Cardiomyopathy, Myocarditis and pericarditis

Question 1

dilated cardiomyopathy

Answer 1

heart condition where the ventricular function is impaired

- Can be a primary problem or the end result of almost any pathological insult to the myocardium

Question 2

Causes of dilated cardiomyopathy?

Answer 2

ischaemia

valvular

genetic + familial - muscular dystrophy

inflammatory, infectious, autoimmune, postpartum

toxic:- drugs, exogenous chemicals, endocrine

injury, cell loss, scar replacement

Question 3

which gene commonly causes cardio myopathy if altered

Answer 3

SCN5A gene

Question 4

which chambers of the heart can dilated myocardiopathy affect/

Answer 4

Both L and R ventricles mainly but can also affect atria

Can be one chamber but most often all chambers become dilated and functionally impaired

So systolic and diastolic dysfunction can occur

thrombosis in chambers is not uncommon

Question 5

when is flow slower through a ventricle?

Answer 5

when it is dysfunctional/ not contracting properly

Question 6

Reversible causes of dilated cardiomyopathy (6)

Answer 6

Alcohol
endocrine
tropical disease
post partum - after having a baby - often fixes itself
haemaochromatosis sarcoid

Question 7

is DCM common?

Answer 7

no

incidence = 5-8 per 100000

Question 8

symptoms of DCM

Answer 8

Progressive
slow onset
dyspnoea
fatigue
orthopnoea
PND - paroxysmal nocturna dyspnoea
ankle swelling
weight gain of fluid overload
cough

Question 9

what are you likely to find on examination of a patient with DCM? (10)

Answer 9

Poor superficial perfusion

thready pulse, irreg if in AF

SOB at rest

narrow pulse pressure
JVP elevated+/- TR waves

displaced apex

S3 and S4

MR murmur often

pulmonary, sacral and ankle oedema pleural effusions

acites - protein containing fluid in abdomen

hepatomegally

Question 10

Basic investigations to carry out for DCM diagnosis? (7)

Answer 10

ECG - almost always abnormal

CXR - fluid overload, pleural effusion

ECHO - simplest, least invasive

basic bloods - FBC

CMRI - best imaging modality - show scar burden in myocardium and maybe see thrombus

coronary angiogram

sometimes a biopsy depending if caught at early stage or not

Question 11

general treatment of DCM

Answer 11

Correct anaemia

Remove exacerbating drugs eg NSAIDs - these retain salt/water so not good if already have fluid overload

Correct any endocrine disturbance
Advise on fluid and salt intake, reduce it
Advise on managing weight to identify fluid overload
HF nurse referral

Question 12

specific treatment of DCM

Answer 12

• ACE Inhibitors, - Angiotensin II blockers - diuretics
• Beta blockers
  Spironolactone
• Anticoagulants as required
• Sudden Cardiac Death risk assessment with ICD or CRT-D/P implant
• Cardiac transplant – last resort, if everything else fails

Question 13

prognosis of DCM? which cause of DCM shows worst outcome

Answer 13

generally poor and often influenced by the causes

Cardiomyopathy due to HIV

Question 14

restrictive and infiltrative cardiomyopathy - what is it? prevalence?

Answer 14

not a high prevalence - cardiologist might see 1 a year?

heart is restricted - has to stretch to accommodate blood and increased venous return - ventricle can’t fill well due to reduced compliance

impairment of diastolic function

about 50% related to specific clinical conditions, the rest remain unknown

Question 15

2 causes of infiltrative cardiomyopathy?

Answer 15

sarcoid - inflammation of organs

amyloid - protein collection in tissues/organs

Question 16

storage diseases that can cause restrictive and infiltrative cardiomyopathy

Answer 16

haemachromatosis

Fabry’s disease - glycogen storage disease

Question 17

endomyocardial causes of restrictive and infiltrative cardiomyopathy?

Answer 17

Fibrosis, carcinoid, radiation, drug effects

Question 18

is there increased or reduced compliance in Restrictive and infiltrative cardiomyopathy?

Answer 18

reduced

Question 19

is relaxation of the ventricular wall an active or passive process?

Answer 19

active

requires functioning intact myocytes

Question 20

investigations carried out for restrictive and infiltrative cardiomyopathy (10)

Answer 20

repeated ECG - note if there is Left bundle branch block (LBBB) -cardiac conduction abnormality seen on ECG. In this condition, activation of the left ventricle of the heart is delayed, which causes the left ventricle to contract later than the right ventricle.

CXR

BNP - increased in heart failure

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

Question 21

what are some specific measures put in place for restrictive and infiltrative cardiomyopathy

Answer 21

Limited diuretic use as low filling pressures will cause problems

Beta blockers limited ACE Inhibitor use

Anticoagulants as required

Sudden Cardiac Death risk assessment with Implantable cardiac defibrillator or Cardiac resynchronization therapy pacemaker implant

Cardiac transplant

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

Endomyocardial fibrosis has little specific treatment

Question 22

hypertrophic cardiomyopathy:- what is it? prevalence? genetic basis?

