CARDIOVASCULAR JOURNAL OF AFRICA • Vol 23, No 5, June 2012
252
AFRICA
Treatment of subaortic stenosis in hearts with single-
ventricle physiology
BULENT SARITAS, EMRE OZKER, CAN VURAN, ÇAĞRI GUNAYDIN, CANAN AYABAKAN, RIZA TURKOZ
Abstract
Background:
We evaluated the patients who had had a
Damus-Kaye-Stansel (DKS) operation for single-ventricular
physiology with the aorta originating from a hypoplastic
ventricle and the pulmonary artery from the systemic
ventricle.
Methods:
Seven patients who were operated on between May
2007 and November 2010 were evaluated retrospectively.
The patients had been diagnosed with a transposed double-
inlet left ventricle and triscuspid atresia, and had been
waiting for a Fontan operation. Systemic outflow stenosis
was defined echocardiographically as those with a gradient
greater than 20 mmHg, and angiographically those with
greater than 5 mmHg in the subaortic region.
Results:
The mean age and weight of the patients was 15
±
9.7 months and 8
±
3.3 kg, respectively. The mean gradient
between the systemic ventricle and the aorta was 35
±
25
mmHg. This gradient decreased to 14.3
±
4 mmHg postoper-
atively. The early hospital mortality was 14% (one patient).
The mean extubation time and mean time in the intensive
care unit (ICU) were 13
±
7.3 hours and 2.2
±
0.5 days,
respectively. The mean follow-up time was 11
±
2 months. No
mortality and semi-lunar valve insufficiency were observed
after discharge.
Conclusions:
One of the major problems that occur while
waiting for a Fontan operation is systemic ventricular hyper-
trophy and deterioration in the compliance of the ventricle
due to systemic ventricular outflow stenosis.When the disad-
vantages of outflow resection are encountered, a DKS proves
to be a good alternative.
Keywords:
pulmonary artery band, univentricular heart, Fontan
procedure, subaortic stenosis
Submitted 11/4/110, accepted 31/5/11
Cardiovasc J Afr
2012;
23
: 252–254
DOI: 10.5830/CVJA-2011-023
In patients with a double-inlet left ventricle (DILV) or tricuspid
atresia (TA) where the aorta originates from the hypoplastic
Department of Cardiovascular Surgery, Baskent University
Hospital, Istanbul, Turkey
BULENT SARITAS, MD,
EMRE OZKER, MD
CAN VURAN, MD
ÇAĞRI GUNAYDIN, MD
RIZA TURKOZ, MD
Department of Paediatric Cardiology, Baskent University
Hospital, Istanbul, Turkey
CANAN AYABAKAN, MD
ventricle, the interventricular connection that is present at birth
may narrow in time.
1
There is increased pulmonary blood flow in
DILV and TA patients with accompanying aortic arch pathology.
In order to prevent pulmonary vascular disease, pulmonary
artery banding is often preferred as a palliative procedure.
However, ventricular hypertrophy caused by the pulmonary
band may lead to narrowing of the interventricular connection.
2-4
A restrictive ventricular septal defect (VSD) restricts flow from
the systemic ventricle to the aorta, hence leading to progression
of subaortic stenosis.
Enlarging the interventricular connection either by resection
or by performing a Damus-Kaye-Stansel operation are the two
most applied techniques.
5
We present cases of DILV or TA
patients who were found to have subaortic stenosis in their
clinical follow up and underwent DKS operations.
Methods
Seven patients underwent DKS operations between May 2007
and November 2010. These patients had DILV and TA without
any chance of bi-ventricular repair and had developed subaortic
stenosis while they were waiting for Fontan operations. For the
purpose of this study, we defined systemic outflow obstruction
as a resting peak instantaneous gradient greater than 20 mmHg
on echocardiography or a resting peak-to-peak gradient greater
than 5 mmHg with cardiac catheterisation. Systemic outflow
obstruction was considered clinically significant if the patient
had findings of left ventricular hypertrophy.
Three patients had concomitant bi-directional cava-pulmonary
connection (BCPC) operations and one patient had a central
shunt operation at the time of the DKS operation. All patients
were evaluated in terms of postoperative surgical morbidity and
mortality, the degree of subaortic stenosis, ventricle function and
rate of re-operation, and semi-lunar valve insufficiency.
Surgical procedures
Pulmonary artery banding:
three patients with excessive
pulmonary blood flow related to a hypoplastic aorta and the
pulmonary artery originating from a non-hypoplastic ventricle
underwent a pulmonary artery banding operation through an
antero-lateral thoracotomy. One patient also had a Blalock-Hanlon
atrial septectomy in addition to pulmonary artery banding, through
a median sternotomy. The pulmonary band is tightened until the
pressuredistaltothebanddecreasestohalfofthesystemicpressure.
Among these four patients on whom palliative pulmonary
band operations were performed, one patient died in the early
postoperative period. The other three patients were followed up.
The mean age and weight of these four patients was 22
±
12 days
and 3.1
±
1.9 kg, respectively.
Bi-directional cava-pulmonary connection:
three patients with
balanced pulmonary blood flow underwent BCPC operation.
The operations were performed under cardiopulmonary bypass.