Journal of Pediatric Cardiology and Cardiac Surgery 3(1): 50‒55 (2019)

Case Report

Two Cases of Staged Repair of Anomalous Origin of
the Right Pulmonary Artery from the Ascending Aorta
Kensaku Matsuda, MD1), Hideki Tatewaki, MD, PhD1), Yusaku Nagatomo, MD2),
Yuichiro Hirata, MD2), Hazumu Nagata, MD, PhD2), Kazuhiro Hinokiyama, MD, PhD1),
Shouichi Ohga, MD, PhD2), and Akira Shiose, MD, PhD1)
1) Department of Cardiovascular Surgery, Kyushu University Hospital, Fukuoka, Japan
2) Department of Pediatrics, Kyushu University Hospital, Fukuoka, Japan

Anomalous origin of the right pulmonary artery from the ascending aorta (AORPA) is a rare congenital cardiac malformation. Clinical manifestations usually appear in infants or, more rarely, in newborns and include
respiratory distress or congestive heart failure due to increased pulmonary resistance. Primary total correctionof AORPA reportedly can result in excellent survival with a low incidence of reintervention. We report here twocases of staged repair of AORPA. Case 1 involved a 25-day-old girl who had a complicating respiratory syncytial(RS) virus infection. RS virus infection in children with congenital heart disease is associated with high mortality
and morbidity, and cardiac surgery performed with cardiopulmonary bypass during symptomatic RS virus infection is associated with a high risk of postoperative complications, especially postoperative pulmonary hypertension. Therefore, we decided that the initial palliation should consist of right pulmonary artery banding, and total
correction was achieved 2 months later. Case 2 involved an almost 2-month-old girl who initially underwent
right pulmonary artery banding due to severe pulmonary hypertension, and total correction was achieved 13days later. Both patients were discharged in good condition without any clinical symptoms. us, right pulmonary
artery banding appears to be a good surgical option for patients with AORPA and complicated condition.

Keywords: respiratory syncytial virus infection, anomalous origin of the right pulmonary artery from theascending aorta, right pulmonary artery banding

Introduction
Anomalous origin of the right pulmonary artery fromthe ascending aorta (AORPA) is a rare congenital cardiac malformation constituting <0.1% of all congenital
heart diseases.1) The right pulmonary artery (PA) arises
from the ascending aorta in the presence of separated
aortic and pulmonary valves. Whereas excessive pulmonary arterial blood flow causes hypertension in theanomalous PA, the blood pressure in the contralateral
PA arising from the pulmonary arterial trunk is wellknown to be elevated simultaneously, sometimes above
the systemic arterial pressure. This anomaly results

in progressive pulmonary hypertension (PH) in both
lungs. Clinical manifestations usually appear in infants
or, more rarely, in newborns and include respiratory
distress or congestive heart failure. Therefore, AORPA
must be repaired as early in life as possible. Meanwhile,
cardiac surgery performed during symptomatic respiratory syncytial (RS) virus infection is associated witha high risk of postoperative complications, especially
postoperative intractable PH.2) We herein describe twopatients with AORPA who initially underwent right PA
banding (PAB) because of RS virus infection in Case 1
and severe PH in Case 2.

Received: November 13, 2018; Accepted: January 4, 2019
Corresponding author: Hideki Tatewaki, MD, PhD, Department of Cardiovascular Surgery, Kyushu University Hospital, 3-1-1
Maidashi, Higashi-ku, Fukuoka 812-8582, Japan
E-mail: tatewaki@heart.med.kyushu-u.ac.jp
Originally published in Pediatric Cardiology and Cardiac Surgery, Vol. 34(2018), No. 1, pp. 46‒51
doi: 10.24509/jpccs.190109© 2019 Japanese Society of Pediatric Cardiology and Cardiac Surgery

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Case Presentations
Case 1
A 25-day-old girl with a complicating RS virus
infection was transferred to our hospital because of
respiratory distress. Enhanced computed tomography(CT) showed that the right PA originated from theposterolateral wall of the ascending aorta (Fig. 1). Echocardiography revealed that the right ventricle (RV) was
remarkably dilated, with a flattened interventricular septum (Fig. 2A) and moderate tricuspid regurgitation (TR)with a pressure gradient of 68 mmHg, suggesting excessive PH. Echocardiography also showed a 5.7-mm-wide
atrial septal defect (ASD) with bidirectional shunting.
Due to the RS virus infection and requirement for ventilator support, right PAB was planned for initial pallia-

