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CARDIOLOGY CHAPTER OF INDIAN ACADEMY OF PEDIATRICS
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Discussion from National IAP Consensus meeting on pediatric
acute rheumatic fever and Rheumatic Heart
disease2007Indian Academy of Pediatrics
(Vision2007)

E-book available : www.cardioiap.org

Coorrespondance190, Sukhdev Vihar First floor, New Delhi 110025


editorcardioiap@gmail.com

Discussion from National IAP Consensus meeting on pediatric acute


rheumatic fever and Rheumatic Heart disease2007
Indian Academy of Pediatrics (Vision2007)
Consensus Guidelines On Pediatric Acute Rheumatic fever & Rheumatic Heart Diseases
Formulated at National Consultative Meeting On 20th May 2007, (Program under IAP Vision
2007)
Majority of our members cater to child health as practitioners and I strongly believe that capacity
building and empowering them will improve the health of the children of our country .
(Dr Naveen Thacker ,Presidential Address- IAP annual conference 2007)

Nodal Committee
Chairperson
Dr. R. K. Agarwal
Consultant Pediatrician. Udaipur
President elect central IAP 2008
Convener
Dr. Smita Mishra
Consultant Pediatric Cardiologist,
Max Heart & vascular Institute, New Delhi.

Co-Chairperson
Dr. Zulfikar Ahamed
HOD, Govt Medical College,Trivandrum
Chairperson Cardiology Chapter, Central IAP
Co-convener
Dr. Rani Gera
Consultant Dept of Pediatrics LNJP hospital
Secretary, Cardiology Chapter Central IAP

Faculty Members of Consultative meeting


Prof (Dr.) R.Tandon
HOD, Cardiology, Sitaram Bharatiya, Hospital of Medical Sciences. New Delhi
Previously ,HOD Deptt Of Cardiology AIIMS.New Delhi
Dr. Anita Saxena

Professor Pediatric Cardiology AIIMS, New Delhi


Dr. S. Radhakrishnan
Director, Pediatric Cardiology, EHIRC, New Delhi
Dr .R. Krishna kumar
HOD Pediatric Cardiology AIMS, Kochi.
Dr Simmi Manocha
Consultant Cardiologist Escorts Hospital, Faridabad
Dr. Meenakshi Sharma
Sr. Research Officer, Div of NCD, ICMR.
Dr Yugal K. Mishra
Director, Cardio Vascular Surgery EHIRC, New Delhi
Dr. Jyoti Singh
Associate Professor, Pediatrics, SS Medical College, Rewa (M.P.)
Dr. Arun Gupta
Senior Consultant Pediatrician, SunderLal Jain Hospital. New Delhi.
Core Committee
Dr R. Tandon,
Dr. Anita Saxena,
Dr R.Krishna Kumar,
Dr Z Ahmed
S. Radhakrishnan
Dr Smita Mishra

Advisory Committee
Dr. R.K. Agarwal,
Dr. Rani Gera
Dr J Singh,
Dr Y K Mishra
, Dr M Sharma,
Dr Simi Manocha,
Dr A Gupta

Special Contribution:
Dr Ashok Seth
Chairman, Max Heart & Vascular Institute
Saket New Delhi
Dr Anil Bhan
Director Cardio vascular Surgery
MHVI Saket New Delhi
Dr Pradipta Acharya,
Clinical Associate, MHVI Saket New Delhi

Conceptualization
Dr Naveen Thackar,
President IAP (year2007)
Dr. Deepak Ugra
Secretary IAP (Year 2007)
Dr H. P. Singh,
Professor of Pediatrics,
SS Medical college Rewa(MP)
Organization
Dr Ajay Gambhir
Vice President IAP(Year 2007)
Dr Anupam Sachdev
Consultant Pediatric Gastroenterologist,
Sir Ganga ram Hospital New Delhi

Mr Verma IMA
Content Author & correspondence : Dr Smita Mishra
Pediatric Cardiologist, MHVI Saket New Delhi
Contact : Dr Ashok Mittal Coordinator, Cardiology chapter IAP Andhra Pradesh
(dr_ashok_mittal@yahoo.co.in)

AIMS & OBJECTIVE


This consultative meet aims at formulation of
1.Guidelines for management of streptococcal sore throat.
(After discussion on: Problems with diagnosis.Treatment and alternative treatment)
2.Guidelines for diagnosis of acute rheumatic fever
(After discussion on various aspects
Role of Jones criteria
(Modifications: WHO,Australian)
Role of echocardiography - echo features)
Use of lab criteria .use of lab markers(str)
other than ASLO
Cost benefit of lab testing. Define the recurrence. Recurrence in patients recently underwent surgery or intervention to
recognize the recurrence)
3.Guidelines for management of ARF/RHD
ARF/ No carditis
ARF/Transient carditis
Established Valvular heart disease with or without ARF
Valvular disease with CHF with or without ARF
Atrial fibrillation
RHD with pregnancy
(Discussion on duration of bed rest, activity levels at school , use of penicillin or alternative drugs, use of digoxin, diuretic
and vasodilators, anticoagulation. Steroids and Aspirins . Side effects and management. )
4. Guidelines for interventions and surgery:
(Discussion on Interventions: Indication guidelines. Problems in pediatric patients. Role of closed mitral valvuloplasties.
Cost benefit ratio between BMV Vs CMV. Results. Chances of re- stenosis . Precautions. Interventions for aortic and
tricuspid valve. Surgical management: Indication and feasibility of repair in rheumatic valves. Results. Indication of
replacement. Current choice of valves. Cost. Long-term management. INR management. Diet alterations. When to
suspect prosthetic valve dysfunction.
5. Guidelines for secondary prophylaxis
( discussion on duration of Sec prophylaxis. Doses How much and what interval( 27 vs 30 kg - 15 days vs 21 days) .
Problems with secondary prophylaxis. Penicillin as an anaphylactic drug Penicillin as a member of special drug category.
How to ensure continuous supply. Method of injection. Site of injection . Pain relieving methods. Control of anaphylaxis
or allergic reaction.(name of drugs their use)
6.Guidelines for Bacterial endocarditis -prophylaxis and management
(Defining bacterial endocarditis . D/D Acute attack of carditis presenting with chordal rupture and SBE. clinical
presentation criteria to diagnose. Treatment Hospital vs home therapy after first two weeks.
I.V. vs oral use of newer antibiotics. No of cultures to be taken . Type of cultures (aerobic, anaerobic fungal ) cost benefit.
Should all be taken in first instance or if patient is not responding. Availability of facilities in peripheral medical colleges.
Treatment protocol, Use of second line drugs in culture negative endocarditis is it justified?)
7.Guidelines for general pediatric care:
Dental care
Vaccination
Nutritional management

Care of co- morbid situation like worm Infestation, Tuberculosis


8. Guidelines for surveillance, case detection, Notification and Data collection. (Formation of registries in area of high
population density and low income. Role of Community education. Establishment of supervised rheumatic clinic with
training of paramedics. District work shop and supplying guideline booklets to local practitioners. Role of medical colleges
and dispensaries )
By establishing guidelines we propose RHD prevention program under the supervision of IAP core committee group.
About 63% of deaths accounting for 90% of child deaths can be prevented if interventions with both sufficient and limited
evidence are implemented with universal coverage. there is an urgent need to strengthen existing interventions. From
Presidential address.
On sidelines of Consultative meeting central IAP conducted a nationwide survey with very good response from our
esteemed and knowledgeable pediatricians. Results were in concordance with our understanding related to subject.
There were clear indication of frustration about unavailability of proper guidelines, lack of supply of Benzathine penicillin.
According to this questionnaire we tried to bring out relevant answers in simpler manner.
Participation of pediatricians is the most and guidelines are being proposed for making registries to start special clinics
and to invite government to procure Benzathine penicillin for the masses.
Final Document may seem lengthy , but in prevailing conditions our pediatricians get more untreated children then treated
ones and this group has all kinds of complications. These guidelines contain all practical information which one may
need while looking after a patient with ARF/RHD, like how to monitor INR. Interaction of Food and drugs with
anticoagulants. One may get detailed information from articles given in references.
Literature search : Publications from India and abroad.(on line & journals). All available guidelines on the subject were
reviewed .
Classification categories: Class I: General agreement exists
Class II: Reasonable agreement, but conflicting evidence/divergence of
Opinion
IIa: Weight of evidence/ opinion is in favor
IIb: Credibility less well established, but most agree
Class III: Intervention not indicated, may be harmful
All recommendations fall in class one category except cross over aspirin vs continuous steroids for ARF/Moderate to
severe carditis.

Discussion National IAP Consensus meeting on pediatric acute rheumatic fever and Rheumatic Heart disease2007

Content:
Chapter I (Introduction & Management of streptococcal Pharyngitis)
1. Introduction
2. Management of streptococcal pharyngitis
Prevention
Diagnosis
Investigation
Treatment
Chapter II (Diagnosis, treatment of ARF and associated manifestation)
.Management of Acute Rheumatic fever
1. Diagnosis
i. Description of major criteria
ii. Clinical features of carditis
iii .Defining the Recurrence of carditis
iv. Echocardiography
v Valve involvement in carditis
vi. Terminology
vii. Association of carditis
viii. Polyarthritis
ix. Chorea
x. Subcutaneous nodule
xi. Erythema Marginatum
xii. Minor criteria
xiii. Supportive evidence of preceding str infection
2..ARF: Goal of treatment:
a-Eradication of Group A Streptococcal Infection.
b- Symptom Relief & Anti-inflammatory treatment
General measures

7-9
7
7
7
8
8
9
9-19
9-19
9
10-13
10
11-12
11
11
12
12
12
12,13
13
13
13,14
14
1414
15
15,16

Anti-inflammatory treatment
i. Aspirin
16
ii. Steroids
17
Treatment of Heart Failure
17,18
Treatment of Chorea
18
Treatment of Atrial Fibrillation
18
Chapter III LONG TERM MANAGEMENT OF ACUTE RHEUMATIC FEVER & RHEUMATIC HEART DISEASE
1. Prevention of recurrences
19-23
Secondary prophylaxis
19,20
BPG sensitivity test/Pen administration
.Management of anaphylaxis
2. Prevention & Management of Infective Endocarditis
3. Anticoagulation for Prosthetic valve
Embolization in RHD
4. Intervention in valvular heart disease
a. Percutaneous valvuloplasties
b. Surgery
Chapter IV Guidelines for community out reach
Appendix:
.Acronyms & Abbriviation
Table1:Rheumatic Fever/Rheumatic Heart Disease status in India
Table 2. Definitions ARF/RHD
Table 3.. Investigations
Table 4. Clinical features of CHF
Table 5.Auscultation and other finding of valve disease
Table 6. NYHA classification
Table 7. Echo evaluation of regurgitant lesion
Table8 The prevalence of physiological valvular regurgitation in normal people:
Table 9. Warfarin Doses adjustment
Flow Chart: Management of ARF
Table 10. Vit K content of vegetables
Table11: Drugs affecting INR
Table 12. Clinical evaluation of prosthetic valve
Table 13. Thrombolytic Therapy for prosthetic valve
Table 14. X- ray evaluation of prosthetic valve
Table 15. Post intervention follow-up
Table16. SBE prophylaxis recommendations
Table17. SBE prophylaxis Recommendation for
Dental procedure

20-21
22,23,
24-27
27-28
28 -29
29-33
29-32
32-33
33-34
35
36
37
37
37
38
38
39
39
39
40
41
42
43
43
44
44

Table 18. SBE prophylaxis Recommendation for


other procedure.
Table 19. Endocarditis Prophylactic Regimens.
Table20 Management pregnancy with RHD
Table 21:RHD Pregnancy with RHD and AF
Table 22. Cardiac evaluation of post intervention patient.
Table22. Follow-up plan of Acute Rheumatic fever
Table 23:Drugs and dosages
Table 24 AHA Guidelines Anaphylaxis Management
RF/RHD REGISTRY PROJECT Suggested Protocol
References

45
46
47
47
47
48
49-53
54
55-57
57-61

Chapter I
1. Introduction
2. Management of streptococcal Pharyngitis
1 Introduction 1-7 & 20
Acute rheumatic fever is a non suppurative complication of Group A beta hemolyticus gram positive
streptococcal (GABHS) sore throat. It affects joints, skin, subcutaneous tissue, brain and heart1,.Only
cardiac complications are significant, chronic and life threatening and require proper interventions. 2
Cardiac damage usually results from recurrent episodes of acute rheumatic fever. Early diagnosis and
secondary prophylaxis to prevent recurrent attacks, is the best possible way to avoid long term
cardiac sequelae.
Historically, Chorea, a neurological complication was probably first to get attention in 15 th -16th
century5, full spectrum of disease was recognized subsequently in early part of 19th century6. This
growing understanding resulted in evolution of clinical and laboratory based criteria for diagnosis , by
Dr T D Jone in 19447. These criteria underwent many revisions and modifications. Most latest revision
was done in1992 and later was accepted by WHO with some modification20..
Acute Rheumatic Fever and Rheumatic Heart Disease remains a cause of concern in rural and low
socioeconomic parts of India. This disease actually has its roots in child hood (5-15 yrs)2.
Prevalence in Indian population varies from 0.5 /1000 to 11/1000 in various studies. In developed
countries it is around 5 /100,000. Pediatric age group is the vulnerable population for residual cardiac
defects. Therefore, Indian academy of Pediatrics took the initiative and convened a national
consultative meeting at Delhi on 20th May 2007.
2. Streptococcal Pharyngitis: Prevention. Diagnosis and Treatment 12,13
Prevention:
There is no chance of getting a cost effective vaccine in near future.*14 Hence suggested preventive
measures are:
Use of handkerchief while coughing

Hand wash./ Isolation .


