Cardiomyopathy, Myocarditis and Pericarditis

Question 1

Causes of dilated cardiomyopathy

Answer 1

Ischaemic and valvular causes
Genetic and familial: SCN5A gene, muscle dystrophy
Inflammatory, infections, autoimmune, postpartum
Injury, cell loss, scar replacement
Toxic, drugs, exogenous chemicals, endocrine
Irreversible specific causes:
- Alcohol
- Endocrine
- Tropical disease
- Post partum
- Haemaochromatosis (build up of iron)
- Sarcoid

Question 2

Types of cardiomyopathy

Answer 2

Dilated Cardiomyopathy
Restrictive and Infiltrative cardiomyopathy
Hypertrophic cardiomyopathy

Question 3

Pathophysiology of DCM

Answer 3

Ventricular function is impaired

Question 4

Pathophysiology of RCM+ICM

Answer 4

Systolic function may or may not be impaired

Ventricle has reduced compliance and so inability to fill the ventricle well

Question 5

Types of ICM and RCM

Answer 5

• Non-infiltrative; familial, forms of HCM, scleroderma, diabetic, pseudoxanthoma, elasticum
• Infiltrative; amyloid, sarcoid
• Storage diseases: Haemochromataosis, fabry disease
• Endomyocardial; fibrosis, carcinoid, radiation, drug effects

Question 6

Pathology of HCM

Answer 6

Myocyte hypertrophy and disarray
Can be generalised or segmental wall thickness
Can be apical, septal or generalised
Impaired relaxation and so behaves in a restrictive manner
If septal hypertrophy this can with mitral valve defect lead to LVOT obstruction
Coronary arteries also affected - small vessel narrowing and consequent ischemia and fibrosis.
Arrhythmias are common

Question 7

Key causes of pericarditis

Answer 7

Bacterial
Post MI
Dissection of proximal aorta
Neoplasia

Question 8

Symptoms of dilated cardiomyopathy

Answer 8

Progressive, slow onset
Dyspnoea and PND
Fatigue
Orthopnoea
Ankle swelling
Weight gain of fluid overload
Cough

Question 9

Signs of DCM

Answer 9

Poor superficial perfusion
Thready pulse, irregular in AF
SOB at rest
Narrow pulse pressure
Displaced apex
S3 + S4
MR murmur often
Pulmonary oedema
Pleural effusions
Ankle oedema
Sacral oedema
Ascites
Hepatomegaly

Question 10

Investigations for DCM

Answer 10

Repeated ECG noting if LBBB is present
CXR
N terminal pro BNP
Basic bloods: FBC, U and Es
ECHO
CMRI
Coronary angiogram
Sometimes biopsy depending on the course of cardiomyopathy

Question 11

Investigations of RCM and ICM

Answer 11

Repeated ECG noting LBBB if present and other conduction defects
CXR
N terminal pro BNP
Basic bloods: FBC, U and Es, be on the lookout for sarcoid and haemachromatosis
Auto antibodies for sclerotic CT diseases
Amyloid needs non-cardiac biopsy to help establish the diagnosis
ECHO
Fabry; low plasma alpha galactosidae A activity
CMRI

Question 12

Symptoms of HCM

Answer 12

Asymptomatic for many
Fatigue
Dyspnoea
Palpitations
Anginal like chest pain
Exertional pre syncope
Syncope related to arrhythmias or LVOT obstruction
SCD

Question 13

Signs of HCM

Answer 13

Can be none
Notched pulse pattern
Irregular pulse if in AF or ectopy
Double impulse over apex, thrills and murmurs, often dynamic, LVOT, murmur will increase
JVP can be raised in very restrictive filling

Question 14

Investigations for HCM

Answer 14

ECG: often abnormal but can be normal if phenotype is poorly expressed in genotype +ve individuals
ECHO
CMRI
Risk stratification for SCD, may need ICD

Question 15

Pathology of myocarditis

Answer 15

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

Question 16

Symptoms of myocarditis

Answer 16

Heart failure with SOB, CP in only 26%
Shorter course of a few weeks
May not have fever
Signs of HF

Question 17

Investigations for myocarditis

Answer 17

ECG usually abnormal
Biomarkers often elevated but not falling into 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

