Images in Cardiovascular Medicine Atresia of the Ascending Aorta in Hypoplastic Left Heart Syndrome Christoph Haller, MD; Osami Honjo, MD, PhD; Timothy Bradley, MD; Andreea Dragulescu, MD; Glen S. Van Arsdell, MD
T
he spectrum of hypoplastic left heart syndrome spans a continuum from borderline hypoplasia to extreme forms with retrograde blood flow from the ductus arteriosus to the cerebral and coronary arteries. A male neonate, born at 37 weeks’ gestation, had an antenatal diagnosis of hypoplastic left heart syndrome. Postnatal echocardiographic assessment confirmed the diagnosis of hypoplastic left heart syndrome with minimal antegrade flow in the aortic sinus, retrograde arch perfusion, and nondemonstrable ascending aorta. As a result of initial instability with recurrent infections, bilateral pulmonary artery banding was performed. Intraoperatively, a thread-like ascending aorta was found (Figure 1A), which warranted further investigation. Focused echocardiographic assessment could not visualize flow in the ascending aorta but demonstrated antegrade flow through a severely stenotic aortic valve with laminar color Doppler flow in the coronaries (Figure 2). Computed tomography angiography confirmed that the ascending aorta ended blindly above the sinotubular junction with antegrade coronary artery perfusion (Figure 3). Because of the unusual anatomy, he was deemed unsuitable for single-ventricle–type palliation. Worsening episodes of insufficient coronary perfusion with severe distress and irritability, associated with ST-segment changes and elevated troponin levels, further aggravated the course. Subsequently, he underwent heart transplantation at 2 months of age. Pathological examination of the explanted heart confirmed a blind-ending atretic ascending aorta with a relatively welldeveloped aortic root and usual coronary artery origins. In this rare anatomic anomaly, we speculate that competing blood flow antegrade through the stenotic aortic valve and retrograde through the aortic arch in fetal life created no
flow and atresia of the ascending aorta, as has been suggested previously.1,2 In our case, the Norwood-type aortic reconstruction was abandoned mainly as a result of the short height of the aortic root, which makes Damus-Kaye-Stansel anastomosis impossible without coronary artery distortion. The short height of the aortic root and the muscle structure between the great arteries would also have precluded direct implantation of the ascending aorta to the pulmonary trunk. Weidenbach et al3 reported a similar anatomy in which a neonate had the atresia of the ascending aorta with a large blind pouch of the proximal aortic root. This patient underwent the modified Norwood procedure with the Damus-Kaye-Stansel anastomosis and died of myocardial dysfunction presumably caused by poor myocardial protection. We believe that this anomaly essentially contraindicates conventional Norwood procedure and primary transplantation would be recommended unless the blind pouch is large and long enough to perform DamusKaye-Stansel anastomosis without coronary artery distortion.
Disclosures None.
References 1. Bass JL, Berry JM, Einzig S. Flow in the aorta and patent ductus arteriosus in infants with aortic atresia or aortic stenosis: a pulsed Doppler ultrasound study. Circulation. 1986;74:315–322. 2. Taber LA, Eggers DW. Theoretical study of stress-modulated growth in the aorta. J Theor Biol. 1996;180:343–357. doi: 10.1006/jtbi.1996.0107. 3. Weidenbach M, Daehnert I, Razek V, Beller A, Janousek J, Kostelka M, Anderson RH. Interruption of the ascending aorta: a hitherto undescribed lesion. Ann Thorac Surg. 2008;85:1451–1453. doi: 10.1016/j. athoracsur.2007.10.078.