Early use of appropriate antibiotics.
ICMR(Indian council for medical research) is trying to evolve an effective vaccine according to
streptococcal Epidemiology in India. Immunological response only to homologous strain and findings
like sequencing of C terminal region of M protein being extreme unsuitable in south India are the
major road blocks. Assumptions are that an Immunological effective vaccine may not be costeffective
.14 .
Tab 1. GAS PHARYNGITIS VS NONSTREPTOCOCCAL PHARYNGITIS
Streptococcal Pharyngitis

Nonstreptococcal
Pharyngitis**

Age
Mode of onset
Initial symptoms
Fever
Characteristics of sore throat

5- 15 yrs
All
Sudden
More gradually
Sore throat with pain on swallowing
Mild sore throat
High - 38C
Not so high
Redness, tenderness of anterior Redness of the pharynx
cervical lymph nodes, hyperemia of Cough
the pharynx, petechiae on the palate, Hoarseness of voice
scabby erosions on the edge of Watery nasal secretions
nostrils. Clinical picture of scarlet Conjunctivitis
fever.
Throat cultures: Obtain specimen by vigorous swabbing of both tonsils and posterior pharynx.
Streptozyme tests for detection of GAS antigen: Not available easily.
**Non streptococcal Pharyngitis :Viral pharyngitis, adenovirus, enterovirus, herpes virus
influenza virus, other bacteria, Streptococci group C and group G, Neisseria gonorrhoeae
Mycoplasma pneumoniae, Chlamydia pneumoniae, Arcanobacterium haemolyticum
McIsaac Score: fever>=38c (+1),No cough (+1), Tonsillar exudates(+1),Tender ant cervical lymph node(+1)
Age<15(+1)Score >4highly predictive.

Relevant Lab Tests:


Throat Cultures / Rapid Streptococcal Antigen Test.
Antistreptolysin O (ASO), Erythrocyte sediment rate (ESR), C- reactive protein (CRP), Complete
blood count (CBC).
Treatment of Streptococcal sore Throat
Recommendations (NCMRHD IAP 2007):
Benzathine Penicillin(Deep IM single dose After sensitivity test)
*Penicillin V:
*Azithromycin
*Cephalexin

1.2 Million Units (> 27KG)


0.6 Million Units (<27 Kg)
250 mg QID X 10 days (ped)
500mg QID(adult)
12.5 mg/kg single dose X 5 days
15/kg/day BID/TID X 10days

Current recommendations are based on discussions on safety, cost effectiveness, acceptability and
availability of drugs. Penicillins as a group show excellent eradication rate. Single dose of Benzathine
penicillin12,13 can eradicate the streptococci effectively with a very low cost and can be used for
supervised injections, at community level and strongly recommended for regular use as a first line of
drug. Adequate blood levels persist at least for 3 weeks with 1.2 MU & 2 weeks with 0.6 MU. Though

pain at injection site may often persist for 1-2 days, other adverse effects are far less common and
fear for them seems unjustified..(Anaphylaxis: see section on secondary prophylaxis)
However, lately, availability of injections has been a problem. Oral penicillin is cost effective and
needs a 10 days course. Penicillin V 12,13, unlike pen G is an acid resistant drug and yields greater
blood levels. Compliance with Azithromycin15 is expected to better due to single daily dose and five
shorter duration.. It is the third choice. Although superiority over BPG has been established.. Till now
resistance has not been reported. First and second generation Cephalosporins have shown good
sensitivity12,13. The older brands are available in market at reasonable price.
Antimicrobials preferably not to be used for GABHS pharyngitis
Tetracycline
High prevalence of resistant strain.
Sulfonamide
and
sulfamethoxazole
Chloramphenicol

trimethoprim- Does not eradicate GABHS in pharyngitis.


Unpredictable efficacy and high toxicity.

Other Anti microbial Agents


Erythromycin a drug being used regularly for penicillin sensitive patients for secondary prophylaxis is
effective till now, Roxithromycin is not an established drug for GABHS pharyngitis. Clarythromycin
and Cephalosporins (Cefuroxime, Cefpodoxime) are newer effective drugs but are not recommended
as they carry no added advantage and are expensive.

10

Chapter II
1.Diagnosis
2.treatment of acute rheumatic fever and associated manifestation
1. Diagnosis of Rheumatic Fever: 16,17&23
Diagnosis: WHO adoption of (2004) revised Jones criteria 1992.9
Clinical & Lab criteria

Supportive evidence of preceding Streptococcal infection


(essential except for diagnosis of Chorea)

Major Criteria (More specific)


Carditis
Poly Arthritis
Chorea
Subcutaneous Nodule
Erythema Marginatum
Minor Criteria (Less Specific)
Fever
Poly arthralgia*
ESR, CRP, Poly morph. Leukocytosis
(follow standard lab values)
ECG: Prolonged PR interval ( Normal
value)**

Anti streptolysin O
anti-deoxyribonuclease B:
History of (within previous 45 days)
Streptococcal sore Throat
Scarlet fever
Positive Sore throat culture
Positive Rapid streptococcal antigen detection test

*Arthralgia is not considered as a minor criteria in presence of poly-arthritis.


** Not considred as minor criteria in presence of carditis.
First episode of ARF: Two major or one major and two minor criteria+ supportive evidence of previous streptococcal
throat infection
Recurrence of ARF in a patient without established Heart disease: Two major or one major and two minor criteria+
Supportive evidence of Previous Streptococcal throat infection
Recurrence of ARF in a patient with established Heart disease: two minor criteria+ supportive evidence of previous
Streptococcal throat infection
Rheumatic Chorea &Insidious onset Rheumatic Carditis: No requirement of other major manifestations or supportive
evidence of streptococcal sore throat infection
Chronic Valve lesions of RHD (Patient presenting with pure mitral stenosis(MS) or MS/mitral regurgitation (MR) with or
without aortic valve disease): Do not require any other criteria to diagnose as rheumatic heart disease.

11

Description of Major Criteria 16,17,21,23:

RHEUMATIC CARDITIS - Clinical diagnosis


Valvulitis
Apical Pan Systolic Murmur
Apical Mid Diastolic Murmur
Basal Early Diastolic Murmur

??Myocarditis19,20
(Literature suggests the so called
features of myocarditis are secondary
to valve regurgitation and not a true
myocarditis)
Unexplained Cardiomegaly.
Tachycardia
Congestive Heart Failure (CHF)
Soft S1/ S3 Gallop

Pericarditis
Pain
Rub
Effusion

Indicators of Recurrence of RF in established heart disease 21,22,23


1.
New murmur.
2.
Change in pre-existing murmur.
3.
Pericardial rub (and other evidence of Pericarditis).
4.
Unexplained CHF (differentiate it with decompensated chronic progressive heart disease).
5.
Enlargement of heart size, clinically and on X- Ray.
Difficult to diagnose if previous status of carditis is not known .
Echo findings suggestive of Acute Carditis 21,22:
1. Nodule- focal, present at body and tip. Showing no independent chaotic movement, disappearing
on follow up. Not seen in established RHD.
2. Prolapse of tip of valve due to minor chordal rupture.
3. Pericardial inflammation/Effusion.
Echo finding of established RHD21,22:
1.
Thickening of valve.
2.
Restriction of leaflet movements.
3.
Valve prolapse, annular dilatation (not common in first attack).
4.
Rupture of chordae tendineae
5.
Thickening of sub-valvar mitral apparatus.
6.
Elongation of chordae.
7.
Annular/ commissural calcification.
8.
Ventricular dilatation (usually secondary to valvular lesion)

12

9.
10.
11.

Mitral stenosis. Mitral Regurgitation. Aortic Valve disease.


Simultaneous involvement of Tricuspid valve.
Ventricular dysfunction: is not a feature of RF in absence of significant valvular disease.

Role of Echocardiography : ECHO is not required to establish a diagnosis of ARF but in absence of
overt clinical signs, it helps to recognize undetected carditis (sub-clinical carditis)
It helps in differentiating the acute carditis from Endocarditis
Unlike ESR or CRP it is not a major or minor criteria but plays very significant role in establishing the
diagnosis of carditis, a major Jones criteria.
Terminology :
a. Recurrence: A new episode of RF following another GABHS infection; occurring > 8 wks
following stopping treatment.
b. Rebound: Manifestations of RF occurring within 4-6 wks of stopping treatment or while
tapering drugs.
c. Relapse: Worsening of RF while under treatment and often with Carditis (4%)
d. Sub clinical carditis: When clinical examination is normal but Echo cardiogram (ECHO) is
abnormal. (Around 30 percent of patients having chorea present as sub clinical carditis.)
e. Indolent Carditis: It is a common entity in our country. Patient presents with persistent
features of CHF, Murmur and Cardiomegaly. There are no or very few features of carditis
Association of severe Carditis with joint involvement
Arthritis
Arthralgia
No joint involvement

10%
33%
50%

Association of Carditis with extra Cardiac Manifestations


PolyarthritisChorea
SC Nodule

60-75%
- 60 - 75 %
- > 95 %

POLY ARTHRITIS 16,,23,25


Onset : Poly arthritis is often, the first presenting complaint in general and common presentation in
Adolescents (75% patients have it in first attack of ARF)

13

Clinical presentation: Usually big joints are affected most (Knee> Ankle> Wrist> elbow >
Hip>shoulders rarely neck).Joints are warm, swollen, red and painful. Synovial fluid is sterile but has
inflammatory cells. Pain is out of proportion.
Character: Fleeting or Migratory. Inflammation in first joint starts to recede before involvement of
next joint.
Course : Occurs in first 2-4 weeks .Spontaneous resolution without residual defects. Magical
response to Aspirin.
Variant: Additive Arthritis (Involvement of many joint at a time).
Monoarthritis (? Early use of non-steroidal anti-inflammatory drugs). Must be differentiated
from post streptococcal reactive arthritis and monoarticular rheumatoid arthritis. In high endemic
areas possibility of rheumatic arthritis must be kept and close observation is indicated. Secondary
prophylaxis for a year can be considered during follow-up.
Polyarthralgia : Included in minor criteria.
Co-existence with other major criteria: : 50-75% may have associated carditis .10% cases have
moderate to severe carditis. Can co-exist with other major manifestations except Chorea which
presents late.
Jaccouds arthritis is a progressive deforming arthro-pathy of the hands and feet in young adults
following recurrent rheumatic fever/Systemic Lupus erythematosus.
Post streptococcal reactive Arthritis : This non-migratory arthritis occurs after a relatively short
latent period of a week following the episode streptococcal sore throat and lacks in dramatic response
to anti-inflammatory treatment. Mostly it is difficult to differentiate it from
True rheumatic arthritis. It may be wise to give secondary prophylaxis in presence of other Jones
criteria and evidence of recent streptococcal infection.
SYDENHAMS CHOREA 5,16,23-24,26 (Chorea derived from Greek word Khoreia means dance)
Onset: Chorea is a delayed manifestation (1-7 months) It has an insidious onset.
Clinical Presentation : Clinically it presents with irritability , emotional lability, uncoordinated
movements, muscular weakness and choreiform movement.. Many clinical signs can be seen or
elicited like, milkman grip, pronator sign, and Spoon disfiguration of wrist.
It can last for few days to several years.
Course : can persists for months to years. Recurrences are common.
Coexistence with other Major manifestations: association with Carditis is high. Arthritis almost
never co-exists.
Variants: Hemi chorea and chorea gravidorum can be manifestation of acute ARF.
PANDAS: Pediatric Autoimmune Neuro-psychiatric Disorders Associated with Streptococcal
Infections: it is believed that something very similar to rheumatic Chorea occurs. Till now diagnosis is
taken as hypothetical and no recommendations are made on it.

14

SUBCUTANEOUS NODULE (SCN)27,28,29


Clinical Presentation: SCN are round, firm, freely movable, painless lesions of varying size (0.52.0
cm.). Usually occur in crops over bony prominences or extensor tendons like elbow, wrists, knees,
ankles , Achilles tendons and spinal process.
Course: Spontaneous resolution in two to three weeks, without residual lesions.
Coexistence with other Major manifestations: > 90% are associated with severe carditis. May
coexist with Polyarthritis & Erythema Marginatum, rarely with chorea.
They can be induced using autologous Buffy coat for the diagnosis of Rheumatic activity. .
ERYTHEMA MARGINATUM 30
Clinical Presentation: Evanescence in nature. Multiple asymptomatic outwardly spreading pink
macule or papule with circular or serpiginous margins on trunk & proximal extremities and rarely may
be found in distal extremities. Never occurs on face.
Course: May persist or disappear and reappear.
Co-existent Major Manifestation: Carditis may coexist but EM is not an indicator of severity.
EM tends to present with subcutaneous nodules. In Indian population rarely seen probably due to
darker skin.

MINOR MANIFESTATIONS OF ACUTE RHEUMATIC FEVER 18,23,31:

15

Minor

Clinical Presentation

Coexistent Major

Course

Manifestation

criteria
1. Fever

Occurs at onset >1020 F. No characteristic

Persists for weeks

Present with all Except

pattern

Chorea

2.Arthralgia

: Usually involves large


Asymmetrical, migratory.
Intensity of pain: mild
to incapacitating.

3. ECG

Prolonged PR interval.
Cause:? due to effect of RF on
conduction system
. PR Interval (Normal Value)

ESR

4.
LAB

CRP
Leuko
cytosis

joints. days to weeks, often varying High correlation with


in severity.
severe carditis

PR prolongation is seen in
absence of Carditis.