Fig. 1 Preoperative enhanced computed tomography with three-dimensional reconstruction inCase 1
The right branch of the pulmonary artery originatedfrom the posterolateral wall of the ascending aorta.
RPA: right pulmonary artery, Asc Ao: ascending aortation to avoid cardiopulmonary bypass.
At 36 days of age, right PAB was performed througha median sternotomy using a 2-mm-wide expanded
polytetrafluoroethylene (ePTFE) tape. The right PA was
gradually tightened to a circumference of 14.5 mm withthe band. At that time, the peak blood flow velocity
across the right PA was 4.2 m/s. The systemic arterialpressure was finally elevated to 20 mmHg under 100%
oxygen and nitric oxide (NO) inhalation. After thissurgery, we were able to start aggressive PH therapy
using tadalafil and macitentan to improve RV function.
This patient was extubated on postoperative day (POD)
24, and NO inhalation was discontinued on POD 32.
Echocardiography showed that the deformity of theintraventricular septum had improved, and the TR was
trivial with a pressure gradient of 42 mmHg (Fig. 2B).
Postoperative cardiac catheterization after right PAB
demonstrated that the left pulmonary vascular resistance (PVR) was still high at 10.7 units·m2. However,
her left lung reacted well to oxygen supplementation,and the patient was therefore considered to be a goodcandidate for total correction. After the development ofbacterial pneumonia, her cardiac function and PH deteriorated, and mechanical ventilation was required on
POD 45. After improvement of the PH and heart failureby antibiotic therapy, total correction was achieved 72
days after the initial palliation.
After a repeat sternotomy, cardiopulmonary bypass
was established using the innominate artery with a
3.5-mm ePTFE graft and bicaval cannulation. The operative schema for right PA reconstruction is depicted inFig. 3. This right PA reconstruction was performed withthe heart beating. The patient underwent extubation

Fig. 2 (A) Preoperative and (B) postoperative echocardiography in Case 1(A) The RV was remarkably dilated, with a ﬂattened interventricular septum. (B) The deformity of the interventricular septum
was improved. RV: right ventricle, LV: left ventricle© 2019 Japanese Society of Pediatric Cardiology and Cardiac Surgery

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Fig. 3 Operative schema of Case 1(A) The ascending aorta was transected to create the aortic ﬂap, just above and beneath the origin of the RPA. (B) The aorta
was reconstructed by end-to-end direct anastomosis. A posterior pulmonary arterial ﬂap was directly anastomosed to an
anterior aortic ﬂap without the use of any augmentation material anterior to the ascending aorta. (C) Newly created communication between the anomalous RPA and the MPA. LPA: left pulmonary artery, MPA: main pulmonary artery, Asc Ao:
ascending aorta, RPA: right pulmonary artery

Fig. 4 Operative schema of Case 2(A) The RPA was dissected from the ascending aorta to create the aortic ﬂap. (B) The ascending aorta was directly sutured.
A posterior pulmonary arterial ﬂap was directly anastomosed to an anterior aortic ﬂap anterior to the ascending aorta. (C)RPA augmentation was performed with an autologous pericardial patch. LPA: left pulmonary artery, MPA: main pulmonary
artery, Asc Ao: ascending aorta, RPA: right pulmonary arteryand cessation of NO inhalation on POD 8. Echocardiography showed improvement of the intraventricularseptal deformity. Enhanced CT 1 month after the total
correction showed no stenosis in the repaired right PA.
Postoperative cardiac catheterization 2 months after
the total correction demonstrated an MPA pressure of
35/15 (21) mmHg, calculated PVR of 3.43 units·m2, andpulmonary-to-systemic arterial pressure ratio of 0.34.
No significant pressure gradient was noted between the
RV and both PAs. The patient was discharged on POD
58 with administration of tadalafil and macitentan. One
year after surgery, she was asymptomatic with a good
general condition.
Case 2
A 1-month-old girl was transferred to our hospital
because of AORPA, which had been diagnosed by echocardiography in another hospital. Echocardiography
revealed that the RV was remarkably dilated, with a flattened interventricular septum and severe TR with presJ Pediatr Cardiol Card Surg Vol. 3, No. 1 (2019)

