D.I. SADYKOVA¹, D.R. SABIROVA¹, N.A. SOLOVYEVA¹, G.A. KULAKOVA¹, E.A. KURMAEVA, A.A. MALOV^{1, 2}, T.I. KOCHETKOVA²

¹Kazan State Medical University Ministry of Health of the Russian Federation, Kazan

²Children’s Republican Clinical Hospital of the Ministry of Health of Republic of Tatarstan, Kazan

Contact details:

Solovyeva N.A. — PhD (medicine), Associate Professor of the Department of Hospital Pediatrics

Address: 49 Butlerov St., Russian Federation, Kazan, 420012, tel.: +7 (843) 556-74-52, e-mail: nailya-soloveva@mail.ru

Atresia of the pulmonary artery with an intact interventricular septum is a rare critical heart disease (CHD) in which there is no communication between the right ventricle and the pulmonary artery and the life of a newborn depends on the functioning of the open arterial duct. The article presents a clinical observation of a child diagnosed in the first hours of life with Atresia of the pulmonary artery valve. Open arterial duct. Open oval window. Ductus-dependent pulmonary circulation. In the Cardiac Surgery Department, an operation was performed: open valvuloplasty of the pulmonary artery valve and creation of a systemic pulmonary anastomosis in modification. After discharge on the 18^th day of life, the patient was under the supervision of a district pediatrician with regular monitoring of clinical and laboratory data and instrumental research methods (ECG, echocardiography, measurement of oxygen saturation) and consultation with a cardiologist and cardiac surgeon. At the age of 2 years 5 months radical surgery was performed: Plastic valve pulmonary artery, tricuspid valve plasty, plasty of the right branch of the pulmonary artery, the elimination of systemic-pulmonary anastomosis, plastic ASD in conditions of artificial circulation. The patient was discharged with recovery. Careful monitoring of the child’s health by a pediatrician, cardiologist and cardiac surgeon allowed preventing the occurrence of complications in the postoperative period and determining the optimal timing of anatomical radical correction of the defect, which led to full recovery and ensured the harmonious development of the girl.

Key words: children, critical heart disease, correction, condition monitoring.

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