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Bilateral Left-Sidedness Heterotaxy Syndrome
Journal of the American College of Cardiology © 2011 by the American College of Cardiology Foundation Published by Elsevier Inc.
Vol. 58, No. 1, 2011 ISSN 0735-1097/$36.00 doi:10.1016/j.jacc.2010.08.655
IMAGES IN CARDIOLOGY
Bilateral Left-Sidedness Heterotaxy Syndrome Tatyana Danilov, MD, Muhamed Saric, MD, PHD, Monvadi B. Srichai, MD, Itzhak Kronzon, MD New York, New York
From the Leon H. Charney Division of Cardiology, New York University School of Medicine, New York, New York. Manuscript received August 10, 2010, accepted August 25, 2010.
A
25-year-old man with a history of atrioventricular (AV) canal defect repair in childhood underwent transesophageal echocardiography that revealed left atrial appendage morphology of the right atrial appendage (A) and a prominent azygos vein (B). Three-dimensional transesophageal echocardiography showed a cleft anterior mitral valve (Online Video 1). Cardiac magnetic resonance imaging confirmed the diagnosis of bilateral left-sidedness syndrome, including left atrial isomerism (C), absence of the intra-hepatic segment of the inferior vena cava (IVC), azygos continuation of the IVC (D, Online Video 2), and hyparterial bronchi (E). Heterotaxy syndrome represents a group of disorders of abnormal lateralization of the abdominal and thoracic organs. Sidedness of the heart is determined by the atrial appendage morphology. Cardiovascular findings of bilateral left-sidedness syndrome include bilateral left atrial appendage morphology, AV canal defects, azygos continuation of the IVC, as well as conduction abnormalities. Extracardiac findings include midline liver, polysplenia, hyparterial bronchi, and bi-lobed lungs. SVC ⫽ superior vena cava.
Vol. 58, No. 1, 2011 ISSN 0735-1097/$36.00 doi:10.1016/j.jacc.2010.08.655
IMAGES IN CARDIOLOGY
Bilateral Left-Sidedness Heterotaxy Syndrome Tatyana Danilov, MD, Muhamed Saric, MD, PHD, Monvadi B. Srichai, MD, Itzhak Kronzon, MD New York, New York
From the Leon H. Charney Division of Cardiology, New York University School of Medicine, New York, New York. Manuscript received August 10, 2010, accepted August 25, 2010.
A
25-year-old man with a history of atrioventricular (AV) canal defect repair in childhood underwent transesophageal echocardiography that revealed left atrial appendage morphology of the right atrial appendage (A) and a prominent azygos vein (B). Three-dimensional transesophageal echocardiography showed a cleft anterior mitral valve (Online Video 1). Cardiac magnetic resonance imaging confirmed the diagnosis of bilateral left-sidedness syndrome, including left atrial isomerism (C), absence of the intra-hepatic segment of the inferior vena cava (IVC), azygos continuation of the IVC (D, Online Video 2), and hyparterial bronchi (E). Heterotaxy syndrome represents a group of disorders of abnormal lateralization of the abdominal and thoracic organs. Sidedness of the heart is determined by the atrial appendage morphology. Cardiovascular findings of bilateral left-sidedness syndrome include bilateral left atrial appendage morphology, AV canal defects, azygos continuation of the IVC, as well as conduction abnormalities. Extracardiac findings include midline liver, polysplenia, hyparterial bronchi, and bi-lobed lungs. SVC ⫽ superior vena cava.