Rheumatic Heart Disease DR. DIBBENDHU KHANRA DM CARDIOLOGY

Rheumatic Heart Disease DR. DIBBENDHU KHANRA DM CARDIOLOGY AIIMS RISHIKESH

Pathology Fibrinous necrosis: exudative (bread and butter appearance) Proliferative (Aschoff nodules/Antishkow/ caterpiller cells) – McCallum patch Healing and fibrosis (milk spots)

Series of Events SORE THROAT (GABHS) ACUTE RHEUMATIC FEVER RHEUMATIC HEART DISEASE COMPLICATIONS ACUTE RHEUMATIC ACTIVITY

PREVALENCE 5-15 YRS 15 YRS RF 0.75/1000 (Mishra) 0.4/1000 (Verma) RHD 4.5/1000 (Lalchandani) 4.5/1000 (Lalchandani) 5-15 YRS All age Low risk pop 2/1 lac 1/1000 High risk pop 2/1 lac 1/1000

GABHS Sore Throat SORE THROAT (GABHS) MODIFIED CENTOR CRITERIA 1. AGE 5-15 YRS 2. HIGH GRADE FEVER 3. ANT CERVICAL LN 4. TONSILLAR EXUDATE 5. COUGH ABSENT 0-1 : NO AB* 2-3 : THROAT SWAB RAPID AG DET AB IF POSITIVE 4-5 : AB SORE THROAT to ARF: 3% (epidemic) 0.3% (endemic) Once RF after sore throat, 50% chance of RF recurrence after another sore throat THROAT SWAB: YIELD 5-10% *AMOXICILLIN/ AZITHROMYCIN

SN 77 SP 97

ARF: Modified Jones Criteria MAJOR PANCARDITIS MIGRATORY ARTHRITIS CHOREA SC NODULES ERYTHEMA MARGINATUM MINOR HIGH FEVER ARTHRALGIA ESR 30 CRP 3 PROLONGED PR H/O ARF IN RHD BLAND & JONES 30% PADMAVATI 30% PAUL WOOD 60% SB ROY 60% GAS INFECTION RAPID AG TEST THROAT SWAB ASO ANTI-DNAase Jones criteria exempted MS Chorea

INDIAN VS WESTERN INDIAN (PADMAVATI, SANYAL) 1/3 COMMENTS CARDITIS WESTERN (BLAND & JONES) 2/3 ARTHRITIS 1/3 2/3 ARTHALGIA ARTHRITIS CHOREA 50% 10% UNCOMMON SCN 5% 1% UNCOMMON EM 5% - RARE LESS IN INDIANS

PANCARDITIS ENDOCARDITIS Regurgitations MC-MR PSM Careycoumb EDM (AR) Long PR/ AF MYOCARDITIS Cardiomegaly S3 Parchment carditis Vs viral carditis: No murmer Symp improves PERICARDITIS Rub Effusion Rare w/o endocarditis

VALVULAR INV IN ARF VALVE MITRAL MITRAL AORTIC AORTIC TRICUSPID PULMONARY INVOLVEMENT 75% 20% 3% 2% - FATE OF MR/ PSM 1/3 DISAPPEARS 1/3 SAME 1/3 PROGRESSES

VALVULAR LOAD SVC 5 PV 10 RA 5 LA 10 RV 25/0-5 LV 120/0-10 PA 25/10 AO 120/80 TCV 20 mmHg PV 5 mmHg MV 110 mmHg AV 70 mmHg TCVA 8-10cm2 MVA 4-6cm2 PVA 2-4cm2 AVA 2-4cm2 TCVA 2 mmHg/ cm2 PVA 1 mmHg/ cm2 MVA 40 mmHg/cm2 AVA 25 mmHg/cm2

Carditis Acute: Dyspnea at rest Subacute: DOE Insidious: no symps, murmer Subclinical: no symp, no murmer, echo In jones criteria: No role of Murmer

