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CVS Pathology > Cardiomyopathy, Myocarditis and Pericarditis > Flashcards

Cardiomyopathy, Myocarditis and Pericarditis Flashcards

1
Q

What is dilated cardiomyopathy? What is hypertrophic obstructive cardiomyopathy?

A

DCM - A structural and functional description, where the ventricular function is impaired
Dilated heart leads to predominately systolic dysfunction - all 4 chambers dilated but the left ventricle is more than the right. Eccentric hypertrophy is seen

HOCM - AD disorder of the muscle tissue caused by defects in the genes coding for contractile proteins

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2
Q

Causes of dilated cardiomyopathy

A

Genetic and familial DCM; SCN5A gene, muscular dystrophy
Inflammatory
Infections (COXSACKIE B)
Autoimmune
HTN
Post partum
Drugs, exogenous chemicals, endocrine, ALCOHOL
Injury, cell loss, scar replacement (IHD)
Tropical disease

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3
Q

Types of cardiomyopathy

A

Dilated (DCM)
Restrictive and infiltrative cardiomyopathy
Hypertrophic obstructive cardiomyopathy

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4
Q

Possible pathology of restrictive and infiltrative cardiomyopathy

A

Filling and myocytes relaxation capacity dysfunction

Systolic function may or may not be impaired

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5
Q

Causes of restrictive and infiltrative cardiomyopathy

A 
Familial
Forms of HCM 
Scleroderma
DM
Psudoxanthoma elasticum 
AMYLOIDOSIS
Sarcoidosis 
Haemachromatosis 
Fabry disease
Endomyocardial fibrosis, carcinoid, radiation, drug effects 
POST RADIOTHERAPY
50% unknown
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6
Q

Pathology of hypertrophic cardiomyopathy

A

Morphological description
PREDOMINATELY DIASTOLIC DYSFUNCTION
LVH -> Decreased compliance -> decreased cardiac output
Myocyte hypertrophy and disarray
Impaired relaxation - restrictive
Systolic function usually adequate
If septal hypertrophy this can with mitral valve defect lead to LVOT obstruction
Arrhythmias common due to coronary arteries being affected

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7
Q

Types of hypertrophic cardiomyopathy

A

Apical
Septal
Generalised

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8
Q

Definition of pericardium

A

Reflected lining over the epicardium (visceral pericardium) and the parietal pericardium that is the inner portion of the exterior sac around the heart and the proximal great vessels

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9
Q

What is pericarditis?

A

Inflammation of the pericardial layers with or without myocardial involvement

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10
Q

Causes of pericarditis

A 
BACTERIAL
POST MI 
PERFORATION
DISSECTION OF PROXIMAL AORTA
Idiopathic
Viral
Fungal 
Connective tissue disease 
Arteritis
IBD 
Drug induced
Neoplastic disease
Haemopericardium (trauma, post MI rupture, iatrogenic)
Trauma
Congenital cysts or congenital absence 
Hypo/hyper thyroidism 
Amyloidosis 
Aortic dissection
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11
Q

What is the genetics of hypertrophic cardiomyopathy?

A

Sacromere gene defect

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12
Q

Inheritance of hypertrophic cardiomyopathy

A

Autosomal dominant - incomplete penetrance

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13
Q

Presentation of dilated cardiomyopathy

A 
CLASSICAL FINDINGS OF HF
Progressive, slow onset
SOB
Fatigue
Orthopnoea
PND
Ankle swelling
Weight gain (due to fluid overload)
Cough
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14
Q

What is orthopnoea?

A

SOB when lying flat

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15
Q

PMH related to dilated cardiomyopathy

A 
Systemic illness
Travel 
HTN
Vascular disease
Neuromuscular disease
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16
Q

Signs of dilated cardiomyopathy

A 
Poor superficial perfusion 
Thready pulse, irregular if in AF
SOB at rest
Narrow pulse pressure
JVP elevated +/- TR waves
Displaced apex 
S3 + S4
Systolic murmur 
MR murmur often (possibly TR as well)
Pulmonary oedema
Pleural effusions
Ankle + sacral oedema
Ascites
Hepatomegaly
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17
Q

What is ascites?

