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\begin{document}

\title{A Calcified Unicommissural Unicuspid Aortic Valve: is it the trigger to
the calcified amorphous tumor?}



\author[1]{Samet Uysal}%
\author[2]{CETIN GECMEN}%
\author[3]{Ender Cakmak}%
\author[4]{Gonca Gecmen}%
\affil[1]{Kartal Kosuyolu Training and Research Hospital}%
\affil[2]{KOSUYOLU HEART\&RESEARCH HOSPITAL}%
\affil[3]{Kartal Kosuyolu Heart Education and Research Hospital}%
\affil[4]{Dr. Lutfu Kirdar Education and ResearchHospital}%


\vspace{-1em}



  
  \date{May 22, 2024}


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\selectlanguage{english}
\begin{abstract}
Unicommissural unicuspid aortic valve (UAV) is a rare valvular
malformation. It may lead to severe aortic stenosis with or without
aortic regurgitation. UAVs have many characteristic changes such as
premature dysmorphic valvular calcification and premature valvular
dysfunction. Transesophageal echocardiography (TEE) was described as the
gold standard for the diagnosis of UAVs. We reported the case of a 58
years old man who presented with syncope and dyspnea due to severe
aortic stenosis secondary to a heavily calcified unicommissural UAV.
After the surgery pathological specimen showed severe amorphous
calcification just like a calcified amorphous tumor (CAT).%
\end{abstract}%



\sloppy


\textbf{Introduction}

Isolated aortic valve stenosis in adults, with or without aortic
regurgitation, is almost always secondary to a congenital valvular
malformation.{[}1{]} Unicuspid aortic valve (UAV) is a rare form of
these valvular malformations. The prevalence of which is 0.02\% in the
adult population.{[}2{]} Unicommissural UAVs generally present in the
4\textsuperscript{th} to 6\textsuperscript{th} decade of life.{[}5{]}
UAVs have many characteristic changes such as premature dysmorphic
valvular calcification, aortic root dilatations.{[}5{]} In valves,
dysmorphic calcification may occur aggressively and may lead to dense
calcified masses like a calcified amorphous tumor. Cardiac amorphous
tumors (CAT) are rare non-neoplastic intracardiac tumors that consist of
nodular calcium deposits in a background composed of fibrin or amorphous
fibrillary materials.{[}6{]} Size can vary from small punctate lesions
to very large masses, moreover diffuse myocardial infiltrations. In this
report, we reported a 59 years old man who was diagnosed with severe
aortic stenosis concomitant severe aortic insufficiency secondary to
unicommissural UAV. After aortic valve and ascending aorta replacement,
the surgical specimen revealed unicommissural unicuspid aortic valve
with severe calcification. Histological examination revealed nodular
calcification in the amorphous material, including dense fibrin and
blood capillaries, confirming the diagnosis of CAT.

\textbf{Case Report}

A 58-year-old man was admitted to the emergency department because of
syncope and progressive dyspnea for several months. A grade 5/6 systolic
murmur and a grade 4/6 diastolic murmur were auscultated.
Electrocardiography showed normal sinus rhythm. Brain computed
tomography and diffusion magnetic resonance imaging was revealed normal
for cerebrovascular pathologies. Computed tomography (CT) showed heavily
calcified CAT like mass (Figure 1.A) on the aortic valve and aneurysm of
the ascending aorta (5.2 cm in diameter). Transthoracic echocardiography
(TTE) revealed concentric left ventricular hypertrophy with normal
ejection fraction. The aortic valve was thickened and heavily calcified,
especially in the middle portion. There was a fissure like opening with
CAT like calcification on the aortic valve suggesting unicommissural
UAV. The maximal velocity through the aortic valve was 4.3 m/sec, the
mean pressure gradient between the aorta and the aortic valve was 43 mm
Hg and the calculated aortic valve area was 0.7 cm\textsuperscript{2}.
There was concomitant severe aortic regurgitation with ascending aorta
dilatation. Transesophageal echocardiography (TEE) confirmed
unicommissural unicuspid aortic valve (Figure 1.B-C) and CAT like heavy
calcification in the middle portion with substantial ascending aorta
dilatation (Figure 2.A-B). Due to symptomatic severe aortic stenosis and
aortic regurgitation with concomitant ascending aorta dilatation, the
patient was consulted with the cardiovascular surgeons. The aortic valve
and ascending aorta replacement were successfully performed.
Postoperatively, the patient was transferred to the cardiac intensive
care unit then discharged with warfarin in a week. Histopathologic
examination showed calcified nodules on an amorphous background of
fibrin material on the aortic valve just like a calcified amorphous
tumor (Figure 3.A). And degeneration with cystic changes in the
ascending aortic media which were described as cystic medial
degeneration (Figure 3.B).

