ОПЫТ ХИРУРГИЧЕСКОГО ЛЕЧЕНИЯ РЕБЕНКА ГРУДНОГО ВОЗРАСТА С СИНДРОМОМ ЯТАГАНА: КЛИНИЧЕСКОЕ НАБЛЮДЕНИЕ

Abstract

Scimitar syndrome (SS) is an extremely rare congenital heart disease (CHD). There are two main clinical forms of the disorder: adult and infant. The usual course of the infantile form is characterized by an unfavorable prognosis. According to some reports the mortality could reach 100% without surgical treatment. The results of surgical treatment in this age group are currently unsatisfactory. Authors represent a clinical case of SS in a 4.5 months old male patient, who was in moderate and relatively stable condition upon admission. The severity of the patient’s condition was mainly caused by the progressive cardiac and respiratory failure. The diagnoses of CHD, SS were established during the clinical and instrumental examination: partial anomalous drainage of the right pulmonary veins into the inferior vena cava, hypoplasia of the right branch of the pulmonary artery, hypoplasia of the right lung, sequestration of the right lung, dextrocardia, secondary atrial septal defect (ASD), therefore, the indications for surgical correction were also identified. Ligation of the systemic pulmonary anastomoses, reimplantation of the right pulmonary vein collector into the left atrium, suturing of ASD under cardiopulmonary bypass and hypothermic circulatory arrest had been performed. The postoperative period was difficult: the patient was supported with an artificial lung ventilation for long time. He was transferred to the general somatic department on the 28th day after the surgical intervention, which was then followed by a favorable long-term result. Conclusion: Authors represent a successful case of radical surgical treatment of a child aged 4.5 months old with a rare CHD using the non-standard technique for reimplantation of the pulmonary vein collector as well as the features of intraoperative support.