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Unexpected anatomical obstacle detected during emergency coronary intervention: type A interrupted aortic arch in an adult

Published online by Cambridge University Press:  11 November 2025

Murat KucukukurOpen the ORCID record for Murat Kucukukur[Opens in a new window]
Murat Kucukukur*
Affiliation:
Department of cardiology, Ministry of Health Izmir City Hospital , Izmir, Türkiye
*
Corresponding author: Murat Kucukukur; Email: drmuratkucuk055@gmail.com
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Abstract

Interrupted aortic arch is a rare congenital heart anomaly characterised by a complete discontinuity between the ascending and descending aorta, accounting for approximately 1–1.5% of CHDs. Its incidence in live births ranges from 3 to 20 per million. Survival into adulthood is only possible with the presence of a well-developed collateral circulation. In this case, a 49-year-old male patient presented with acute anterior myocardial infarction, and during emergency coronary angiography, discontinuity of the aortic arch was noticed. The procedure was successfully completed via radial access after femoral access failed. Subsequent thoracic computed tomography angiography confirmed the diagnosis of Type A interrupted aortic arch and revealed widespread collateral arterial circulation. No blood pressure difference was detected between the right-left arms and lower extremities, and the patient remained asymptomatic. This case demonstrates that this rare anomaly in adults can be incidentally detected during emergency procedures and that radial access is an effective alternative in overcoming anatomical obstacles.

Keywords

Emergency angiographytype A interrupted aortic archcollateral circulationcongenital anomalyradial access

Information

Type
Case Report
Information
Cardiology in the Young , Volume 35 , Issue 11 , November 2025, pp. 2347 - 2349
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Copyright
© The Author(s), 2025. Published by Cambridge University Press

Introduction

Interrupted aortic arch is an extremely rare congenital anomaly characterised by a complete anatomical discontinuity between the ascending and descending aorta. It constitutes less than 1–1.5% of CHDs. Reference Boutaleb, Tabat, Mekouar, Bennani, Drighil and Habbal1 In the neonatal period, it causes severe haemodynamic disorders and organ perfusion impairment, often resulting in rapid mortality. Survival into adulthood is only possible through the maintenance of ductus arteriosus patency or sufficient systemic circulation via well-developed collateral vessels. Reference Chen, Li and Pu2 Adult cases are usually diagnosed incidentally during investigations conducted for reasons such as hypertension, coronary artery disease, or suspected aortic coarctation. Reference Dibaba, Bekele and Gebrewahd3

Case presentation

A 49-year-old male patient presented to the emergency department with chest pain. The electrocardiogram showed ST-segment elevation in the anterior leads. During emergency coronary angiography initiated via femoral access, the catheter failed to advance into the descending aorta, and contrast imaging showed no contrast filling in the descending aorta, raising suspicion of an anatomical variant. The procedure was continued and successfully completed via the right radial artery. Coronary angiography revealed 100% occlusion in the left anterior descending artery, 90% stenosis in the circumflex artery, and a fistula between the circumflex artery and the pulmonary artery. Successful primary percutaneous coronary intervention was performed at the left anterior descending artery–first diagonal branch bifurcation. Subsequent thoracic computed tomography angiography confirmed the diagnosis of Type A interrupted aortic arch, revealing complete interruption of the aortic arch distal to the left subclavian artery. Prominent collateral arterial networks originating from the brachiocephalic and subclavian arteries were also observed. There was no significant blood pressure difference between the right and left upper extremities and the lower extremities. The patient was clinically asymptomatic. Cardiothoracic surgery was consulted, but surgery was not recommended due to stable condition, adequate systemic perfusion, and asymptomatic course. The patient was discharged with a recommendation for close follow-up. At three-month follow-up, the patient remained asymptomatic, and physical examination findings were normal (Figures 1 and 2).

Figure 1.

Caudal view showing 100% occlusion of the left anterior descending artery, 90% stenosis of the circumflex artery, and a fistula from the circumflex artery to the pulmonary artery.