\textbf{Discussion}

Unicommissural unicuspid aortic valve (UAV) is a rare valvular
malformation which prevalence is \%0.02 in the adult population.{[}2{]}
It may clinically present with aortic stenosis, aortic regurgitation, or
aortic root dilations.{[}5{]} Premature dysmorphic valvular
calcification on the valves is one of the characteristic changes in
UAVs. UAV patients generally show symptoms earlier in their lives,
usually in 30-50 years.{[}5{]} Premature dysmorphic calcification and
valvular dysfunction develop earlier and progress faster.{[}5{]} Our
patient was diagnosed with unicommissural UAV, severe aortic stenosis
concomitant severe aortic regurgitation by TEE which was described as
the gold standard for diagnosis UAVs.{[}7{]} Ascending aortic dilatation
with mild histological changes in media has been investigated in UAV
patients in some studies. {[}2{]} Ascending aortic dilatation which was
measured 5.2 cm in diameter. Heavily calcified CAT-like mass was
revealed on the valve both CT and TTE. CAT is a rare non-neoplastic
intracardiac tumor and mostly concomitant with pre-existing valve
disease or end-stage renal disease. They can be small punctuate lesions
to very large masses. In our patient, severe aortic stenosis with
unicomissural UAV morphology might lead to heavy calcification like CAT
especially in the middle portion of the aortic valve. Stenotic valve
area might lead to earlier valvular dysfunction and premature dysmorphic
calcification on the valve. In histopathological examination showed
densely calcified nodules on an amorphous background of fibrin deposits
that were also similar findings in CATs. Also degeneration with cystic
changes in the ascending aortic media which were described as cystic
medial degeneration.

\textbf{Conclusion}

Unicommissural UAV is a rare congenital disorder that leads to severe
aortic stenosis with or without aortic regurgitation. It can be
accompanied by aortic dilatation. Valvular dysfunction and dysmorphic
calcification develop earlier and calcification on the valve can mimic
cardiac amorphous tumors. In young patients with heart failure symptoms
and systolic murmurs, it must be considered in the differential
diagnosis. Deciding the appropriate time for surgery, it is important to
diagnose and follow up earlier these patients.

\textbf{Ethics Statement}

The patient has provided written, informed consent to have the details
of his case published. Institutional approval was not required for
publication.

\textbf{Conflict of interests}

The authors report no conflicts of interest in this work.

\textbf{Author Contributions}

SU, CG, and EC contributed to the drafting of this manuscript. GG
contributed to pathologically diagnosis and discussion

\textbf{Funding information}

None.

\textbf{References}

{[}1{]} W. C. Roberts and J. M. Ko, ``Frequency by decades of unicuspid,
bicuspid, and tricuspid aortic valves in adults having isolated aortic
valve replacement for aortic stenosis, with or without associated aortic
regurgitation,'' \emph{Circulation} , 2005

{[}2{]} G. M. Novaro, M. Mishra, and B. P. Griffin, ``Incidence and
echocardiographic features of congenital uniscupid aortic valve in an
adult population,'' \emph{J. Heart Valve Dis.} , 2003.

{[}3{]} R. H. Anderson, ``Editorial: Understanding the structure of the
unicuspid and unicommissural aortic valve,'' \emph{Journal of Heart
Valve Disease} . 2003.

{[}4{]} F. Mookadam, V. R. Thota, A. M. G. Lopez, U. R. Emani, and A. J.
Tajik, ``Unicuspid aortic valve in children: A systematic review
spanning four decades,'' \emph{J. Heart Valve Dis.} , 2010.

{[}5{]} F. Mookadam \emph{et al.} , ``Unicuspid aortic valve in adults:
A systematic review,'' \emph{Journal of Heart Valve Disease} . 2010.

{[}6{]} C. Reynolds, H. D. Tazelaar, and W. D. Edwards, ``Calcified
amorphous tumor of the heart (cardiac CAT),'' \emph{Hum. Pathol.} , 1997

{[}7{]} J. W. Chu, M. H. Picard, A. K. Agnihotri, and M. G. Fitzsimons,
``Diagnosis of congenital unicuspid aortic valve in adult population:
The value and limitation of transesophageal
echocardiography,''\emph{Echocardiography} , 2010

\textbf{Supplementary Material:}

Movie Clip 1. Unicuspid unicommissural aortic valve motion in systole
and diastole at TEE. LA: Left atrium, RA: Right atrium, RVOT: Right
ventricular outflow tract.

Movie Clip 2. 3D unicuspid unicommissural aortic valve motion in systole
and diastole at TEE. LA: Left atrium, RVOT: Right ventricular outflow
tract.

\textbf{Figure Legends:}

Figure 1. A- Heavily calcified mass (1.14x1.63cm) on the aortic valve
(yellow arrow) and ascending aorta dilatation (yellow star) at CT. B-
Opened unicuspid unicommissural aortic valve (yellow arrow) in systole
at TEE. C- Closed unicuspid unicommissural aortic valve (yellow arrow)
in diastole at TEE. Ao: Aorta, Pa: Pulmonary artery, LA: Left atrium,
RA: Right atrium, LV: Left ventricle. RVOT: Right ventricular outflow
tract.

Figure 2. A- 3D opened unicuspid unicommissural aortic valve in systole
at TEE and heavily calcified mass on the valve (yellow arrow) B- 3D
closed unicuspid unicommissural aortic valve (yellow arrow) in diastole.
LA: Left atrium, Pa: Pulmonary artery, RVOT: Right ventricular outflow
tract.

Figure 3. A- The histopathologic examination showed calcified nodules
(yellow arrows) on an amorphous background of fibrin material. B- The
slide showed degeneration with cystic changes (yellow stars); loss of
elastic and muscle fibers in the ascending aortic media. These
histopathologic features were described as cystic medial degeneration.\selectlanguage{english}
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