www.worldcardiocongress.

org

Chairpersons:
Bongani M. Mayosi
Jonathan Carapetis
Press conference:
Rheumatic Heart Disease a forgotten but
devastating disease
www.worldcardiocongress.org

www.worldcardiocongress.org

RHD The Real World Situation

L.Zhlke, G.Karthikeyan, M.Engel, B.Cupido, A.Joachim , R.Daniels
S.Rangarajan, S.Yusuf, K.Teo, B.Mayosi.
for the
Rheumatic Heart Disease Global Registry (REMEDY) Investigators

www.worldcardiocongress.org

Burden of Disease:Heart Failure

CHD

Mayosi, B et al Heart 2007

7%

3%

12%

35%

Renal

Rheumatic Heart Disease

17%
26%

CMO

EMF

Ellis J et al Trop Doct 2007

Tamponade

Gunther et al The Lancet 2006

Diao M et al Arch Cardiovasc Dis. 2011

RhEuMatic Heart Disease RegistrY - REMEDY

Rationale and Design
Prospective, international (Africa and India),
hospital-based registry; follow-up and outcome
ascertainment at two-year follow-up
N = 3000
The full study will aim to enroll 10,000 patients.

Main objectives
Karthikeyan G et al.
Rationale and design of a Global Rheumatic Heart
Disease Registry: The REMEDY study. American
Heart Journal. 2012.

To describe demographic and disease

characteristics of contemporary patients
To describe prevailing treatment patterns
(Focus on adherence to and quality of oral
anticoagulation, and adherence to secondary
penicillin prophylaxis)
Major outcomes (all-cause mortality,
stroke/systemic embolism, major bleeding, RF)

Preliminary Results: Previous History n=690

15%

10%

13%

6%

6%

6%
2%

5%

1%

0%
Infective
Endocarditis

Major
Bleeding

Peripheral
Embolism

Stroke

Valvulopasty

Previous
surgery

Preliminary Results- n=690

Mild
Moderate
Severe
120
100

23 % NYHA 3 or 4
60 % diagnosed with Congestive
Heart Failure

80
60
40
20
0
AR

AS

MR

MS

TS

Preliminary Results- n=690

Oral Anticoagulants
Prescribed

Awareness of Goal INR

35%
44.7%

55.3%

Yes
No

Yes
No

65%

Measurements of INR in past 6

months
40%
29%

33%

30%
20%

24%
14%

10%
0%
1-2

3-5

6 or more

none

RhEuMatic Heart Disease RegistrY - REMEDY

Preliminary data

Young patients (mean age)

30 years

Proportion with AF

31.6%

High-risk population

4.4% (15/341) all-cause mortality at 10-month follow-up

Mean age of death: 50 years

In conclusion
There is an irrefutable burden of disease relating to RHD in Africa
and middle and low-income countries.
REMEDY presents an opportunity for collaboration, in describing
and documenting the course of a neglected disease.
Initial results indicate significant disease burden and serious
morbidity.
There are important gaps in the prescription of and adherence to
guideline-recommended treatments. This presents opportunities for
focused intervention.

REMEDY will be a source of valuable information for governments

in formulating policy and guidelines for the control and treatment of
RHD.

Thank you

www.worldcardiocongress.org

Dr. Antonio Grimaldi, M.D.

Cardiovascular and Thoracic Department,
San Raffaele Scientific Hospital, Milan, Italy
Rheumatic Heart Disease Remains a Major Cause
of Heart Failure in Developing Nations The
Ugandan Experience
www.worldcardiocongress.org

BACKGROUND
The increasing global crisis in Non-Communicable Diseases (heart disease,

stroke, cancer, diabetes, etc.), is a barrier to UN development goals including

poverty reduction, health equity, economic stability, and human security (Lancet
2011).
In Africa, cardiovascular diseases are the second leading cause of mortality and
the first under the age of 50 years.
Uganda, like many other african countries, has an
accelerated epidemiological transition with a marked
increase in cardiovascular non-communicable diseases
(Mayosi, Science 2009).

BACKGROUND
Heart failure (HF) is a progressive disorder in which damage to the
heart causes weakening of the cardiovascular system.
HF is defined as the inability of the heart to supply sufficient blood to
meet the needs of the body.
HF is a major cause of mortality in the community.
Identifying HF aetiology is crucial when planning interventions aimed to
reduce mortality and morbidity.

