Notoriously, the valvular disease of the right heart have always received less attention than the left heart valvular disease both by echocardiographers and by researchers, probably due to the long period of latent asymptomatic and for the intrinsic difficulties of examination. However, it is increasingly recognized that right-sided valve disease is not a benign lesion and has a significant and independent impact on morbidity and mortality. Pulmonary regurgitation (PR) is common after surgical or percutaneous relief of pulmonary stenosis and following repair of tetralogy of Fallot. This case report describes the natural history of an adult patient with grown-up congenital heart (GUCH) who became competitive athlete and who showed signs of extreme morphological and functional adaptation of the right heart resulting in the outcome of a previous run surgical valvotomy at the age of 5 years for a congenital pulmonary stenosis. These anatomic changes of the right ventricle and pulmonary circulation have requested the replacement of the pulmonary valve for the symptomatic pulmonary hypertension, with subsequent gradual return to sports activity.
Keywords: GUCH; pulmonary hypertension; pulmonary regurgitation; pulmonary valve replacement.