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\begin{document}

\title{Yacoub valve-sparing aortic root replacement with a concomitant repair
of Tetralogy of Fallot: a case report}



\author[1]{Yongjun Qian}%
\author[2]{Qi An}%
\author[2]{Juan Wu}%
\affil[1]{Sichuan University}%
\affil[2]{West China Hospital of Sichuan University}%


\vspace{-1em}



  
  \date{May 17, 2024}


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\textbf{Yacoub valve-sparing aortic root replacement with a concomitant
repair of Tetralogy of Fallot: a case report}

Juan Wu\textsuperscript{1}, MB, Qi An\textsuperscript{2}, MD, Yongjun
Qian\textsuperscript{2}, MD, PhD

\textsuperscript{1}Out-patient Department, West China Hospital, Sichuan
University, 37 Guoxue Lane, Chengdu, Sichuan 610041, China

\textsuperscript{2}Department of Cardiovascular Surgery, West China
Hospital, Sichuan University, 37 Guoxue Lane, Chengdu, Sichuan 610041,
China

Corresponding Author: Dr. Yongjun Qian, MD, PhD

E-mail: qianyongjun@scu.edu.cn



\textbf{Yacoub valve-sparing aortic root replacement with a concomitant
repair of Tetralogy of Fallot: a case report}

Juan Wu\textsuperscript{1}, MB, Qi An\textsuperscript{2}, MD, Yongjun
Qian\textsuperscript{2}, MD, PhD

\textsuperscript{1}Out-patient Department, West China Hospital, Sichuan
University, 37 Guoxue Lane, Chengdu, Sichuan 610041, China

\textsuperscript{2}Department of Cardiovascular Surgery, West China
Hospital, Sichuan University, 37 Guoxue Lane, Chengdu, Sichuan 610041,
China

\textbf{Abstract}

Enlargement of the aorta is a severe sequela of tetralogy of Fallot
(TOF) that can cause fatal complications including aortic regurgitation,
dissection, aneurysm or even rupture. Valve sparing aortic root
replacement (VSARR), also known as the Yacoub technique, is a surgical
maneuver that corrects regurgitation and avoids requirement for
long-term anticoagulation treatment at the same time. Several studies
have demonstrated the effectiveness of VSARR in patients with aortic
regurgitation complicated by aortic root dilation following a repair of
congenital heart defects. To date, little data exists on VSARR in TOF
patients. Herein we report a case of a previously unoperated 35-year-old
male TOF patient. He was admitted due to dyspnea, initial physical
examination demonstrated cyanosis and heart murmurs. Echocardiography
suggested secundum atrial septal defect, perimembranous ventricular
septal defect and mild-severe aortic regurgitation. Computed tomography
angiography indicated aortic root dilation and suspicious right coronary
artery aneurysm. Concurrent repair of TOF and VSARR was performed
successfully and the patient had a satisfactory recovery.

\textbf{KEYWORDS:} Aortic root replacement; Congenital heart disease;
Tetralogy of Fallot



\textbf{1 | INTRODUCTION}

Enlargement of the aorta is a severe sequela of tetralogy of Fallot
(TOF), which can lead to fatal outcomes like aortic regurgitation (AR),
dissection, aneurysm, or even rupture with fatal consequences, even in
those patients who have had a repair of TOF.{[}1{]} Valve sparing aortic
root replacement (VSARR), also known as the Yacoub technique, is a
surgical procedure that has the advantages of correcting AR, preserving
native aortic valves, avoiding prosthetic valves and subsequent
requirement for long-term anticoagulation treatment.{[}2{]} Several
studies have demonstrated the effectiveness of VSARR in patients with AR
complicated by aortic root dilation following a repair of congenital
heart defects.{[}2, 3{]} To date, VSARR has not been performed on TOF
patients undergone no intervention. Here we described a case of a
previously unoperated TOF patient undergoing VSARR and concurrent repair
of TOF successfully.



