Surgical clipping of an aortopulmonary window: a valid strategy?—case report

Introduction

An aortopulmonary window (APW) is a congenital malformation in which an abnormal connection exists between the ascending aorta and the pulmonary artery. The incidence is rare with 0.1% of all congenital cardiac anomalies (1), and associations with other congenital conditions frequently occur (2,3). There are three types of APW, depending on the localization of the connection (4). An APW creates a shunt between the systemic and pulmonary circulation, with subsequent lung overflow and if untreated, evolution towards pulmonary arterial hypertension (PAH). Commonly, the diagnosis is made with echocardiography, but rarely before the child is born (5).

Therapy consists of occlusion of this connection. Historically, this was achieved with conventional cardiac surgery, in which the connection was detached and the defects filled with a patch (6). The need for cardiopulmonary bypass and cardioplegic arrest is critical. Because of the latter, newer techniques are developed to further decrease complications. Nowadays, the use of cardiopulmonary bypass for neonatal congenital heart surgery is well established, but patients remain at risk for bleeding due to heparinization, acute kidney injury and neurological complications (6-8). In this report, we describe our experience with surgical clipping of the APW, thereby effectively occluding the shunt. This technique is infrequent, rarely reported, and excludes the need for heparinization and cardiopulmonary bypass in neonates. Additionally, the operation time is much shorter. We present this case in accordance with the CARE reporting checklist (available at https://jovs.amegroups.com/article/view/10.21037/jovs-24-23/rc).

Case presentation

All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). Written informed consent was obtained from the patient’s parents for the publication of this case report, accompanying images and videos. A copy of the written consent is available for review by the editorial office of this journal.

An eight-day-old boy was transferred to our institution because of breathing difficulties, needing non-invasive ventilation on day 10 after birth. He was born after an uneventful pregnancy (gestational age 38+5 weeks) with a birth weight of 3.45 kg and saturations of 99%. The Apgar scores were 9,10 and 10 after 1, 5 and 10 minutes. There was no known disease or genetic problem. The family history was negative for cardiorespiratory disease. On clinical examination, a high frequency systolic murmur was heard, and the patient was tachypneic. There was sinus rhythm with a frequency of 150 per minute, respiratory rate was 50 breaths per minute. Saturation was 96%. Echocardiography revealed a small atrial septal defect, good biventricular function, grade 1 tricuspid regurgitation and a short but large connection of 2.8 mm between the aorta and the pulmonary artery (Figure 1A,1B), with a left to right shunt. The APW type 1 was confirmed by computed tomography scanning (Figure 2). A multidisciplinary team discussion favored closure of the shunt to facilitate respiratory weaning and avoid further PAH.

Figure 1 Echocardiography shows the aortopulmonary window (white arrow) between the aorta and the PA (left). (A) Plain 2D view. (B) Color doppler image. PA, pulmonary artery.

Figure 2 Computed tomography revealing the aortopulmonary window (white arrow).

Through a median sternotomy, the pericardium was opened. After careful dissection of the aorta and pulmonary artery, the APW was clearly visualized (Video 1). Occlusion was performed by placing 2 titanium clips of 5.6 mm on the vessel, resulting in complete obstruction of the shunt. This was done without extracorporeal circulation or the administration of heparin (Video 2). There were no issues with perioperative bleeding or infection. The architecture of the root of both aorta and pulmonary artery was preserved, with optimal function. The procedure took 90 minutes, and a 6 mm titanium vascular clip was used. The patient was extubated one day later and could be dismissed from the intensive care unit on the fourth postoperative day. He was transferred to the referring hospital and discharged home on the seventh postoperative day. The immediate postoperative course was uneventful, but the child developed post pericardiotomy syndrome for which he received anti-inflammatory therapy with corticosteroids and aspirin. On echocardiography six months later, biventricular function was normal, with a preserved function of the aortic and pulmonary root and valve. No residual connection could be seen.

Discussion

An APW creates a large left to right shunt causing pulmonary overflow. The lesion is often discovered in infancy since these children have dyspnea and failure to thrive. If untreated, evolution towards therapy resistant PAH is very likely. Therefore, occlusion is warranted as soon as possible. The majority of reports describe a complete surgical reconstruction of both aorta and pulmonary artery, often realized by placing a patch in the defect. The use of cardiopulmonary bypass and cardioplegic arrest is warranted (9). Less invasive options are suggested as an attractive alternative, since the use of cardiopulmonary bypass can be complicated by bleeding, renal and neurological complications (6-8) In a previous report, we already discussed this clipping technique in a premature baby with bronchial bleeding (10). Avoiding systemic heparinization and cardiopulmonary bypass was beneficial for bronchial bleeding, and we observed an excellent geometric and hemodynamic result. This patient is currently doing excellent, three years later. The anatomy of both vessels is preserved, even with further growth. There is an unremarkable aortic and pulmonary valve function, and the patient is free from any sign of PAH. Together with the present report, these two cases also show favorable results towards architecture and function of the aortic and pulmonary root. However, more reports are necessary to evaluate this strategy, and the long-term follow-up is currently lacking. This patient experienced post pericardiotomy syndrome, sometimes seen after cardiac operations in children (11). We do not believe this is because of the clipping, but rather after sternotomy and opening of the pericardium. However, no data are available to support this. Reports on transcatheter closure are becoming available (12,13), highlighting the evolution in transcatheter techniques and devices in smaller patients. The availability of different techniques will probably favor patient specific treatment and improve short and long-term results.

Conclusions

Surgical clipping of an APW in neonates is a valid option to prevent further left-to-right shunting, as an alternative to complete repair needing cardiopulmonary bypass and systemic heparinization. The short and mid-term results are promising, with preservation of great vessel architecture and function. Further and definite conclusions, however, should be supported by more data, especially on long-term results of the discussed technique.

Acknowledgments

Funding: None.

Footnote

Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://jovs.amegroups.com/article/view/10.21037/jovs-24-23/rc

Peer Review File: Available at https://jovs.amegroups.com/article/view/10.21037/jovs-24-23/prf

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://jovs.amegroups.com/article/view/10.21037/jovs-24-23/coif). T.M. serves as an unpaid editorial board member of Journal of Visualized Surgery from September 2023 to August 2025. The other authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). Written informed consent was obtained from the patient’s parents for the publication of this case report, accompanying images and videos. A copy of the written consent is available for review by the editorial office of this journal.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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