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\begin{document}

\title{Absent Pulmonary Valve with L-Transposition of Great Arteries: A Case
report}



\author[1]{Wataru Sakai}%
\author[2]{Hidetsugu Asai}%
\author[1]{Takafumi Oyasu}%
\author[2]{Yosuke Arai}%
\author[2]{Noriyoshi Ebuoka}%
\author[2]{Jun Oba}%
\affil[1]{Hokkaido Medical Center for Child Health and Rehabilitation}%
\affil[2]{Hokkaido Daigaku}%


\vspace{-1em}



  
  \date{May 19, 2024}


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\selectlanguage{english}
\begin{abstract}
Absent pulmonary valve syndrome and L-transposition of the great
arteries are rare congenital anomalies. To our knowledge, patients with
both have not been previously reported. We present a case of surgical
success in the patient with absent pulmonary valve, L-transposition of
the great arteries, dextrocardia, double outlet left ventricle,
hypoplastic anatomical right ventricle, valvular aortic stenosis and
tracheomalacia. We performed anatomical left ventricle to pulmonary
artery shunt using an 8-mm expanded polytetrafluoroethylene
bicuspid-valved conduit, pulmonary artery plication, and tracheostomy.
The patient was discharged in a stable condition while waiting for the
Glenn procedure until getting her parent's permission.%
\end{abstract}%



\sloppy


\textbf{Introduction}

Absent pulmonary valve syndrome (APVS) is a rare congenital cardiac
malformation that has absent, dysplastic, or rudimentary pulmonary valve
leaflets. The characteristic feature of APVS is dilation of the
pulmonary artery, which leads to compression of the tracheobronchial
tree and respiratory distress. Most cases of APVS are associated with
tetralogy of Fallot (ToF). L-transposition of the great arteries (L-TGA)
is also a rare congenital anomaly that is associated with ventricular
septal defect, pulmonary stenosis, and abnormalities of the tricuspid
valve. There are no previous reports on patients with both APVS and
L-TGA.

\textbf{Case presentation}

A female infant with a birth weight of 2,231 g was delivered vaginally
at a gestational age of 37 weeks and 6 days. A fetal echocardiogram
revealed a heart abnormality with severe aortic and pulmonary stenosis.
Tracheobronchial tree and lung abnormality were not reported in fetal
diagnosis. After birth, she was tachydyspneic and cyanotic with a loud
``to-and-fro'' murmur over the left sternal border. Hepatomegaly was
observed. Chest radiography revealed a cardiothoracic ratio of 80\%.
Echocardiography showed the absence of pulmonary valve with pulmonary
insufficiency and stenosis, L-TGA, dextrocardia, double outlet left
ventricle, hypoplastic anatomical right ventricle, valvular aortic
stenosis, atrial septal defect, and patent ductus arteriosus with a
bidirectional shunt. Computed tomography (CT) showed a severe
hypertrophic anatomical left ventricle connected to the left displaced
and dilated main pulmonary artery, with dilation of both branches
(Figure 1). The compression of the left main bronchus by the dilated
pulmonary artery was diagnosed as tracheomalacia (Figure 2A). The left
lung was compressed by the enlarged heart and was suspected to be
hypoplastic. It had a less vascular shadow than the right lung.

A tracheal tube was placed immediately after birth because the
spontaneous breathing effort was severe, and left lung sounds could not
be heard on auscultation. A Ventricular arrhythmia requiring adrenalin
infusion immediately after birth indicated severe heart failure. Six
hours after delivery, the patent ductus arteriosus was ligated because
of increased pulmonary insufficiency and exacerbation of heart failure.
On postnatal day 5, percutaneous balloon aortic valvuloplasty was
performed for critical aortic valve stenosis. This reduced the peak
pressure gradient from 25 mmHg to 20 mmHg. At 4 months age, anatomical
left ventricle to pulmonary artery shunt using an 8-mm expanded
polytetrafluoroethylene bicuspid-valved conduit with bulging sinuses
(Stretch Vascular Graft, W.L. Gore \& Associates, Inc., Flagstaff, AZ,
USA) and pulmonary artery plication were performed. Although pulmonary
insufficiency could be controlled completely with the operation,
tracheomalacia of the left main bronchus remained (Figure 2B), and a
high positive ventilation setting was still required after the
operation. Tracheostomy was performed at the age of five months. There
was poor postoperative weight gain and repeated necrotizing
enterocolitis. Cartelization showed that her Qp:Qs was 1.6:1, and
aortopulmonary collaterals were observed from the right intrathoracic
artery. Embolization of the aortopulmonary collateral was performed to
decrease the pulmonary blood flow at the age of nine months. Conduit was
clipped at the age of ten months to decrease pulmonary blood flow. There
was good weight gain, and positive ventilation was not required after
the surgery, although tracheomalacia of the left main bronchus persisted
on CT. Despite recurrent respiratory tract infections, the patient was
discharged at the age of 14 months in a stable condition while waiting
for the Glenn procedure until getting her parent's permission.

\textbf{Discussion}

APVS includes rudimentary, dysplastic, or absent pulmonary valve
leaflets, dilated main pulmonary artery with or without dilation of its
branches, to-and-fro flow at the site of the absent pulmonary valve, and
systolic pressure gradient across the narrowed pulmonary
valve\textsuperscript{1}. APVS is a rare variant of ToF, which accounts
for approximately 3--6\% of patients with ToF \textsuperscript{2}.

