RHEUMATIC HEART DISEASE

Definition

• Rheumatic fever is a systemic, post-streptococal, inflammatory disease, principally affecting the heart, joints, central nervous system, skin and subcutaneous tissues.
• Rheumatic heart disease is a chronic condition characterized by scaring and fibrosis of valves and layers of the heart secondary to rheumatic fever
• Rheumatic Heart Disease caused by recurrent episodes of rheumatic fever; characterized by changes in the myocardium or scarring of the heart valves that reduce the power of the heart to pump blood.

Etiology

• Rheumatic fever occurs as a delayed sequale of a group A β-hemolytic streptococcal infection of upper respiratory tract usually a pharyngeal infection.

Incidence

• Acute rheumatic fever appear most often in children between the age 5 and 15 years,
• About 20% of 1st attacks occur in middle to later stage of life.
• Prognosis for the primary attack is generally good
• 1% of patients die from rheumatic fever.
• Increased vulnerability to reactivation of the disease with subsequent pharyngeal infections.
• Carditis is likely to worsen with each recurrence and damage is cumulative.
• 99% of cases of mitral stenosis is due to RHD.
• Mitral valve alone-65 to 70% of the cases.
• Mitral and aortic valve -25% .

Pathophysiology

Clinical Manifestations

• After confirming antecedent rheumatic fever (the modified Jones criteria)

Major Criteria

• Carditis (50%) - Inflammation of the heart muscle (myocarditis) which can manifest as congestive heart failure.
• Polyarthritis (70-75%)- A temporary migrating inflammation of the large joints, usually starting in the legs and migrating upwards.
• Chorea (10-30%) - A characteristic series of rapid movements without purpose of the face and arms. This can occur very late in the disease for at least three months from onset of infection.
• Subcutaneous nodules (0-8%) - 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.
• Erythema marginatum (5-13%) - 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. This rash typically spares the face and is made worse with heat.

Minor Criteria

• Fever of 101–102 °F
• Arthralgia: Joint pain without swelling (Cannot be included if polyarthritis is present as a major symptom)
• Raised ESR or C reactive protein
• Leukocytosis
• ECG showing features of heart block - prolonged PR interval (not included if carditis-present)
• Previous episode of rheumatic fever or inactive heart disease

Laboratory Findings

• High ESR
• Anemia, leukocytosis
• Elevated C-reactive protein
• ASO titer >200 Todd units.(Peak value attained at 3 weeks, then comes down to normal by 6 weeks)
• Anti-DNAse B test
• Throat culture-GABH streptococci
• ECG- prolonged PR interval, 2nd or 3rd degree blocks, ST-depression, T- inversion
• 2D Echocardiography- valve edema, mitral regurgitation, LA & LV dilatation, pericardial effusion, decreased contractility
• Chest X-ray - Cardiomegaly and increased broncho-vascular markings reflecting pulmonary venous congestion may be noted

Diagnosis

• Rheumatic fever is mainly a clinical diagnosis
• No single diagnostic sign or specific laboratory test available for diagnosis
• Diagnosis based on Modified Jones Criteria
• The presence of 2 major or 1 major and 2 minor criteria for the diagnosis of rheumatic fever

Histologic Findings

• Group A Streptococcal Infections Microscopic appearance of an Aschoff body in rheumatic heart disease.
• The Aschoff nodule is considered as a pathognomonic sign of Acute Rheumatic Fever.

Prevention and management strategies

Management

• Step I - Primary Prevention (eradication of streptococci)
• Step II - Anti Inflammatory Treatment (aspirin, steroids)
• Step III- Supportive Management & management of complications
• Step IV- Secondary Prevention (prevention of recurrent attacks)

 

STEP I: PRIMARY PREVENTION OF RHEUMATIC FEVER (Treatment of Streptococcal Tonsillopharyngitis)
Agent	Dose	Mode	Duration
Benzathine penicillin G	600,000 U for patients up to 27 kg (60 lb) 1,200,000 U for patients >27 kg	IM	Once
	"OR"
Penicillin V (phenoxymethyl penicillin)	Children: 250 mg 2-3 times daily Adolescents and adults: 500 mg 2-3 times daily	Oral	10 day
For individuals allergic to penicillin
Erythromycin Estolate	20-40 mg/kg/d 2-4 times daily (maximum 1 g/d)	Oral	10 day
	"OR"
Ethylsuccinate	40 mg/kg/d 2-4 times daily (maximum 1 g/d)	Oral	10 day

*Recommendations of American Heart Association

STEP II: ANTI-INFLAMMATORY TREATMENT
Clinical Condition	Drugs
Arthritis only	Aspirin 75-100 mg/kg/day,give as 4 divided doses for 6 weeks (Attain a blood level 20-30 mg/dl)
Carditis	Prednisolone 2-2.5 mg/kg/day, give as two divided doses for 2 weeks Taper over 2 weeks & while tapering add Aspirin 75 mg/kg/day for 2 weeks. Continue aspirin alone 100 mg/kg/day for another 4 weeks

*Recommendations of American Heart Association

STEP III: SUPPORTIVE &COMPLICATIONS MANAGEMENT

•    Bed rest •    Treatment of congestive cardiac failure - digitalis, diuretics •    Treatment of chorea - diazepam or haloperidol •    Rest to joints & supportive splinting

*Recommendations of American Heart Association

STEP I: SECONDARY PREVENTION (Prevention of Recurrent Attacks)
Agent	Dose		Mode
Benzathine penicillin G	1 200 000 U every 4 weeks*		Intramuscular
	"OR"
Penicillin V (phenoxymethyl penicillin)	250 mg twice daily		Oral
	"OR"
Sulfadiazine	0.5 g once daily for patients 27 kg (60 lb) 1.0 g once daily for patients >27 kg (60 lb)		Oral
For individuals allergic to penicillin
Erythromycin Estolate	250 mg twice daily		Oral
*In high-risk situations, administration every 3 weeks is justified and recommended
Duration of Secondary Rheumatic Fever Prophylaxis
Category		Duration
Rheumatic fever with carditis and residual heart disease (persistent valvar disease*)		At least 10 yrs. since last episode and at least until age 40 yrs., sometime life long
Rheumatic fever with carditis (no valvar disease*)		10 yrs. or well into adulthood whichever is longer
Rheumatic fever without carditis		5 yrs. or until age 21 yrs., whichever is longer
*Clinical or echocardiographic evidence.

*Recommendations of American Heart Association

Surgical Care

• Forty percent of patients with acute rheumatic heart disease subsequently develop mitral stenosis as adults
• Patients with critical stenosis, mitral valvulotomy, percutaneous balloon valvuloplasty, or mitral valve replacement may be indicated
• Due to high rates of recurrent symptoms after annuloplasty or other repair procedures, valve replacement appears to be the preferred surgical option

Prognosis

• Rheumatic fever can recur whenever the individual experience new GABH streptococcal infection, if not on prophylactic medicines
• Good prognosis for older age group & if no carditis during the initial attack
• Bad prognosis for younger children & those with carditis with valvular lesions

Nursing Diagnoses

• Pain related to inflammation of the Cardiac muscles
• Activity intolerance related to imbalance between oxygen supply and demand
• Impaired breathing pattern related to pulmonary congestion
• Imbalanced nutrition less than body requirements related to inadequate intake of food
• Deficient knowledge regarding prophylaxis and follow up care