Cardiomyopathy

Question 1

What is DCM?

Answer 1

Impaired ventricular function.
Can be primary, or the end result.
Can affect one or (often) all chambers.
Generally poor prognosis.

Question 2

What are the signs of DCM?

Answer 2

Thready and irregular pulse (AF), narrow pulse pressure, raised JVP, S3 and S4, MR murmur.
SOB at rest, displaced apex beat, pleural effusions, ascites, hepatomegaly.
Ankle/sacral/pulmonary oedema.

Question 3

What are the symptoms of DCM?

Answer 3

Progressive and slow onset.
SOB, fatigue, orthopnoea, postnatal depression, ankle swelling, weight gain, cough.

Question 4

What are the risk factors of DCM?

Answer 4

Genetics, muscular dystrophy, infection, autoimmunity, post-partum, alcohol, trauma.

Question 5

What are the investigations of DCM?

Answer 5

ECG (LBBB), CXR, bloods (FBC, U&E, NT-proBNP), ECHO, CMRI, cor angio, biopsy.
PMH - systemic illness, travel, hypertension.
SH - alcoholism, occupation.

Question 6

What is the treatment of DCM?

Answer 6

General - correct anaemia and endocrine disturbance, remove exacerbating drugs, advice of fluid and salt intake, management of weight.
Specific - ACEis, diuretics, BBs, entresto, spironolactone, anticoagulants, sudden cardiac death risk assessment, cardiac transplant.

Question 7

What is restrictive and infiltrative cardiomyopathy?

Answer 7

Less common. ~50% are related to disorders.
Pathology - the inability to fill a ventricle well, whose wall has reduced compliance.

Non-infiltrative - familial, forms of HCM, diabetic
Infiltrative - amyloid, sarcoid.
Endomyocardial - fibrosis, carcinoid, drugs.

Question 8

What are the investigations for restrictive and infiltrative cardiomyopathy?

Answer 8

ECG (LBBB), CXR, bloods (FBC, U&E, NT-proBNP), ECHO, CMRI, biopsy (false negative rate), non-cardiac biopsy (amyloid).
Autoantibodies (sclerotic CT diseases).
Fabry (low plasma alpha-galactosidase A activity).

Question 9

What is the treatment for restrictive and infiltrative cardiomyopathy?

Answer 9

(Specific) - BBs, anticoagulants, sudden cardiac death risk assessment, cardiac transplant, amyloid or Fabry’s for iron overload, ICD.

Limited diuretic use (low filling pressures cause problems), limited ACEi use.

Question 10

What is HCM?

Answer 10

Impaired relaxation.
Myocyte hypertrophy and disarray.
Generalised or segmental thickness.
Septal hypertrophy can cause mitral valve defects, leading to LVOT obstruction.
Coronary arteries are affected by narrowing and consequent ischaemia, fibrosis and arrhythmias.

Question 11

What is the prevalence of HCM?

Answer 11

60% - sarcomere gene mutation.
30% - idiopathic.
10% - other.

Prevalence - high (autosomal dominant).

Question 12

What are the signs, symptoms and investigations of HCM?

Answer 12

Signs - irregular pulse (AF), double impulse over apex, thrills, raised JVP, LVOT murmur (increases with Valsalva, decreases with squatting).

Symptoms - asymptomatic, fatigue, SOB, anginal-like chest pain, exertional pre-syncope, syncope related to arrhythmias.

Investigations - ECG, ECHO, CMRI, ETT.

Question 13

What is the treatment for HCM?

Answer 13

General - avoid heavy exercise and dehydration, explore family history, consider genetic testing, regular follow ups to assess risks/progress.

Specific - ICD, BBs, verapamil, disopyrimide, anticoagulants (AF), surgical septal ablasion.

Question 14

What is myocarditis?

Answer 14

Acute or chronic inflammation of the myocardium; can be associated with pericarditis and arrhythmias; can appear as DCM.
The most common cause is viral.

Inflammatory cells infiltrate the myocardium, leading to HF and arrhythmias.

Question 15

What are the symptoms and investigations of myocarditis?

Answer 15

Symptoms - HF, fatigue, SOB, cerebral palsy, fever.

Investigations - ECG, biomarkers (MI), ECHO, CMRI, biopsy, viral DNA PCR, autoantibodies, strep antibodies, HIV.

Question 16

What are the treatment and prognosis of myocarditis?

Answer 16

General - support of HF and arrhythmias, immunotherapy, stop possible drugs/toxins.

Prognosis - 30% recovery, 20% mortality at 1yr, 55% by 4yrs.

Question 17

What is pericarditis?

Answer 17

Inflammation of the pericardial layers +/- myocardial involvement.
Majority of causes - viral or idiopathic.
Consider bacterial, post-MI, perforation, dissection of proximal aorta, and neoplasia.

Question 18

What are the signs and symptoms of pericarditis?

Answer 18

Signs - fever, pericardial rub, raised JVP (effusion), low BP, muffled HS.
Very unwell, high fever, no effusion - bacterial.

Symptoms - ~2wks, chest pain (pleuritic), sitting forward helps, lying back worsens.

Question 19

What are the investigations and treatments for pericarditis?

Answer 19

Investigations - ECG (widespread ST changes, PR depression), raised Troponin I (if myocardial involvement), ECHO.

Treatment (general) - conservative (viral), colchicine and limited NSAIDs (idiopathic), chest drain (bacterial, even if small effusion; large effusion), antibiotics (bacterial).

Question 20

What is pericardial effusion?

Answer 20

Has the same causes as pericarditis.
Where tamponade is present.

Question 21

What are the signs and symptoms of pericardial effusion?

Answer 21

Signs - pulsus paradoxus, raised JVP, low BP, +/- rub and muffled HS.
Symptoms - fatigue, SOB, dizziness, chest pain.

Question 22

What are the investigations and treatments of pericardial effusion?

Answer 22

Investigations - ECHO (urgent), CXR (large cardiac shadow), ECG (alternates if large).
Treatment - drainage, proteins/LDH (exudate), neoplastic cells, surgery (if persistent).

Question 23

What is constrictive pericarditis?

Answer 23

Causes - idiopathic, radiation, post-surgery, autoimmunity, renal failure, sarcoid.
Pathology - impaired filling, normal myocardium (most of the time).

Question 24

What are the signs and symptoms of constrictive pericarditis?

Answer 24

Signs - right HF with oedema, ascites, high JVP, jaundice, hepatomegaly, AF, TR, pleural effusion, pericardial knock.
Symptoms - fatigue, SOB, cough.

Question 25

What are the investigations and treatment of constrictive pericarditis?

Answer 25

Investigations - right heart cath (exclude restrictive cardiomyopathy), ECHO.
Treatment (careful!) - limited diuretics, pericardectomy.