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Year 2 - BRS > 5.3 - Structural heart diseases 2/2 > Flashcards

5.3 - Structural heart diseases 2/2 Flashcards

1
Q

Define structural heart disease?

A
  • structural heart disease covers a number of defects which affect the valves and chambers of the heart and the aorta
  • some defects are present at birth (congenital) while others form later in life (due to damage by infections etc)
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2
Q

What are some examples of congenital heart defects? (6)

A
  • atrial septal defect (ASD)
  • ventricular septal defect (VSD)
  • coarctation of aorta
  • patent foramen ovale (PFO)
  • patent ductus arteriosus (PDA)
  • teratology of fallot (TOF)
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3
Q

What are some examples of heart defects that develop later in life? (2)

A
  • valvular dysfunctions (aortic stenosis, aortic regurgitation)
  • muscular dysfunctions (cardiomyopathies)
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4
Q

What is an atrial septal defect (ASD)?

A

Hole in wall between the two atria

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5
Q

What is a ventricular septal defect (VSD)?

A
  • wall between the two ventricles fails to develop in foetus –> hole in wall
  • leads to mixing of oxygenated blood from LV with deoxygenated blood in RV
  • child - poor weight gain, poor feeding, palpitations
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6
Q

What happens in tetralogy of fallot (TOF)?

A
  1. ventricular septal defect - hole in wall between ventricles
  2. pulmonary stenosis - pulmonary trunk is stenosed/narrowed
  3. overriding aorta - aorta sits above VSD hole so it sits above both LV and RV and allows for mixing of blood between them and into aorta
  4. right ventricular hypertrophy - thickening of RV
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7
Q

What is coarctation of the aorta?

A
  • narrowing of aorta
  • blood has to force its way through, so ventricle has to work harder to push more blood through narrowing
  • leads to thickening of ventricles or heart failure
  • serious condition, urgent repair needed
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8
Q

What is stenosis of aortic/mitral valve?

A

Narrowing of the valve

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9
Q

What is regurgitation of aortic/mitral valve?

A

Incompetence of valve (flappy, not tightly closed)

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10
Q

What is the epidemiology of aortic and mitral valve disease like?

A
  • age 60+ = all types of valve disease become very prevalent
  • mitral > aortic prevalence
  • (mild) mitral regurgitation is the most common form of valve disease
  • MR > AS > MS > AR
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11
Q

What is aortic stenosis?

A

When the aortic valve narrows and does not open fully, reducing or blocking blood flow into the aorta

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12
Q

What are some epidemiological facts about aortic stenosis?

A
  • most common valvular disease in the US and Europe requiring treatment
  • second most frequent cause for cardiac surgery
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13
Q

What is aortic stenosis preceded by?

A

Aortic sclerosis - aortic valve thickening without flow limitation (asymptomatic)

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14
Q

How is aortic stenosis suspected?

A

Often suspected by the presence of an early-peaking systolic ejection murmur and confirmed by echocardiography (heart walls are thickened)

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15
Q

What age does aortic stenosis most likely happen in?

A

It is largely a disease of older people >70 years

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16
Q

What are the risk factors of aortic stenosis? (8)

A
  • hypertension
  • high LDL
  • smoking
  • elevated C-reactive protein
  • congenital bicuspid valves (aortic valve has 2 cusps, not 3)
  • chronic kidney disease
  • radiotherapy
  • older age
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17
Q

What are the causes of aortic stenosis? (3)

A
  • rheumatic heart disease
  • congenital heart diseases
  • calcium build up
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18
Q

What events trigger the pathophysiology of aortic stenosis?

A
  • degeneration (age-related) or congenital bicuspid valve –> wear and tear of valve / disruption of valve endothelium
  • untreated Group A Streptococcus URTI –> anti-Strep antibodies wrongly attack valves leading to inflammation of valve endocardium
  • both these lead to fibrosis and calcification of aortic valve
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19
Q

What is the pathophysiology of aortic stenosis?