\textbf{2| CASE PRESENTATION}

A 35-year-old male previously diagnosed to have TOF at the age of 5 but
did not undergo any intervention for an unknown reason, presented with
dyspnea after physical exercises and occasional cyanosis of the lips. He
reported no symptoms of chest pain, palpitation, amaurosis, syncope,
swelling of limbs, or squatting position. His other past medical history
included pulmonary tuberculosis that was fully treated at the age of 25,
and an abdominal angioma diagnosed approximately 4 weeks prior to his
hospitalization in our institution.

The physical examination revealed evidence of cardiac arrhythmia,
tremors, grade 3/6 systolic ejection murmur at the second intercostal
space and the left margin of the sternum, grade 4/6 systolic blowing
murmur at the fourth intercostal space and the left margin of the
sternum. Echocardiography was performed which showed unoperated TOF. A
dilated right atrium measuring 52 mm in diameter was evident, while the
right ventricle appeared hypertrophic with a thickness of 14 mm. The
infundibulum of the right ventricular outflow tract appeared severely
narrowed with a diameter of 8 mm. The pulmonary artery measured 18 mm in
diameter and the pulmonary artery valves were slightly restrictive. The
left ventricle appeared normal in size and the ejection fraction was
73\%. The aorta seemed to have moved forward and 50\% of its area
covered the interventricular septum. Furthermore, there was dilation of
the aortic root (55.4 mm) \textbf{(Fig.1)} and a mild central AR (VC =
5.7 mm) \textbf{(Fig.2)} . The right coronary artery showed evidence of
an anomaly with a diameter of 7.5mm. Moreover, there was a secundum
atrial septal defect showing bidirectional low-speed shunt and a 15 mm
perimembranous ventricular septal defect (VSD). Given the above
findings, surgery was recommended to correct the above abnormalities.

In surgery, a median sternotomy was performed and a total
cardiopulmonary bypass was established between the distal ascending
aorta and the vena cavae. Myocardial protection strategies including
systemic hypothermia at 32, antegrade cold cardioplegia, and consistent
retrograde cardioplegia through coronary sinus were applied. The right
ventricular outflow tract was incised longitudinally and the excessive
infundibular muscle was resected. The VSD extending towards the
infundibular and perimembranous portion of the right ventricle was
clearly visualized. An autologous pericardium was harvested for the
closure of the VSD and secured with continuous sutures. The aortic root
was then transected to explore the aortic annulus and aortic valves.
There was no thickening or calcification found, and all the leaflets
appeared equally. Following that, both the coronary arteries were freed,
and all the aortic sinuses with the entire ascending aorta were
resected. A 3 mm high aortic valvuloplasty ring was made out of a 26 mm
artificial vessel. Then, the valvuloplasty ring, aortic root, and the
proximal end of the artificial vessel were assembled by continuous
suturing to reconstruct the Valsalva sinus. An end-to-end anastomosis
was performed between the ascending aorta and the distal end of the
artificial vessel with running sutures. Openings of 8 mm in size were
made on the artificial vessel and both the coronary arteries were then
anastomosed to the openings with continuous suturing using 5-0 Prolene.
An autologous pericardium was utilized as a patch to enlarge the
pulmonary artery trunk. Partial closure of the atrial septal defect was
performed using running sutures, leaving a small remaining 3 mm opening.
Upon completion of the surgical procedures, patient rewarming, lung
recruitment, discontinuation of cardiopulmonary bypass, and cardiac
resuscitation were uneventful. An intraoperative transesophageal
echocardiogram was performed, which revealed trivial central AR and an
unobstructed left ventricular outflow tract. Postoperatively, the right
ventricular systolic pressure measured 45 mmHg, the pulmonary arterial
pressure was 18 mmHg, and the peripheral blood pressure was 88/45 mmHg.

