Cardiomyopathy, Myocarditis and Pericarditis Flashcards by Annie Caldwell

What is dilated cardiomyopathy? What is hypertrophic obstructive cardiomyopathy?

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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Causes of dilated cardiomyopathy

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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Types of cardiomyopathy

Dilated (DCM)
Restrictive and infiltrative cardiomyopathy
Hypertrophic obstructive cardiomyopathy

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Possible pathology of restrictive and infiltrative cardiomyopathy

Filling and myocytes relaxation capacity dysfunction

Systolic function may or may not be impaired

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Causes of restrictive and infiltrative cardiomyopathy

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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Pathology of hypertrophic cardiomyopathy

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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Types of hypertrophic cardiomyopathy

Apical
Septal
Generalised

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Definition of pericardium

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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What is pericarditis?

Inflammation of the pericardial layers with or without myocardial involvement

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Causes of pericarditis

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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What is the genetics of hypertrophic cardiomyopathy?

Sacromere gene defect

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Inheritance of hypertrophic cardiomyopathy

Autosomal dominant - incomplete penetrance

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Presentation of dilated cardiomyopathy

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

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What is orthopnoea?

SOB when lying flat

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PMH related to dilated cardiomyopathy

Systemic illness
Travel 
HTN
Vascular disease
Neuromuscular disease

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Signs of dilated cardiomyopathy

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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What is ascites?

Fluid in abdominal cavity

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Investigations of dilated cardiomyopathy

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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Investigation of restrictive and infiltrative cardiomyopathy

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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Presentation of hypertrophic obstructive cardiomyopathy

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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Signs of hypertrophic cardiomyopathy

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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Investigations for hypertrophic cardiomyopathy

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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What is myocarditis?

Acute or chronic inflammation of the myocardium

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What can myocarditis be in association with?

Pericarditis

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What can be the results of myocarditis?

Impair myocardial function Impair myocardial conduction Generate arrythmia

Most common cause of myocarditis

Viral

Pathology of myocarditis

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

Presentation of myocarditis

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

Investigations of myocarditis

``` 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 ```

Examples of pericardial disease

Pericarditis Pericardial effusion Constrictive pericarditis

Symptoms of pericarditis

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

Signs of pericarditis

``` Fever Pericardial rub LSE JVP Low BP Muffled heart sounds and raised JVP ```

What would muffled heart sounds and raised JVP indicate?

Pericarditis + effusion

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

Bacterial

Investigations of pericarditis

ECG ECHO Troponin (elevated if myocardial involvement)

What would be seen on ECG in pericarditis?

Widespread ST changes - Saddle shaped ST elevation | PR depression of pericarditis

Presentation of pericardial effusion

``` Overt Fatigue SOB Dizzy with low BP Occasionally chest pain ```

Signs of pericardial effusion

``` Overt Pulsus paradoxus JVP raised Low BP +/- rub +/- heart sounds ```

What is very rare in pericardial effusion/tamponade?

Pulmonary oedema

Investigations for pericardial effusion

Urgent ECHO CXR (Large cardiac shadow) ECG

How common is constrictive pericarditis?

Rare

Causes of constrictive pericarditis

``` Idiopathic Radiation Post surgery Autoimmune Renal failure Sarcoid ```

What is the usual pathology of constrictive pericarditis?

Impaired filling although myocardium is normal most of the time

Presentation of constrictive pericarditis

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

Investigations of constrictive pericarditis

ECHO | Right heart catheter

Treatment of dilated cardiomyopathy

``` 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 ```

Prognosis of dilated cardiomyopathy

Generall poor

Treatment of restrictive and infiltrative cardiomyopathy

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

Prognosis of restrictive and infiltrative cardiomyopathy

Unless reversible then poor prognosis

Treatment of hypertrophic cardiomyopathy

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

Treatment of myocarditis

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

Prognosis of myocarditis

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

Treatment of pericarditis

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

Treatment of pericardial effusion

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

What cause of pericarditis has a high death rate?

Bacterial

Treatment of constrictive pericarditis

Careful and limited diuretics | Pericardectomy

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

Hypertrophic obstructive cardiomyopathy

Usual mutation causing hypertrophic obstructive cardiomyopathy

Mutation in gene encoding B-myosin heavy chain protein

When does peripartum cardiomyopathy occur?

Between last month of pregnancy to 5 months post partum

Who is peripartum cardiomyopathy more common in?

Older women Greater parity Multiple gestations

How many patients with DCM have a genetic predisposition?

1/3rd

Associations of hypertrophic cardiomyopathy

Freidrechs ataxia | WPW

Drugs to avoid in hypertrophic obstructive cardiomyopathy

Nitrates ACEIs Inotropes

What sign is seen in constrictive pericarditis?

Kussmauls sign | Raised JVP that does NOT fall with inspiration / increases with inspiration

Common cause of constrictive pericarditis

Recent cardiac surgery

What may HOCM present with?

Exertional dyspnoea

What is HOCM associated with in the young and why?

Sudden death in young atheletes | Due to ventricular arrhythmia on extreme exertion

What pulse may HOCM be associated with?

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

Inheritance of HOCM

Autosomal dominant

ECG features of HOCM

LVH - deep ST depression - T wave inversion

What ECHO findings support HOCM?

Asymmetric septal hypertrophy | Systolic anterior movement of the anterior leaflet of the mitral valve or cMR

Treatment of pericarditis

Combination of NSAID and colcheine

Management of HOCM

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

Drugs to avoid in HOCM

Nitrates ACEIs Inotropes

Who is at risk of dilated cardiomyopathy?

Alcoholics

Cardiomyopathy, Myocarditis and Pericarditis Flashcards

(75 cards)