CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
13
surgical re-intervention were risk factors for prolonged hospital stay.
Conclusion:
Truncus arteriosus communis repair with or without
right ventricle-to-pulmonary artery conduit provided similar early
and mid-term results.
209: CONGENITAL PORTAL VEIN ANOMALY AS A CAUSE
OF SEVERE PULMONARY HYPERTENSION
Iveta Simkova
1
, Monika Kaldararova
2
, Marian Hrebik
2
, Pavel Jansa
3
1
Department of Cardiology , Slovak Medical University and National
Institute of Diseases, Slovakia
2
National Institute of Cardiovascular Diseases, Children’s Cardiac
Centre, Bratislava, Slovakia
3
Department of Internal Medicine II, Division of Cardiology and
Angiology, Czech Republic
Background:
Congenital absence of the portal vein (CAPV) as a
consequence of aberrant venous development in the early embry-
onic period is an extremely rare condition, with only about 40 cases
published. Due to the porto–systemic shunt, the mesenteric–splenic
venous drainage bypasses the liver and drains directly into the
systemic circulation.
Methods:
Although considered rare, in our centre in a period of four
years we detected three cases of CAPV due to portal vein agenesis
with porto–systemic shunting presenting as severe porto–pulmonary
hypertension (PPHT).
Results:
Two young adults (34-year-old female, 36-year-old male)
and a 34-month-old girl presented with pulmonary arterial hyperten-
sion (PAH). Right heart catheterisation confirmed severe PAH in all
three patients (mean PAP: 65/85/47 mmHg and PVR 8/13 WU, 10.4
WU/m
2
, respectively). Due to the clinical course and suspicion of
PPHT, abdominal ultrasonography, MR and CT were performed and
the diagnosis of CAPV with porto–systemic shunts to the inferior
vena cava in all three cases was established, and the diagnosis of
PPHT was confirmed. Due to the functional status (WHO II/III/II),
specific therapy for PAH was initiated as follows: ambrisentan, silde-
nafil and bosentan. In two adult patients, treatment (over seven and
three years) improved continually to functional status WHO I, and
physical performance was highly significantly improved (6 MWT:
+ 128 m/+ 68 m). The follow up as well as therapy of the child was
interrupted after one year due to incompliant parents and according
to the GP’s information, died three months later due to acute respira-
tory failure.
Conclusion:
CAPV is a rare condition, with only a few cases
presented. It is usually associated with cardiac, skeletal and visceral
malformations. The association of CAPV with severe PPHT is
extremely rare but may be more frequent than expected. However
it often remains undiagnosed. The prognosis is dependant on early
diagnosis, as well as management with PAH-specific treatment
including an experienced PH centre.
215: MODIFIED EXPOSURE IN SURGICAL REPAIR OF
TOTAL ANOMALOUS PULMONARY VENOUS CONNEC-
TION: FIVE-YEAR EXPERIENCE
Lyubomyr Bohuta, Evgeniy Segal, Kateryna Chornen’ka, IllyaYemets
Ukrainian Children’s Cardiac Centre, Kyiv, Ukraine
Objective:
Surgery for total anomalous pulmonary venous connec-
tion (TAPVC) requires either anastomosing pulmonary venous
confluence (PVC) to the left atrium (LA) or intra-cardiac baffling.
Limited convenience and lack of space during the creation of an
anastomosis can potentially compromise the repair and outcomes.
The aim of this study was to present our experience with a modified
approach in the repair of TAPVC, which provides superior exposure
and facilitates repair.
Methods:
One-hundred and twenty patients with isolated TAPVC
were operated on in the years 2000 to 2010. Conventional approaches
to surgical exposure were used in 72 patients (group I). Starting from
2006, modified exposure was used in 48 consecutive patients (group
II). For the modified approach, the right pleural cavity is widely
opened, the heart is rotated around the axis of the caval veins into a
pleural cavity and this maneouvre provides better exposure for crea-
tion of anastomosis.
Results:
There were two early deaths (4.2%) in the modified-expo-
sure group. Multifactorial analysis has shown the modified exposure
to be among the factors responsible for improved outcomes. Follow
up was 93.1% with no late deaths or re-interventions in either group.
All survivors were asymptomatic at a median follow up of 31.7
months.
Conclusion:
Anastomosing the LA to the PVC is a key component of
TAPVC repair. Modified surgical approaches to repair may provide
better exposure and superior outcomes. Long-term outcomes are
good with all approaches.
216: DISTINCTIVE HAEMODYNAMICS IN THE IMME-
DIATE POST-OPERATIVE PERIOD OF PATIENTS WITH
A LONGER CARDIAC INTENSIVE CARE STAY POST
TETRALOGY OF FALLOT REPAIR
Babar Hasan, Kisha Beg, Anwarul Haq, Muneer Amanullah, Sobia
Laique, Saleem Sadqani, Mehnaz Atiq
The Aga Khan University, Karachi, Pakistan
Background
: The majority of tetralogy of Fallot (ToF) patients
have up to two days’ stay in the cardiac intensive care unit (CICU)
while some stay longer. We undertook this study to investigate the
differences in immediate postoperative (first four hours) variables in
patients who had shorter compared to those who had a longer stay
in CICU.
Methods
: Patients who underwent ToF repair at the Aga Khan
University, Pakistan between July 2006 and December 2011 were
considered. Exclusion criteria were: mortality in the first 24 hours,
pulmonary atresia, absent pulmonary valve syndrome, infection or
other non-cardiac cause of prolonged CICU stay. Clinical parameters
were compared between the shorter-stay group (SSG) (
≤
2 days) and
the longer-stay group (LSG) (
>
2 days). Continuous variables are
presented as medians.
Results
: Eighty-nine patients (LSG 57, SSG 32) were included.
There was no difference in age at repair (in years) between the
groups (LSG 5 vs SSG 6,
p
=
0.07). LSG had a lower pre-operative
saturation (78%) compared to SSG (85%),
p
=
0.04. LSG had a
significantly longer total bypass time (150 vs 137 min,
p
=
0.02).
Averaged over the initial four post-operative hours, patients in the
LSG had a significantly higher heart rate (136 vs 122 beats/min,
p
=
0.04), central venous pressures (CVP) (11 vs 9 mmHg,
p
=
0.001)
and inotropic score (IS) (10 vs 8,
p
=
0.01). Averaged over the total
CICU stay, the LSG had a significantly higher IS (10 vs 6,
p
=
0.009),
CVP (9 vs 8 mmHg,
p
=
0.01), longer duration on inotropes (2 vs 1
days,
p
=
0.001) and mechanical ventilation (1 vs 0.5 days,
p
=
0.001)
when compared to SSG.
Conclusion
: Patients who ended up staying longer in the CICU had
features that were distinctive in the immediate postoperative period.
These clinical parameters can be used to predict patients who may
need more support and longer CICU stay and thus help in parent
counselling.
217: HEARTFELT: MOTHERS EXPERIENCES OF THEIR
INFANTS FOLLOWING CARDIAC SURGERY
Jennifer Re
1,2,3
, Samuel Menahem
2,3,4
, Suzanne Dean
2
1
School of Public Health and Psychosocial Studies, Auckland
University of Technology, New Zealand
2
School of Psychology and Psychiatry, Monash University, Australia
3
Murdoch Children’s Research Institute (MCRI), Australia
Monash Heart, Monash Medical Centre, Australia
5
Royal Children’s Hospital, Australia
Background
: Many infants with congenital heart disease require
major surgery within weeks of birth. Attachment theory and research