sure gradient of 97 mmHg. The right PA originated fromthe ascending aorta. Echocardiography also revealed asmall ASD with bidirectional shunting. Enhanced CT
showed that the right PA originated from the posterolateral wall of the ascending aorta. Preoperative cardiac
catheterization demonstrated a pulmonary arterialpressure of 76/27 (52) mmHg and calculated PVR of
10.83 units·m2. Because of severe PH and a poor reaction
to oxygen loading, we hesitated to perform primary total
correction. Instead, we decided that right PAB should be
performed for initial palliation.
On hospitalization day 7, right PAB was performed
through a median sternotomy using a 1.5-mm-wide tapemade of an ePTFE graft. The banding tape encircling theright PA was tightened to a circumference of 15 mm.
At that time, the peak velocity across the right PA was
3.6 m/s. The systemic arterial pressure was elevatedunder 100% oxygen and NO inhalation, and intraoperative transesophageal echocardiography showed that
the TR and RV function had improved. After right PAB,

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Discussion

Fig. 5 Postoperative angiography in Case 2
No stenosis in the reconstructed RPA or signiﬁcantpressure gradient was noted from the RV to bothbranches of the pulmonary arteries.

Table 1 Postoperative cardiac catheter data in Case 2
LPA (s/d/m)
RPA (s/d/m)

Pressure (mmHg)

RpI (units·m2)

Pp/Ps

20/7 (10)19/4 (10)

2.80

0.17

echocardiography showed only mild TR and an ASD
with dominant left-to-right shunting. The RV function
further improved with administration of sildenafil, oxygen therapy, and NO inhalation. Although the patientwas extubated on POD 2, she required reintubation
because of deterioration of the PH due to atelectasis on
POD 4. After stabilization of her respiratory condition,
total correction was achieved 13 days after right PAB.
Fig. 4 shows the operative findings in Case 2. Right PA
plasty was performed using almost the same procedure
as in Case 1 except for the use of patch augmentation
with fresh autologous pericardium for right PA reconstruction because of concern about stretching the right
PA. After right PA augmentation, the RV-to-systemicartery pressure ratio decreased to 0.55. The patient waswas extubated on POD 3, and her postoperative course
was uneventful. Postoperative cardiac catheterization 20
days after the total correction showed no stenosis in thereconstructed right PA (Fig. 5). The pulmonary arterialpressure was normalized to 20/7 (10) mmHg, and thecalculated PVR was 2.8 units·m2 (Table 1).
The patient was discharged on POD 25 with administration of sildenafil and macitentan. Five months after
surgery, she was asymptomatic and in a good generalcondition.

AORAP was first described by Fraentzel in 18683)
and classified into two subgroups according to the morphological features: a proximal and distal type. In theproximal form, the anomalous PA originates from theright posterior aspect of the proximal ascending aorta
close to the aortic valve. About 85% of AORPAs are
regarded as the proximal form according to the report by
Kutsche & Van Mierop.4) In contrast, in the distal type,the anomalous PA originates from the ascending aorta
just proximal to the innominate artery or from the baseof the artery itself. In patients with the distal type, theanomalous RPA has a narrow site that is usually distantfrom its origin.5) The most common anomaly associatedwith AORPA is a patent ductus arteriosus, followed byan aortopulmonary septal defect, interrupted aortic
arch, patent foramen ovale, ventricular septal defect,
ASD, coarctation of the aorta, and tetralogy of Fallot.
No associated anomalies are found in approximately
15% of patients.6) The natural history of AORPA ischaracterized by the onset of severe pulmonary vascularobstructive disease, and the prognosis is dismal without
an early operation.7) However, it is generally recognizedthat the early diagnosis and prompt surgical treatment
of AORPA results in excellent early and late outcomes.
Nathan et al.3) reported that the probability of freedom
from death at 20 years was 94%. Therefore, surgicaltreatment is recommended as soon as a diagnosis is confirmed during the neonatal period.
Armer et al.8) reported the first successful surgicalcorrection of AORPA with interposition of a Dacron
graft between the right PA and the main PA in 1961.
Kirkpatrick et al.9) performed the first anatomicalcorrection using only autologous tissue. In 1996, vanSon and Hanley7) described aortic and main PA flaps
for repair of AORPA, thereby reducing tension on theright PA‒main PA anastomosis and decreasing therisk of kinking or stenosis of either branch of the PA.
Furthermore, in 2002, Prifti et al.10) described a double
surgical flap technique. Other modifications of aortic
and pulmonary arterial flap techniques have also been
described.11) In our two patients, we successfully performed an aortic and pulmonary arterial flap techniqueusing only autologous tissue for the right PA reconstruction. The ascending aorta, which was removed with theflap for the right PA and reanastomosed by itself, tended© 2019 Japanese Society of Pediatric Cardiology and Cardiac Surgery

