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STRUCTURAL HEART DISEASE Flashcards

1
Q

List examples of congenital structural heart diseases

A

Ventricular septal defect (VSD)
Tetralogy of Fallot (TOF)
Atrial septal defect (ASD)
Coarctation of the aorta

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2
Q

Describe the condition of ventricular septal defect (VSD) and the presentations

A

Septum fails to develop normally causing holes between ventricles causing the mixing of oxygenated and deoxygenated blood

Presentations:

  • poor weight gain
  • poor feeding
  • palpitations
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3
Q

What is the treatment of VSD?

A

Open heart surgery/cardiac catherization to manually close it .

Sometimes if hole is small enough it closes when child grows older

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4
Q

Describe the condition of tetralogy of Fallot (TOF)

A

Occurrence 4 different effects which can happen together

  • VSD
  • Pulmonary stenosis
  • Widening of aortic valve (causes mixing of blood)
  • Right ventricular hypertrophy
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5
Q

Describe the condition of atrial septal defect (ASD)

A

Wall between atria fails to develop properly causing a hole and mixing of blood

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6
Q

Describe the condition of coarctation of the aorta

A

Narrowing of the descending aortic artery increasing afterload and causing hypertrophy of ventricles and heart failure

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7
Q

List some valvular defects

A

Aortic stenosis
Aortic regurgitation

Mitral stenosis
Mitral regurgitation

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8
Q

Which valve disease is more prevalent in all age ranges, mitral or aortic?

A

Mitral valve disease

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9
Q

What is the most common valvular disease in the UK and Europe?

A

Aortic stenosis

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10
Q

What age group does aortic stenosis mainly affect?

A

Older people 70-80 yrs old

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11
Q

What is aortic stenosis often preceded by?

A

Aortic sclerosis

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12
Q

How is aortic stenosis often suspected?

A

Presence of an early-peaking, systolic ejection murmur and confirmed by echocardiography

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13
Q

What are the risk factors of aortic stenosis?

A 
Hypertension
LDL levels
Smoking
Elevated C-reactive protein
Congenital bicuspid valves (more prone to wear and tear)
CKD (more exposed to infection)
Radiotherapy
Older age
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14
Q

What are the causes of aortic stenosis?

A

Rheumatic heart disease
Congenital heart disease
Calcium build up causing calcification

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15
Q

Describe the pathophysiology of an aortic stenosis?

A

Valvular endocardium is damaged due to abnormal blood flow across congenital bicuspid aortic valve or by unknown trigger

Inflammation causing fibrosis and deposition of calcium on valve

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16
Q

What is the pathophysiology of aortic stenosis in rhuematic disease?

A

Autoimmune inflammation triggered by prior streptococcus infection that targets the valvular endothelium eventually leading to calcification

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17
Q

How does aortic stenosis lead to systolic heart failure?

A

The pressure overload causes left ventricular hypertrophy as an adaptive mechanism to maintain normal wall stress
As stenosis gets worse, adaptive mechanism fails and wall stress increases eventually causing systolic heart failure

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18
Q

What investigations can you undergo to diagnose aortic stenosis?

A

Transthoracic echocardiography
ECG chest X ray
Cardiac catheterisation
Cardiac MRI

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19
Q

For what aortic stenosis patients should aortic valve replacement be done for?

A

Primary treatment of symptomatic aortic stenosis

Asymptomatic patients with severe AS with LVEF < 50% or who are undergoing other cardiac surgery

Asymptomatic patients with severe AS with rapid progression, abnormal exercise test or elevated serum BNP levels

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20
Q

What are the symptoms of aortic stenosis and what may they have in their history?

A

Exertional dyspnoea and fatigue
Chest pain
Ejection systolic murmur

History of:

  • rheumatic fever
  • high lipoprotein
  • high LDL
  • CKD
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21
Q

What is aortic regurgitation?

A

Diastolic leakage of blood from the aorta into the left ventricle due to incompetence of valve leaflets from either intrinsic valve disease or dilation of aortic root

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22
Q

What happens in chronic aortic regurgitation?

A

Gradually increase in LV volume leading to LV enlargement and eccentric hypertrophy.

Early stages: ejection fraction normal or slightly increase
After some time ejection fraction falls and LV end systolic volume rises. Eventually LV dyspnoea lowering coronary perfusion and causing ischemia, necrosis and apoptosis

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23
Q

What occurs in acute aortic regurgitation?