Answer 22

thickened heart muscle

impaired relaxation is a common feature. Ventricles can’t hold much blood, and the walls can’t relax properly and may stiffen. Behaves in restrictive manner

relatively high prevalence 1:500

over 1500 genes now identified - sarcomere gene defect

autosomal dominant but gene can be expressed differently and not known how until time passes

Question 23

what parts of the heart thicken in hypertophic cardiomyopathy?

Answer 23

doesn’t have to be whole heart, can be part of it

Can be apical, septal or generalised

Question 24

if septal hypertrophy what can an additional defect in the mitral valve lead to?

Answer 24

Left ventricular outflow tract obstruction

Question 25

common further heart/vessel problems associated with hypertrophic cardiomyopathy

Answer 25

Coronary arteries also affected with small vessel narrowing and consequent ischaemia and fibrosis arrhythmias are common

Question 26

symptoms of hypertrophic cardiomyopathy

Answer 26

Breathless, palpitations, syncope, exertional symptoms, Sudden Cardiac Death ``` Asymptomatic for many fatigue dyspnoea anginal like chest pain exertional pre syncope syncope related to arrhythmias or LVOT obstruction ```

Question 27

examination findings:- hypertrophic cardiomyopathy

Answer 27

Can be none! 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

Question 28

investigations/tests for hypertrophic cardiomyopathy?

Answer 28

ECG - often abnormal but a few are normal where phenotype is poorly expressed in genotype +ve individuals Echo - see thickness in muscle and dynamic effects of blood flow CMRI ``` Risk stratification for Sudden Cardiac Death, may need ICD Holters repeatedly (monitors heart rate) ```

Question 29

general measures suggested for hypertrophic cardiomyopathy

Answer 29

Avoid heavy exercise Avoid dehydration Explore FH and first degree relatives, ECGs and echoes may be required Consider genetic testing Regular follow up to re appraise the risks and progress

Question 30

specific measures suggested for hypertrophic cardiomyopathy

Answer 30

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

Question 31

myocarditis:- what is it? acute/chronic? prevalence?

Answer 31

can be acute or chronic inflammation of the myocardium canimpair myocardial function, conduction and generate arrhythmia 8-10 100000 most common is viral

Question 32

common viruses causing myocarditis?

Answer 32

``` adenovirus parvovirus B19 influenza hep C epstein barr virus ```

Question 33

pathology of myocarditis?

Answer 33

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

Question 34

symptoms of myocarditis

Answer 34

Heart failure with fatigue, SOB, Chest Pain in only 26% Shorter course of a few weeks May not have fever Signs of Heart Failure

Question 35

Expected investigation/test outcomes for myocarditis?

Answer 35

ECG usually abnormal Biomarkers often elevated but not falling in a pattern consistent with MI Echo, can get RWMA (regional wall motion abnormalities - LV wall motion abnormalities usually) CMRI can see oedema in certain images Low threshold for biopsy ``` Viral DNA PCR Auto antibodies Step antibodies Lyme B burgdorferi HIV ```

Question 36

general measures/management for myocarditis

Answer 36

Supportive with treatment of heart failure and support for brady and tachy arrhythmias Immunotherpay if biopsy or other Ix point to a specific diagnosis Stop possible drugs or toxic agent exposure 20% mortality at 1 year and 56% by 4 years.

Question 37

pericarditis

Answer 37

Inflammation of the pericardial layers with or without myocardial involvement

Question 38

causes of pericarditis (10)

Answer 38

``` idiopathic viral - infectious bacterial post MI perforation dissection of proximal aorta neoplasia - presence or formation of new, abnormal growth of tissue immune-inflammatory trauma congenital ```

Question 39

symptoms of pericardial disease

Answer 39

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

Question 40

signs of pericardial disease

Answer 40

Raised temp pericardial rub Left Sternal Edge look for JVP as if an effusion is present and substantial or haemodynamically relevant then it will be raised low BP muffled HS and raised JVP should make you consider not just pericarditis but effusion High fever and very unwell despite no effusion may suggest bacterial

Question 41

investigations for pericardial disease

Answer 41

ECG and echo, troponin (protein in muscle fibres) may be raised if myocardial involvement too (myocardial injury)

Question 42

general measures/management for pericardial disease:- viral, idiopathic and bacterial

Answer 42

Viral is conservative - non invasive treatment 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

Question 43

what signs and symptoms would a patient present with if tamponade was present

Answer 43

signs:- raised JVP, low BP, +/- rub or muffled HS. Large drop in stroke vol, systolic BP and pulse wave amplitude during inspiration (pulsus paradoxus) symptoms:- fatige, SOB, dizzy with low BP, occasionally chest pain.

Question 44

tests/investigations:- tamponade

Answer 44

urgent echo CXR - show large cardiac shadow drainage - treatment mechanical circulatory support

Question 45

what does persistant effusion need

Answer 45

a surgical pericardial window made to allow flow to abdomen

Question 46

constrictive pericarditis

Answer 46

rare, impaired filling although myocardium is normal most of the time causes: - idiopathic, radiation, post-op, autoimmune, renal failure, sarcoid symptoms: - fatigue, SOB, cough signs: - more of RHF with oedema, ascites, high JVP, jaundice, hapetomegally, AF, TR, pleural effusion investigations: - echo, RH catheter to differentiate from restrictive cardiomyopathy - v difficult treatment: - careful and limited diuretics and pericardectomy