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\begin{document}

\title{Congenital Unicuspid Aortic Valve Repair without Cusp Patch Augmentation}



\author[1]{Jama Jahanyar}%
\author[1]{Gaby Aphram}%
\author[1]{Daniel E. Munoz}%
\author[1]{Stefano Mastrobuoni}%
\author[1]{Emiliano Navarra}%
\author[1]{Laurent de Kerchove}%
\author[1]{Gebrine Elkhoury}%
\affil[1]{Cliniques universitaires Saint-Luc}%


\vspace{-1em}



  
  \date{May 22, 2024}


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\selectlanguage{english}
\begin{abstract}
Unicuspid aortic valves are rare congenital malformations. Surgical
repair is feasible in aortic regurgitation, and in some cases of aortic
stenosis. The standard surgical approach is a bicuspidization and
symmetrization with pericardial patch augmentation of valve cusps.
Herein, we are describing our original technique for bicuspidization of
a unicuspid aortic valve without cusp patch augmentation. We also
address the surgical management of a commissural diastasis.%
\end{abstract}%



\sloppy


\textbf{Introduction}

The aortic valve (AV) is a semilunar valve and is derived from
endocardial cushions of the primitive heart tube. The right and left
cusps stem from conotruncal cushions, whereas the non-coronary cusp
originates from right-posterior intercalated cushions. Cavitation of the
cushions leads to separation of the cusps with formation of a lumen, and
this is followed by elongation and thinning of the cusps. Dysregulation
of extracellular matrix remodeling for instance, can lead to valve
malformations, and thus different valve phenotypes \textsuperscript{1}.

The normal AV-phenotype is tricuspid, but we know that AVs can range
from unicuspid (UAV) to quadricuspid valves, with even further
variability beyond this spectrum \textsuperscript{2}. Nonetheless, UAVs
are rare congenital heart valve malformations, with an estimated
prevalence of 0.02\% \textsuperscript{3}. Although there is no official
classification for UAVs, possibly due to its' rarity, we generally
distinguish between an acommissural- and unicommissural
phenotype\emph{(Figure1A,B)} .

UAVs can be repaired through biscuspidization. This is generally
achieved through patch augmentation of both cusps (e.g. butterfly
patch), with or without realignment of the commissural angle or aortic
annuloplasty \textsuperscript{4, 5}. As in other AV-phenotypes, there is
a spectrum of UAV morphologies. However, the commissure is usually
positioned posteriorly (Figure1B) and the height of the two raphae is
generally lower (5-15mm) than the level of the
commissure\textsuperscript{6}, as in bicuspid AVs \textsuperscript{6,
7}. Nonetheless, there is no one technique fits all approach for
UAV-repair, and herein we are presenting our original technique for
bicuspidization of a regurgitant UAV without the usual patch
augmentation of the cusps.

\textbf{Technique}

The surgical technique is illustrated in the Video (Video 1,
supplementary material). A transverse aortotomy is performed 1.5cm above
the origin of the right coronary artery, after the heart is arrested
with normothermic blood cardioplegia. The valve is assessed and
confirmed to be unicuspid with a true posterior commissure at the
left/non-coronary commissural position, and two raphae at the
non/right-, and right/left-position \emph{(Figure1C,D)} . The height of
both raphae is below the height of the true posterior commissure. An
additional unusual finding is a diastasis of the posterior commissure,
which had to be addressed separately \emph{(Figure2C).} Although the
aortic root is only moderately enlarged at 4.2cm, we performed our
modified reimplantation technique, since it provides the best
stabilization of the functional aortic annulus, with an excellent aortic
annuloplasty and support of the Sino-tubular
junction\textsuperscript{7}.

