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Cardiovascular > Structural heart defects and other heart disease > Flashcards

Structural heart defects and other heart disease Flashcards

1
Q

What are cardiomyopathies?

A

Group of diseases of the myocardium that affect the mechanical or electrical function of the heart

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2
Q

Give some key characteristics of hypertropic cardiomyopathies

A
  • Caused by sarcomeric protein gene mutations
  • Thickened heart muscle which decreases the chamber size
  • Muscle fibrosis in the heart
  • Powerful heart contraction but it is less compliant so relaxation takes longer
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3
Q

What might be shown on an ECG of ahypertropic cardiomyopathy?

A

Very large voltages and repolarisation T waves changes

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4
Q

Give some key characteristics of dilated cardiomyopathies

A
  • Can be LV/RV or all 4 chamber dilatation and dysfunction
  • The heart wall is either normal or thin
  • Contractility is impaired
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5
Q

What is the most commonly seen type of cardiomyopathy?

A

Dilated cardiomyopathies

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6
Q

Give some key characteristics of restricted cardiomyopathies

A
  • There is a decreased or normal volume of both ventricles
  • Bi-atrial enlargement
  • Poor dilatation of the heart
  • Rigid myocardium which restricts ventricular filling
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7
Q

Name some causes of restricted cardiomyopathies

A
  • Idiopathic
  • Amyloidosis
  • Sarcoidosis
  • End-myocardial fibrosis
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8
Q

What is the physiological cause of arrhythmogenic cardiomyopathies?

A

Desmosome gene mutations so adhesion between the cells is lost

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9
Q

What characteristics would you seen on ECGs for arrhythmogenic cardiomyopathies?

A
  • Abnormalities typically over RV (V1-3) anterior leads

* Characteristic epsilon waves

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10
Q

What is Naxos disease?

A

A subset of cardiomyopathies with a physical phenotype

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11
Q

What physical characteristics are seen in Naxos disease?

A
  • Palmoplantar keratoderma due to separation in abrasions (e.g. from crawling)
  • Wooley hair
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12
Q

Is Naxos disease dominant or recessive?

A

Recessive

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13
Q

What are inherited arrhythmias?

A

Genetically mediated rhythm disturbances due to ion channel abnormalities

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14
Q

Name some inherited arrhythmias

A
  • Long QT syndrome
  • Brugada syndrome
  • Catecholaminergic polymorphic VT (CPVT)
  • Wolff-Parkinson White
  • Prognostic conduction disease
  • Idiopathic VF
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15
Q

What is aortic stenosis?

A

Narrowing of the aortic valve resulting in obstruction to the left ventricular stroke volume

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16
Q

What is the normal area of the aortic valve?

A

3-4 cm squared

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17
Q

When do symptoms occur in aortic stenosis?

A

When the valve area is a quarter of normal

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18
Q

What are the 3 types of aortic stenosis?

A
  1. Supravalvular
  2. Subvalvular
  3. Valvular
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19
Q

What is the most common type of aortic stenosis?

A

Valvular

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20
Q

Give 2 congential causes of aortic stenosis

A
  • Congenital aortic stenosis

* Congenital bicuspid aortic valve (BAV)

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21
Q

Give 2 acquired causes of aortic stenosis

A
  • Calcific aortic valvular disease (CAVD)

* Rheumatic heart disease

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22
Q

Describe the pathophysiology of aortic stenosis

A
  • Due to obstructed LV emptying , a pressure gradient develops between the LV and the aorta (increased afterload)
  • LV function is initially maintained by compensatory pressure hypertrophy
  • When compensatory measures are exhausted , LV function declines rapidly
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23
Q

What are clinical presentations of aortic stenosis?

A
  • Syncope (exertional)
  • Angina
  • Heart failure
  • Dyspnoea (exertional) due to systolic and diastolic heart failure
  • Sudden death
  • Slow rising carotid pulse and decreased pulse amplitude
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24
Q

Describe what heart sounds might be heard in aortic stenosis

A
  • Soft or absent 2nd heart sound
  • Prominent 4th heart sound due to LV hypertrophy
  • Ejection systolic murmur crescendo-decrescendo hypertrophy
  • Loudness tells you nothing about severity
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25
Q

Give 2 differential diagnoses to aortic stenosis

A
  • Aortic regurgitation

* Subacute bacterial endocarditis

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26
Q

How would you diagnose arotic stenosis?

A
  • Echocardiography – 2 measurements obtained
  • ECG
  • CXR
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27
Q

Why is an echocardiogram performed in aortic stenosis?

