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Indexed/Abstracted in: EMBASE, Scopus, Emerging Sources Citation Index
Online ISSN 1827-1782
Andrzej JURASZEK 1, Anna DROHOMIRECKA 2, Elżbieta K. BIERNACKA 3, Anna KLISIEWICZ 3, Mirosław KOWALSKI 3, Piotr HOFFMAN 3, Jacek RÓŻAŃSKI 1, Mariusz KUŚMIERCZYK 1
1 Department of Cardiac Surgery and Transplantation, The Cardinal Stefan Wyszyński Institute of Cardiology, Warsaw, Poland; 2 Department of Heart Failure and Transplantation, The Cardinal Stefan Wyszyński Institute of Cardiology, Warsaw, Poland; 3 Department of Congenital Heart Defects, The Cardinal Stefan Wyszyński Institute of Cardiology, Warsaw, Poland
We present a case of a 33-year-old woman who was diagnosed with Bland-White-Garland Syndrome (BWGS) during her second pregnancy. Patient was presented with systolic murmur and frequent ventricular extrasystoles. An echocardiogram showed hypokinesis of all apical segments with mildly decreased global systolic function of enlarged left ventricle (LVEF 50%, LVEDD 70mm), mild mitral insufficiency and blood flow between pulmonary trunk and left coronary artery. As the patient was mildly symptomatic further diagnostics was postponed after delivery. Interesingly, the coronary angiogram revealed left anterior descending (LAD) artery originating from pulmonary trunk; circumflex artery and right coronary artery (RCA) originated separately from the right sinus of Valsalva. Cardiopulmonary exercise test revealed good exercise capacity. Magnetic resonance imaging (MRI) confirmed the spatial relation of the vessels. Late gadolinium enhancement presented myocardial ischemic scar of the anterior wall and apex (up to the 50% and 75% of wall thickness, respectively). Patient was referred to surgery. Takeuchi’s repair was performed by making an intrapulmonary baffle connecting the aorta and the origin of LAD. Both surgery and recovery proceeded uneventful. Postoperative echocardiogram showed improvement of systolic function (LVEF 55-60%), hypokinetic apex and adequate blood flow in the baffle. BWGS should be always corrected as soon as it diagnosed even in asymptomatic patients.