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Late presentation of distal aortopulmonary window with anomalous origin of right coronary artery from main pulmonary artery with isolated left subclavian artery

Published online by Cambridge University Press:  24 February 2025

Anish GuptaOpen the ORCID record for Anish Gupta[Opens in a new window],
Abisho R. Starlet and
Yash Shrivastava
Anish Gupta*
Affiliation:
Department of cardiothoracic and vascular surgery, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India
Abisho R. Starlet
Affiliation:
Department of cardiothoracic and vascular surgery, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India
Yash Shrivastava
Affiliation:
Department of Pediatric cardiology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India
*
Corresponding Author: Anish Gupta; Email: drash_06@yahoo.co.in
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Abstract

We present the case of a 9-year-old girl with a distal aortopulmonary window (APW) with anomalous origin of right coronary artery from the main pulmonary artery and an isolated left subclavian artery arising from the main pulmonary artery. This report highlights the unusual combination of the three anomalies and late presentation of APW.

Keywords

Aortopulmonary windowanomalous origin of right coronary artery from main pulmonary arteryisolated left subclavian artery

Information

Type
Brief Report
Information
Cardiology in the Young , Volume 35 , Issue 3 , March 2025, pp. 655 - 658
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Copyright
© The Author(s), 2025. Published by Cambridge University Press

Clinical summary

A 9-year-old girl presented to cardiology outpatient with complaints of shortness of breath while playing in school (NYHA class II) since she was 5 years old, which then progressed to NYHA class III. She also gave a history of palpitations. She has been experiencing episodes of chest pain on exertion for the last one month. There was no history of cyanotic spells. She was a twin-born child, but the other twin died within 5 h of birth, the cause of which is not known. Her physical examination revealed a pulse rate of 98 beats per minute, blood pressure of 130/70 mmHg in the left arm supine position without any significant variation in other limbs, respiratory rate of 24 breaths per minute, and oxygen saturation of 91% in room air. She was afebrile and had no cyanosis or clubbing. A precordial bulge was present with grade 3/6 mid-systolic murmur, which was best heard at the apex. Routine blood investigations were within normal limits. Chest X-ray showed cardiomegaly and increased bronchovascular markings with a prominent upper mediastinal shadow (Figure 1). Electrocardiography suggests sinus rhythm with bi-atrial enlargement and left ventricular hypertrophy (Figure 2). Echocardiography shows a right aortic arch (RAA) with dilated left atrium and left ventricular chambers and severe pulmonary arterial hypertension (Figure 3). There was a large distal aortopulmonary window (APW) with an unclear origin of the right coronary artery. Cardiac CT angiography showed a large APW (27 mm) between the distal ascending aorta and main pulmonary artery, with anomalous origin of right coronary artery from the main pulmonary artery (ARCAPA) (Figure 4) and an isolated left subclavian artery arising from the left pulmonary artery (Figure 5). Catheterisation study at room air showed pulmonary artery pressure of 97/58 (76) mmHg and aortic pressure of 98/53 (72) mmHg. Baseline Pulmonary Vascular Resistance Index (PVRI) was 9 Wood units, which decreased to 5 Wood units on 100% oxygen. The patient’s family refused high-risk surgical intervention and is on medical management.

Figure 1.

Chest X-ray showing cardiomegaly and increased bronchovascular markings with a prominent upper mediastinal shadow.

Figure 2.

Electrocardiography showing sinus rhythm with bi-atrial enlargement and left ventricular hypertrophy.

Figure 3.

Echocardiography showing a right aortic arch (RAA) with dilated left atrium and left ventricular chambers and severe pulmonary arterial hypertension.

Figure 4.

Cardiac CT angiography showed a large APW (27 mm) between the distal ascending aorta and main pulmonary artery, with anomalous origin of right coronary artery from the main pulmonary artery (ARCAPA).

Figure 5.

3-dimensional reconstruction showing isolated left subclavian artery arising from the left pulmonary artery.

Discussion

The embryological basis for APW is the abnormal separation of the truncus arteriosus by the conotruncal septum. This can be associated with other cardiac anomalies like interrupted aortic arch, ventricular septal defect, or tetralogy of Fallot Reference Talwar, Agarwal and Choudhary1,Reference Alakhfash, Tagelden, Almesned and Alqwaiee2 and, as in our case, with ARCAPA and a left aberrant subclavian artery. But, to our knowledge, the combination of APW and ARCAPA with an isolated left subclavian artery has not been reported.

