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Autologous pulmonary artery flap repair for aortopulmonary window with interrupted aortic arch and anomalous origin of the right coronary artery from the pulmonary artery

Published online by Cambridge University Press:  03 November 2026

Masaru Kumae Open the ORCID record for Masaru Kumae [Opens in a new window] ,
Naoki Masaki Open the ORCID record for Naoki Masaki [Opens in a new window]  and
Sadahiro Sai
Masaru Kumae*
Affiliation:
Cardiovascular Surgery, Miyagi Kenritsu Kodomo Byoin , Japan
Naoki Masaki
Affiliation:
Cardiovascular Surgery, Miyagi Kenritsu Kodomo Byoin , Japan
Sadahiro Sai
Affiliation:
Cardiovascular Surgery, Miyagi Kenritsu Kodomo Byoin , Japan
*
Corresponding author: Masaru Kumae; Email: hirosaki_somu@yahoo.co.jp
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Abstract

We describe a surgical technique using a pedicled autologous pulmonary artery wall flap for repair of aortopulmonary window in association with interrupted aortic arch and anomalous origin of the right coronary artery from the pulmonary artery. A 29-day-old infant underwent definitive repair in which the pulmonary artery flap was used to close the defect and to achieve wide rerouting of the right coronary artery to the reconstructed ascending aorta. The pulmonary artery was reconstructed with a fresh autologous pericardial patch, and the aortic arch was repaired by direct anastomosis. Postoperative CT demonstrated patency of the right coronary artery and satisfactory aortic configuration. This anatomical repair using autologous tissue may reduce the risk of coronary distortion and provide a favourable coronary geometry in selected patients. However, further follow-up is required to evaluate coronary growth and the long-term durability of the reconstructed vessels.

Keywords

aortopulmonary windowanomalous origin of the right coronary artery from the pulmonary arteryinterrupted aortic archcoronary translocationCHD

Information

Type
Case Report
Information
Cardiology in the Young , First View , pp. 1 - 3
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Copyright
© The Author(s), 2026. Published by Cambridge University Press

Introduction

Aortopulmonary window is a rare congenital cardiac lesion (approximately 0.2% of cases). Reference Konstantinov, Karamlou and Williams1 Its coexistence with an interrupted aortic arch and anomalous right coronary artery from the pulmonary artery, as in the present case, is extremely uncommon and creates unique surgical challenges. We report this unusual anatomical combination and describe an anatomy-tailored reconstruction using a pedicled autologous pulmonary artery wall flap.

Case report

A female newborn, weighing 2.4 kg, was born at 36 weeks of gestation. Postnatal echocardiography revealed aortopulmonary window type I and interrupted aortic arch type A. Three days after birth, she underwent bilateral pulmonary artery banding due to heart failure caused by pulmonary overcirculation. Post-banding CT demonstrated an anomalous origin of the right coronary artery arising from the pulmonary artery wall adjacent to the aortopulmonary window, with the orifice located only a few millimetres from the edge of the aortopulmonary window, in addition to the previously identified aortopulmonary window and interrupted aortic arch (Figure 1 a ). After the initial procedure, she showed no signs of heart failure or myocardial ischaemia. At 29 days old, her body weight had increased slightly to 2.6 kg, and she underwent definitive surgical repair.

Figure 1.

Preoperative computed tomography image showing a type I aortopulmonary window between the ascending aorta and pulmonary artery, interrupted aortic arch type A, and an anomalous origin of the right coronary artery arising from the pulmonary artery adjacent to the defect. ( a ) Postoperative computed tomography image demonstrating patency of the reimplanted right coronary artery and satisfactory reconstruction of the ascending aorta and aortic arch. ( b ) Ao = aorta; PA = pulmonary artery; RCA = right coronary artery.

A diagram of the heart’s major arteries before and after surgery.