SEVERITY OF CARDITIS Severity Mild Mod Severe Fulminant Cl/F NYHA 2-3 NYHA 3-4 NO CARDIOMEGALY NYHA 3-4 CARDIOMEGALY PERICARDIAL EFFUSION SC NODULES JACCOUDS ARTHOPATHY NYHA 3-4 CARDIOMEGALY LV FUNCTION DEPRESSED

SUB CLINICAL CARDITIS

CONSEQUENCE OF CARDITIS SANYAL ET AL ARF CARDITIS (60%) 2/3 RHD (40%) NO CARDITIS (40%) 1/10 RHD (4%)

Which murmur disappears? No CHF/ cardiomegaly Low grade PSM Single valve Early penicillin First attack Male Which ARFwill lead to RHD? CARDIOMEGALY / CHF GR2 EDM OVERCROWDING MALNUTRITION NO PEN PROPHX RECURRENT ATTACK

HOW MANY DIES? BLAND & JONES 10% IN 10 YRS 20% IN 20 YRS TOTAL 30% (1/3) IN 3 YRS CHF CARDIOMEGALY 50% DIES

Arthralgia/ arthritis! Fever and joint pain 1 week after sore throat Migratory Stereotypic Large joints No small joints NOT INVOLVED: TMJ, STERNOCLAV JOINT, ATLANTOAXIAL JOINT Back rarely involved Severely painful/ tender/ swollen/ red/ hot L/O function Symp signs Each joint Lasts for 1 week Dramatic response to salicylates Total episode resolves in 4 week No residual deformity

Arthralgia/ arthritis!DD VS PSRA 1. Short incubation period 2. Affects small joint 3. No response to salicylates 4. Often renal involvement 5. No carditis TO RX PENICILLIN PROPHYLAXIS FOR 1 YEAR VS JIA 6. MP rash incl face 7. Back inv 8. Small joints inv 9. LN 10. LFT deranged

Signifies ARA Non-erosive Can involve lower limbs

Subcutaneous nodules Extensor surface Elbow forearm Knee joints knee Severe carditis/ active carditis Painless Freely mobile Not attached to tendon Good response to salicylate DD Rheumatoid nodules/JIA -Larger -Painful -Attached to tendons Osler’s node Painful Pulp of fingers Smaller Janeway lesion Macular Palm soles blanching

Erythema marginatum In crops Painless Axilla/ thighs Never on face Annular Evanescent Itchy Rare to find in indians Carditis No response to salicylates DD Scarlet fever Scalding

Sydenhams Chorea Late manifestation Never with arthritis Carditis More in females Rare in postpubertal boys Resolves in 6m in 75% cases Jerky speech Pronator sign Jack in the box Worms in the tongue Milkmaids grip Spoon-like configuration Pendular knee jerk OCD Poor school performance Things fall from hands No sensory or motor inv

Sydenhams Chorea/ DD PANDAS Early after sore throat OCD Tics Epilepsy TO RX PENICILLIN TX IVIG/PLEX WILSONS Liver inv No carditis Hereditary HUNTINGTONS Anticipation Psychiatric prob Genetic/ Imaging

Antibodies ASO 240 TU in adults, 330 in children ASO rises after 1 week peaks after 3 weeks Anti DNAase B 120 TU in adults, 240 in children Anti DNAase B rises after 2 weeks peaks after 6 weeks Sensitivity ASO only 65% Anti DNAase B 85% Together 95% ESR 30, 50 in CHF (ESR falsely high in 50% pts of CHF) CRP 3 Throat swab can not differentiate b/w active inf/ carrier Multiple samples required Yield 10% Rapid antigen test also can not differentiate b/w active inf/ carrier

ECG features of active carditis Heart blocks PR prolongation despite tachy Relative brady VPCs Small voltage DD Dengue Diphtheria

