Case presentation:
A ten-year-old boy presented to our hospital with progressive exertional
dyspnea and episodes of chest pain. He had undergone surgical repair of
subaortic stenosis and supramitral ring 7 years ago. Transthoracic
echocardiogram (TTE) revealed the following findings: situs inversus
with dextrocardia, LVOTO with peak gradient (PG) of 90 mmHg along with
moderate to severe aortic insufficiency (AI), moderate mitral
regurgitation (MR) with mitral stenosis (MS) (PG across the mitral valve
was 35 mmHg). Diagnostic cardiac catheterization was performed, and
showed tunnel type LVOTO (Figure 1). Based upon these findings, the
indication of Konno-Rastan operation was established. The patient was
prepared appropriately and scheduled for elective surgical repair. The
operation was performed via median sternotomy with great caution to
avoid inadvertent rupture of any cardiac cavity due to the heavy
adhesions from previous operation, and dextrocardia was kept in mind.
The aorta was cannulated just below the take off of the innominate
artery to gain as much length as possible on the ascending aorta.
Bicaval cannulation was performed on the left side (Dextrocardia) to
have a bloodless field. We placed a left ventricular vent via the left
superior pulmonary vein. Aortic cross clamp was applied and the
ascending aorta was opened longitudinally on the anterior aspect (Figure
2), and the cardioplegic solution was administered via the coronary
ostia due to the severe AI. This incision was extended into the right
coronary sinus to the right of the right coronary ostium (Dextrocardia)
(Figure 3). By staying close to the right/left commissure, the
conduction system is protected. The right ventricular outflow tract was
opened and then cutting through the aortic annulus and
ventriculo-infundibular fold into the ventricular septum (Figure 4).
This incision is made between the right ostium and right/left
commissure, staying closer to the right/left commissure as shown in
Figure 3. The septal incision is usually about 10-15 mm in length and
allows the aortic annulus to separate nicely (Figure 5). A sizer was
placed to estimate the patch width and decide if the septal incision is
adequate. The width of the patch will equal the additional annular
circumference. The inferior aspect of the patch was sewn to the defect
created in the ventricular septum (Figure 6). The superior aspect of the
patch was used to augment the aortic sinus and sinotubular junction.
Then, 2-0 ethibond horizontal mattress sutures were passed through the
aortic annulus as per any aortic valve replacement and some of these
sutures were passed through the patch in correspondence with the aortic
annulus. The superior aspect of the patch was sutured to the aortotomy,
and a second patch was used to augment the right ventricular outflow
tract. The remainder of the operation was completed uneventfully.
Postoperative TTE showed residual subaortic stenosis with PG of 40 mmHg,
and there was significant improvement of MS with PG across the mitral
valve of 12 mmHg. The patient was followed up for 6 months and was
asymptomatic with normal physical activity.