Hypertrophic Cardiomyopathy, Part 1

Transcript

00:00 --> 00:06You're listening to the moon liners. The Yale
00:06 --> 00:11Internal Medison podcast, talking with
00:11 --> 00:13expert guests dropping
00:13 --> 00:18expert knowledge. This is your morning report
00:18 --> 00:20fix on the radio.
00:20 --> 00:23Your daily dose of internal Medison.
00:23 --> 00:26Welcome to the Moonlighters everybody.
00:26 --> 00:27Thanks for listening.
00:27 --> 00:31Today we have an excellent episode for you.
00:31 --> 00:34We have actually a special guest here.
00:34 --> 00:36One of our second year residents
00:36 --> 00:38at Yale Doctor Keith Love.
00:38 --> 00:41He has a special interest in
00:41 --> 00:43Cardiology and he's going to
00:43 --> 00:46kind of lead the episode today.
00:46 --> 00:48We also have our famous
00:48 --> 00:49cohost Gabriel Wilson,
00:49 --> 00:52as well as usual will be here too.
00:52 --> 00:55So thanks for coming, guys.
00:55 --> 00:56Yeah,
00:56 --> 00:57thanks so much for having us.
00:57 --> 00:59So today's topic, we're going to go
00:59 --> 01:00through Hypertrophic Cardiomyopathy
01:00 --> 01:03and we have a special guest with us.
01:03 --> 01:04Our very own doctor Daniel Jacobi of
01:04 --> 01:06the all New Haven Hospital doctor
01:06 --> 01:08Kobe received his MD here at Yale
01:08 --> 01:10before completing his residency over
01:10 --> 01:12at Mount Sinai in his fellowship in
01:12 --> 01:13Cardiology at Columbia Presbyterian.
01:13 --> 01:16He then found his way back to New Haven,
01:16 --> 01:17where he now specializes in the
01:17 --> 01:19diagnosis and treatment of heart
01:19 --> 01:20failure and cardiomyopathy.
01:20 --> 01:21He's the director of the comprehensive
01:21 --> 01:23heart failure program and is
01:23 --> 01:25the founder of the director of
01:25 --> 01:25the cardiomyopathy program.
01:25 --> 01:27He focuses on the diagnosis and
01:27 --> 01:28the treatment of hypertrophic
01:28 --> 01:29dilated arrhythmogenic,
01:29 --> 01:30an restrictive cardiomyopathy.
01:30 --> 01:32As well as the evaluation for
01:32 --> 01:33causes of sudden death,
01:33 --> 01:35and he also runs a pretty mean
01:35 --> 01:385K. He's got a lot going for now.
01:38 --> 01:39Thanks for the boost.
01:39 --> 01:41Build you up for the episode.
01:41 --> 01:42It's good, it's good.
01:42 --> 01:44I run a pretty good one, K.
01:44 --> 01:46Run half of them,
01:46 --> 01:48lock the other half but. All
01:48 --> 01:49right, so we're talking about
01:49 --> 01:50Hypertrophic Cardiomyopathy today.
01:50 --> 01:53And why is this an important topic?
01:53 --> 01:55So our focus will take us
01:55 --> 01:56through common presentations,
01:56 --> 01:58an manifestations of Hypertrophic
01:58 --> 02:00Cardiomyopathy will have a chance to
02:00 --> 02:02kind of tease apart the heterogeneous
02:02 --> 02:04ideologies of what is likely an
02:04 --> 02:05under recognized spectrum of disease,
02:05 --> 02:07and talk about how the underlying
02:07 --> 02:09pathophysiology should guide our
02:09 --> 02:10initial and chronic management of
02:10 --> 02:12the disease will also discuss how
02:12 --> 02:15to counsel our patients about life
02:15 --> 02:16with Hypertrophic Cardiomyopathy.
02:16 --> 02:18So let's get started with the case.
02:18 --> 02:21Great, so we're going to talk about Mr.
02:21 --> 02:23H Mr age 37 year old man with no past
02:23 --> 02:25medical history and the first comes
02:25 --> 02:27into his primary care providers office.
02:27 --> 02:29After passing out reports that one week ago
02:29 --> 02:31he's playing pickup basketball an experience,
02:31 --> 02:32sudden chest tightness, followed by
02:32 --> 02:34Lightheadedness and loss of consciousness.
02:34 --> 02:35He regained consciousness seconds later.
02:35 --> 02:37All his friends were standing around him.
02:37 --> 02:39He was asymptomatic at that point and
02:39 --> 02:41then he denied any symptoms there.
02:41 --> 02:43After he did said he felt normal
02:43 --> 02:44before the game felt normal.
02:44 --> 02:46After the game, he tells you this is
02:46 --> 02:49the first time he's ever passed out.
02:49 --> 02:51And he wonders if maybe he was just
02:51 --> 02:53dehydrated today in the office.
02:53 --> 02:55Again, he states that he's feeling
02:55 --> 02:56quite well, Sasha, Kobe.
02:56 --> 02:58So with this patient PCP,
02:58 --> 02:59the primary care physician,
02:59 --> 03:01what more history would you
03:01 --> 03:02want right off the
03:02 --> 03:03bat? Yeah, this is.
03:03 --> 03:05I mean, this is a scary episode.
03:05 --> 03:0737 year old healthy person
03:07 --> 03:08should not pass out.
03:08 --> 03:10I mean, in general I would guess
03:10 --> 03:12that no one sitting in this
03:12 --> 03:14room has passed out recently.
03:14 --> 03:16It's a rare event for someone to
03:16 --> 03:18pass out and for someone to pass out
03:18 --> 03:20while they're doing sports activity.
03:20 --> 03:22Is extremely rare and usually
03:22 --> 03:24is a big red flag for bad stuff,
03:24 --> 03:26so I'm already very worried
03:26 --> 03:27as the persons PCP.
03:27 --> 03:29First thing I'm saying to the guys.
03:29 --> 03:31Good job coming in. Well done.
03:31 --> 03:34I'm glad you didn't blow this off.
03:34 --> 03:36I would want to really try in a
03:36 --> 03:38little bit more into whether there was
03:38 --> 03:40any prodrome associated with this.
03:40 --> 03:42He mentions that he is a little
03:42 --> 03:43bit of Lightheadedness before
03:43 --> 03:45the loss of consciousness,
03:45 --> 03:46and I think that could potentially
03:46 --> 03:48be an important piece of information.
03:48 --> 03:51He mentions that he thinks he was dehydrated.
03:51 --> 03:53I'd like to dig in.
03:53 --> 03:55And find out exactly what he means by that.
03:55 --> 03:57Did he go out and was out till
03:57 --> 03:582:00 in the morning drinking
03:58 --> 04:00the night before you know?
04:00 --> 04:01Got up,
04:01 --> 04:03skip breakfast and went to play his game?
04:03 --> 04:04If that's the case,
04:04 --> 04:06maybe it is dehydration of.