Triggers pathophysiology:

  • degeneration (age-related) or congenital bicuspid valve –> wear and tear of valve / disruption of valve endothelium
  • untreated Group A Streptococcus URTI –> anti-Strep antibodies wrongly attack valves leading to inflammation of valve endocardium
  • both these lead to fibrosis and calcification of aortic valve
A
  1. fibrosis and calcification of aortic valve
  2. disrupted blood flow through aortic valve
  3. LV has to contract harder to pump blood through the stenotic valve
  4. over time, continuous forceful contraction of LV causes concentric LV myocardial hypertrophy
  5. hypertrophic LV becomes stiff overtime and harder to fill (increased muscle reduces volume) –> decreased cardiac output (diastolic dysfunction)
  6. pressure overload in LV backs up in the LA, causing it to dilate and ultimately leading to increase in pressure in lungs causing pulmonary congestion
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20
Q

What kind of hypertrophy is seen in aortic stenosis?

A

Concentric hypertrophy (increased thickness, reduces volume of LV) - remember that due to the narrowed valve, the LV contracts with more FORCE to allow all blood through (rather than there being a buildup of blood in LV)

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21
Q

In aortic stenosis, what clinical finding is associated with disrupted blood flow through the valve?

A
  • turbulent blood flow through valve during systole on auscultation
  • ejection systolic murmur (crescendo-decrescendo)
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22
Q

In aortic stenosis, what clinical finding is associated with LV contracting harder to get blood from aorta –> body?

A

Syncope on exertion - during exercise, decreased output to brain

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23
Q

In aortic stenosis, what clinical finding is associated with hypertrophied muscle (high oxygen demand) and increase in pressure in ventricles (resulting in less coronary perfusion)?

A

Angina on exertion

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24
Q

In aortic stenosis, what clinical finding is associated with increase back pressure in lungs –> pulmonary congestion?

A
  • diffuse crackles on auscultation of lungs
  • dyspnoea
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25
Q

What clinical findings are found in aortic stenosis? (5)

A
  • turbulent blood flow through valve during systole on auscultation –> ejection systolic murmur (crescendo-decrescendo)
  • syncope on exertion
  • angina on exertion
  • diffuse crackles on auscultation of lungs
  • dyspnoea
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26
Q

How do patients with aortic stenosis present? (5)

A
  • exertional dyspnoea and fatigue
  • chest pain, angina
  • syncope
  • heart failure
  • ejection systolic murmur
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27
Q

What do patients with aortic stenosis usually have a history of? (5)

A
  • rheumatic fever
  • high lipoprotein
  • high LDL
  • chronic kidney disease
  • age >65
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28
Q

What investigations are done in aortic stenosis?

A

Doppler echo is essential to the diagnosis (for the pressure gradient)

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29
Q

How is aortic stenosis managed?

A

Aortic valve replacement (AVR)

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30
Q

How is severe aortic stenosis treated?

A
  • transcatheter valve replacement
  • surgical valve prosthesis
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31
Q

What should be taken into account when deciding treatment for aortic stenosis?

A
  • lifetime risks and benefits associated with the type of approach:
  • transcatheter vs surgical?
  • type of valve - mechanical vs bioprosthetic?
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32
Q

What is aortic regurgitation?

A

Diastolic leakage of blood from the aorta into the left ventricle

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33
Q

What kind of murmur does aortic regurgitation cause?

A

Early diastolic ejection murmur

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34
Q

Why does aortic regurgitation occur?

A

Due to incompetence of valve leaflets, resulting from either intrinsic valve disease or dilation of the aortic root

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35
Q

What happens if aortic regurgitation is acute?

A

Medical emergency - presenting with sudden onset of pulmonary oedema and hypotension or cardiogenic shock

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36
Q

What happens if aortic regurgitation is chronic?

A

Culminates into congestive heart failure

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37
Q

What is the epidemiology of aortic regurgitation like?