Embryologically, the involution of the distal left dorsal aorta can lead to a connection between the left seventh intersegmental artery and the main pulmonary artery. Reference Chaturvedi, Prasad, Ashwath, Strainic and Snyder3 An isolated subclavian artery can present with weak pulses, differential blood pressure, vertebrobasilar insufficiency, or arm claudication, which were absent in our case, probably due to severe pulmonary artery hypertension caused by the APW.

Late presentation of APW beyond infancy is uncommon, and many of these cases are inoperable due to the development of progressive pulmonary hypertension and Eisenmenger syndrome. Reference Das, Dutta and Das4

ARCAPA is a rare coronary anomaly, the exact embryological basis of which remains unknown. Unlike Anomalous Left Coronary Artery from Pulmonary Artery (ALCAPA), it can be asymptomatic and can be incidentally detected, and survival to adulthood is common. Higher pulmonary pressures caused by the APW are thought to cause antegrade flow of blood through the anomalous right coronary artery, which decreases the risk of left to right shunting and coronary steal or myocardial ischaemia. Reference Guenther, Sherazee, Wisneski, Gustafson, Wozniak and Raff5

Conclusion

APW is a rare congenital heart disease and may be associated with other anomalies. ARCAPA is the most commonly associated coronary artery anomaly, but an isolated subclavian artery presenting together with APW, ARCAPA, and RAA is unique.

Financial support

This research received no specific grant from any funding agency, commercial, or not-for-profit sectors.

Competing interests

None.

Ethical standard

Not applicable.

Declaration of patient consent

The authors certify that they have obtained the appropriate patient’s family consent. They were informed that anonymous echocardiographic and CT images would be posted in the journal.

References

Talwar, S, Agarwal, P, Choudhary, SK et al. Aortopulmonary window: morphology, diagnosis, and long-term results. J Card Surg 2017; 32: 138–144.10.1111/jocs.12936CrossRefGoogle ScholarPubMed
Alakhfash, AA, Tagelden, M, Almesned, A, Alqwaiee, A. Aortopulmonary window with anomalous right coronary artery from the pulmonary artery. Case report and literature review. Cardiol Young 2020; 30: 47–49.10.1017/S1047951119002543CrossRefGoogle ScholarPubMed
Chaturvedi, K, Prasad, D, Ashwath, R, Strainic, JP, Snyder, CS. Isolatedleft subclavian artery, complete atrioventricular block, and tricuspid atresia in a neonate. Tex Heart Inst J 2016; 43: 546–549.10.14503/THIJ-15-5692CrossRefGoogle Scholar
Das, D, Dutta, N, Das, P et al. Late presentation of aortopulmonary window: a contemporary series. Indian J Thorac Cardiovasc Surg 2022; 38: 403–407.10.1007/s12055-021-01306-xCrossRefGoogle ScholarPubMed
Guenther, TM, Sherazee, EA, Wisneski, AD, Gustafson, JD, Wozniak, CJ, Raff, GW. Anomalous origin of the right coronary artery from the pulmonary artery: a systematic review. Ann Thorac Surg 2020; 110: 1063–1071.10.1016/j.athoracsur.2020.01.082CrossRefGoogle ScholarPubMed
Figure 0

Figure 1.Chest X-ray showing cardiomegaly and increased bronchovascular markings with a prominent upper mediastinal shadow.

Figure 1

Figure 2.Electrocardiography showing sinus rhythm with bi-atrial enlargement and left ventricular hypertrophy.

Figure 2

Figure 3.Echocardiography showing a right aortic arch (RAA) with dilated left atrium and left ventricular chambers and severe pulmonary arterial hypertension.

Figure 3

Figure 4.Cardiac CT angiography showed a large APW (27 mm) between the distal ascending aorta and main pulmonary artery, with anomalous origin of right coronary artery from the main pulmonary artery (ARCAPA).

Figure 4

Figure 5.3-dimensional reconstruction showing isolated left subclavian artery arising from the left pulmonary artery.