CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
90
AFRICA
reported so far. We describe a foetus with Uhl’s anomaly that initially
mimicked Ebstein’s disease.
Case report:
A 22-year-old pregnant woman was referred for
suspicion of congenital heart disease at 21 weeks’ gestation (WG).
Initial echocardiographic evaluation concluded apical displacement
of the tricuspid septal leaflet compatible with an Ebstein’s anomaly,
with mild RV and right atrial (RA) dilatation. The RV free wall was
surprisingly thin and hypokinetic. Two additional muscular ventricu-
lar septal defects (VSDs) were also reported. There was no tricuspid
regurgitation and pulmonary flow, and flow in the ductus arteriosus
(DA) and ductus venosus (DV) were normal. At 27 WG, significant
cardiomegaly and profound A waves in the DV were reported, with
moderate tricuspid regurgitation and RA dilatation. The DA showed
bidirectional flow reflecting decreased RV output. At 30 WG foetal
supraventricular tachycardia at 230 bpm occurred leading to foetal
hydrops. There was no antegrade pulmonary blood flow. The RV
free wall was even more abnormally thin and Uhl’s anomaly was
suspected. Foetal demise occurred at 31 WG.
Discussion:
Uhl’s anomaly is a rare cause of foetal RV dilatation
and can be associated with tricuspid dysplasia or pulmonary atresia.
A cautious analysis of the RV free wall showing an abnormally thin
myocardium and decreased myocardial thickening should help making
the differential diagnosis between tricuspid disease and Uhl’s anomaly.
The evolution is poor with low RV output, progressive dilatation of the
right heart chambers and rhythm disturbances leading to foetal demise.
1333: INTERMEDIATE AND LONG-TERM OUTCOME OF
DEVICE CLOSURE OF PATENT ARTERIAL DUCT WITH
SEVERE PULMONARY HYPERTENSION
Masood Sadiq
1
, Asif-Ur Rehman
1
, Najam Hyder
1
, Ahmad Usaid
Qureshi
1
, Tehmina Kazmi
1
, Shakeel Ahmed Qureshi
2
The Children’s Hospital and Punajb Institute of Cardiology, Lahore,
Pakistan
2
Evelina Children’s Hospital, London, UK
Background:
A subset of patients with large patent ductus arteriosus
(PDA) and severe pulmonary arterial hypertension (PAH) progress-
es to adulthood without established pulmonary vascular disease.
Whether to close or not to close and how to close these PDAs is often
a difficult decision.
Objectives:
To evaluate the results of device closure of large PDA
with severe PAH after 2 years of age and report their intermediate
and long-term outcome.
Methods:
Fifty-seven patients with large PDA and severe PAH
(pulmonary artery pressure (PAP) more than 2/3rd systemic) with no
desaturation were considered for closure. The reversibility of PAH
was assessed with 100% O
2
and trial balloon/device occlusion. Mean
age was 12.5 + 1.4 (2–55) years and mean weight 27.4 + 2.1 (9–70)
kg. The follow-up was primarily based on echocardiography while 4
patients with persistent PAH underwent repeat catheterisation.
Results:
Fifty patients demonstrated a decrease in PAP and device
closure was successful in 47/49 (96%). Three patients underwent
surgery, two after unsuccessful device closure. Seven (12.3%) patients
did not demonstrate a decrease after trial occlusion. Amplatzer ductal
occluder (ADO) was used in 30/49 (61.2%), MVSD device in 14
(28.6%) and Amplatzer septal occluder (ASO) in 5 (10.2%) patients.
There was a significant decrease in peak systolic PAP (81
±
4 vs 61
±
3 mmHg,
p
<
0.001) and mean PAP (63
±
2 vs 46
±
2 mmHg,
p
<
0.001) following device occlusion. The device embolised to the left
pulmonary artery (LPA) in one patient needing surgery. At a mean
follow-up of 49.2
±
3.7(6–121) months, there were no deaths, late
embolisation or aortic protrusion. Mild LPA stenosis in 2 patients
did not progress. Significant PAH has persisted in 4/50 (8%) patients.
Conclusions:
Device closure of large PDA causing severe PAH with
Amplatzer devices is safe and effective. Despite symptomatic improve-
ment, PAH may not regress in 8% of the patients. In addition to more
diligent non-invasive assessment, an accurate measurement of pulmo-
nary vascular resistance, together with direct measurement of PAP
after trial occlusion may help further exclusion of borderline patients.
