Cardiomyopathy- hypertrophic, dilated, restrictive and myocarditis, pericardial disease, pericarditis, effusion and tamponade Flashcards
dilated cardiomyopathy
ventricular function is impaired, can be primary problem or end result of any insult to myocardium. can be one but more often all chambers dilated and functionally impaired. sometimes thrombosis
etiological background of dilated cardiomyopathy
genetic and familial, inflammatory, infectious, autoimmune, postpartum, toxic, injury, cell loss, scar replacement
examples of toxic insults causing dcm
alcohol, drugs, exogenous chemicals, endocrine
symptoms of DCM
progressive slow onset dyspnoea, fatigue, orthopnaea, PND, ankle swelling, weight gain and fluid overload, cough
examination findings for DCM
poor superficial pulse, thready pulse, irrefutable if in AF, SOB at rest, narrow pulse pressure, JVP elevated and or TR waves, displaced apex, S3 S4 and mitral regurgitant murmur often, pulmonary oedema, pleural effusions, crepitations, pericytes and hepatic mycelium may also be present
investigations for DCM
repeated ECG, CXR, basic bloods FBC and U+E, echo, CMRI, coronary angiogram,, N terminal pro BNP
general measures in treatment of cardiomyopathy
correct anaemia, remove exacerbating drugs, correct endocrine disturbance, advise on fluid and salt intake, advise on managing weight to identify fluid overload, HF nurse referral
specific measures in treatment of DCM
ACEI, ATII blockers, diuretics, Sac/Val, beta blockers, anticoagulants, spironolactone, SCD risk assessment with ICD or CRT-D/P implant, cardiac transplant
restrictive and infiltrative cardiomyopathy
less common, describes the physiology of filling and myocyte relaxation capacity, the systolic function may or may not be impaired. about 50% related to specific clinical disorders the rest unknown
types of restrictive and infiltrative cardiomyopathy
non infiltrative, infiltrative, storage disease, endomyocardial
types of non infiltrative cardiomyopathy
familial, forms of HCM, scleroderma, diabetic, pseudoxanthoma elasticum
types of infiltrative cardiomyopathy
amyloid, sarcoid
types of storage diseases
haemachromatosis, fairy disease
types of endomyocardial cardiomyopathy
fibrosis, carcinoid, radiation, drug effects
pathology of restrictive and infiltrative cardiomyopathy
inability to fill well a ventricle whose wall has reduced compliance. relaxation of ventricular wall is an active process that needs functioning intact myocytes it is not passive.
investigation for restrictive and infiltrative cardiomyopathy
repeated ECG, CXR, N terminal BNP, basic bloods, FBC, U+E, be on look out for sarcoid and haemachromatosis, auto antibodies, amyloid may need non cardiac biopsy, fairy has low plasma alpha galactosidase A activity, echo, CMRI, biopsy
specific measure of treatment for restrictive and infiltrative 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, specific treatments for iron overload, amyloid, fabrys. endomyocardial fibrosis has little specific treatment
prognosis for restrictive and infiltrative cardiomyopathy
unless reversible then poor
hypertrophic cardiomyopathy
impaired relaxation is common feature and systolic function is usually adequate albeit with some functional abnormality. relatively high prevalence, sarcomere gene defect autosomal dominant but variable expression and incomplete penetrance
pathology of hypertrophic cardiomyopathy
mycoyte hypertrophy and disarray, can be generalised or segmental wall thickness greater than 14mm or 12mm in primary relative, can be apical septal or generalised, impaired relaxation so behaves in a restrictive manner, if septal hypertrophy this can with mitral valve defect lead to LVOT obstruction, coronary arteries also affected
symptoms of hypertrophic cardiomyopathy
asymptomatic for many, fatigue, dyspniae, angineal like chest pain, exertion pre syncope, syncope related to arrhythmias or LVOT obstruction
examination of hypertrophic cardiomyopathy
can be none, notched pulse pattern, irreg pulse if in AF or ectopy, double impulse over apex, thrills and murmurs, LVOT murmur will increase with Valsalva and decrease with squatting, JVP raised