From the Divisions of Cardiovascular Surgery (C.H., O.H., G.S.V.A.) and Cardiology (T.B., A.D.), The Labatt Family Heart Centre, Hospital for Sick Children, Toronto, ON, Canada; and Department of Thoracic and Cardiovascular Surgery, University Hospital Tuebingen, Germany (C.H.). Correspondence to Glen Van Arsdell, MD, Hospital for Sick Children, Division of Cardiovascular Surgery, 555 University Ave, M5G 1X8 Toronto, ON, Canada. E-mail [email protected] (Circulation. 2015;131:925-926. DOI: 10.1161/CIRCULATIONAHA.114.013069.) © 2015 American Heart Association, Inc. Circulation is available at http://circ.ahajournals.org
DOI: 10.1161/CIRCULATIONAHA.114.013069
Downloaded from http://circ.ahajournals.org/ at New York 925University/ Medical Center--New York on June 2, 2015
926 Circulation March 10, 2015
Figure 1. Intraoperative photograph and corresponding ultrasound image. A, Intraoperative findings of the thread-like ascending aorta. B, Twodimensional image showing the thread-like atretic ascending aorta in a modified parasternal long-axis view. Arrows show the atretic aortic segment. PA indicates pulmonary artery.
Figure 2. Preoperative echocardiographic images. A, Parasternal short-axis image at the aortic sinus level showing the origin of the left coronary artery. B, Color Doppler image of A demonstrating antegrade flow in the aortic root (red flow). Ao indicates aorta; LMCA, left main coronary artery; and PA, pulmonary artery.
Figure 3. Computed tomography (CT) angiography and pathological photograph. A, CT angiography confirmed that the ascending aorta ended blindly above the sinotubular junction with antegrade coronary artery perfusion. B, Gross pathology of the explanted heart showing the aortic root and coronary arteries. Asterisks indicate left and right coronary arteries. Ao indicates aorta; and LV, left ventricle.
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Atresia of the Ascending Aorta in Hypoplastic Left Heart Syndrome Christoph Haller, Osami Honjo, Timothy Bradley, Andreea Dragulescu and Glen S. Van Arsdell Circulation. 2015;131:925-926 doi: 10.1161/CIRCULATIONAHA.114.013069 Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Copyright © 2015 American Heart Association, Inc. All rights reserved. Print ISSN: 0009-7322. Online ISSN: 1524-4539
The online version of this article, along with updated information and services, is located on the World Wide Web at: http://circ.ahajournals.org/content/131/10/925
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T
he spectrum of hypoplastic left heart syndrome spans a continuum from borderline hypoplasia to extreme forms with retrograde blood flow from the ductus arteriosus to the cerebral and coronary arteries. A male neonate, born at 37 weeks’ gestation, had an antenatal diagnosis of hypoplastic left heart syndrome. Postnatal echocardiographic assessment confirmed the diagnosis of hypoplastic left heart syndrome with minimal antegrade flow in the aortic sinus, retrograde arch perfusion, and nondemonstrable ascending aorta. As a result of initial instability with recurrent infections, bilateral pulmonary artery banding was performed. Intraoperatively, a thread-like ascending aorta was found (Figure 1A), which warranted further investigation. Focused echocardiographic assessment could not visualize flow in the ascending aorta but demonstrated antegrade flow through a severely stenotic aortic valve with laminar color Doppler flow in the coronaries (Figure 2). Computed tomography angiography confirmed that the ascending aorta ended blindly above the sinotubular junction with antegrade coronary artery perfusion (Figure 3). Because of the unusual anatomy, he was deemed unsuitable for single-ventricle–type palliation. Worsening episodes of insufficient coronary perfusion with severe distress and irritability, associated with ST-segment changes and elevated troponin levels, further aggravated the course. Subsequently, he underwent heart transplantation at 2 months of age. Pathological examination of the explanted heart confirmed a blind-ending atretic ascending aorta with a relatively welldeveloped aortic root and usual coronary artery origins. In this rare anatomic anomaly, we speculate that competing blood flow antegrade through the stenotic aortic valve and retrograde through the aortic arch in fetal life created no
flow and atresia of the ascending aorta, as has been suggested previously.1,2 In our case, the Norwood-type aortic reconstruction was abandoned mainly as a result of the short height of the aortic root, which makes Damus-Kaye-Stansel anastomosis impossible without coronary artery distortion. The short height of the aortic root and the muscle structure between the great arteries would also have precluded direct implantation of the ascending aorta to the pulmonary trunk. Weidenbach et al3 reported a similar anatomy in which a neonate had the atresia of the ascending aorta with a large blind pouch of the proximal aortic root. This patient underwent the modified Norwood procedure with the Damus-Kaye-Stansel anastomosis and died of myocardial dysfunction presumably caused by poor myocardial protection. We believe that this anomaly essentially contraindicates conventional Norwood procedure and primary transplantation would be recommended unless the blind pouch is large and long enough to perform DamusKaye-Stansel anastomosis without coronary artery distortion.
