CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
127
Acute onset of breathlessness or heart failure was the common-
est manifestation (5/8). Clinical examination revealed a continuous
murmur in 7/8 patients. Two patients were asymptomatic at time of
diagnosis and one with Klippel-Feil syndrome died suddenly.
Echocardiography demonstrated a continuous shunt across the
rupture site in 7/8 cases. Seven patients had a successful procedure (6
surgical, 1 device closure) and remain well to date. Histopathological
findings in the patients with concomitant BAV were focal disor-
ganisation of the aortic wall elastic layers with increase in smooth-
muscle cells and medial and subintimal fibrosis. In the Klippel-Feil
fatal case, a linear rupture was found in the non-coronary SVA with
thinning of the aneurysmal wall and medial elastin replaced by fibro-
myxoid connective tissue.
Conclusions:
SVAs arise mainly from congenital defects of the aortic
media. Sudden onset of dyspnoea with continuous murmur suggests
aneurysm rupture. Rarely it can result in sudden death. Commonly
coexisting cardiac lesions are BAV and VSD. Echocardiography is
the key to diagnosis. Timely surgical or device closure has a good
long-term outcome.
1440: PULMONARY CONDUIT FUNCTION AFTER THE
ROSS OPERATION: LONGITUDINAL ANALYSIS OF THE
GERMAN-DUTCH ROSS REGISTRY EXPERIENCE
Mostafa Mokhles
1
, Efstratios Charitos
2
, Ulrich Stierle
2
, Jeevanantham
Rajeswaran
3
, Eugene Blackstone
3,4
, Ad Bogers
1
, Johanna Takkenberg
1
,
Hans-Hinrich Sievers
2
1
Erasmus Medical Center, Department of Cardiothoracic Surgery,
Rotterdam, Netherlands
2
University of Luebeck, Department of Cardiac and ThoracicVascular
Surgery, Germany’
3
Research Institute, Department of Quantitative Health Sciences,
Cleveland, USA
4
Cleveland Clinic, Department of Thoracic and Cardiovascular
Surgery, Cleveland, USA
Background:
The Ross operation presents with excellent mid-term
results. However, the prevalence and predictors of late pulmonary
conduit failure after the Ross procedure have been addressed only in
few reports with small patient numbers. The objective of this study
was to determine the natural dynamics of pulmonary conduit stenosis
and regurgitation.
Materials and methods:
Among 1 775 consecutive patients (mean
age
±
standard deviation (SD), 43.7
±
12.0, range 16.1–70.5 years)
that underwent the Ross procedure, 1 645 (93%) received an allo-
graft (pulmonary 1 612, aortic 12, unknown 21), 120 (6%) received
a bioprosthesis, and 5 (0.3%) received a bovine jugular vein for the
reconstruction of the right ventricular outflow tract. Using non-linear
longitudinal models, serial echocardiographic records (
n
=
6 950)
from these patients were studied with a maximum echocardiographic
follow-up of 22.4 years (mean
±
SD, 5.5
±
4.3 years).
Results:
A slight increase in pulmonary conduit regurgitation grade
was observed during follow-up. Freedom from regurgitation grade
≥
2+ was 95% after 14 years of follow-up. Female
patient
gender, the
use of an allograft (compared to bioprosthesis), male
donor
gender,
antibiotic treatment of the allograft, and specific surgical adjustments
were associated with a significantly higher regurgitation grade. Mean
conduit gradient increased from about 4.7 mmHg at 1 month to about
10 mmHg by 14 years after the procedure. Peak gradient increased
from about 8.4 mmHg at 1 month to about 18.5 mmHg by 14 years
after the procedure. Smaller conduit diameter, male
patient
gender,
younger
patient
age, younger
donor
age, and use of bioprosthesis
were associated with significantly higher mean and peak gradi-
ent. The changes in echocardiographic measurements were mainly
observed in the first 2 years after surgery.
Conclusion:
Echocardiographic follow-up of pulmonary conduits
shows outstanding conduit durability. Clinically important conduit
regurgitation and stenosis is rare in patients after the Ross operation.
Consideration of risk-associated predictors may improve pulmonary
conduit outcome.