A
  • LV size and function: LV hypertrophy, dilation and ejection fractions
  • Doppler derived gradient and valve area (AVA) – assess pressure gradient
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28
Q

What might a chest X-ray in aortic stenosis show?

A
  • LV hypertrophy

* Calcified aortic valve

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29
Q

How can you treat aortic stenosis?

A
  • Good dental hygiene to avoid infective endocarditis
  • Aortic valve replacement
  • Transcatheter aortic valve implantation (TAVI)
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30
Q

What is the role of medical treatment in aortic stenosis?

A
  • Limited role since AS is a mechanical issue

* Vasodilators contraindicated in severe AS

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31
Q

What are the indications for intervention in aortic stenosis?

A
  • Any symptomatic patient with severe AS (including symptoms with exercise)
  • Any patient with decreasing ejection fraction
  • Any patient undergoing CABG with moderate/severe AS
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32
Q

What is mitral regurgitation?

A

Backflow of blood from the LV to the LV during systole

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33
Q

What is the most frequent cause of mitral regurgitation?

A

Myxomatous degeneration

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34
Q

Give some causes of mitral regurgitation?

A
  • Myxomatous degeneration
  • Ischaemic mitral valve
  • Rheumatic heart disease
  • Infective endocarditis
  • Papillary muscle dysfunction/rupture
  • Dilated cardiomyopathy
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35
Q

What is myxomatous degeneration?

A

Weakening of the chordae tendinae leading to mitral valve prolapse

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36
Q

Give 5 risk factors for mitral regurgitation

A
  1. Being female
  2. Lower BMI
  3. Advanced age
  4. Renal dysfunction
  5. Prior MI
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37
Q

What are compensatory mechanisms for mitral regurgitation?

A
  • LA enlargement

* LV hypertrophy

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38
Q

What are some clinical presentations of mitral regurgitation?

A
  • Exertional dyspnoea
  • Fatigue and lethargy
  • Increased SV felt as palpitation
  • Heart failure
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39
Q

In mitral regurgitation what might you hear on ascultation?

A
  • Soft S1 and pan systolic murmur at the apex radiating to the axilla
  • Prominent S3 in congestive heart failure/LA overload
  • Displaced hyperdynamic apex beat
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40
Q

How would you diagnose mitral regurgitation?

A
  • ECG
  • CXR
  • Echocardiogram
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41
Q

What might an ECG show in mitral regurgitation?

A
  • LA enlargement
  • AF and LV hypertrophy with severe MR
  • ECGs aren’t diagnostic in MR
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42
Q

What might an CXR show in mitral regurgitation?

A
  • LA enlargement

* Central pulmonary artery enlargement

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43
Q

How would you manage mitral regurgitation?

A
  • Vasodilators e.g. ACE-i
  • Beta blockers for AF heart rate control
  • Calcium channel blockers and digoxin
  • Anticoagulation in AF and AFl
  • Diuretics for fluid overload
  • Serial echocardiography
  • IE prophylaxis
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44
Q

How often would serial echocardiography occur for patients with mitral regurgitation?

A
  • Mild: 2-3 years
  • Moderate: 1-2 years
  • Severe: 6-12 months
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45
Q

What are indications for surgery in severe mitral regurgitation?

A
  • Patients with any symptoms at rest or exercise

* Asymptomatic but ejection fraction <60%, LV end systolic dimension >45mm or new onset AF

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46
Q

What is aortic regurgitation?

A

Leakage of blood into the LV from the aorta in diastole due to ineffective coaptation (drawing together) of the 3 aortic cusps

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47
Q

Give 2 chronic causes of aortic regurgitation

A
  1. Congenital bicuspid aortic valve (BAV)

2. Rheumatic fever

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48
Q

Give an acute cause of aortic regurgitation

A

Infective endocarditis

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49
Q

Give 5 risk factors of arotic regurgitation

A
  1. SLE
  2. Marfan’s and Ehlers-Danlos syndrome
  3. Aortic dilatation
  4. Infective endocarditis
  5. Aortic dissection
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50
Q

Give 2 compensatory mechanisms in aortic regurgitation

A
  1. LV dilatation

2. LV hypertrophy

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51
Q

Give 6 clinical presentations of aortic regurgitation

A
  1. Exertional dyspnoea
  2. Palpitations
  3. Angina
  4. Syncope
  5. Wide pulse pressure
  6. Apex beat is displaced laterally
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52
Q

What heart sounds might you hear in aortic regurgitation?

A
  • Diastolic blowing murmur at the left sternal border

* Systolic ejection murmur; due to increased flow across the aortic valve

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53
Q

Give 3 differential diagnoses of aortic regurgitation

A
  1. Heart failure
  2. Infective endocarditis
  3. Mitral regurgitation
54
Q

What would you use to diagnose aortic regurgitation?

