Circulation. 1999;100:1757-1759
(Circulation. 1999;100:1757-1759.)
© 1999 American Heart Association, Inc.
Images in Cardiovascular Medicine |
Ebstein's Anomaly Associated With an Anomalous Coronary Artery, 2 Myocardial Bridges, and a Mahaim Fiber
James A. de Lemos, MD;
Etienne Delacretaz, MD;
Robert N. Piana, MD;
Toussaint Smith, MD;
Jose Rivero, MD;
Daniel I. Simon, MD;
Peter L. Friedman, MD, PhD
From the Cardiovascular Division, Brigham and Women's Hospital,
Harvard Medical School, Boston, Mass.
Correspondence to James A. de Lemos, MD, Cardiovascular Division, Brigham and Women's Hospital, 75 Francis St, Boston, MA 02115. E-mail jdelemos{at}rics.bwh.harvard.edu
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Introduction
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 Top
 Introduction
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A61-year-old man with
Ebstein's anomaly was referred for evaluation
of palpitations and
recurrent chest pain. A
99mTc-sestamibi
exercise
test showed no evidence of ischemia, and a Holter
monitor and
ECG event recorder were unrevealing. The patient's
hospital
course is summarized in the accompanying images.
An echocardiogram confirmed the diagnosis of Ebstein's anomaly (Figure 1
), without associated tricuspid
regurgitation or right heart failure. Cardiac
catheterization revealed an anomalous right
coronary artery arising from the left sinus of Valsalva and
passing between the aorta and pulmonary artery (Figure 2). This congenital anomaly is associated
with an increased risk for sudden cardiac death, particularly during
exercise. Angiography also demonstrated myocardial bridging in the mid
left anterior descending coronary artery (Figure 3) and in several obtuse marginal
branches of the circumflex artery (Figure 4). Myocardial bridges have been
associated with ischemic chest pain, myocardial infarction,
ventricular arrhythmias, and sudden cardiac
death.
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Figure 1. A 4-chamber echocardiographic view
showing displacement of tricuspid leaflets (TV) into right ventricle, a
finding characteristic of Ebstein's anomaly.
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Figure 2. Right anterior oblique (left) and left anterior
oblique (right) views of right coronary artery (arrows),
illustrating an anomalous origin from left sinus of Valsalva. Left,
artery can be seen coursing in anterior direction, between aorta and
pulmonary artery.
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Figure 3. Images of mid left anterior descending
coronary artery in diastole (left) and systole
(right), demonstrating systolic constriction consistent
with a myocardial bridge (arrow).
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Figure 4. Diastolic (left) and systolic
(right) images of circumflex artery and its branches. In several small
obtuse marginal branches (arrows), systolic flow is
obliterated, consistent with myocardial bridging.
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Electrophysiological study (Figure 5) identified antidromic
atrioventricular reentrant tachycardia
(AVRT) with anterograde conduction over a decrementally
conducting accessory pathway (Mahaim fiber). Endocardial mapping
localized this pathway to the anterolateral tricuspid annulus.
Application of radiofrequency current to this site instantly terminated
the tachycardia, after which no residual accessory pathway
conduction remained and no reentrant tachycardia could be
induced.
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Figure 5. Sinus rhythm (left) and
antidromic AVRT (right) recorded during endocardial catheter
mapping. From top of each panel are surface ECG leads I, III,
V1, and V6 and bipolar electrograms from high
right atrium (HRA), His bundle (His), ablation catheter (Abl), and
right ventricular apex (RVA). During sinus rhythm, right
bundle-branch block is present without ventricular
preexcitation. Ablation catheter positioned at anterolateral aspect of
tricuspid annulus, far away from His bundle recording site,
records an accessory pathway potential (AP) that is inscribed much
later than His (H) deflection. QRS during sustained antidromic AVRT has
a left bundle-branch morphology. Earliest atrial electrogram
(A) during AVRT is recorded in His lead, consistent with
retrograde AV nodal conduction. AV interval during AVRT is long, and an
accessory pathway potential precedes each ventricular
electrogram (V), consistent with anterograde conduction
over decremental accessory pathway.
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The patient subsequently underwent single-artery coronary
artery bypass graft surgery, with a right internal mammary artery graft
to the right coronary artery. In addition, the myocardial
bridge in the left anterior descending artery was "unroofed." The
patient recovered uneventfully and remains free of chest pain and
palpitations.
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Footnotes
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The editor of Images in Cardiovascular Medicine is Hugh A. McAllister,
Jr, MD, Chief, Department of Pathology, St Luke's Episcopal
Hospital and Texas Heart Institute, and Clinical Professor of
Pathology, University of Texas Medical School and Baylor College
of Medicine.
Circulation encourages readers to submit cardiovascular images to Dr Hugh A. McAllister, Jr, St Luke's Episcopal Hospital and Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.
This article has been cited by other articles:
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I. Adachi, S. Y. Ho, and H. Uemura
Reply to the Editor
J. Thorac. Cardiovasc. Surg.,
August 1, 2009;
138(2):
515 - 515.
[Full Text]
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