3-12 yrs:0. 16sec;12-14y:0. 18sec>17y:0.


20sec
Significant in presence of major criteria. Have markers of inflammation!:
diagnostic & prognostic value.
Normalize with anti-inflammatory
Polymorpho nuclearcytosis is
also included but less reliable.
treatment or on spontaneous
Normal Value : ESR <30 mm/hr
resolution.
Positive CRP <12 mg/dl

Usual association of all


major
criteria
except
chorea. Anti-inflammatory
treatment is required if they
positive in presence of
chorea.

Standardized lab should be used. The value


of different lab can differ. Values should be
accepted according to standard value of lab.

Supportive evidence of previous streptococcal sore throat 17,18,23,25.


Chorea is absolved from fulfilling these criteria due to delayed presentation
Evidence of preceding streptococcal throat infection like positive throat culture antigen detection test,
history of scarlet fever within 45 days. ASO titer: >333 for children and > 250 for grown ups .
(Anti DNAs normal values are 1:60 TU in preschool,1:480 in school children & 1:340 in adults)

.
ASO
ASO + Anti DNAs

POLYARTHRITIS
80%
95%

CARDITIS
80%
95%

CHOREA
30%
80%

The blood titers of antistreptolysin-O raised against extra cellular antigens of streptococci appear in
10 15 days and reach a peak in 34 weeks after the acute infection, and usually are maintained for
23 months before declining. 20% case may remain positive till 6 months.
In absence of any major Jones criteria, isolated antibody titer rise of any level just suggest a past or
present streptococcal infection and not the acute rheumatic activity. Hence unlike ESR and CRP it is
not a minor criteria. There is no need to treat high ASO titers with secondary prophylaxis (Benzathine

16

penicillin). To show the rising titer, in selected patients ( having high suspicion of ARF but normal
ASO titer) one can repeat it after 1 week . Routinely it is not recommended.
ASO titer must be done in a standardized lab and values must be used according to that lab standard.
The Streptozyme test :Is a screening test for antibodies to the streptococcal antigens NADase,
DNase, streptokinase, streptolysin O, and hyaluronidase. Although it detects several antibodies in a
single assay and is expected to be more sensitive. However, it does not determines specific
antibodies. It is less sensitive in children than adults. In fact, borderline antibody elevations which
could be significant in children, may not be detected at all. Hence it is not recommended as a routine
test.
ACUTE RHEUMATIC FEVER: GOAL OF TREATMENT 16,17&23
A. Relevant Lab Tests:
Throat Culture/ Rapid Streptococcal Antigen detection tests.
ASO, ESR, CRP, Hemoglobin (Hb), CBC, Platelet count
Kidney Function test, Liver Function test
CXR, ECG,
Echocardiography.- see above.
?? Blood c/s & Coagulation profile in selected group of patients
Appropriate serological/ blood investigation according to clinical scenario.
B. Treatment of acute rheumatic fever and associated manifestation
I.
Eradication of Group A Streptococcal Infection.
II. Treatment of acute Rheumatic episodes
I. Eradication of Group A Streptococcal Infection.
(Treatment recommendations are same as for the streptococcal sore throat.)
II. Treatment of acute Rheumatic episodes:

Symptom Relief & Treatment of Inflammatory processa. General measures


b. Anti-inflammatory treatment
c. Treatment of Heart Failure
d. Treatment of Chorea
e. Treatment of Atrial Fibrillation
.a. GENERAL MEASURES:
1. Pain relief : Paracetamol and codeine can be used till diagnosis is settled and Aspirin must be
given after establishing the diagnosis.
2.Rest:: (No definite guidelines) Arthritis, Carditis & Chorea are indication for rest.

17

i.
ii.
iii.
iv.

v.

vi.

In absence of carditis and chorea, rest for 15-20 days.


Absolute bed rest is only needed if carditis is severe. Rest in presence of CHF must
be given till control of CHF otherwise for carditis without congestive failure rest can
be for 4-6 weeks. (Heart size may be simple and a better indicator)
Chorea requires restriction of activity to avoid accidents.
Sedatives are helpful in chorea and carditis with CHF to ensure rest
Children should be ambulated and monitored at home before going to school to
functional capacity and accordingly activity must be allowed.

assess the

In absence of carditis and chorea ambulatory restrictions can be relaxed once


arthritis has subsided.

3. Diet:: One should ensure adequate nutrition to growing child. Preventive measures like salt
restriction should be logically used. Enforcement of too rigid diet restrictions may lead to further
nutritional compromise.
4. Constitutional symptoms: Fever, mayalgia, loss of appetite and anemia improve with
management of RF and do not need specific measures.
5. Hospitalization:
i. If Diagnosis is not clear.
ii. To start secondary prophylaxis in group of patients carrying the high risk for allergic reactions like
history of atopic dermatitis, Asthma, reaction to any other substance.
Iii Acute Rheumatic fever with severe carditis, uncontrolled CHF, chorea and some times with severe
arthritis
iv. Patient is not doing well on appropriate treatment.
v. Patient having prolonged or unexplained fever.
vi. Illiterate patients /Patients with anxious parents: To make patient comfortable and to provide
adequate supervision and counseling to the family.

b.CONTROL OF INFLAMMATION (Therapy to be continued for 12 weeks)


ANTI INFLAMMATORY DRUGS 16,17,23,33--36
Treatment to be given for 12 weeks to prevent relapse & rebound.

Management of Acute inflammation in ARF:


Polyarthritis+/- mild carditis

Aspirin*
To

achieve

serum

level

Full doses:100 mg/kg/day for 2-3 weeks


optimum (Adult 6-8gm/day) divide in 4-5 doses .
of

20- Tapering doses :once symptoms resolved taper to 60-70

30mg/100dl.#

mg/kg/day. ( forOlder children 50mg/kg/day )$

Naproxen **(If Aspirin

10-20mg/kg/day

intolerance detected)
No response to Aspirin Then rule out other conditions like Chronic
in 4 days

inflammatory/ myelo-proliferative disorders before


switching over to steroids.

18

Moderate to

Steroids ***

Prednisolone : 2mg/Kg/d,max 80mg/day

severe Carditis

till ESR normalizes usually 2 weeks. Taper over 2-4


weeks, reduce dose by 2.5-5mg every 3rd day.
* Start aspirin 50-75mg/kg/d simultaneously ,to complete
total 12 weeks. (Aspirin Cross over Therapy)##

Non responders

Methyl Prednisolone23

If no response to oral steroid therapy then start IV methyl

(Intravenous)

Prednisolone 30mg/kg/day for 3 days

$Alternative Treatment : 50 to 60 mg//kg /day for full 12 weeks(IIb).


*. ** & *** Consider Antacids. Avoid Gastric irritants. Allow frequent feeding. Medicines must not be taken in empty
stomach. #Drug levels (Not realistic in most situations) Ask history of tinnitus daily early symptom of toxicity. ##
Prednisolone same doses x 3-4 weeks. Taper 5mg/wk to cover total period of 10-12 weeks (No Cross Over)

*This group of experts strongly believed that Aspirin can be given in doses of 50 to 60 mg//kg /day for
full 12 weeks with good response, better gastric tolerance and less chances of toxicity. Although this
observation has never been mentioned in literature. With this observation treating pediatrician/
cardiologist can use his or her own discretion. Aspirin dose can be upgraded in absence of predicted
response.
One must ask for black stools and other features of gastric intolerance. Medicine must not be taken
empty stomach. Antacids must be supplemented.
If possible children should get vaccinated for Influenza and chickenpox.
Monitoring for Rheumatic Activity:
Monitor ESR and CRP to monitor anti-inflammatory Response after 7 days , 15th day and 30th day
sand 3 months (rebound/relapse).
Monitoring for Drug Side Effects : Watch for complaints of epigastric pain, black stools or frank
blood in stool & steroid effects like Cushingoid facies. Patients on steroid have propensity to catch
infections or reactivation of dormant co-morbid condition like tuberculosis. Sever infection may go unnoticed. Parents must be counseled for these effects.
Prolonged steroid therapy has less chances of aspirin induced GI bleeding but may have growth
related issues. There is no additional advantage in terms of residual cardiac effects

Management of Chorea 17,23,37-40


Mild Chorea:
Quiet environment, reassurance

Sedative

like

Severe (life style limiting)


chorea:

Phenobarb, Haloperidol: 0.5-2.5 mg tid

diazepam is useful.

Valproate: 15 mg/kg/day

Chlorpromazine can be used

Carbamazepine (7-20 mg /kg/d)

19

Resistant Chorea:
Plasmapheresis
Pimozide

If features of rheumatic activity (ESR, CRP, ASO) are present treat with Anti-inflammatory drugs as
given above.
Treatment should be continued 2-4 weeks after clinical improvement.

c. Management of cardiac complications


1. Treatment of Heart Failure 17,23,41,42
Congestive heart failure may present with acute valvulitis or established valve diseases. It causes
major morbidity & mortality. A successful management of patients is possible in any secondary care
center.
i. Restrict physical activities to reduce or eliminate symptoms.
ii. Routine measures like daily weight record, continuous monitoring of vitals & input / output fluid
balance.
iii.In anemic patients blood Transfusion with Packed cell and Iron therapy must be recommended
Medical management of Congestive heart failure.
Digoxin

30 mcg/kg Total digitalization dose, 7.5 mcg/kg /day maintenance dose

Diuretics

Frusemide 0.5 2 mg/kg/day Metolazone:0.2-0.4mg/kg/po daily. Adults 2.5-10mg/day

ACE inhibitors

Captopril: 0.25 mg/kg: Test dose. Build up doses from 1.5mg/day to 3 mg/kg/day in three
divided doses.

Sodium Nitroprusside

0.5-10 mcg/kg/min infusion . Monitor cyanide level.

(Uncontrolled CHF)
Inotropes

Dobutamine 2-20mcg/kg/min infusion; Dopamine,2-20mcg/kg/min infusion;


Milrinone: 0.5-1mcg/kg/min infusion

Surgery

Severe Mitral regurgitation Due to chordal rupture leading to refractory CHF

2. ATRIAL FIBRILLATION 18,23,43


AF is a supraventricular tachyarrhythmia characterized by uncoordinated atrial activation.
AF clinically presents with irregularly irregular pulse rate (slow/fast/normal rate)
Electrocardiogram : is diagnostic & shows replacement of consistent P waves by rapid oscillations
or fibrillatory waves that vary in amplitude, shape, and timing, associated with an irregular, frequently
rapid ventricular response when atrioventricular (AV) conduction is intact. The ventricular response to
AF depends on electrophysiological (EP) properties of the AV node and other conducting tissues, the

20

level of vagal and sympathetic tone, the presence or absence of accessory conduction pathways, and
the action of drugs.
Treatment of Atrial Fibrillation
Associated with chronic Valvular heart disease. Clinical presentation :Irregularly Irregular pulse.
ECG: Fibrillatory wave .
Rate Control:

Digoxin, Ca++channel blocker: (Slows down the AV conduction. Before


starting the treatment Rule out accessory pathways). Beta blockers.

Rhythm control

Must be attempted in AF of recent onset or Hemodynamically unstable


patients. Without correction of valvular heart disease, rhythm control can
not be sustained.

Electrical:

Cardio-version.*

Pharmacological

Amiodarone. Flecainide

Anticoagulant

Warfarin to achieve INR of 2-3. Avoid Vitamin K containing food like green
leafy vegetables.

Surgery

Modified Maze procedure done during surgery

* Rule out left atrial clot and start anticoagulant before cardioversion
3. Chronic Valvular Heart Disease
Management 64-89 RHD primarily targets mitral and aortic valve and rarely tricuspid valve . Chronic
regurgitant lesions are better tolerated and can be medically managed for longer duration. Basically
management requires:
1.Good adherence to Benzathine penicillin prophylaxis preventing the second attack of ARF.
2.Diuretics (Thiazide or loop diuretic + /- K+ sparing diuretic like aldactone)
3.Vasodilators: Angiotensin converting enzymes inhibitors like Captopril.
4.Digoxin, most commonly indicated in presence of Atrial fibrillation &CHF
5.Management of associated arrhythmias. Commonest arrhythmia is Atrial fibrillation (see above)
6.Anticoagulation
Acute lesions or obstructive lesion may need immediate intervention.
Patients with surgical and cath intervention has to be followed up at long term basis for various issues
like secondary prophylaxis , anticoagulation, cardiac function and associated valvular lesion.
Valvuloplasties:
Most encouraging results are seem in patients with mitral stenosis only.
Mitral Stenosis:
Clinical features (ref:Table3)

21

Echocardiography trans thoracic or Tran esophageal is adequate for decision making.


