CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
229
Background:
Marfan syndrome (MFS) is a connective tissue disor-
der with major features in cardiovascular, ocular and skeletal systems.
The diagnostic criteria were revised in 2010 and more weight was
given to the aortic root dilatation, ectopia lentis and genetic study.
Methods:
This was a retrospective study of every patient evaluated
for MFS in our centre over the last 15 years. We aimed to analyse the
practical clinical implications of the revised nosology in a paediatric
population.
Results:
The study included 18 patients (nine girsl), with the first
evaluation in paediatric cardiology at the mean age of 6.7 years (6
days
–
16.8 years). There was a positive family history in four cases.
The diagnosis of MFS by the classical Ghent nosology was estab-
lished in 12 patients; eight patients at the first consultation and in the
rest, 2.8 years later, confirming that MFS has an evolving phenotype.
With the revised criteria, the diagnosis of MFS remained in 11/12
patients and two new patients were diagnosed. The diagnosis of the
five rejected patients were: two as MASS phenotype (in the absence
of aortic dilatation), two as mitral valve prolapse syndrome and one
as potential MFS in patients under 18 years. The diagnosis was not
confirmed because the
z
-score of the aortic root was
<
2 or 3.
Conclusions:
The revised Marfan nosology led to a different diagnosis
in three cases; one of the MFS patients was reclassified as MASS;
conversely, two were reclassified as MFS in the presence of aortic
dilatation. The diagnosis of MFS was rejected mostly because of the
absence of aortic root dilatation defined as
z
-score
≥
2 or 3. All patients
suspected of MFS maintain follow up in paediatric cardiology, consid-
ering the possibility that they may develop the classic MFS with time.
1308: THE RELATIONSHIP OF A LEFT ATRIAL MYXOMA
WITH THE MITRAL VALVE IS BEST EVALUATED BY 3D
ECHOCARDIOGRAPHY
Marie Jose Raboisson
1
, Ahmed Abdul Salam
2
, Hatem Hosny
2
, Nancy
Poirier
1
1
Ste Justine University Hospital Centre, Montreal, Canada
2
Aswan Heart Centre, Aswan, Egypt
Background:
Seventy-five per cent of myxomas are located in the
left atrium, the majority originating from the interatrial septum. They
can come in contact with vital structures. We used intra-operative
3D TEE to reassess a myxoma of the left atrium just before surgery.
Method and Results:
A 51-year-old woman consulted the Aswan
Heart Centre for progressive mild dyspnoea. Her physical findings
were compatible with bronchial reactivity without signs of cardiac
pathology. Her chest X-ray and ECG were normal. The TTE showed
a sessile mass of 13
×
18 mm compatible with a myxoma attached to
the left side of the inter-atrial septum without other anomalies. She
was brought to the operating room for elective resection of the myxo-
ma. A real-time 3D TEE was performed pre-operatively, followed by
a full-volume acquisition with a systematic cropping of the 3D TEE
dataset in multiple plans. Reconstructions showed a tumour measur-
ing 14
×
19 mm attached to the lower part of the interatrial septum
in close contact with the anterior leaflet of the mitral valve as well
as the mitral annulus. The space between the mitral annulus and the
myxoma was estimated at 5 mm. Transmitral blood flow was normal.
There were echolucent areas in the tumour compatible with necrosis.
After cardiopulmonary bypass and cardiac arrest with blood cardio-
plegia, a right atriotomy was performed and the myxoma completely
resected without traumatising the mitral valve. The septum was then
reconstructed with an autologous pericardial patch. The postoperative
course was simple and the patient discharged on day six.
Conclusion:
3D echocardiography facilitated description of a left
atrial myxoma better than 2D. It provided a better understanding of
the relationships with adjacent structures, especially regarding the
space between the tumour and vital structures, and should be consid-
ered as first-line examination in order to help plan surgery and avoid
complications.
1314: PERSISTENT RETINAL VASCULAR CHANGES IN
KAWASAKI SYNDROME: POTENTIAL ROLE IN CORO-
NARY RISK STRATIFICATION
CWT Lim
1
, IBY Wong
2
, A Pang
4
, XP He
1
, CPP Hia
1
, C Cheung
3
, TY
Wong
2
, SC Quek
1
1
Department of Paediatrics, National University Health System,
Singapore
2
Department of Ophthalmology, National University Health System,
Singapore
3
Singapore Eye Research Institute, Singapore
4
Department of Ophthalmology, Tan Tock Seng Hospital, Singapore
Background
: We hypothesised that the inflammatory effects of
Kawasaki syndrome (KS) causes longstanding changes in the retinal
vasculature akin to traditional coronary risk factors such as diabetes
mellitus and hypertension.
Methods:
We compared the retinal vascular dimensions of a cohort
with a history of KS to matched controls. All subjects underwent
high-resolution digital retinal photography in which the diameters
of all arterioles and venules coursing through a specified area one-
half to one disc diameter from the optic disc were measured with a
computer program (IVAN), according to a published standardised
protocol. Central retinal arteriolar equivalent (CRAE), central retinal
venular equivalent (CRVE), and arteriole-to-venule ratio (AVR) were
calculated for each retinal photo.
Results:
Thirty-two subjects with a history of KS and 138 controls
were examined. KS subjects had a mean CRAE of 149.77 µm, a mean
CRVE of 211.61 while controls had a mean CRAE of 147.25 µm (
p
=
0.300), and a mean CRVE of 221.11(
p
<
0. 001). Cases were matched
for age, gender, ethnicity, body surface area and also controlled for
calibre of neighboring vessels.
Conclusions
: KS results in independent, persistent and substantial
narrowing of the retinal venules. Such an effect may be related to
endothelial dysfunction and serve as a potential marker for incipient
coronary vasculopathy.
1320:TWO CASES OF POST-FONTANWARFARIN-INDUCED
TRACHEOBRONCHIAL CARTILAGE CALCIFICATION
Luke Eckersley, Nigel Wilson, Chris Occleshaw, John Stirling
Green Lane Paediatric and Congenital Cardiac Service, Starship
Children’s Health, Auckland, New Zealand
Background
: This study identifies tracheobronchial cartilage calci-
fication in children with congenital heart disease. Calcification of
the tracheobronchial airways has previously been found to be more
common in adults taking warfarin, and has been described in children
who are receiving warfarin following mitral valve replacement.
Methods
: A nine-year-old female who had undergone a Fontan
repair six years previously underwent a cardiac CT to further image
her pulmonary arteries. An incidental finding of extensive tracheo-
bronchial cartilage calcification was noted. We then conducted a
retrospective review of all the paediatric Fontan patients who had
undergone cardiac CT to look for calcification of the tracheobron-
chial cartilage.
Results
: Ten paediatric Fontan patients had undergone cardiac CT
scan. Two patients with extensive calcification of the tracheobron-
chial airways were identified. A nine-year-old female with a cardiac
diagnosis of hypoplastic left heart syndrome had undergone a staged
repair with Fontan completion at age three years. A 16-year-old male
with a cardiac diagnosis of tricuspid atresia underwent staged repair
and Fontan completion at age 3.5 years. They had received continu-
ous warfarin for six and 13 years, respectively. Other common causes
of airway calcification were excluded.
Conclusions
: We describe warfarin-induced tracheobronchial calci-
fication in patients following the Fontan procedure. This finding has
potential implications for airway growth and vascular calcification.