A

Not as common as aortic stenosis and mitral regurgitation

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38
Q

What are the congenital and acquired causes of aortic regurgitation? (5)

A
  • rheumatic heart disease
  • infective endocarditis
  • aortic valve stenosis
  • congenital heart defects
  • congenital bicuspid valves
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39
Q

What are the causes of aortic regurgitation through aortic root dilation? (5)

A
  • Marfan’s syndrome
  • connective tissue disease/collagen vascular diseases
  • idiopathic
  • ankylosing spondylitis
  • traumatic
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40
Q

What events trigger the pathophysiology of aortic regurgitation?

A
  • Marfan’s syndrome, ankylosing spondylitis, syphilis, idiopathic –> aortic root dilation
  • post inflammatory response (untreated group A streptococcus rheumatic fever, endocarditis); collagen vascular disease (i.e. good pasteurs disease); congenital bicuspid valve –> inflammation of valvular endocardium leading to abnormal valve leaflet
  • aortic root dilation + inflammation of valvular endocardium leading to abnormal valve leaflet –> valve leaflets close poorly when aortic pressure is higher than LV during diastole
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41
Q

What is the pathophysiology of aortic regurgitation?

Triggers pathophysiology:

  • Marfan’s syndrome, ankylosing spondylitis, syphilis, idiopathic –> aortic root dilation
  • post inflammatory response (untreated group A streptococcus rheumatic fever, endocarditis); collagen vascular disease (i.e. good pasteurs disease); congenital bicuspid valve –> inflammation of valvular endocardium leading to abnormal valve leaflet
  • aortic root dilation + inflammation of valvular endocardium leading to abnormal valve leaflet –> valve leaflets close poorly when aortic pressure is higher than LV during diastole
A
  1. valve leaflets close poorly when aortic pressure > LV pressure during diastole
  2. blood flow back from aorta to LV
  3. volume and pressure overload in LV = increase LV preload and afterload
  4. acutely, the dilation = increase in SV due to Frank Starling law
  5. chronically, LV dilates and eccentrically hypertrophies to accommodate increase in volume
  6. later excessive stretching weakens the myocardium and unable to contract properly leading to systolic heart failure (5 <–> 6)
  7. back pressure in LV to atria and ultimately lung vasculature leading to congestion
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42
Q

In aortic regurgitation, what clinical finding is associated with backflow from aorta to LV?

A

Diastolic murmur (between S2 and S1)

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43
Q

In aortic regurgitation, what clinical finding is associated with chronic dilation of LV –> early filling of heart during systole?

A

The S3 gallop sound that occurs in early diastole, produced by rapid filling and expansion of ventricles

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44
Q

In aortic regurgitation, what clinical finding is associated with hypertrophied muscle (high oxygen demand), reduced aortic pressure (compromised coronary circulation) and decrease in SV?

A

Angina on exertion, fatigue

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45
Q

In aortic regurgitation, what clinical finding is associated with back pressure in lungs –> pulmonary congestion?

A
  • diffuse crackles on auscultation of lungs
  • dyspnoea
  • orthopnoea
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46
Q

What is a bounding/corrigan/collapsing pulse found in aortic regurgitation?

A

Results from large stroke volume followed by the exaggerated collapse of the large vessels on the diastolic return of blood to the LV (large upstroke and downstroke)

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47
Q

What clinical findings are associated with aortic regurgitation? (8)

A
  • diastolic murmur
  • S3 gallop in early diastole (rapid filling and expansion of ventricles)
  • angina on exertion
  • fatigue
  • diffuse crackles on auscultation of lungs
  • dyspnoea
  • orthopnoea
  • bounding/corrigan/collapsing pulse
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48
Q

How does acute aortic regurgitation present? (6)

A
  • hypotension
  • pulmonary oedema
  • cardiogenic shock
  • tachycardia
  • cyanosis
  • diastolic murmur
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49
Q

How does chronic aortic regurgitation present? (2)

A
  • wide pulse pressure (high systolic BP, low diastolic BP - due to LV hypertrophy increasing systolic BP, and reduced blood in aorta reducing diastolic BP)
  • corrigan pulse (water hammer pulse)
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50
Q

How do you investigate aortic regurgitation?

