123I-BMIPP/201Tl dual myocardial SPECT proves the efficacy of surgical treatment for an adult with Bland–White–Garland syndrome

Abstract Bland–White–Garland (BWG) syndrome is a rare congenital heart disease in which the left coronary artery originates from the pulmonary artery (PA). Surgical treatment to rebuild a dual coronary system is recommended at the time of the diagnosis. However, no effective operative procedure has been established for adult-type BWG patients because of the paucity of such cases. We herein report a case of adult-type BWG that was successfully treated by patch closure of the orifice of the left main tract from the main PA and coronary artery bypass grafting. 123I-15-(p-iodophenyl)-3-(R,S)-methylpentadecanoic acid (BMIPP) and 201thallium (Tl) dual myocardial single-photon emission computed tomography (SPECT) were performed before surgery, early after surgery, and at three months after surgery. Before surgery, dual SPECT showed myocardial perfusion defects in the anterior and septal wall, which corresponded to the cardiovascular magnetic resonance imaging findings. Early after surgery, only 201Tl images demonstrated an improvement in the defect area. At three months after surgery, both the 201Tl and 123I-BMIPP imaging findings demonstrated an improvement in the defect area, which was correlated with the recovery of the left ventricular function. These results showed the effectiveness of this surgical approach for BWG syndrome.