Hemodynamic of Mitral Stenosis: Patients of mitral valve stenosis have high LA pressure and
pulmonary venous hypertension due to restricted flow across the mitral valve. Pulmonary arterial
hypertension & high RV pressure are late manifestations. Patients clinically present with history of
dyspnoea on exertion & nocturnal dyspnoea due to restricted systemic cardiac output & pulmonary
congestion. Atrial fibrillation sets in due to dilatation of left atria . Inefficient atrial contractions
secondary to AF predispose these patients for later thrombo-embolic events.
Mitral Valvuloplasties:
Type of mitral valvuloplasties:
Percutaneous done by
I. spherical balloon(Inoue balloon)
II. Double balloon(not common)
Surgical Valvotomy
I. Closed Mitral valvotomy
II. Open mitral commissurotomy

Indications of BMV :
BMV required after basal assessment

BMV required after excercise testing in


patients with MVA>1.5 (AHA guidelines 2006)
43

MVA <1 cm2 (Absolute Indication)


PASP>60mmHg
MVA 1-1.5 cm2 (if associated with raised PA PAWP>25mmHg
pressure)
MVG >15mmHg may need intervention.
PAH TR gradient>50mmHg
Episodic acute pulmonary edema
AF/ flutter(paroxysmal/persistent)
Embolism
Contemplating future pregnancy
Occupation that pose high risk to patient /public

Balloon Mitral Valvotomy:


MECHANISM
-Splitting of the fused
commissure toward the mitral
annulus
-Similar to -surgical mitral

CONTRAINDICATION
-LA clot (contraindication to BMV)
-Subvalvar apparatus: Unsuitable
Calcified valve
-Associated lesions of other

22

Successful BMV:
-MVA-> 1.5 cm2
-Increase in MVA->50 %
-< grade 2 MR
-Qp/Qs-<1.5

commissurotomy
-Balloon increases mitral valve
flexibility by fracturing calcified
deposits in MV leaflets

valves
-Commissural severe fibrosis
-MR grade III or more

-Immediate results: End


diastolic gradients < 4 mm
Hg in normal Cardiac
output.

Follow up: (see appendix)


6 month to yearly interval
Close watch for
1. Patients with suboptimal valvotomy or significant MR
2. Patient having additional valve lesions
3. Use of ACE inhibitors/ vasodilators in significant MR
4. Medical therapy : selected patients with decongestive, Digoxin
5. Anticoagulation: Patients with Atrial Fibrillation-INR 2- 2.5
6. Surgical intervention in symptomatic patients.
7. SBE prophylaxis

Surgical Intervention in Valvular Heart disease:


Indication of surgery in Mitral Valve Disease
Mitral Stenosis

Mitral Regurgitation

Indication:

ACUTE severe MR with

MVA <1 cm2 (Absolute Indication)


MVA 1-1.5 cm2 (if associated with raised PA pressure)
PAH TR gradient>50mmHg
Episodic acute pulmonary edema
AF/ flutter(paroxysmal/persistent)
Embolism
Contemplating future pregnancy
Occupation that pose high risk to patient /public.
TREATMENT MODALITY:
If suitable: Balloon mitral valvotomy otherwise surgery:
Repair/replacement

Uncontrolled CHF
-LVEF FALLING<55%
-.Fractional shortening falling < 30%
-.LVEDd: > 7.5 cm
-.LVEDs: > 5cm or 2.6cm/m2
--LVESV: >60ml/m2
-.Radius: wall thickness ratio at end systole multiplied by
systolic pressure: 195mmHg
-.PA pressure> 50mmHg
Treatment Modalities
MV repair: Early intervention
MV replacement : One must wait

Indicators of poor Outcome after MV surgery:


Low EF
Giant LA with AF
H/o Wide variation in INR

23

Aortic Valve
Isolated Aortic stenosis is less common than combined lesion.
If AS is isolated, think of pathology like Bicuspid AV.
Rheumatic AS : Result of balloon valvuloplasties are bad and valve replacement is choice. Ross
procedure is not a good choice in these cases & must be avoided.
Indication if intervention in Aortic valve disease
Aortic Stenosis
Aortic Regurgitation
Symptomatic patient
EF falling <40%
Mean gradient >40mmHg
LVES:<25 %
Aortic valve area <1.0cm2
LVEDd: 7 mm or 3.8cm/m2
Calcified valve
LVEDs: >5cm or >2.6cm/m2
AR is associated
Radius/ wall thickness ratio at end diastole
Presence Of Vegetations
multiplied by systolic BP >600mmHg
Ross J Jr: J Am Coll Cardiol 5: 811,1985
Treatment:
Repair / replacement
In patient showing symptom/systemic congestion, RV dysfunction
e. Treatment of Rheumatic Fever- Specific Points
Diagnosis must be established before initiation of treatment.
When CHF is intractable Invariably due to severe MR or AR. Valve replacement may be the only
chance of survival.
Aspirin and steroids suppress acute phase reactants, but do not modify the course of illness.
(Table :see Appendix)

24

Chapter III
LONG TERM MANAGEMENT OF ACUTE RHEUMATIC FEVER & RHEUMATIC HEART
DISEASE
1-Prevention of Recurrences: Secondary Prophylaxis (Section III)
2- Endocarditis Prophylaxis (SectionIV)
3. Management of Thrombo-embolism in RHD- Anticoagulation (Section V)
4. Intervention in valvular heart disease (Section VI)

1.Prevention of Recurrences
a. Secondary Prophylaxis 44-50
b.. Management of Anaphylaxis 51-52
a. SECONDARY PROPHYLAXIS 16,17.23
Definition : Secondary Prophylaxis (World Health Organization 2001)
Secondary prevention of rheumatic fever is defined as the continuous administration of
Specific antibiotics to patients with a previous attack of rheumatic fever, or well-documented
Rheumatic heart disease. The purpose is to prevent colonization or infection of the upper
Respiratory tract with group A beta-hemolytic streptococci and the development of recurrent
attack of rheumatic fever.
SIGNIFICANCE
Prevents recurrence of GAS infection, which can lead to recurrent ARF.
Reduces severity of RHD (can result in cure of RHD after many years).
Helps prevent death from severe RHD.
{RHD control programme (NHFA)}
Indications
ARF confirmed by the Jones Criteria
RHD confirmed on echocardiogram
Chorea
Points to be remembered:
a. Benzathine penicillin Remains the corner stone for Rheumatic Fever Prophylaxis.
b. ISOLATED ASO TITRE IS NOT A CRITERIA TO START SECONDARY PROPHYLAXIS.

25

c. Secondary prophylaxis is mandatory after cath or surgical intervention (2004 update of WHO
recommendation )
d. Patients with previous drug allergies must be treated more cautiously.
e. Benzathine penicillin should be continued during anticoagulant therapy. (except if INR is very
high.)
f. Allergic reactions to Benzathine penicillin are rare but may be fatal.(Alternative drug:
Erythromycin)
g. Anaphylaxis is a life threatening complication of BPG injection but it is not the only cause of
death. Death usually may occur after inadvertent IV injection leading to embolism and due to
vaso-vagal attack in patients with severely decompensated carditis. Occasional report of
Gangrene is most probably due to intra vascular injection)
Secondary prophylaxis in Pregnancy
Penicillin causes no risk to fetus during pregnancy.
Erythromycin can be continued during pregnancy to prevent ARF.
Doses of Benzathine Penicillin G
Injectable Benzathine Penicillin G most effective for secondary prophylaxis.
Deep intramuscularly
1.2 lakh for ALL people >=27 Kg every 21 days
6 lakh units for children< 27kg every 15 days.
Alternative Treatment
1. Penicillin V: is used in absence of Benzathine penicillin or if there is bleeding disorder.
Oral penicillin is not a very effective drug.
2. Erythromycin
Dose: ref table
Drugs preferably not to used for secondary prophylaxis
Benzyl penicillin IM/IV
Procaine Penicillin IM
Duration Secondary Prophylaxis
1.ARF (No carditis): Minimum of 5yrs after ARF or until 18 yrs age (whichever is longer)
2.Mild to Moderate Carditis (Healed Carditis): Minimum 10 years after last ARFor until age 25 years
(whichever is longer)
3.Severe RHD or following cardiac surgery:
Continue for life (WHO 2004 update) (Class 1 evidence).Second option is to stop it at the age of 40
years. (Class 2 evidedence)

Senstivity test for benzathine penicillin


Recommendations that Only standard extracts, which include major and minor determinants of
penicillin, should be used. e.g. Prepen (benzylpenicilloyl polylysine Hollister-Stier Laboratories,
United States of America) and the minor determinants include benzyl penicillin (penicillin-G),

26

benzylpenicilloate and benzylpenilloate. Unfortunately, This is difficult to comply in India where these
reagents are not available commercially .
Due to the fact that BPG is unsuitable for Intradermal injections sensitivity testing ,current IAP
recommendations are to use Benzyl penicillin for the skin testing. This method is not capable of
detecting all cases of possible penicillin allergy. Up to 4% of patients with a negative skin test to both
the major and minor determinants will develop non-life-threatening allergic reactions if they receive
penicillin again. A penicillin skin test predicts only the presence of IgE antibodies for the major or
minor penicillin determinants at the time of application and does not predict the future development of
IgE-mediated reactions during subsequent courses of penicillin. A penicillin skin test does not predict
non-IgE-mediated reactions caused by other immune mechanisms, such as cytotoxic antibodymediated reactions, antibody-antigen immune complex-mediated reactions, and delayed-type cellmediated reactions.
How to do senstivity test
Benzyl Pen 10,000 U/ml to be given for sensitivity test. Prick test must be used before intra dermal
test for the patients getting their first injection (A drop of Benzyl Penicillin 10,000/ml to be kept on
forearm volar surface- scratch with bifurcated needle).Then Intradermal test with both Benzyl pen
and control saline must be done (approximately 0.02ml at volar surface of fore arm or lat surface of
arm). A wheel 2 mm more than control or 4 mm more than initial edema must be taken as positive
test. Rest of injections must be preceded by Benzyl pen Intradermal test.: ( Test reading time:1530min)
(Control saline helps in recognizing the initial oedema due to intra dermal injection, if available, use of
histamine is helpful to recognize person incapable of producing skin response. )
The patient should not have taken antihistamines recently (e.g., chlorpheniramine maleate or
terfenadine during the preceding 24 hours, diphenhydramine hydrochloride (HCl) or hydroxyzine
during the preceding 4 days, or astemizole during the preceding 3 weeks).
Administration of benzathine penicillin
a. Site of injection
Deep intramuscular injection into upper and outer quadrant of buttock or anterolateral thigh (can be
converted in two injections at two different site)
b. Check medication and expiry date
c. Use 23 gauge needle if 12 lakhs is diluted in 6 ml water for injection.
c. Can be given through 20 G needle if 12lakhs is diluted in 3 ml of sterile water.
d. Benzathine penicillin is poor water-soluble drug. It should properly shaken after taking it in syringe.
Needle should be cleared and immediately prick must be done into deep muscular tissue. One must
withdraw the piston to detect any amount of blood. Once sure, push the drug without changing the
position of tip of needle to avoid any vascular access. Benzathine penicillin containing syringe is
prone for both- stuck needle and stuck syringe. One should not try to re-adjust needle position. Bring
it out and again check and prick.
e. Dispose used needles and syringes in puncture proof container.
Patient must be enquired about pain any nodule at the site of injection and h/o limping or weakness of
limb.
Secondary prophylaxis is a prolonged process. Every attempt should be made to take family in
confidence. Risk of anaphylaxis, injection related pain and risk of avoiding it must be explained in

27

detail. It might be a good idea to start secondary prophylaxis in second or third visit and to start in
presence of two doctors. It may be a good idea to get it endorsed by senior most Person from the
set-up and at least first injection to be given in a place, which is fully equipped with life saving drugs
and equipment.
Pain Reduction
a. Warm cold syringes to room temperature between hands
b. Apply gentle pressure for 10 seconds with finger or thumb before injection
c. Ensure skin swabbed with alcohol is dry before injection
d. Deliver the injection slowly over 2-3 minutes
e. Encourage movement before injection
Ceasing Secondary prophylaxis
Date for ceasing should be recorded
Assessment of patient should be recorded
Time since last ARF illness
Specialist review
Echocardiogram
b. Anaphylaxis:
a. Sudden and generalized reaction to the injection or ingestion of some antigen to a previously
hypersensitive individual.
b. Anaphylactic reaction: Occur at any age
c. Incidence: 0.004 to 0.04% in patients treated with penicillin- (Kucers & Bennett, 1987)
d. 0.001% of patients die due to anaphylaxis after use of penicillin ( 1/3 have previous history of
reaction to the drug)
e. Most commonly seen with Parenteral injection;
f. Rare with ingestion and after Intradermal skin test
Management of anaphylaxsis in the outpatient clinic
Clinical features:
The manifestations are many and variable.The most dramatic and constant feature of this process is
the rapidity of onset.

PREMONITORY SYMPTOM
Frequent.
commonest are not feeling well, itching
,around
the
eyes,
sneezing,
hoarseness of voice, anxiety and
lacrimation

SYSTEM SPECIFIC SYMPTOM


POTENTIAL FATAL REACTION
Respiratory:
Wheeze,
Acute Angioedema of tongue, acute laryngeal
pulmonary edema, laryngeal edema
edema, cardiovascular collapse, Acute
pulmonary edema, Acute severe asthma.
Cardio Vascular: Vasodilatation,
Increase in capillary permeability,
Vasoconstriction, Hypovolumic shock
Cutaneous:
Diffuse
erythema,
pruritus,urticaria, angioedema.

28

Principles in Management of Anaphylaxis(Detailed guidelines Ap: table24)


Key to successful resuscitation is anticipation and the preparedness for this eventuality. It is life
threatening but is imminently reversible.
Success depends on the promptness in detection and instituting treatment
The key drug is Adrenalin and not steroids.
Occasional patient will need intubations for airway management
Out patient facility

Personnel: At least one nurse trained in cardiac resuscitation in addition to the physician.
Drugs: Adrenalin (1: 1000) loaded in 2 ml syringe
Adrenalin (1: 10,000) loaded in 10ml syringe
Venous access: Before the test dose is administered or be ready for IM injection. Given at
right time equally effective
Volume replacement : Normal saline,Haemaccel, Haestril-6%, 10% dextrose.