1454: AVERY RARE CASE OF UNOPERATEDADULTWITH
TRICUSPID ATRESIA AND SUCCESSFUL PREGNANCY
Srilatha Alapati, Gurur Biliciler-Denktas
University of Texas, USA
A 46-year-old female with tricuspid atresia, ventricular septal defect
(VSD) and d-transposition of great arteries (d-TGA) was diagnosed
during infancy and her family deferred surgical palliation becaue of
lack of symptoms (1966). The patient transferred her care to us in
2009 for routine yearly follow-up. Her review of symptoms included
cyanosis, occasional palpitations, and dyspnoea on exertion (NYHA
class II) but no chest pain, dizziness or easy fatigability. She works
at an office. Current medications include digoxin 0.25 mg once
daily. Her obstetric history was G1P1L1A0. She had an uneventful
pregnancy and delivered a healthy baby girl at 36 weeks’ gestation.
The imaging studies including transthoracic echocardiogram and
magnetic resonance imaging (MRI) studies revealed tricuspid atresia
type IIb, d-TGA and bilateral branch pulmonary artery stenosis, and
adequate sized VSD and atrial septal defects (ASDs). The electrocar-
diograph (ECG) showed sinus rhythm and normal QRS axis, which
is usually seen in about 50% of cases with type II tricuspid atresia.
The reason for her long-term survival was very well balanced
pulmonary and systemic circulation. This is facilitated mainly by
adequate size of ASD which allowed blood flow from right atrium
to left atrium and adequate size of VSD with free flow of blood into
aorta. She also had bilateral branch pulmonary artery, controlling
amount of pulmonary blood flow.
Long-term survival is very rare in unoperated tricuspid atresia
patients. Only three patients including our patient have survived into
the 5
th
decade. Our patient had long-term survival without surgery
because of her favourable anatomy for this condition and well-
balanced circulation. To our knowledge, this is the first report of
successful pregnancy in an unrepaired tricuspid atresia with TGA.
1471: ASSOCIATION OF EMERGENCY ADMISSION WITH
HOSPITAL LENGTH OF STAY INADULTSWITH CONGEN-
ITAL HEART DISEASE
Ju Ryoung Moon
1
, June Huh
2
, I-Seok Kang
2
, Ji-HyukYang
3
, Tae-Gook
Jun
3
, Sung A Chang
4
, Seung Woo Park
4
, Heung Jae Lee
2
1
GUCH clinic, Samsung Medical Center, Seoul, Korea
2
Department of Pediatrics, Sungkyunkwan University School of
Medicine, Seoul, Korea
3
Department of Thoracic and Cardiovascular Surgery, Sungkyunkwan
University School of Medicine Seoul, Korea
4
Department of Internal Medicine, Sungkyunkwan University School
of Medicine, Seoul, Korea
Background
: In the field of emergency care, the increasing needs of
adults with congenital heart disease (CHD) are going to add to the
burden on medical resources. The aim of this study was to investi-
gate the reasons for emergency admission and its associations with
increased hospital length of stay (LOS).
Methods
: We evaluated 2 720 adults with CHD in a single tertiary
cardiac centre database. Patients who required emergency admission
between September 1994 and December 2011 were reviewed from
medical records.
Results
: A total of 669 adult CHD patients (24%) required emer-
gency admission, culminating in a total of 1 726 admissions. Mean
age was 48.4
±
18.5 years, with a female predominance (57%). Most
were either studying or working (62%) and were married (64%).
Atrial septal defect (43.8%), ventricular septal defect (21.5%)
and tetralogy of Fallot (7.2%) formed the majority of diagnoses.
One-third of the admissions were for cardiac reasons including
heart failure, endocarditis, arrhythmias, haemoptysis and thrombo-
embolism. The remaining two-thirds were admitted for non-cardiac
reasons. Median hospital LOS was 14.5
±
5.6 days. Those who were
older (
p
=
0.02), and neither employed nor studying (
p
=
0.021) had
longer LOS. Endocarditis accounted for longer LOS (
p
<
0.001);
41% of the admissions that required interdisciplinary referrals had
