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Isolated Innominate Artery From the Main Pulmonary Artery in DiGeorge Syndrome
Journal of the American College of Cardiology © 2011 by the American College of Cardiology Foundation Published by Elsevier Inc.
Vol. 57, No. 6, 2011 ISSN 0735-1097/$36.00 doi:10.1016/j.jacc.2010.03.110
IMAGES IN CARDIOLOGY
Isolated Innominate Artery From the Main Pulmonary Artery in DiGeorge Syndrome Joe Kreeger, RDCS, RCCS, Brian Schlosser, BS, RDCS, RDCM, Denver Sallee, MD, William Border, MBCHB, MPH, Shriprasad Deshpande, MBBS, MS Atlanta, Georgia
From Children’s Healthcare of Atlanta/Emory University, Pediatric Cardiology, Atlanta, Georgia. Manuscript received February 16, 2010; accepted March 9, 2010.
A
3-month-old patient with a known diagnosis of perimembranous ventricular septal defect and DiGeorge syndrome was admitted for an aspiration event. On admission, transthoracic echocardiography confirmed the known diagnosis of perimembranous ventricular septal defect. Suprasternal aortic arch imaging failed to demonstrate normal innominate artery origin. On careful evaluation, a right-sided aortic arch was noted, with isolated innominate artery originating from the main pulmonary artery (A, Online Videos 1 and 2). The right carotid and right subclavian arteries had normal origins from the aorta. Cardiac magnetic resonance imaging confirmed the diagnosis of isolated innominate artery from the main pulmonary artery (B to D). Isolated innominate artery from the pulmonary artery is an exceedingly rare anomaly (1,2). There are only 2 previous reports of association with DiGeorge syndrome (3,4). Embryologically, the hypothetical double aortic arch of Edwards can explain the occurrence of isolated innominate artery (E) (5). Clinically, in the absence of an intracardiac shunt, differential cyanosis between the right and left arm should raise the suspicion for this anomaly. In most cases, including ours, the innominate artery can be relocated to the aorta with good results.
REFERENCES
1. Singh B, Satyapal KS, Moodley J, Rajaruthnam P. Right aortic arch with isolated left brachiocephalic artery. Clin Anat 2001;14:47–51. 2. Le Bret E, Leobon B, Roubertie F, et al. Right aortic arch and isolated left innominate artery from a left sided patent ductus arteriosus: a very rare aortic arch anomaly. J Pediatr Surg 2009;44:E29 –31.
3. Duke C, Chan KC. Isolated innominate artery in 22q11 microdeletion. Pediatr Cardiol 2001;22:80 –2. 4. Miyaji K, Hannan RL, Burke RP. Anomalous origin of innominate artery from right pulmonary artery in DiGeorge syndrome. Ann Thorac Surg 2001;71:2043–9. 5. Edwards JE. Anomalies of derivatives of the aortic arch systems. Med Clin North Am 1948;19:925–94.
Vol. 57, No. 6, 2011 ISSN 0735-1097/$36.00 doi:10.1016/j.jacc.2010.03.110
IMAGES IN CARDIOLOGY
Isolated Innominate Artery From the Main Pulmonary Artery in DiGeorge Syndrome Joe Kreeger, RDCS, RCCS, Brian Schlosser, BS, RDCS, RDCM, Denver Sallee, MD, William Border, MBCHB, MPH, Shriprasad Deshpande, MBBS, MS Atlanta, Georgia
From Children’s Healthcare of Atlanta/Emory University, Pediatric Cardiology, Atlanta, Georgia. Manuscript received February 16, 2010; accepted March 9, 2010.
A
3-month-old patient with a known diagnosis of perimembranous ventricular septal defect and DiGeorge syndrome was admitted for an aspiration event. On admission, transthoracic echocardiography confirmed the known diagnosis of perimembranous ventricular septal defect. Suprasternal aortic arch imaging failed to demonstrate normal innominate artery origin. On careful evaluation, a right-sided aortic arch was noted, with isolated innominate artery originating from the main pulmonary artery (A, Online Videos 1 and 2). The right carotid and right subclavian arteries had normal origins from the aorta. Cardiac magnetic resonance imaging confirmed the diagnosis of isolated innominate artery from the main pulmonary artery (B to D). Isolated innominate artery from the pulmonary artery is an exceedingly rare anomaly (1,2). There are only 2 previous reports of association with DiGeorge syndrome (3,4). Embryologically, the hypothetical double aortic arch of Edwards can explain the occurrence of isolated innominate artery (E) (5). Clinically, in the absence of an intracardiac shunt, differential cyanosis between the right and left arm should raise the suspicion for this anomaly. In most cases, including ours, the innominate artery can be relocated to the aorta with good results.
REFERENCES
1. Singh B, Satyapal KS, Moodley J, Rajaruthnam P. Right aortic arch with isolated left brachiocephalic artery. Clin Anat 2001;14:47–51. 2. Le Bret E, Leobon B, Roubertie F, et al. Right aortic arch and isolated left innominate artery from a left sided patent ductus arteriosus: a very rare aortic arch anomaly. J Pediatr Surg 2009;44:E29 –31.
3. Duke C, Chan KC. Isolated innominate artery in 22q11 microdeletion. Pediatr Cardiol 2001;22:80 –2. 4. Miyaji K, Hannan RL, Burke RP. Anomalous origin of innominate artery from right pulmonary artery in DiGeorge syndrome. Ann Thorac Surg 2001;71:2043–9. 5. Edwards JE. Anomalies of derivatives of the aortic arch systems. Med Clin North Am 1948;19:925–94.