04:06 --> 04:07I'd still be highly suspicious
04:07 --> 04:08and then I automatically jump
04:08 --> 04:10into whether there was any family
04:10 --> 04:11history of similar episodes,
04:11 --> 04:14because as a young person with no other
04:14 --> 04:16problems that would be of interest to me,
04:16 --> 04:17by the way,
04:17 --> 04:18also want to know this guy,
04:18 --> 04:19smoke, you know,
04:19 --> 04:21we would already know as he'll be,
04:21 --> 04:24so it's his BMI we want to kind of lay the.
04:24 --> 04:26Lay the groundwork of understanding
04:26 --> 04:28what are his cardiovascular risk
04:28 --> 04:30factors for Atheros sclerosis
04:30 --> 04:32because all things being equal,
04:32 --> 04:33even though he's young,
04:33 --> 04:35what's the most common cause
04:35 --> 04:37of cardiovascular disease?
04:37 --> 04:39It's Atheros sclerosis.
04:39 --> 04:41Sensor ties in a little bit with
04:41 --> 04:43what immediately hops up sword are
04:43 --> 04:44differential diagnosis and you
04:44 --> 04:46alluded to some scary things and
04:46 --> 04:48some common things at this point,
04:48 --> 04:50what would you say are your top
04:50 --> 04:52three things that you're thinking
04:52 --> 04:54for this guy and what do you
04:54 --> 04:56have so far to support those?
04:56 --> 04:58OK, so I once got made fun of
04:58 --> 05:00mercilessly as a resident just once,
05:00 --> 05:02because it was only happened to
05:02 --> 05:05me one time all the other times,
05:05 --> 05:06people will totally laudatory,
05:06 --> 05:08but this one totally unusual time
05:08 --> 05:10someone came up to me was, like,
05:10 --> 05:12you know, it has so many differential
05:12 --> 05:14diagnosis that it's hilarious.
05:14 --> 05:16It's so obvious that guy has a UTI.
05:16 --> 05:17Why do you have this long
05:17 --> 05:18differential diagnosis?
05:18 --> 05:19But I've always been someone to kind of
05:19 --> 05:21play out all the different possibilities,
05:21 --> 05:22because if you don't,
05:22 --> 05:24and this is kind of something that
05:24 --> 05:25I'm sure everybody's heard before.
05:25 --> 05:27But if you don't think about
05:27 --> 05:28it out of the gate,
05:28 --> 05:30you're probably not going to think about it.
05:30 --> 05:33It's whatever you put into the basket in the
05:33 --> 05:35beginning is what you pick out of the basket.
05:35 --> 05:37You pick something out of the basket at the
05:37 --> 05:40end so I would be a really broad with this.
05:40 --> 05:41I'd be thinking about
05:41 --> 05:42Cornering Atheros sclerosis.
05:42 --> 05:44I'd be thinking about all the billions of
05:44 --> 05:46different kinds of sudden cardiac death.
05:46 --> 05:47Associated syndromes I'd be thinking
05:47 --> 05:48about autonomic dysfunction.
05:48 --> 05:50Everything about drugs,
05:50 --> 05:51alcohol in Alpha listed substances,
05:51 --> 05:52behavior, lifestyle items.
05:52 --> 05:54Afraid to say you got to
05:54 --> 05:56think about malingering.
05:56 --> 05:57I mean,
05:57 --> 05:59I don't think about that all
05:59 --> 06:01the time with my patients,
06:01 --> 06:04but if you don't have it in the basket,
06:04 --> 06:05you know Munchausen's type
06:05 --> 06:06behavior and lingering.
06:06 --> 06:10You're never going to pick it up.
06:10 --> 06:12So there's a big broad differential
06:12 --> 06:14for this kind of thing at the very
06:14 --> 06:16top of it is Athero Sclerosis.
06:16 --> 06:17An inherited,
06:17 --> 06:19sudden death and coronary anomalies.
06:19 --> 06:21So when you see someone who's
06:21 --> 06:22Young was exercising the anomalous
06:22 --> 06:25coronary artery has to be one of
06:25 --> 06:26your differential diagnosis as well.
06:27 --> 06:29Yeah, so this is yeah,
06:29 --> 06:31this is pretty interesting case.
06:31 --> 06:33We created it so of course it's interesting,
06:33 --> 06:34but it's perfect.
06:34 --> 06:35Essentially syncope, you know,
06:35 --> 06:37and I always think syncope
06:37 --> 06:39is like a great case.
06:39 --> 06:40This white differential and wanted
06:40 --> 06:43to kind of get your opinion on
06:43 --> 06:44the pro drum like water keywords
06:44 --> 06:47that you hear where you're like.
06:47 --> 06:48Red flags are going off in
06:48 --> 06:50your like oh this is serious.
06:50 --> 06:52You know this isn't a vasovagal.
06:52 --> 06:54This is, you know something else.
06:54 --> 06:55Well, we
06:55 --> 06:57said one of them which is exercise
06:57 --> 06:59so people don't have vasovagal
06:59 --> 07:01episodes in the exercise. I mean,
07:01 --> 07:03it's very unlikely immediately post exercise.
07:03 --> 07:06You do see it. You can see someone,
07:06 --> 07:09but in the middle of exercise, not as much.
07:09 --> 07:12So exercise is a red flag.
07:12 --> 07:14This guy reports a little bit of
07:14 --> 07:16Lightheadedness and chest tightness,
07:16 --> 07:19which is a program that makes you think
07:19 --> 07:22that there could be a cardiac etiology.
07:22 --> 07:23The light headedness alone.
07:23 --> 07:25If you just have someone say
07:25 --> 07:27Well and is not rocket science,
07:27 --> 07:28we just have someone say,
07:28 --> 07:30well I sort of got lightheaded.
07:30 --> 07:32Everything started spinning and I went down.
07:32 --> 07:33That could be vasovagal,
07:33 --> 07:36but usually the prodrome is pretty extensive.
07:36 --> 07:37People feel not well,
07:37 --> 07:39they start to sweat, you know,
07:39 --> 07:40they realize that things
07:40 --> 07:41aren't going well frequently.
07:41 --> 07:43There's an opportunity to sit
07:43 --> 07:44down that they don't take.
07:44 --> 07:45That's common,
07:45 --> 07:48but I have had patients who have what in the
07:48 --> 07:50end turns out to be autonomic dysfunction,
07:50 --> 07:51vasovagal syncope,
07:51 --> 07:52nonagram, genic syncope.
07:52 --> 07:54That happens really, really fast,
07:54 --> 07:56so it's not a total rule out,
07:56 --> 07:59and this guy, the chest pressure,
07:59 --> 07:59the Lightheadedness,
07:59 --> 08:02and then what I interpret as probably
08:02 --> 08:04very rapid loss of consciousness is,
08:04 --> 08:05I think,
08:05 --> 08:07end during exercise are all red
08:07 --> 08:10flags for non vasovagal syncope for,
08:10 --> 08:12or what we would batch as
08:12 --> 08:14cardiovascular cardiac syncope.
