CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
231
resistance index (PVRI) decreased from 19.73
±
9.31 Wood U/m
2
prior to inhalation to 15.49
±
8.87 Wood U/m
2
following inhalation
(
p
<
0.01), but there was no significant change in systemic blood
pressure (prior to inhalation 85
±
13 mmHg, following inhalation 83
±
11 mmHg,
p
>
0.05).
Conclusion
: Inhalation of iloprost during cardiac catheterisation in
children with severe PAH associated with systemic-to-pulmonary
shunt-type CHD can lower pulmonary vascular resistance, while
causing no significant change in systemic blood pressure and without
producing any obvious adverse reactions.
1328: OUTCOME OF BOSENTAN TREATMENT IN PAEDIAT-
RIC PATIENTSWITH PULMONARYARTERIAL HYPERTEN-
SIONASSOCIATEDWITH CONGENITAL HEART DISEASE
Hong Gu, Aijie Li, Chen Zhang, Xiaofeng Wang, Qiangqiang Li,
Bangjun Wu, Baojing Guo, Yinglong Liu
Beijing Anzhen Hospital affiliated to Capital Medical University,
China
Objective:
To describe the outcome of paediatric patients with
pulmonary arterial hypertension (PAH) associated with congenital
heart disease (CHD) treated with bosentan, which is known to be
safe and effective in patients with idiopathic pulmonary hyperten-
sion (IPAH).
Methods:
In this monocentre, open-label, uncontrolled, observational
study, 23 patients with PAH associated with CHD were treated with
bosentan, The mean age was 9.12
±
3.6 years (range 2.1–14.7) and they
were treated for a mean of 13.3
±
7.5 months (range 6–31). Six-minute
walking test, percutaneous blood oxygen saturation (SpO
2
%), New
York Heart Association functional class (NYHA) and cardiac catheteri-
sation data were compared before and after bosentan treatment.
Results:
After treatment, 6MWT (
n
=
17) improved from 458
±
16 to
496
±
69 m (
p
=
0.035). SpO
2
% increased from 89
±
5 to 91
±
5% (
p
=
0.009). In addition, the NYHA class improved. One patient in NYHA
class IV moved to class III, and one in class III moved to class II,
others in class II were stable (
p
=
0.001). Twelve patients had cardiac
catheterisation before and after bosentan treatment, Qp/Qs increased
from 0.97
±
0.40 to 1.16
±
0.40, PVRI decreased from 20.8
±
8.8 to
18.1
±
7.7 Wood unit/m
2
, but the changes had no statistical signifi-
cance. Bosentan was well tolerated by all patients, except one patient
had temporary lower gastrointestinal tract bleeding.
Conclusion:
Bosentan was safe and effective. Bosentan caused
significant improvements in 6MWT SpO
2
and NYHA functional
class and improvements in Qp/Qs and PVRI in children with PAH
associated with CHD.
1331: SUBVALVAR ANEURYSMS IN CHILDREN AT A
SOUTHERN AFRICAN TERTIARY CARE CENTRE
Antoinette Cilliers, Paul Adams, Gcina Dumani, Barend Fourie
Division of Paediatric Cardiology, Chris Hani Baragwanath Hospital,
University of the Witwatersrand, Johannesburg, South Africa
Introduction:
Subvalvar aneurysms, which may be subaortic or
submitral, were described originally in young Africans in the 1960s,
but later reports have included other race groups. Proposed aetiolo-
gies include infections, particularly tuberculosis, post traumatic, post
surgery and ischaemia. An underlying congenital or developmental
weakness between the muscular ventricular wall and the fibrous
valve annulus is postulated. Complications such as rupture of the
aneurysm, coronary artery compression, thromboembolism, and
sudden death support the need for surgical intervention.
Methods:
Children with subvalvar aneurysms were sourced from a
paediatric cardiology computerised database compiled over a 20-year
period at a southern African tertiary care centre.
Results:
Nine children with an average age of 8.8 years (range 1–16)
were diagnosed with submitral aneurysms using echocardiography.
