A Long-Term Survivor of Bland-White-Garland Syndrome
Background: land-White-Garland syndrome (anomalous origin of the left coronary artery from the pulmonary artery) is a rare disease which may result in myocardial infarction, congestive heart failure and sometimes death during the early infantile period.
Case Presentation: A successfully treated case of a 45-year-old mother of 2 children with Bland-White-Garland syndrome and concomitant severe mitral regurgitation is presented. Subsequent therapy consisted of ligation of the anomalous origin of the left coronary artery, anastomosis of the left internal mammary artery to the left anterior descending branch and mitral valve replacement. Continuous blood flow from the left coronary artery ostium during extracorporeal circulation and aorta clamping suggested systemic collateral supply.
Conclusions: Recognition and diagnosis of Bland-White-Garland syndrome is important due to its potentially life-threatening complications.
Anomalous origin of the left coronary artery arising from the pulmonary artery (Bland-White-Garland syndrome also known as ALCAPA syndrome), is a rare congenital abnormality affecting 1 in 300.000 live births, accounting for 0,5% of cases of congenital heart disease.
Patients with Bland-White-Garland (BWG) syndrome who survive past childhood often have varying symptoms of myocardial ischaemia, impaired left ventricular function, mitral regurgitation and progressive heart failure, depending on the development of collateral circulation. The majority of patients die in infancy.
A case of a 45-year-old woman referred to our institution with the diagnosis of mitral regurgitation is presented. Detailed diagnostics revealed BWG syndrome. Subsequent therapy consisted of ligation of the anomalous origin of the left coronary artery, anastomosis of left internal mammary artery (LIMA) to the left anterior descending (LAD) branch and mitral valve replacement with a mechanical prosthesis.