A

Increased blood volume in LV during systole due to blackflow causing LV end diastolic pressure to increase. This increases pulmonary venous pressure leading top dyspnea and pulmonary oedema and heart attack cumulating into cardiogenic shock

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24
Q

List the causes of aortic regurgitation

A 
Rheumatic heart disease
Infective endocarditis
Aortic valve stenosis
Congenital heart defects
Congenital bicuspid valves

Aortic root dilation

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25
What are the causes of dilation of the aortic root and thus aortic regurgitation?
``` Marfan's sundrome Connective tissue diseases Idiopathic Akylosing spondilytis Traumatic ```
26
What are some causes of acute aortic regurgitation?
Infective endocarditis causing rupture of leaflets or paravalvular leaks Vegetations on valvular cusps causing inadequate closure of leaflets resulting in leakage of blood Chest trauma causing tear in ascending aorta
27
What are some causes of chronic aortic regurgitation?
Bicuspid aortic valve | Rheumatic fever leading to fibrotic changes causing thickening and retraction of leaflets
28
How does acute AR present?
``` Cardiogenic shock Tachycardia Cyanosis Pulmonary oedema Austin flint murmur ```
29
How does chronic AR present?
Wide pulse pressure Corrigan's pulse (water hammer pulse) Pistol shot pulse (traube sign)
30
What are the investigations for aortic regurgitation?
Transthoracic echocardiography Chest X ray Cardiac catheterisation Cardiac MRI/CT scan
31
What is the management of aortic regurgitation?
Acute AR - ionotropes/vasodilators, valve replacement Chronic symptomatic - valve replacement with vasodilator therapy Chronic asymptomatic - drugs/reassurance if LV function normal PREVENTION IS KEY - treat rheumatic fever/infective endocarditis with ABs
32
What is the aetiology of mitral stenosis?
``` - Rheumatic fever (MS typically occurs decades after an episode of this) - Carcinoid syndrome - Ergot/serotonergic drugs - SLE - Mitral annular calcification due to ageing - Amyloidosis - RA - Whipple disease - Congenital deformity ```
33
What is the pathophysiology of mitral stenosis?
Acute insult e.g. rheumatic fever leads to multiple foci formation and infiltrates in the endo/myocardium and along walls of valves Over time it is thickened, calcified and contracted causing stenosis
34
What does mitral stenosis cause and thus how will the patient present?
Initially: - Exertional dyspnoea (exercise) due to increased left atrial pressure Severe: - Increase in left atrial pressure and increased back pressure into pulmonary veins causing transudation of fluid into lung interstitium and dyspnoea at rest/exertion and or orthopnea Pulmonary hypertension - hemoptysis if bronchial vein rupture Decreased cardiac output due to limited left ventricular filling
35
What is the management of mitral stenosis?
Progressive asymptomatic - no therapy needed Severe asymptomatic - usually no therapy, maybe balloon valvotomy Severe symptomatic - diuretic, balloon valvotomy, valve replacement/repair with adjunct beta blockers
36
What are the acute causes of mitral regurgitation?
``` Mitral valve prolapse Rheumatic heart disease infective endocarditis Following valvular surgery Prosthetic mitral valve dysfunction ```
37
What are the chronic causes of mitral regurgitation?
``` Rheumatic heart disease SLE Scleroderma Hypertrophic cardiomyopathy Drug related ```
38
What does chronic mitral regurgitation lead to?
Increased atrial pressure and volume due to back flow. This larger volume in atrium flows back into ventricle during LV diastole causing: - Increased ventricular pressure - Eccentric LV hypertrophy - Elongation of myocardial fibres and increased left ventricle end diastolic volume/diameter. Increase in preload and decrease in afterload
39
How does infective endocarditis cause mitral regurgitation?
Abscess formation, vegetations, rupture of chordae tendinae and leaflet perforation
40
How does a patient with mitral regurgitation present?
``` Dyspnea Diminished S1 Fatigue Orthopnea Chest pain Atrial fibrillation ```
41
What investigations can be done for a patient suspected with mitral regurgitation?
``` ECG Transthoracic echocardiography Cardiac catheterisation Chest Xray Cardiac MRI/CT scan ```
42
What investigations can be done for a patient suspected with mitral stenosis?
``` ECG Transthoracic echocardiography Cardiac catheterisation Chest Xray Cardiac MRI/CT scan ```
43
What is the management for a patient with mitral regurgitation?
Acute - emergency surgery with preoperative diuretics, intra-aortic balloon counterpulsation Chronic asymptomatic - ACE inhibitors, beta blockers. If left ventricular EF is < 60% first line surgery Chronic symptomatic - surgery plus medical treatment. If left ventricular EF < 30% first line intra-aortic balloon counterpulsation
44
What are cardiomyopathies?
Disease of heart muscle which makes it harder for heart to pump blood to rest of body and can lead to heart failure
45
What are the main types of cardiomyopathies?
Dilated Hypertrophic Restrictive
46
What age group does dilated cardiomyopathy most commonly occur at?
