Rheumatic Heart Disease

Introduction:

Acute Rheumatic fever:

Acute Rheumatic Fever (ARF) is an acute autoimmune disease that occurs as a sequel  of group A- streptococcal infection which is characterized by inflammation of connective tissue, primarily affecting the joints and heart.

Rheumatic Heart Disease is a condition that occurs when rheumatic fever causes long term damage to the heart and its valves.

Etiology:

1. Most initial attack of ARF occur 1 to 5 weeks (average 3 weeks) after a streptococcal throat infection or infections of the upper respiratory tract.
2. Peak incidence among children between ages 6 to 15. 
3. A family history of Rheumatic fever and lower economic status are additional factors.

Pathophysiology:

Clinical Manifestations:

Major Manifestations: (JONES Criteria)

1. Carditis: Manifested by systolic or diastolic murmur, prolonged PR and QT interval on ECG, and possibly by signs of CHF in echo.
2. Poly-arthritis: Pain and limited movement of two or more joints. Joints are swollen, red, warm, and tender.
3. Syndenham's Chorea: Purposeless, involuntary, rapid movements commonly associated with muscle weakness, involuntary facial grimaces, speech disturbances, and emotional liability.
4. Erythema Marginatum: non- puritic pink, macular rash mostly of the trunk with pale central areas; migratory.
5. Subcutaneous nodules: firm, painless nodules over scalp, extensor surface of joints such as wrists, elbows, knees and vertebral column.

Minor Manifestations:

1. Arthralgia: Pain in one or more joints without evidence of inflammation, tenderness, or limited movement.
2. Fever: temperature greater than 100.4 degree F or 38 degrees C.
3. Laboratory abnormalities: elevated erythrocyte sedimentation rate (ESR), positive C reactive protein, elevated white blood cells count.
4. ECG changes: Prolonged PR interval.

Diagnostic evaluation:

Other diagnostic measures are:

1. ECG to evaluate PR interval and other changes.
2. Laboratory tests: ESR, C-reactive protein, WBC count, Group A streptococcal culture and/ or antistreptolysin-O titer to detect streptococcal antibodies from recent infection.
3. Chest X-ray for cardiomegaly, pulmonary congestion, or edema.

Management:

Medical Management:

1. Eradicate Infection:

• Preventive and prophylactic therapy is indicated after Rheumatic fever and Rheumatic heart disease to prevent further damage to heart valves. 
• Primary prophylaxis (initial course of antibiotics administered to eradicate the streptococcal infection) also serves as first course of secondary prophylaxis.
• Usually penicillin is given IM in a single dose or a 10 day course of oral Penicillin V.
• 10 days course of oral Penicillin V.
• Oral Erythromycin may be used for children who are allergic to Penicillin.
  
• An injection of 0.6 to 1.2 million units of benzathine penicillin G intramuscular every 4 weeks is the recommended regimen for secondary prophylaxis.
• Penicillin V or Erythromycin 250 mg twice per day for secondary prophylaxis.

2. Symptomatic Management:

• Oral Salicylates (aspirin) or NSAIDS are used to control pain and inflammation of joints. Aspirin is taken for 4-6 weeks for carditis.
• Corticosteroids for cardiac inflammation.
• Phenobarbital, Diazepam or other neurologic agent to control chorea.

3. Maximize Cardiac Output:

• Corticosteroids for carditis.
• If Heart failure develops treatment including Ace Inhibitors, beta blockers and diuretics is effective.

4. Bed rest:

• Depends on type and severity of manifestation.
• One week for isolated arthritis and several weeks for severe carditis.
• Full activity is allowed when ESR becomes normal.

5. Mitral valve replacement may be necessary in some cases.

Surgical Management:

When heart failure persists or worsens after aggressive medical treatment for Acute Rheumatic heart disease, surgery to decrease valve inefficiency  may be life saving.

1. Cummisurotomy: It is a mitral valve repair surgery which can be done to widen the valves.
   
2. Mirtal Valvulotomy or per-cutaneous balloon valvuloplasty or mitral valve replacement may be indicated in patients with critical stenosis.

                  

Nursing Management: 

• Acute Pain related to inflammatory response in the joints.

1. Assess level, intensity, frequency and duration of pain.
2. Complete bed rest and position changes.
3. Provide diversional therapy and psychological support.
4. Administer analgesics ans anti inflammatory medications as required.

• Decreased cardiac output related to valve dysfunction/ cardiac failure/ carditis.

1. Assess for symptoms of heart failure and decreased cardiac output such as diminished quality of peripheral pulses, cool skin or extremities, increased respiration and pulse and so on.
2. Assess heart sound.
3. Monitor blood pressure, intake and output.
4. Administration of cardiac glycosides as prescribed.

• Risk for injury related to involuntary movements (chorea).

1. Use padded side rails if chorea is severe.
2. Assist the child with feeding, ambulation and other fine motor activities as needed.
3. Avoid the use of straws and sharp utensils if chorea involves the face.
4. Make sure that the child consumes nutritious diet with recommended vitamins, proteins and calories.
5. Provide emotional support and protect the child from stress.
6. Administration of Phenobarbital or other medication for chorea as directed. Observe for drowsiness.

• Knowledge deficit related to disease condition and long term treatment.

1. Assess the level of knowledge of client and family.
2. Teach the appropriate administration of all medications, including antibiotics.
3. Encourage all family members to get screened for streptococus infection and receive the appropriate treatment.
4. Advise the parents that child cannot return to school and bed rest is essential.
5. Instruct on followup with usual health care provider for immunization, well- child evaluation, hearing and vision screening and other health maintenance needs.
6. Clarify the doubts of client and family.

• Anxiety related to disease condition and treatment.

1. Assess the level of anxiety.
2. Clarify the doubts of client and family by using non- medical terms.
3. Explain all activities, procedures and issues that involves the patient.
4. Explain the disease condition and prophylactic treatment.

Complications:

1. Heart Failure.
2. Valvular heart disease.
3. Cardiomyopathy.
4. Atrial arrhythmias.
5. Pulmonary and systemic embolism.

Prepared By:

Anusha Shrestha (RN)

Kathmandu, Nepal.