A.V. KRASNOPOLSKAYA ¹, L.A. BALYKOVA ¹, N.V. SCHEKINA ², E.S. GLUKHOVA ¹, T.I. KORNILOVA ²

 ¹ National Research Mordovian State University named after N.P. Ogarev, 68 Bolshevistskaya Str., Saransk, Russian Federation, 430005

² Children’s Republican Clinical Hospital of the Ministry of Health of the Republic of Mordovia, 15 R. Luksemburg Str., Saransk, Russian Federation, 430032

Krasnopolskaya A.V. ― post-graduate student of the Department of Pediatrics, tel. +7-927-276-33-63, e-mail: [email protected]

Balykova L.A. ― D. Med. Sc., Professor, Corresponding Member of RAS, Head of the Department of Pediatrics, Director of the Medical Institute, tel. (8342) 32-19-83, e-mail: [email protected]

Shchekina N.V. ― Head of the Unit of Cardiology, tel. (8342) 35-32-15, e-mail: [email protected]

Glukhova E. S. ― the 6^th year student, tel. (8342) 35-30-02, e-mail: [email protected]

Kornilova T.I. ― Cand. Med. Sc., cardiologist-consultant, tel. (8342) 35-32-15, e-mail: [email protected]

The article gives a description of a clinical case that peculiarities of pulmonary hypertension (PH) development in a girl with complex congenital heart disease. Relatively late appeal to a specialized cardiosurgical hospital in combination with the inability in 2002 in Russia of radical surgical correction of this defect against the background of rapid development of high pulmonary hypertension has made pharmacotherapy the only possible method of treatment. The article presents anamnestic data, dynamics of clinical symptoms and echocardiographic indices. The effectiveness of sildenafil and bosentan in correction of clinical and echocardiographic signs of high pulmonary hypertension associated with congenital heart disease is demonstrated. The authors compare the obtained results with international researches data, Russian and European clinical guidelines.

Key words: pulmonary hypertension, congenital heart disease, sildenafil, bosentan

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