investigations for hypertrophic cardiomyopathy
ECG, often abnormal but a few are normal where phenotype is poorly expressed in positive individuals, echo, CMRI, risk stratification for SCD may need ICD, holters
general measures for hypertrophic cardiomyopathy
avoid heavy exercise, avoid dehydration, explore FH and first degree relatives, ECGs and echoes, consider genetic testing, regular FU to reappraise the risks and progress
specific measures for hypertrophic cardiomyopathy
drugs to try and enhance relaxation eg beta blockers, varapamil, disopyrimide, if in AF anticoagulate, obstructive form- surgical or alcohol septal ablation, ICD if required
myocarditis
acute or chronic inflammation of the myocardium, can be associated with pericarditis, can impair myocardial function, conduction and generate arrhythmia, long list of possible causes but usually cause is not found, can take on the dilated cardiomyopathy appearance, most common is viral
myocarditis pathology
infiltration of inflammatory cells into the myocardial layers, reduced function and heart failure, heart block as conduction system is involved and arrhythmias
symptoms of myocarditis
heart failure with fatigue, SOB, CP in only 26%, shorter course of a few weeks, may not have fever, signs of Heart failure
investigation of myocarditis
ECG usually abnormal, biomarkers often elevated but not falling in a pattern consistent with MI, echo can get RWMA, CMRI can see oedema, low threshold for biopsy, viral DNA PCR, auto antibodies, step antibodies, Lyme B burgdorferi, HIV
measures of treatment for myocarditis
supportive with treatment of heart failure and support for Brady and tachycardia arrhythmias, immunotherapy if biopsy or other lx point to a specific diagnosis, stop possible drugs or toxic agent exposure
prognosis for myocarditis
30% recovery, 20% mortality at 1 year and 56% by 4 years. at 11 years those still alive are transplant free
pericardial disease
inflammation of the pericardial layers with or without myocardial involvement, substantial number of causes
key causes of pericarditis
bacterial, post MI, perforation, dissection of proximal aorta, neoplasia
symptoms of pericarditis
chest pain with pleuritic features and postal features, sitting forward usually improves it lying back worse, fever
examination of pericarditis
temp up, pericardial rub LSE, raised JVP if effusion present or substantial or haemodynamically relevant, low BP, muffled HS, high fever and very unwell despite no effusion may suggest bacterial
investigations for pericarditis
ECG and echo, troponin may be raised if myocardial involvement too
general treatment measure of pericarditis
viral is conservative, idiopathic gets colchicine and limited used 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
pericardial effusion
may be haemodynamically significant = tamponade or not, often some causes of pericarditis where tamponade is present
symptoms of pericardial effusion
overt, fatigue, SOB, dizzy with low BP, chest pain occasionally
signs of pericardial effusion
overt, pulsus paradoxus, JVP raised, low BP and or rub, and or muffled HS, pulmonary oedema is very rare in pericardial effusions and tamponade
investigations for pericardial effusion
key test is urgent echo, CXR can show large cardiac shadow, drainage, send for MCS, neoplasia cells, protein and LDH , persistent effusion needs surgical pericardial window made to allow flow to abdomen
constrictive pericarditis
rare, causes are idiopathic, radiation. posy surgery, autoimmune, renal failure, sarcoid
pathology of constrictive pericarditis
impaired filling although myocardium is normal most of the time
symptoms of constrictive pericarditis
fatigue, sob, cough
signs of constrictive pericarditis
right heart filature with oedema, ascites, high JVP, jaundice, hepatomegaly, AF, TR, pleural effusion, pericardial knock
investigations for constrictive pericarditis
echo and right heart catheterisation to differentiate from restrictive cardiomyopathy which can be very difficult
treatment of constrictive pericarditis
careful and limited diuretics and pericardectomy
