Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2025 Feb 4;77(1):20.
doi: 10.1186/s43044-025-00614-6.

Unusual presentation of anomalous origin of the right pulmonary artery from the ascending aorta: case report

Mohamed Elhudairy  1 , Naif Alkhushi  1 , Osman Al-Radi  2 , Khadijah Maghrabi  1 , Gaser Abdelmohsen  3   4
Affiliations

Unusual presentation of anomalous origin of the right pulmonary artery from the ascending aorta: case report

Mohamed Elhudairy et al. Egypt Heart J. .

Abstract

Background: Anomalous origin of the right pulmonary artery (AORPA) from the ascending aorta is a rare congenital anomaly, representing approximately 0.12% of all congenital heart defects. Early diagnosis and timely intervention are essential to prevent severe complications such as heart failure and pulmonary vascular disease.

Case presentation: We report a case of a full term neonate presented with respiratory distress and cyanosis. Echocardiography revealed an anomalous right pulmonary artery (RPA) origin from the ascending aorta, a large patent ductus arteriosus (PDA) with right-to-left shunt, and moderate tricuspid regurgitation. Despite initial management with prostaglandin E1 (PGE1) infusion, discontinuation of the drug led to clinical deterioration characterized by severe metabolic acidosis and low cardiac output syndrome. Resuming PGE1 infusion stabilized the patient's hemodynamics and improved systemic blood flow, allowing for successful surgical repair.

Conclusion: In cases of AORPA associated with aortic arch flow reversal, pulmonary hypertension, and inadequate interatrial communication, maintaining PDA patency with PGE1 infusion until surgical repair is critical for survival. The right-to-left flow across the PDA counteracts the steal from the aorta and decompresses the right ventricle, preventing right ventricular failure and maintaining systemic blood flow.

Keywords: AORPA; Anomalous pulmonary artery; Congenital heart defects; PDA; Prostaglandin E1 bolus; Pulmonary hypertension.

PubMed Disclaimer

Conflict of interest statement

Declarations. Ethics approval and consent to participate: Written informed consent was obtained directly from the patient’s parents to publish this case report, so ethical approval is not applicable. Consent for publication: Written informed consent was obtained from the patient’s parents to publish this case report. Competing interests: The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
A 2D image of parasternal long axis view showing the RPA arising from the ascending aorta (white asterisk). B Color Doppler of parasternal long axis view showing the RPA (white asterisk). C: Suprasternal long axis view showing RPA (white asterisk) and the retrograde flow in the aortic arch (yellow arrow). D The parasternal short-axis view shows the right-to-left PDA flow (yellow arrow). E An apical four-chamber view shows moderate tricuspid regurgitation (TR) with dilated RA and RV F Continuous wave Doppler at TR showing a TR gradient of 70 mmHg (indicating severe pulmonary hypertension). G Suprasternal long axis view showing the aortic arch and the proximal descending aorta with no coarctation. Ao: aorta: LA: left atrium, LPA: left pulmonary artery, LV: left ventricle, MPA: mean pulmonary artery, PDA: patent ductus arteriosus, RA: right atrium, RPA: right pulmonary artery, RV: right ventricle, TR: tricuspid regurgitation
Fig. 2
Fig. 2
A MDCT angiography with 3D reconstruction and right lateral projection showing the anomalous RPA from the ascending aorta (white arrow). B MDCT angiography with 3D reconstruction and posterior projection showing the aberrant right subclavian artery (white arrow) with no coarctation of the aorta. C MDCT angiography with 3D reconstruction and left lateral projection showing the large PDA (white arrow) connected to the descending aorta. D The parasternal short-axis view shows constriction of the PDA with right-to-left shunting after the discontinuation of prostaglandins (yellow arrow). E Preoperative chest x-ray showing plethoric right lung. F The parasternal short-axis view shows postoperative RPA stenosis (yellow arrow). G continuous wave Doppler showing postoperative RPA stenosis with diastolic runoff (yellow arrows). MDCT: multidetector computed tomography, MPA: mean pulmonary artery, PDA: patent ductus arteriosus, RPA: right pulmonary artery
See this image and copyright information in PMC

References

    1. Alkhushi N, Al-Radi OO, Ajlan A, Abdelmohsen G, Attia W (2017) Coronary-pulmonary arterial fistula in a neonate with pulmonary atresia-ventricular septal defect and single coronary artery. Echocardiography 34(10):1536–1539. 10.1111/echo.13674 - DOI - PubMed
    1. Fukae K (2009) Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta. In: Yasui H, Kado H, Masuda M (eds) Cardiovascular Surgery for Congenital Heart Disease. Springer Japan, Tokyo, pp 159–161. 10.1007/978-4-431-99470-1_26
    1. Prifti E, Bonacchi M, Murzi B et al (2004) Anomalous origin of the right pulmonary artery from the ascending aorta. J Card Surg 19(2):103–112. 10.1111/j.0886-0440.2004.04023.x - DOI - PubMed
    1. Ueno R, Yagi S, Bando M, Sata M (2019) Long-surviving anomalous origin of the right pulmonary artery from the ascending aorta complicated with pulmonary arteriovenous fistula. Intern Med 58(18):2749–2750. 10.2169/internalmedicine.2455-18 - DOI - PMC - PubMed
    1. Bu H, Gong X (2020) Image diagnosis: an anomalous origin of right pulmonary artery from the ascending aorta. J Cardiovasc Comput Tomogr 14(5):e37–e39. 10.1016/j.jcct.2018.12.053 - DOI - PubMed

LinkOut - more resources