1352: MEDIUM-TERM FOLLOW UP OF ENDOVASCULAR
STENTING FOR NATIVE AND RECURRENT COARCTA-
TION OF THE AORTA: A SINGLE CENTRE EXPERIENCE
Jonathan Mervis
1
, Michael Mullin
2
, Michael Rigby
1
, Anselm Uebing
1
,
Alan Magee
1
1
The Royal Brompton and Harefield NHS Foundation Trust, London,
UK
2
The Heart Hospital University College, London Hospital Trust
Aim
: To describe the complete single-centre experience of transcath-
eter stenting of native and recurrent coarctation of the aorta (CoA).
Patients and methods
: From December 2000 to October 2011 160
patients (92 male) underwent transcatheter stenting for coarctation of
the aorta; 107 had native coarctation (67%) and 53 re-coarctation, 11
of whom had previously undergone balloon aortoplasty. Median age
and weight at procedure were 20.5 yrs (6–65) and 63 kg (24–116)
respectively. Bare metal stents were used in 131 patients and covered
stents were used in 29 (14%). Four patients had more than one stent
inserted during the procedure. Follow-up data were available for 129
at a median interval of 56 months (range 4 months – 10 years).
Results
: Invasive peak systolic gradient decreased from mean 28
mmHg (
±
13) to 5 mmHg (
±
6). Coarctation site increased from mean
6 mm (
±
3) to 13 mm (
±
2.5). There were no deaths, 8 (5%) patients
suffered procedure-related complications (1 acute pulmonary oede-
ma, 1 para-aortic aneurysm, 1 aortic rupture, 5 embolised stents).
Right arm systolic blood pressure decreased from mean of 150
±
20
mmHg to 128
±
16 mmHg (
p
<
0.0001). Seventy-five (47%) remain
on antihypertensive medication. Echocardiographic Doppler-derived
gradient decreased from mean of 58.7 mmHg (
±
15.8) to 25.1 mmHg
(
±
14.3) (
p
<
0.0001). Eighty patients (50%) had post-procedural
computed tomography (CT) angiograms at approximately 3 months.
Only one aneurysm was identified which was unchanged from the
post-procedure angiogram. There were no late complications.
Conclusions
: Transcatheter stenting of native and recurrent CoA is a
safe and effective therapeutic option in this age group with benefits
maintained in the medium term. Complications tend to occur at the
time of the procedure. Routine post-procedural CT angiography does
not appear to be necessary and the place of covered stents has still
to be established.
1356: SUCCESSFUL TRANSCATHETER CLOSURE OF
A PATENT DUCTUS VENOSUS WITH AN AMPLATZER
VASCULAR PLUG II IN AN INFANT
Jayneel Joshi, Belinda Jane Mitchell, SAhmad, C Kock
1
, FF Takawira
Steve Biko Academic Hospital, University of Pretoria, South Africa
The ductus venosus is an embryological vascular structure that
connects the umbilical vein with the inferior vena cava. The ductus
venosus rarely persists beyond 2 weeks after birth. If however it
remains patent beyond this period and the resulting portosystemic
shunt is significant, it can be an extremely rare cause of liver failure.
We present a rare case of a large patent ductus venosus causing chole-
static jaundice and severe liver dysfunction in a 6-day-old neonate.
Abdominal sonography demonstrated a large vascular channel adja-
cent to the ligamentum teres which was identified to be patent ductus
venosus.
Computed tomography (CT) angiography was unsuccessful
because of technical difficulties, and because of the deteriorating
liver function a combined diagnostic and interventional catheterisa-
tion was planned.
At catheterisation a 6F sheath was inserted into the right internal
jugular vein. Angiography demonstrated a large patent ductus veno-
sus measuring 6 mm at its widest diameter and 26 mm in length.
Balloon occlusion was performed with a wedge pressure catheter in
order to measure the portal pressures once the ductus was occluded,
which showed no significant increase.
The decision was then made to occlude the ductus venosus using
a 12 mm Amplatzer vascular plug type II. The device was deployed
with some initial occlusion to the normal hepatic venous drainage. It
