Anomalous aortic origin of a coronary artery with an intraseptal course: novel techniques in haemodynamic assessment

Description

A 7-year-old boy presented to the emergency room after an episode of syncope while at home. Although, it was non-exertional, it lasted 2 min and was preceded by angina-like chest pain. Vitals, physical examination, chest radiograph and ECG were normal. Subsequent cardiology evaluation led to an echocardiogram that showed anomalous origin of the left main coronary artery from the opposite sinus of Valsalva with adjacent ostia which was confirmed on CT angiography. In addition, it showed a long 2.5–3 cm deep intraseptal course of the left anterior descending (LAD) and short intraseptal course of the left circumflex (LCX) artery. Stress nuclear perfusion scan and Holter monitoring were negative, but stress cardiac MRI showed an inducible perfusion defect along the anterior interventricular septal wall most prominent at the mid-ventricular and basilar levels. Coronary angiography (figure 1 and video 1) and intravascular ultrasound demonstrated significant systolic compression of the intraseptal segment...

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