Diseases of Heart Muscle

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Acute myocarditis (Inflammatory cardiomyopathy)

This describes acute inflammation of the myocardium.

It is most commonly due to a viral infection. The most common virus is Coxsackie A and B (although usually the causal virus is not identified).

The most common cause worldwide is Trypanosoma cruzi (Chagas disease).

It can also be due to drugs including ethanol, clozapine and doxorubicin.

Half of patients recover in a month whereas many go on to develop dilated cardiomyopathy and heart failure.

 

Symptoms

Patients may have signs of a recent viral infection e.g. fever, joint pain, fatigue

Disease mirrors acute coronary syndrome e.g. acute chest pain, palpitations with shortness of breath

 

Key tests

ECG shows diffuse T wave inversions and saddle-shaped ST elevations

Raised inflammatory markers CRP/ESR with raised troponin and BNP

Heart MRI shows inflammation of the heart muscle

 

Management

Treat underlying cause, e.g., if parasitic or bacterial then give antibiotics

Anti-inflammatories, e.g., NSAIDs and colchicine, steroids if refractory to treatment

Symptomatic treatment of heart failure and antiarrhythmics

 

Dilated Cardiomyopathy (DCM)

The most common cardiomyopathy which refers to dilation of all 4 heart chambers.

It gives systolic dysfunction with biventricular heart failure and valve incompetence.

 

Causes

Usually idiopathic

Acute myocarditis (usually due to Coxsackie A/B)

Haemochromatosis

Ischaemic heart disease

Hypertension

Vitamin B1 deficiency

Drugs – Doxorubicin

dilated cardiomyopathy

Symptoms

Symptoms of heart failure – fatigue, oedema, ascites, S3 gallop heart sounds

Systolic murmur due to mitral/tricuspid regurgitation

 

Key tests

CXR shows “balloon” appearance of the heart

Echocardiogram shows LV dilation and systolic dysfunction with impaired global contractility

 

Management

Medications for heart failure (beta-blockers, ACE-i, mineralocorticoid antagonist).

If refractory to medical treatment, the definitive management is heart transplant

 

Hypertrophic Obstructive Cardiomyopathy (HOCM)

This refers to left ventricular hypertrophy which causes obstruction and turbulence in the left ventricular outflow tract.

It is the leading cause of sudden cardiac death in the young.

It is usually due to a genetic mutation in sarcomere proteins (β-myosin heavy chain).

It is inherited in an autosomal dominant fashion, but also associated with conditions like Friedrich’s ataxia and WPW syndrome.

Hypertrophy means that the ventricle cannot fill in diastole reducing cardiac output.

 

Symptoms

Can be asymptomatic and lead to sudden cardiac death due to ventricular fibrillation

The outflow tract obstruction gives rise to symptoms of aortic stenosis in young patients, e.g., exertional dyspnoea, angina, syncope following exercise

Gives ejection systolic murmur with jerky pulse

 

Key tests

ECG shows left ventricle hypertrophy with deep Q waves

Echo shows MR SAM ASH (mitral regurgitation, systolic anterior motion of mitral valve and asymmetric hypertrophy)

Cardiac biopsy shows hypertrophy of the muscle fibres

 

Management

This involves management of the complications: heart failure (e.g., beta-blockers), atrial fibrillation (rhythm control and anticoagulation), obstructive heart failure (surgical myomectomy) and prevention of sudden cardiac death (ICD).

 

Restrictive Cardiomyopathy

This is decreased compliance of the ventricular endocardium that restricts filling during diastole. This gives it similar symptoms to constrictive pericarditis.

 

Causes

Amyloidosis – build-up of amyloid plaques in organs

Haemochromatosis – inherited condition in which iron levels rise over many years

Endomyocardial fibrosis – fibrosis of the endocardium

Loffler’s syndrome – endomyocardial fibrosis with eosinophilic infiltrate and eosinophilia.

 

Symptoms

Presents as right heart failure –> Increased JVP, hepatomegaly, oedema, ascites

Classic finding is low-voltage ECG with diminished QRS amplitude

 

Key tests

Echocardiogram

MRI

 

Management

Treat the underlying cause

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