Acute Rheumatic Fever (ARF)

Acute Rheumatic Fever (ARF)

• In the third world, ARF is a prevalent cause of cardiac disease, especially in those aged 5-15 years

• Pharyngeal infections caused by Lancefield group A streptococcus are the main cause

• A subsequent ARF attack can be triggered 2-3 weeks later, affecting about 3% of children

• Antigenic mimicry occurs because a carbohydrate in the streptococcus cell wall is similar to a glycoprotein in the human cardiac valve

• Antibodies to the streptococcal cell wall cross-react with valve tissue causing an autoimmune reaction

• Cross-reacting antibodies have been found in the myocardial sarcolemma and caudate nucleus

Diagnosis of ARF

• Diagnosis is purely clinical
• Look for evidence of a preceding Lancefield group A B haemolytic infection (culture or elevated ASO titre)
• Jones criteria was established in 1904

Jones Criteria

• Major criteria include carditis, arthritis, Sydenham's chorea, erythema marginatum, and subcutaneous nodules
• Minor criteria include fever, previous rheumatic fever, raised ESR or CRP, arthralgia, and a long PR interval
• Additional findings may include recurrent streptococcal infection, a history of scarlet fever, a positive throat swab, and elevated ASO titre

Clinical Features

• Carditis and arthritis are considered common features

Carditis Details

• Murmurs include PSM (apex) MR, mid-diastolic murmurs apex, Carey Coombs murmur, and 1st-degree HB

Arthritis Details

• Arthritis presents as a migratory polyarthritis, usually affecting large joints, that does not cause chronic arthritis

Subcutaneous Nodules Details

• Subcutaneous nodules are rare, appearing in less than 5% of cases
• They are small, mobile, and painless, located on extensor surfaces of joints

Erythema Marginatum Details

• Erythema marginatum is rare, mainly on the trunk, not on the face
• It presents as an evanescent geographical-type rash with slightly raised edges and a clear centre without itching or induration

Sydenham's Chorea Details

• Sydenham’s chorea, or St. Vitus dance, is a late manifestation
• It can be unilateral or bilateral, involving involuntary purposeless movements associated with facial grimacing

ARF Investigations

• Conduct ESR/CRP/ASO titre, ECG, and Echo tests

ARF Treatment

• Treatments include salicylates at 100mg/kg/day and steroids at 3mg/kg/day
• Medications include benzathine penicillin
• Implement a 2nd dry prophylaxis and administer L.A. penicillin 1,200,000 U IM every 3 weeks

Mitral Stenosis

• Aetiology is almost always rheumatic or congenital
• Fusion of the commissures occurs during pathogenesis
• Leaflets thicken and calcification occurs
• The chordae thicken, fuse, and shorten, resulting in fish mouth and buttonhole orifice

Pathophysiology and Symptoms Diagram

• Mitral stenosis can cause an increase in left atrial size, leading to AF/thrombosis
• Mitral stenosis can cause RHF
• Mitral stenosis can cause pulmonary hypertension that can increase in left atrial pressure which causes pulmonary venous pressure and subsequent SOB and Orthopnoea

Mitral Stenosis Symptoms

• Symptoms include dyspnoea and fatigue
• The following symptoms can also indicate Mitral Stenosis: haemoptysis, systemic embolization, chronic bronchitis, chest pain, palpitations, and symptoms of RHF
• Symptoms of left atrial enlargement include left recurrent laryngeal nerve palsy
• The symptoms can also include hoarseness of voice, oesophagus and dysphagia
• Additional symptoms can be left main bronchus and left lung collapse
• Infective endocarditis is a rare symptom

Physical Signs of Mitral Stenosis

• Signs include mitral facies and pulse volume/AF
• Other signs are JVP (PHTN) and tapping apex impulse (not displaced)
• Listen for loud 1st HS/OS and MDM rumbling
• Further signs include loud P2 (PHTN), ECG SR or AF, and P mitrale

Echocardiography and Mitral Stenosis

• Echocardiography shows normal left ventricle size and function, and a dilated left atrium
• Echocardiography can further highlight mitral valve stenosis by area and gradient, associated lesions, PA pressure, and thrombus formation

Medical Treatment for Mitral Stenosis

• Treatments should lower the patients heart-rate and relieve congestion via diuretics
• AF anticoagulation should be administered

Interventions for Mitral Stenosis

• PTMC / MBV is an intervention

• Open commissurotomy is an intervention

• Valve replacement is an intervention

Mitral Regurgitation Aetiology

• Mitral valve apparatus, annulus, leaflets, chordae tendineae, and papillary muscles are all involved
• Aetiologies include primary and secondary

Mitral Regurgitation - Primary Aetiology

• Primary causes include annulus calcification
• Leaflets issues that can cause mitral regurgitation include either from rheumatic IE MVP
• Ruptured chordae (idiopathic- MVP IE ischaemia) and papillary muscle dysfunction are causes

Mitral Regurgitation - Secondary Aetiology

• Secondary causes include dilated MV annulus, papillary muscle malalignment, ICM, DCM, and atrial fibrillation

Mitral Regurgitation Symptoms

• Mild cases may be asymptomatic
• Most patients fall into one or two groups depending on time onset of events and size/compliance of the left atrium