A

Echocardiography is the best non-invasive test to grade the severity of AR

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51
Q

What is the main way of managing acute aortic regurgitation?

A

Aortic valve replacement (AVR) - acute AR is a medical emergency

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52
Q

How do we manage asymptomatic patients with chronic severe aortic regurgitation?

A

Vasodilator therapy improves haemodynamics and delays the need for AVR

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53
Q

What can we do to prevent aortic regurgitation? (Prevention is key)

A

Treat rheumatic fever and infective endocarditis

54
Q

What is mitral stenosis?

A

Obstruction to left ventricular inflow at the level of mitral valve due to structural abnormality of the mitral valve

55
Q

What does mitral stenosis lead to as the disease progresses?

A

Pulmonary hypertension and therefore right heart failure

Mitral disorders closer to lungs than aortic disorders

56
Q

What is the main cause of mitral stenosis in developing countries?

A

Rheumatic fever

57
Q

What are some causes of mitral stenosis? (9)

A
  • rheumatic fever
  • carcinoid syndrome
  • use of ergot/serotonergic drugs
  • SLE
  • mitral annular calcification due to ageing
  • amyloidosis
  • rheumatoid arthritis
  • whipple disease
  • congenital deformity of the valve
58
Q

What events trigger the pathophysiology of mitral stenosis?

A

Rheumatic fever, IE, calcific (age-related), congenital stenosis –> recurrent inflammation

59
Q

What is the pathophysiology of mitral stenosis?

Triggers pathophysiology:
Rheumatic fever, IE, calcific (age-related), congenital stenosis –> recurrent inflammation

A
  • recurrent inflammation
  • fibrous deposition and calcification of the mitral valve leaflets and chordae tendinae –> thickening and shortening of chordae tendinae
  • thick and stiff leaflets and fusion of the junction between leaflets
  • decrease in area of orifice –> obstructed blood flow through MV (–> impaired emptying of LA and impaired filling of LV)
  • impaired emptying of LA –> increase in left atrial pressure –> back pressure in LA and ultimately lung vasculature = congestion
  • impaired filling of LV –> decrease in SV and CO = congestive heart failure <–> increase in RV pressure leads to hypertrophy of RV - right-sided heart failure
60
Q

In mitral stenosis, what clinical finding is associated with turbulent blood flow across the valve?

A

Opening snap, mid-diastolic murmur

61
Q

In mitral stenosis, what clinical finding is associated with left atrial enlargement (stretch of conduction fibres)?

A

Afibrillation

62
Q

In mitral stenosis, what clinical finding is associated with left atrial enlargement (compression of surrounding structures - rare)?

A

Dysphagia and hoarseness (rare)

63
Q

In mitral stenosis, what clinical finding is associated with decreased SV and CO?

A

Cardiogenic shock / congestive heart failure

64
Q

In mitral stenosis, what clinical finding is associated with RV hypertrophy?

A

Right-sided heart failure

65
Q

In mitral stenosis, what clinical finding is associated with increased pulmonary vasculature pressure (transudation of fluid in interstitium, pulmonary oedema)?

A

Dyspnoea

66
Q

What clinical findings are associated with mitral stenosis? (6)

A
  • opening snap, mid-diastolic murmur
  • afibrillation
  • dysphagia and hoarseness (rare)
  • cardiogenic shock/congestive heart failure
  • right-sided heart failure
  • dyspnoea
67
Q

How do patients with mitral stenosis present? (7)

A
  • history of rheumatic fever
  • dyspnoea
  • mid-diastolic murmur
  • opening snap/loud S1 in early stages
  • dysphagia (enlargement LA can compress left recurrent laryngeal nerve)
  • atrial fibrillation (palpitations)
  • haemoptysis (bronchial vein rupture from pulmonary HTN)
68
Q

What investigations can be done for mitral stenosis? (3)

A
  • ECG
  • chest X-ray
  • transthoracic echocardiography
69
Q

How do we manage progressive asymptomatic mitral stenosis?