08:14 --> 08:14Alright,
08:14 --> 08:16so on further discussion he reports
08:16 --> 08:18mild worsening of exercise tolerance
08:18 --> 08:19over the last year in discussing
08:19 --> 08:21his family history reports,
08:21 --> 08:23his father died in a drowning
08:23 --> 08:25accident and a paternal uncle died
08:25 --> 08:27suddenly of a heart attack at age 45.
08:27 --> 08:29Otherwise, he has two children
08:29 --> 08:30who are in good health.
08:30 --> 08:32So what is sudden cardiac death?
08:32 --> 08:35An how do you kind of confirm or refute
08:35 --> 08:38this? Well, this is very
08:38 --> 08:40interesting piece of information.
08:40 --> 08:43Do you ask your patients about drowning?
08:43 --> 08:48Not know. Unexplained deaths I always
08:48 --> 08:51ask my patients about this because when I
08:51 --> 08:53first started practicing in this field.
08:53 --> 08:55The guy who I was working with,
08:55 --> 08:57this Ganim Professor William McKenna,
08:57 --> 09:00who was a big huge figure in this field,
09:00 --> 09:03would see all these patients with me and when
09:03 --> 09:05patient would say the usual thing of yeah,
09:05 --> 09:06well my uncle died.
09:06 --> 09:09You know he would say well how did he die?
09:09 --> 09:12So we had a heart attack while
09:12 --> 09:12describing what happened.
09:12 --> 09:14Hardtack, well, he drowned.
09:14 --> 09:16The doctor said he had a heart
09:16 --> 09:17attack while he was swimming,
09:17 --> 09:19and then the following question
09:19 --> 09:21was he a good swimmer?
09:21 --> 09:22Where was he swimming?
09:22 --> 09:24Was swimming in the swimming pool?
09:24 --> 09:26Was he in huge waves at Misquamicut?
09:26 --> 09:26You know,
09:26 --> 09:29is he surfing 30 foot waves or was he
09:29 --> 09:32just like paddling around in a Lake?
09:32 --> 09:34Because also drowning for adults
09:34 --> 09:37who know how to swim who are not in
09:37 --> 09:39a bad situation is extremely rare
09:39 --> 09:41and you get pulled out by a riptide.
09:41 --> 09:43OK you're surfing whatever you're
09:43 --> 09:44trying to save somebody.
09:44 --> 09:44Yes,
09:44 --> 09:47you hear about those cases but for
09:47 --> 09:49kids sadly or for people wearing
09:49 --> 09:50driving alcohol it happens,
09:50 --> 09:52but for grownups who know how to swim
09:52 --> 09:55is a rare event to drown because you
09:55 --> 09:58kind of know your limits and you get.
09:58 --> 10:00You don't get into that situation,
10:00 --> 10:03so the fact that his father
10:03 --> 10:05died drowning is odd.
10:05 --> 10:07That jumps that jumps out immediately,
10:07 --> 10:09and there are certain sudden death syndrome
10:09 --> 10:10that are associated with swimming fact.
10:10 --> 10:12One of the long cuties I'm
10:12 --> 10:13going to get it wrong.
10:13 --> 10:14Which one?
10:14 --> 10:16I don't know if it's 1,
10:16 --> 10:18two or three is actually associated
10:18 --> 10:20with sudden death during swimming.
10:20 --> 10:21And so when you hear about
10:21 --> 10:23sudden death with with drowning,
10:23 --> 10:25it sets off a red flag and then the
10:25 --> 10:27other thing that happens is paternal
10:27 --> 10:29uncle died suddenly heart attack.
10:29 --> 10:31We get this all the time because people,
10:31 --> 10:32doctors, pathologist,
10:32 --> 10:34so we have to give an answer
10:34 --> 10:35to the struggling family.
10:35 --> 10:37And historically you either tell
10:37 --> 10:39the person it was a stroke you
10:39 --> 10:40tell him was a heart attack.
10:40 --> 10:42So I always have the follow-up
10:42 --> 10:44question what do you mean?
10:44 --> 10:45What happened? Were you there?
10:45 --> 10:47Do you know the details?
10:47 --> 10:49Did he grab his chest with you have
10:49 --> 10:50known atherosclerotic heart disease?
10:50 --> 10:52Do you have an autopsy?
10:52 --> 10:53Did he just die suddenly standing
10:53 --> 10:55at the bus stop in the Doc said,
10:55 --> 10:57well, this is, you know,
10:57 --> 10:58the heart attack because you don't
10:58 --> 10:59have any other explanation frequently.
10:59 --> 11:01Also what you see is not to get
11:01 --> 11:03too long winded about it, but.
11:03 --> 11:05There is a very strong belief,
11:05 --> 11:08probably by all of us in this room,
11:08 --> 11:10even though we don't really probably
11:10 --> 11:12obviously admit to it that somehow
11:12 --> 11:13illness is a moral failure and
11:13 --> 11:16we in the old days 2000 years ago
11:16 --> 11:17illness was totally a moral failure.
11:17 --> 11:19If you got leprosy,
11:19 --> 11:20it's 'cause you did something wrong.
11:20 --> 11:23Now we've kind of gotten away from that,
11:23 --> 11:24but not completely particularly
11:24 --> 11:25important vascular disease, right?
11:25 --> 11:27Are you a smoker You diabetic
11:27 --> 11:28or you fat the exercise?
11:28 --> 11:29You not exercise.
11:29 --> 11:31There's this perception that if you
11:31 --> 11:33do the right stuff, you shouldn't
11:33 --> 11:35get something bad could happen to you.
11:35 --> 11:36So very frequently,
11:36 --> 11:38family will say my uncle died,
11:38 --> 11:39but he was a drinker.
11:39 --> 11:41I didn't take care of himself,
11:41 --> 11:43but I take care of myself so
11:43 --> 11:45you get that in families too.
11:45 --> 11:48But you gotta sort of push that out to the
11:48 --> 11:50side because everybody needs an explanation.
11:50 --> 11:52So this history of two
11:52 --> 11:53people with sudden death,
11:53 --> 11:55presumably before age 50,
11:55 --> 11:57we didn't get the age of the dad,
11:57 --> 11:59but let's just say two people will send
11:59 --> 12:02at a relatively young age is already
12:02 --> 12:04jumping off the page as concerning.
12:04 --> 12:05What is Sunday's son?
12:05 --> 12:08Death is death within an hour of symptoms.
12:08 --> 12:10Technically for like for like a trial,
12:10 --> 12:12or you know that would be sudden
12:12 --> 12:14death from within an hour of symptoms.
12:14 --> 12:16But like you got your chest,
12:16 --> 12:18your chest pain dead within an hour,
12:18 --> 12:19that's pretty much like sudden death.
12:19 --> 12:22But when we think of sudden death?
12:22 --> 12:24We think of it as like immediate
12:24 --> 12:26like I'm talking to you right now.
12:26 --> 12:28Head goes down on the table, dead,
12:28 --> 12:30and that's what we see in the hospital too.
12:30 --> 12:32When patient codes you know
12:32 --> 12:33they're talking to you,
12:33 --> 12:35and then they're not talking to you.
12:35 --> 12:36So that sudden death.