A median sternotomy was performed, and cardiopulmonary bypass was initiated via bicaval drainage and arterial return from the brachiocephalic artery and the descending aorta. The ductus arteriosus was ligated. Cardiac arrest was induced using antegrade cardioplegia with clamping of the ascending aorta and the pulmonary artery. A transverse incision was made just above the sino-tubular junction of the pulmonary artery to identify the pulmonary valve and the orifice of the right coronary artery. The right coronary artery orifice was positioned just above the annulus of the pulmonary valve. A 10 mm pedicled pulmonary artery flap was meticulously designed and resected to match the size of the aortopulmonary window defect, using the anterior wall of the pulmonary artery (Figure 2 a ). A careful incision was made between the right coronary artery orifice and the pulmonary valve annulus, and the aorta and pulmonary artery were divided (Figure 2 b ). The proximal portion of the right coronary artery was dissected and mobilised. The pulmonary artery flap was then sutured to the aortic wall using 7-0 running sutures to reconstruct the ascending aorta (Figure 2 c ). The aortic reconstruction was completed in 35 minutes of aortic cross-clamp time. The descending aorta was directly anastomosed to the lesser curvature of the aorta, opposite to the brachiocephalic artery, thereby reconstructing the aortic arch. The pulmonary artery was reconstructed using a fresh autologous pericardial patch (Figure 2 d ). The patient was weaned from cardiopulmonary bypass without difficulty.

Figure 2.

Schematic illustration of the surgical technique. ( a ) The size of the aortopulmonary window defect was approximately 10 mm × 10 mm. ( b ) The orifice of the right coronary artery was close to the pulmonary valve. We carefully made a precise incision between them, similar to creating a coronary button, and fashioned a pedicle flap from the pulmonary artery. ( c ) With meticulous attention to the coronary orifice, we sutured the flap to reconstruct the aorta. ( d ) The defect of the pulmonary artery was closed with a fresh autologous pericardial patch. The descending aorta was then anastomosed to the lesser curvature of the aortic arch, opposite the origin of the brachiocephalic artery.

Diagram of a surgical technique involving the aorta, pulmonary artery, and right coronary artery.

The postoperative course was uneventful, and the patient was extubated on postoperative day 4. The ICU stay lasted 12 days. Postoperative CT confirmed patency of the right coronary artery orifice and a favourable configuration of the reconstructed ascending aorta and aortic arch (Figure 1 b ). At 1-year follow-up, the patient remains asymptomatic, with preserved ventricular systolic function, no ischaemic electrocardiographic changes, and no clinical or echocardiographic evidence of myocardial ischaemia.

Discussion

Anomalous right coronary artery from the pulmonary artery is distinct from anomalous aortic origin of the right coronary artery, with substantially different anatomical, physiological, and surgical implications. In typical anomalous right coronary artery from the pulmonary artery, collateral-dependent coronary flow may drain into the low-pressure pulmonary circulation; however, in the present patient, this physiology was likely modified by the aortopulmonary window, which exposed the pulmonary arterial origin of the right coronary artery to systemic-level pressure before repair.

Several surgical strategies have been reported for anomalous right coronary artery from the pulmonary artery in association with aortopulmonary window, including direct implantation Reference Leobon, Bret and Roussin2 and intrapulmonary baffle rerouting of the coronary artery to the aorta through the aortopulmonary window using patch materials. Reference Ginde, Earing and Bartz3 Direct implantation is now considered one of the standard options for repairing the anomalous coronary artery, owing to the extensive surgical experience with the arterial switch operation for transposition of the great arteries. However, this technique can be challenging in some cases due to the risk of coronary artery stretching or kinking and anatomical difficulties caused by the significant distance from the aorta to the anomalous coronary artery orifice. In the present case, the anomalous coronary orifice was located immediately adjacent to the aortopulmonary window, only a few millimetres from its edge. This close proximity made it possible to incorporate the coronary orifice into the pedicled pulmonary artery flap and reroute it to the reconstructed ascending aorta without a separate coronary transfer. Direct implantation was not considered anatomically impossible; rather, the flap reconstruction was selected because it made direct use of this favourable proximity while avoiding a separate coronary reimplantation. Alternatively, baffle rerouting similar to the Takeuchi method has shown good long-term outcomes. Reference Gabbieri, Guadalupi and Stefanelli4 However, concerns remain about potential interference with the pulmonary artery or valve, baffle-related complications such as narrowing and leakage Reference Ginde, Earing and Bartz3 and the complexity of future reoperations. Unlike intrapulmonary baffle techniques, the present approach avoids an intrapulmonary conduit and its potential complications, including narrowing or leakage.

In our case, we employed a repair technique involving a pedicled autologous pulmonary arterial wall flap for the aortopulmonary window, Reference Matsuki, Yagihara and Yamamoto5 achieving wide rerouting of the right coronary artery to the reconstructed aorta. Autologous pulmonary arterial tissue has been used in other congenital cardiovascular reconstructions, including pulmonary artery autograft interposition. Reference Bilal, Yildirim, Avsar and Ozyuksel6 In the present case, the pedicled pulmonary artery wall flap was applied to the rare combination of aortopulmonary window, interrupted aortic arch, and anomalous right coronary artery from the pulmonary artery. Because the anomalous right coronary artery orifice was located only a few millimetres from the edge of the aortopulmonary window, the flap could be designed to close the defect while incorporating the coronary orifice and creating a wide, short route to the reconstructed ascending aorta. Care must be taken to avoid occlusion or kinking of the coronary artery. This technique may therefore provide a favourable configuration for coronary rerouting and aortic reconstruction with a short suture line.