Progression of RHD - Bland & Jones 20 yrs In india 5-10 yrs CMC Vellore 3months Depends on: Host factors (no penicillin prohpx) Environmental factors (overcrowding, malnutrition) Agent factors (Virulent strain, eg. Outbreak in Utah 1987)

RHD Manjunath et el: Mitral 60% 1/3 MS 1/3 MR 1/3 MS MR Mitral aortic 25% Aortic only 10% Tricuspid only 10% (TR TS) Pulm valve only not reported from India MVD 1/3

Complications of RHD PVH PAH LV dysfunction CHF AF Embolic stroke IE

Sudden worsening of symptoms Carditis/ ARA AF LV dysfunc Preg (carditis gravidarum) Vol overload Bact inf Thyrotoxicosis IE Thrombus

Recurrences SB Roy 1. 2. 3. 4. 5. Musical murmer Rub Cardiomegaly CHF Sleeping tachycardia Also 6. SC nodules 7. Prolonged PR despite tachy 8. Heart blocks 9. VPCs w/o digoxin 10.Pericardial effusion Bland & Jones 1/5 in 5 yrs 1/10 in 5-10 yrs 1/20 in 10-15 yrs 1/40 in 15-20 yrs Sanyal Carditis in 1st attack 30% Vaishnab Carditis in all attacks 90%

RHD in Young 5 yrs: 5% (Chockalingum) 12 yrs: 10% (Vaishnab) – Pediatric MS 20 yrs: 20% (SB Roy) – Juvenile MS 40 YRS: 40% Juvenile MS (SB Roy) - Predominant MS - Low ca - Less AF - Severe PAH - Small aorta - Cuspal: symp signs - Good result to BMV

ARF: Management Bed rest 4-6 weeks Good nutrition Benz Pen ( 27 kgs) 6lac IU ( 27 kgs) 12lac IU deep IM in buttock, small needle OR oral Pen V 250 TDSX5d (children) 500TDSX5d (adult) OR Erythromycin 250 QDSx10d (2omg/kg/d upto 1 gram in 3-4 divided doses) OR azithromycin 500 in day and 250 ODX4d (12.5 mg/kg/d x 5d) Arthtitis: ASA 100mg/kg/day in 3-4 divided doses Carditis: ASA 100mg/kg/day in 3-4 divided doses Salicylism: Resp alk (hyperventilation) – paradoxical aciduria – met acidosis CHF: prednisolone 1mg/kg/d in two divided doses Review after 1 weeks, 2 weeks and 4 weeks and FU with echo/ clin NO PROPHYLAXIS FOR ASYMP CARRIERS/ CONTACTS

Rebound/ Recurrence? On treatment: Initial recovery. But later worsening relpase Treatment completed Symptoms reappeared after completion of tx 6wks rebound 8wks recurrence

Secondary prophylaxis

Secondary prophylaxis

Penicillin - Recurrences w/o pen: 10% With oral pen: 3% With IM pen: 0.5% - Complications allergy: 3% Anaphylaxis: 0.5% Death: 0.05% Why 3wks? Incubation period: 9 days Achieves t1/2: 19 days Dose: 4 weekly For developing countries: 3 wkly (Pen level drops after 20 days, Taiwan)

Infective endocarditis prophylaxis

Q1: Commonest cutaneous manifestation in ARF? 1. 2. 3. 4. SC Nodules Eryhtme Marginatum Oslers Node Janeway Lesion

Q2: what is the most common cause of Jaccouds arthropathy in India? 1. 2. 3. 4. SLE ARF RA TB

Q3: MS/MR patient had recurrence at 45 yrs. 2’ prophyx how long? 1. 2. 3. 4. None 1 yrs 5 yrs 10 yrs

Q4: McCallum patch commonest in? Ventr side of LV Atrial side of LA Ventr side of AML Atrial side of PML

Q4: In RHD least involved mitral scallops is? 1. 2. 3. 4. A2 A3 P2 P3

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