Disclosures None.
References 1. Bass JL, Berry JM, Einzig S. Flow in the aorta and patent ductus arteriosus in infants with aortic atresia or aortic stenosis: a pulsed Doppler ultrasound study. Circulation. 1986;74:315–322. 2. Taber LA, Eggers DW. Theoretical study of stress-modulated growth in the aorta. J Theor Biol. 1996;180:343–357. doi: 10.1006/jtbi.1996.0107. 3. Weidenbach M, Daehnert I, Razek V, Beller A, Janousek J, Kostelka M, Anderson RH. Interruption of the ascending aorta: a hitherto undescribed lesion. Ann Thorac Surg. 2008;85:1451–1453. doi: 10.1016/j. athoracsur.2007.10.078.
From the Divisions of Cardiovascular Surgery (C.H., O.H., G.S.V.A.) and Cardiology (T.B., A.D.), The Labatt Family Heart Centre, Hospital for Sick Children, Toronto, ON, Canada; and Department of Thoracic and Cardiovascular Surgery, University Hospital Tuebingen, Germany (C.H.). Correspondence to Glen Van Arsdell, MD, Hospital for Sick Children, Division of Cardiovascular Surgery, 555 University Ave, M5G 1X8 Toronto, ON, Canada. E-mail [email protected] (Circulation. 2015;131:925-926. DOI: 10.1161/CIRCULATIONAHA.114.013069.) © 2015 American Heart Association, Inc. Circulation is available at http://circ.ahajournals.org
DOI: 10.1161/CIRCULATIONAHA.114.013069
Downloaded from http://circ.ahajournals.org/ at New York 925University/ Medical Center--New York on June 2, 2015
926 Circulation March 10, 2015
Figure 1. Intraoperative photograph and corresponding ultrasound image. A, Intraoperative findings of the thread-like ascending aorta. B, Twodimensional image showing the thread-like atretic ascending aorta in a modified parasternal long-axis view. Arrows show the atretic aortic segment. PA indicates pulmonary artery.
Figure 2. Preoperative echocardiographic images. A, Parasternal short-axis image at the aortic sinus level showing the origin of the left coronary artery. B, Color Doppler image of A demonstrating antegrade flow in the aortic root (red flow). Ao indicates aorta; LMCA, left main coronary artery; and PA, pulmonary artery.
Figure 3. Computed tomography (CT) angiography and pathological photograph. A, CT angiography confirmed that the ascending aorta ended blindly above the sinotubular junction with antegrade coronary artery perfusion. B, Gross pathology of the explanted heart showing the aortic root and coronary arteries. Asterisks indicate left and right coronary arteries. Ao indicates aorta; and LV, left ventricle.
Downloaded from http://circ.ahajournals.org/ at New York University/ Medical Center--New York on June 2, 2015
Atresia of the Ascending Aorta in Hypoplastic Left Heart Syndrome Christoph Haller, Osami Honjo, Timothy Bradley, Andreea Dragulescu and Glen S. Van Arsdell Circulation. 2015;131:925-926 doi: 10.1161/CIRCULATIONAHA.114.013069 Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Copyright © 2015 American Heart Association, Inc. All rights reserved. Print ISSN: 0009-7322. Online ISSN: 1524-4539
The online version of this article, along with updated information and services, is located on the World Wide Web at: http://circ.ahajournals.org/content/131/10/925
Permissions: Requests for permissions to reproduce figures, tables, or portions of articles originally published in Circulation can be obtained via RightsLink, a service of the Copyright Clearance Center, not the Editorial Office. Once the online version of the published article for which permission is being requested is located, click Request Permissions in the middle column of the Web page under Services. Further information about this process is available in the Permissions and Rights Question and Answer document. Reprints: Information about reprints can be found online at: http://www.lww.com/reprints Subscriptions: Information about subscribing to Circulation is online at: http://circ.ahajournals.org//subscriptions/
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