The aortic root is prepared and annular sutures are placed at the level
of the virtual basal ring as previously described by our
group\textsuperscript{7}. A commissurotomy is then performed at the
non/right-raphe to create a second functional commissure, and the free
margins are thinned with a blade \emph{(Figure2A,B)} . The remaining
raphe is then shaven and thinned towards the hinge point of the anterior
cusp, to allow for better mobility \emph{(Figure2C)} . Attention has to
be paid towards not accidentally perforating the cusp at its' insertion.

The commissural diastasis of the posterior commissure is then addressed.
The commissure is incised towards its' tip, in order to readjust the
width and to exclude some abnormal tissues in-between the cusps. The
commissure is then remodeled with a 4.0 Prolene suture\emph{(Figure2D)}
. This resolved the commissural diastasis and excluded the abnormal
tissues in-between the cusps and led to improved apposition of cusp free
margins.

A 30mm Valsalva-graft is then seated onto the aortic annulus and tied
down. The commissure and modified raphe are resuspended at 180\selectlanguage{ngerman}º. The
nadir of the fused cusp, at the raphe, is lowered to match the nadir of
the non-fused cusp \emph{(Video1)} . The graft is then sewn to the
proximal aortic root remnant and the aortic valve is re-assessed
thereafter. The anterior cusp is prolapsed and the neo-commissure is
lower than the posterior commissure. We usually perform central cusp
plications to treat a prolapse, but due to the good quality of cusp
tissues in proximity to the neo-commissure, and the bulk of tissues
centrally, we opted to perform a lateral cusp plication instead. This
lateral plication at the neo-commissure can also help to somewhat
elevate the commissure, if at the same time a small bite of the aortic
wall is taken laterally\emph{(Figure3A,B).} In addition to this, the
neo-commissure was further resuspended, 5-10mm higher to match the
height of the posterior commissure, utilizing a 5.0 Prolene suture with
emphasis on the anterior prolapsing cusp \emph{(Figure3C,D).} This is
feasible, due the good quality and large amount of cusp tissues, as well
as the annuloplasty, which increases cusp mobility due to a relative
increase of free margin length.

There was good coaptation, with an adequate effective- and geometric
height thereafter. Both coronary buttons were then reimplanted in the
usual fashion, and the distal aortic anastomosis was performed,
resecting the diseased aorta and wrapping the very distal ascending
aorta with a piece of prosthetic graft.

\textbf{Comments}

Unicuspid AVs are rare congenital malformations. However, the true
prevalence of this valve morphology is likely underreported. For
instance, Noly et al. reported that only 14\% of patients with UAV were
diagnosed preoperatively \textsuperscript{8}. Even intraoperatively, UAV
may remain under-recognized due to severe calcifications for instance;
or inexperience of the surgeon or echocardiographer with identification
of complex AV-morphologies.

Unicuspid AVs, without severe calcifications, can be repaired through
bicuspidization. The standard technique is a butterfly patch
repair\textsuperscript{4}, with symmetrization of the cusps and
improvement of cusp mobility, through additional modifications of the
functional aortic annulus (e.g. annuloplasty, sinus plication), and
thinning of the raphe.

In selected patients, Matsushima et al. have achieved a UAV-repair
without patch material, through cusp nadir relocation and root
remodeling. This achieved a relative elevation of the newly created
commissure, which then more closely matched the height of the true
commissure \textsuperscript{6}. The reason for this approach is to
obviate the accelerated degeneration of autologous or xenogenic
pericardial patch material, which renders this a palliative repair
approach, and thus only allows for deferment of valve replacement later
in life. Nonetheless, this is still an advantage in children and
adolescents, and we think it is also of benefit when employed in adults,
as the better survival of valve repair compared to prosthetic valve
replacement is now well documented. Moreover, this may also function as
a bridge to a Ross-procedure later in life, in children but also in
younger to middle-aged adults.