12:36 --> 12:38It's really hard to confirm or refute it.
12:38 --> 12:39I've got an autopsy papers
12:39 --> 12:41from people a lot of times.
12:41 --> 12:43Families do keep autopsies from their
12:43 --> 12:45relatives, and you can get those sent to you.
12:45 --> 12:47So it just depends how hard
12:47 --> 12:48you want to dig in.
12:48 --> 12:50I would go full blast on this
12:50 --> 12:52guy because he's got two kids.
12:52 --> 12:52He's alive.
12:52 --> 12:55Either tried to diet or just had a vasovagal
12:55 --> 12:57'cause he drank too much in there before but.
12:57 --> 12:58You don't care about that.
12:58 --> 13:00If that happened, who cares?
13:00 --> 13:01You care that he,
13:01 --> 13:02you know someone who has
13:02 --> 13:03this happened to him,
13:03 --> 13:04is at risk of dropping dead.
13:04 --> 13:06So either and he's got two kids and
13:06 --> 13:08they're going to be potentially at risk.
13:08 --> 13:09If there's a genetic problem.
13:09 --> 13:10So you're going to really
13:10 --> 13:12dig into this family history.
13:12 --> 13:14I'll keep my next answer shorter.
13:14 --> 13:18I mean, I say from steam. Proof.
13:18 --> 13:21That was full of awesome information.
13:21 --> 13:24I feel like we can end the episode there.
13:24 --> 13:26No, I never thought it was.
13:26 --> 13:28My wife is calling on the other line.
13:29 --> 13:31Alright, so on physical exam his
13:31 --> 13:33PCP notices a systolic murmur along
13:33 --> 13:35the left sternal border and Apex,
13:35 --> 13:37which becomes louder with Valsalva maneuver.
13:37 --> 13:39The murmur does not radiate to the Carotids,
13:39 --> 13:41though there is a soft radiation
13:41 --> 13:43to the excella. So doctor to Kobe.
13:43 --> 13:46When you're approaching a patient like this,
13:46 --> 13:47do you think you could take
13:47 --> 13:49us through just in general?
13:49 --> 13:51I guess how you approach the physical exam
13:51 --> 13:54and then specifically if you hear a murmur,
13:54 --> 13:56how you practically go through these
13:56 --> 13:58different maneuvers to try and figure
13:58 --> 14:01out what the source of the murmur is.
14:01 --> 14:03Oh yeah, Well, you know when you tell your
14:03 --> 14:05patient that they have a heart murmur.
14:05 --> 14:07They get very worried.
14:07 --> 14:09Do you ever go through that you say Oh,
14:09 --> 14:12I hear a little more than what remember
14:12 --> 14:14what what is that so you know the
14:14 --> 14:16problem with murmurs are that we
14:16 --> 14:17now have echocardiography and the
14:17 --> 14:19stethoscope is 100 year old technology.
14:19 --> 14:22So I'm not trying to say that's not
14:22 --> 14:24important and I'm going to tell you
14:24 --> 14:27what I think about it but I just my
14:27 --> 14:29minute for like plugging point of care,
14:29 --> 14:30echo an advancing technology
14:30 --> 14:31of physical exam.
14:31 --> 14:33And I think you know the day I teach
14:33 --> 14:35cardiac physical exam to the PS.
14:35 --> 14:37I used to teach the medical students
14:37 --> 14:40for many years and so I'm I'm in favor
14:40 --> 14:41of knowing the cardiac physical exam.
14:41 --> 14:43But I think we have to recognize that
14:43 --> 14:45there are some significant limitations to
14:45 --> 14:48it that can be resolved with very simple,
14:48 --> 14:49readily available technology that
14:49 --> 14:51you can get in your iPhone right now.
14:51 --> 14:52So That being said,
14:52 --> 14:54there's the overall gestalt.
14:54 --> 14:56So you got to look at the patient
14:56 --> 14:57from head to toe.
14:57 --> 14:59How do the years lock hasnat Clock?
14:59 --> 15:01Is there a shock of white hair?
15:01 --> 15:03There are certain things that
15:03 --> 15:04can trigger you're thinking.
15:04 --> 15:06Oh, maybe this is a syndromic episode.
15:06 --> 15:08I'm always looking to see whether
15:08 --> 15:08there's underlying myopathy.
15:08 --> 15:11What's a muscle strength like as a
15:11 --> 15:13person able to get up from the Chair,
15:13 --> 15:13Easilly?
15:13 --> 15:14They have hypertrophied calves,
15:14 --> 15:16is there in muscular dystrophy
15:16 --> 15:17there that's been missed,
15:17 --> 15:19that they have normal grip strength?
15:19 --> 15:20They have neuropathy,
15:20 --> 15:21I don't have a needle to
15:21 --> 15:22prick for neuropathy,
15:22 --> 15:24but usually they will tell
15:24 --> 15:26you if they can feel stuff.
15:26 --> 15:28And then for the cardiac
15:28 --> 15:29specific physical exam,
15:29 --> 15:31the right way to do it is to really
15:31 --> 15:33have the patient lying down.
15:33 --> 15:35Listen to them when they're free,
15:35 --> 15:37breathing in all the usual places,
15:37 --> 15:39and then really take a careful
15:39 --> 15:40listen during expiration.
15:40 --> 15:41You're listening really hard for
15:41 --> 15:43any kind of murmurs over this
15:43 --> 15:44sort of aortic region,
15:44 --> 15:46because you want to know whether
15:46 --> 15:48there's any outflow obstruction.
15:48 --> 15:49I think because one of the
15:49 --> 15:50things that leads to syncope
15:50 --> 15:52is hypertrophic cardiomyopathy,
15:52 --> 15:54with left ventricular outflow obstruction,
15:54 --> 15:56and this is pretty typical murmur for that.
15:56 --> 15:58And it sounds a heck of a lot
15:58 --> 16:00like aortic stenosis.
16:00 --> 16:01The difference between this murmur in
16:01 --> 16:04aortic stenosis is that you can really
16:04 --> 16:05provoke an outflow obstruction murmur.
16:05 --> 16:07There can be almost no outflow
16:07 --> 16:09obstruction murmur when the person
16:09 --> 16:10is just lying comfortably free,
16:10 --> 16:11breathing at rest.
16:11 --> 16:14But if you set them up and have
16:14 --> 16:16them do a big chess valsalva,
16:16 --> 16:17you can almost always hear a murmur
16:17 --> 16:19come up immediately after they've
16:19 --> 16:21released their breath and start
16:21 --> 16:22breathing again with the Valsalva.
16:22 --> 16:25An if you hear that kind of provokes murmur,
16:25 --> 16:27it's really a hallmark of hypertrophic
16:27 --> 16:28obstructive cardiomyopathy.
16:28 --> 16:30And that can lead to syncope.
16:30 --> 16:32My drug rotation doesn't lead to syncope.
16:32 --> 16:33Aortic stenosis, of course.
16:33 --> 16:35We all know can lead to
16:35 --> 16:37syncope, but it shouldn't be provoke Obel
16:37 --> 16:39in that particular way with Valsalva.
