Aim: To analyze the cardiovascular (CV) causes of disqualification from competitive sports in young athletes (aged ≤ 35 years) consecutively screened at our Sports Medicine Unit in a 10 years time interval and to collect the long term follow-up data. Methods: During the 2001-2010 period, 32473 athletes were screened (31% females), according to Italian protocol (history, physical examination, 12-lead electrocardiography, exercise stress testing). CV conditions causing disqualification were analyzed on the basis of the reasons for proceeding with further cardiovascular examinations. Results: Sixty-three (0.2%) young athletes were disqualified from sport participation because of CV causes. They were referred for further examination because of positive history for CV diseases in 15 (24%), heart murmurs or systemic hypertension at physical examination in 5 (8%), 12-lead ECG or stress test (arrhythmias or myocardial ischemia) changes in 39 (62%); reasons remained unknown in 4 (6%). CV causes of disqualification were: bicuspid aortic valve (12), mitral valve prolapse (10), arrhythmogenic cardiomyopathy (3), hypertrophic cardiomyopathy (3), congenital coronary artery diseases (3), healed myocarditis (2), dilated cardiomyopathy (1), atherosclerotic coronary artery disease (1), atrial septal defect (1), previous surgical repair of atrial septal defect (1), coronary artery aneurysms post-Kawasaki disease (1), left ventricular diverticulum (1), systemic hypertension (2), pulmonary hypertension (1), junctional or supraventricular arrhythmias (3), 3rd-degree AV block (1), long QT syndrome (1), atrial fibrillation (1), and idiopathic ventricular arrhythmias (15). Drug or interventional therapies have seen undertaken when deemed necessary. In a mean follow up of 63±34 months (range 12-132 months), the clinical course of these athletes disqualified from competitions was unremarkable. Altogether, CV diseases at risk of sudden death in the young (hypertrophic cardiomyopathy, arrhythmogenic cardiomyopathy, congenital coronary artery anomalies) were identified in 8 athletes, which were referred for further examination because of positive 12-lead ECG or ventricular arrhythmias. In 3 athletes the diagnosis of CV disease was not confirmed by additional tests. Conclusions: Our data confirm the usefulness of pre-participation screening and the key role of 12-led ECG and stress test for the identification of CV disease at risk of sudden death during sport. Although coronary artery disease is rare at this age, a thorough assessment of risk factors even in athletes <35 years of age seems advisable.