Do you understand what this means?

[Rainey]

I got some of my records from my cardio today so that I could take them with me to my son's echo on Friday. I just wanted to run this by everyone and see if the numbers mean anything to anyone. It is part of the results from my TEE back in November when HOCM was diagnosed.

"doppler interrogation is unremarkable at rest. No resting aortic outflow tract gradient is identified. At rest following and isolated PVC, the outflow tract velicity is 7.3 m/sec. Following amyl nitrate administration, the post PVC outflow tract velocity is 8.1 m/sec. Following amyl nitrate administration, there is marked systolic anterior motion of the anterior mitral leaflet into the outflow tract with an outflow tract velocity of 6.8 m/sec."

Huh? I do know that I only have a "mild" case of this but I'm confused. It says in my report that "left ventricular chamber size is normal. There is disproportionate subaortic septal thickening present. Maximum septal thickness below the aortic valve is approximately 1.8 cm." Does anyone know what the normal measurements are? I thought that I had thickeness in all the walls of my left ventricle - now it seems like its JUST the septum. I also remember him telling me that my measurements were 1.6 - 2.2 but right here in the report it clearly says that the MAXIMUM is 1.8. I'm a little confused. Can anyone help me with any of this terminology and measurements?

[Reenie]

Measurements can vary depending on the technique used and the technician who does the echo. Does it say anywhere on your report about gradient, measured in mmHG? That would be milimeters of mercury and it tells how much, if any, obstruction there is.

Normal septal measurement is about 1 cm and it's the same size throughout. Many with HCM have a "bump" that's thick in the septum. Some have thickening throughout the left ventricle but the septum is usually the most affected.

As far as you having a mild case, I'm not going to say you're really sick, but that might be a misleading thing to tell you. To paraphrase Lisa Salberg, having HCM is like being pregnant. Either you have it or you don't, you can't be a little bit pregnant. There are people with very large measurements who have relatively few symptoms and people with smaller measurements who have huge symptoms. A lot of that has to do with placement of the "bump" but there are electrical issues and other things to consider too.

Have you called the HCMA office yet? I'm sure they can do a much better job than I can of explaining what's probably going on with you and can direct you to a specialist who can explain your specifics to you and help you with medical management.

[Pam Alexson]

Normal cavity just refers to the fact that it has not gotten smaller from the hypertrophy. The volume of blood holding capacity is normal but other factors like stiffness can effect the filling phase of heart circulation and its capadity over time. The variable #'s could be that he is referring to your overall hypertrophy measurements. Mostly cardios refer to the septal measurements as those are where the issues are of greatest impact.

Using amyl nitrate is like valsalva or an athletic challenge for ones heart ( increasing the heart rate artificially). Yours obstructs showing you do have obstruction ...we just do not know how much ..he must have put that # somewhere on there.
Likewise your mitral leaflets impact your septum when your heart activity is challenged, interfering with the outflow of blood flow velocity as evidenced in the value 6.8 mm/sec. It is marked ( significant). Systolic anterior motion (SAM) of the mitral valve leaflet is significant. The anterior mitral leaflet is actually getting sucked into the aortic outflow tract and there is probably some mention of mitral valve regurgitation on your paper as well. IT is all normal at rest( good news) BUT not out of the woods:

"It is interesting that the severity of left ventricular hypertrophy and the resting left ventricular outflow tract gradient are not particularly predictive of the degree of symptoms experienced by patients. Since most patients have symptoms only with exertion, the resting gradient should not be used as assessment of efficacy of medical therapy. It is the exercise-induced consequences that are important to treat. Thus, if a patient becomes minimally symptomatic on therapy, then treatment failure should not be determined based upon only the resting gradient."
By Ommen and Nishimura in "Hypertrophic cardiomyopathy"

The 1.8 cm or 18 mm is above normal.. ( 10mm or 1.0cm - 12mm or 1.2cm or less = normal). Your biggest thickness is right below the outflow tract of the aorta. It is disproportionate (asymetrical). Even @ 1.8 when the ventricle contracts to expel the blood a ridge of papillary muscle can be scrunching up and offering obstruction to the outflow tract as yours is under that spot( where the blood exits) so it is not soley the mitral leaflets that may be interfering w/ outflow. You just need to get that #. That # will reflect the pressure value that increases in your heart when the blood TRIES*to exit. The blood does get out as evidenced by Ejection Fraction %. Usually an HCM heart is hyperdynamic (works very hard) to get the circulating blood out and we therefore often have higher then normal EF's. WE do not also know what he wrote for your EF.

Expert oversight and monitoring of this is best and an expert in HCM should always be consulted at some point and a great plan is in place.

If you contact the HCMA they can go over those values w/ you.

