Restrictive Cardiomyopathy: Pathophysiology and Manifestations (Nursing)

00:01 Hi, in this part of our video series, we're going to talk about the most uncommon form of cardiomyopathy.

00:08 And it's called restrictive cardiomyopathy.

00:11 Remember whether it's any one of the three types, whether it's dilated, hypertrophic or restrictive, that's going to be a cue to you as to what's going on with this patient's heart.

00:21 So we start again, just like we have in the other two discussions with a picture of the heart and what it looks like.

00:28 We're going to slice it right down the middle, like we have before.

00:32 Kind of open that heart up to show you the impact on the left side of the heart.

00:37 So here, we have a normal heart, right? It has appropriate contractility.

00:42 And you know, in order for the heart to contract, that muscle needs to be strong and flexible.

00:48 In restrictive cardiomyopathy, you can see how that could be problematic.

00:53 In restrictive cardiomyopathy, like the artists put that for you.

00:57 Looks like a little brick wall.

00:59 That's a pretty good visual picture for you to understand what's going on, because that heart wall has what they call decreased compliance of the ventricles, meaning, maybe like trying to compress a brick wall.

01:12 It's really, really stiff.

01:14 So, the heart muscle is not dilated, like we've seen in other forms of cardiomyopathy.

01:19 But that wall is just not going to be compliant or flexible, and able to contract down well.

01:27 So, it's called diastolic dysfunction. Why is that? Well, remember, systole diastole.

01:35 All of a sudden, it can't really move.

01:37 You've got this weird filling of the ventricle.

01:41 Systole, diastole. When it relaxes, that's when it fills.

01:46 So, in restrictive cardiomyopathy, you have this impaired or decreased filling of the ventricles because of the damage to the heart wall.

01:56 Let's look at the epidemiology of restrictive cardiomyopathy.

02:00 Now, remember, this is the rarest form of cardiomyopathy only represents about 5% of all the cases.

02:08 When you start to look at the causes of this particular type of cardiomyopathy, the restrictive cardiomyopathy, they can kind of be grouped into category of familial.

02:18 They can be some very specific or known gene mutations.

02:22 Now, remember, idiopathic means we kind of have an idea but not exactly in some cases.

02:28 So, it can also be associated with other conditions.

02:32 Now, looking at amyloidosis, that's the most common cause.

02:35 But here's additional things they can bring on and cause restrictive cardiomyopathy, sarcoidosis, and then look at that last one, endomyocardial fibrosis.

02:48 So, myocardial, we're talking about the muscle of the heart, endomyocardial.

02:53 Fibrosis is stiff.

02:56 Since, this is restrictive cardiomyopathy you saw at the beginning, the artists put little bricks in the walls of the heart to remind you that it's really stiff, and not able to function or to relax.

03:09 So, endomyocardial fibrosis may be something that you also see.

03:14 Now, people after radiation, they can end up with fibrosis, because it's after radiation.

03:20 They can end up with a thrombus.

03:21 If you have a big fat clot in the ventricle, it's also going to make that wall stiff because it's pushing against a solid thrombus.

03:30 Tumors can cause a similar type of situation that you would see with a ventricular thrombus.

03:36 So let's go back and recenter. Let's refocus and see.

03:40 In a normal heart, there's going to be appropriate contractility.

03:45 Right, it's going to have the ability to do that.

03:48 Now, in a heart that suffering problems, you see those little arrows going on there, that is to represent continuous injury to that myocardium to that wall of the heart.

04:00 When that happens, the wall becomes fibrous or thick and stiff.

04:06 So the difference between a normal heart and a heart experiencing restrictive cardiomyopathy is what's going on to that wall.

04:16 If it hasn't had continuous injury, it can definitely contract in a strong and healthy way that moves blood throughout the rest of the body.

04:25 If it's a suffered continuous injury, by those signs that you see there, the arrows that you see there, that's when the wall becomes fibrotic or stiff, and does not function at the way a healthy heart would.

04:40 So, if you have chronic fibrosis, there's our friends, the bricks to help you see become so difficult for the heart to contract and to fill.

04:50 So you have that what we refer to before as decreased compliance of the ventricles.

04:56 Now, this is non dilated, right.

04:58 You can see that on the left side there the ventricle is not exactly dilated like we've seen in other ones, but you have dystonic dysfunction.

05:07 So, wow, that is a lot of big words.

05:09 Pause for just a moment and let's reset.

05:12 You see the heart there.

05:15 The thing that's different from a normal heart is that we see the ventricular wall is very stiff.

05:20 That's why we put the bricks there just to help you remember that.

05:22 So, what happens when a left ventricular wall is that stiff? Well, the ventricles are less compliant.

05:30 You don't have a dilated heart muscle, you have a nondilated heart muscle, and you have diastolic dysfunction.

05:38 Meaning it cannot feel like when you need it to filled in it will to push blood out to the rest of the body.

05:45 So, if the ventricles have filling in, you can't get blood out to the rest of the body, blood is going to back up from the ventricle.

05:52 If I'm in the left ventricle, it's going to backup to where? All right the left atrium.

05:59 Now, in this case of restrictive cardiomyopathy, since the left ventricle is not functioning, blood is backing up into the left atrium, then you could have some real challenges in that left atrium.

06:11 This patient is also going to experience dyspnea.

06:14 They can also have orthopnea which means they cannot even lay flat without becoming short of breath.

06:20 They can also have chest pain and palpitations because the heart is not able to receive adequate oxygenation, because of all the changes that have happened.

06:29 When the body is not well oxygenated, the patient can become dizzy and they become agitated as a sign of their hypoxemia or low oxygen in the blood.

06:39 These patients can also have periods of syncope.

06:43 Now, as blood is backing up from that left ventricle you can also experience signs of right sided heart failure, jugular vein distension, peripheral edema.

06:52 These are clear cues that you should be watching for as a safe and effective nurse.