rim I won't be to worried about bending or breaking...actually going to put it on the stock Bontrager Race X that came with the Superfly...make it just a Single Speed for now (since thats the way the "Fly" is set up) and yet it will be a hub that could always become "geared" again if need be...and will be a "sweet" hub at that...

Going to try riding tonight, will take it easy, don't want to rip the stiches open, but dying to ride...so I'll wrap the leg REAL GOOD to hold it all together...and no POWERING up the climbs, if need be just hike-a-bike...should be well enough for Laurel Classic this Saturday (crossing fingers)...

Hope everyone had a great time riding this past LOOOOONG weekend..let us all know about your rides....


Ed