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\begin{document}

\title{Repair of anomalous origin of right pulmonary artery in Tetralogy of
Fallot with anomalous coronary artery}



\author[1]{ashish katewa}%
\author[1]{Balswaroop  Sahu}%
\author[1]{Shivani Gajpal}%
\affil[1]{Sri Sathya Sai Sanjeevani Hospital}%


\vspace{-1em}



  
  \date{May 04, 2024}


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\begin{abstract}
We present an unusual combination of lesions in an eight months old
child diagnosed with Tetralogy of Fallot (TOF), Anomalous origin of
Right Pulmonary artery (AORPA) and anomalous coronary artery (ACA)
crossing the pulmonary annulus. The association of AOPA and TOF is
extremely rare with an incidence of 0.4\%. (1) The incidence of
anomalous coronary artery in TOF is 10.3\%. (3) However a combination of
all three lesions poses challenges to surgical repair and has not been
previously reported.%
\end{abstract}%



\sloppy


\textbf{Case Report}

An eight months old male child weighing 4.5 Kg presented with complains
of breathlessness, repeated respiratory tract infections and failure to
thrive. The child was malnourished and had tachycardia and tachyopnea.
He had mild cyanosis with an oxygen saturation of 85\% on room air. An
ejection systolic murmur was heard over the pulmonary area on
auscultation. Chest radiogram revealed cardiomegaly and differential
pulmonary vascularity with features of increased pulmonary blood flow on
the right lung field. The electrocardiogram suggested bi-ventricular
hypertrophy. Echocardiogram examination revealed anomalous origin of
right pulmonary artery (AORPA) from ascending aorta and hypoplastic
pulmonary annulus. The main pulmonary artery (MPA) continued as left
pulmonary artery. The interatrial septum was intact and there was a
large mal-aligned ventricular septal defect. The obstruction in the
right ventricular outflow tract was predominantly deemed to be valvular.
A decision to disconnect right pulmonary artery (RPA) from aorta and
create pulmonary confluence, close the VSD and enlarge the pulmonary
outflow was made.

Surgical approach was median sternotomy. During intra-operative
inspection, the pulmonary annulus was found to be severely hypoplastic
and a large coronary artery was found crossing the pulmonary annulus.
(Figure 1) It was a branch from right coronary artery crossing the right
ventricular outflow tract and running parallel to the inter-ventricular
groove as an accessory left anterior descending artery (LAD). Pulmonary
arteries were extensively mobilised upto the lobar branches..
Cardiopulmonary bypass was initiated after aorto-bicaval cannulation.
RPA was snared immediately prior to initiation of CPB. The ligamentum
ductus was divided. Del Nido's Cardioplegia at 30ml/ Kg was administered
to achieve cardiac arrest. RPA was disconnected from the side of aorta.
Defect in the aorta was sutured primarily in two layers. RPA was
anastomosed to MPA directly and pulmonary confluence was created. The
ventricular septal defect (VSD) was routed to aorta using bovine
pericardial patch. MPA was opened longitudinally without cutting across
the pulmonary annulus in order to preserve the anomalous coronary
artery. A tri-leaflet valved conduit was created using bovine
pericardium (St. Jude Medical) and 0.1 mm Polytetrafluoroethylene
(Gore-Tex, WL Gore and Associates Inc, Flagstaff, Ariz). The valve
leaflets are created with 0.1mm PTFE membrane which is sutured to a
sheet of bovine pericardium. The bovine pericardial sheet is rolled into
a tube to make the valved conduit. The internal diameter of the conduit
was 12mm and it was interposed between the right ventricle and MPA.
(Figure 2) The native right ventricular outflow tract was also preserved
making a double barrel right ventricle outflow tract. The postoperative
course was uneventful. The child was weaned from mechanical ventilation
and extubated after 48 hours and was discharged on sixth postoperative
day. The child is asymptomatic at 36 months after surgery. The native
pulmonary valve has grown from 6 mm to 8 mm in diameter and the RV to PA
conduit is functioning well with mild regurgitation.

