Cardiovascular Journal of Africa: Vol 24 No 1 (February 2013) - page 21

CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013 AFRICA 19
Background: We report on a retrospective analysis of the demo-
graphic, morphological and clinical profiles of patients along with
results of operative repair for total anomalous pulmonary venous
connection.
Methods: In the last five years, 65 patients (45 males, 20 females)
underwent repair for total anomalous pulmonary venous connec-
tion. Ages ranged from two days to nine years (median six, mean 17
months) and 46 were under one year old. Weight ranged from 2.4
to 18 kg (median 5 kg). The anomalous connection was supracar-
diac in 35 (54%), cardiac in 18 (27%), infracardiac in seven (10%)
and mixed in five (8%) patients. Twenty-five (38%) patients had
obstructed drainage and 40 (61%) had moderate or severe pulmonary
arterial hypertension; 21 patients (32%) had to be operated on as an
emergency.
Results: Mortality was more in obstructed drainage patients (26%)
compared with patients without obstruction (8%).The major causes
of early death were weight < 10% (OR 1.1; 95% CI: 0.1–6.5, p =
0.009), obstruction (OR 9.8; 95% CI: 1.6–60, p = 0.006) and sepsis
(OR 23.3; 95% CI: 3–177, p = 0.002). Follow up ranged from one to
45 months (median 24 months). There was one re-operation due to
late pulmonary vein stenosis but the patient later died.
Conclusions: In a developing country such as Pakistan, mortal-
ity continues to be high in infants with total anomalous pulmonary
venous connection. Weight < 10th percentile, obstruction, severe
pulmonary arterial hypertension and postoperative sepsis appear to
be the most important predictors of operative mortality.
408: MORTALITY OUTCOMES UPTO ONEYEAR FOLLOW-
ING PAEDIATRIC CARDIAC SURGERY IN WESTERN
AUSTRALIA (2001–2010)
Gitanjali Mansukhani, David Andrews and James Ramsay
Children’s Cardiac Centre, Princess Margaret Hospital for Children,
Perth, Western Australia
Background: Paediatric cardiac surgical outcomes have been tradi-
tionally described as 30-day or in-hospital mortality. There are
limited reports of late deaths up to one year after surgery. This study
aimed to determine the mortality outcomes for cardiac surgeries
performed on patients from western Australia (WA) from 1 January
2001 to 31 December 2010 up to one year after the procedures, and
to ascertain possible causes.
Methods: Data were obtained and cross-linked from the Department’s
customised surgical and clinical databases, and medical records were
reviewed. Inclusion criteria: paediatric cardiac surgical cases oper-
ated on between 1 January 2001 and 31 December 2010 in WA and
interstate; and deaths occurring up to one year post surgery. Thoracic,
non-cardiac surgeries and pre-term neonates with PDA ligation were
excluded.
Results: A total of 1 198 cardiac surgical procedures were performed
on WA children, of which 128 surgeries were performed interstate;
30-day (early) and one-year (> 30 < 365 day/late) mortality rates
were 2.3 and 1.4%, respectively. In-hospital mortality was 2.6%. The
cause of the majority of late deaths (10/17) was cardiac but non-
surgical related, with pulmonary hypertension and pulmonary venous
obstruction being common. Two/17 late deaths appeared directly
related to cardiac surgery. The remainder (5/17) were unrelated to the
cardiac problem or unknown. Other high-risk factors included indig-
enous children living in remote locations, and trisomy 21 following
AVSD repair.
Conclusions: The overall mortality rate at one year following all
paediatric cardiac surgeries between 2001 and 2010 on WA children
was 3.7%. The 30-day mortality rate was 2.3%. Late mortality up to
one year added 1.4%. Some of the high-risk factors identified for
late deaths are being approached by specific changes in management
and follow up. Parents should be informed regarding the potential for
ongoing risk of mortality in high-risk situations.