30-40 years old
47
What is the aetiology of dilated cardiomyopathy?
Primary: - Familial - Idiopathic Secondary: - Heart valve disease - After child birth - Thyroid disease - Myocarditis - Alcoholism - Autoimmune disorders - Drugs - Mitochondrial disorders
48
Is dilated cardiomyopathy reversible?
Usually irreversible
49
What occurs in dilated cardiomyopathy?
Ventricular chamber enlargement and systolic dysfunction with normal left ventricular wall thickness Lower ejection fraction and increase in ventricular wall stress and end systolic volumes Compensatory mechanisms kick in but become overwhelmed and heart fails
50
What is the hallmark of dilated cardiomyopathy?
Ventricular dilation usually > 4 cm at autopsy
51
What are the compensatory mechanisms against dilated cardiomyopathy?
Increase heart rate Increase tone of peripheral vascular system Neurohumoral activation of renin-angiotensin aldosterone system and an increase in circulating levels of catecholamines Increase natriuretic peptides
52
How does dilated cardiomyopathy present?
``` Dyspnoea Systolic murmur Fatigue Angina Pulmonary congestion Low cardiac output Displaced apex beat, S3 ```
53
What investigations can you do for a patient suspected with dilated cardiomyopathy?
``` Genetic testing Viral serology ECG Chest Xray Cardiac catheterisation Cardiac MRI/CT Exercise stress test Echocardiography ```
54
What is the management of a patient with dilated cardiomyopathy?
Counselling since long term condition Diet changes Treat underlying ACEi, beta blockers for heart failure symptoms - (if ineffective consider heart transplant) Anticoagulants
55
What is the aetiology of hypertrophic cardiomyopathy?
Genetic- 50% are autosomal dominant Mendelian-inherited disease
56
What is the leading cause of sudden cardiac death in preadolescent and adolescent children?
Hypertrophic cardiomyopathy
57
What is hypertrophic cardiomyopathy and where does it often occur?
Increase in left ventricular wall thickness that is not solely explained by abnormal loading conditions Often involves interventricular septum causing obstruction to left ventricular outflow tract
58
What is the hallmark of hypertrophic cardiomyopathy?
Inappropriate, often asymmetrical myocardial hypertrophy that occurs in absence of an obvious hypertrophy stimulus
59
What does hypertrophic cardiomyopathy cause?
Abnormal diastolic function which impairs ventricular filling and increases filling pressure Abnormal calcium kinetics SDubendocardial ischaemia
60
How may a patient with hypertrophic cardiomyopathy present?
``` Sometimes asymptomatic with sudden cardiac death Double carotid artery impulse S3 gallop Syncope Presyncope Ejection systolic murmur Congestive heart failure Dizziness Palpitations Angina ```
61
What investigations can be done for a patient with hypertrophic cardiomyopathy?
``` Haemoglobin level (anaemia exacerbates chest pain and dyspnea) Elevated BNP levels Elevated troponin T levels Echocardiography Chest Xray Cardiac MRI ```
62
What is the management of hypertrophic cardiomyopathy?
``` Symptomatic: - Beta blocker - Verapamil - Disopyramide (LVOT gradient + persistant symptoms + absence of contraindication to drug) ``` Mechanical therapy (if refractory symptoms) - Pacemaker with AV delay - Septal myectomy/ablation
63
What is restrictive cardiomyopathy?
Less well-defined since diagnosis based on presence of restrictive ventricular filling pattern
64
What is the pathophysiology behind restrictive cardiomyopathy?
Characterised by diastolic dysfunction/heart failure with restrictive ventricular physiology and normal systolic function. Atrial enlargement due to impaired ventricular filling Impaired stiffness of myocardium causes ventricular pressure to rise very high but with only slight increase in volume Prominent ventricular filling in early diastole but stops abruptly at end of rapid filling phase due to reduced compliance and thus reduced cardiac output Systolic dysfunction and ventricular arrhythmias in advanced cases
65
What is the aetiology behind restrictive cardiomyopathies?
Idiopathic Familial Systemic disorders e.g. haemochromatosis, amyloidosis, sarcoidosis, carcinoid syndrome, Fabry's, scleroderma...
66
How might a patient with restrictive cardiomyopathy present?
Ascities, pitting oedema of lower extremities, fluid in abdo or lung - comfortable in sitting position Enlarged liver (maybe painful, full of fluid) Weight loss, cardiac cachexia Systemic findings e.g. easy bruising, periorbital purpura, macroglossia, carpal tunnel syndrome indicate amyloidosis Increase jugular venous pressure Decrease stroke volume, cardiac output and pulse volume
67
What investigations can be done for a patient with restrictive cardiomyopathy?
``` CBC Serology Amyloidosis check Chest Xray ECG Echocardiography Cardiac catheterisation Cardiac MRI/biopsy ```
68
What is the management for restrictive cardiomyopathy?
- Heart failure medication (ACEi, ARBs, diuretics, aldosterone inhibitors) - Antiarrhythmic therapy if conduction abnormalities - Immunosuppression (steroids) - Pacemaker - Transplant

CARDIORESPIRATORY 2 (8 decks)

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