Features Of Diagnosis and Choice of Treatment Tactics of The First-Diagnosed Bland-White-Garland Syndrome with Severe Coexisting Somatic Pathology (Clinical Case)

ALCAPA (Anomalous Left Coronary Artery from the Pulmonary Artery) is a rare and dangerous congenital anomaly that is one of the leading causes of myocardial ischemia and infarction in pediatric patients. Without timely treatment and correction, the disease can lead to extremely severe consequences: up to 90 % of patients die within the first year of life. In adults, patients suffering from this syndrome may face various complications such as left ventricular dysfunction, mitral regurgitation, asymptomatic myocardial ischemia and infarction, and a high risk of sudden cardiac death. Given that this disease is rarely diagnosed, it is important to detect it early for preventive treatment and avoidance of serious disorders.This paper describes a unique case of first-diagnosed ALCAPA syndrome in a 27-year-old female patient. In addition to the malformation, the woman suffered from severe concomitant pathology — primary sclerosing cholangitis, which was complicated by liver cirrhosis and hypersplenism. The presence of these diseases significantly complicated the choice of therapeutic tactics, requiring a personalized approach. Due to the high risk of postoperative complications, preference was given to minimally invasive endovascular embolization of the left coronary artery. The operation showed good results and led to significant improvement of the patient’s condition, including the achievement of heart failure compensation up to functional class II (NYHA). This case highlights the importance of an individualized approach in the treatment of rare adult congenital heart disease, especially in the presence of serious concomitant diseases of other organs and systems.