Cardiomyopathy- hypertrophic, dilated, restrictive and myocarditis, pericardial disease, pericarditis, effusion and tamponade

Question 1

dilated cardiomyopathy

Answer 1

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

Question 2

etiological background of dilated cardiomyopathy

Answer 2

genetic and familial, inflammatory, infectious, autoimmune, postpartum, toxic, injury, cell loss, scar replacement

Question 3

examples of toxic insults causing dcm

Answer 3

alcohol, drugs, exogenous chemicals, endocrine

Question 4

symptoms of DCM

Answer 4

progressive slow onset dyspnoea, fatigue, orthopnaea, PND, ankle swelling, weight gain and fluid overload, cough

Question 5

examination findings for DCM

Answer 5

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

Question 6

investigations for DCM

Answer 6

repeated ECG, CXR, basic bloods FBC and U+E, echo, CMRI, coronary angiogram,, N terminal pro BNP

Question 7

general measures in treatment of cardiomyopathy

Answer 7

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

Question 8

specific measures in treatment of DCM

Answer 8

ACEI, ATII blockers, diuretics, Sac/Val, beta blockers, anticoagulants, spironolactone, SCD risk assessment with ICD or CRT-D/P implant, cardiac transplant

Question 9

restrictive and infiltrative cardiomyopathy

Answer 9

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

Question 10

types of restrictive and infiltrative cardiomyopathy

Answer 10

non infiltrative, infiltrative, storage disease, endomyocardial

Question 11

types of non infiltrative cardiomyopathy

Answer 11

familial, forms of HCM, scleroderma, diabetic, pseudoxanthoma elasticum

Question 12

types of infiltrative cardiomyopathy

Answer 12

amyloid, sarcoid

Question 13

types of storage diseases

Answer 13

haemachromatosis, fairy disease

Question 14

types of endomyocardial cardiomyopathy

Answer 14

fibrosis, carcinoid, radiation, drug effects

Question 15

pathology of restrictive and infiltrative cardiomyopathy

Answer 15

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.

Question 16

investigation for restrictive and infiltrative cardiomyopathy

Answer 16

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

Question 17

specific measure of treatment for restrictive and infiltrative cardiomyopathy

Answer 17

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

Question 18

prognosis for restrictive and infiltrative cardiomyopathy

Answer 18

unless reversible then poor

Question 19

hypertrophic cardiomyopathy

Answer 19

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

Question 20

pathology of hypertrophic cardiomyopathy

Answer 20

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

Question 21

symptoms of hypertrophic cardiomyopathy

Answer 21

asymptomatic for many, fatigue, dyspniae, angineal like chest pain, exertion pre syncope, syncope related to arrhythmias or LVOT obstruction

Question 22

examination of hypertrophic cardiomyopathy

Answer 22

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

Question 23

investigations for hypertrophic cardiomyopathy

Answer 23

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

Question 24

general measures for hypertrophic cardiomyopathy

Answer 24

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

Question 25

specific measures for hypertrophic cardiomyopathy

Answer 25

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

Question 26

myocarditis

Answer 26

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

Question 27

myocarditis pathology

Answer 27

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

Question 28

symptoms of myocarditis

Answer 28

heart failure with fatigue, SOB, CP in only 26%, shorter course of a few weeks, may not have fever, signs of Heart failure

Question 29

investigation of myocarditis

Answer 29

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

Question 30

measures of treatment for myocarditis

Answer 30

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

Question 31

prognosis for myocarditis

Answer 31

30% recovery, 20% mortality at 1 year and 56% by 4 years. at 11 years those still alive are transplant free

Question 32

pericardial disease

Answer 32

inflammation of the pericardial layers with or without myocardial involvement, substantial number of causes

Question 33

key causes of pericarditis

Answer 33

bacterial, post MI, perforation, dissection of proximal aorta, neoplasia

Question 34

symptoms of pericarditis

Answer 34

chest pain with pleuritic features and postal features, sitting forward usually improves it lying back worse, fever

Question 35

examination of pericarditis

Answer 35

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

Question 36

investigations for pericarditis

Answer 36

ECG and echo, troponin may be raised if myocardial involvement too

Question 37

general treatment measure of pericarditis

Answer 37

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

Question 38

pericardial effusion

Answer 38

may be haemodynamically significant = tamponade or not, often some causes of pericarditis where tamponade is present

Question 39

symptoms of pericardial effusion

Answer 39

overt, fatigue, SOB, dizzy with low BP, chest pain occasionally

Question 40

signs of pericardial effusion

Answer 40

overt, pulsus paradoxus, JVP raised, low BP and or rub, and or muffled HS, pulmonary oedema is very rare in pericardial effusions and tamponade

Question 41

investigations for pericardial effusion

Answer 41

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

Question 42

constrictive pericarditis

Answer 42

rare, causes are idiopathic, radiation. posy surgery, autoimmune, renal failure, sarcoid

Question 43

pathology of constrictive pericarditis

Answer 43

impaired filling although myocardium is normal most of the time

Question 44

symptoms of constrictive pericarditis

Answer 44

fatigue, sob, cough

Question 45

signs of constrictive pericarditis

Answer 45

right heart filature with oedema, ascites, high JVP, jaundice, hepatomegaly, AF, TR, pleural effusion, pericardial knock

Question 46

investigations for constrictive pericarditis

Answer 46

echo and right heart catheterisation to differentiate from restrictive cardiomyopathy which can be very difficult

Question 47

treatment of constrictive pericarditis

Answer 47

careful and limited diuretics and pericardectomy