[mtlieb]

A left ventricular outlflow tract velocity of 6.8 m/sec translates to a gradient of roughly 185 mmHg.

That's: 6.8² x 4.

Jim

[Pam Alexson]

Thank you Jim! I just knew you would give those conversions.

AND..that gradient is quite significant!

[Pam Alexson]

Pathophysiology ( directly from this link:http://www.mayoclinic.org/hypertrophic-cardiomyopathy/physiciansguide.html )
The classic symptoms in hypertrophic cardiomyopathy are the triad of exertional dyspnea, angina, and syncope. While much attention is directed toward systolic function and events in hypertrophic cardiomyopathy, diastolic dysfunction is a major contributing cause of symptoms in all patients. Prolongation of ventricular relaxation and decreased ventricular compliance from the abnormal hypertrophied myocardium are present in every patient who presents with symptoms. Because this diastolic dysfunction impairs filling of the ventricle, circumstances that cause an increase in heart rate (exertion) result in decreased diastolic filling period and further compromise of left ventricular filling. This will increase left atrial pressure, and thereby cause shortness of breath. Increased left ventricular diastolic pressure also causes an oxygen supply/demand mismatch. Combined with the large muscle mass, which enhances the mismatch, symptoms of angina pectoris will arise even in the presence of normal coronary arteries (the contribution of myocardial "bridging" to these symptoms is unknown). Although these symptoms usually arise during exertion, there may be patients with such severe diastolic dysfunction that they are short of breath either at rest or with minimal exertion. Severe resting symptoms due to diastolic dysfunction are seen mainly in patients with an apical variant or end-stage dilated variant of hypertrophic cardiomyopathy.

About half of the patients will have a dynamic obstruction to left ventricular outflow tract. This is due to significant hypertrophy of the basal septum, which projects into the left ventricular outflow tract, and is accompanied by systolic anterior motion of the mitral valve. The systolic anterior motion of the mitral valve may result from suction and drag forces of the accelerating blood flow but may also be pushed into the outflow tract by a abnormal mitral valve apparatus. The obstruction can decrease effective cardiac output and result in syncope or near syncope with effort. The systolic anterior motion of the mitral valve also causes significant mitral regurgitation due to distortion of the mitral valve apparatus. In patients with severe symptoms of dyspnea accompanied by an outflow tract obstruction, the mitral regurgitation itself is often the primary cause of the dyspnea. Both the obstruction and the mitral regurgitation are dynamic: dependent upon the loading conditions and contractile force of the myocardium. The obstruction can cause increased left ventricular afterload, increased intercavitary pressure, and decreased coronary perfusion, all of which worsen the oxygen supply/demand mismatch. Thus, the obstruction itself will worse diastolic function and cause further myocardial ischemia. Relief of obstruction will improve forward cardiac output, decrease mitral regurgitation, enhance coronary blood flow, and improve diastolic function.

Syncope and even sudden death may occur in hypertrophic cardiomyopathy. Arrhythmias are common, with atrial arrhythmia occurring in anywhere from 35-50 percent of patients. Rapid tachycardia and loss of atrial contraction, in this disease with impaired ventricular filling may result in significant hemodynamic compromise. Ventricular arrhythmias also occur frequently, presumably due to the electrical instability cause from the myocardial disarray. It is felt that the primary cause of sudden death in many of these patients is ventricular arrhythmias, primarily ventricular tachycardia. However, atrial arrhythmias, bradycardias, and abnormal autonomic responses may also be involved in causing the sudden catastrophe events.

[Rainey]

Thanks. This is so much to take in. Last night was horrible. I FINALLY had an unstuffed nose when I went to bed after over a week of having the flu. So, I decided to go ahead and try to start wearing my sleep apnea mask (I only have a nasal mask so I HAVE to be able to breath out of my nose). At first I just couldn't get to sleep because I was so stressed about the day and all the new information. Then, after about an hour of just flopping around like a fish, I realized that the covers started feeling really heavy - especially on my right arm. So, I pulled it out and tried to fall asleep with that one arm out. Then, it just started getting worse and I started to notice a lot of flip flopping in my chest. So, I thought I'd better get an anxiety pill (haven't taken one in weeks). So, I unhooked my mask and realized that my nose was almost completely stuffed on one side and partially on the other but I couldn't tell because the air was forced. So, now I'm sure that I was oxygen poor last night for at least a little while. I'm usually SO good at dealing with stress but this is just too much. Then, to make matters worse, I have my four-year old son's echo on Friday. I'm going to take his doc all my numbers and see if she thinks I shouldn't get a second opinion. Wish me luck.