Steps of resuscitation in severe anaphylaxis (See table24 in appendix)


Administer adrenalin*
Adults: 0.5 ml of 1:1000 adrenalin- IM/SC
3 to 5 ml of 1:10,000 adrenalin-IM or IV
Children: 0.1ml/kg(max0.3ml/kg/dose) of 1:1000 adrenalin-IM/SC
Repeat dose can be given at 5 minute interval.
Administer Volume-. Colloids are better than crystalloids in the setting of leaky capillaries(weight
appropriate volume)
-**CPR should be initiated as and when necessary.
-Brochospasm: Adrenalin, Steroids, Salbutamol inhalation, IV Aminophylline.
-Angioneurotic edema: Adrenalin, Antihistaminic
-Pulmonary edema: Difficult to manage, will need ventilation**,

CONCLUSION 44-52
-Anaphylaxis is a rare but dreaded complication with Benzathine penicillin which is completely
reversible if managed with timely adrenaline , volume replacements and rarely with active

29

resuscitation. Hence -Anticipation, preparedness and promptness is the key to successful


resuscitation.

2. Prevention & Management of Infective Endocarditis17,23, 53(Ap : Table16-18)


IE is a serious complication, if untreated, is often fatal. Lifetime risk of IE is 308-440 per 100,000
patient years for native & prosthetic valves. Emerging antibiotic resistance to IE pathogens make its
management more challenging. It can attack native valve as well as prosthetic valve leading to
complication like embolization, mechanical obstruction, abscesses at intra and extra cardiac sites
paravalvular leaks in post op patients.
Penicillin does not protect against infective endocarditis.
(See appendix tab:14-18 for details)
Risk stratification for IE
Highest risk
Prosthetic heart valves
Previous episodes of IE
Complex cyanotic CHD

Moderate risk
Acquired valvular heart disease (RHD)
Residual lesion after valve repair
Others

Endocarditis prophylaxis includes :


1.Use of prophylactic antibiotics before certain procedures
2. Routine Measures:
Adequate use of antibiotics must be under supervision of pediatrician and with strong indications.
Use of total counts, blood cultures and CRP when strong doubt of infection.
Strict prohibition of SELF MEDICATION.
Promotion of general oral hygienic measures like brushing of teeth, twice, particularly at morning
and bed time. Use of mouth wash., avoidance of frequent feeding without proper cleaning of mouth.
Cardiac condition associated with risk of endocarditis (Detailed list see appendix)
-Bacteremia resulting from daily activities is much more likely to cause IE than bacteremia with a
dental procedure.
-Only a small number of cases of IE can be prevented even if prophylaxis is 100% effective
In general, IE prophylaxis is recommended for patients with the highest risk for adverse outcome
(AHA/ ACC Updated Document for Prevention of IE 2007)See tables in appendix for detailed
recommendations.
Evaluation for Infective Endocarditis:
Following signs and symptoms in a patient with prolonged, unexplained fever (>48hrs)and the
valvular abnormalities indicates IE:

30

Clinical Indicators of Infective Endocarditis


Non-Cardiac Symptom:

Cardiac Symptom:

1.Fevers, Arthralgia, Myalgia, Clubbing, Splenomegaly -Appearance of new murmur


-CHF
2. Musculo-skeletal symptoms of arthritis and back -Conduction disorder.
pain.
3. Emboli with infarction of spleen, kidney, bowel and
brain
4. Immunological phenomenon:
-Glomerulonephritis
-Oslers node
-Roths spots
-Embolic episode to different parts of body.

Confirmation of Infective Endocarditis.


(Refer the patient where culture facilities are available)
At least two sets of blood cultures should be taken from different sites prior to starting antibiotics
Transoesophageal echocardiography may be necessary for diagnosing prosthetic valve endocarditis
.Before starting: Take a detailed history general and treatment in particular history of recent
procedure.
Clinically look for features of rheumatic activity
Record basal RR, HR, BP
Any focus of infection any where in body, rashes, petechiae.
Investigate: Total count, ESR, CRP, ASO, Renal/ Liver function test, Coagulation profile.
Detailed echo.
Evaluation should be detailed and complete. Association of rheumatic activity must be ruled out.
Before starting therapy, drug allergies, previous treatment (adequate or inadequate) kidney and liver
functional status (Many
drugs might be toxic in presence of dysfunction.), LVEF, nature of disease and detailed record of
surgery must be known to decide about antibiotic and supportive therapy.

31

Treatment of infective endocarditis


Bacteria
Streptococcal Infection

Native valve
IV Penicillin G 200,000 U/kg/d in 46doses
/Ceftriaxone
sodium100mg/kg/d
once daily +
gentamicin3mg/kg/d in 2-3 dose
x 4weeks
If
allergic
to
penicillin:
IV
Vancomycin40mg/kg/d in 2 doses +
gentamicin
B.Staphylococcal Infection Oxacillin/Nafcillin 200mg/kg/d IV in 4-6
doses
for 4-6 weeks
(+Gentamicin for first 3-5 days)
If allergic to penicillin: Vancomycin
in place of oxacillin/Nafcillin
Enterococcal Infection
Penicillin G + Gentamicin for 4 weeks
If
allergic
to
penicillin/penicillin
resistant:
Vancomycin+Gentamicin for 6 weeks

Prosthetic valve
IV Penicillin G/Ceftriaxone sodium +
gentamicin x6 weeks (same dosage)
If allergic to penicillin: IV Vancomycin +
gentamicin

Treatment of
Negative
Endocarditis

Prosthetic valve (< 1 year old)Vancomycin +


Gentamicin + Cefepine + Rifampin for 8 weeks
(Cefepime dose: 150mg/kg/d in 3 doses)
Prosthetic valve (> 1 year old)
Ceftriaxone + Gentamicin/Rifampin for 8 weeks
If suspecting Bartonella, add Doxycycline 24mg/kg/d

If
suspecting
infection:

Culture Vancomycin
+
gentamicin
+
Infective Ciprofloxacin
for
4-6
weeks
(Ciprofloxacin dose: 20-30mg/kg/d
IV/PO in 2 doses)

fungal AmphotericinB IV
PO

Gentamicin to be given for first 2 weeks


Add Rifampin 20mg/kg/d IV/PO in 3
doses
Duration of treatment: 6-8 weeks
Linezolid 20mg/kg/d IV in 2 doses for 8 weeks
Imipencin/cilastatin+Ampicillin for 8
weeks

+ flucytosine AmphotericinB IV

+ flucytosine PO

Summary of treatment of endocarditis prophylaxis

Crystalline Penicillin is still a very effective antibiotic for streptococcal and enterococcal
endocarditis
For those allergic to penicillin/cephalosporines, vancomycin is the best alternative (if renal
parameters are normal)
Prosthetic valve endocarditis is difficult to treat, carries high mortality. Rifampin should be
added to antibiotic regimen and duration of treatment should be longer (8 weeks)
For culture negative endocarditis treatment should start with vancomycin plus gentamicin
Outpatient IV or oral antibiotic treatment for IE ?; till now no role.

Indication for surgery


a. Homodynamic deterioration/heart failure
b. Fungal endocarditis
c. Uncontrolled infection

32

d.
e.
f.
g.
h.

Heart block
Annular abscess/destructive lesions
Dehiscence of prosthetic valve
Recurrent embolisation
Large, mobile vegetations (> 10 mm in size)

3.anticoagulation in RHD - Management of Thrombo-embolism in RHD 54-62


Anticoagulation is an integral part of management of long term management of RHD for both pre-op & post op patients.
In pre-op patients it is required in atrial fibrillation as these patients are prone fo thromboembolic episodes. .

Anticoagulation for Prosthetic Heart Valve (See appendix tab 9-12)


Recommendations;
a. Lifelong for all patients with mechanical valves
b. For three months after bioprostheses implantation
c. For three months after mitral valve repair involving use of prosthetic annuloplasty ring
Poor anticoagulation control with high variability of INR (International Normalized Ratio) is the
strongest independent predictor of reduced survival after valve replacement
Anticoagulation for Prosthetic Heart Valve
Important issues in India
a. Poor compliance
b. Lack of facilities in close proximity to monitor INR
c. Lab errors for calculating INR
Anticoagulation for Prosthetic Heart Valves:
Mechanical Valve(Lifelong)
Recommended INR
MVR
2.5-3.5
AVR
2.0-3.0
Bioprosthesis (for 3 months)
Recommended INR
MVR/AVR
2.0-3.0
No consensus on routine use of Aspirin

Anti coagulation Management During Non Cardiac Surgery:


For short procedures like dental extraction, INR up to 2.0 is acceptable
If low risk of thrombosis (e.g. AVR), warfarin may be stopped 48-72 hours before and started 24 hours
later
For cases with MVR, interruption of oral anticoagulants is required and heparin is used in the interim
period.
No definite data on efficacy of low molecular weight heparin

33

Interruption of Anticoagulation for Non Cardiac Surgery


-2 day
Stop Warfarin & Start UF Heparin I.V.
O day

Stop Heparin 4-6 hrs before Non cardiac surgery


& Restart Heparin 6 hrs later

1 day
3-4 day

Start Warfarin
INR 2.5, stop IV Heparin

Prosthetic Valve Thrombosis


Serious complication, not uncommon
High mortality if not treated promptly
Patient is often in NYHA class III-IV
Less dramatic presentation with bileaflet prosthetic valves (e.g. St. Jude)
An INR of > 2.0 does not rule out the diagnosis

Management of Prosthetic Valve Thrombosis( Appendix tab13)


Emergency surgery if NYHA class III or IV
Fibrinolytic therapy (IV streptokinase) for;
- NYHA class I or II
- Surgery high risk or not available
- Small clot burden
Disadvantages of fibrinolytic therapy
- Cerebral embolism in 12-15%
- Major bleeding in 5%
May be ineffective
Care of Prosthetic Heart Valve (For follow-up protocol see appendix)
Patients/ parents education about anticoagulation, recognition of symptoms of valve thrombosis etc.
Patients receiving penicillin for secondary prevention of ARF require additional antibiotic coverage for
IE prophylaxis
Antibiotics for treating IE should be based on culture report or circumstantial evidence
Anticoagulation for prosthetic heart valves presents many logistic difficulties in developing countries
Valve repair surgery has the advantage of not requiring anticoagulation.
Embolization in RHD
I.
Thrombus: leading to infarction of affected organ like brain & kidney
II. Vegetation: Leading to Infarction and abscess of affected organ.

34

If thrombus or vegetations are seen one must be aggressive in therapy. Prosthetic valve thrombus or
vegetation may need urgent surgery. CNS event may be life threatening hence hospitalization is
essential.

Chapter IV
Guidelines for community out reach
Guidelines for community out reach and collecting Epidemiological Information for Rheumatic
Fever and Rheumatic Heart Disease:
A number of studies have attempted to document RF incidence and RHD prevalence in India. School surveys are
traditionally considered useful for this purpose because the denominator is clear. However school surveys have serious
limitation due to possible non inclusion of susceptible population due to school drop outs or sick children not attending the
school and poor staying at home.
It is important to recognize, however, that data from these highly selected regions are not perhaps representative of the
country as a whole. The parts of the country with the highest prevalence today are also those regions with the poorest
health care infrastructure. Most comparative studies have reported a substantially higher prevalence in rural regions.
Because of serious limitations in the health care delivery systems, the magnitude of the problem remains unrecognized in
many poorly served regions. Very few systematic surveys are available from rural populations with a poor health care
infrastructure, urban slums and tribal colonies. Given the extraordinary variations in indices of human development across
the country and even within regions, it is difficult to make generalizations for the entire country
This observation was endorsed by all participant in meeting . Our group of experts have proposed verification of data from
the states having low per capita income.
Guidelines for Community out reach:
To take RHD prevention program to community levels: Use of existing Health care system.
Role of Government:
Role of Medical colleges/Civil hospitals and associated ancillary services
Role of IAP.:
Role of Paramedics:
Role of Government:
-Accept RHD prevention
programme
-Set-up goals & budget
allocation
-Sensitize health care
system
-Procure BPG & ensure turn
over
Cardiac patients must be

Role of Medical colleges/Civil


hospitals and associated
ancillary services:
-To run RHD prevention
programme.
-To run regular rheumatic
clinic frpm pediatric deptt.
Training progrmme for
medical & paramedical staff. To sensitize them for
secondary prophylaxis.
-Research/ thesis at 5-7 year
interval or creation of
registries

Role of IAP & Pediatrician

Role of Paramedics:

-To ensure the participation


at all levels.
-To improve awareness by
publications.
IAP branches can bring out
their programme for helping
the patients.
Role Of Pediatrician:
Only pediatrician can
successful run supervised
RHD prevention programme

Must know
-doses and how to dilute the
Benzathine penicillin
- be trained for IM injection
and learn precautions.
- how to do sensitivity test.
-be trained in recognizing and
managing Anaphylaxis.
-t be trained to handle life
support .
-Record Keeping.
-RHD card: entry of every inj
like in Vaccination./INR
monitoring Card

considered as physically
handicapped and given
facilities for treatment.
Introduce insurance
policies.