In this patient however, we performed an AV-repair without a patch,
through commissurotomy of a raphe to create a second commissure and two
symmetric cusps. We further enhanced symmetry through reimplantation of
the commissures at 180º. Cusp mobility was improved through
annuloplasty, as well as thinning of the free margins and raphe, and
repair of a commissural diastasis. The anterior commissure was elevated
through cusp plication and additional resuspension. Since no additional
patch material was used, we hope that we were able to minimize the risk
of cusp degeneration with overall improvement in valve longevity,
similar to our bicuspid AV-repairs \textsuperscript{9}.

\textbf{References}

\textbf{1.} Martin PS, Kloesel B, Norris RA, Lindsay M, Milan D, Body
SC. Embryonic Development of the Bicuspid Aortic Valve. \emph{J
Cardiovasc Dev Dis.} 2015;2:248-272.

\textbf{2.} Jahanyar J, Aphram G, de Kerchove L, Khoury GE. A Unique
Hybrid Tricuspid Aortic Valve. \emph{Ann Thorac Surg.} 2021.

\textbf{3.} Novaro GM, Mishra M, Griffin BP. Incidence and
echocardiographic features of congenital unicuspid aortic valve in an
adult population. \emph{J Heart Valve Dis.} 2003;12:674-678.

\textbf{4.} Lorenz V, Gonthier S, Jahanyar J, de Kerchove L, El Khoury
G. Original repair after Ross failure: A case report of bicuspidized
unicuspid autograft. \emph{JTCVS Tech.} 2021;8:141-143.

\textbf{5.} Aicher D, Bewarder M, Kindermann M, Abdul-Khalique H,
Schafers HJ. Aortic valve function after bicuspidization of the
unicuspid aortic valve. \emph{Ann Thorac Surg.} 2013;95:1545-1550.

\textbf{6.} Matsushima S, Hess A, Glenske M, Hoffmann K, Raddatz A,
Schafers HJ. Cusp Nadir Relocation by Root Remodeling in Unicuspid
Aortic Valve Repair. \emph{Ann Thorac Surg.} 2019;108:e409-e412.

\textbf{7.} Jahanyar J, El Khoury G, de Kerchove L. Commissural geometry
and cusp fusion insights to guide bicuspid aortic valve
repair.\emph{JTCVS Tech.} 2021;7:83-92.

\textbf{8.} Noly PE, Basmadjian L, Bouhout I, Viet Le VH, Poirier N,
El-Hamamsy I. New Insights Into Unicuspid Aortic Valve Disease in
Adults: Not Just a Subtype of Bicuspid Aortic Valves. \emph{Can J
Cardiol.} 2016;32:110-116.

\textbf{9.} de Meester C, Vanovershelde JL, Jahanyar J, et al. Long-term
durability of bicuspid aortic valve repair: a comparison of 2
annuloplasty techniques. \emph{Eur J Cardiothorac Surg.} 2021.

\textbf{Figures}

\textbf{Figure1. A, B.} 2 Unicuspid valve morphologies; A. Acommissural
Unicuspid Aortic Valve; B. Unicommissural Unicuspid Aortic
Valve.\textbf{C.} Intraoperative assessment. \textbf{D.} Echo assessment
(short axis).

\textbf{Figure 2. A.} Commissurotomy. \textbf{B.} Cusp
shaving.\textbf{C.} Raphe thinning\textbf{. D.} Remodeling Commissural
Diastasis.

\textbf{Figure 3. A.} Non-fused cusp lateral plication. \textbf{B.}Fused
cusp lateral plication. \textbf{C.} Fused cusp elevation.\textbf{D.}
Further commissural elevation.

\textbf{Supplementary material}

\textbf{Video 1.} Unicuspid aortic valve repair without patch material.\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/unicuspid-collage-1/unicuspid-collage-1}

\end{center}
\end{figure}\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/Unicuspid-collage-2/Unicuspid-collage-2}

\end{center}
\end{figure}\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/Unicuspid-collage-3---edited/Unicuspid-collage-3---edited}

\end{center}
\end{figure}

\selectlanguage{english}
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