\textbf{Comment}

Anomalous origin of branch pulmonary artery from aorta (AOPA) is an
extremely rare condition accounting for only 0.1\% of all congenital
heart defects. (1) Aortopulmonary septum develops by fusion of right and
left conotruncal ridges. Severe mal-alignment of these conotruncal
ridges results in anomalous origin of the RPA from the ascending aorta.
AOPA can be isolated or it may be associated with other cardiac defects.
The most common associated lesion is patent ductus arteriosus, seen in
50\% of cases. Rarely, it is associated with TOF, ventricular septal
defect (VSD), Aortopulmonary window ( APW), Interrupted aortic arch and
atrial septal defect. Anomalous origin of right pulmonary artery (AORPA)
is far more common than anomalous origin of left pulmonary artery
(AOLPA). (2) However, in tetralogy of Fallot AOLPA is far more common
than AORPA. Clinical features are those of increased pulmonary blood
flow and congestive heart failure. The cyanosis associated with
tetralogy of Fallot may not be apparent due to increased blood flow to
the ipsilateral lung. Differential lung vascularity on chest radiogram
may be suggestive of the diagnosis. The repair should be performed
immediately after diagnosis to prevent congestive heart failure and
ipsilateral pulmonary hypertension. Our patient had an added complexity
in the form of anomalous coronary crossing the pulmonary annulus. The
incidence of anomalous coronary artery or a large conal artery crossing
the RVOT is 10.2\% based on a meta analysis of 28 studies. (3) The
combination of AOPA and ACA with TOF has not been previously reported in
the literature. ACA was circumvented by interposing a hand-sewn bovine
pericardial tube with a tri-leaflet valve constructed from PTFE
membrane. The integrity of the native pulmonary valve was maintained
allowing future growth as has been proposed in literature. (4) This was
demonstrated in our patient on echocardiogram examination. The native
pulmonary annulus has increased in diameter from 6 mm to 8 mm over a
period of 30 months. A promise of this growth potential combined with a
larger than required RV to PA conduit will delay or perhaps prevent
re-operations for the RVOT.

\textbf{Declaration of conflicting interests}

The author(s) declared no potential conflicts of interest with respect
to the research, authorship, and/os publication of this article.

\textbf{Funding}

The author(s) received no financial support for the research,
authorship, and/or publication of this article.

\textbf{Consent}

The patient's family gave permission to publish this case report.

\textbf{References}

John C, Maheshwari S. Unusual cardiac associations with tetralogy of
fallot- A descriptive study, paediatric Cardiol, 2010, vol. 31: 785-91

Pankaj Garg, Sachin talwar, Shyam Sunder Kothari, Anita Saxena, Rajnish
Juneja, Shiv Kumar Choydhary, Balram Airen. The anomalous origin of the
branch pulmonary artery from the ascending aorta. Intract Cardiovasc
Thorac Surg 2012 Jul; 15(1): 86--92.

Claire J Koppel, Monique R M Jongbloed, Philippine Kies, Mark G
Hazekamp, Bart J E Mertens, Martin J Schalij, Hubert W Vleigen. Coronary
anomalies in Tetralogy of Fallot : A Meta analysis. International
Journal of Cardiology, 2020;306:78-85

Karl G. Reyes, MD, Charles C. Cuaso, MD, and Reina Bianca M. Tan, MD.
Double-Barrel Right Ventricular Outflow: Tetralogy of Fallot Annulus
Preservation Technique. Ann Thorac Surg 2012;94:1023--5

\textbf{Legend for figures}

Figure 1 Sketch of preoperative anatomy: Ac LAD: Accessory Left
Descending artery; AORPA: Anomalous origin of right pulmonary artery;
LPA: Left pulmonary artery

Figure 2 Sketch of postoperative anatomy: Ac LAD: Accessory Left
descending artery; AORPA: Anomalous origin of right pulmonary artery;
LPA: Left pulmonary artery

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