Background A unicuspid aortic valve is a rare congenital cardiac abnormality. Despite its uncommon finding on an initial presentation, aortic insufficiency is accompanied with unicuspid aortic valve and this might reflect the natural history of progression in the morphology of unicuspid aortic valve. Case presentation We describe a 65-year-old Japanese man who was evaluated for endocarditis and found to have a unicuspid aortic valve concomitant with moderate aortic insufficiency, which was, owing to the lack of evidence of valve membrane destruction, independent of underlying infectious endocarditis. In addition, aortic insufficiency was progressed because of nonbacterial thrombotic endocarditis on the ventricular side, in areas of high turbulence around the heart valve. Conclusions Our case is unusual given the unicuspid aortic valve concomitant with aortic insufficiency, which was presumably independent of underlying infectious endocarditis because of the location of the vegetation and the lack of evidence of valve destruction. Therefore, attention should be paid to a variety of complications in the setting of unicuspid aortic valve.

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. 123 I-15-(p-iodophenyl)-3-(R,S)-methylpentadecanoic acid (BMIPP) and 201 thallium (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 201 Tl images demonstrated an improvement in the defect area. At three months after surgery, both the 201 Tl and 123 I-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.

No abstract