A
  • Echocardiogram
  • CXR
  • ECG
55
Q

How would you use an echocardiogram to diagnose aortic regurgitation?

A
  • Evaluation of the aortic valve and aortic root

* Measurement of left ventricle dimensions and function

56
Q

What might you see on a chest X-Ray of someone with aortic regurgitation?

A
  • Enlarged cardiac silhouette and aortic root enlargement

* Left ventricular enlargement

57
Q

What might you see on an ECG of someone with aortic regurgitation?

A
  • Tall R waves
  • Deeply inverted T waves in the left-sided chest leads
  • Deep S waves in the right-sided leads
58
Q

How would you manage someone with aortic regurgitation?

A
  • Consider prophylaxis IE treatment
  • Vasodilators e.g. ACE-I if the patient is symptomatic/hypertensive
  • Serial echocardiograms to monitor progress
  • Valve replacement surgery
59
Q

What are the indications for surgery in someone with aortic regurgitation?

A
  • Any symptoms at rest/on exertion
  • Asymptomatic if ejection fraction <50% or LV becomes dilated
  • ECG deterioration (T wave inversion in lateral leads)
60
Q

What is mitral stenosis?

A

Obstruction of LV inflow that prevents proper filling during diastole

61
Q

What is the normal area of the mitral valve?

A

4-6cm squared

62
Q

In mitral stenosis, at what valve area do symptoms usually manifest?

A

<2cm squared

63
Q

Is mitral stenosis more common in men or women?

A

Men

64
Q

What are usually the causes of mortality in mitral stenosis?

A
  • Progressive pulmonary congestion
  • Infection
  • Thromboembolism
65
Q

Give 2 risk factors for mitral stenosis

A
  • History of rheumatic fever

* Untreated streptococcus infections

66
Q

What is the most common cause of mitral stenosis?

A

Rheumatic heart disease

67
Q

What is mitral stenosis caused by rheumatic heart disease usually secondary to?

A

Rheumatic fever due to a group A β-haemolytic streptococcus

68
Q

What are the causes of mitral stenosis?

A
  • Rheumatic heart disease
  • Infective endocarditis
  • Mitral annular calcification
69
Q

Give some clinical presentations of mitral stenosis

A
  • Progressive dyspnoea
  • Haemoptysis
  • Right heart failure in advanced disease
  • AF
  • Systemic emboli
  • Prominent “a” wave in jugular venous pulsations
  • Mitral facies/malar flush
70
Q

What is mitral facies and why does it occur?

A

In severe mitral stenosis it is pinkish-purple patches on the cheeks from vasoconstriction due to diminished cardiac output

71
Q

What heart sounds might be heard in mitral stenosis?

A

• Diastolic murmur
- Low-pitched diastolic rumble most prominent at the apex
- Heard best with patient lying on the left side in held expiration
• Loud opening S1 snap
- Due to the abrupt halt in leaflet motion in early diastole, after a rapid initial opening, due to fusion at the leaflet tips
• More severe disease might have an opening snap closer to S2

72
Q

What would you use to diagnose mitral stenosis?

A
  • Echocardiogram
  • ECG
  • CXR
73
Q

What is the gold standard for diagnosis of mitral stenosis?

A

Echocardiogram

74
Q

What would you assess on an echocardiogram of mitral stenosis?

A
  • Mitral valve mobility

* Gradient and mitral valve area

75
Q

What might you see on a chest X-Ray of mitral stenosis?

A
  • LA enlargement
  • Pulmonary oedema/congestion
  • Occasionally calcified mitral valve
76
Q

How would you manage mitral stenosis?

A
  • Serial echocardiography
  • Medicatons for symptoms
  • Percutaneous mitral balloon valvotomy (PMBV)
  • IE prophylaxis
  • Mitral valve replacement
77
Q

Why don’t medications prevent progression of mitral stenosis?

A

Because mitral stenosis is a mechanical problem

78
Q

What drugs might be given to manage symptoms of mitral stenosis?

A
  • Beta blockers/CCBs/digoxin – control HR to prolong diastole and improve diastolic filling
  • Diuretics – for fluid overload
79
Q

What are indications for mitral valve replacement in mitral stenosis?

A
  • Any symptomatic patient with NYHA class III or IV symptoms

* Asymptomatic moderate or severe stenosis with pliable valve suitable for PMBV

80
Q

What is infective endocarditis?

A

An infection of the endocardium or vascular endothelium of the heart

81
Q

What are the 5 types of infective endocarditis?