35

APENDIX
:ACRONYMS AND ABBREVIATIONS
2DE

two-dimensional echocardiography

anti-DNase B

anti-deoxyribonuclease B

ARF

acute rheumatic fever

ASO

anti-streptolysin O

AVA

aortic valve area

BP

blood pressure

BPG

benzathine penicillin G

CRP

C-reactive protein

ECG

electrocardiogram

ESR

erythrocyte sedimentation rate

GAS

group A streptococcus

HR

heart rate

INR

international normalised ratio

IVIG

intravenous immunoglobulin

LMWH

low molecular weight heparin

LVEF

Left ventricular ejection fraction

LVEDD

ventricular end diastolic diameter

LVESD

ventricular end systolic diameter

LVOT

ventricular outflow tract

MVA

mitral valve area

NHFA

National Heart Foundation of Australia

NSAIDs

non-steroidal anti-inflammatory drugs

NYHA FC

New York Heart Association Functional Class


associated with streptococcal infections

PAS

pulmonary artery systolic (pressure)

PBMV

percutaneous balloon mitral valvuloplasty

RHD

rheumatic heart disease

RR

respiratory rate

Str

Streptococci

TDD

Total digitalization dose

UFH

unfractionated heparin

ULN

upper limit of normal

velocity

WHO

World Health Organization

INR: International Normalized Ratio = (x/y)z , where x = Prothrombin Time of sample (sec)
y = Mean Normal Prothrombin Time (sec) ;z = [ ISI of Thromboplastin}]

36

Table I: Rheumatic Fever/Rheumatic Heart Disease status in India

79-83

ICMR

Delhi

Year
19821990

Age (yrs)
515

Population studied
13,509

Prevalence (per 1000)


2.9

Padmawati
Grover et al
Lalchandaniet al
Jose and Gomathi
et al

Delhi Urban
Rajpurrani
Kanpur
Vellore

1984-1994
1988-91
2000
2001-2002

5-10
5-15
7-15
5-18

40,000
31,200
3953
229,829

3.9
2.1
4.5
0.68

Soman et al**

Ernakulam

2002-04

5-16

25 033

0.12*

Author

Place

Mishra et al

Gorakhpur

2003-2006

4-18

118,212

0.5

* These studies used strict diagnostic criteria (echocardiogram confirmation of diagnosis)

** Unpublished data of the Jaivigyan ICMR rheumatic fever and rheumatic heart disease in Ernakulam district

Table2:acute rheumatic fever and rheumatic heart disease


Definitions (As laid down by WHO):
Rheumatic Fever: A new (current) case with acute illness which fulfills the Jones criteria(revised 1992) with or without
cardiac disease.
Rheumatic Heart disease: A new or old case without rheumatic activity with a valvular lesion confirmed either by
reliable auscultation or by echocardiography.
Past history of Rheumatic Fever: A case without valvar lesion with a past history of documented RF(confirmed) or a
past history suggestive of RF but undocumented.
Incidence of RF: Total number of new case with RF(with or without cardiac involvement recorded in a period of one
year per 1.00.000 of the specific population group usually school children )
Prevalence of RF: Total no of old cases with documented past h/o RF(without a valvar lesion) and total number of new
cases with RF(without valvar lesion) per 1000 in a selected area, period of time and specific population group usually
school children.
Prevalence of RHD: Total no of all cases with confirmed RHD and total number of new cases with RF(with cardiac
involvement) per 1000 in a selected area, period of time and specific population group usually school children.
Prevalence of RF/ RHD:Total no of RF plus RHD cases per thousand population.
Group A streptococcal throat infection: Case with throat illness and positive culture for GAS organism.
Compliance in Prophylaxis: Patient who received atleast 90%of long acting Benzathine penicillin injections due per
year is taken as compliant. Centre compliance is reported as the percent of total no of compliant pt/ the total no of
registered pts.

37

Diagnosis of RHD
Reliable auscultation is sufficient for the diagnosis of RHD in most of cases. However , some case may require
confirmatory echo.
Definite: Isolated MS. MS/M R. MS or MR with AR. Isolated MR with documented h.o ARF. Isolated MR(echo)
Probable: Isolated AR(Bicuspid AV excluded on echo).Clinical exclusion of Marfans syndrome.
Suspected: Rest of case can be referred to cardiologist for echo confirmation.
All suspected valvular lesion in absence of special clinical setting like Marfans syndrome should be taken as rheumatic
and should be subjected to further investigation.

Table3: Investigations:
White blood cell count/ Erythrocyte sedimentation rate/ C-reactive protein
Blood cultures if febrile
Electrocardiogram (repeat in 2 weeks and 2 months if prolonged P-R interval or other rhythm abnormality)
Chest x-ray if clinical or echocardiographic evidence of carditis
Echocardiogram (consider repeating after 1 month if negative)
Throat swab (preferably before giving antibiotics) culture for group A streptococcus
Anti-streptococcal serology: both anti-streptolysin O (anti-DNase B titers) if available (repeat 1014 days later if first test not
confirmatory)
Rapid group A strep Ag tests : Can be done in 5 minutes with specificity of 98%.All rapid group A strep tests require a sample from
the infected patient's throat. The sample is obtained by depressing the tongue and swabbing the back of the throat and tonsils,
while avoiding the tongue, saliva and lips. Swabs made of rayon or Dacron should be used. Swabs containing cotton, calcium
alginate, or wooden shafts, or that have been placed in transport medium containing charcoal are not recommended.(Not available
)

Table 4:Clinical features of Congestive heart failure:


Tachycardia
Tachypnoea
Venous congestion
Right-sided
Left-sided
Hepatomegaly
Tachypnea
Ascites
Retractions
Pleural effusion
Nasal flaring or grunting
Edema
Rales
Jugular venous distension
Pulmonary edema
X-ray: cephalization of upper lobe
pulmonary veins.
Kerley A,B,C lines, Pulmonary oedema

38

Low cardiac output


Fatigue or low energy
Pallor
Sweating//Cool extremities
Poor growth
Dizziness
Altered consciousness
Syncope

Table 5.Clinical diagnosis of Valvular Heart Disease


Findings
S1
S2

MS
Loud
Loud P2
Normal S2

S3
S4
Murmur
Syst/Diastolic
Radiation of
murmur
Best heard on
precordium
Chest X-Ray
findings

ECG findings

MR
Soft/Absent
Wide Variable split
(A2 advances)
Low pitch

If Ac Severe MR +
Opening snap followed Holosystolic murmur
by delayed diastolic
+MDR
murmur with pre
systolic accentuation
Axilla
At apex pt in Lt lat.
position

AS
Narrow paradoxical
split
If LV H
S4+(severe AS)
ESM

AR
soft
A2 delayed or
accentuated
S3 s/o Significant LV
dysfunction
High pitch blowing
Decrescendo+ ESM
+Graham steel M

Carotid Artery

Peripheral signs of ARcollapsing pulse


2nd/3rd ICS
Third ICS. Increased by
pt leaning forward with
breath held expiration
Rounding of LV apex Apex down and to left
.Post stenotic
Asc Aorta and Aortic
dilatation of aorta
knob are moderately
Valve calcification
dilated

Apex increased by
isometric strain
decreased by Valsalva
Straightening of left
LA enlargement
Heart border Prominent Pulm venous
MPA .Prominent upper congestion. Interstitial
lobe veins.
edema
Kerley s B line
Valve calcification
P Mitrale, RVH
LA enlargement.AF may LVH LV strain pattern LVH
be present+
T inversion Lead1AVL LV strain+
LVH+LAD
Lt Precordial lead
QRS prolongation.

Table6.New York Heart Association (NYHA) Classification:


Class I: patients with no limitation of activities; they suffer no symptoms from ordinary activities.
Class II: patients with slight, mild limitation of activity; they are comfortable with rest or with mild exertion.
Class III: patients with marked limitation of activity; they are comfortable only at rest.
Class IV: patients who should be at complete rest, confined to bed or chair; any physical activity brings on discomfort and
symptoms occur at rest.

Table7:Echocardiographic Evaluation Of Regurgitant Lesion(Subclinical Carditis)


AORTIC REGURGITATION
Colour:
substantial colour jet seen in 2 planes extending well beyond* the valve leaflets
Continuous wave or pulsed Doppler:
holodiastolic with well-defined, high-velocity spectral envelope
MITRAL REGURGITATION Colour:
substantial colour jet seen in 2 planes extending well beyond* the valve leaflets
Continuous wave or pulsed Doppler:

39

holosystolic with well-defined, high-velocity spectral envelope

Table8 A The prevalence of physiological valvular regurgitation in normal people:


Valve
Mitral regurgitation
Aortic regurgitation
Tricuspid regurgitation
pulmonary regurgitation

Percentage (%) of normal people


2.445%
033%
6.395%
21.992%

Physiological regurgitation is characteristically localized at the region immediately below or above the plane of valve
leaflets (or within 1.0 cm), and the signals are short and the maximum regurgitant area small.
The appearance of physiological valvular regurgitation in healthy subjects with structurally normal hearts varies with the
devices, sensitivity, penetration power and techniques used, with changes in systemic and
pulmonary vascular resistance and pressure, and with body habitus and age.

Table 8 B : Valvular involvement in RHD


MV involvement

92- 95%

Aortic Valve involvement (+MV)

20-25 %

Isolated Aortic valve involvement

5-8%

Tricuspid valve

7-9 %

Pulmonary valve

Very rare

Table9.Warfarin Sodium: Monitoring and Dosage Adjustment(Use standardized lab for the INR
monitoring)

INR

Action

>10.0

Stop warfarin. Contact patient for examination.

7.0-10.0 Stop warfarin for 2 days; decrease weekly dosage by 25% or by 1 mg/d for next week (7 mg total); repeat
PT in 1 week.
4.5-7.0 Decrease weekly dosage by 15% or by 1 mg/d for 5 days of next week (5 mg total); repeat PT in 1 week.
3.0-4.5 Decrease weekly dosage by 10% or by 1 mg/d for 3 days of next week (3 mg total); repeat PT in 1 week.
2.0-3.0

No change.

Increase
1.5-2.0 weWeekly dosage by 10% or by 1 mg/d for 3 days of next week (3 mg total); repeat PT in 1 week.

Increase
<1.50 weWeekly dose by 15% or by 1 mg/d for 5 days of next week (5 mg total); repeat PT in 1 week.

40

Flow chart 1 : Management Plan Of Acute Rheumatic Fever

(ICMR guidelines )
Suspected RHD

Hospitalize if diagnostic dilemma, Chorea, Carditis, severe arthritis, Family


councilling, First injection of Benzathine pen(duration see pg )

Carditis

Poly Arthritis

Salcylates
100mg/kg
72hrs Dramatic
improvement
Contd x 2wks
Taper: 5080mg/kg(upto12 wks)
Monitor
for Rh.
Activity
/rebound
NO

Onset of carditis Do
echo if available

Chorea

Yes ------------------

Monitor Carditis

No
Carditis

Mild

carditis

Echo if required

Steroid 2-3 weeks+


start supportive Tt
Overlap Salicylate
Taper steroids after 2 wks(2.5 to 5 mg
every third day)
Next 2 wks over lap
The Steroid + salcylates(50-80 mg/kg)
Total duration:12 week
Salicylate: complete 12 weeks of
therapy

Monitor for activity rebound

SECONDARY PROPHYLAXIS

No
carditis

Mod to severe

Salcylate
(same as in arthritis)

Sedative Halperidol (0.5


1gm oral) see text

Table11: Drugs affecting INR


Can increase INR:
Erythromycin,Clarythromycin
Amiodarone, Propafenone
INH/ Clofibrate, simvastatin
Fluconazole, Metronidazole
NSAID, Thyroid hormones
Herbal product:/Ginger ,Garlic
Fish oil/Vit E

Decreased INR:
Carbamazepine,Barbiturates
Rifampicin
Green pea
Phenytoin first increases then decreases the INR

41

s
.Dietary restriction is utmost important once the anticoagulants are started. Vegetables particularly rich in Vit K counter the effect
of Warfarin which has its action through vit K inhibition. If we know the vit K content of food Item we can make a flexible menu by
adjusting the dose of warfarin . Making food more palatable is important for growing child whos nutritional requirements are
much more than adults. For values see : home page www.cardioiap.org
Table 12:Clinical Evaluation Prosthetic heart valve:
Aortic prosthetic valves:
Accentuated Sounds(Inflow valve-S1 ; Outflow valve S2)
Ejection Systolic Murmur: All prosthetic AV have ESM because of their smaller orifice size. Loudest sound is produced by
Caged ball and small porcine valves.
Low Grade diastolic murmur : Tilting disc valves and bileaflet valves do not occlude their outflow tract completely when
closed, allowing some back flow.
. Suspect prosthetic aortic valve failure in a patient with Muffling of sounds, a greater than 2/6 diastolic murmur. Caged ball
and tissue valves cause no diastolic murmur since they completely occlude their outflow tract in the closed position.
Consider any degree of diastolic murmur in these patients pathologic until proven otherwise.
Mitral prosthetic valves:
Low Grade Systolic murmur:Caged ball valves may cause a low-grade systolic murmur due to the turbulent flow caused by
the cage projecting into the left ventricle. Consider any holosystolic murmur greater than 2/6 pathologic in a patient with an
artificial mitral valve.
Short diastolic murmurs: may be heard with bioprostheses and, occasionally, with the St. Jude bileaflet valve. These are
best heard at the apex with the patient in the left lateral decubitus position.
Absence of a normal valve closure sound (clicks) or presence of an abnormal regurgitant murmur is an important clue to the
presence of prosthetic valvular failure.
Subacute valve failure may present with hemolytic anemia or gradually worsening of CHF.
Patients with acute valvular failure present with cardiogenic shock and severe hypotension.
Evidence of poor tissue perfusion is present, including diminished peripheral pulses, cool or or mottled extremities, confusion
or unresponsiveness, and decreased urine output mottled extremities, confusion or unresponsiveness, and decreased urine
output.

