CARDIOVASCULAR JOURNAL OF AFRICA • Vol 23, No 5, June 2012
AFRICA
253
The main pulmonary arteries were tied up in all patients. The
mean age and weight of the patients was 6
±
2.1 months and 8
±
3.4 kg, respectively.
Damus-Kaye-Stansel procedure:
the decision to do a DKS
operation was taken for seven patients who had subaortic stenosis
in the follow-up period. An 11-day-old patient with pulmonary
stenosis and restrictive interventricular connection underwent a
DKS and central shunt operation. The procedure was performed
under cardiopulmonary bypass and cardiac arrest by the same
surgical team. Cardiopulmonary bypass was achieved with
aortic and bi-caval cannulation. In patients in whom BCPC
operations were planned, the innominate veins were cannulated.
In these patients BCPC operations were performed without the
use of aortic cross clamping. Cardiac arrest was maintained with
antegrade intermittent normothermic blood cardioplegia using
the miniplegia technique.
After removing the pulmonary band, which was placed in
the initial operation, the pulmonary artery and the aorta were
transected. The distal pulmonary artery orifice was closed either
with a patch or primarily, according to the orifice diameter. Then
the adjacent walls of the pulmonary artery and aorta were joined.
The two facing sinuses of both great vessels were sewn together.
In order to prevent a mismatch between the diameter of the new
artery and the distal aorta, the anterior side of the distal aorta was
incised 3 to 4 mm. The new artery was sewn to the distal aorta.
Results
The demographic data are shown in Table 1. The early hospital
mortality was 14% (one patient). After the DKS operation, the
pulmonary blood flow was maintained with a BCPC operation
in six patients, and with a central shunt in one patient. The
11-day-old patient with transposed TA who underwent DKS and
central shunt procedures was taken to ICU with a left ventricular
assist device. The patient could not be weaned from high doses
of inotropic support and died on the fifth postoperative day due
to septicaemia and low cardiac output. The rest of the patients
had an uneventful postoperative course. The mean extubation
time and stay in ICU were 13
±
7.3 hours and 2.2
±
0.5 days,
respectively.
The mean duration between the first palliative operation and
the DKS operation was 6
±
1.8 months. There was no statistical
difference between patients who had pulmonary banding and
patients who had BCPC operations, in terms of timing of the
DKS procedure.
When the pulmonary arteries which had been tied up in
order to create pulmonary atresia in the initial BCPC operation
were transected during the DKS operation, no malformation
was observed in the pulmonary valve. However, there was a
thrombus on the pulmonary valve in two patients. When the
thrombi were removed, the valve structures were found to be
normal. All six patients were discharged home on the sixth
postoperative day in a good condition.
The mean duration of follow up was 11
±
1.2 months.
There was no mortality in the interim. In routine follow-up
echocardiographical measurements, the mean gradient in the
systemic ventricular outflow tract was 14.3
±
4 mmHg. No semi-
lunar valve insufficiency was observed in the follow up. None
of the patients was re-hospitalised and none of those awaiting a
Fontan operation needed re-operation.
Discussion
Double-inlet left ventricle and tricuspid atresia with transposed
great arteries (TA-TGA) are two forms of a single left ventricle
at risk of developing systemic outflow obstruction and poor
outcomes.
2
In these patients, the only way that blood can be
delivered to the aorta is through the VSD. Even when the VSD
is non-restrictive at birth, it may narrow over time and hence
subaortic obstruction becomes apparent.
1
On the other hand, in
patients with distal aortic arch anomalies, pulmonary vascular
disease may develop due to the excessive pulmonary flow.
2,3
In these patients, in order to reduce pulmonary blood
flow, palliative treatment strategies have been established and
pulmonary artery banding is the most common method used.
However, since banding induces ventricular hypertrophy, the
VSD becomes restrictive and subaortic stenosis develops.
4
The
restrictive VSD limits the flow of blood from the systemic
ventricle to the aorta and reduces cardiac output. In addition,
ventricular hypertrophy leads to the decrease in the compliance
of the systemic ventricle. In patients awaiting a Fontan operation,
the ventricle with impaired compliance is unable to maintain the
Fontan circulation.
5
Franklin and colleagues reported 11% survival rate at 10
years in patients with excessive pulmonary blood flow and
systemic outflow obstruction. They also reported 79% survival
rate at 10 years in patients with pulmonary stenosis without
subaortic stenosis.
6
Therefore, subaortic stenosis should be
corrected surgically as early as possible. In order to achieve this
goal, the most commonly used methods are BVF resection and
a DKS operation.
Direct BVF resection was found to have a high incidence
of atrio-ventricular block and high mortality rates.
7
Lan and
colleagues reported 15 complete heart blocks in 44 subaortic
resection patients and found that pacemaker requirement and
the presence of tachyarrhythmia were important risk factors for
mortality.
2
Postoperative semi-lunar valve insufficiency is the most
important disadvantage of the DKS operation. Matitiau and
colleagues reported a 10% rate of postoperative semi-lunar valve
insufficiency. In our echocardiographical evaluations, we did
not detect any semi-lunar valve insufficiency and measured mild
gradients between the systemic ventricle and aorta. However our
mean duration of follow up was not long enough.
There has been no consensus on the optimal timing of
TABLE 1. PRE-OPERATIVEAND PERI-OPERATIVE
FINDINGS OF THE PATIENTS
Age (month)
15
±
9.7
Weight (kg)
8
±
3.3
IVC area index (cm
2
/m
2
)
1.88
±
1.18
Systemic ventricular pressure (mmHg)
132
±
43
Aortic pressure (mmHg)
91
±
14
Gradient (mmHg)
35
±
25
CPB time (min)
113
±
28
ACC (min)
39
±
14
IVC: interventricular connection, CPB: cardio-pulmonary bypass,
ACC: aortic cross clamp.