A

No therapy required

70
Q

How do we manage severe asymptomatic mitral stenosis?

A
  • no therapy generally required
  • can do adjuvant balloon valvotomy (not very effective)
71
Q

How do we manage severe symptomatic mitral stenosis? (4)

A
  • diuretics
  • balloon valvotomy
  • valve replacement
  • repair adjunct beta blockers
72
Q

What is mitral regurgitation?

A
  • most frequent valvular heart disease
  • abnormal reversal of blood flow from the left ventricle to the left atrium
  • caused by disruption in any part of the mitral valve apparatus
73
Q

What is the most common cause of acute mitral regurgitation leading to heart failure?

A

Mitral valve prolapse (leads to mitral valve’s leaflets bulging back into left atrium during heart contraction, which can prevent the mitral valve from closing tightly and leads to regurgitation)

74
Q

What are the causes of acute mitral regurgitation? (5)

A
  • mitral valve prolapse
  • rheumatic heart disease
  • infective endocarditis
  • following valvular surgery
  • prosthetic mitral valve dysfunction
75
Q

What are the causes of chronic mitral regurgitation? (5)

A
  • rheumatic heart disease
  • SLE
  • scleroderma
  • hypertrophic cardiomyopathy
  • drug-related
76
Q

What events trigger the pathophysiology of mitral regurgitation?

A
  • coronary heart disease (IHD and MI/myocarditis) - dilation of valve leaflets and tethering of chordae tendinae
  • papillary muscle rupture - valve leaflets flail
  • infective endocarditis - vegetations on the leaflets
  • rheumatoid heart disease - dilatation of mitral valve annulus inflammation of the leaflets
  • connective tissue disorder - weak valve leaflets
  • mitral valve prolapse - structurally abnormal valve
  • these all cause MR - back flow of blood from LV to LA due to impaired closure of the valve
77
Q

What is the pathophysiology of mitral regurgitation?

Triggers pathophysiology:

  • coronary heart disease (IHD and MI/myocarditis) - dilation of valve leaflets and tethering of chordae tendinae
  • papillary muscle rupture - valve leaflets flail
  • infective endocarditis - vegetations on the leaflets
  • rheumatoid heart disease - dilatation of mitral valve annulus inflammation of the leaflets
  • connective tissue disorder - weak valve leaflets
  • mitral valve prolapse - structurally abnormal valve
  • these all cause MR - back flow of blood from LV to LA due to impaired closure of the valve
A
  • MR - back flow of blood from LV to LA due to impaired closure of valve
  • increase volume and pressure in left atrium
  • increase volume pushed back into left ventricle in next diastole
  • LV dilation - remodelling - decrease in LV systolic function
    • LV dilation –> back pressure in left atria and ultimately lung vasculature leading to congestion
    • LV dilation –> decreased SV and CO - congestive heart failure
78
Q

In mitral regurgitation, what clinical finding is associated with blood flow constantly backwards in systole?

A

Holosystolic murmur radiating to axilla

79
Q

In mitral regurgitation, what clinical finding is associated with increase volume pushed back into LV?

A

S3 heart sound

80
Q

In mitral regurgitation, what clinical finding is associated with decreased oxygen to kidneys due to decreased organ perfusion (parenchymal damage)?

A

Increase in serum creatinine

81
Q

In mitral regurgitation, what clinical finding is associated with decrease in stroke volume and cardiac output?

A

Cardiogenic shock / congestive heart failure

82
Q

In mitral regurgitation, what clinical finding is associated with congestive heart failure?

A

Peripheral oedema

83
Q

In mitral regurgitation, what clinical finding is associated with fluid extravasates out of vessels and into the lungs?