411: 29 YEARS OF FOLLOW UP IN THE DAMUS-KAYE-
STANSEL PROCEDURE: ANASTOMOSIS REVISION AND
NEED FOR VALVE REPAIR
Callie Rzasa1,2, Vishal Nigam1,2, Howaida El-Said1,2, John Moore1,2,
John Lamberti1,2, James Perry1,2
1University of California, San Diego, USA
2Rady Children’s Hospital, San Diego, USA
Background: While the Damus-Kaye-Stansel (DKS) procedure has
been used for complex congenital heart disease and systemic outflow
tract obstruction, limited data are published on late outcomes. This
study examined the incidence of surgical intervention in patients with
more than one year of follow up after DKS over 29 years.
Methods: This was a retrospective study on 54 patients who under-
went DKS (not including Norwood) from 1983 to 2007. Patients were
excluded if there was no follow up beyond one year postoperatively
(three), or if death occurred within one year of surgery (14). Survival
was calculated from the date of DKS to the last known follow up or
time of death from 1984 to 2012.
Results: Thirty-seven patients met the study criteria, with a median
follow up of 12.9 years (2–28 years). Diagnoses included double-
inlet ventricle (16), double-outlet right ventricle variants (12), tricus-
pid atresia (six), and others (three). Seven patients (18.9%) experi-
enced death over one year after DKS (4.6–24.2 years). Five deaths
were sudden at ages 13 to 30 years. One death occurred due to sepsis
complicating a MRSA-infected pseudoaneurysm of DKS. One death
occurred awaiting transplant. The mean number of cardiac surger-
ies was three, median 3.5 for the 37 patients reviewed; 28 patiens
(75.7%) obtained Fontan status. Eight patients (21.6%) required
DKS anastomosis revision due to aneurysmal DKS connections in
four patients and outflow tract stenosis in four; 15 patients (40.5%)
required repair/replacement of a semilunar valve.
Conclusions: Our review demonstrates a high incidence of aneu-
rysmal DKS connections, stenosis of the systemic outflow tract,
valvular insufficiency and sudden death. These patients warrant close
long-term surveillance with imaging, arrhythmia and heart-failure
assessments.
443: HEART TRANSPLANTATION IN PATIENTS WITH
SINGLE VENTRICLE: A SINGLE-CENTRE EXPERIENCE
Martin B Riester, Julia Birnbaum, Rainer Kozlik-Feldmann, Robert
Dalla Pozza, Alexandra Fuchs, Anja Lehner, Heinrich Netz, Christoph
Schmitz, Edward Malec, Katarzyna Januszewska
Department of Paediatric Cardiology, Ludwig Maximilian University,
Munich, Germany
Background: In the 1990s, heart transplantation was a common
first-line therapeutic option for many single-ventricle congenital
heart diseases. The aim of this study was to present our centre’s expe-
rience with heart transplantation in these patients.
Methods: A retrospective review of 105 children and adult patients
with congenital heart malformation who underwent cardiac trans-
plantation between 1988 and 2012 revealed 22 patients who were
transplanted for a single right (n = 18) or left (n = 4) ventricle. Group
1 (n = 13) had not been operated on before transplantation or under-
gone preliminary palliative surgery. Group 2 (n = 9) had undergone
a partial (n = 3) or total ( n = 6) cavopulmonary anastomosis. Median
age at transplantation was 0.2 years (5 days to 21.2 years) in group 1
and 15.2 years (2.4 to 34.6 years) in group 2.
Results: The median follow up in the study series was 8.3 years
(3.5 to 18.1 years) and was complete in 100%. The overall mortal-
ity rate was 45% (n = 10) with a hospital mortality rate of 27% (n =
6). Three patients had to be re-transplanted. The five-year survival
rates after transplantation were 69% in group 1 and 53% in group
2. Kaplan-Meier analysis showed no significant difference between
the two groups in the log-rank test (p = 0.39) and an overall median
survival of 11 years. For comparison, the five-year survival rate of
all 105 patients was 82%.