16:39 --> 16:42In fact, if you sort of reduce
16:42 --> 16:42the ventricular filling,
16:42 --> 16:45the aortic murmur can go down without Salve,
16:45 --> 16:47as opposed to up with obstructive
16:47 --> 16:48hypertrophic cardiomyopathy.
16:48 --> 16:49So you can go through
16:49 --> 16:50these various maneuvers.
16:50 --> 16:52SWAT stand Valsalva and see whether
16:52 --> 16:54you can provoke any kind of
16:54 --> 16:55member that would make you think
16:55 --> 16:57this left ventricular outflow.
16:57 --> 16:59Tract obstruction radiation to the Carotids.
16:59 --> 17:01Is any member that occurs near
17:01 --> 17:02at the aortic valve,
17:02 --> 17:03I would assume would be something will
17:03 --> 17:05be hard to differentiate by radiation.
17:05 --> 17:07The crowd is and I'll be.
17:07 --> 17:08Honestly, I don't use that as a
17:08 --> 17:10significant factor in my physical exam,
17:10 --> 17:12although historically I guess there
17:12 --> 17:14are certain things you can do and
17:14 --> 17:15then I don't know if you can see
17:15 --> 17:17this part of the physical example.
17:17 --> 17:19You should always do ortho statics.
17:19 --> 17:22Which is a real pain, but you got it.
17:22 --> 17:24You can't build for that actually.
17:24 --> 17:26Order it in the hospital
17:26 --> 17:28and then never done it.
17:28 --> 17:30After 2 liters of fluids you can start.
17:30 --> 17:33You have to do it yourself exactly when
17:33 --> 17:35we talk about this murmur that gets
17:35 --> 17:37invoked by the Valsalva and you.
17:37 --> 17:39Talk about doing this sort of
17:39 --> 17:41sit forward big chest valsalva.
17:41 --> 17:42What do you actually hearing
17:42 --> 17:44there with the outflow tract that
17:44 --> 17:47is coming up and more? Are you
17:47 --> 17:48hearing that well?
17:48 --> 17:49You've taken airplane flights before
17:49 --> 17:52and the plane lifts off the runway
17:52 --> 17:54for the same reason that you hear.
17:54 --> 17:56A murmur during Valsalva with
17:56 --> 17:57Hypertrophic Cardiomyopathy.
17:57 --> 17:58Because there's something
17:58 --> 17:59called the Bernoulli effect,
17:59 --> 18:02an when a fluid or gas has to
18:02 --> 18:04travel more rapidly next to
18:04 --> 18:06one that's traveling slowly,
18:06 --> 18:09the pressure is decreased in the area
18:09 --> 18:12where the broad is traveling more rapidly.
18:12 --> 18:14So what happens with Hypertrophic
18:14 --> 18:16cardiomyopathy is that the anterior
18:16 --> 18:18mitral leaflet or leaflet apparatus is
18:18 --> 18:20relatively closely approximating the
18:20 --> 18:21hypertrophied interventricular septum
18:21 --> 18:24an as the hard squeezes the plug out.
18:24 --> 18:26The blood has to accelerate.
18:26 --> 18:28Around this convex hypertrophic
18:28 --> 18:30overgrown piece of muscle that's
18:30 --> 18:33close to the mitral valve and as the
18:33 --> 18:35blood gets through there it decreases
18:35 --> 18:37the pressure in that area and sucks
18:37 --> 18:40the mitral leaflet apparatus over
18:40 --> 18:42to touch the interventricular septum
18:42 --> 18:44and just like rocks in a stream
18:44 --> 18:46that caused the water to gurgle,
18:46 --> 18:49if you distort the Lamb and or blood
18:49 --> 18:51flow you hear a murmur and so you're
18:51 --> 18:53actually hearing the blood squirting
18:53 --> 18:56passed in this turbulent fashion.
18:56 --> 18:58This obstruction between the mitral valve.
18:58 --> 19:00And the interventricular septum.
19:00 --> 19:03And we refer to that as Sam,
19:03 --> 19:04Systolic anterior motion of
19:04 --> 19:05the mitral leaflet.
19:05 --> 19:07There's many factors that
19:07 --> 19:09kind of feed into having that,
19:09 --> 19:12but with Valsalva one of the things
19:12 --> 19:15that you see that happens is that you
19:15 --> 19:18temporarily under fill your left ventricle,
19:18 --> 19:20and so there's less preload,
19:20 --> 19:22and so you get this increased
19:22 --> 19:24approximation of the hypertrophic
19:24 --> 19:25segment with mitral leaflets.
19:25 --> 19:27And so you can actually
19:27 --> 19:29provoke more bernoulli effect.
19:29 --> 19:31Or make them do in fact more affected
19:31 --> 19:33by bringing those two things closer
19:33 --> 19:35together by having less filling
19:35 --> 19:36of the left ventricle. And can
19:36 --> 19:39we use that to explain why we're
19:39 --> 19:40seeing syncope in that patient
19:40 --> 19:42population is a huge factor?
19:42 --> 19:44I keep them. Glad you asked that,
19:44 --> 19:45because there are many
19:45 --> 19:46patients walking around today.
19:46 --> 19:48Have defibrillators because they
19:48 --> 19:49had hemodynamic syncope with
19:49 --> 19:50hypertrophic cardiomyopathy for sure,
19:50 --> 19:53because you can have syncope just from that
19:53 --> 19:55phenomenon and we don't know in this patient.
19:55 --> 19:57Now again, we have all this other
19:57 --> 19:59stuff is dad died, his uncle died.
19:59 --> 20:01He died very young.
20:01 --> 20:02He passed out very quickly,
20:02 --> 20:05so if this turns out to be the diagnosis.
20:05 --> 20:06You know we would start to be
20:06 --> 20:08very suspicious of arrhythmia,
20:08 --> 20:09but People do pass out.
20:09 --> 20:11I have patients who pass out all
20:11 --> 20:13the time from this or get to near
20:13 --> 20:15syncope from this and then they
20:15 --> 20:17would get in my activity or not call
20:17 --> 20:18blasian and they would feel better
20:18 --> 20:19and they would stop happening.
20:19 --> 20:21One of the things that you note
20:21 --> 20:22about that though is there's
20:22 --> 20:24almost always a lead in a program
20:24 --> 20:25and it's almost always associated
20:25 --> 20:27postural change or activity.
20:27 --> 20:28So the classic is Doc.
20:28 --> 20:29I drove my car to work,
20:29 --> 20:31there's a very it's an hour drive.
20:31 --> 20:33Is it very slight Hill up to the
20:33 --> 20:35stairs that I have to take up to
20:35 --> 20:37the front door when I get to the
20:37 --> 20:39top of the stairs of the front
20:39 --> 20:41door I always feel like I'm about
20:41 --> 20:43to pass out and one time I did.
20:43 --> 20:45Or I was I had a glass of
20:45 --> 20:45wine Thanksgiving dinner.
20:45 --> 20:47I sat and watched the football
20:47 --> 20:49game for two hours and then I
20:49 --> 20:50had to go to the bathroom.
