June 2017 – Bland-White-Garland Syndrome (ALCAPA)

A 38 year old female presented to the emergency department for increased shortness of breath and dry cough. The patient had a history of congenital heart disease during childhood, reactive airway disease and seasonal allergies. A CT Coronary Angiogram was ordered, which revealed an Anomalous Left Coronary Artery arising from the Pulmonary Artery (ALCAPA), also known as Bland-White-Garland syndrome.

ALCAPA is a heart defect in the left coronary artery, which is connected to the pulmonary artery instead of the aorta (Fig. 1). This occurs early in the pregnancy when the baby’s heart is developing. If caught early, surgery can correct the problem and the patient can live a normal life. Without surgery, death can occur within weeks or months of birth. In rare instances, the patient can reach adulthood without being diagnosed, but once identified surgery is required to correct the problem.

Using sophisticated software tools, technologists in the 3DQ Lab perform post processing of the CT Coronary angiogram to create 3D volume rendered (VR) images that display the anomaly (Fig. 2). Curved planar reformats (CPR) are also reconstructed to evaluate patency of the coronary arteries (Fig. 3).