ST. RAPHAEL OF ST. FRANCIS HOSPITAL NSAMBYA

AIMS
During 2009-2010, 5 non-governmental organizations missions carried out by
Italian medical teams (30 weeks) had the opportunity to prospectively collect
clinical and echocardiographic data from patients referred to a urban Hospital in
Kampala, Uganda.
The objective of the study was to provide a hospital-based epidemiological picture
of HF.

RESULTS
Among 217 subjects referred for suspicion of
heart disease, 150 were affected by heart
disease.
Most patients (97; 65%) presented with HF
(median age 32 years, IQR 12-65, female 52%).

The main cause of HF was RHD (n=29, 30%),

mainly related to mitral valve regurgitation (60%).
RHD is a complication of rheumatic fever and
represents the predominant form of valvular
disease affecting children and young adults.
Among HF patients affected by RHD, 23 (79%)
had an indication for cardiac surgery.

CONCLUSIONS
RHD still remains the most common cause of HF in Uganda, with late
presentation in most cases and heart failure in adulthood.
The results should promote efficient cost-effective preventive measures focused
on RHD:
Screening programmes aimed to increase the community awareness concerning the
RHD also promoting a wider diffusion of portable technologies.
Education concerning major cardiovascular risk factors and chronic alcohol intake

Beyond the preventive strategy there is an actual need for surgical interventions for
heart failure related to RHD since cardiac surgery is not available in Uganda.

www.worldcardiocongress.org

Andrea Z. Beaton, M.D.

Childrens National Medical Center, Washington DC
Echocardiography-Based Screening for the Early
Diagnosis of Rheumatic Heart Disease
www.worldcardiocongress.org

Echocardiography for the Early Diagnosis of RHD

Traditionally RHD
was only
diagnosed
through clinical
exam

Disease was
usually advanced

Echocardiography for the Early Diagnosis of RHD

Finds more cases

Is more accurate
Can detect very
early disease

Echocardiography for the Early Diagnosis of RHD

Rheumatic Heart Disease found on

echocardiogram before clinical symptoms
exist is called subclinical RHD

Echocardiography for the Early Diagnosis of RHD

Echocardiography for the Early Diagnosis of RHD

Since 2004 the World Health Organization has

recommended echocardiography for RHD
screening in high-prevalence regions

Echocardiography for the Early Diagnosis of RHD

5006 Available Children enrolled in 6
Screening Sites

4869 Children
Screened (97%)
130 Children with
Abnormal Screening Echo (2.4%)
72 Total RHD Cases
(1.5%)

Echocardiography for the Early Diagnosis of RHD

5006 Available Children enrolled in 6
Screening Sites

Prevalence rate
of 1.5%

4869 Children
Screened (97%)
130 Children with
Abnormal Screening Echo (2.4%)

72 Total RHD Cases

(1.5%)

Echocardiography for the Early Diagnosis of RHD

5006 Available Children enrolled in 6
Screening Sites

47
Sub-clinical
RHD

4869 Children
Screened (97%)
130 Children with
Abnormal Screening Echo (2.4%)

72 Total RHD Cases

(1.5%)

Echocardiography for the Early Diagnosis of RHD

Children attending schools with lower

socioeconomic representation were more
likely
Have RHD
Have advanced disease

Mean age of RHD positive (10.1 yrs) children

higher than RHD negative children (9.3 yrs)
May have implications for screening protocols

Echocardiography for the Early Diagnosis of RHD

12-month Follow-Up Data

We are just beginning
to learn about patients
with subclinical RHD
What is the natural
history of subclinical
RHD?
Do they benefit from
secondary prophylaxis?

