The Ross procedure was developed as an aortic valve substitute to avoid the need for anticoagulation and provide young patients with a long-lasting aortic valve substitute. Forty adults (28 males, 12 females; mean age 33.3 years) underwent the Ross procedure for congenital aortic stenosis (n = 6), aortic insufficiency (n = 17) and mixed disease (n = 17). Myocardial protection was with cold blood cardioplegia. Postoperative evaluation was by transthoracic echocardiography, office visit and/or telephone interview. NYHA class, aortic and pulmonary valve function and aortic root dimensions were evaluated. One patient died postoperatively from global left ventricular dysfunction. Four patients (10%) developed pulmonary homograft stenosis (peak gradient >40mmHg); six developed mild pulmonary stenosis. One patient had aortic insufficiency seven years postoperatively that required valve replacement. Eight patients developed mild dilatation (>37 mm) of the neoaortic root; five of these had aortic insufficiency. One patient required transplantation at 40 months for restrictive cardiomyopathy. In conclusion, the Ross procedure is an effective means of AVR with low perioperative morbidity and mortality. At mid-term follow up, a significant number of patients developed moderate pulmonary trunk stenosis, though echo characterization showed good valve function.