CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
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AFRICA
Conclusions:
PVR can be performed with low operative mortality.
The bovine pericardium biological prosthesis presents good short-
and mid-term results, although a longer follow-up is necessary to
confirm the initial results regarding its haemodynamics and durabil-
ity. As with the ideal substitute for PVR, the optimal timing of PVR
remains controversial.
1323: ADULT CONGENITAL HEART DISEASE SUPERVI-
SION IN REGIONAL NEW ZEALAND
Graeme Porter
1
, Jamie Voss
2
, Tim Hornung
2,3
1
Tauranga Hospital, Bay of Plenty, New Zealand
2
Auckland City Hospital, Auckland, New Zealand
3
Starship Hospital, Auckland, New Zealand
Background:
Patients born with congenital heart disease now
commonly live into adult life. Over the last 10 years the care model
in New Zealand for adult congenital heart disease (ACHD) has
changed from follow-up centrally in Auckland to regional follow-
up in conjunction with Starship ACHD service. Tauranga Hospital
(TH) drains a population of just over 210 000 and is approximately a
3-hour drive from Auckland.
Patients and methods:
Our research describes the cohort of 103
patients with ACHD under active follow-up at TH at the end of
August 2012 for the purposes of audit as well as service planning.
Median age is 27 y (range 15–79 y); females 50.5%. The primary
congenital cardiac lesion was classified as severe in 46%, simple
shunt in 23%; the other 31% in accordance with the schema used by
Mackie
et al
. In addition, 3 patients have Eisenmenger physiology
and 1 patient has uncorrected cyanotic congenital heart disease. In
the 2 years until August 2012 196 clinic appointments were attended,
and 140 echocardiograms and 27 cardiac magnetic resonance
imaging (MRI) scans performed. While under local follow-up 14
patients have undergone cardiac surgery (including a single cardiac
transplant) and there have been 16 pregnancies resulting in 13 live
births. Five patients have died (age range 23–55 y) and are not
included in the main analysis. Two of the five patients suffered from
Eisenmenger syndrome.
Conclusions:
A significant proportion of ACHD patients under local
follow-up at TH have severe or complex congenital heart lesions.
Although ACHD patients represent a small proportion of patients
seen in the outpatient setting, they utilise a significant amount of
health resource, particularly during pregnancy. Furthermore, given
that most ACHD patients require lifelong follow-up, clinical leaders
should account for this group when projecting for growth in service
demands.
1366: THE LATE OUTCOMES OF SURGICAL TREATMENT
OF SCIMITAR SYNDROME: NO SIMPLE SOLUTION TO
A COMPLEX FORM OF ANOMALOUS PULMONARY
VENOUS DRAINAGE
Lou Hofmeyr, Andrie Stroebel, Theresa Malpas, Chris Whight, Peter
Pohlner, Mugur Nicolae, Dorothy Radford
The Prince Charles Hospital, Brisbane, Australia
Background:
Scimitar syndrome is a rare congenital cardiac defect
where right-sided pulmonary veins drain to the inferior vena cava.
Complications arise in adulthood in both patients who have had
surgical correction by intra-atrial baffle, and those who have had no
intervention.
Methods:
We contrast the presentation, treatment plans and outcomes
of 18 patients (11 female) with mean age 41 yrs followed between
1978 and 2012 at an adult congenital heart disease unit in Brisbane,
Australia. Age of surgical repair in 13 cases was a mean of 28.1 yrs
(range 3–72). Five patients were never operated on. Clinical and
imaging follow-up was obtained.
Results:
A broad spectrum of presenting complaints was seen.
Associated lesions included right lung hypoplasia and abnormal
right lung arterial supply in 12 patients (66%) and atrial septal
defect (ASD) in 3 (17%). The treatment plan varied between
conservative management, medical therapy and surgical intervention.
Complications in adulthood in the operated group included occlusion
of intra-atrial baffles, right heart dilatation, haemoptysis, pulmonary
hypertension and atrial arrhythmia.
Conclusions:
Management of scimitar syndrome in adulthood
remains complex as patients treated both conservatively and surgi-
cally develop complications. Care should be individualised.
1367: ADVANCED THERAPIES TO TREAT PULMONARY
HYPERTENSION IN COMPLEX CYANOTIC CONGENITAL
HEART DISEASE WITHOUT EISENMENGER PHYSIOL-
OGYAREAN EFFECTIVEAND SAFE LONG-TERMTREAT-
MENT OPTION
Lou Hofmeyr, Catherina Tjahadi, Dorothy Radford, Theresa Malpas,
Mugur Nicolae, Fiona Kermeen
The Prince Charles Hospital, Brisbane, Australia
Background
: Significant improvement in exercise capacity has been
reported in patients with Eisenmenger syndrome with advanced
pulmonary hypertension (PAH) therapies. There is limited evidence
of safety and efficacy in complex congenital heart disease (CHD)
patients who do not have Eisenmenger physiology with shunt reversal.
Aim
: To report the long-term efficacy and safety data with use
of advanced PAH therapies in patients with PAH associated with
complex CHD.
Methods
: A prospective, single-centre study of 16 patients (11
female) with mean age 31.3 years (range 15–52 years) was done;
Fontan circulation (2), pulmonary atresia with ventricular septal
defect (VSD)/MAPCAs (11), truncus arteriosus (1), single ventricle
with VSD/transposition of great arteries (TGA) (1), and TAPVD/atri-
al septal defect (ASD) (1). Thirteen patients were prescribed mono
PAH therapy and 3 combination, between 2004 and 2012. Outcome
measurements of oxygen saturation (SaO2), NYHA-FC, 6-minute
walk test distance (6MWD) and adverse events were analysed.
Results
: Mean duration of therapy was 33.7 months. Significant
improvement was noted: NYHA-FC (baseline mean 3.19 to 2.4)
and 6MWD (pre therapy 343.7 m to 420.3 m). There was no signifi-
cant change in SaO2 or echocardiographic parameters. Two deaths
occurred because of failure of Fontan circulation; 2 patients switched
to sildenafil because of worsening heart failure.
Conclusion
: This single-centre study suggests a significant improve-
ment in functional class and exercise capacity after treatment of PAH
in complex CHD with advanced PAH therapies.
1393: RUPTURED SINUS OF VALSALVA ANEURYSMS IN
ADULT CONGENITAL HEART DISEASE - CLINICAL PRES-
ENTATION, ECHOCARDIOGRAPHIC DIAGNOSIS AND
HISTOPATHOLOGICAL FINDINGS IN 8 CASES
Maryanne Caruana
1
, Mary N Sheppard
2
, Wei Li
1
1
Adult Congenital Heart Disease Unit, Royal Brompton Hospital,
London
2
Imperial Cardiac Pathology Unit, Royal Brompton Hospital, London
Background:
Sinus of Valsalva aneurysms (SVAs) are rare anoma-
lies, often associated with congenital cardiac malformations and
connective tissue disorders. Aneurysm rupture usually triggers clini-
cal deterioration.
Materials and methods:
We searched our echocardiography, histo-
pathology and electronic patient databases between 2004 and 2012.
Clinical details and procedures were obtained from case notes.
Results:
A total of 8 patients (5 females; mean age 35.7 years)
were studied. Aneurysm of right coronary sinus was found in 5 and
non-coronary sinus in 3 cases. Rupture into the right ventricle (RV)
occurred in 5 cases (3 into RV body; 2 into outflow tract) and the
right atrium in 2. Five patients had congenital defects: bicuspid aortic
valve (BAV) 2, ventricular septal defect (VSD) 1, resected subaortic
stenosis 1. Another patient had Klippel-Feil syndrome.
