Coronary steal from an anomalous circumflex artery diagnosed 25 years after ventricular septal defect closure: a case...

Association of anomalous origin of a coronary artery arising from pulmonary artery (PA) with other congenital heart defects, such as ventricular septal defects (VSDs), is uncommon. In such cases, coronary anomalies may be overlooked and underdiagnosed.

We report a case of late diagnosis of a circumflex coronary artery arising from the right PA, 25 years after VSD-closure during infancy with a complicated post-operative course with severe left ventricular dysfunction. Eventually being discharged, she remained asymptomatic until reaching adulthood, when she developed atypical chest pain during moderate exertion initially with no further investigation being performed. The patient further developed palpitations and underwent a CT-scan, showing an anomalous circumflex artery arising from the right PA. Surgical circumflex artery re-implantation was performed and the symptoms have resolved.

This case report highlights the unique pathophysiology of coronary artery steal when arising from the PA. Severe ventricular dysfunction, frequently occurring post-operatively after VSD-closure is usually related to increased afterload. However, other causes should not be neglected especially if function does not improve. Long-term follow-up is therefore mandatory for patients diagnosed with congenital heart disease, even after supposedly simple defects.