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1st Year Systems > Cardiomyopathy, Myocarditis and Pericarditis > Flashcards

Cardiomyopathy, Myocarditis and Pericarditis Flashcards

1
Q

Learning Outcomes

A
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2
Q

What are the different types of cardiomyopathy?

A

Hypertrophic

Dilated

Restrictive

Myocarditis

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3
Q

What are thedifferent types of pericardial disease?

A

Pericarditis and effusion (with or without tamponade)

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4
Q

What is dilated cardiomyopathy?

A

A condition when the heart becomes enlarged and cannot pump blood efficiently, affects other body systems such as the lungs, liver.

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5
Q

Why does dilated cardiomyopathy result in ventricular remodelling?

A

Left or right systolic pump is impaired leading to progressive heart enlargement via ventricular hypertrophy and ventricular dilation,

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6
Q

What is dilated cardiomyopathy often a cause of?

A

Congestive heart failure

Thrombosis in chambers is not uncommon

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7
Q

What are the possible genetic aetiological backgrounds for dilated cardiomyopathy?

A

Mutations in the SCN5A gene (a heart sodium channel gene)

Muscular dystrophy

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8
Q

What are the possible aetiological backgrounds for dilated cardiomyopathy? (besides genetic causes)

A

Pregnancy (post partum)

Chagas disease

Toxic insults, including drugs, chemicals and hormones (doxorubicin (Adriamycin), and cobalt are included)

Injury

Inflammatory response

Infection

Autoimmune disease

Chronic severe ventricular extrasystole

Fibrous change of the myocardium from a previous myocardial infarction

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9
Q

Which chambers are affected in dilated cardiomyopathy?

A

Can be one but more often all chambers dilated and functionally impaired

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10
Q

What is the prognosis for dilated cardiomyopathy?

A

Some causes are specifically sought as they are reversible/part reversible but most are progressive and irreversible

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11
Q

What are the symptoms of dilated cardiomyopathy?

A

•Progressive, slow onset,

Dyspnoea

Fatigue

Orthopnoea

PND (paroxysmal nocturnal dyspnoea)

Ankle swelling

Weight gain of fluid overload

Cough.

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12
Q

What is a typical past medical history for someone with dilated cardiomyopathy?

A

Systemic illness

Travel

Hypertension

Vascular disease

Thyroid

Neuromuscular disease

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13
Q

What would you find on examination of someone with dilated cardiomyopathy?

A

Poor superficial perfusion

Thready pulse

Irregular pulse if in atrial fibrillation

SOB at rest

Narrow pulse pressure

JVP elevated

Displaced apex beat

S3 and S4 heart sounds

MR murmur

oedema (pulmonary, ankle and sacral)

Pleural effusions

Acites

Hepatomegaly

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14
Q

What are the relevant investigations for dilated cardiomyopathy?

A

Repeated ECG noting LBBB if present

CXR

Basic bloods

N terminal pro Brain Natriuretic peptide

Basic bloods FBC, U + E

Echo

CMRI (best imaging modality)

Coronary angiogram

Sometimes biopsy depending on time course of cardiomyopathy

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15
Q

What is general measures in the treatment of dilated cardiomyopathy?

A
  • Correct anaemia
  • Remove exacerbating drugs eg NSAIDs
  • Correct any endocrine disturbance
  • Advise on fluid and salt intake, reduce it
  • Advise on managing weight to identify fluid overload
  • HF nurse referral
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16
Q

What are the more specific measures in the treatment of dialted cardiomyopathy?

A

ACEi

Angiotensin receptor blockers

Diuretics

Beta Blockers

Spironolactone

Anticoagulants as required

Risk of sudden cardiac death is reduced with implantable cardioversion devices (small defibrillators) or Cariac resynchronisation therapy defibrillators (these devices resynchronizes the contractions of the heart’s ventricles by sending tiny electrical impulses to the heart muscle, which can help the heart pump blood throughout the body more efficiently, they also have the function of a defibrillator)

Cardiac Transplant

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17
Q

What is restrictive cardiomyopathy?

A

When the walls of the heart are rigid and the heart is restricted from stretching and filling with blood properly.

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18
Q

What are the non infilatrative forms of restrictive cardiomyopathy?

A

Familial

Forms of hypertrophic cardiomyopathy

Scleroderma (a chronic hardening and contraction of the skin and connective tissue, either locally or throughout the body - autoimmune disease)

Diabetic pseudoxanthoma elasticum (degeneration of elastic fibres within the body)

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19
Q

What are the infiltrative forms of restrictive cardiomyopathy?

A

Amyloidosis and sarcoidosis

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20
Q

What are the storage disease associated with restrictiva cardiomyopathy?

A

Haemachromatosis (a hereditary disorder in which iron salts are deposited in the tissues, leading to liver damage, diabetes mellitus, and bronze discoloration of the skin.)

