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Case Reports
. 2025 Jan 20;9(2):ytaf012.
doi: 10.1093/ehjcr/ytaf012. eCollection 2025 Feb.

Left coronary ostial isolation in a young boy caused by a dysplastic aortic valve: a case report

Nicholas Fitzgerald  1   2 , Matthew Liava'a  1 , Ganesh Gnanappa  1 , Julian Ayer  1   3
Affiliations
Case Reports

Left coronary ostial isolation in a young boy caused by a dysplastic aortic valve: a case report

Nicholas Fitzgerald et al. Eur Heart J Case Rep. .

Abstract

Background: Ischaemic cardiac chest pain and coronary artery abnormalities are uncommon in children. The long-term implications of missed or delayed diagnosis are myocardial ischaemia and risk of sudden cardiac death. Improvement in non-invasive imaging has made diagnosis and surgical planning possible with multi-modal imaging.

Case summary: A 12-year-old boy with ischaemic chest pain caused by isolation of the left coronary ostium in the context of a dysplastic aortic valve. There was a delay to formal diagnosis. Surgical aortic valve repair resulted in complete resolution of symptoms. Ethics approved (SCHN: CCR2023/5).

Discussion: Isolation of the left coronary ostium caused by a dysplastic aortic valve (without supravalvar stenosis) is an example of a rare cause of ischaemic chest pain in children. To our knowledge, only 10 paediatric case reports are published in English. In reported cases, presenting features varied from poor feeding and a murmur in infants to chest pain, syncope, or cardiac arrest in adolescents. Historically, angiography during a cardiac catheter procedure was required for diagnosis; however, improvements in non-invasive imaging techniques have resulted in the diagnosis being possible on echocardiography (supported by computed tomography angiography or cardiac magnetic resonance imaging).

Keywords: Case report; Chest pain; Dysplastic aortic valve; Isolation of the left coronary ostium; Myocardial ischaemia; Paediatric Surgery.

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Conflict of interest statement

Conflict of interest: None declared.

Figures

Figure 1
Figure 1
Twelve-lead ECG immediately following the treadmill exercise stress test demonstrating infero-lateral ST segment depression.
Figure 2
Figure 2
Cardiac magnetic resonance comparing cine and delayed gadolinium enhanced images in four chamber (A and B) and short axis views (C and D) demonstrating subendocardial infarct pattern in the left ventricle.
Figure 3
Figure 3
Transoesophageal echocardiogram with the aortic valve in short axis demonstrating the hypoplastic left coronary cusp (LCC), isolated coronary ostium, and obstructed coronary artery flow.
Figure 4
Figure 4
Long axis (A) and coronal (B) reconstructions from computed tomography angiogram demonstrating the fused left coronary cusp (LCC) and pathway to the left coronary artery (LCA).
Figure 5
Figure 5
Transoesophageal echocardiogram demonstrating unobstructed left coronary artery flow post repair.
None
See this image and copyright information in PMC

References

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