Rheumatic Heart Disease
Rheumatic heart disease is a condition in which the heart valves have been permanently damaged by rheumatic fever.
Rheumatic Heart Disease can also be defined as a chronic stage of Rheumatic Fever involving all the layers of the heart causing major cardiac sequelae
So what is Rheumatic Fever?
Rheumatic Fever is an autoimmune, systemic, post-streptococcal, inflammatory disease, principally affecting the heart, joints, central nervous system, skin and subcutaneous tissues.
‘rheumatism licks the joint, but bites the whole heart’.
Causes/Etiology of Rheumatic Heart Disease
- Infection: The heart damage may start shortly after untreated streptococcal throat infection referred to as strep throat or scarlet fever. The disease is caused by rheumatic fever and the bacteria responsible is group A beta-hemolytic streptococci. (Streptococcus pyogenes).
> The heart valve can be inflamed and become scarred over time.
> This can result in narrowing or leaking (regurgitation) of the heart valve making it harder for the heart to function normally.
> The commonest valves affected are the mitral valve and the aortic valve however all 4 valves may be affected. This may take years to develop and can result to heart failure.
Pathophysiology of Rheumatic Heart Disease.
Causative agent (Group A Beta hemolytic streptococci) causing Strep throat, untreated strep throat infection leads to rheumatic fever several weeks after a sore throat has resolved (only infections of the pharynx have been shown to initiate or reactivate rheumatic fever.
Severe scarring of the valves develops during a period of months to years after an episode of rheumatic fever, and recurrent episodes may cause progressive damage to the heart valves. The mitral valve is affected most commonly and severely (65-70% of patients) followed by aortic valve. This eventually can lead to heart failure.
Clinical features of Rheumatic Fever.
- Fever (39 degrees Celcius)
- Swollen, tender, red and extremely painful joint –particularly the knees and ankles. (Migrating Poly arthritis)
- Nodules (lumps under the skin)
- Shortness of breath and chest discomfort. Uncontrolled movement of arms, legs or facial muscle
- General weakness
- Carditis presenting with chest pain, dyspnea, palpitations,
Clinical features of Rheumatic Heart Disease.
- Carditis: Carditis can involve the pericardium (pericarditis), myocardium (myocarditis), and endocardium (endocarditis) /
- Polyarthritis: Acute pain and swelling in the joints, starting with one joint and onto the other(migratory polyarthritis),less often in kids.
- Chorea: involuntary, irregular, unpredictable muscle movement
- Erythema marginatum: A long-lasting reddish rash that begins on the trunk or arms as macules, which spread outward and clear in the middle to form rings, which continue to spread and coalesce with other rings, ultimately taking on a snake-like appearance
- Subcutaneous nodules: Painless, firm collections of collagen fibers over bones or tendons. They commonly appear on the back of the wrist, the outside elbow, and the front of the knees
Diagnosis of Rheumatic Heart Disease
- People with rheumatic heart disease will have or recently had strep throat infection ( throat culture / blood test may be used to check for streptococcus)
- The patient may have the murmurs or rub that may be heard during auscultation this may be due to blood leaking around the damaged valves
- Along with complete medical history and physical examination, test to diagnosed rheumatic heart disease may include:
Echocardiogram (cardiac echo).
> Electrocardiogram (ECG): valve insufficiency and ventricular dysfunction. are observed in patients with rheumatic heard disease.
> Cardiac MRI and Chest Radiography: Cardiomegaly, pulmonary congestion, and other findings consistent with heart failure may be observed on chest radiograph in individuals with rheumatic fever.
> Blood test: C-reactive protein and erythrocyte sedimentation rate are elevated in individuals with rheumatic fever due to the inflammatory nature of the disease
Modified JONES criteria guideline for the diagnosis of Rheumatic Heart Disease.
(A) Major criteria
Major criteria is a Jones criteria
- J – Joint involvement which is usually migratory and inflammatory joint involvement that starts in the lower joints and ascends to upper joints
- O – (“O” Looks like heart shape) – indicating that patients can develop myocarditis or inflammation of the heart
- N – Nodules that are subcutaneous
- E – Erythema marginatum which is a rash of ring-like lesions that can start in the trunk or arms. When joined with other rings, it can create a snake-like appearance
- S – Sydenham chorea is a late feature which is characterized by jerky, uncontrollable, and purposeless movements resembling twitches
(B) Minor Criteria
Minor criteria include
- C – CRP Increased (C-reactive Protein) High in cases of inflammation. (above 3mg/dl)
- A – Arthralgia ( Joint pain)
- F – Fever (> 38.5 degrees Celicius)
- E – Elevated ESR (inflammation indicative) (>60mm/hr)
- P – Prolonged PR Interval
- A – Anamnesis (suggestive of rheumatism)
- L – Leukocytosis
- Evidence of Group A Streptococcal Infection + 2 major criteria
- 1 major + 2 minor criteria
A prolonged PR interval
represents a delay in the time it takes for the
signal to move across the atria at the top of the heart,
which receive blood flowing in from the veins,
into the ventricles at the bottom of the heart,
which pump blood out into the arteries
(C) Supportive evidence of preceding group A streptococcal infection including;
Positive throat culture for group A streptococci, raised titers of streptococcal antibodies (ant streptolysin O and S, ant streptokinase), and recent scarlet fever.
