Bland-White-Garland syndrome: clinical features of the course and treatment in young patients
Authors:   PhD, associate professor at the Department Hospital therapy, occupational diseases and clinical pharmacology of the Medical Institute of M.K. Ammosov North-Eastern Federal University, Russian Federation, Yakutsk, Palshina A.M. , Silina N.N. ,   head of the cardiological department with ICU for patients with ACS of the Republican Cardiovascular Center Republican Hospital No1 – M.E. Nikolaev National Center of Medicine, Russian Federation, Yakutsk, Okorokova A.B. ,   cardiologist at the cardiological department with ICU for patients with ACS of the Republican Cardiovascular Center Republican Hospital No1 – M.E. Nikolaev National Center of Medicine,   Russian Federation, Yakutsk, Ivanov K.I. ,   MD, Director of the Republican Cardiovascular Center Republican Hospital №. 1 - M.E. Nikolaev National Center of Medicine,  Russian Federation, Yakutsk, Rosseykin E.V. ,   MD, Professor, Chief doctor of the Federal Center for Cardiovascular Surgery of the Ministry of Health of the Russian Federation, Russian Federation, Khabarovsk, Kobzev E.E. ,   cardiovascular surgeon, head of cardiac surgery department №2 at the Federal Center for Cardiovascular Surgery of the Ministry of Health of the Russian Federation, Khabarovsk, Borisov A.I. ,   1-year resident of the specialty ‘Cardiology’, assistant of the Department of ‘Propaedeutic and Faculty therapy with Endocrinology and Physical Therapy’ of the Medical Institute of M.K. Ammosov North-Eastern Federal University, Russian Federation, Yakutsk, Gogolev V.A. ,   a 2-year resident of the specialty ‘Internal medicine’ assistant of the Department ‘Hospital therapy, occupational diseases and clinical pharmacology’ of the Medical Institute of M.K. Ammosov North-Eastern Federal University, Russian Federation, Yakutsk
Published: January 2025

Bland-White-Garland syndrome (SBUG) is a rare, high–risk fatal outcome in infancy and sudden death in adults, an anomaly of the divergence of the left coronary artery (LCA) from the pulmonary artery (PA) (ALCAPA - abnormal left coronary artery arising from the pulmonary artery). In 85-90% of cases, children without radical surgical treatment do not live up to a year. In our clinical case, in a 36-year-old patient, the disease debuted with recurrent ventricular arrhythmias, Imaging diagnostic methods coronary angiography, spiral computed tomography, magnetic resonance imaging of the heart revealed in the patient, in addition to the pathology of the LCA, individual anatomical features of the coronary bed, which provided adequate myocardial perfusion and maintained sufficient myocardial reserve. A combined operation was performed on a patient at the "Federal Center for Cardiovascular Surgery" of Khabarovsk: prosthetics of the LCA trunk with a vascular prosthesis "Vascutek PTFE" № 6. Plastic surgery of the pulmonary trunk (PT) with a patch from the autopericardium. Mitral valve repair (MV) on the "MedInj" support ring № 28. The postoperative period was uneventful. The period of dynamic observation was 22 months. Currently, the patient is in a satisfactory condition and is able to work. Given the different clinical variants of SBUG, specialists providing primary health care - district pediatricians, internists, cardiologists - should be wary of this rare but life-threatening congenital malformation.
Keywords: Bland-White-Garland syndrome, anomaly of the left coronary artery, ALCAPA.