Figure 2.

Image showing the absence of the aortic arch in the descending aortogram, and 3D computed tomography angiography image showing a view consistent with Type A aortic interruption (black circle).

Discussion

Type A interrupted aortic arch is typically a congenital anomaly with fatal outcomes in the neonatal period and rarely survives into adulthood. Reference Boutaleb, Tabat, Mekouar, Bennani, Drighil and Habbal1 However, in some cases, well-developed collateral circulation preserves systemic perfusion, allowing long-term asymptomatic survival. Adult diagnoses of interrupted aortic arch are extremely rare and are often incidentally discovered during imaging performed for reasons such as hypertension, differential pulse, or dyspnoea. Reference Dibaba, Bekele and Gebrewahd3,Reference Dong, Di and Cheng4 In this case, the diagnosis emerged during emergency percutaneous coronary intervention due to failure of femoral access, which is rarely reported in the literature. Reference Vadalà5 Radial access provided a significant advantage in continuing percutaneous coronary intervention in the presence of such anatomical anomalies. Reference Patel, Maldjian and Lovoulos6 While similar cases in the literature were mostly diagnosed by computed tomography or echocardiography, the diagnosis in this case being made during the procedure increases its uniqueness. In the case reported by Chen et al. (Reference Chen, Li and Pu2018), the diagnosis was made by computed tomography in a patient presenting with hypertension and treated surgically. Reference Dong, Di and Cheng4 Dong et al. (Reference Dong, Di and Cheng2021) diagnosed a patient presenting with dyspnoea using echocardiography and computed tomography and also performed surgery. Reference Vadalà5 In our case, however, the diagnosis was made directly during the intervention, the patient remained asymptomatic, and surgery was not required.

Conclusion

Interrupted aortic arch in adults is extremely rare and may remain undiagnosed for a long time. This case demonstrates that Type A interrupted aortic arch can be recognised during emergency percutaneous coronary intervention and that radial access offers a safe alternative for overcoming anatomical barriers. When guidewire advancement fails, contrast distribution appears unusual, or abnormal vascular structures are seen during coronary interventions, congenital anomalies should always be considered.

Ethical standards

Written informed consent was obtained from the patient for publication of this case report and accompanying images.

References

Boutaleb, AM, Tabat, M, Mekouar, Y, Bennani, G, Drighil, A, Habbal, R. Rare case series of adult interrupted aortic arch. J Cardiol Cases 2023; 28: 206–209.Google ScholarPubMed
Chen, GW, Li, H, Pu, H. Isolated interrupted aortic arch diagnosed using computed tomography angiography: case report and literature review. Medicine (Baltimore) 2018; 97: e10569.CrossRefGoogle ScholarPubMed
Dibaba, AD, Bekele, AK, Gebrewahd, SH. A case of interrupted aortic arch in an adult: an unsuspected cause of uncontrolled hypertension. Case Rep Radiol 2024; 2024: 1–4.CrossRefGoogle Scholar
Dong, SW, Di, DD, Cheng, GX. Isolated interrupted aortic arch in an adult: a case report. World J Clin Cases 2021; 9: 992–998.CrossRefGoogle Scholar
Vadalà, G. Incidental finding of interrupted aortic arch in an adult patient who underwent percutaneous coronary intervention; challenges of radial access. Austin Clin Cardiol 2023; 9: id1101.Google Scholar
Patel, DM, Maldjian, PD, Lovoulos, C. Interrupted aortic arch with post‐interruption aneurysm and bicuspid aortic valve in an adult: case report & literature review. Radiol Case Rep 2015; 10: 5–8.CrossRefGoogle Scholar
Figure 0

Figure 1.Caudal view showing 100% occlusion of the left anterior descending artery, 90% stenosis of the circumflex artery, and a fistula from the circumflex artery to the pulmonary artery.

Figure 1

Figure 2.Image showing the absence of the aortic arch in the descending aortogram, and 3D computed tomography angiography image showing a view consistent with Type A aortic interruption (black circle).