Echocardiography for the Early Diagnosis of RHD

43 (60%) children presented for follow-up at 6 months
41 (57%) children presented for follow-up at 12 months

60

62%

40
30

29

53%
88%

17

20
10

58/88 (66%)

47

50

16

Definite

Probable

75%
12

Total Patients

Possible
Follow-up

No Disease

Echocardiography for the Early Diagnosis of RHD

12 Month Disease Progression

0
Definite

5
5

10

15

20

25

30

13/58 (22%) showed improvement

7/58 (12%) showed worsening

Probable

Possible
None

2
22

3
Unchanged

Improved

Worsened

35

Echocardiography for the Early Diagnosis of RHD

12 Month Disease Progression
0
Definite

5
5

10

15

20

25

30

Resolution of MR
Probable

Possible
None

2
22

3
Unchanged

Improved

Worsened

35

Echocardiography for the Early Diagnosis of RHD

12 Month Disease Progression

0
Definite

5
5

10

15

20

25

30

New regurgitant murmur

Probable

Possible
None

2
22

3
Unchanged

Improved

Worsened

35

Echocardiography for the Early Diagnosis of RHD

At 6 months 13/17 (76%)
children prescribed
penicillin showed >90%
compliance
At 12 months 8/15 (53%)
had >90% compliance

9/9 boarding students

receiving PCN from school
nurse had 100%
compliance at both visits

Echocardiography for the Early Diagnosis of RHD

Follow-up of children diagnosed with RHD in a

school-based echo-screening program is feasible
Early follow-up shows dynamic disease
development: both improvement and worsening
More research is needed into improving rates of
compliance with secondary prophylaxis
The effectiveness and need of secondary
prophylaxis in children with subclinical disease
remains unclear

Echocardiography for the Early Diagnosis of RHD

New evidence-based guidelines will allow for

collaborations to determine the significance of
sub-clinical disease

Echocardiography for the Early Diagnosis of RHD

www.worldcardiocongress.org

Dr. Abdulkader A. Sharafadden, MMed

Head of Cardiac Department at Algomhori Teaching
Hospital, Taiz, Yemen
Interventional Therapy and the Need for Earlier
Intervention
www.worldcardiocongress.org

Rheumatic Mitral Valve Stenosis

An acquired progressive form of valvular heart disease
caused by rheumatic fever
Characterized by diffuse thickening of mitral valve leaflets,
fusion of the commissures, shortening and fusion of the
chordae tendineae
Resulting in an obstruction of the blood flow from the left
atrium to the left ventricle
Leaflets

Mitral
valve

Chordae
papillary

Normal

Mitral stenosis

Therapy

Medical
- Prevention of recurrent rheumatic fever,
- Prevention and treatment of complications
- Monitoring disease progression to allow intervention
at the optimal time point.

Interventional
- Percutaneous Balloon Mitral Valvuloplasty
( PBMV )
- Surgical intervention

PBMV

1st of June 2008 to 28th of February 2009

87 patients

Inclusion criteria
All patients were admitted and confirmed to have
pure rheumatic mitral valve stenosis by
echocardiography.

Cases with compound and other rheumatic valves

lesions were just included for statistical purposes.

Distribution of patients with RHD admitted to

Cardiology Department at Algomhori Hospital
Rheumatic valve
lesion

From Rheumatic Heart

Disease
(n= 206)

From total
cardiac patients
(n=1082)

Mitral stenosis

119

57.8

Pure mitral stenosis

Mitral regurgitation

89
68

43.2
33

Aortic stenosis

35

17

11
8.2
4.4
2.5

Aortic regurgitation

14

6.8

1.1

Total RHD

206

19%

Distribution of sex and mean age within patients

with pure Rheumatic Mitral Stenosis

28.7 %

Males
Females

M : F Ratio
1 : 2.4

71.3 %

Mean age
36.02 14.13
years

Degree of stenosis in patients with pure Rheumatic

Mitral Valve Stenosis

Mild
11.5%
Severe
51.7%

Moderate
36.8%

Recurrent Rheumatic Fever in patients with pure

Rheumatic Mitral Stenosis

Negative
60.9%

Positive
39.1%

Adherence to secondary prophylaxis for

Rheumatic Fever

Positive
28.7%
Negative
71.3%

Complications according to the degree of

stenosis
Degree of stenosis
Mild

Moderate

Severe

( n=10 )

( n=32 )

(n=45 )

Pulmonary Hypertension ( n=83 )

10.8

29

34.9

45

54.2

0.1

Pulmonary Congestion ( n=45 )

13.3

18

40

21

46.7

0.61

Atrial Fibrillation ( n=35 )

8.6

11

31.4

21

60

0.43

Right Ventricular Failure ( n=17 )

11.8

29.4

10

58.8

0.77

Stroke ( n=11 )