A
  1. Left sided native IE (mitral or aortic)
  2. Left sided prosthetic IE
  3. Right sided IE
  4. Device related IE 5. Prosthetic (early or late)
82
Q

How can infective endocarditis be acquired?

A
  • Having an abnormal valve
  • Infectious material introduced into the blood stream or directly into the heart during surgery
  • Previous IE infection
83
Q

What are the locations that can be infected by infectious endocarditis?

A
  • Valves with congenital or acquired defects
  • Normal valves with virulent organisms such as Strep.pneumoniae or S.aureus
  • Prosthetic valves and pacemakers
84
Q

What are the 4 main groups of people who are likely to get infective endocarditis?

A
  1. The elderly
  2. Young IV drug abusers
  3. Young people with congenital heart disease
  4. Anyone with prosthetic heart valves
85
Q

Are men or women more likely to get infective endocarditis?

A

Men

86
Q

What is the most common cause of infective endocarditis?

A

Staphylococcus aureus

87
Q

What are the main causes of infective endocarditis?

A
  • Staphylococcus aureus (IVDU, diabetes and surgery)
  • Pseudomonas aeruginosa
  • Streptococcus viridans (dental problems)
88
Q

What are the risk factors for infective endocarditis?

A
  • IV drug use
  • Poor dental hygiene
  • Skin and soft tissue infection
  • Dental treatment
  • IV cannula
  • Cardiac surgery
  • Pacemaker
89
Q

What are the common clinical presentations of infective endocarditis?

A
  • Signs of systemic infection e.g. fever, sweats, etc.)
  • Embolism: stroke, PE, bone infections, kidney dysfunction, MI
  • Valve dysfunction leading to heart failure and arrhythmia
90
Q

What are specific, characteristic manifestations of infective endocarditis?

A
  • Finger clubbing
  • Splinter haemorrhages on nail beds of fingers
  • Embolic skin lesions • Osler nodes
  • Janeway lesions
  • Roth spots
  • Petechiae
91
Q

What are Osler nodes?

A

Tender nodules in the digits

92
Q

What are Janeway lesions?

A

Haemorrhages and nodules in the fingers

93
Q

What are Roth spots?

A

Retinal haemorrhages with white or clear centres seen on fundoscopy

94
Q

What are petechiae?

A

Small red/purple spots caused by bleeds in the skin

95
Q

What makes up the modified Duke’s criteria for infective endocarditis?

A 
• 2 Major criteria
- Microorganisms grown from blood cultures
- Evidence of endocarditis on echo, or new valve leak
• 5 Minor criteria
- Predisposing factors
- Fever
- Vascular phenomena
- Immune phenomena
- Equivocal blood cultures
96
Q

What elements from Duke’s criteria would indicate definite infective endocarditis?

A

2 major or 1 major + 3 minor or 5 minor criteria

97
Q

What elements from Duke’s criteria would suggest possible infective endocarditis?

A

1 major or 1 major + 3 minor or 5 minor criteria

98
Q

How would you diagnose infective endcarditis?

A
  • Modified Duke’s criteria
  • Blood clutures
  • Blood tests
  • Urinalysis
  • CXR
  • ECG
  • Echocardiogram
99
Q

What is important to remember when taking blood cultures for suspected infective endocarditis?

A
  • Take 3 sets from different sites over 24hrs
  • Take cultures before starting antibiotics
  • Cultures only identify IE in 75% of cases
100
Q

What would blood tests for infective endocarditis show?

A
  • CRP and ESR raised
  • Normochromic, normocytic anaemia
  • Neutrophilia
101
Q

What might be seen on a chest X-Ray in infective endocarditis?

A

Cardiomegaly

102
Q

What might be seen on an ECG in infective endocarditis?

A

Long PR interval at regular intervals

103
Q

Compare the use of transthoracic (TTE) and transoesophageal (TOE) echos

A
  • TTE: Safe, non-invasive, low sensitivity

* TOE: Very uncomfortable, more sensitive so better at diagnosing

104
Q

How can you prevent/reduce risk of infective endocarditis?

A
  • Good oral hygiene

* IE prophylaxis

105
Q

How can you treat infective endocarditis?

A
  • Antibiotic treatment for 4-6 weeks
  • Treat complications
  • Surgery to remove valve and replace with a prosthetic one
106
Q

What are the indications for surgery in infective endocarditis?

A
  • If infection cannot be cured with antibiotics
  • Complications e.g. aortic root abscess, severe valve damage
  • To remove infected devices
  • To replace valve after infection has been cured
  • To remove large vegetations before they embolise
107
Q

What antibiotics might be used to treat infective endocarditis?