A
  • decrease in oxygen saturation
  • tachypnoea
  • wheeze
  • crackles
  • frothy sputum
84
Q

What clinical findings are associated with mitral regurgitation? (10)

A
  • holosystolic (pansystolic) murmur radiating to axilla
  • S3 heart sound
  • increase in serum creatinine
  • cardiogenic shock/congestive heart failure
  • peripheral oedema
  • decrease in oxygen saturation
  • tachypnoea
  • wheeze
  • crackles
  • frothy sputum
85
Q

How do patients with mitral regurgitation present? (4)

A
  • dyspnoea
  • holosystolic murmur
  • S3 heart sound
  • signs of congestive heart failure
86
Q

What investigations are done for mitral regurgitation? (4)

A
  • transthoracic echocardiography
  • ECG
  • chest X-ray
  • cardiac MRI/CT scan
87
Q

How do we manage acute severe mitral regurgitation?

A
  • regurgitation corrected by repairing or replacing the supporting valve structures
  • prosthetic ring can be inserted to reshape the valve
88
Q

How do we manage chronic severe asymptomatic mitral regurgitation?

A

Watchful waiting or surgery

89
Q

How do we manage chronic symptomatic mitral regurgitation?

A

1st line is surgery plus medical treatment

90
Q

What are cardiomyopathies?

A

Diseases of the heart muscle that make it harder for your heart to pump blood to rest of your body

91
Q

What can cardiomyopathies lead to?

A

Heart failure

92
Q

What are the three main types of cardiomyopathies?

A
  • dilated cardiomyopathy
  • hypertrophic cardiomyopathy
  • restrictive cardiomyopathy
93
Q

How common is dilated cardiomyopathy?

A
  • estimated prevalence is 1:2500
  • among the most common causes of heart failure
94
Q

When in life does dilated cardiomyopathy usually manifest?

A

Most commonly in third or fourth decade of life

95
Q

What is dilated cardiomyopathy?

A

Progressive, usually irreversible disease causing global systolic (contractile) dysfunction with heart failure

96
Q

What is the hallmark gross finding of dilated cardiomyopathy at autopsy?

A

Left ventricular dilatation, usually more than 4cm

97
Q

How common are familial causes of dilated cardiomyopathy?

A

25% of all cases

98
Q

What is the primary cause of dilated cardiomyopathy without family history?

A

Idiopathic

99
Q

What are the secondary causes of dilated cardiomyopathy? (8)

A
  • myocardial ischaemia/heart valve disease
  • after childbirth
  • thyroid disease
  • myocarditis
  • alcoholism
  • autoimmune disorders
  • ingestion of drugs
  • inherited disorders
100
Q

What is dilated cardiomyopathy characterised by?

A

Ventricular chamber enlargement and systolic dysfunction with normal left ventricular wall thickness

101
Q

What events trigger the pathophysiology of dilated cardiomyopathy?

A
  • viral myocarditis, autoimmune diseases, idiopathic, myocardial ischaemia –> inflammatory damage and death of the myocytes
  • chronic alcoholism, inherited disorders, drugs –> toxic damage and death of the myocytes
  • these lead to eccentric fibrosis and volume increases
102
Q

What is the pathophysiology of dilated cardiomyopathy?

Triggers pathophysiology:

  • viral myocarditis, autoimmune diseases, idiopathic, myocardial ischaemia –> inflammatory damage and death of the myocytes
  • chronic alcoholism, inherited disorders, drugs –> toxic damage and death of the myocytes
  • these lead to eccentric fibrosis and volume increases
A
  • eccentric fibrosis and volume increases
  • enlargement of LV chamber without corresponding increase in myocardial mass
  • Frank-Starling law initially - contractility is okay
  • gradually over-distension and systolic dysfunction
  • decreased cardiac output and increased EDV/EDP
  • volume overload - congestive heart failure
103
Q

How do patients with dilated cardiomyopathy present? (7)

A
  • dyspnoea (low CO = insufficient tissue oxygenation)
  • displaced apex beat (enlarged LV)
  • fatigue (low CO = decreased organ perfusion)
  • angina (low coronary perfusion)
  • pulmonary congestion (diffuse crackles)
  • peripheral oedema (heart failure)
  • sudden cardiac death
104
Q

What investigations do we do for dilated cardiomyopathy? (7)

A
  • ECG
  • chest X-ray
  • cardiac catheterisation
  • cardiac MRI/CT scan
  • echocardiography
  • genetic testing
  • viral serology
105
Q

How do we manage dilated cardiomyopathy?