Investigations for rheumatic heart disease
- Throat swab/ culture.
- Rapid antigen detection test
- Anti-streptococcal antibody titers.
- Physical examination; murmurs? Abnormal rhythms?
- Chest x-ray may show cardiomegaly, congestion.
- Echocardiogram/ Doppler echocardiography may show effusion, vulvular dysfunction
Management of Rheumatic Heart Disease.
The treatment depends on how much damage has been done to the heart valves. In severe cases, treatment may include surgery to replace or repair the badly damaged valves. The medical treatment is divided into three parts i.e.
- Prevent and eradicate infection
- Maximize cardiac output
- Promote comfort
1. To prevent and eradicate infection
´The best treatment is to prevent rheumatic fever by giving antibiotic for throat infection and keep rheumatic fever from developing hence prevent damage to the valves.
> i.m benzathine penicillin 0.6-1.2 mu every 4 weeks, the same dose is given every 3 weeks in areas where rheumatic fever is endemic.
> Note. patients with rheumatic fever and have developed carditis and valve damage should receive antibiotic for at least 10 years or until age of 40 years. Patients who had rheumatic fever without valve damage do not need this prophylaxis.
2. To maximize cardiac output
- Anti-inflammatory. Treatment of the acute inflammatory manifestations of acute rheumatic fever consists of salicylates and steroids; aspirin in anti-inflammatory doses effectively reduces all manifestations of the disease except chorea.
- Analgesics for pain relief such as Paracetamol are preferred to opioids.
- Corticosteroids. If moderate to severe carditis is present as indicated by cardiomegaly, third-degree heart block, or Congestive Heart Failure, add orally prednisone to salicylate therapy.
- Anticonvulsant medications. For severe involuntary movements caused by Sydenham chorea, prescribe an anticonvulsant, such as valproic acid or carbamazepine (Carbatrol, Tegretol, others).
- Antibiotics. Such as penicillin or erythromycin or another antibiotic to eliminate remaining strep bacteria.
- Surgical care. When heart failure persists or worsens after aggressive medical therapy for acute Rheumatic Heart Disease, surgery to decrease valve insufficiency may be lifesaving; approximately 40% of patients with acute rheumatic fever subsequently develop mitral stenosis as adults.
- Diet. Advise nutritious diet without restrictions except in patients with Congestive heart failure, who should follow a fluid-restricted and sodium-restricted diet; potassium supplementation may be necessary because of the mineralocorticoid effect of corticosteroid and the diuretics if used.
- Activity. Initially, place patients on bed rest, followed by a period of indoor activity before they are permitted to return to school or work; do not allow full activity until the PRs have returned to normal; patients with chorea may require a wheelchair and should be on homebound instruction until the abnormal movements resolve.
- ACE Inhibitors e.g captopril, enapril, beta blockers e.g. bisoprolol, metoprolol, diuretics and digitalis e.g digoxin.
- Therapy for congestive heart failure. Heart failure in Rheumatic heart disease probably is related in part to the severe insufficiency of the mitral and aortic valves and in part to pancarditis; therapy traditionally has consisted of an inotropic agent (digitalis) in combination with diuretics (furosemide, spironolactone) and afterload reduction (captopril).
3. To promote comfort
- Patients with arthritic complication are given salicylate e.g. aspirin
- Encouraged to have bed rest
- Warm compress on the joints
- And use of bed cradle to lift the weight of bed linen from the affected joints
Complications of rheumatic heart disease
- Heart failure: This occur from either narrowed or leaking heart valve.
- Bacterial endocarditis .infection of the inner lining of the heart this occur when the rheumatic fever has damaged the heart valves.
- Raptured heart valves. this is a medical emergency that require urgent surgery to replace or repair the damaged heart valve.
- Cerebral stroke: this occur when a piece of vegetation dislodges itself and join circulation to the cerebral vessels.
- Pulmonary hypertension due to systemic congestion with blood.
- Atrial fibrillation. happens when abnormal electrical impulses suddenly start firing in the atria. These impulses override the heart’s natural pacemaker, which can no longer control the rhythm of the heart. This causes you to have a highly irregular pulse rate.
- Infective Endocarditis, pericarditis and myocarditis.
- Decreased cardiac output related to valve stenosis as evidenced by shortness of breath, fatigue, dizziness.
- Acute pain related to inflammation of synovial membranes as evidenced by patient verbalizing about painful joints.
- Hyperthermia related to inflammation of synovial membranes and heart valves as evidenced by a thermometer reading of 38 degrees Celsius.
- Activity intolerance related to muscle weakness as evidenced by prolonged bed rest.
- Self care deficit related to polyarthritis, therapy, bed rest.
- Impaired skin integrity related to skin inflammation as evidenced by subcutaneous nodules and skin rash.
- Risk for impaired Gas exchange related to blood accumulation in the lungs due to atrial filling.
- Risk for injury related to chorea.
- Risk for non-compliance with prophylactic drug therapy related to financial or emotional burden of lifelong therapy.