Open or closed surgical valvotomy or BAV may
offer interim palliation in infants with critical aortic
stenosis with normal biventricular heart. However,
severe aortic regurgitation or residual aortic stenosis
may subsequently develop.[
3,
4] Therefore, a rapid and
radical management approach may be required, as in
our case. The main disadvantages of prosthetic valve
replacement in neonates and infants with critical aortic
stenosis include implanting an appropriate prosthetic
valve by expanding aortic annulus, need for re-do
surgery in the following years, and challenges in
using anticoagulants.[
6] On the other hand, aortic
valve replacement (AVR) using homografts is not
a reasonable alternative due to the lack of growth
potential and rapid degeneration of allografts in
pediatric population, as well as difficulty in homograft
supplying.[
7]
The Ross procedure is the most reasonable treatment
of choice in neonates and infants requiring AVR.
However, an optimal compliance between pulmonary
autograft and aortic annulus, growth potential,
acceptable durability profile of pulmonary autograft in
the mid- and long-term and no need for anticoagulants are the main advantages of the procedure. In addition,
it can be combined with the Konno procedure in the
presence of complex LVOTO.[1,5,8-10] In our case, aortic
annulus was within normal size (9 mm). We would
also perform the Ross-Konno procedure in case of
annulus hypoplasia.[9]
Although the Ross procedure is a technically
challenging and time-consuming intervention, it can
be safely applied with a mortality rate below 5%.[1,4,8-10]
However, there are still concerns regarding putting at
a risk of both valves, autograft dilatation in the longterm
and need for re-do surgery of the right ventricular
outflow tract conduit.[11-13] There are several studies
showing less annular and sinotubular dilatation of
the autograft and aortic regurgitation in neonates and
infants.[1,4] Maeda et al.[1] reported that 74% (7±12.9) of
infants had very mild aortic regurgitation at five years
following the Ross-Konno procedure. Shinkava et
al.[5] also reported that 95.2% of infants had excellent
autograft functions which tended to grow with
increasing age in a 10-year follow-up period. This may
be explained by an ongoing histological structuring
of semilunar valves (mucopolysaccharide and collagen
balance) in neonates and infants. Pulmonary arteries
and valves may release histological adaptation against
systemic pressure, thereby leading to less autograft
dilatation and dysfunction in this population.[5] In
addition, improved neoaortic valve functions in the
long-term following arterial switch operation support
this assumption.
Nonetheless, the most important disadvantage of
the Ross procedure is the risk of re-do surgery due to
the possible RV-PA conduit-related complications. The
Contegra valved bovine jugular vein graft (Contegra;
Medtronic, Inc., Minneapolis, Minn) have a very low
early re-do surgery rate for RVOT reconstruction and
could be therefore used in neonates and children under
the age of three years, unless a blood group-compatible
homograft can be found.[13,14]
There are several reports demonstrating functional
RV-PA conduit in 50-70% of the patients at
10 years.[5,7,10-12] A large-size conduit as much as
possible should be placed during the initial operation
to ensure a more durable RV-PA conduit. It is wellestablished
that conduits less than 14 mm in diameter
may result in early re-do surgery. In our study, we
implanted a 14 mm Contegra conduit (Medtronic
Inc., Minneapolis, Minn, USA), as his weight was
2.5 kg. At 42 months of surgery, conduit stenosis was observed preserving its function. We believe that we
can increase the lifespan of the conduit by pulmonary
balloon angioplasty.
In conclusion, the Ross procedure and Ross-Konno
procedure are among the first treatment of choices
in the management of aortic valve pathologies and
LVOTO in neonates and infants.
Declaration of conflicting interests
The authors declared no conflicts of interest with
respect to the authorship and/or publication of this
article.
Funding
The authors received no financial support for the
research and/or authorship of this article.
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