Over the last decades, progress in pediatric cardiac surgery and interventional cardiology allowed reaching adulthood to a great number of children suffering from congenital heart diseases (CHD). Apart from corrected CHD patients, grownup congenital heart diseases (GUCH) also comprise adults with newly diagnosed cardiac defect. Ebstein's anomaly, coarctation of the aorta, atrial septal defect and congenitally corrected transposition of the great arteries may remain undiagnosed for many years or occasionally identified on echocardiographic evaluation and at least manifest in adult life for the first time with infective endocarditis or effort dyspnea [1]. Therefore it is difficult to estimate the exact prevalence of CHDs in adults. The management of GUCH subjects requires particular expertise by physicians in order to provide the most appropriate treatments.It is known that patients over 60 years of age with moderate or severe CHD have worse survival prospective than general population: coronary artery disease, heart failure symptoms and NYHA class represent the strongest prognostic determinants. As compared to GUCH subjects aged between 20 and 60 years, those over 60 years of age need greater healthcare resources as outlined by considering the number of outpatient clinic visits and number and length of hospitalization of these patients [2]. The cardiovascular causes of admission to the hospital depend on complexity of CHD: congestive heart failure is the most frequent condition induced hospital admission and it is more frequent in patients suffering from complex disorders, including tetralogy of Fallot, truncus arteriosus and transposition of great arteries; valvular disease related cardiac decompensation is found in patients with simple disorder without atrial septal defect (ASD) or patent foramen ovale; finally, cerebrovascular event are the main cause of admission in case of patients suffering from simple atrial septal defect or patent foramen ovale [3]. Pulmonary arterial hypertension (PAH) represents an important complication of CHD, affecting about 10% of GUCH patients [4]. The risk of developing PAH is related to volume and pressure overload of the pulmonary circulation.Size of defect, degree of blood shunting and repair status are the main key pathogenic factors inducing PAH and right cardiac chambers enlargement. When the left and right ventricular pressures become similar, the direction of blood flow across the defect depends on pulmonary vascular resistance: when the latter exceeds systemic vascular resistance the reversal of flow and cyanosis occur (Eisenmenger syndrome).It has been estimated that about 44% of adult CHD subjects with a systemic-to-pulmonary shunt are at risk of PAH, and the prevalence of PAH in these subset of patients is 7.4% [5]. The beneficial effects in terms of pulmonary pressure values reduction in adults undergoing ASD occlusion are well known. Surgical closure of ASD significantly decreased right ventricular dimension and mean pulmonary pressure in patients over 35 years of ag...