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Year One - Cardiovascular System > Hypertrophic Obstructive Cardiomyopathy > Flashcards

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
Q

What are the three conservative management options of hypertrophic obstructive cardiomyopathy?

A

Increased Hydration

Alcohol Cessation

Weight Loss

26
Q

How do we remember the pharmacological and surgical management options of hypertrophic obstructive cardiomyopathy?

A

AABCD

Amiodarone

Anticoagulation

Beta-Blockers

Calcium Channel Blockers

implantable cardioverter Defibrillator (ICD)

27
Q

What are the four pharmacological management options of hypertrophic obstructive cardiomyopathy?

A

Amiodarone

Anticoagulation

Beta-Blockers

Calcium Channel Blockers

28
Q

When is amiodarone used to manage hypertrophic obstructive cardiomyopathy?

A

When the complications of atrial fibrillation have developed

29
Q

When is anticoagulation used to manage hypertrophic obstructive cardiomyopathy?

A

When the complications of atrial fibrillation have developed - as stroke prophylaxis

30
Q

Name an anticoagulant used to manage hypertrophic obstructive cardiomyopathy

A

Warfarin

31
Q

When are beta-blockers used to manage hypertrophic obstructive cardiomyopathy?

A

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
Q

Name a beta-blocker used to manage hypertrophic obstructive cardiomyopathy

A

Bisoprolol

33
Q

When are calcium channel blockers used to manage hypertrophic obstructive cardiomyopathy?

A

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
Q

Name two calcium channel blockers used to manage hypertrophic obstructive cardiomyopathy

A

Verapamil

Diltiazem

35
Q

Which five pharmacological management options are contraindicated in hypertrophic obstructive cardiomyopathy?

A

ACEI

Digoxin

Flecainide

Inotropes

Nitrates

36
Q

When are ICDs used to manage hypertrophic obstructive cardiomyopathy?

A

They are used as prophylactic management of sudden cardiac death in individuals with high risk factors and a supporting clinical context

37
Q

When is ventricular septal myomectomy used to manage hypertrophic obstructive cardiomyopathy?

A

When the complications of left ventricular outflow tract obstruction have developed

38
Q

What is ventricular septal myomectomy?

A

It involves removal of the thickened heart tissue to improve blood flow

39
Q

What are the four complications of hypertrophic obstructive cardiomyopathy?

A

Sudden Cardiac Death

Atrial Fibrillation

Heart Failure

Infective Endocarditis

Year One - Cardiovascular System (20 decks)

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