DIFFICULTIES IN THE ECHOCARDIOGRAPHIC DIAGNOSIS OF BLAND-WHITE-GARLAND SYNDROME

Abstract

SUMMARY The Bland-White-Garland Syndrome (BWG) is a rare, but serious congenital cardiac anomaly, found in 1/300 000 life born children or is 0,25-0,5% of the congenital heart anomalies (CHA). At the same time it is the most frequent congenital coronary anomaly - 90%. It is described for the first time in 1866, but the full clinical-anatomic description is given from Bland, White and Garland in 1933, and that is why it takes their names. It represents anomaly of the start of the left coronary artery from the trunk of the pulmonary artery (ALKAPA), most often from the left sinus with signs of myocardial ischemia in the relevant zones. In evolution develop collaterals between both coronary arteries and the syndrome of “stealing” because of the presence of significant left-right shunt on vascular level. Connected to this there are two forms of the disease: infantile and adult. The aim of this report is to present the ECG and EchoCG results, clinical manifestation and result of the held conservative and operative treatment in three children, hospitalized in the clinic during the last years, establishing diagnostic difficulties in the echocardiograph examination and specifying of the type of the congenital cardiac malformation. ECG, clinical manifestation, outcome of the disease. The Bland-White-Garland Syndrome (BWG) is a rare, but serious congenital cardiac anomaly, found in 1/300 000 life born children or is 0,25 - 0,5% of the congenital cardiac anomalies (CCA). At the same time it is the most frequent congenital coronary anomaly - 90%. It is described for the first time in 1866, but the full clinical-anatomic description is given from Bland, White and Garland in 1933, and that is why it takes their names. It represents anomaly of the start of the left coronary artery from the trunk of the pulmonary artery (ALCAPA), most often from the left sinus with signs of myocardial ischemia in the relevant zones. In evolution develop collaterals between both coronary arteries and the syndrome of “stealing” because of the presence of significant left-right shunt on vascular level. Connected to this there are two forms of the disease: infantile and adult. The anomaly most often if isolated, but it can be combined with other CCA - interventricular defects, persisting arterial channel, tetralogy of Fallot or aortic coarctation. The early diagnosis is often crucial for the prognosis and outcome of the operative treatment.