A supracristal ventricular septal defect (VSD), an outlet VSD situated in subaortic and subpulmonic regions, has a strong association with prolapse of the aortic valve cusp. The authors report the case of a patient operated for a supracristal VSD with prolapse of the right coronary cusp. The VSD was found to be in the subaortic position on preoperative transthoracic echocardiography, which failed to detect the presence of a double-chamber right ventricle. Intraoperative transesophageal echocardiography correctly recognized the supracristal nature of the VSD and identified the double-chamber right ventricle, subsequently altering the course of surgery.
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