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

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).