20:50 --> 20:52So I got up off the couch and boom,
20:52 --> 20:53'cause you're always dilated.
20:53 --> 20:54Glass of wine, big meal,
20:54 --> 20:56but it's in your stomach to watch a
20:56 --> 20:58football game and boom, you go down.
20:58 --> 21:00So there are some environmental factors
21:00 --> 21:02that can kind of tell you what it is.
21:02 --> 21:03Uh.
21:03 --> 21:05OK, excellent,
21:05 --> 21:07so enough of that stethoscope nonsense,
21:07 --> 21:09and we're going to complete garbage,
21:09 --> 21:11not rubbish. It's a good decoration.
21:11 --> 21:14Patients like it, but so we get
21:14 --> 21:17an EKG on this guy in the office.
21:17 --> 21:19It reveals increased record-eagle voltage
21:19 --> 21:20with left ventricular hypertrophy,
21:20 --> 21:23an prominent Q Waves in the inferior, and the
21:23 --> 21:26lateral leads. We also get a little
21:26 --> 21:28blood work right off the bat.
21:28 --> 21:30It's notable for an elevated BNP two
21:30 --> 21:32800 and his primary care provider
21:32 --> 21:35then orders a transthoracic Echo.
21:35 --> 21:36Anne refers to your office.
21:36 --> 21:39So can we just unpack this a little bit
21:39 --> 21:41will start off with the ECG an again,
21:41 --> 21:43to reiterate that we're seeing LVMH
21:43 --> 21:44or left ventricular hypertrophy
21:44 --> 21:46prominent Q Waves in the inferior
21:46 --> 21:48and lateral leads and then some
21:48 --> 21:48increase recorded voltage.
21:49 --> 21:51Yeah, Well you spelled it out.
21:51 --> 21:53I mean this is this is looking a lot
21:53 --> 21:55like Hypertrophic Cardiomyopathy.
21:55 --> 21:57You have to be a little bit cautious
21:57 --> 21:58'cause the guys in athlete.
21:58 --> 22:00I'm assuming he played basketball.
22:00 --> 22:02The relatively young guy, so you know,
22:02 --> 22:04if the guys on the basketball court 3
22:04 --> 22:06four days a week if he's exercising,
22:06 --> 22:09he can have increased voltage. the Q waves.
22:09 --> 22:11You said there in the inferior leads. Yeah.
22:11 --> 22:13Q Waves in fear leads are suspicious.
22:13 --> 22:14Not they're not.
22:14 --> 22:15Diagnostic could be.
22:15 --> 22:17I don't know how how tall the guy is
22:17 --> 22:19could be something about his body.
22:19 --> 22:20Habitus could be.
22:20 --> 22:22Doing that so LVH some QA is
22:22 --> 22:24in fairly I'm suspicious,
22:24 --> 22:26but I'm not sure if you had told
22:26 --> 22:29me he's also got significant T wave
22:29 --> 22:31inversion across the anterior precordium.
22:31 --> 22:33Now I'm really worried about it,
22:33 --> 22:34or even out laterally.
22:34 --> 22:36Then I really think hypertrophic
22:36 --> 22:38cardiomyopathy is coming big into
22:38 --> 22:39the differential diagnosis here,
22:39 --> 22:41but this could be a hypertrophic
22:41 --> 22:42cardiomyopathy, EKG,
22:42 --> 22:44and then you said the BMP is
22:44 --> 22:45elevated elevated 800 OK,
22:45 --> 22:47so that's really really interesting fact.
22:47 --> 22:48So I mean,
22:48 --> 22:50we always associate elevated
22:50 --> 22:51BNP with heart failure,
22:51 --> 22:52reduced ejection fraction.
22:52 --> 22:54Sometimes our fair preserved
22:54 --> 22:55ejection fraction,
22:55 --> 22:57but we you know it's not usually associated
22:57 --> 22:59with hypertrophic cardiomyopathy.
22:59 --> 23:02I would guess that most people walking
23:02 --> 23:04around don't necessarily think about BMP,
23:04 --> 23:06asmark, fireproof, according me,
23:06 --> 23:08but it is an we are.
23:08 --> 23:10This is actually public data,
23:10 --> 23:12so we presented at European
23:12 --> 23:14side of Cardiology in August.
23:14 --> 23:17The I think it was 36 week data from
23:17 --> 23:20the pioneer study which was a phase
23:20 --> 23:23two study of an ATP myosin modulator.
23:23 --> 23:25That really is a negative on it.
23:25 --> 23:26Rope used for obstructive
23:26 --> 23:27hypertrophic cardiomyopathy.
23:27 --> 23:28We had 21 patients.
23:28 --> 23:29The initial study,
23:29 --> 23:31one who dropped out of the
23:31 --> 23:33initial study because of A-fib.
23:33 --> 23:35Then I think it was six or Seven
23:35 --> 23:37patients ended up going for surgical
23:37 --> 23:39myectomy in between the end of that
23:39 --> 23:41study and the initiation of the
23:41 --> 23:43long-term access to the drug study.
23:43 --> 23:45So there were 13 patients who
23:45 --> 23:46continued on in this study.
23:46 --> 23:49So the 13 pages to continue for
23:49 --> 23:5136 months there BMP started an
23:51 --> 23:52average of around 1500.
23:52 --> 23:55These are patients who had class two
23:55 --> 23:58or three hard failure type symptoms.
23:58 --> 24:00Some limitation from some limitation to
24:00 --> 24:02a lot of limitation with obstructive
24:02 --> 24:03hypertrophic cardiomyopathy.
24:03 --> 24:05Normal hyperdynamic ventricles
24:05 --> 24:07did not have volume overload,
24:07 --> 24:08just obstructive hypertrophic
24:08 --> 24:10cardiomyopathy and their BNP levels.
24:10 --> 24:11Basically normalized on the
24:11 --> 24:13therapy that also reduced their
24:13 --> 24:15obstruction to basically normal
24:15 --> 24:17without dropping the F substantially.
24:17 --> 24:20So my point in telling that
24:20 --> 24:22long story is to say that BMP.
24:22 --> 24:24Looks at this point like a reasonable
24:24 --> 24:26marker of symptomatic obstruction
24:26 --> 24:27and Hypertrophic Cardiomyopathy,
24:27 --> 24:29so I don't know what to say
24:29 --> 24:30exactly about the BNP.
24:30 --> 24:32The BNP at this point that you
24:32 --> 24:34going to be NPR nonspecific.
24:34 --> 24:36The guys gotta murmur.
24:36 --> 24:37I don't really know.
24:37 --> 24:39I don't trust my physical exam enough
24:39 --> 24:42to say conclusively whether this is ASM are.
24:42 --> 24:44I don't know whether he had
24:44 --> 24:46the Valsalva response or not at
24:46 --> 24:47this point in my differential,
24:47 --> 24:48diagnosis is still dilated.
24:48 --> 24:50Cardiomyopathy with with Mia versus
24:50 --> 24:51hypertrophic cardiomyopathy.
