Hypertrophic Obstructive Cardiomyopathy Flashcards

1
Q

What is hypertrophic obstructive cardiomyopathy (HOCM)?

A

It is defined as a genetic condition in which there is an asymmetrical increase in left ventricular wall thickness – usually affecting the septum

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2
Q

Does hypertrophic obstructive cardiomyopathy lead to predominately systolic or diastolic dysfunction?

A

Diastolic

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3
Q

What is the inheritance of hypertrophic obstructive cardiomyopathy?

A

Autosomal dominant

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4
Q

Which genetic mutation is associated with hypertrophic obstructive cardiomyopathy?

A

Cardiac sarcomere proteins encoding genes

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5
Q

Name three cardiac sarcomere proteins

A

Beta-myosin heavy chain

Myosin-binding protein C

Cardiac troponin C

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6
Q

What is the function of cardiac sacromere proteins?

A

They are important structural components of cardiac muscle

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7
Q

Due to the genetic mutation of the cardiac sarcomere protein genes, describe the pathophysiology of hypertrophic obstructive cardiomyopathy?

A

This results in diastolic dysfunction, which in turn leads to decreased cardiac output and thus left ventricular hypertrophy

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8
Q

What are the two associations of hypertrophic obstructive cardiomyopathy?

A

Friedrich’s Ataxia

Wolff-Parkinson White Syndrome

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9
Q

What are the eleven clinical features associated with hypertrophic obstructive cardiomyopathy?

A

Exertional Dyspnoea

Exertional Syncope

Palpitations

Chest Pain

Jerky Pulse Rate

Double Apex Beat

Systolic Murmurs

S4 Sound

Split S2 Sound

Prominent A Wave of JVP

Sudden Cardiac Death

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10
Q

What two heart murmurs are associated with hypertrophic obstructive cardiomyopathy?

A

Ejection Systolic

Mitral Regurgitation

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11
Q

Which heart murmur is most commonly associated with hypertrophic obstructive cardiomyopathy?

A

Ejection Systolic

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12
Q

Describe three features of the ejection systolic murmur associated with hypertrophic obstructive cardiomyopathy

A

It is loudest between the apex and left sternal border

It is worsened upon the Valsalva manoeuvre and expiration

It improves upon squatting

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13
Q

What does the development of the ejection systolic murmur in hypertrophic obstructive cardiomyopathy indicate?

A

Left ventricular outflow obstruction

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14
Q

How is sudden cardiac death a clinical feature of hypertrophic obstructive cardiomyopathy?

A

It can result in the development of ventricular arrhythmias

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15
Q

What are the five murmur features of mitral regurgitation?

A

Holosystolic, High Pitched, Whistling Murmur

Murmur Radiation To Axilla

Quiet, Soft S1

S3 Sound

Widely Split S2

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16
Q

What is the most common cause of sudden cardiac death in young athletic males? How?

A

Hypertrophic obstructive cardiomyopathy

It can lead to the development of ventricular arrhythmias

17
Q

What three investigations are used to diagnose hypertrophic obstructive cardiomyopathy?

A

ECG Scan

ECHO Scan

Cardiac MRI Scan

18
Q

What are the five features of hypertrophic obstructive cardiomyopathy on ECG scans?

A

Non-Specific ST Segment Abnormalities

Non-Specific T Wave Abnormalities

Progressive T Wave Inversion

Deep, Narrow Q Waves

Atrial Fibrillation Features

19
Q

What is the first line investigation used to diagnose hypertrophic obstructive cardiomyopathy?

A

ECHO scans

20
Q

What are the three features of hypertrophic obstructive cardiomyopathy on ECHO scans?

A

Asymmetrical Left Ventricular Wall Thickness > 15mm During Diastole

Mitral Regurgitation Features

Systolic Anterior Motion of the Anterior Mitral Valve Leaflet

21
Q

How can we remember the features of hypertrophic obstructive cardiomyopathy on ECHO scans?

A

MR SAM ASH

Mitral Regurgitation

Systolic Anterior Motion

ASymetric Hypertrophy

22
Q

Which ECHO scans are preferred to diagnose hypertrophic obstructive cardiomyopathy - transoesophageal or transthoracic?

A

Transthoracic

23
Q

How are cardiac MRI scans used to investigate hypertrophic obstructive cardiomyopathy?

A

They are not required for a diagnosis, however if local resources permit should be considered as part of the assessment

24
Q

What is the histological feature of hypertrophic obstructive cardiomyopathy?

A

Myofibrillar hypertrophy with chaotic and disorganised fashion myocytes and fibrosis

25
What are the three conservative management options of hypertrophic obstructive cardiomyopathy?
Increased Hydration Alcohol Cessation Weight Loss
26
How do we remember the pharmacological and surgical management options of hypertrophic obstructive cardiomyopathy?
AABCD Amiodarone Anticoagulation Beta-Blockers Calcium Channel Blockers implantable cardioverter Defibrillator (ICD)
27
What are the four pharmacological management options of hypertrophic obstructive cardiomyopathy?
Amiodarone Anticoagulation Beta-Blockers Calcium Channel Blockers
28
When is amiodarone used to manage hypertrophic obstructive cardiomyopathy?
When the complications of atrial fibrillation have developed
29
When is anticoagulation used to manage hypertrophic obstructive cardiomyopathy?
When the complications of atrial fibrillation have developed - as stroke prophylaxis
30
Name an anticoagulant used to manage hypertrophic obstructive cardiomyopathy
Warfarin
31
When are beta-blockers used to manage hypertrophic obstructive cardiomyopathy?
To provide provide symptomatic relief of exertional angina-like chest pain and heart failure – in the absence of significant left ventricular outflow tract obstruction and coronary artery disease
32
Name a beta-blocker used to manage hypertrophic obstructive cardiomyopathy
Bisoprolol
33
When are calcium channel blockers used to manage hypertrophic obstructive cardiomyopathy?
To provide provide symptomatic relief of exertional angina-like chest pain and heart failure – in the absence of significant left ventricular outflow tract obstruction and coronary artery disease
34
Name two calcium channel blockers used to manage hypertrophic obstructive cardiomyopathy
Verapamil Diltiazem
35
Which five pharmacological management options are contraindicated in hypertrophic obstructive cardiomyopathy?
ACEI Digoxin Flecainide Inotropes Nitrates
36
When are ICDs used to manage hypertrophic obstructive cardiomyopathy?
They are used as prophylactic management of sudden cardiac death in individuals with high risk factors and a supporting clinical context
37
When is ventricular septal myomectomy used to manage hypertrophic obstructive cardiomyopathy?
When the complications of left ventricular outflow tract obstruction have developed
38
What is ventricular septal myomectomy?
It involves removal of the thickened heart tissue to improve blood flow
39
What are the four complications of hypertrophic obstructive cardiomyopathy?
Sudden Cardiac Death Atrial Fibrillation Heart Failure Infective Endocarditis