Shone complex is a rare congenital disorder that involves 4 obstructive lesions of the left heart, as follows: parachute mitral valve, supravalvular mitral ring, subaortic stenosis, and coarctation of the aorta. Incomplete forms with 2 or 3 of these lesions in adult patients have been rarely reported in the literature, meaning that insufficient general data exist concerning the surgical strategy and clinical follow-up. Herein, we report the case of a 31-year-old woman with a diagnosis of incomplete form of Shone complex with parachute mitral valve and coarctation of the aorta who underwent successful single-stage surgical repair.
Adult ; Aortic Coarctation ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Constriction, Pathologic ; Diagnosis ; Female ; Follow-Up Studies ; Heart ; Heart Defects, Congenital ; Humans ; Mitral Valve

Shone complex is a rare congenital disorder that involves 4 obstructive lesions of the left heart, as follows: parachute mitral valve, supravalvular mitral ring, subaortic stenosis, and coarctation of the aorta. Incomplete forms with 2 or 3 of these lesions in adult patients have been rarely reported in the literature, meaning that insufficient general data exist concerning the surgical strategy and clinical follow-up. Herein, we report the case of a 31-year-old woman with a diagnosis of incomplete form of Shone complex with parachute mitral valve and coarctation of the aorta who underwent successful single-stage surgical repair.

Background: It is generally agreed that using a bilateral internal thoracic artery (BITA) composite graft improves long-term survival after coronary artery bypass grafting (CABG). Although the left internal thoracic artery (LITA)-based Y-composite graft is widely adopted, technical or anatomical difficulties necessitate complex configurations. We aimed to investigate whether BITA configuration impacts survival or patency in patients undergoing coronary revascularization. Methods: Between January 2006 and June 2017, 1,161 patients underwent CABG at Seoul National University Bundang Hospital, where the standard technique is a LITA-based Y-composite graft with the right internal thoracic artery (RITA) sequentially anastomosed to non-left anterior descending (LAD) targets. Total of 160 patients underwent CABG using BITA with modifications. Their medical records and imaging data were reviewed retrospectively to investigate technical details, clinical outcomes, and graft patency. Results: Modifications of the typical Y-graft (group 1, n=90), LITA-based I-graft (group 2, n=39), and RITA-based composite graft (group 3, n=31) were used due to insufficient RITA length (47%), problems using LITA (28%), and target vessel anatomy (25%). The overall 30- day mortality rate was 1.9%. Among 116 patients who underwent computed tomography or conventional angiography at a mean interval of 29.9±33.1 months postoperatively, the graft patency rates were 98.7%, 95.3%, and 83.6% for the LAD, left circumflex artery, and right coronary artery territories, respectively. Patency rates for the inflow, secondary, and tertiary grafts were 98.2%, 90.5%, and 80.4%, respectively. The RITA-based graft (group 3) had the lowest patency rate of the various configurations (p<0.011). Conclusion LITA-based Y composite graft, showed satisfactory clinical outcomes and patency whereas modifications of RITA- based composite graft had the lowest patency and 5-year survival rates. Therefore, when using RITA-based composite graft, other options should be considered before proceeding atypical configurations.