42

Table 13:Thrombolytic Therapy for prosthetic valves


Diagnosis: clinical, Fluoroscopy & echo : Stop regular anticoagulant
Streptokinase 250,000 IU over 30 min
Streptokinase 100,000 IU /hr
OR
Urokinase: same dose
OR
Recombinant tissue-type plasminogen activator (rt-PA): 100 mg over a period of 25 h
Monitor clot (Echo), Fibrinogen and fibrin degradation product.
No Improvement over 24 hrs: Surgery after 24 hrs or 2 hrs later after neutralization with protease inhibitors.
If thrombolysis is successful: Heparin infusion is started (APTT upto two fold of control)
Followed by conversion to oral anticoagulation
Combined with aspirin (100 mg/. day
INR is adjusted to 34 for aortic and 3.54.5for mitral prosthesis .
In Complete Thrombolysis in a stable patient: S.C heparin +oral anticoagulation can be given.

Table14::X- Ray Evaluation of Prosthetic valve:


X- Ray AP view and lateral (Deep penetrating)view
Identifying valve in X- ray chest (Mehlman ,1988).
Starr-Edwards caged ball valve Radiopaque base ring /Radiopaque cage Three struts for the aortic valve;

4 struts for the mitral or tricuspid valve

Silastic ball impregnated with barium that is mildly radiopaque (but not in all models)

Medtronic-Hall tilting disc valve Radiopaque base ring Radiopaque struts that project into base ring:

3 small ones and 1 large hook-shaped one Occluder disc that is mildly opaque

But often cannot be seen. The base ring and two struts are radiopaque.

St. Jude medical bileaflet valve Mildly radiopaque leaflets are best seen when viewed on end. Seen as radiopaque lines
. Base ring is not visualized on most models. when

the leaflets are fully open

The valve may not be visualized on some radiographs.

Carbo Medics bileaflet valves: Valve housing and leaflets are radiopaque and easily visible.
Carpentier-Edwards porcine valve: The tall serpiginous wire support is the only visualized portion.
Hancock porcine valve :The radiopaque base ring is the only visible part in some models.

Other models have radiopaque stent markers with or without a visible base ring.

43

Table15:SBE prophylaxis recommendations in RHD


Endocarditis prophylaxis recommended

Endocarditis prophylaxis not recommended


Negligible-risk category (no greater risk than the general
population
*Mitral valve prolapse without valular regurgitation1
*Physiologic, functional, or innocent heart murmurs

High-risk category
*Prosthetic cardiac valves, including bioprosthetic and
homograft valves
*Previous bacterial endocarditis
Moderate-risk category
*Acquired valvular dysfunction (eg, rheumatic heart
disease)

*Previous rheumatic fever without valvar dysfunction


*Cardiac pacemakers (intravascular and epicardial) and
implanted defibrillators

*Mitral valve prolapse with valvular regurgitation and/or


thickened leaflets

Table 16: SBE recommendation for Dental Procedure


Endocarditis prophylaxis recommended1

Endocarditis prophylaxis not recommended

*Dental extractions

*Restorative dentistry2 (operative and prosthodontic) with or without

*Periodontal procedures including surgery, scaling and root planing,

retraction cord3

probing, and recall maintenance

*Local anesthetic injections (nonintraligamentary)

*Dental implant placement and reimplantation of avulsed teeth

*Intracanal endodontic treatment; post placement and buildup

*Endodontic (root canal) instrumentation or surgery only beyond the apex

*Placement of rubber dams

*Subgingival placement of antibiotic fibers or strips

*Postoperative suture removal

*Initial placement of orthodontic bands but not brackets

*Placement of removable prosthodontic or orthodontic appliances

*Intraligamentary local anesthetic injections

*Taking of oral impressions

*Prophylactic cleaning of teeth or implants where bleeding is anticipated

*Fluoride treatments
*Taking of oral radiographs
*Orthodontic appliance adjustment
*Shedding of primary teeth

44

Table18: SBE recommendation for other Procedures


Endocarditis prophylaxis recommended

Endocarditis prophylaxis NOT recommended

Respiratory tract:

Respiratory tract:

*Tonsillectomy and/or adenoidectomy

*Endotracheal intubation

*Surgical operations that involve respiratory mucosa

*Bronchoscopy with a flexible bronchoscope, with or without biopsy|

*Bronchoscopy with a rigid bronchoscope

*Tympanostomy tube insertion

Gastrointestinal

tract1:

Gastrointestinal tract:

*Sclerotherapy for esophageal varices

*Tran esophageal echocardiography|

*Esophageal stricture dilation

*Endoscopy with or without gastrointestinal biopsy|

*Endoscopic retrograde cholangiography with biliary obstruction


*Biliary tract surgery
*Surgical operations that involve intestinal mucosa
Genitourinary tract:

Genitourinary tract:

*Prostatic surgery

*Vaginal hysterectomy2

*Cystoscopy

*Vaginal delivery3

*Urethral dilation

*Cesarean section
-In uninfected tissue:
*Urethral catheterization
*Uterine dilatation and curettage
*Therapeutic abortion
*Sterilization procedures
*Insertion or removal of intrauterine devices
Other:*Cardiac catheterization, including balloon angioplasty
*Implanted cardiac pacemakers,implanted defibrillators, and coronary stents
*Incision or biopsy of surgically scrubbed skin, Circumcision

45

Table19:Endocarditis Prophylactic Regimens for Dental, Oral, Respiratory Tract and Esophageal Procedures
Situation

Agent

Regimen*

Standard general prophylaxis

Amoxicillin

Adults: 2 g
Children: 50 mg per kg
Taken orally one hour before the procedure

Patient is unable to take oral medications

Ampicillin

Adults: 2 g
Children: 50 mg per kg
Given IM or IV within 30 minutes before the procedure

Patient is allergic to penicillin

Clindamycin (Cleocin)

Adults: 600 mg
Children: 20 mg per kg
Taken orally one hour before the procedure

or
Cefadroxil (Duricef)
or cephalexin
(Biocef, Keflex)

Adults: 2 g
Children: 50 mg per kg
Taken orally one hour before the procedure

or
Azithromycin (Zithromax)Adults: 500 mg
or clarithromycin
Children: 15 mg per kg
(Biaxin)
Taken orally one hour before the procedure
Patient is allergic to penicillin and is unable to take oral medication Clindamycin

Adults: 600 mg
Children: 20 mg per kg
Given IV within 30 minutes before the procedure

or
Cefazolin (Ancef, Kefzol) Adults: 1 g
Children: 25 mg per kg
Given IM or IV within 30 minutes before the procedure

46

RHD with Pregnancy


PREGNANCY IN RHD PATIENT:
RHD patients during pregnancy are specially at risk due to altered hemodynamics and increase in total cardiac output and increased load on heart.
Obstructive as well as regurgitatant lesion may become apparent owing to this altered hemodynamics.
If possible patient must be examined and counseled and pregnancy must be a planned one . Valvotomy if required must be done before
conception,which is not possible in our circumstance. A severe valvular disease is indication for MTP below 20 weeks.

Tab 20 :Pregnancy with Valvular Abnormality


Pregnancy with
Regurgitant Lesion(MR/AR)

Pregnancy with
Obstructive lesion(MS/AS)

Usually well tolerated


Treat medically with diuretics,
Vasodilators
(no ACE inhibitors/angiotensin II
receptor blockers but hydralzine ,
Nitrates Ca channel blocker can be
used) for heart failure
Beta-blockers, digoxin
for rate control of AR

Mild to moderate MS Medical Tt


Moderate to severe MS (MVA
<1.5cm2) BMV at late second
trimester if patient remains
symptomatic and
PAS pressure >50mmHg
Aortic stenosis (rare) Mild to
moderate well tolerated
Diuretics for heart failure
Beta-blockers, digoxin for rate control
of AF. Severe AS (AVA >50mmHg
mean gradient) BAV if severely
symptomatic
Avoid surgery, high risk of foetal loss

Pregnancy with Prosthetic Valve:

High risk to fetus & mother


Risk of warfarin embryopathy in first
trimester may be avoided if warfarin
dose 5mg
. ANTICOAGULENTS
LMWH throughout pregnancy,
weight-adjusted dose with anti-Xa
level monitoring
2 .Warfarin throughout pregnancy if
can keep warfarin 5mg, eg INR 2.0
3.0 in aortic
prosthesis, sinus rhythm; change to
LMWH or UFH at 36 weeks
3. LMWH until 13 weeks and then
warfarin and aspirin until 36 weeks;
change to
4 .LMWH or UFH until labour. Monitor
anti-Xa levels with LMWH

Table21:Atrial Fibrillation in Pregnancy:


Rate control:
Digoxin
Beta-blocker,
Calcium channel antagonist

Hemodynamically Unstable
Cardio version

47

Anti coagulation:
Target INR 2-3
Principle as for prosthetic valve

Table22: Cardiac evaluation of post intervention patient.


Post Intervention patient
Post Valvuloplasty: Functional capacity/ Rate and Rhythm
E/o pulmonary hypertension.
Level of regurgitation. Vasodilators for moderate MR/AR
Rule out restenosis/ recurrence of rheumatic activity
Status of other valve/ventricular function
Secondary prophylaxis/ SBE prophylaxis
Post Surgery patient :
All of above.
INR monitoring.
Clinical evaluation of valve sounds.
Slightest doubt of valve dysfunction or IE patient must be referred to tertiary care level.
Secondary prophylaxis/ SBE prophylaxis

Table 23: Drugs & Dosages

Drugs

Indications

Doses

Comments

Str pharyngitis

AST, Deep IM Single dose

ARF

AST, Deep IM Single dose

Secondary prophylaxis

< 27 kg 6 lakh every 15 days

No carditis:18 yrs age/ 5 years

>27 kg 12lakhs every 21 days

after last attack whichever longer

Antibiotics
Benzathine Pen

Mild to mod carditis 10 years /25


years of age which ever longer
Established RHD :lifelong /?40
years
Follow the protocol

Penicillin V p/o

Sec prophylaxis

250 mg BD children

Follow up protocol

500mg BD grown up/adults


Str sore throat (10 days)

250 mg QID children


500mg QID grown up/adults

Erythromycin ethyl

Str sore throat (10 days)

succinate po (only if allergic

48

10days

to penicillin)

Sec Prophylaxis

20mg/kg BD

Follow-up protocol

Azithromycin

Str sore throat

12.5mg/kg

5 days

Cephalexin

Str Sore throat

25 mg / kg /dose twice a thrice a day

10 days

Pain relieve in ARF before establishment of diagnosis


Paracetamol p/o

Codeine p/o

Arthritis or

60mg/kg/day (max 4g) given in

Until symptoms relieved

arthralgia mild

46 doses/day; may increase to

or NSAID started

or until diagnosis

90mg/kg/day if needed, under

confirmed

medical supervision

same

0.51.0mg/kg/dose (adults

Until symptoms relieved

1560mg/ dose) 46hrly

or NSAID started

80100mg/kg/day (48 g/day in

See page no 18

Anti inflammatory therapy


Aspirin p/o

Confirmed ARF

adults) given in 45 doses/day


Reduce to 6070mg/kg/day when
symptoms improve

Naproxen p/o

Prednisolone

Arthritis (if aspirin

10-20 mg/kg/d (max dose: 1250 mg)

intolerant)

bid

Severe Carditis, Heart

1-2 mg/kg/d (Max dose 80 mg)

failure,pericardiotis with effusion

See Page no 18

till ESR normalizes usually 2


weeks Taper over 2-4 weeks,
(+start Aspirin 75 mg/kg/d)
reduce 2.5-5mg every 3rd day.
Continue with Aspirin to
complete 12 weeks.

Diuretic Therapy
Frusemide po/IV (can also be Heart failure

Children: 12mg/kg stat, then

Until failure controlled

given IM)

0.51mg/kg/dose 624 hrly

and carditis improved

(max 6mg/kg/dose)
Adults: 2040mg/dose 1224 hrly,up to
250500mg/day

Spironolactone p/o

same

Children: 12mg/kg stat, then


0.51mg/kg/dose 624 hrly
(max 6mg/kg/dose)

49

Same

Adults: 2040mg/dose 1224 hrly,up to


250500mg/day

Angiotensin Converting Enzymes Inhibitors


Captopril p/o

Valve Regurgitation/ Heart failure

0.5 1.mg/kg/dose 8hrly

Same

(test dose:0.12 mg/kg)


Enalapril p/o

Same

Children: 0.1mg/kg/day in 12 doses,

Same

increased gradually over 2 weeks to


max of 1mg/kg/day in 12 doses
Adults initial: 2.5mg daily;
Maintenance: 1020mg daily
(max 40mg)
Lisinopril p/o

Same

Children: 0.10.2mg/kg once daily,

Same

up to 1mg/kg/dose
Adults: 2.520mg once daily
(max 40mg/day)

Digoxin
Digoxin po/IV

Heart failure /Atrial

Children:

Fibrillation

5mcg/kg /dose bd max 125mcg)

Clinical decision

Adults: 125250mcg daily

Management of Chorea
Carbamazepine

Severe chorea

720mg/kg/day (710mg/kg/day

Until chorea controlled for

usually sufficient) given tds

several weeks, then trial off


medication

Valproic acid po

Same

Usually 1520mg/kg/day

Same

(can increase to 30mg/kg/day)


given tds (may affect salcylate
metabolism)
Haloperidol

Same

0.5-2.5mg tid

Same

Pimozide

Chorea

0.2 mg/kg/d

Same

Control of Atrial Fibrillation

50

Flecainide

AF (Pharmacological conversion)

50-100mg/m2/day or 6-9mg/kg/day in
three divided doses

Propanolol

AF (rate control)

2-5mg/kg/d 3-4 divided doses

Atenolol

AF (Rate control)

1-2 mg/kg/d Single dose

Metoprolol

Rate control AF

Sotalol
Amiodarone

2-8mg/kg/d bid
Pharmacological cardioversion

IV 25mcg/kg /min first 4 hrs. then 10-15


mcg/kg/min. depending on response
tapper down to 5mcg/kg/min and start
oral therapy.
Oral: load with 10-15 mg/kg/d for 1-2
wks . maintenance 2.5-5/kg/

Diltiazem

AF(adult)

loading:10-15mg/kg/d
0.25mg/kg/iv over 2 min. Maintenance
:2mcg/kg/min 120 360 mg /day

D-C shock

AF

1-2 j/kg 0.5-1 for conversion.


