Echocardiographic Marker for Bland-White-Garland Syndrome in Adult

Bland-White-Garland syndrome, also known anomalous origin of the left main coronary artery, is a rare congenital malformation. The authors report the case of a 51-year-old woman with anomalous origin of the left main coronary artery from the pulmonary artery. A prominent large ostium of the right coronary artery and intercoronary “steal” collaterals should raise suspicion for this pathology and subsequently guide a step-by-step echocardiographic approach to identify the left main coronary artery from the pulmonary artery.

Bland-White-Garland syndrome is a rare congenital malformation particularly affecting the adult population. It is identified by an anomalous origin of the left main coronary artery (LMCA) from the pulmonary artery (ALCAPA) that leads to abnormal perfusion of the left ventricle, myocardial dysfunction, arrhythmias, and ischemic mitral regurgitation. The majority of those afflicted die in the first year, only a small minority of patients survive past childhood without surgical repair, and up to 90% die suddenly at a mean age of 35 years. Survival to adult life without treatment depends on the development of adequate collateralization and other anatomic factors, such as high pulmonary artery pressure or stenosis of the anomalous origin of LMCA, that limit coronary steal and increase myocardial perfusion pressure. Postoperative complications such as occlusion and stenosis of the LMCA after reimplantation of the ALCAPA are extremely rare. It is usually related to the excessive tension placed on this vessel to reach the aorta and from its distant position on the posterolateral aspect of the main pulmonary artery (MPA).

Case Presentation

A 51-year-old asymptomatic woman was referred to our institution for further evaluation because of a syncopal episode. Physical examination revealed blood pressure of 120/75 mm Hg, with an irregular heart rate of 98 beats/min and a grade 4/6 holosystolic murmur along the left sternal border and apex. Electrocardiography showed atrial fibrillation, loss of the R waves in precordial leads V 1 and V 2 , Q waves in leads V 1 and V 2 , and ischemic changes in V 5 and V 6 . Transthoracic echocardiography showed an enlarged left atrium, a moderately enlarged left ventricle, anteroseptal hypokinesis, mildly reduced pump function, and pulmonary hypertension. Mitral valve prolapse with severe mitral regurgitation was also seen ( Video 1 ). From the parasternal long-axis view, a prominent, large (7-mm) origin of the right coronary artery (RCA) arising from the right sinus of Valsalva was visualized ( Figure 1 A). Using Doppler color flow imaging, it was possible to follow the course of the very large tortuous RCA and to identify extensive intercoronary collateral vessels ( Figures 1 B and 1 C, Videos 2 and 3 ). The origin of the LMCA was absent from the left sinus of Valsalva but was seen with abnormal retrograde coronary blood flow to arise from the pulmonary trunk ( Figures 1 D and 1 E, Video 4 ). Coronary angiography showed a massively dilated RCA that gave rise to abundant collateral vessels. The origin of the LMCA was not visualized. Multidetector computed tomography revealed a normally originating, dilated, and tortuous RCA ( Figure 2 A); multiple collateral vessels; and an abnormal origin of the LMCA from the MPA ( Figure 2 B).

Figure 1

(A) Parasternal long-axis view revealed a large origin of the RCA (arrowhead). (B) With color flow Doppler. (C) Modified apical two-chamber view showed color flow Doppler image of massive steal collaterals. (D) Parasternal short-axis view. The LMCA is seen to arise from the MPA. (E) Color flow Doppler image indicates retrograde filling of the LMCA from the RCA.

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Jun 11, 2018 | Posted by in CARDIOLOGY | Comments Off on Echocardiographic Marker for Bland-White-Garland Syndrome in Adult

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