L-TGA is also rare, with a published incidence ranging from 0.02 to 0.07
per 1000 births \textsuperscript{3}. The most common anatomic cardiac
abnormalities with L-TGA include ventricular septal defect, pulmonary
stenosis, and abnormalities of the tricuspid valve \textsuperscript{3}.

A dilated pulmonary artery with APVS can compress the tracheobronchial
tree and the esophagus, which is a significant indicator of poor
postnatal prognosis \textsuperscript{4}. One study showed that complete
repair with a valved conduit and reduction pulmonary arterioplasty can
help improve tracheomalacia \textsuperscript{5}. Although most patients
might require pulmonary arterioplasty, it is still controversial whether
a pulmonary valve or a valved/unvalved conduit should be inserted and
how the degree of size reduction of the pulmonary artery should be
determined. The standard medical therapy for tracheomalacia is
positive-pressure ventilation with or without a
tracheostomy\textsuperscript{5}. We performed anatomical left ventricle
to pulmonary artery shunt with a bicuspid conduit made of
polytetrafluoroethylene, which has excellent biocompatibility and low
antigenicity\textsuperscript{6}. Our patient required tracheostomy and
positive pressure ventilation from birth till the age of one year. The
anatomical left ventricle instead of the right ejected blood to the
pulmonary artery directly because of cTGA. This, along with aortic valve
stenosis, could lead to pulmonary over circulation and dilate the
pulmonary artery easily. Therefore, the complex of APVS, cTGA, and
aortic stenosis might cause pulmonary artery dilation and tracheomalacia
more easily than APVS with TOF.

\textbf{Conclusion}

To our knowledge, this is the first reported case of a patient with APVS
and L-TGA who underwent anatomical left ventricle to pulmonary artery
shunt, pulmonary artery plication, and tracheostomy. The patient was
discharged in a stable condition while waiting for the Glenn procedure.

\textbf{Acknowledgments:} None

\textbf{Abbreviations}

APVS: Absent Pulmonary Valve Syndrome

TOF: tetralogy of Fallot

L-TGA: L-transposition of great arteries

CT: Computed tomography

\textbf{Disclosures}

\textbf{Ethics approval and consent to participate:} The Ethics
Committee at Hokkaido Medical Center for Child Health and Rehabilitation
approved this study.

\textbf{Conflict of Interest Disclosure:} None

\textbf{Availability of data and material:} Not applicable.

\textbf{Author contribution:}

Wataru Sakai and Hidetsugu Asai: Concept, Interpretation, Drafting
article, Critical revision of article, Approval of article

Takafumi Oyasu, Yosuke Arai, Noriyoshi Ebuoka and Junichi Oba:
Interpretation, Drafting article, Approval of article

\textbf{References}

1. Grewal DS, Chamoli SC, Saxena S. Absent pulmonary valve syndrome -
Antenatal diagnosis. Med J Armed Forces India. 2014;70(2):198-200.

2. Nair AK, Haranal M, Elkhatim IM, Dillon J, Hew CC, Sivalingam S.
Surgical outcomes of absent pulmonary valve syndrome: An institutional
experience. Ann Pediatr Cardiol. 2020;13(3):212-219.

3. Sam\selectlanguage{ngerman}ánek M, Vorísková M. Congenital heart disease among 815,569
children born between 1980 and 1990 and their 15-year survival: a
prospective Bohemia survival study. Pediatr Cardiol. 1999;20(6):411-417.

4. Volpe P, Paladini D, Marasini M, Buonadonna AL, Russo MG, Caruso G,
et al. Characteristics, associations and outcome of absent pulmonary
valve syndrome in the fetus. Ultrasound Obstet Gynecol.
2004;24:623--628.

5. Brown JW, Ruzmetov M, Vijay P, Rodefeld MD, Turrentine MW. Surgical
treatment of absent pulmonary valve syndrome associated with bronchial
obstruction. Ann Thorac Surg. 2006;82(6):2221-2226.

6. Yoshida M, Wearden PD, Dur O, Pekkan K, Morell VO. Right ventricular
outflow tract reconstruction with bicuspid valved
polytetrafluoroethylene conduit. Ann Thorac Surg. 2011;91(4):1235-1238

Figure 1. Preoperative CT. The left displaced and dilated main pulmonary
artery with dilation of both branches.

aLV, anatomical left ventricle; Ao, aorta; MPA, main pulmonary artery;
RPA, right pulmonary artery; LV, left pulmonary artery.

Figure 2. The arrow (\selectlanguage{english}-) shows the compression of the tracheobronchial
tree by the dilated pulmonary artery at the same level of left bronchial
stenosis (A) Preoperative and (B) postoperative CT at the same level of
left bronchial stenosis. The arrow (\selectlanguage{english}-) shows the left main bronchus that
is compressed by the dilated pulmonary artery. The arrowhead (\selectlanguage{english}>\selectlanguage{english}[?]) shows
anatomical left ventricle to pulmonary artery shunt.

MPA, main pulmonary artery; RPA, right pulmonary artery; LPB, left
pulmonary bronchus; conduit, anatomical left ventricle to pulmonary
artery shunt

Figures: 2\selectlanguage{english}
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\includegraphics[width=0.70\columnwidth]{figures/Figure2/Figure2}

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