Long-term efficacy of specific therapy in patients with different clinical variants of pulmonary arterial hypertension associated with congenital heart disease

Aim: evaluate the frequency of prescription and long-term efficacy of different types of specific therapy in patients with pulmonary arterial hypertension (PAH) associated with congenital heart defects (CHD). Materials and methods. The retrospective part of the study included patients first admitted to the National Medical Research Center of Cardiology in the period 2015-2017 — 30 patients with Eisenmenger syndrome, 25 with left-to-right shunt, 26 with residual PAH. 58 patients were followed up 12 and 24 months after the first hospitalization. Results. The most commonly prescribed drugs for PAH-CHD pts were sildenafil and bosentan as monotherapy and in combination. By the 24th month of followup there was a twofold increase of patients with combined specific therapy in all groups, the largest increase in patients with residual PAH. After 24 months, all modes of therapy showed positive dynamics with an increase in distance in the 6-minute walk testing, functional class improvement, a decrease in the mean pulmonary artery pressure, a decrease in the size of the right ventricle, and normalization of the left heart. The survival rate of patients with Eisenmenger syndrome was 80,5%, PAH-CHD with bidirectional shunt — 93,5%, residual PAH — 47,9%, idiopathic pulmonary hypertension — 48%. Conclusion. Patients with Eisenmenger syndrome receive specific monotherapy for a long time, while maintaining low/intermediate risk, are characterized by the best survival. Patients with residual PAH have the most severe hemodynamic impairment, rapid progression of the disease, despite the ongoing combination specific therapy, and low survival.