A
  • counselling
  • consider cause
  • symptomatic treatment, rehabilitation
  • diet modification - fluid and Na restriction
  • treatment of underlying diseases
  • treat symptoms of heart failure - ACEi, beta blockers, diuretics, ARBs, surgery (LVAD)?
  • treat arrhythmias - amiodarone
  • treat thrombotic events - anticoagulants
106
Q

What is hypertrophic cardiomyopathy (HCM)?

A

A genetic cardiovascular disease defined by an increase in left ventricular wall thickness not solely explained by abnormal loading conditions

107
Q

What is hypertrophic cardiomyopathy the leading cause of?

A

Sudden cardiac death in preadolescent and adolescent children/young ATHLETIC person

108
Q

What is the worrying thing about the symptoms of hypertrophic cardiomyopathy?

A
  • most patients are asymptomatic
  • the first clinical manifestation in these people may be sudden death, likely from ventricular tachycardia or fibrillation
109
Q

How commonly is hypertrophic cardiomyopathy familial?

A

Familial HCM occurs as an autosomal dominant Mendelian-inherited disease in approximately 50% of cases

110
Q

What is the pathophysiology of hypertrophic cardiomyopathy?

A
  • genetic, storage diseases, neuromuscular or mitochondrial disorders, malformation syndromes
  • thickening and disarray of left ventricular myocardium - can occur in any region of LV
  • frequently involves interventricular septum, which results in an obstruction of flow through the left ventricular outflow tract
  • disorganised myocytes disrupt signal conduction
  • ventricular arrhythmias
  • sudden cardiac death
111
Q

How does hypertrophic cardiomyopathy present? (7)

A
  • S4 (forceful atrial contraction into a hypertrophied LV)
  • syncope (reduced CO to peripheries and head)
  • fatigue (low CO and decreased organ perfusion)
  • angina (low coronary perfusion)
  • pulmonary congestion and oedema (diffuse crackles)
  • systolic murmur (passage of blood through narrow outflow tract)
  • sudden cardiac death
112
Q

How do we investigate hypertrophic cardiomyopathy? (3)

A
  • echocardiography
  • chest X-ray
  • cardiac MRI
113
Q

How do we manage hypertrophic cardiomyopathy with symptoms?

A

Beta blockers - if contraindicated veramapil

114
Q

How do we manage hypertrophic cardiomyopathy if refractory and drugs fail?

A

Mechanical therapy with pacemaker or surgery (septal myectomy or ablation)

115
Q

How do we manage asymptomatic hypertrophic cardiomyopathy?

A

Regular echocardiograms to monitor condition

116
Q

What is restrictive cardiomyopathy?

A
  • a less well-defined cardiomyopathy as its diagnosis is based on establishing the presence of a restrictive ventricular filling pattern
  • diastolic dysfunction with restrictive ventricular physiology but systolic function normal
117
Q

What % of all cases of diagnosed cardiomyopathies does restrictive cardiomyopathy account for?

A

5%

118
Q

What could the causes of restrictive cardiomyopathy be? (10)

A
  • idiopathic (mainly)
  • familial (related to troponin I or desmin mutations, latter often in association with a skeletal myopathy)
  • haemochromatosis
  • amyloidosis
  • sarcoidosis
  • Fabry’s disease
  • carcinoid syndrome
  • scleroderma
  • anthracycline toxicity
  • previous radiation
119
Q

What is the pathophysiology of restrictive cardiomyopathy?

