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\begin{document}

\title{A rare combination of cardiovascular anomaly: Supravalvular aortic
stenosis and congenital absence of right coronary artery}



\author[1]{Yunfei Ling}%
\author[1]{Song Jiayi}%
\author[1]{Tiange Li}%
\author[1]{Ke Lin}%
\affil[1]{Sichuan University West China Hospital}%


\vspace{-1em}



  
  \date{May 07, 2024}


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\selectlanguage{english}
\begin{abstract}
We reported a case of a 3-month-old infant presented with supravalvular
aortic stenosis with congenital right coronary artery deficiency.
According to cardiovascular CT results, Doty technique was adopted to
restore the aortic root geometry under cardiopulmonary bypass. An
angioplasty was performed to establish right coronary blood flow at the
same time. The patient had no abnormal cardiac symptoms after surgery.%
\end{abstract}%



\sloppy


\textbf{Title Page}

\textbf{A rare combination of cardiovascular anomaly: Supravalvular
aortic stenosis and congenital absence of right coronary artery}

Yunfei Ling\textsuperscript{1}, Jiayi Song\textsuperscript{1}, Tiange
Li\textsuperscript{1}, Ke Lin\textsuperscript{1\#}

\textsuperscript{1}Department of cardiovascular surgery, West China
Hospital, Sichuan University, Chengdu 610041, People's Republic of China

\textsuperscript{\textbf{\#}} Corresponding author: Ke Lin, MD, PhD

Phone: + 86 28 85422897

Fax: + 86 28 85422897

Email: pangzai356249@126.com



\textbf{A rare combination of cardiovascular anomaly: Supravalvular
aortic stenosis and congenital absence of right coronary artery}

Yunfei Ling\textsuperscript{1}, Jiayi Song\textsuperscript{1}, Tiange
Li\textsuperscript{1}, Ke Lin\textsuperscript{1\#}

\textsuperscript{1}Department of cardiovascular surgery, West China
Hospital, Sichuan University, Chengdu 610041, People's Republic of China

\textsuperscript{\textbf{\#}} Corresponding author: Ke Lin, MD, PhD

Phone: + 86 28 85422897

Fax: + 86 28 85422897

Email: pangzai356249@126.com

\textbf{Abstract}

We reported a case of a 3-month-old infant presented with supravalvular
aortic stenosis with congenital right coronary artery deficiency.
According to cardiovascular CT results, Doty technique was adopted to
restore the aortic root geometry under cardiopulmonary bypass. An
angioplasty was performed to establish right coronary blood flow at the
same time. The patient had no abnormal cardiac symptoms after surgery.

\textbf{Keywords:} Congennital heart disease

A 3-month-old infant was referred for further intervention due to
systolic murmurs found at birth. Echocardiography demonstrated a
supravalvular aortic stenosis (SVAS) at the sinutubular junction level,
bicuspid aortic valves (BAV) with the raphe on the left cusp, mild
aortic regurgitation and secundum atrial septal defect (SASD) (Fig. A,
B). Computed tomography (CT) and three-dimensional reconstruction
suggested the absence of right coronary artery (Fig. C, D). Left
coronary was demonstrated features of stenosis at the ostium (Fig. E).
The patient then received surgical intervention under cardiopulmonary
bypass. Exploration during surgery revealed that main branch of the
right coronary artery was present but not connected to the aorta, so an
angioplasty was performed to recover right coronary blood flow. The SASD
was repaired using autologous pericardium and left coronary artery
received patch augmentation. Doty technique was adopted to restore the
aortic root geometry. This technique of extended aortoplasty for
symmetric reconstruction of the aorta should provide more predictable
relief of aortic obstruction and improved function of the aortic
valve\textsuperscript{1}.

SVAS is characterized by the stricture of the lumen of the ascending
aorta, especially in the supravalvular aorta at the sinutubular
junction. SVAS may occur as an isolated defect or combined with other
angiostenosis, especially stenosis of pulmonary artery, and usually with
arrhythmia at the same time\textsuperscript{2}. SVAS can be classified
into two categories based on the degree of involvement of the ascending
aorta described by echocardiogram reports: discrete SVAS and diffuse
SVAS\textsuperscript{3}. Discrete type is characterized by a localized
ring-like thickening at the sinutubular junction that results in an
hourglass-shaped aortic root while diffuse SVAS is characterized by
tubular hypoplasia and thickening of the entire ascending
aorta\textsuperscript{4}.

Some successful cases reported angioplasty of coronary artery performing
SVAS repair at the same time, but these studies primarily described
cases in the left coronary artery. Congenital absence of RCA is probably
related to dysplasia or congenital occlusion of the RCA during the
development of embryo. This kind of may lead to the deficiency of blood
supply in sinoatrial and atrioventricular node, eventually causing their
dysfunction. Until now, there are few reports on congenital absence of
RCA. This may because RCA has non-specific clinical features. We adopted
angioplasty to establish right coronary blood flow for the patient.
Angioplasty can improve blood supply of the heart without increasing the
risk of major complications.

This case image also suggested that compared to echocardiography or
catheter angiography, cardiovascular CT can provide excellent
visualization of complex congenital anatomies. Anyway, we treated this
case with Doty technique and angioplasty procedures mentioned above.
Short-term prognosis of the patient revealed good.

\textbf{CONFLICT OF INTERESTS}

The authors declare that there are no conflict of interests.

\textbf{ETHICS STATEMENT}

This manuscript and all of its content meet the ethical guidelines,
including adherence to the legal requirements of the study country. The
need for patient consent was waived.

\textbf{FUNDING}

This study was funded by 1\selectlanguage{ngerman}·3·5 project for disciplines of
excellence-Clinical Research Incubation Project, West China Hospital,
Sichuan University (ZYJC21064).

\textbf{Figure 1} (A) Echocardiography of the aorta revealed the
stenosis at the sinutubular junction level with a diameter of 4.7 mm and
BAV with mild aortic regurgitation. (B) Echocardiography on the atrium
level suggested SASD and mild pulmonary arterial hypertension. (C, D and
F) CT and three-dimensional reconstruction confirmed the significant
SVAS and demonstrated the absence of right coronary artery. (E) Left
coronary origin was above the level of Valsalva sinus and demonstrated
features of stenosis at the ostium. (G and H) Exploration during surgery
revealed that main branch of the right coronary artery was present but
not connected to the aorta.

\textbf{Reference}

1. Doty DB, Polansky DB, Jenson CB: Supravalvular aortic stenosis.
Repair by extended aortoplasty. J Thorac Cardiovasc Surg 74:362-371,
1977.

2. Mirko, Baglivo, Sarah, et al: Atrial septal defects, supravalvular
aortic stenosis and syndromes predisposing to aneurysm of large vessels.
Acta bio-medica : Atenei Parmensis 90:53-57.

3. Ibarra C, Spigel Z, Msn R, et al: Surgical Techniques in Management
of Supravalvular Aortic Stenosis in Children. The Annals of Thoracic
Surgery, 2020.

4. Mitchell MB, Goldberg SP: Supravalvar aortic stenosis in infancy.
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 14:85-91, 2011.\selectlanguage{english}
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