Rheumatic heart disease

             Usually affects children (most commonly between 5 and 15 years)

            Licks the joint ,bites the heart

               Features occur  2–3 weeks after an episode of streptococcal pharyngitis

                  Multisystem disorder

Pathogenesis

      Ab against  Group A Streptococcal antigens that cross-react with cardiac myosin and sarcolemmal membrane protein  and joint connective tissue causing pancarditis , arthritis and skin inflammation

      Histologically-Fibrinoid degeneration and aschoff nodules(Multinucleated giant cell) are found

      Aschoff is only found in heart-Giant cell ,macrophage ,T cell ,plasma cell with central necrosis

Jones criteria

Major criteria-(C2ARE : Carditis ,Chorea ,Arthritis ,Rheumatic nodule/Subcutaneous.nodule ,Erythema marginatum)

1.Pancarditis-

            For pericarditis-

                      Central chest pain varies with posture(If IHD-No variation with posture)

                      Pericardial rub(May be absent later?-Due to resolution/effusion)

                      ST elevation with concavity upward

            
            For myocarditis-

                      Palpitation ,Heart block  ,left/right heart failure

                      S3 gallop

                      T wave inversion

           
             For endocarditis-

                   Soft heart sound

                   Appearance of new murmur or change of character of previous murmur                            

                   Pansystolic murmur [Mitral regurgitation]

                   Soft mid-diastolic murmur(Carey coombs murmur) [Due to valvulitis]

                   Eearly diastolic murmur[Aortic regurgitation]

2.Polyarthritis-

• Most commonest of the major
• Asymmetrical ,migratory of knee ,elbow ,wrist ,ankle joints
• Never small joints
• Migratory means: Involve 1st joint>Peak effect of 1st joint>Slightly reduced from peak pain of 1st joint>Affect 2nd joint>Then other joint in same manner
• Respond to aspirin is diagnostic

[Other migratory-Gonococcal/syphilitic arthritis ,lyme arthritis ,septicemia ,bacterial endocartitis]

3.Sydenham's chorea/St. vitus dance-

         Sudden ,non repetitive

         Purposeless involuntary ,choriform movement of hands ,feet and face

         3 months after Rheumatic fever

        Occurs  when all other S/S disappear

        More common in female

[Other chorea-Huntington's chorea in Huntington's disease ,chorea gravidarum in SLE]

4.Erythema marginatum

                        Rounded

                        Macule

                        Faded centre with red margin

                        Trunk , proximal limbs ,but never in face

                        Blunch on pressure

5.Subcutaneous nodule-

               Small ,firm ,painless ,over extensor surface

               Freely moveable

               Confirms diagnosis ,rather making diagnosis

Minor criteria-(F2ARE : Fever ,First degree heart block ,Arthralgia ,Rheumatic fever/previous ,ESR/CRP raised)

Evidence-

       Supporting evidence of preceding streptococcal infection: recent scarlet fever, raised antistreptolysin O(300units) or other streptococcal antibody titre, positive throat culture(Patient's family ,teachers ,neighbours ,friends)

Diagnostic

              Two or more major manifestations +evidence

              Or, one major and two or more minor manifestations, along with evidence of preceding streptococcal infection.

Investigation

Evidence of a systemic illness (non-specific)

                         Leucocytosis, raised ESR and CRP

Evidence of preceding streptococcal infection (specific)

Throat swab culture: group A β-haemolytic streptococci

                            (also from family members and contacts)

 Antistreptolysin O antibodies (ASO titres): rising titres, or ,levels of > 200 U (adults) or  > 300 U (children)

Evidence of Carditis

Chest X-ray: cardiomegaly; pulmonary congestion

ECG: first- and rarely second-degree AV block; features of pericarditis; T-wave inversion; reduction in QRS voltages

                   
                  Echocardiography: cardiac dilatation and valve abnormalities

Management

Acute attack-

•       A single dose of benzyl penicillin 1.2 million U i.m. or oral phenoxymethylpenicillin 250 mg 6-hourly for 10 days

•       Erythromycin if allergic to penicillin

Secondary prevention-    

          Benzathine penicillin  1.2 million  unit I/M  monthly

Or,

          Oral phenoxymethylpenicillin 250mg 12 hourly

#Continue upto 5 years after last attack or upto 18 years age or whichever is longer

#If carditis already developes ,then continue  upto 10 years after last attack or upto 40 years age or whichever is longer

To whom give secondary prevention?

High prevalence area ,teachers