Clinical features of pulmonary arterial hypertension associated with congenital systemic-to-pulmonaryshunts

Pulmonary arterial hypertension (PAH), associated with congenital heart disease (CHD) (PAH-CHD), despite the similarities of histological lesions, is а different from other forms of PAH. PAH-CHD is represented by a heterogeneous patient population with varying defect locations, concomitant diseases, indications and contraindications to surgical correction; also, some patients have a history of a defect closure. According to the European guidelines for the diagnosis and treatment of pulmonary hypertension 2015, there are four main groups in the clinical classification of PAH-CHD: 1) Eisenmenger's syndrome, 2) PAH associated with prevalent systemic-to-pulmonary shunts, 3) PAH with small/concidental defects, 4) PAH after defects correction. The evolution of the guidelines for the surgical correction of CHD has seen in recent years. Risk stratification has a great importance for further prognosis, response to treatment and timing of surgical correction. For optimal treatment carefulness and expertise in diagnosis and differential diagnosis should be exercised. A better understanding of clinical features, risk stratification and the assessment of the impact of a genetic background will help to determine the best clinical management, which is associated with better quality of life and improved survival in patients with PAH-CHD.