Anesth Analg 2007;104:1348-1349
© 2007 International Anesthesia Research Society
doi: 10.1213/01.ane.0000261257.66969.28
CARDIOVASCULAR ANESTHESIA
Transesophageal Echocardiography in Adult-Type Bland-White-Garland Syndrome
Leonid L. Minkovich, MD, PhD*,
Stephanie J. Brister, MD, FRCS(C)
, and
Peter D. Slinger, MD, FRCPC*
From the *Department of Anesthesia; and Division of Cardiovascular Surgery of Toronto General Hospital, University Health Network, University of Toronto, Toronto, Ontario, Canada.
Address correspondence and reprint requests to Dr. Leonid L. Minkovich, Department of Anesthesia, Toronto General Hospital, University Health Network, 3-410 Eaton North Wing, 200 Elizabeth Street, Toronto, ON M5G 2C4, Canada. Address e-mail to leonid.minkovich{at}uhn.on.ca.
The origin of the left main coronary artery (LMCA) from the pulmonary artery (PA), known as Bland-White-Garland syndrome (BWGS), is a rare anomaly with mortality of 90% during infancy (1). If collateral circulation between right and left coronary systems ensues, BWGS patients may reach adulthood (1,2). However coronary flow directs preferentially into the lower pressure PA, away from the left ventricle (LV) creating "coronary steal," and left-to-right shunt. Mitral regurgitation (MR) is further common in BWGS most likely due to multiple factors (3). Surgical correction of BWGS is strongly recommended, even in asymptomatic patients, because of the high risk of developing heart failure, myocardial infarction, ventricular arrhythmias, and sudden death (1,2).
A 44-yr-old man presented with recent onset of fatigue and atrial fibrillation. Transthoracic echocardiography demonstrated severe MR with a posterior-directed jet, mild anterior mitral leaflet prolapse, a dilated mitral annulus, enlarged left atrium (5.8 cm), preserved global LV function, a moderately dilated right ventricle with moderate tricuspid regurgitation, and estimated right ventricle systolic pressure of 60 mm Hg. Because of the patient’s occasional chest pain, a persantine–thallium study was performed demonstrating a significant perfusion defect of the anterior LV wall. Cardiac catheterization revealed the absence of the LMCA in the left sinus of Valsalva. Contrast dye injected into the right coronary artery (RCA) filled the main branches of the LMCA with anomalous drainage through the LMCA into the PA.
The patient was referred for mitral valve (MV) repair and correction of anomalous LMCA. Intraoperative transesophageal echocardiography (TEE) mostly confirmed the preoperative echocardiography findings but now demonstrated a large RCA (17 mm of diameter) that was identified at the right sinus of Valsalva. Color flow Doppler examination showed that the LMCA passed close to the left sinus of Valsalva with no connection to the aortic root. The LMCA origin was identified as arising from the PA, 18 mm distal to the pulmonic valve with retrograde flow into the PA (Figs. 1 and 2; please see video clip available at www.anesthesia-analgesia.org).
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Figure 1. Preoperative multiplane transesophageal echocardiography (TEE), mid-esophageal aortic valve long axis view. The large orifice of the right coronary artery can be seen with laminar diastolic antegrade flow demonstrated by Color Doppler mapping. (LA, left atrium; MV, mitral valve; LVOT, left ventricle outflow tract, IVS, interventricular septum; AoV, aortic valve; AO, ascending aorta; RCA, right coronary artery.)
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Figure 2. Preoperative multiplane transesophageal echocardiography (TEE), modified mid-esophageal aortic valve short axis view. The ascending aorta just above the aortic valve can be recognized. Color Doppler shows the left main coronary artery (LMCA) passing close to the left sinus of Valsalva however with no identified connection to the Aortic root. (LA, left atrium; RA, right atrium; IAS, interatrial septum; AO, ascending aorta.)
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The patient underwent uncomplicated reimplantation of the LMCA into the aortic root and MV repair. The postoperative TEE showed LMCA orifice reimplanted into the left sinus of Valsalva, and the MV annuloplasty ring with mild MR (Fig. 3).
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Figure 3. Postoperative multiplane transesophageal echocardiography (TEE), mid-esophageal aortic valve short axis view. The LMCA orifice now reimplanted into the left sinus of Valsalva can be seen. (LA, left atrium; RA, right atrium; IAS, interatrial septum; LCC, left coronary cusp; RCC, right coronary cusp; NCC, noncoronary cusp; LMCA, left main coronary artery.)
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Identification of the coronary orifices and normal coronary flow pattern is an important part of a TEE examination. Young adults with no risk factors for coronary disease may present with MR and unrecognized BWGS. In the presence of a single enlarged RCA orifice, BWGS should be considered.
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Footnotes
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Accepted for publication February 8, 2007.
This study was supported entirely with departmental funds.
The authors have no conflicts to report.
This article has supplementary material on the Web site: www.anesthesia-analgesia.org.
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REFERENCES
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- Perloff JK Anomalous origin of coronary arteries from the pulmonary trunk. In: Perloff JK, ed. The clinical recognition of congenital heart disease. 4th ed. Philadelphia: WB Saunders, 1994;546–61.
- Azakie A, Russell JL, McCrindle BW, et al. Anatomic repair of anomalous left coronary artery from the pulmonary artery by aortic reimplantation: early survival, patterns of ventricular recovery and late outcome. Ann Thorac Surg 2003;75: 1535–41.[Abstract/Free Full Text]
- Chau EM, Cheng LC, Lee JW. Severe mitral regurgitation due to mitral valve prolapse associated with Bland-White-Garland syndrome. Hong Kong Med J 2001;7:307–10.[Medline]
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