Transesophageal Echocardiography in Adult-Type Bland-White-Garland Syndrome
Notice bibliographique
Résumé
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).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.)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.)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).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.)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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Prédiction distillée sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Apprise à partir de 10 348 étiquettes directes de Codex et de 10 348 étiquettes directes de Gemma. Le mode candidate est l'union des têtes enseignantes seuillées; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont ni des étiquettes humaines ni des étiquettes directes de modèles de pointe.
Scores Codex et Gemma par catégorie
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,001 | 0,000 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,001 | 0,000 |
| Bibliométrie | 0,001 | 0,001 |
| Études des sciences et des technologies | 0,000 | 0,000 |
| Communication savante | 0,000 | 0,000 |
| Science ouverte | 0,000 | 0,000 |
| Intégrité de la recherche | 0,000 | 0,000 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,000 | 0,000 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule tête enseignante, pas un consensus.
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