Espiard S, Vantyghem MC, Assie G, et al. - Experts conducted a multicenter national prospective study involving individuals with Carney complex (CNC), primary pigmented nodular adrenal disease or relatives carrying a pathogenic PRKAR1A mutation followed during 3 years with annual standardized investigation after a full initial work-up. In this cohort, 70 patients were enrolled (50 females, mean age: 35.4 years +/- 16.7, 81% of patients carrying PRKAR1A mutation). In 6 patients, the newly diagnosed manifestations of the disease were subclinical acromegaly, bilateral testicular calcifications in 1 patient and cardiac myxomas in 2 patients at the end of the 3-year follow-up. They found recurrences of cardiac myxoma in 4 patients during the 3-year follow-up of this study. Results highlighted the significance of a systematic follow-up of the CNC manifestations, particularly a biannual screening for cardiac myxoma. Leading to a lighter and more acceptable follow-up for patients, regular screening for the other manifestations after a first extensive work-up could be spaced by contrast. In addition, these are significant outcomes for recommendations for long-term management of CNC individuals.