64-Slice multidetector computed tomography visualization of myocardial calcium in adult-type Bland-White-Garland syndrome.

A 50-year-old woman with adult-type Bland-White-Garland (BWG) syndrome, antero-lateral left ventricular (LV) exercise ischemia, syncope, and sustained monomorphic ventricular tachycardia (VT) induction at electrophysiological testing was referred for cardiac 64-slice multi-detector computed tomography (MDCT) to further assess the LV myocardium after negative cardiac magnetic resonance (CMR) studies for perfusion anomalies (C and D). The MDCT confirmed the angiographic anomalous origin of the "wrinkled" left coronary artery (LCA) arising from the pulmonary artery, retrogradely perfused through abundant and enlarged intercoronary anastomoses (E and F, white arrows) from a markedly tortuous and dilated right coronary artery (RCA), and showed LV enlargement with an ejection fraction of 0.40 and thinned, hypokinetic, and focally calcified antero-lateral walls (A and B, black arrows). The treatment strategy consisted of LCA ostium ligation with aorto-coronary bypass grafting, and cardioverter-defibrillator implantation, which delivered 2 appropriate shocks at 27-month follow-up. The patient survived past childhood owing to the development of extensive collateral coronary circulation, which preserved the ischemia extension in the LCA territory, but provided an irreversible anatomical substrate for the genesis of life-threatening ventricular arrhythmias, such as areas of fibrosis and calcification, that did not benefit from surgical dual anterograde coronary artery flow restoration. The MDCT provided further insight into formulating the rationale for individual treatment needs.