[gfox42]

Regarding Jim's unit conversions . . . I've wondered where this came from since the last time he mentioned this, and finally got around to checking. There's a lot of math involved, which I won't trouble others with, but one reference is an echocardiography book at http://books.google.com/books?id=E_S8RI1Hf5IC&pg=PA139&lpg=PA139&dq=%22simplified+bernoulli%22+velocity+echocardiog ram&source=web&ots=O17fHOHImm&sig=a91hkwgIWYcYWlD8KNrg4k9ZGUA#PPA60,M1

It turns out that this calculation (gradient = 4 X velocity^2) is a simplified version of an equation used to understand how fluids move through pipes. The simplifications come because we can ignore a lot of things like gravity (since it has a negligible effect over the elevation difference between your left ventricle and your aorta). There are several other simplifications; the bottom line is that of course this conversion is an approximation, but it's a pretty good one for hearts.

The factor 4 is the "mass density" of blood -- the mass per unit volume. Pressure is obviously related to this!

A more general way of putting the relationship Jim described is
pressure = mass density X velocity^2.
This should actually make some intuitive sense -- the pressure depends on the mass density and on the speed with which the blood is moving.

Since the mass density of blood is fixed at about 4, the pressure gradient varies only because of the velocity with which blood is leaving the ventricle. And that depends on how narrow the outflow tract is. As people have pointed out before, it's like putting your thumb over the end of a hose -- the water leaving the hose does so at a much greater velocity and under greater pressure than it would otherwise.

Gordon

[Pam Alexson]

Sorry things are a bit overwhelming presently...w/ regards to your mask , I also have a head cold w/ stuffiness and for me what works is a bit of vicks vapor rub right under the nose before I put my nose mask on. I have to prop up real high on firm pillows and stay on my back as my airway seems to collapse worse on either side. I try to hold the mask out a bit until the vicks opens up my breathing and the stuffiness subsides. It takes a little while to adjust and work.
Talk to your doctor about what you could try.
My pulmonary doctor has also recommended those breath right strips.. I have not tried them but he says they work for a lot of patients w/ the same nasal congestion and who must wear the c-pap or bi-pap.

I hope you get more answers on Friday... is your sons doc well versed in HCM?
See what is said and then YOU make that decision which is best for you. Many w/ HCM have gone a similar route and in the end a specialty eval is the best course of action for someone w/ this diverse disease could enter into. Wishing you lots of luck.

Pam

[gfox42]

Regarding Jim's unit conversions . . . I've wondered where this came from since the last time he mentioned this, and finally got around to checking. There's a lot of math involved, which I won't trouble others with, but one reference is an echocardiography book at http://books.google.com/books?id=E_S8RI1Hf5IC&pg=PA139&lpg=PA139&dq=%22simplified+bernoulli%22+velocity+echocardiog ram&source=web&ots=O17fHOHImm&sig=a91hkwgIWYcYWlD8KNrg4k9ZGUA#PPA60,M1

It turns out that this calculation (gradient = 4 X velocity^2) is a simplified version of an equation used to understand how fluids move through pipes. The simplifications come because we can ignore a lot of things like gravity (since it has a negligible effect over the elevation difference between your left ventricle and your aorta). There are several other simplifications; the bottom line is that of course this conversion is an approximation, but it's a pretty good one for hearts.

The factor 4 is the "mass density" of blood -- the mass per unit volume. Pressure is obviously related to this!

A more general way of putting the relationship Jim described is
pressure = mass density X velocity^2.
This should actually make some intuitive sense -- the pressure depends on the mass density and on the speed with which the blood is moving.

Since the mass density of blood is fixed at about 4, the pressure gradient varies only because of the velocity with which blood is leaving the ventricle. And that depends on how narrow the outflow tract is. As people have pointed out before, it's like putting your thumb over the end of a hose -- the water leaving the hose does so at a much greater velocity and under greater pressure than it would otherwise.

Gordon

[Rainey]

Thanks. I don't know how well-versed she is. She is part of the cardiac team at Nationwide Children's Hosptial here in Columbus, Ohio. It is one of the best pediatric hospitals. I have chatted with the cardiac team over there and there is one guy, who, if any of my kids are diagnosed, that we'll switch to. Right now we're going to see this lady doctor because of ease of location, really - she's has hours in a satelite office only minutes from my house and this way I feel like my kids won't be as taken aback by the big, scary hospital. Hoping we never have to step foot in there! Thanks for the advice on the mask, too. My symtoms were worse than ever after about an hour of having that mask on - I seriously slept on the couch with a phone near me in case I needed to call 9-1-1. Now that I think about it, I was doing pretty good until I rolled over onto one of my sides. Maybe I'll try the Vicks, propping up and sleeping on my back thing tonight. I figure a few hours wearing it are better than none! I'll also use my Neti Pot before going to bed. That might help, too.