A
  • For Staph use vancomycin and rifampicin (if MRSA)

* If not Staph use penicillin - ideally benzylpenicillin and gentamycin

108
Q

Give some causes of congenital heart disease

A
  • Maternal prenatal rubella infection
  • Maternal alcohol misuse
  • Gene association e.g. Down’s
  • Drugs e.g. thalidomide, amphetamines, lithium
  • Maternal diabetes
  • Genetic abnormalities
109
Q

Give 5 common clinical presentations of congenital heart disease

A
  • Central cyanosis
  • Pulmonary hypertension
  • Clubbing of the fingers
  • Growth retardation
  • Syncope
110
Q

In Tetralogy of Fallot, what are the 4 things wrong with the heart?

A
  1. Ventricular septal defect
  2. Pulmonary atresia
  3. Overriding aorta
  4. RV hypertrophy
111
Q

What is pulmonary atresia?

A

Undergrowth of pulmonary outflow tract

112
Q

What is the pathophysiology of Tetralogy of Fallot?

A
  1. Stenosis of RV outflow leads to RV pressure higher than LV pressure
  2. Deoxygenated blood passes to LV
  3. Cyanosis occurs especially when feeding
113
Q

What are some clinical presentations/features of Tetralogy of Fallot?

A
  • Central cyanosis
  • Low birthweight and growth
  • Dyspnoea on exertion
  • Delayed puberty
  • Systolic ejection murmurs
  • Boot-shaped heart on CXR
114
Q

How would you treat Tetralogy of Fallot?

A
  • Full surgical treatment during first 2yrs of life

* May require pulmonary valve surgery due to valve regurgitation occuring

115
Q

What are ventricular septal defects?

A

Abnormal connection between the two ventricles

116
Q

What are the abnormal physiological changes in ventricular septal defects?

A
  • Low pressure RV and high pressure LV

* So there is increased blood flow to the lungs

117
Q

Give 4 clnical signs in small ventricular septal defects

A
  • Loud systolic murmur
  • Thrill (buzzing sensation)
  • Well grown
  • Normal HR and size
118
Q

Give 5 clnical signs in large ventricular septal defects

A
  • Small, breathless, skinny baby
  • Increased respiratory rate
  • Tachycardia
  • Large heart on CXR
  • Murmur varies in intensity
119
Q

What are atrial septal defects?

A

Abnormal connection between the two atria

120
Q

What are the abnormal physiological changes in atrial septal defects?

A
  • Slightly higher pressure in the LA then the RA
  • Shunt is L to R
  • Increased flow into R heart and lungs
121
Q

Give 4 clinical signs seen in atrial septal defects

A
  • Pulmonary flow murmur
  • Fixed split second heart sounds
  • Big pulmonary arteries and heart on CXR
  • RBBB on ECG
122
Q

What are atrio-ventricular septal defects?

A

Hole in the centre of the heart (partial or complete)

123
Q

How might the valves be affected inatrio-ventricular septal defects?

A

The AV valves are often 1 big, malformed, leaky one

124
Q

What is patent ductus arteriosis?

A

A persistent communication between the L proximal pulmonary artery and descending aorta

125
Q

What are the clinical signs of patent ductus arteriosis?

A
  • Continuous ‘machinery’ murmur
  • Breathless if the heart is too large
  • Eisenmenger’s syndrome
126
Q

What is Eisenmenger’s syndrome?

A

The process in which a long-standing left-to-right cardiac shunt caused by a congenital heart defect leading to shunt reversal and cyanosis

127
Q

What is coarctation of the aorta?

A

Narrowing of the aorta at the site of insertion of the ductus arteriosus

128
Q

What is the physiology of mild coarctation of the aorta?

A
  • Presents with mild hypertension
  • Incidental murmur
  • Should be repaired to prevent long term damage
129
Q

What is the physiology of severe coarctation of the aorta?

A
  • Complete or almost complete obstruction to aortic flow
  • Collapse with heart failure
  • Urgent need of repair
130
Q

What is a possible consequence of mild or moderate pulmonary stenosis?

A

Right ventricular hypertrophy

131
Q

What are possible consequences of severe pulmonary artery stenosis?

A
  • RV failure as neonate
  • Collapse
  • Poor pulmonary blood flow
  • RV hypertrophy
  • Tricuspid regurgitation
132
Q

How can you treat pulmonary artery stenosis?

A
  • Balloon valvotomy
  • Open valvotomy
  • Open trans-annular patch
  • Shunt to bypass the blockage

Cardiovascular (5 decks)

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