0.0

0.0

11

100

0.003
0.003

Frank Pulmonary Edema ( n=9 )

0.0

33.3

66.7

0.44

Left Atrial Thrombus ( n=3 )

0.0

0.0

100

0.24

Infective Endocarditis ( n=2 )

0.0

0.0

100

2.3

Peripheral Embolisation ( n=1 )

0.0

0.0

100

0.62

Complications

Hospital stay duration according to

complications
Hospital Stay
<1 week

1-2 weeks

> 2 weeks

( n=40 )

( n=39 )

( n=8 )

Pulmonary Hypertension ( n=83 )

38

45.8

37

44.6

9.6

0.8

Pulmonary Congestion ( n=45 )

19

42.2

23

51.1

6.7

0.41

Atrial Fibrillation ( n=35 )

20

25

71.4

8.6

0.00
0.00

Right Ventricular Failure ( n=17 )

47

41.2

11.8

0.89

Stroke ( n=11 )

27.3

9.1

63.6

0.00
0.00

Frank Pulmonary Edema ( n=9 )

66.7

33.3

0.0

0.34

Left Atrial Thrombus ( n=3 )

33.3

66.7

0.0

0.69

Infective Endocarditis ( n=2 )

0.0

0.0

100

0.00
0.00

Peripheral Embolisation ( n=1 )

100

0.0

0.0

0.53

Complications

Case fatality according to the degree of

Mitral Valve Stenosis
Outcome

Degree of
mitral
stenosis

Mild

10

12.7

0.0

32

40.5

0.0

37

46.8

100

79

90.8

9.2

Alive

Died

(n= 10)

Moderate
(n= 32)

Severe
(n= 45)

Total

Case fatality according to complications

80

75

70

Percentages

60
50
40
30

37.5

37.5
25

20
10
0

Plan of therapy in patients with

Mitral Valve Stenosis
60%

52.9
Percentages

50%
40%

28.7

30%

18.4

20%
10%
0%

BMVP

Surgical
intervention

Follow up

Performance of interventional therapy

SurgicalBMVP
intervention
Interventional Therapy
Performed
4%
Performed
Performed
23.9%
34.8%
Not
Not
performed
Not
performed
64.2%performed
76.1%
96%

Conclusions

Mitral stenosis was the most common RHD within our

community accounted for 57.8% while pure mitral stenosis
accounted for 43.2%

Females were more affected than males (M:F ratio 1:2.4)

The detection rate of the Attack of rheumatic fever was low
with
only
39.1%
and
adherence
to
secondary
chemoprophylaxis was weak among patients with only 28.7%
.
.

Conclusions

Most

patients were in a late stage of the disease

necessitating interventional therapy

Case fatality was 9.2%, all noted among severe degree of

stenosis

Performance of interventional therapy was far lower than

that required (23.9 %)

Recommendations

Mitral stenosis requires 3 early:

Early diagnosis
Early prevention
Early intervention

Recommendations

Great efforts must be made to detect the first signs of

Rheumatic fever and Rheumatic heart disease as this maybe
the only clinically manifested visit at an early stage before it
becomes subclinical and so be missed until the progression
of disease or complications occur.

Any auscultated murmur must be thoroughly investigated

by echocardiography because secondary prevention and
outcome of the disease relies on accurate case detection.

Recommendations

Establishing a RHD Surveillance System for a better

documentation and registration all over the country to
facilitate health care planning.

Screening programs for Rheumatic heart disease must

be performed among elementary school children by
echocardiography.

Recommendations

Great efforts should be done to implement primary and

secondary prevention programs for rheumatic fever and
RHD including establishing health educational programs
through different media.

Advanced cardiac centers must be established throughout

the

country to facilitate accessibility to appropriate

management including interventional therapies with less

effort and free of charge.

Recommendations

Reconsideration of the health strategy applied in Yemen

concerning general practioners practice where they should

be sent immediately after graduation to central hospitals to

gain the adequate experience before sending them for rural
services (primary care centers).

Benzathine
penicillin

BMVP

Mitral valve
replacement

10 years
cost

200 $

2000 $

5000 $

20 years
cost

400$

???

???

Special Thanks

Assoc. Prof. Ali Ahmed Ali

Assoc. Prof. Hilal Lashuel
Dr. Enas Ali Dammag

Q&A Session