A

Fluid in abdominal cavity

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18
Q

Investigations of dilated cardiomyopathy

A 
Repeated ECG noting if LBBB is present
CXR - BALLOOON APPEARANCE 
N terminal pro BNP
FBC
U and Es
ECHO
CMRI
Coronary angiogram 
Sometimes biopsy depending on time course
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19
Q

Investigation of restrictive and infiltrative cardiomyopathy

A

Repeat ECG noting if LBBB is present or other conduction defects
CXR
N terminal pro BNP
FBC
U and Es
Autoantibodies for sclerotic CT diseases
Amyloid needs non cardiac biopsy to help establish diagnosis
Fabry; low plasma alpha galactosidase A activity
ECHO
CMRI
Biopsy

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20
Q

Presentation of hypertrophic obstructive cardiomyopathy

A 
Asymptomatic for many 
Fatigue 
Exertional SOB
Palpitations
Anginal like chest pain 
Exertional pre syncope 
Syncope related to arrthymias or LVOT obstruction 
- typically following exercise 
- from functional aortic stenosis 
Sudden cardiac death
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21
Q

Signs of hypertrophic cardiomyopathy

A

Can be none
Notched pulse pattern / jerky pulse
Large ‘a’ waves
Irregular pulse if in AF or ectopy
Double impulse over apex, thrills and murmurs, often dynamic, LVOT,
Ejection systolic murmur
- murmur will increase with Valsalva
- decrease with squatting
JVP can be raised in very restrictive filling
May impair mitral valve closing and therefore MVP

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22
Q

Investigations for hypertrophic cardiomyopathy

A 
ECG - often abnormal 
- LVH
- non specific ST and T wave abnormalities
- deep Q waves 
- AF occasionally seen 
ECHO (MR SAM ASH)
- MR
- Systolic anterior motion of anterior mitral valve leaflet 
- asymmetrical septal hypertrophy (ASH)
CMRI
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23
Q

What is myocarditis?

A

Acute or chronic inflammation of the myocardium

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24
Q

What can myocarditis be in association with?

A

Pericarditis

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25
Q

What can be the results of myocarditis?

A

Impair myocardial function
Impair myocardial conduction
Generate arrythmia

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26
Q

Most common cause of myocarditis

A

Viral

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27
Q

Pathology of myocarditis

A

Infiltration of inflammatory cells into the myocardial layers, reduced function and heart failure, heart block as conduction system is involved, and arrhythmias

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28
Q

Presentation of myocarditis

A

Heart failure with fatigue, SOB, CP in only 26%
Short course
Possible fever
Signs of HF

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29
Q

Investigations of myocarditis

A 
ECG abnormal 
Biomarkers - often elevated but not falling in  a pattern consistent with MI
ECHO
CMRI (oedema)
Biopsy (low threshold)
Autoantibodies 
Viral DNA PCR
Strep antibodies 
Lyme B burgdorferi 
HIV
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30
Q

Examples of pericardial disease

A

Pericarditis
Pericardial effusion
Constrictive pericarditis

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31
Q

Symptoms of pericarditis

A 
1-2 weeks 
Chest pain with pleuritic and postural features
- Sitting forward improves pain 
- Lying back makes pain worse
Fever
32
Q

Signs of pericarditis

A 
Fever
Pericardial rub LSE
JVP 
Low BP
Muffled heart sounds and raised JVP
33
Q

What would muffled heart sounds and raised JVP indicate?

A

Pericarditis + effusion

34
Q

What would high fever and very unwell despite no effusion suggest?

A

Bacterial

35
Q

Investigations of pericarditis

A

ECG
ECHO
Troponin (elevated if myocardial involvement)

36
Q

What would be seen on ECG in pericarditis?

A

Widespread ST changes - Saddle shaped ST elevation

PR depression of pericarditis

37
Q

Presentation of pericardial effusion

A 
Overt
Fatigue
SOB
Dizzy with low BP
Occasionally chest pain
38
Q

Signs of pericardial effusion

A 
Overt
Pulsus paradoxus
JVP raised
Low BP 
\+/- rub 
\+/- heart sounds
39
Q

What is very rare in pericardial effusion/tamponade?

A

Pulmonary oedema

40
Q

Investigations for pericardial effusion

A

Urgent ECHO
CXR (Large cardiac shadow)
ECG

41
Q

How common is constrictive pericarditis?

A

Rare

42
Q

Causes of constrictive pericarditis

A 
Idiopathic
Radiation 
Post surgery 
Autoimmune
Renal failure
Sarcoid
43
Q

What is the usual pathology of constrictive pericarditis?