Fabry disease (a rare genetic lysosomal storage disease - alpha-galactosidase A deficiency)

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21
Q

What are the Endomyocardial conditions asoociated with Restrictive cardiomyopathy?

A

Fibrosis, carcinoid, radiation, drug effects

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22
Q

Why is the process of relaxation of the ventricular walls described as active rather than passive?

A

It needs functioning intact myocytes

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23
Q

What are the relevant investigations for restrictive cardiomyopathy?

A
  • Repeated ECG noting LBBB if present and other conduction defects
  • CXR
  • N termial pro Brain Natriuetic Peptide
  • Basic bloods FBC, U+E, be on the look out for sarcoid and haemachromatosis
  • Auto antibodies for sclerotic CT diseases
  • Amyloid needs non cardiac biopsy to help establish the diagnosis
  • Fabry; low plasma alpha galactosidase A activity
  • Echo
  • CMRI, probably best imaging modality

Biopsy more helpful but still has high false negative rate

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24
Q

What is meant by amyloidosis?

A

Amyloidosis is a rare and serious disease caused by accumulation of proteins in the form of abnormal, insoluble fibres, known as amyloid fibrils, within the extracellular space in the tissues of the body

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25
Q

What are the general measures for restrictive cardiomyopathy?

A
  • Limited diuretic use as low filling pressures will cause problems
  • Beta blockers limited ACEI use
  • Anticoagulants as required
  • SCD risk assessment with ICD or CRT-D/P implant
  • Cardiac transplant

If iron overload, specific forms of amyloid or Fabrys then specific treatments are available

Endomyocardial fibrosis has little specific treatment

Prognosis

Unless reversible then poor prognosis

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26
Q

Define hypertrophic cardiomyopathy

A

Hypertrophic cardiomyopathy (HCM) is a disease in which a portion of the myocardium (heart muscle) is hypertrophic (enlarged) without any obvious cause, creating functional impairment of the heart.

The left ventricle (one of your heart’s four chambers) is almost always affected, and in some people the muscle of the right ventricle also thickens.

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27
Q

What gene is responsible for hypertrophic cardiomyopathy?

A

Sarcomere gene

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28
Q

What is the inheritance pattern for hypertrophic cardiomyopathy?

A

Autosomal dominant but with variable expression and incomplete penetrance

50% chance of inheriting the gene but how it is expressed is not known until time passes

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29
Q

Where are the possible places that hypertrophy can occur in the heart?

A

Apical, septal or generalised

30
Q

Why does cardiac hypertrophy often present in a restrictive manner?

A

Because there is impaired relaxation

31
Q

What is the complication associated with septal hypertrophy?

A

With mitral valve defect can lead to Left ventriculr outflow tract obstruction

LVOT is nearly indistinguishable from the rest of the ventricle

32
Q

What is the effect of hypertrophic cardiomyopathy on the coronary arteries?

A

Causes small vessel narrowing and consequent ischaemia and fibrosis, arrhythmias are common

33
Q

What are the symptoms of hypertrophic cardiomyopathy?

A

Asymptomatic for many

Fatigue

Dyspnoea

Anginal like chest pain

Exertional pre syncope

Syncope related to arrhythmias

LVOT obstruction

34
Q

What are the examination findings for hypertrophic cardiomyopathy?

A
  • Notched pulse pattern
  • Irreg pulse if in AF or ectopy
  • Double impulse over apex, thrills and murmurs, often dynamic, LVOT murmur will increase with valsalve and decrease with squatting
  • JVP can be raised in very restrictive filling
35
Q

What is the assessment for hypertrophic cardiomyopathy?

A
  • ECG, often abnormal but a few are normal where phenotype is poorly expressed in genotype +ve individuals
  • Echo
  • CMRI
  • Risk stratification for SCD, may need ICD

Exercise tolerance test

FH

36
Q

What are the general measures in the treatment of hypertrophic cardiomyopathy?

A
  • Avoid heavy exercise
  • Avoid dehydration
  • Explore FH and first degree relatives, ECGs and echoes may be required
  • Consider genetic testing
37
Q

What are the specific measures in the treatment of hypertrophic cardiomyopathy?

A
  • Drugs to try and enhance relaxation, variable results but often if symptomatic, beta blockers, verapamil, disopyrimide
  • If in AF anticoagulate
  • Obstructive form; surgical or alcohol septal ablation
  • ICD if required based on risk stratification
38
Q

What is myocarditis?

A

Acute or chronic inflammation of the myocardium

39
Q

What is restrictive myocarditis often confused for?

A

Constrictive pericarditis

40
Q

What is the effect of cardiomyopathy?

A

Can impair myocardial function, conduction and generate arrhythmia

41
Q

What are a few of the possible causes of myocarditis?

A

Viral - Adenovirus, Hep C, HIV

Bacterial - Mycobacterial Species, Chlamydia pneumoniae, Streptococcal species, treponum pallidum

Fungal - Aspergillus, candida

Toxins - anthracylines, cocaine

Autoimmune activation - Giant cell myocarditis, small pox vaccination

Protozoal - trypanosoma cruzi

Parasitic - Schistosomiasis, larva migrans

Hypersensitivity - clozapine, penicillin

42
Q

Where are the inflammatory cells in myocarditis?