24:51 --> 24:53I don't think at this point he
24:53 --> 24:55has a primary rhythmic disease.
24:55 --> 24:57Or like I don't think that was a
24:57 --> 24:59seizure or something like that.
24:59 --> 25:01Like I think you know,
25:01 --> 25:03I think we're dealing with the structural
25:03 --> 25:05heart disease here at this point,
25:05 --> 25:07and probably I think top of the
25:07 --> 25:08list is hypertrophic cardiomyopathy
25:08 --> 25:10and after that some
25:10 --> 25:11other version of Nonischemic Cardiomyopathy.
25:12 --> 25:13Quick question that came to
25:13 --> 25:14mind after discussing that EKG,
25:14 --> 25:16so you talked a little bit about how
25:16 --> 25:19the key waves can point you in the
25:19 --> 25:20direction of Hypertrophic Cardiomyopathy.
25:20 --> 25:22Though not definitely specific for that,
25:22 --> 25:24whi can you explain why you would get
25:24 --> 25:27Q Waves on EKG and a patient like this?
25:27 --> 25:29Is it because there's fibrosis in
25:29 --> 25:30the area of hypertrophied ventricle,
25:30 --> 25:32or 'cause I CQ waves and I think
25:32 --> 25:34of old infarct, that's you know.
25:34 --> 25:35I've trained myself.
25:35 --> 25:37So what makes you think that this
25:37 --> 25:38could potentially point towards
25:38 --> 25:39Hypertrophic cardiomyopathy?
25:39 --> 25:39Well,
25:39 --> 25:41you raise a good point and I
25:41 --> 25:43didn't say that, but obviously.
25:43 --> 25:45Ischemic heart disease is still
25:45 --> 25:47in the differential diagnosis as
25:47 --> 25:49our as is now most coronary artery
25:49 --> 25:52and there is no way to be specific
25:52 --> 25:54about that with regards to the EKG,
25:54 --> 25:56I don't know physiologically why patients
25:56 --> 25:58with hypertrophic cardiomyopathy get Q waves,
25:58 --> 26:00and specifically needs, and some don't.
26:00 --> 26:02There are some patients with
26:02 --> 26:04recording app that have normal EKG's.
26:04 --> 26:05It's not, though,
26:05 --> 26:07for the same reason that patients
26:07 --> 26:09for the schema cardiomyopathy get it
26:09 --> 26:12because in those patients the QA can
26:12 --> 26:14actually tell you where the infarct was.
26:14 --> 26:15In this case,
26:15 --> 26:17if you do an MRI in patient with
26:17 --> 26:19hypertrophic cardiomyopathy with Q waves,
26:19 --> 26:21you're not going to see transmural
26:21 --> 26:23fibrosis in the inferior wall
26:23 --> 26:24because of those Q waves.
26:24 --> 26:26You may see fibrosis over 70%
26:26 --> 26:27of patients with hypertrophic
26:27 --> 26:29cardiomyopathy have fibrosis apparent.
26:29 --> 26:31an MRI when you do the MRI,
26:31 --> 26:33but it's in all kinds of
26:33 --> 26:33different distributions.
26:33 --> 26:36Most of it is not very, very severe,
26:36 --> 26:38and it's rare to see transmural.
26:38 --> 26:41You can see it, but it's much more rare,
26:41 --> 26:43and it doesn't distribute according to
26:43 --> 26:44the EKG. OK, that was
26:44 --> 26:46kind of my question.
26:46 --> 26:48Me if you see dagger Q waves,
26:48 --> 26:50it's hypertrophic cardiomyopathy.
26:50 --> 26:52Is that just like a Association
26:52 --> 26:54that is like a knee jerk reaction?
26:54 --> 26:57Or is that something that holds
26:57 --> 26:59weight send that person to me?
26:59 --> 27:01I don't know. I've never heard of
27:01 --> 27:02that before.
27:02 --> 27:04The dagger, the word Daggeron,
27:04 --> 27:05hypertrophic cardiomyopathy,
27:05 --> 27:06go together on echo.
27:06 --> 27:09OK, so you see, these dagger shaped.
27:09 --> 27:11Continuous wave Doppler tracings going
27:11 --> 27:13through the left ventricular outflow
27:13 --> 27:15tract because as sisterly progress is
27:15 --> 27:16the obstruction becomes more severe,
27:16 --> 27:19so the so instead of being a nice
27:19 --> 27:21curves review, shape parabola like you
27:21 --> 27:23get with them are where it goes up,
27:23 --> 27:25and then it goes down.
27:25 --> 27:27This one gets worse and worse and worse.
27:27 --> 27:30So it kind of cuts out like a
27:30 --> 27:32dagger on one side it's inverse,
27:32 --> 27:34and then it goes straight down.
27:34 --> 27:35So that's the dagger that
27:35 --> 27:37I know about in HCM,
27:37 --> 27:38but you know,
27:38 --> 27:39I'm open to new
27:39 --> 27:42information. I might be getting that
27:42 --> 27:47wrong and mix the old echo with the kids.
27:47 --> 27:50Yeah, yeah. So here we are.
27:50 --> 27:52We got this case and you know it sounds
27:52 --> 27:55a lot like hypertrophic cardiomyopathy.
27:55 --> 27:57I'm not a steam diagnostician,
27:57 --> 27:59but does sound like that,
27:59 --> 28:02and that is the title of the episode, but.
28:02 --> 28:04So doctor Kobe suspend your disbelief
28:04 --> 28:06so you know they come to you.
28:06 --> 28:08They probably already have an echo
28:08 --> 28:10before they get referred to you,
28:10 --> 28:11but I'm sure you're going
28:11 --> 28:13to be looking at this.
28:13 --> 28:14Echo yourself and like.
28:14 --> 28:16What specifically like you're
28:16 --> 28:17sitting down at the computer?
28:17 --> 28:19What are you looking for
28:19 --> 28:20and what is important to
28:20 --> 28:22you is the key thing,
28:22 --> 28:23because the diagnosis of
28:23 --> 28:24hypertrophic cardiomyopathy,
28:24 --> 28:25it's an image in diagnosis.
28:25 --> 28:28So whether you look at echo or MRI,
28:28 --> 28:30you need a picture of the heart to make
28:30 --> 28:32diagnosis of Hypertrophic Cardiomyopathy.
28:32 --> 28:34If you really want to be sure,
28:34 --> 28:36because the diagnostic criteria are
28:36 --> 28:38dependent upon finding greater than 15
28:38 --> 28:40millimeter wall thickness in at least
28:40 --> 28:41one segment in the left ventricle,
28:41 --> 28:43in the absence of abnormal afterloader,
28:43 --> 28:45other stimulus for hypertrophy.
28:45 --> 28:47So finding 50 millimeter wall segment
28:47 --> 28:49and some will severe IIS not good
28:49 --> 28:51enough can give the diagnosis.
28:51 --> 28:53Persons blood pressure is 180 over 110
28:53 --> 28:55every time they come to the office.
28:55 --> 28:58Not good enough, can't give the diagnosis,
28:58 --> 29:00but person comes in even mild hypertension.
