Rheumatic fever

This is a complication after being infected with group A β-haemolytic streptococcus.

Antibodies directed against the streptococcus pyogenes M-antigen cross react with myosin and smooth muscle.

This leads to a systemic condition which occurs in acute attacks, taking about three months to recover. It can also cause chronic inflammation leading to permanent damage to heart valves.

It typically causes stenosis of valves (usually mitral) with a “fish mouth” appearance.

Symptoms

History of previous streptococcal infection (e.g., pharyngitis)

Infective signs – fever and arthralgia

Cardiac symptoms (e.g., mitral stenosis) may occur after multiple attacks of RF

Key tests

Jones criteria is used, which requires evidence of recent infection plus 2 major, or 1 major and 2 minor criteria to be filled.

Evidence of prior group A B-haemolytic streptococcal infection

By throat culture or anti-streptolysin O (ASO) antibodies/anti-DNase B titres

Major criteria

Cardiac inflammation, including endocarditis (small vegetations along mitral valve) and myocarditis (Aschoff bodies, characterised by foci of chronic inflammation)

Migratory polyarthritis – joint inflammation that resolves and migrates to a new joint

Subcutaneous nodules on joints and spine

Erythema marginatum – a non-pruritic rash with red borders on trunk and limbs

Sydenham chorea – jerky, uncontrollable and purposeless muscle movements

Minor criteria

Fever

Prolonged PR interval on ECG

Raised inflammatory markers (ESR/CRP)

Management

Penicillin based antibiotics and anti-inflammatories

Important to treat the complications (e.g., mitral valve stenosis) as they occur

Individual Valve Conditions

These conditions affect specific valves and are usually divided into stenotis (narrowed) or regurgitation (incompetency of the valve) leading to backflow of blood.

Key tests

The key diagnostic for identifying valvular conditions is an echocardiogram

Management

The definitive management for valve conditions is a valve replacement.

If less severe or surgery not suitable, medical management is considered.

Tricuspid regurgitation

This refers to incompetency of the tricuspid valve which connects the right atrium and ventricle, which allows blood to flow back from the right ventricle to the right atrium.

This raises right atrial pressure and reduces venous return.

Therefore, it produces similar symptoms to right sided heart failure.

Causes

Right ventricle hypertrophy (after pulmonary hypertension) or right ventricle MI

Classically seen in infective endocarditis of IV drug users

Rheumatic fever, Ebstein’s anomaly, carcinoid syndrome

Symptoms

Gives signs of right heart failure (peripheral oedema, active pulsing in JVP)

Pulsatile hepatomegaly with left parasternal heave

Gives a pansystolic murmur head over the left sternal edge

Mitral Regurgitation

This refers incompetency of the mitral valve which allows blood from the left ventricle to enter the left atrium during systole.

Causes

Mitral valve prolapse

Myocardial infarction, causing ischaemia of the papillary muscles

Infections – rheumatic fever and infective endocarditis

Arrhythmias – long-standing fast AF can cause progressive atrial dilatation and reduce coaptation of the mitral valve 

Symptoms

Most patients are asymptomatic until the MR becomes very severe

Can give signs of left-sided heart failure (dyspnoea, fatigue, oedema)

Pansystolic “blowing” murmur at the apex radiating to the axilla with a soft S1

Mitral Stenosis

This is narrowing of the mitral valve orifice, usually due to chronic rheumatic fever.

This reduces blood flow from the left atrium into the ventricle and will cause enlargement of the left atrium.

This reduces the cardiac output from the left ventricle and so produces symptoms of left-sided heart failure.

Symptoms

Heart failure – dyspnoea, chest pain

Malar flush on cheeks due to decreased cardiac output

Fatigue, worse with activity

Atrial fibrillation and palpitations

Can lead to secondary pulmonary hypertension and right sided heart failure

Gives an opening snap followed by a mid-late diastolic murmur

Mitral Valve Prolapse

This refers to ballooning of the mitral valve into the left atrium during systole.

It is a type of myxomatous valve disease, which means the tissue of the mitral valve leaflets and chordae are abnormally stretchy, making the valve “floppy”.

Whilst it is mostly asymptomatic, it predisposes to complications such as valve regurgitation, arrythmias, emboli and infective endocarditis.

Causes

Connective tissue disorders (Marfan, Ehlers-Danlos syndrome)

Genetic conditions, e.g., polycystic kidney disease, Turner’s syndrome

Symptoms

Usually asymptomatic, but patients can get atypical chest pain or palpitations

Produces a mid-systolic click and late systolic murmur

Aortic stenosis

This is the narrowing of the aortic valve orifice, usually due to fibrosis and calcification from “wear and tear”.

It usually presents in late adulthood (> 60 years) and means that less blood is able to flow out from the ventricle into the aorta, which reduces cardiac output.

It is associated with a triad of chest pain, exertional dyspnoea and syncope. 

Causes

Calcification of the valve due to age

Bicuspid aortic valve (seen more in younger patients)

Rheumatic fever, William’s syndrome

Symptoms

Can be asymptomatic until the narrowing gets severe

Gives chest pain, breathlessness and syncope with exercise

Narrow pulse pressure with slowly rising pulse

Leads to eventual left ventricular hypertrophy or failure

Systolic ejection murmur radiating to carotids and a soft S2

May hear additional 4th heart sound

Management

If symptomatic, will require valve replacement

If asymptomatic, measure valvular gradient – if > 40 mmHg (severe) with ventricular dysfunction may need surgery

Aortic Regurgitation

This condition refers to aortic valve incompetency, which results in the backflow of blood from the aorta into the left ventricle during diastole.

This means that the left ventricle must compensate by pumping the blood harder to maintain a sufficient cardiac output.

This leads to left ventricular hypertrophy over time.

Left untreated, this will eventually lead to heart failure.

Causes

These are divided into valvular causes and aortic root causes

Valvular – rheumatic fever, IE, connective tissue disease, bicuspid aortic valve

Aortic root disease – aortic dissection, hypertension

Symptoms

Heart failure symptoms – breathlessness, orthopnoea, palpitations

A hyperdynamic circulation with wide pulse pressure (difference between systolic and diastolic). The diastolic is lower due to regurgitation, whilst systolic higher due to increased stroke volume.

Gives a water-hammer/collapsing pulse

Left ventricular dilation and eccentric hypertrophy

Early, blowing diastolic murmur heard in the tricuspid region

Rarer signs include pulsating nail bed (Quincke pulse) and head bobbing with each heartbeat (de Musset’s sign)

Management

Main goal is to reduce systolic hypertension with medication

Valve replacement if there is heart failure and dysfunction of the left ventricle