Acute vs Chronic Mitral Regurgitation

• Acute MR presents as a small LA burden on pulmonary veins with acute pulmonary oedema

• Chronic MR presents as a large LA burden on ventricles; symptoms are similar to mitral stenosis

• PHTN and RHF are less frequent in mitral regurgitation than pure mitral stenosis

• IE is more common in MR

Common Causes of Mitral Regurgitation

• Acute mitral regurgitation causes include chordae rupture, acute papillary muscles dysfunction, and infective endocarditis
• Chronic MR causes include RHD and a floppy valve (MVP)

Physical Signs of Chronic Mitral Regurgitation

• Pulse will be regular or AF
• Apex will be displaced
• Hear a systolic thrill or soft S1 S3 PSM with any of these
• The ECG might show SR or AF as well as a P mitrale

Chest X-Ray for Mitral Regurgitation

• An X-Ray highlights a dilated LV, an LA enlargement, and MV calcification

• Signs of pulmonary venous congestion (Kerley B lines)

Echocardiography of Mitral Regurgitation

• Echocardiography shows LA size, LV size and function, MR etiology, other valve problems, and PAP

Medical Treatment of Mitral Regurgitation

• Administer diuretics and ACEI for chronic cases
• For acute cases, administer diuretics, vasodilators, IV nitroprusside, and IV nitrates

Interventions for Mitral Regurgitation

• Percutaneous repair
• Surgical repair or replacement

Indications for Intervention

• Symptoms
• LV dysfunction or dilation
• Situation beyond accepted limits
• Acute MR

Aortic Stenosis:

• There are several levels like:
  • 1. valvar aortic stenosis
  • 2. supravalvular aortic stenosis
  • 3. subvalvular aortic stenosis
• Valvar aortic stenosis is common.
• Most common are: congenital (bicuspid aortic valve 40-60 y - Unicuspid less common), senile (heavy calcification of the cusps), or inflammatory (RHD commissural fusion).
• Another cause is severe hypercholesterolemia

Aortic Stenosis Symptoms

• Shortness of breath
• Angina
• Syncope
• Sudden death

Aortic Stenosis Pathophysiology

• LV pressure overload

Aortic Stenosis Signs

• Small volume slow rising pulse
• Low pulse pressure
• Heaving apex impulse
• Systolic thrill in aortic area
• Soft A2
• Mid systolic murmur
• Aortic area base and carotids

Aortic Stenosis ECG

• Usually SR
• LVH

Aortic Stenosis Echocardiography

• LVH LV function
• Cause of AS
• Severity of AS (gradient - AV area)

Aortic Stenosis Intervention

• Aortic valve replacement is recommended once symptoms develop with an average survival of 2-3 years with cardiac failure, also LV dysfunction

Aortic Regurgitation Aetiology

• Primary disease of the aortic valve
• Aortic root disease with dilatation and stretching of the valve’s ring

Aortic Regurgitation Aetiology Details

• Aortic Regurgitation occurs by valve disease (congenital bicuspid valve, calcific RHD IE CTD)
• The Regurgitation may develop from aortic root disease or aortic aneurysm

Aortic Regurgitation and Symptoms

• Aortic Regurgitation is well tolerated lesion

Aortic Regurgitation Symptom Type

• When chronic one experiences SOB, angina and palpitations
• When acute one will experience SOB

Aortic Regurgitation Pathophysiology

• LV volume overload

Aortic Regurgitation Signals

• Corrigan's signs
• Large volume collapsing pulse
• Diastolic thrill AA
• Wide pulse pressure
• Displaced apex
• Hyperdynamic
• EDM AA
• Systolic murmur
• Austin flint murmur

Aortic Regurgitation Findings

• The ECG will find for SR - LVH
• Echocardiography:
  • LV D&F
  • Aetiology of AR
  • Severity
  • Ascending aorta

Aortic Regurgitation Indications for Interventions

• Symptoms
• LV dysfunction
• LV dilatation

Pulmonary Stenosis

• Obstruction of the RVOT may be at several levels
• Physical signs includes Characteristics faces, Noonan's Williams'
• Listen for a Systolic thrill in RVOT
• Check for LPH and JVP giant a wave
• Listen to a ESM pulmonary area and for Soft P2
• ECG :- SR P pulmonate RAD RBBB RVH
• ECHO :-RVH level of stenosis severity (gradient )

Pulmonary Stenosis Symptoms

• Dyspnoea and fatigue
• Symptoms of RHF
• Angina
• Syncope

Pulmonary Stenosis Treatment

• Pulmonary valvuloplasty surgery

Tricuspid Regurgitation

• Functional dilatation of the tricuspid valve ring due to PHTN chronic AF
• Ascites is a clear sign as is LL oedema

Tricuspid Regurgitation Symptoms

• Fatigue hepatic pain
• Pulsation in throat and fullness of face

Tricuspid Regurgitation Signals

• Large V waves in JVP
• Pulsatile liver
• PSM left sternal border

Tricuspid Regurgitation Treatment

• Diuretics may help; there are also repair or replacement options

TV Stenosis

• Almost always rheumatic and is associated with mitral stenosis

TV Stenosis Signals

• JVP prominent a wave
• Diastolic murmur at LSE hepatic congestion
• Ascites & Oedema

TV Stenosis Treatment

• Treatment options are Valvuloplasty or valve replacement