Question 18

Types of pericardial disease

Answer 18

Pericarditis
Pericardial effusion
Constrictive pericarditis

Question 19

Symptoms of pericarditis

Answer 19

Fever
Usually 1-2/52 duration
Chest pain with pleuritic and postual features
Sitting forward usually improves the chest pain and lying back makes it worse

Question 20

Signs of pericarditis

Answer 20

Fever
Pericardial rub LSE
Look for JVP as if effusion is present or substantial haemodynamically relevant then It will be raised
Low BP
Muffled heart sounds and raised JVP should make you think effusion as well as pericarditis
High fever and very unwell despite no effusion may suggest bacterial

Question 21

Investigations for pericarditis

Answer 21

ECG: widespread ST changes and PR depression of pericarditis
ECHO
Troponin may be raised if myocardial involvement too

Question 22

Symptoms of pericardial effusion

Answer 22

Fatigue
SOB
Dizzy with low BP
Occasionally chest pain

Question 23

Signs of pericardial effusion

Answer 23

Pulsus paradoxus
JVP raised
Low BP
\+/- rub
\+/- muffled heart sounds
Pulmonary oedema very rare in pericardial effusions/tamponade

Question 24

Investigations of pericardial effusion

Answer 24

Urgent ECHO
CXR can show large cardiac shadow
ECG: with electrical alternans if large effusion

Question 25

Causes of constrictive pericarditis

Answer 25

Idiopathic
Radiation
Post-Surgery
Autoimmune
Renal failure
Sarcoid

Question 26

Symptoms of constrictive pericarditis

Answer 26

Fatigue
SOB
Cough

Question 27

Signs of constrictive pericarditis

Answer 27

More of right heart failure with oedema
Ascites
High JVP
Jaundice
Hepatomegaly
AF
TR
Pleural effusion
Pericardial knock

Question 28

Investigations for constrictive pericarditis

Answer 28

ECHO

Right heart catheter: to differentiate from restrictive cardiomyopathy which can be very difficult

Question 29

Treatment for DCM

Answer 29

GENERAL MEASURES:
Correct anaemia
Remove exacerbating drugs e.g. NSAIDs
Correct any endocrine disturbance
Advise on reduction of fluid and salt intake
Advise on managing weight to identify fluid overload. 
HF nurse referral 
SPECIFIC MEASURES:
ACEI, ATII blockers
Diuretics
Spironolactone
Anticoagulants as required
SCD risk assessment with ICD or CRT-D/P implant
Cardiac transplant

Question 30

RCM + ICM treatments

Answer 30

GENERAL MEASURES:
MORE SPECIFIC MEASURES
limited diuretic use as low filling pressures will cause problems
Beta blockers, limited ACEI use
Anticoagulants as required
SCD assessment with ICD or CRT-D/P implant
Cardiac transplant

Question 31

HCM treatments

Answer 31

GENERAL MEASURES
Avoid heavy exercise
Avoid dehydration
Explore FH and 1st degree relatives, ECGs and ECHOs may be required
Consider genetic testing
Regular FU to reappraise the risks and progressive
SPECIFIC MEASURES
If in AF: anticoagulate
Drugs to try and reenhance relaxation, variable results but often if symptomatic, beta blockers, verapramil, disopryimide
Obstructive forms; surgical or alcohol septal ablation
ICD if required based on risk stratification

Question 32

Myocarditis treatment

Answer 32

GENERAL MEASURES
Supportive treatment of heart failure and support for brady and tachy arrythmias
Immunotherapy if biopsy or other Ix point to a specific diagnosis
Stop possible drugs or toxic agent exposure

Question 33

Pericarditis treatment

Answer 33

GENERAL MEASURES
Viral is conservative
Idiopathic gets colchine 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, drain

Question 34

Pericardial effusion treatment

Answer 34

Drainage is the destination treatment
Send for MCS, neoplastic cells, protein and LDH, most are exudates
Persistent effusion needs a surgical pericardial window made to allow flow to the abdomen

Question 35

Constrictive pericarditis treatment

Answer 35

Careful and limited diuretics and pericardetomy