The male-to-female ratio was 1:2 and all were black. Five presented in
heart failure and with varying degrees of mitral valve incompetence,
one with an incidental murmur, one with syncope, one with a pneumo-
nia and one with abdominal TB and human immunodeficiency virus
infection. Two patients were found to have additional left ventricular
cavity aneurysms and two aneurysms were found at surgery to have
ruptured into the left atrium. Four patients had positive Mantoux tests
and one was confirmed to have myocarditis and non-compaction of
the myocardium on MRI scan. Surgery was undertaken in six patients.
Histological examination of operative specimens showed peri-cardiac
tuberculosis in two, features suggestive of rheumatic fever in one, a
false aneurysm in one, and non-specific changes in the remaining two.
Two deaths were recorded. One patient died suddenly pre-operatively
and one immediately post-operatively.
Conclusion:
Subvalvar aneurysms are a rare cause of mitral regur-
gitation and heart failure in children. Diagnosis is readily confirmed
on echocardiography. Tuberculosis is a frequent association, but the
aetiology remains inconclusive. There may be an underlying congeni-
tal predisposition.
1340: CONGENITAL CENTRAL HYPOVENTILATION
SYNDROME (UNDINE SYNDROME) WITH RECURRENT
HYPERCAPNIAAND HYPOXAEMIA IS LIKELY TOACT AS
ENDOTHELIAL PRECONDITIONING
Damian Hutter
1
, Alain Wimmersberger
2
, Roman Brenner
3
, Stefano
Rimoldi
3
, Emrush Rexhaj
3
, Jean-Pierre Pfammatter
1
, Mladen
Pavlovic
1
, Urs Scherrer
3
, Yves Allemann
3
1
Centre of Congenital Heart Disease, University Hospital, Bern,
Switzerland
2
Santa Maria Hospital, Visp, Switzerland
3
University Hospital, Bern, Switzerland
Introduction
: Undine syndrome (US) is a rare disease with severely
impaired central autonomic control of the breathing and dysfunc-
tion of the autonomous nervous system. The incidence is estimated
to be one in of 200 000 livebirths. Due to reccurent hypercapnia
and hypoxia we hypothesised that these patients have higher risk
for pulmonary arterial hypertension (PHT) and long-term systemic
vascular dysfunction. We examined seven patients with US at base-
line and high altitude with regard to pulmonary artery pressure and
systemic vascular disease and compared them with six age- and
gender-matched subjects.
Methods
: Seven patients with US (five female, two male, mean age
19
±
3 years) and six age- and gender-matched healthy controls (three
female, three male, mean age 20
±
2 years) were examined at 550 m
and at 3 883 m above sea level with echocardiography (to measure
pulmonary artery pressure). Vascular function was measured at 550
m by flow-mediated dilatation with and without oxygen.
Results
: All US patients had mild-to-moderate PHT at 550 m above
sea level. PHT was more pronounced in males than in females (41
±
5 vs 27.4
±
3 mmHg) while no PHT was found in the controls. All US
patients had high-normal systemic vascular function while controls
had normal systemic vascular function. At 3 883 m above sea level,
all US subjects showed only mild increase in PHT with regard to
baseline while controls developped moderate-to-severe pulmonary
hypertension (38
±
7 vs 55
±
17 mmHg).
Conclusion
: Despite recurrent hypercapnia, US patients showed
high-normal vascular function. We speculate that endothelial precon-
ditioning (recurrent hypercapnia) could be a stimulus. Until recently,
recurrent hypercapnia was believed to be the most harmful for
endothelial functional properties. The presence of PHT at 550 m
above sea level was not significantly aggravated by high altitude in
comparison with healthy controls. This underlines the hypothesis of
endothelial preconditioning and identifies environmental hypoxia as
a key trigger for PHT in these patients.
1341: RHEUMATIC HEART DISEASE IN A TERTIARY
HOSPITAL IN MALAYSIA
Hung Liang-Choo, Jasminder Kaur Amarjit Singh, Lee Phaik-Ngan
Institute of Paediatrics, Hospital Kuala Lumpur
