Cardiovascular Journal of Africa: Vol 24 No 1 (February 2013)

CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013

AFRICA

157

Methods:

We designed an eight-week training syllabus for primary

health workers. The syllabus included tutorials in basic cardiac anat-

omy and physiology, pathophysiology of RHD, and practical sessions

in basic echocardiographic screening. We developed a 14-step screen-

ing protocol, and devised referral criteria based on identification of

significant mitral or aortic regurgitation. The syllabus included seven

weeks of supervised screening practice in schools.

Results:

Seven nurses from across Fiji participated in the training.

Despite minimal relevant experience, nurses were quick to learn

basic anatomy and pathophysiology for understanding rheumatic

valvular pathology. Nurses demonstrated rapid acquisition of basic

echocardiographic skills. Use of a simplified screening protocol was

highly valuable.

Conclusions:

Training of primary health workers with limited prior

knowledge or experience in basic screening echocardiography for

RHD is feasible. The results of the current fieldwork phase, involving

screening of 2 000 children, will provide further information about

the sensitivity and specificity of this approach to screening. A struc-

tured syllabus, including screening and referral protocols, has been

developed, which may be useful for training other workers in Fiji and

other resource-poor settings.

336: FIRST EXPERIENCES WITH ANGIOTENSIN II

RECEPTOR BLOCKER IN PAEDIATRIC PATIENTS WITH

MARFAN’S SYNDROME AND ENLARGED AORTIC ROOT

Kristoffer Steiner, Lydia Rosenow, Veronika Stark, Goetz Mueller,

Jochen Weil, Yskert V Kodolitsch, Thomas S Mir

Paediatric Cardiology, University Heart Centre of Hamburg, Germany

Background

: Progressive enlargement of the aortic root leading to

dissection or rupture is the main cause of premature death in patients

with Marfan’s syndrome (MFS). Standard prophylaxis is beta-

blockers (BB). A new concept in adults with MFS is the treatment

with angiotensin II receptor blockers (AT). So far, there is a lack of

experience regarding the treatment of children with AT. We present a

comparison of our first patients treated with AT or BB.

Methods

: We identified a cohort of 41 paediatric MFS patients with

aortic root enlargement (mean age 7.8 years). In 28 MFS patients

a prophylaxis with BB (

n

=

15) or AT (

n

=

13) was subscribed. A

control group of 15 children has not been under any medication

during evaluation period while seven of these patients later had to

switch to AT treatment due to progressive enlargement of the aortic

root. Retrospective analysis of the impact of medical treatment

was performed by comparing the rates of change in diastolic aortic

root diameter (DDAR) over a mean period of 8.21

±

5.1 (1.3–30.6)

months.

Results

: Mean DDAR increase over 8.7 months was 0.69

±

1.37

(–0.7–4.0) mm in patients with BB prophylaxis while mean DDAR

increase over a 5.9-month period was –0.08

±

1.11 (–2.0–1.0) mm in

patients with AT prophylaxis respectively (

p

>

0.05). Mean DDAR in

the control group over 6.6 months was 1.59

±

1.22 (–0.5–4.0) mm.

DDAR was significantly lower in patients on medication with BB (

p

=

0.056) and AT (

p

=

0.001) compared to the control group.

Conclusion

: In this small, non-randomised cohort study, the use of

AT or BB therapy in children with Marfan’s syndrome slowed the

rate of progressive aortic root dilation while therapy with AT seemed

to be more effective. These findings require confirmation in further

studies with a prospective and randomised study design.

342: THE SIX-MINUTEWALKING TESTASA PROGNOSTIC

MARKER IN CHILDRENWITH DILATED CARDIOMYOPA-

THY: PRELIMINARY DATA FROM THE CARDIOMYOPA-

THY STUDY (CARS) IN CHILDREN

Suzanne Den Boer

1

, Tim Takken

2

, Gabrielle van Iperen

3

, Ad Backx

4

,

Lukas Rammeloo

5

, Derk-Jan ten Harkel

6

, Ronald Tanke

7

, Gideon du

Marchie Sarvaas

8

, Michiel Dalinghaus

1

Department of Paediatric Cardiology, Sophia Children’s Hospital,

Erasmus MC, Rotterdam, The Netherlands

2

Department of Child Development and Exercise Centre,Wilhelmina

Children’s Hospital, Utrecht, The Netherlands

3

Department of Paediatric Cardiology, Wilhelmina Children’s

Hospital, University MC, Utrecht, The Netherlands

4

Department of Paediatric Cardiology, Emma Children’s Hospital,

Academic MC, Amsterdam, The Netherlands

5

Department of Paediatric Cardiology, Free University Medical

Centre, Amsterdam, The Netherlands

6

Department of Paediatric Cardiology, Leiden University Medical

Centre, Leiden, The Netherlands

7

Department of Paediatric Cardiology, University Medical Centre, St.

Radboud, Nijmegen, The Netherlands

8

Department of Paediatric Cardiology, Beatrix Children’s Hospital,

University MC, Groningen, The Netherlands

Background:

The maximal oxygen uptake (VO

2max

) has been used

as a prognostic marker to stratify adults with (severe) heart failure.

In children, VO

2max

may also have prognostic value, but it may be

challenging to measure, especially at a young age. Therefore, we

determined whether the six-minute walking test (6MWT) could be

used as a prognostic marker in children with heart failure and dilated

cardiomyopathy (DCM). The 6MWT, which measures the distance

that is voluntarily covered in six minutes, is easy to perform and a

good reflection of daily activity.

Methods:

In a prospective longitudinal multicentre study the 6MWT

was performed in children with DCM. Using reference data from

Geiger

et al.

(2007), the 6MWT results were transformed to

z

-scores.

Death, heart transplantation and institution on mechanical support

were defined as primary endpoints. The 6MWT performance of chil-

dren with and without an endpoint was compared.

Results:

Twenty-four children with DCM (mean age 13

±

3 years)

were included and performed 61 6MWTs without adverse events,

during a mean follow-up period of 11

±

5 months. The mean (SD)

6MWT distance

z

-score was –3

±

3.2 compared to reference data

(

p

<

0.01). In children who reached one of the pre-defined primary

endpoints, the distance covered during the 6MWT was significantly

lower (

z

-score –5.7

±

2.9) in the three months before the endpoint

was reached, compared to those not reaching a primary endpoint

(–2.2

±

2.1) (

p

<

0.05).

Conclusion:

The six-minute walking test is easy to perform and

safe in children with DCM. The 6MWT performances of children

with DCM were significantly reduced compared to normative data.

Children reaching a primary endpoint performed significantly worse

than children with a favourable course of the disease. Longitudinal

assessment of the six-minute walking test may have prognostic value

in children with DCM.

344: REDUCED HEALTH-RELATED QUALITY OF LIFE IN

CHILDERENWITH DILATED CARDIOMYOPATHY

Suzanne Den Boer

1

, Lisbeth Utens

2

, Gabrielle van Iperen

3

, Ad

Backx

4

, Derk-Jan ten Harkel

5

, Lukas Rammeloo

6

, Gideon du Marchie

Sarvaas

7