Cardiomyopathy, Myocarditis and Pericarditis Flashcards
Learning Outcomes
What are the different types of cardiomyopathy?
Hypertrophic
Dilated
Restrictive
Myocarditis
What are thedifferent types of pericardial disease?
Pericarditis and effusion (with or without tamponade)
What is dilated cardiomyopathy?
A condition when the heart becomes enlarged and cannot pump blood efficiently, affects other body systems such as the lungs, liver.
Why does dilated cardiomyopathy result in ventricular remodelling?
Left or right systolic pump is impaired leading to progressive heart enlargement via ventricular hypertrophy and ventricular dilation,
What is dilated cardiomyopathy often a cause of?
Congestive heart failure
Thrombosis in chambers is not uncommon
What are the possible genetic aetiological backgrounds for dilated cardiomyopathy?
Mutations in the SCN5A gene (a heart sodium channel gene)
Muscular dystrophy
What are the possible aetiological backgrounds for dilated cardiomyopathy? (besides genetic causes)
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
Which chambers are affected in dilated cardiomyopathy?
Can be one but more often all chambers dilated and functionally impaired
What is the prognosis for dilated cardiomyopathy?
Some causes are specifically sought as they are reversible/part reversible but most are progressive and irreversible
What are the symptoms of dilated cardiomyopathy?
•Progressive, slow onset,
Dyspnoea
Fatigue
Orthopnoea
PND (paroxysmal nocturnal dyspnoea)
Ankle swelling
Weight gain of fluid overload
Cough.
What is a typical past medical history for someone with dilated cardiomyopathy?
Systemic illness
Travel
Hypertension
Vascular disease
Thyroid
Neuromuscular disease
What would you find on examination of someone with dilated cardiomyopathy?
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
What are the relevant investigations for dilated cardiomyopathy?
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
What is general measures in the treatment of dilated cardiomyopathy?
- 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
What are the more specific measures in the treatment of dialted cardiomyopathy?
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
What is restrictive cardiomyopathy?
When the walls of the heart are rigid and the heart is restricted from stretching and filling with blood properly.
What are the non infilatrative forms of restrictive cardiomyopathy?
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)
What are the infiltrative forms of restrictive cardiomyopathy?
Amyloidosis and sarcoidosis
What are the storage disease associated with restrictiva cardiomyopathy?
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)
What are the Endomyocardial conditions asoociated with Restrictive cardiomyopathy?
Fibrosis, carcinoid, radiation, drug effects
Why is the process of relaxation of the ventricular walls described as active rather than passive?
It needs functioning intact myocytes
What are the relevant investigations for restrictive cardiomyopathy?
- 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
What is meant by amyloidosis?
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
What are the general measures for restrictive cardiomyopathy?
- 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
Define hypertrophic cardiomyopathy
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.
What gene is responsible for hypertrophic cardiomyopathy?
Sarcomere gene
What is the inheritance pattern for hypertrophic cardiomyopathy?
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
Where are the possible places that hypertrophy can occur in the heart?
Apical, septal or generalised
Why does cardiac hypertrophy often present in a restrictive manner?
Because there is impaired relaxation
What is the complication associated with septal hypertrophy?
With mitral valve defect can lead to Left ventriculr outflow tract obstruction
LVOT is nearly indistinguishable from the rest of the ventricle
What is the effect of hypertrophic cardiomyopathy on the coronary arteries?
Causes small vessel narrowing and consequent ischaemia and fibrosis, arrhythmias are common
What are the symptoms of hypertrophic cardiomyopathy?
Asymptomatic for many
Fatigue
Dyspnoea
Anginal like chest pain
Exertional pre syncope
Syncope related to arrhythmias
LVOT obstruction
What are the examination findings for hypertrophic cardiomyopathy?
- 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
What is the assessment for hypertrophic cardiomyopathy?
- 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
What are the general measures in the treatment of hypertrophic cardiomyopathy?
- Avoid heavy exercise
- Avoid dehydration
- Explore FH and first degree relatives, ECGs and echoes may be required
- Consider genetic testing
What are the specific measures in the treatment of hypertrophic cardiomyopathy?
- 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
What is myocarditis?
Acute or chronic inflammation of the myocardium
What is restrictive myocarditis often confused for?
Constrictive pericarditis
What is the effect of cardiomyopathy?
Can impair myocardial function, conduction and generate arrhythmia
What are a few of the possible causes of myocarditis?
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
Where are the inflammatory cells in myocarditis?
Infiltration of inflammatory cells into the myocardial layers
What is the result of myocarditis on the electrical function of the heart?
Can result in heart block and arrhythmias
What are the symptoms of myocarditis?
Heart Failure with fatigue
SOB
Chest pain only in a quarter of patients
May not have fever
Signs of Heart Failure
What are the relevant investigations for myocarditis?
- 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
What is the treatment for myocarditis?
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
What is prognosis for myocarditis?
•30% recovery fully but 20% mortality at 1 year and 56% by 4 years. At 11 years those still alive are 93% transplant free.
What is pericarditis?
INflamamtino of the pericardial layers with or without myocardial involvement
What are the most common causes of pericarditis?
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
What are the symptoms of pericardial disease?
Usually 1-2/52 duration, chest pain with pleuritic features and postual features, sitting forward usually improves it lying back makes it worse
Fever
What are the signs of pericardial disease?
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
What are the investigations for pericardial disease?
ECG
ECHO
Troponin may be raised if myocardial involvement too
What are the features of pericardial disease on an ECG?
Diffuse ST segment elevation and PR segment depression
What is the general measures for pericardial disease?
- 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
Define tamponade
Compression of the heart by an accumulation of fluid in the pericardial sac.
What are the causes of pericardial effusion?
•Often same causes as pericarditis
Wat are the symptoms of pericardial effusion where tamponade is present?
Symptoms are overt, fatige, SOB, dizzy with low BP, occasionally chest pain.
Define pulsus paradoxus?
An abnormally large decrease in systolic blood pressure and pulse wave amplitude during inspiration. The normal fall in pressure is less than 10 mmHg.
What are the signs of pericardial disease when tamponade is present?
Pulsus paradoxus
JVP raised
Low blood pressure
Sometimes pericardial rub
Sometimes muffled heart sounds
Pulmonary oedema is very rare
What is the test for pericardial effusion?
Urgent echo
CXR can show large cardia shadow
What is the treatment for pericardial effusion?
Drainage
Persistent effusion needs a surgical pericardial window made to allow flow to abdomen
ECG of tamponade
ECHO with large effusion
What are the causes of constrictive pericarditis?
Causes are idopathic, radiation, post surgery, autoimmune, renal failure, sarcoid.
What is the pathology of constricitve pericarditis?
Pathology is that of impaired filling although myocardium is normal most of the time
What are the symptoms and signs of Constricitve pericarditis
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
What is the investigation for Constricitve pericarditis?
Assess with echo and right heart cath to differentiate from restrictive cardiomyopathy which can be very difficult
What is treatment for Constricitve pericarditis?
Treatment is with careful and limited diuretics and pericardectomy
What is pericardectomy?
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.