Additionally, using autologous tissues, which may accommodate somatic growth, may help reduce long-term complications such as distortion of reconstructed tissues. However, this presumed growth potential remains theoretical and should be regarded as speculative until longer-term imaging and clinical follow-up become available. Potential late concerns include distortion or stenosis of the reconstructed tissues, bronchial compression, and complications related to the aortic arch-descending aorta anastomosis, any of which may require reintervention during follow-up. This approach may represent a useful surgical option in selected patients with this rare combination of anomalies. However, given that this report is based on a single case with limited follow-up, further studies are required to confirm its reproducibility and long-term outcomes.

Data availability statement

The data underlying this article are included in the article.

Financial support

This work received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Competing interests

The authors declare no conflict of interest.

Ethical standard

Written informed consent for publication was obtained from the patient’s guardian. The study was approved by the Institutional Review Board of Miyagi Children’s Hospital (approval no. MCH-644) and was conducted in accordance with the Declaration of Helsinki.

References

Konstantinov, IE, Karamlou, T, Williams, WG, et al. Surgical management of aortopulmonary window associated with interrupted aortic arch: a Congenital Heart Surgeons Society study. J Thorac Cardiovasc Surg 2006; 131(5): 1136–1141.e2. DOI: 10.1016/j.jtcvs.2005.03.051.10.1016/j.jtcvs.2005.03.051CrossRefGoogle ScholarPubMed
Leobon, B, Bret, EL, Roussin, R, et al. Technical options for the treatment of anomalous origins of right or left coronary arteries associated with aortopulmonary windows. J Thorac Cardiovasc Surg 2009; 138(3): 777–778. DOI: 10.1016/j.jtcvs.2008.07.013.10.1016/j.jtcvs.2008.07.013CrossRefGoogle ScholarPubMed
Ginde, S, Earing, MG, Bartz, PJ, et al. Late complications after Takeuchi repair of anomalous left coronary artery from the pulmonary artery: case series and review of literature. Pediatr Cardiol 2012; 33(7): 1115–1123. DOI: 10.1007/s00246-012-0260-5.10.1007/s00246-012-0260-5CrossRefGoogle ScholarPubMed
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Matsuki, O, Yagihara, T, Yamamoto, F, et al. New surgical technique for total-defect aortopulmonary window. Ann Thorac Surg 1992; 54(5): 991–992. DOI: 10.1016/0003-4975(92)90672-q.10.1016/0003-4975(92)90672-QCrossRefGoogle ScholarPubMed
Bilal, MS, Yildirim, O, Avsar, M, Ozyuksel, A. Repair of unilateral absence of right pulmonary artery with contralateral pulmonary artery autograft interposition in an infant. Ann Thorac Surg 2015; 99(4): 1467–1469. DOI: 10.1016/j.athoracsur.2014.12.036.10.1016/j.athoracsur.2014.12.036CrossRefGoogle ScholarPubMed
Figure 0

Figure 1. Preoperative computed tomography image showing a type I aortopulmonary window between the ascending aorta and pulmonary artery, interrupted aortic arch type A, and an anomalous origin of the right coronary artery arising from the pulmonary artery adjacent to the defect. (a) Postoperative computed tomography image demonstrating patency of the reimplanted right coronary artery and satisfactory reconstruction of the ascending aorta and aortic arch. (b) Ao = aorta; PA = pulmonary artery; RCA = right coronary artery.

Figure 1

Figure 2. Schematic illustration of the surgical technique. (a) The size of the aortopulmonary window defect was approximately 10 mm × 10 mm. (b) The orifice of the right coronary artery was close to the pulmonary valve. We carefully made a precise incision between them, similar to creating a coronary button, and fashioned a pedicle flap from the pulmonary artery. (c) With meticulous attention to the coronary orifice, we sutured the flap to reconstruct the aorta. (d) The defect of the pulmonary artery was closed with a fresh autologous pericardial patch. The descending aorta was then anastomosed to the lesser curvature of the aortic arch, opposite the origin of the brachiocephalic artery.