A

Infiltration of inflammatory cells into the myocardial layers

43
Q

What is the result of myocarditis on the electrical function of the heart?

A

Can result in heart block and arrhythmias

44
Q

What are the symptoms of myocarditis?

A

Heart Failure with fatigue

SOB

Chest pain only in a quarter of patients

May not have fever

Signs of Heart Failure

45
Q

What are the relevant investigations for myocarditis?

A
  • ECG usually abnormal
  • Biomarkers often elevated but not falling in a pattern consistent with MI
  • Echo, can get RWMA
  • CMRI can see oedema in certain images
  • Low threshold for biopsy
  • Viral DNA PCR
  • Auto antibodies
  • Step antibodies
  • Lyme B burgdorferi
  • HIV
46
Q

What is the treatment for myocarditis?

A

Treatment of heart failure

Support for brady and tachycardia arrhytmias

  • Immunotherpay if biopsy or other Ix point to a specific diagnosis
  • Stop possible drugs or toxic agent exposure
47
Q

What is prognosis for myocarditis?

A

•30% recovery fully but 20% mortality at 1 year and 56% by 4 years. At 11 years those still alive are 93% transplant free.

48
Q

What is pericarditis?

A

INflamamtino of the pericardial layers with or without myocardial involvement

49
Q

What are the most common causes of pericarditis?

A

Mostly idiopathic or viral (echovirus, adenovirus)

Key ones to pick up are bacterial (streptococcus, staphylococcus, lymes disease) post MI (free wall rupture), perforation, dissection of proximal aorta, neoplasia

50
Q

What are the symptoms of pericardial disease?

A

Usually 1-2/52 duration, chest pain with pleuritic features and postual features, sitting forward usually improves it lying back makes it worse

Fever

51
Q

What are the signs of pericardial disease?

A

High temperature

Pericardial rub

Raised JVP (if there is a substantial or haemodynamically relevant effusion is present)

Low blood pressure

Muffled heart sounds

Very unwell with a high fever and NO effusion can suggest bacterial

52
Q

What are the investigations for pericardial disease?

A

ECG

ECHO

Troponin may be raised if myocardial involvement too

53
Q

What are the features of pericardial disease on an ECG?

A

Diffuse ST segment elevation and PR segment depression

54
Q

What is the general measures for pericardial disease?

A
  • Viral is conservative
  • Idiopathic gets colchicine and limited use of NSAIDs
  • Bacterial must be drained even if small effusion and antimicrobials, high death rate
  • If large effusion present and some haemodynamic effects then drain
55
Q

Define tamponade

A

Compression of the heart by an accumulation of fluid in the pericardial sac.

56
Q

What are the causes of pericardial effusion?

A

•Often same causes as pericarditis

57
Q

Wat are the symptoms of pericardial effusion where tamponade is present?

A

Symptoms are overt, fatige, SOB, dizzy with low BP, occasionally chest pain.

58
Q

Define pulsus paradoxus?

A

An abnormally large decrease in systolic blood pressure and pulse wave amplitude during inspiration. The normal fall in pressure is less than 10 mmHg.

59
Q

What are the signs of pericardial disease when tamponade is present?

A

Pulsus paradoxus

JVP raised

Low blood pressure

Sometimes pericardial rub

Sometimes muffled heart sounds

Pulmonary oedema is very rare

60
Q

What is the test for pericardial effusion?

A

Urgent echo

CXR can show large cardia shadow

61
Q

What is the treatment for pericardial effusion?

A

Drainage

Persistent effusion needs a surgical pericardial window made to allow flow to abdomen

62
Q

ECG of tamponade

A
63
Q

ECHO with large effusion

A
64
Q

What are the causes of constrictive pericarditis?

A

Causes are idopathic, radiation, post surgery, autoimmune, renal failure, sarcoid.

65
Q

What is the pathology of constricitve pericarditis?

A

Pathology is that of impaired filling although myocardium is normal most of the time

66
Q

What are the symptoms and signs of Constricitve pericarditis

A

Symptoms of fatigue, SOB, cough

Signs more of right heart failure with oedema, ascites, high JVP, jaundice, hepatomegally, AF, TR (tricuspid regurgitation?), pleural effusion, pericardial knock

67
Q

What is the investigation for Constricitve pericarditis?

A

Assess with echo and right heart cath to differentiate from restrictive cardiomyopathy which can be very difficult

68
Q

What is treatment for Constricitve pericarditis?

A

Treatment is with careful and limited diuretics and pericardectomy

69
Q

What is pericardectomy?

A

Pericardiectomy is the surgical removal of part or most of the pericardium. This operation is most commonly done to relieve constrictive pericarditis or to remove a pericardium that is calcified and fibrous.

70
Q
A

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