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to be shorter and move posteriorly. There is some concern about narrowing the space behind the ascending
aorta. Therefore, the right PA was reconstructed anterior
to the ascending aorta. This reconstruction procedure
was almost the same as the Lecompte maneuver for thearterial switch operation. However, in the case of transposition of the great arteries, the great arteries exhibit
an anterior‒posterior relationship; i.e., the PA is positioned anterior to the aorta. Conversely, in the normalside-by-side relationship in these cases, such techniques
may cause aortic compression against the reconstructed
right PA. The new right PA coursed over the ascending
aorta and moved down posteriorly immediately behindthe superior vena cava. Therefore, we performed theright PA augmentation with an autologous pericardial
patch in Case 2.
The indication and surgical procedure for unilateral
PAB has not been well established because unilateral
PAB has been rarely performed. In our experience oftreating newborns and young infants, we tighten theright PA to a 14- to 15-mm banding circumference witha 3.5- to 4.0-m/s peak velocity across the band on intraoperative epicardial echocardiography.
Few reports have described staged repair of AORPA.
In Case 1, right PAB was initially performed, and total
correction was delayed because cardiac surgery with cardiopulmonary bypass in patients with symptomatic RS
virus infection is associated with a high risk of postoperative PH.2) In Case 2, we hesitated to perform primary
total correction because preoperative cardiac catheterization demonstrated a PVR of >10 units·m2, and thepatient did not have a good reaction to oxygen loading.
Kido et al.12) performed staged repair of AORPA due tosevere PH and severely impaired RV function. However,
the indication for staged repair of AORPA in patientswith severely depressed RV function and PH has not
yet been established. Although primary total correction
in neonates is the treatment of choice for AORPA, it isimportant to estimate the reactiveness of PVR against
oxygen loading by cardiac catheterization in cases suchas those described herein.
Whereas excessive pulmonary arterial blood flowcauses hypertension in the anomalous PA, the bloodpressure of the contralateral PA from the pulmonary
arterial trunk is elevated, sometimes over the systemicarterial pressure, because the entire cardiac output isreceived from the RV. The advantages of right PAB for
J Pediatr Cardiol Card Surg Vol. 3, No. 1 (2019)

AORPA are 1) the avoidance of cardiopulmonary bypass
and 2) the ability to start NO inhalation, oxygen therapy,
and administration of a pulmonary vasodilator for PHof the left lung. In our patients, TR and RV contraction improved after right PAB. However, complicating
pneumonia or atelectasis may cause the deteriorationof left PH after right PAB. In fact, both of our patientsrequired reintubation because of deterioration of PH
due to bacterial pneumonia in Case 1 and atelectasis inCase 2. In summary, long-term management of AORPA
after right PAB is diﬃcult. Therefore, once stabilizationof the patientʼs general condition has been confirmed,
total correction should be performed as soon as possible.

Conclusion
We have herein reported two cases of staged repair
of AORPA. Although AORPA should be repaired as
early in life as possible, we conclude that staged repair
is a good therapeutic option for AORPA in high-riskpatients undergoing primary total correction.
Conﬂicts of Interest
The authors declare that they have no conflict of interest.

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