A

Impaired filling although myocardium is normal most of the time

44
Q

Presentation of constrictive pericarditis

A 
Fatigue
SOB
Cough
Right heart failure with oedema
Ascites
High JVP
Jaundice
Hepatomegaly 
AF
TR
Pleural effusion 
Pericardial knock
45
Q

Investigations of constrictive pericarditis

A

ECHO

Right heart catheter

46
Q

Treatment of dilated cardiomyopathy

A 
Correct anaemia
Remove exacerbating drugs e.g. NSAIDs
Correct endocrine disturbance
Advise on fluid and salt intake, reduce it 
Advise on managing weight to identify fluid overload 
HF nurse referral
ACEIs, ATII blockers, diuretics
BBs
Spironolactone
Anticoagulants as required 
SCD risk assessment with ICD or CRT-D/P implant
Cardiac transplant
47
Q

Prognosis of dilated cardiomyopathy

A

Generall poor

48
Q

Treatment of restrictive and infiltrative cardiomyopathy

A

General measures
Limited diuretic use (Low filling pressures will cause problems)
BBs limited ACEI use
Anticoagulants as required
SCD risk assessment with ICD or CRT-D/P implant
Cardiac transplant

49
Q

Prognosis of restrictive and infiltrative cardiomyopathy

A

Unless reversible then poor prognosis

50
Q

Treatment of hypertrophic cardiomyopathy

A

General measures
- avoid heavy exercise
- avoid dehydration
- explore FH, ECGs and ECHOs may be required
- consider genetic testing
ABCDE
- A - amiodarone
- B - Beta blockers or verapramil for symptoms
- C - Cardioverter defibrillator
- D - Dual chamber pacemaker
- E - Endocarditis prophylaxis (possibly not)
AF - anticoagulated
Obstructive form; surgical or alcohol septal ablation

51
Q

Treatment of myocarditis

A

General

  • supportive Tx of HF and support for brady and tachy arrythmias
  • immunotherapy if biopsy or indications to specific diagnosis
  • Stop possible drugs or toxic agent exposure
52
Q

Prognosis of myocarditis

A

30% recover fully
20% mortality at 1 year
56% mortality by 4 years

53
Q

Treatment of pericarditis

A 
Viral conservative
Idiopathic
- colchicine and limited use of NSAIDs
Bacterial
- drained even if small effusion 
- antimicrobials
54
Q

Treatment of pericardial effusion

A

Drainage

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

55
Q

What cause of pericarditis has a high death rate?

A

Bacterial

56
Q

Treatment of constrictive pericarditis

A

Careful and limited diuretics

Pericardectomy

57
Q

What is the leading cause of sudden death in young athletes?

A

Hypertrophic obstructive cardiomyopathy

58
Q

Usual mutation causing hypertrophic obstructive cardiomyopathy

A

Mutation in gene encoding B-myosin heavy chain protein

59
Q

When does peripartum cardiomyopathy occur?

A

Between last month of pregnancy to 5 months post partum

60
Q

Who is peripartum cardiomyopathy more common in?

A

Older women
Greater parity
Multiple gestations

61
Q

How many patients with DCM have a genetic predisposition?

A

1/3rd

62
Q

Associations of hypertrophic cardiomyopathy

A

Freidrechs ataxia

WPW

63
Q

Drugs to avoid in hypertrophic obstructive cardiomyopathy

A

Nitrates
ACEIs
Inotropes

64
Q

What sign is seen in constrictive pericarditis?

A

Kussmauls sign

Raised JVP that does NOT fall with inspiration / increases with inspiration

65
Q

Common cause of constrictive pericarditis

A

Recent cardiac surgery

66
Q

What may HOCM present with?

A

Exertional dyspnoea

67
Q

What is HOCM associated with in the young and why?

A

Sudden death in young atheletes

Due to ventricular arrhythmia on extreme exertion

68
Q

What pulse may HOCM be associated with?

A

Bisferiens pulse (double pulse) - occurs due to subaortic stenosis as a result of HOCM

69
Q

Inheritance of HOCM

A

Autosomal dominant

70
Q

ECG features of HOCM

A

LVH

  • deep ST depression
  • T wave inversion
71
Q

What ECHO findings support HOCM?

A

Asymmetric septal hypertrophy

Systolic anterior movement of the anterior leaflet of the mitral valve or cMR

72
Q

Treatment of pericarditis

A

Combination of NSAID and colcheine

73
Q

Management of HOCM

A

An implantable cardiac defibrillator can be inserted to reduce the risk of sudden death
Amoidarone
BBs
Dual chamber pacemaker

74
Q

Drugs to avoid in HOCM

A

Nitrates
ACEIs
Inotropes

75
Q

Who is at risk of dilated cardiomyopathy?

A

Alcoholics

CVS Pathology (19 decks)

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