Cardiomyopathy, Myocarditis and Pericarditis Flashcards
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
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
Types of cardiomyopathy
Dilated (DCM)
Restrictive and infiltrative cardiomyopathy
Hypertrophic obstructive cardiomyopathy
Possible pathology of restrictive and infiltrative cardiomyopathy
Filling and myocytes relaxation capacity dysfunction
Systolic function may or may not be impaired
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
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
Types of hypertrophic cardiomyopathy
Apical
Septal
Generalised
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
What is pericarditis?
Inflammation of the pericardial layers with or without myocardial involvement
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
What is the genetics of hypertrophic cardiomyopathy?
Sacromere gene defect
Inheritance of hypertrophic cardiomyopathy
Autosomal dominant - incomplete penetrance
Presentation of dilated cardiomyopathy
CLASSICAL FINDINGS OF HF Progressive, slow onset SOB Fatigue Orthopnoea PND Ankle swelling Weight gain (due to fluid overload) Cough
What is orthopnoea?
SOB when lying flat
PMH related to dilated cardiomyopathy
Systemic illness Travel HTN Vascular disease Neuromuscular disease
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
What is ascites?
Fluid in abdominal cavity
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
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
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
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
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
What is myocarditis?
Acute or chronic inflammation of the myocardium
What can myocarditis be in association with?
Pericarditis
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