29:00 --> 29:01Even mild aortic stenosis.
29:01 --> 29:03No real good reason to have
29:03 --> 29:04severe hypertrophy.
29:04 --> 29:06You find 50 millimeters of wall
29:06 --> 29:08thickness in the left ventricle.
29:08 --> 29:11You have pretty good case for diagnosis.
29:11 --> 29:13So that's the first thing I'm looking for.
29:13 --> 29:16The other thing that I'm looking for
29:16 --> 29:18is anything in terms of making the
29:18 --> 29:20diagnosis that's going to be what
29:20 --> 29:22I would call a fino copy of this,
29:22 --> 29:24so there's lots of things that
29:24 --> 29:26can cause hypertrophy that aren't
29:26 --> 29:27dependent on after load abnormality
29:27 --> 29:29that can also employed.
29:29 --> 29:29For instance,
29:29 --> 29:31can cause severe hypertrophy
29:31 --> 29:32with no elevated after load,
29:32 --> 29:35and they get wall thickness.
29:35 --> 29:36You know greater than 15 and then
29:36 --> 29:39there are some other sort of fino
29:39 --> 29:40copies of Hypertrophic Cardiomyopathy.
29:40 --> 29:43Some people call them subtypes.
29:43 --> 29:43Mitochondrial disease,
29:43 --> 29:44syndromic diseases,
29:44 --> 29:46infiltrative diseases like glycogen
29:46 --> 29:48storage diseases that can cause
29:48 --> 29:49hypertrophic cardiomyopathy
29:49 --> 29:50like Physiology and imaging,
29:50 --> 29:53but without being sort of the
29:53 --> 29:54classic hypertrophic cardiomyopathy.
29:54 --> 29:57So I'm looking for all that stuff.
29:57 --> 30:00One of the big clues is if you
30:00 --> 30:02see increased wall thickness
30:02 --> 30:04but decreased wall motion,
30:04 --> 30:06it's probably not straightforward
30:06 --> 30:07hypertrophic cardiomyopathy and I've
30:07 --> 30:10seen that mistake made before someone
30:10 --> 30:12coming in with acute Sarcoid Oasys.
30:12 --> 30:14Leading to inflammation.
30:14 --> 30:15Severe hypertrophy,
30:15 --> 30:17but with focal wall motion
30:17 --> 30:19abnormality in that area.
30:19 --> 30:21Given the diagnosis of Hypertrophic
30:21 --> 30:23cardiomyopathy treated for as
30:23 --> 30:25if hypertrophic cardiomyopathy,
30:25 --> 30:28treated as if burnt out.
30:28 --> 30:29Hypertrophic cardiomyopathy later
30:29 --> 30:31with poorly functioning ventricle
30:31 --> 30:34sent for heart transplantation.
30:34 --> 30:35Diagnosis post transplant
30:35 --> 30:38circling so that happens.
30:38 --> 30:39But that's to say,
30:39 --> 30:41like you can't just put your
30:41 --> 30:42Blinders on and see thickness,
30:42 --> 30:44and I get patients with fabreeze.
30:44 --> 30:47Disease is one in about 1 in 500 patients
30:47 --> 30:48referred by bridge overcrowding.
30:48 --> 30:50Map of fabreeze disease
30:50 --> 30:51amyloid much more common.
30:51 --> 30:53You see it all the time people
30:53 --> 30:54referred for HTM actually totally
30:54 --> 30:56different treatment by the way, right?
30:56 --> 30:57We know that.
30:57 --> 31:00And then you're looking at the HL findings.
31:00 --> 31:01They'll vote gradient mitral valve,
31:01 --> 31:02the right ventricle.
31:02 --> 31:03Whether there's an infusion,
31:03 --> 31:05all the usual echo stuff.
31:06 --> 31:09That's why you keep your differential broad.
31:09 --> 31:12Yeah, could be sarcoid on pathology.
31:12 --> 31:15No one wants to look like you to do it.
31:15 --> 31:18Yeah wow, that is crazy. Yeah yeah, if
31:18 --> 31:20you like so. I live on the Safari
31:20 --> 31:23in Africa like that's where,
31:23 --> 31:24so it's easy for Maine.
31:24 --> 31:26I cursed myself out this space
31:26 --> 31:29of practice where I I'm looking
31:29 --> 31:31for the zebra all the time.
31:31 --> 31:32'cause That's my comfort zone.
31:32 --> 31:35That's why I got made fun of in
31:35 --> 31:36residency that one time.
31:36 --> 31:39I feel like you know, like doctors
31:39 --> 31:41have this muscle in their skill is like
31:41 --> 31:42when something doesn't make sense.
31:42 --> 31:45You need to have that alarm that go
31:45 --> 31:47off and be like I gotta stop and I
31:47 --> 31:50gotta look at this a little bit harder.
31:50 --> 31:51Yeah, what's the environment that
31:51 --> 31:53allows you to do that? We don't
31:53 --> 31:55really have an environment like that. You
31:55 --> 31:57have to carve yourself out that environment.
31:57 --> 31:59I mean, that's one of the things that
31:59 --> 32:02you need to do as an individual.
32:02 --> 32:03I think that's the key.
32:03 --> 32:05If all the drivers in your practice
32:05 --> 32:07environment are telling you, don't stop.
32:07 --> 32:08Don't listen to that voice,
32:08 --> 32:10then you're eventually going to Cave.
32:10 --> 32:11Most people are going to Cave 'cause
32:11 --> 32:13it's really hard, but if the drivers
32:13 --> 32:15in your environment are telling you.
32:15 --> 32:16Bonus, you found it,
32:16 --> 32:18you didn't let that patient go by,
32:18 --> 32:20you did the right thing.
32:20 --> 32:21Then you will do that.
32:21 --> 32:23I think that yeah we do that.
32:23 --> 32:24I think we create environment
32:24 --> 32:26where we do tell people hey,
32:26 --> 32:27great job.
32:27 --> 32:28You didn't make the wrong diagnosis,
32:28 --> 32:30both in training and in practice,
32:30 --> 32:32but you gotta watch out in modern
32:32 --> 32:33Medison you can plug yourself into
32:33 --> 32:35an environment where you might end
32:35 --> 32:37up feeling like that's a tough thing
32:37 --> 32:39to do. So
32:39 --> 32:41we're going to actually take a pause here.
32:41 --> 32:44Will break this episode into two parts,
32:44 --> 32:45and so will stop here.
32:45 --> 32:47In next time we'll be back with
32:47 --> 32:49Doctor Jacobi to focus on management
32:49 --> 32:51accounts like these patients. Thanks
32:51 --> 32:53for listening to the moon liners.
32:53 --> 32:55We'll see you next time.

Information

Prepare to hypertrophy your knowledge of cardiomyopathy! This week The Moonlighters sit down with Dr. Daniel Jacoby, the director of The Comprehensive Heart Failure Program and the Cardiomyopathy Program at Yale-New Haven Hospital. We discuss the presentation, workup, and management for patients with Hypertrophic Obstructive Cardiomyopathy.

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