Defibrillation higher doses(2-4j/kg)

Ionotropes & Vasodilators (Uncontrolled CHF)


Dopamine

CHF(uncontrolled)

2-20mcg/kg/min inf

Dobutamine

CHF(uncontrolled)

0.5-1mcg/kg/min inf

Milrinone

CHF(uncontrolled)

0.5-1mcg/kg/min inf

Nitroprusside

CHF(uncontrolled)

0.5-10 mcg/kg/min (monitor cyanide


levels)

Prevention and management of Thrombosis(See Text)


Warfarin

AF/Prosthetic valve
Ped: 0.05-0.3 mg/kg/d dont exceed
adult dose.
Adults: 5-15mg/d po 3-5 d then
maintenance 2-10mg/d with INR
monitoring.

51

Adult:800-1000iu continuous infusion to

Heparin

keep PTT2-2.5 times of control(55-70


sec)
Ped:25iu/kg/hr iv infusion

Tissue plasminogen
activator(t-PA)

0.6-1 mg/kg/in bolous infusion followed Following the thrombolysis


by continuous infusion 0.1-0.5 mg/kg/hr heparin infusion must be started
for 6 hrs followed by reassessment
before continuing for prolonged infusion.

Urokinase

4400units stat then 4400 Units/Kg/hr

Blood fibrinogen
level,activated PTT and fibrin
degradation product must be
monitored. Echo review must
be done
Monitor Fibrinogen, TCTthrombin clotting time,,APTT
PT

Streptokinase

2000 U /kg loading dose then 1000


U/kg/hr then infusion 1000/U/kg /hr 6hrs

52

Same

Tab24:Management Guidelines Penicillin Anaphylaxis*

Initial Management :Administer adrenalin


Adults: 0.5 ml of 1:1000 adrenalin- IM/SC or 3 to 5 ml of 1:10,000 adrenalin-IM or IV
Children: 0.1ml/kg(max0.3/dose) of 1:1000 adrenalin-IM/SC
Repeat dose can be given at 5 minute interval.
Head end low, Raise lower limb. Oxygen. Monitoring. Bagging if required(adult Ambu must be >700ml; in children according to wt
bag must be chosen)
Management in Intensive care
ADULT:
Fluid : Crystalloids-Ringer or normal saline; Adults; 5-10ml/kg first 5 min 1-2 lit/hr.Amount to be adjusted according to
hydration,renal and cardiac status.
Adrenalin: Prepare infusion by adding 1 mg (1 mL) of a 1:1000 Adrenalin to 250 mL of D5W to yield a concentration of 4.0
micrograms/mL. . Infused at a rate of 1 to 4 micrograms/min (15 to 60 drops per minute with a microdrop apparatus [60 drops per
minute = 1 mL = 60 mL/h]), increasing to a maximum of 10.0 micrograms/min.or by an infusion pump-1:100,000 solution of
epinephrine (1 mg [1 mL] in 100 mL of saline) ,administered i.v. at an initial rate of 30 to 100 mL/h (5 to 15 micrograms/min), Then
readjust according to clinical setting.
Children: Fluid: Children:30ml/hr first hour. Then adjust the maintenance fluid.
Adrenalin:dosage of 0.01 mg/kg (0.1 mL/kg of a 1:10,000 solution; maximum dose, 0.3 mg). Alternative pediatric dosage by the
"rule of 6" is as follows:
0.6 X body weight (in kilograms) = the number of milligrams diluted to a total of 100 mL of saline; then 1 mL/h delivers 0.1
microgram/kg/min.
OtherDrugs Used :
diphenhydramine, 1 to 2 mg/kg or 25 to 50 mg per dose (parenterally).
Note: H1 antihistamines are considered second-line therapy to epinephrine and should never be administered alone in the
treatment of anaphylaxis.
Ranitidine, Adults:50 mg Children: 1 mg/kg,
which might be diluted in 5% dextrose to a total volume of 20 mL and injected intravenously over 5 minutes.
Cimetidine (4 mg/kg) can be administered intravenously to adults, but no pediatric dosage in anaphylaxis has been established.
Additional therapy: Broncho dilators and vasopressure drugs like Dopamin.
1.Cardiopulmonary resuscitation and advanced cardiac life support measures.
2.High-dose epinephrine IV (i.e., rapid progression to high dose). A common sequence is 1 to 3 mg (1:10,000 dilution) slowly
administered IV over 3 minutes, 3 to 5 mg administered IV over 3 minutes, and then a 4 to 10 microgram/min infusion.
For children, initial resuscitation dosage : 0.01 mg/kg (0.1 mL/kg of a 1:10,000 solution up to 10 mg/min rate of infusion) repeated
every 3 to 5 minutes for ongoing arrest. Higher subsequent dosages (0.1 to 0.2 mg/kg; 0.1 mL/kg of a 1:1,000 solution) might be
considered for unresponsive asystole or pulseless electrical activity.
3.Rapid volume expansion.
4.Atropine and transcutaneous pacing if asystole and/or pulseless electrical activity are present. .Prolonged resuscitation is
encouraged, if necessary, because efforts are more likely to be successful in anaphylaxis.
*The diagnosis and management of anaphylaxis: an updated practice parameter. J Allergy Clin Immunol 2005 Mar;115(3
Suppl):S483-523.

53

RF/RHD REGISTRY PROJECT Suggested Protocol for Satellite Centers


Objectives:
Creation of a registry
Obtaining prevalence statistics through a school health survey?
Questions:
1.
How do we put these objectives in the national context for RF, RHD prevention?
2.
How many such centers do we create?
3.
How will the national objectives on prevention of RF and RHD be met through these satellite centers?
4.
Do we also integrate the creation of the registry with treatment goals such as provision of penicillin prophylaxis?
Project Initiation
The Principal and the Co-investigator associated with the project should visit one of the main registry (nodal) centers
(closest to theirs) and study the methodology being followed there. The registry center should provide a detailed
description of the project and training material/modules that is being used there. They should then draft a protocol based
on the specific ground realties of their region. This protocol is therefore a rough guideline and will need modification
based on the situation prevailing in the region surrounding the satellite center
Selection of Population and Area for the Registry:
The project co-investigators should conduct a survey on the health care infrastructure in the district using the available
information from standard government sources. A contiguous area with a population of 10 lakhs should be chosen after
consideration of a number of variables including logistics and feasibility. The project area should be clearly defined based
on information obtained. A map should be made. All the gram panchayats in the area should be defined clearly. The
population of the contiguous area selected should be assumed from the results of the most recent national census.
The following information of the project area should be made available:
1.
All demographic information: Area, population, density
2.
Schools in the region: All schools, private and government, primary and secondary, aided and unaided etc.
3.
Health infrastructure of the area: Detailed listing of all health care providers, Govt., Pvt., NGO, Missionary
hospitals etc.
Staffing and Creation of the Project Office
The project office would need to be set-up in the satellite center
The staff will have to be hired as stipulated by ICMR:
Project secretary / data-entry operator
Research officers
Medical social workers
Field Organizers (Field Workers)
Project Office Set Up
Infrastructure should include computer, telephones- one land and one mobile and sufficient furniture. Twowheelers will need to be purchased for the purpose of field and house visits by the project staff as per specifications from
ICMR. Registers of Project activities should be maintained in the office.
Networking Among the Health Care Providers for Passive Reporting: After a detailed listing of the health care providers is
done all associations of the medical profession should be contacted, This should include Indian Medical association (IMA),
Indian Academy of Pediatrics (IAP), State Government Medical Officers Association and Qualified Medical Practioners
Association (QPMPA). The mailing lists of all organizations should be obtained, and the names and addresses of the
doctors practicing in the project area should be filtered out. The concerned district and state health authorities should be
contacted to solicit cooperation from all doctors in the government sector. They should include the district medical officer
(DMO) and Director Health Services.
The doctors in the area should be informed about the project through workshops, meetings and personal visits.
This programme should continue throughout the duration of the project to sustain awareness.
All health care

54

providers in the area, who are likely to encounter patients with RF or RHD, should be instructed to provide the details of
the patients to the registry project. This generally includes general practitioners, physicians, pediatricians and,
cardiologists. All the doctors should be given refresher training on the revised guidelines of diagnosis and treatment of RF
and RHD through training workshops. Further refresher sessions should be given utilizing the various organizational
meetings of doctors and their organizations. Government doctors were further briefed in their monthly conference at the
District Medical Office. General Practitioners should be addressed during the monthly meetings of the local branches
Indian Medical Association (IMA). Other forums that could be utilized to reach out to the doctors include the local
branches of Indian Academy of Paediatrics,), Qualified Private Medical Practitioners Association (QPMPA) and, local
chapter of the Cardiological Society of India (CSI). The Registry Project team should go round the area, with short
presentations and the training modules, to meet the busy practitioners who are not reached through the other means.
Handbooks containing clear guidelines for referral and prepaid postal envelopes will have to be given to all the doctors
that were replenished on a regular basis. All possible modes of communication have to be made available for referral to

the project office (ordinary mail, email, phone, fax, cell phone). After establishing the networking and referral pathways
with the doctors, an intense community awareness campaign should be launched in the area, to ensure all the probable
cases to visit/revisit the nearest doctor. Health awareness sessions for the Anganwadi teachers of the Integrated Child
Development Schemes , schoolteachers, functionaries of the State Poverty Eradication Mission (SPEM) health workers
will have to be done as part of the community mobilization. Health Seminars, photo exhibitions, poster stories, quiz
programs, tele-documentary in local vernacular starring popular serial actors etc can be used to pass the message to the
general public.
Reporting: All the suspected cases referred by doctors should be seen by cardiologists at the referral institute and will
need to be investigated. Echocardiograms will need to be performed when necessary. Feedback detailing the treatment
and follow-up options should be given back to the referring doctor and the patient separately. Penicillin prophylaxis (3
weekly Benzathine penicillin) should be administered free of cost if warranted and requested for by the patient.
From every major hospital in the area, the lists of patients who are on penicillin prophylaxis should be obtained. This
along with the scrutiny of hospital records, wherever permitted helped identification of RHD patients who were already
diagnosed and were on penicillin prophylaxis.
Data collection: Once the project office receives a referral letter, the address of the patient should be crosschecked and
entered in the records and letter of acknowledgement was sent to the referring doctor. The concerned patient /family
need to be contacted by project staff over phone or in person. A convenient date can be fixed for a hospital visit. On the
day of the visit, the patient/family should be interviewed by the project medico social workers. They should then briefed
about the disease entity and its management. The mandate and the purpose of the registry project should also be
explained to them. The cardiologist should see all patients, and if necessary echocardiogram evaluation should be done.
Entry to the registry should be made after confirmation of diagnosis. An additional register containing a list of all
referred/suspected cases should also be maintained.
The data base should be computerized preferably using Epiinfo soft ware.
Training materials to be prepared (they are already available but translations to local language will have to be arranged):
An introductory brochure that will need to be distributed by the field staff to health professionals in the project
area. (Sample in annexure 1)
A training/orientation module for the doctors
A map of the selected project area should be prepared and distributed to the doctors
Training material for Health Workers in local language should be prepared and printed.
Reporting format:
Business Reply Post cards indicating the basic information on the cases that they would like to report to the
registry should be printed for distribution to doctors.
A modified version of the reporting form (available at existing registry centers)
Register to monitor secondary prophylaxis
Reports of all centers have to follow a standardized format

55

Workshop for Doctors: A series of orientation workshops (primarily targeting Physicians and Pediatricians) and doctors in
PHC should be planned. The purpose of the meetings is to brief all the participating doctors about the project and to
define their roles.
The training modules and all the forms should be distributed to all the participants during the workshops. Teaching
material for these workshops is available on CD.
Anganwadi Workers and Community Health Volunteers Training: A list of the Anganwadi in the project area should be
made available. A meeting with ICDS Supervisors to chart out a plan regarding the training programme is desirable.
Efforts should be made to involve all the community health volunteers in the area in the training process.
School survey: A list of schools in the project area (with details of No of students and teachers) should be prepared based
on the information from District Education Office. A random sampling of the schools should be carried out to select 25,000
School Children. This should be a stratified sample and should represent the school profile in the district in terms of
government and private schools.
School teachers and Community Leaders training
School teachers and Community Leaders should also be made aware of the program through special training
sessions.
Community Awareness through Media:
1.
Documentary on RF / RHD for telecast in various health education meetings and in the media. To intensify
public health education a documentary should be made in the local language (dubbed versions of the ones that have been
made may also be acceptable). This documentary film should be short lasting about minutes.
2.
Regular press briefings
3.
Bit notices
4.
Health exhibitions
5.
Health camps

56

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