\textbf{3|DISCUSSION}

Aortic dilation is a common sequela among patients with an unrepaired
TOF. Studies have suggested that aortic dilation may be attributed to
longstanding volume overload of the ascending aorta as a result of right
ventricular outflow tract obstruction and right-to-left shunting across
the ventricular septal defect.{[}4{]}\textsuperscript{,}{[}5{]} VSARR is
a well-established procedure that corrects AR as a result of aortic
dilation, with recent studies suggesting that the modified David V
technique that preserves sinus-like geometry may improve annular
stabilization and hemostasis in the long term compared with those of
remodeling techniques (Yacoub/David II).{[}2{]} Also, VSARR has also
been successfully performed on patients with congenital heart
defects.{[}2, 3{]} Adachi and colleagues have reported a case of TOF
repair with VSARR on a patient who has previously undergone a left
original Blalock-Taussig shunt during infancy.{[}6{]} Our report
represents the first to describe a successfully performed VSARR
procedure on a previously unoperated adult TOF patient. In our patient,
the long-standing VSD fortunately has not caused a significant
impairment to the aortic cusps or annulus. With our previous experience,
extreme care was taken to avoid disrupting the balanced aortic root or
distorting the aortic annulus during VSD repair. To better preserve the
sinuses and considering the hemodynamics of this patient, we chose the
Yacoub technique to reconstruct the aorta.

\textbf{4|CONCLUSION}

In conclusion, valve-sparing aortic root replacement using the Yacoub
technique for aortic root dilation and severe AR, with a concomitant
repair of tetralogy of Fallot in previously unoperated adult patients is
feasible with excellent surgical outcomes. Studies examining long-term
outcomes are warranted to validate the effectiveness of the VSARR
technique further while exploring other innovative surgical approaches
in the management of TOF patients with aortic dilation.



\textbf{AUTHOR CONTRIBUTIONS}

All authors were involved in the conception and design, critical
revision, manuscript writing, final approval, and agreed to be
accountable for all aspects of the work.

\textbf{FUNDING INFORMATION}

No funding was required in the preparation of this case report.

\textbf{DATA AVAILABILITY STATEMENT}

All data generated or analyzed during this study are included in this
published article.

\textbf{ETHICS STATEMENT}

This study is in compliance with the declaration of Helsinki.

\textbf{CONSENT}

Written informed consent was obtained from the patient for publication
of this case report in accordance with the journal's patient consent
policy.



\textbf{REFERENCES}

1. Grotenhuis HB, Dallaire F, Verpalen IM, van den Akker MJE, Mertens L,
Friedberg MK: \textbf{Aortic Root Dilatation and Aortic-Related
Complications in Children After Tetralogy of Fallot Repair} . \emph{Circ
Cardiovasc Imaging} 2018, \textbf{11} (12):e007611.

2. Fraser CD, 3rd, Liu RH, Zhou X, Patel ND, Lui C, Pierre AS, Jacobs
ML, Dietz HC, Habashi J, Hibino N \emph{et al} : \textbf{Valve-sparing
aortic root replacement in children: Outcomes from 100 consecutive
cases} . \emph{J Thorac Cardiovasc Surg} 2019, \textbf{157}
(3):1100-1109.

3. Baliulis G, Ropponen JO, Salmon TP, Kaarne MO: \textbf{Valve-sparing
aortic root replacement in adult patients previously operated for
congenital heart defects: an initial experience} . \emph{Eur J
Cardiothorac Surg} 2016, \textbf{50} (1):155-159.

4. Niwa K: \textbf{Aortic dilatation in complex congenital heart
disease} . \emph{Cardiovasc Diagn Ther} 2018, \textbf{8} (6):725-738.

5. Seki M, Kuwata S, Kurishima C, Nakagawa R, Inuzuka R, Sugimoto M,
Saiki H, Iwamoto Y, Ishido H, Masutani S \emph{et al} :
\textbf{Mechanism of aortic root dilation and cardiovascular function in
tetralogy of Fallot} . \emph{Pediatr Int} 2016, \textbf{58} (5):323-330.

6. Adachi O, Suzuki T, Yoshioka I, Takahashi G, Akiyama M, Kumagai K,
Tatebe S, Saiki Y: \textbf{Concomitant Valve-Sparing Aortic Root
Replacement With Repair of Tetralogy of Fallot} . \emph{Ann Thorac
Surg}2019, \textbf{108} (2):e99-e101.



\textbf{Figure Legends:}

Figure 1 -- Preoperative CTA showed the dilated aortic root with a
diameter of 55.4 mm in the TOF patient.

Figure 2 -- Preoperative TEE indicated existence of dilated aortic root
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