A
  • infiltrative cardiomyopathies characterised by deposition of abnormal substances (e.g. amyloid proteins, noncaseating granulomas, iron) within heart tissue
  • infiltration causes endomyocardial fibrosis leading to ventricular walls stiffening –> diastolic dysfunction
  • atrial enlargement occurs due to impaired ventricular filling, but volume and thickness of ventricles are usually normal
  • restrictive physiology predominates in the early stages, causing contraction abnormalities and diastolic heart failure
  • adverse remodelling may lead to systolic dysfunction and ventricular arrhythmias in advanced cases
  • reduced ventricular filling –> decreased cardiac output
120
Q

How do patients with restrictive cardiomyopathy present? (5)

A
  • ascites and pitting oedema in peripheries (increase venous pressure –> right-sided heart failure)
  • hepatomegaly (hepatic congestion due to right-sided heart failure)
  • S4 (atrium contracts into stiff ventricle)
  • increase in jugular venous pressure (due to right-heart failure)
  • easy bruising, weight loss (heart failure)
121
Q

What investigations do we do for restrictive cardiomyopathy? (8)

A
  • echocardiography
  • ECG
  • chest X-ray
  • MRI/biopsy
  • catherisation
  • CBC (blood count)
  • serology
  • amyloidosis check
122
Q

How do we manage restrictive cardiomyopathy? (5)

A
  • heart failure medication - guideline-directed medical therapy for HF including ACEi/ARBs, diuretics and aldosterone inhibitors for reduced LV
  • antiarrhythmic therapy
  • immunosuppression - steroids
  • pacemaker
  • cardiac transplantation
123
Q

What is the difference between concentric and eccentric hypertrophy?

A
  • concentric hypertrophy is caused by pressure overload, while eccentric hypertrophy is caused by volume overload
  • concentric hypertrophy involves adding new sarcomeres in parallel to existing sarcomeres = wall thickness increases
  • eccentric hypertrophy involves adding new sarcomeres in series to existing sarcomeres = ventricular dilation
124
Q

When are S1, S2, S3 and S4 commonly heard?

A
  • S1 - systole
  • S2 - diastole
  • S3 - mainly dilated cardiomyopathy / mitral dysfunction
  • S4 - mainly hypertrophic cardiomyopathy
125
Q

What kind of murmur is associated with aortic stenosis?

A

Ejection systolic murmur - loudest at right sternal edge 2nd ICS, radiates to carotids, crescendo-decrescendo, louder on expiration, accentuated by patient leaning forward

126
Q

What clinical finding precedes aortic stenosis, and how can you differentiate between these?

A
  • aortic sclerosis - stiffening of the aortic valve without flow limitation, precedes aortic stenosis
  • ejection systolic murmur that RADIATES TO CAROTIDS = aortic stenosis
  • ejection systolic murmur ALONE = aortic sclerosis
127
Q

What three medications do you use in heart failure?

A
  • diuretics (spironolactone, thiazides) - reduce fluid overload e.g. pulmonary oedema
  • antihypertensives (ACEi, ARBs) - reduce strain on heart from high BP
  • beta blockers - reduce heart rate and increase contractility, meaning heart has to work less to pump more blood
128
Q

What kind of murmur is associated with aortic regurgitation?

A

Early diastolic murmur - loudest at right sternal edge 2nd ICS, loudest on expiration, no accentuation manoeuvre

129
Q

What kind of murmur is associated with mitral stenosis?

A

Mid-diastolic murmur - loudest at 5th ICS left MCL, loudest on expiration, accentuate by patient lying in left lateral decubitus

130
Q

What kind of murmur is associated with mitral regurgitation?

A

Pansystolic murmur (radiating to axilla) - loudest at 5th ICS left MCL, loudest on expiration, accentuate by patient lying in left lateral decubitus and moving stethoscope towards left axilla

Year 2 - BRS (84 decks)

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