Case Report
Childhood Presentation of Interrupted Aortic Arch With Persistent Carotid Ducts
World Journal for Pediatric and Congenital Heart Surgery 2015, Vol. 6(2) 335-338 ª The Author(s) 2014 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135114560830 pch.sagepub.com
Somjate Suntratonpipat, MD1, Simon D. Bamforth, PhD2, Amy-Leigh Johnson, PhD2, Michelle Noga, MD, FRCP3, Robert H. Anderson, MD, PhD, FRCPath2, Jeffrey Smallhorn, MBBS, FRACP, FRCP(C)1, and Edythe Tham, MBBS, FRACP1
Abstract Interrupted aortic arch is a rare condition with typical presentation within the first few weeks of life, as the circulation is dependent upon patency of the arterial duct. Most cases are associated with intracardiac anomalies, the most common being a ventricular septal defect with some degree of hypoplasia and/or obstruction of the left ventricular outflow tract. Presentation beyond infancy is uncommon, and suggests the presence of well-developed collateral circulation. This case of childhood presentation of interrupted aortic arch and intact ventricular septum highlights the very unusual finding of bilateral collateral arteries consistent with persistent carotid ducts. Cardiac MRI angiography with three-dimensional reconstruction defined not only the site of interruption in the aortic arch but also the entire collateral circulation. Keywords interrupted aortic arch, carotid duct, Digeorge syndrome Submitted July 24, 2014; Accepted October 22, 2014.
A six-year-old Mexican boy presented with a systolic click, which was heard by his family physician. He was generally healthy but did tire earlier than his peers, with some exercise limitations. There was no family history of congenital heart disease or sudden unexpected death. On examination, he was a well-grown boy in no distress with dysmorphic features of narrow palpebral fissures, short philtrum, and a downturned mouth. His weight was 25.3 kg (75th percentile), height was 114 cm (10th-25th percentile), and heart rate was 88 beats/ min. Blood pressure in his right arm was 115/53 mm Hg, left arm was 66/37 mm Hg, right leg was 63/16 mm Hg, left leg was 62/22 mm Hg, and peripheral capillary oxygen saturation (SpO2) was 96% in room air. There were no heaves or thrills, normal heart sounds with an ejection click, and a soft II/VI murmur at the apex and lower left sternal border. Radial pulses were normal, but his femoral pulses were weak with no radiofemoral delay. An electrocardiogram showed normal sinus rhythm with a rate of 88 beats/min and right ventricular hypertrophy. An echocardiogram demonstrated a bicuspid aortic valve with no stenosis. The ventricular septum was intact, and there was no evidence of obstruction in the left ventricular outflow tract. An obstructive pattern was observed, nonetheless, in the abdominal aorta and there was interruption of the aortic arch (IAA), although the route of supply to the descending aorta
could not be defined by echocardiography. The left ventricular function was normal. A cardiac magnetic resonance imaging (MRI) study was performed with a 1.5T Siemens Aera scanner (Siemens Healthcare Sector). This revealed that the ascending aorta gave rise only to right and left common carotid arteries, with the right subclavian artery having an aberrant cervical origin. The transverse aortic arch was interrupted between the origins of the left common carotid and the left subclavian artery, the overall pattern being consistent with interrupted aortic arch type B (Figures 1 and 2). Bilateral collateral vessels were seen arising from the bifurcation of the common carotid arteries.
1
Stollery Children’s Hospital, University of Alberta, Edmonton, Canada Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, United Kingdom 3 Department of Radiology and Diagnostic Imaging, University of Alberta, Edmonton, Canada 2
Corresponding Author: Edythe Tham, MBBS, Stollery Children’s Hospital, University of Alberta Division of Pediatric Cardiology and Diagnostic Imaging, 4C2.28 WCM Health Sciences Centre, 8440 112th Street, Edmonton, Alberta T6G 2B7 Canada. Email: [email protected]
Downloaded from pch.sagepub.com at Bobst Library, New York University on May 30, 2015
336
World Journal for Pediatric and Congenital Heart Surgery 6(2)
Left Carotid Duct
Right Carotid Duct Right Common Carotid
Left Carotid Duct Left Common Carotid
Right Subclavian
Left Subclavian
Ascending Aorta
Mild Arch Narrowing
Interrupted Aortic Arch
Left Common Carotid Left Vertebral Left Subclavian
Mild Arch Narrowing Descending Aorta
Right Common Carotid Right Vertebral Right Subclavian
Ascending Aorta Interrupted Aortic Arch
Descending Aorta
Figure 1. Cardiac magnetic resonance imaging (MRI) with threedimensional (3D) reconstruction of the thoracic aorta, and the major branching anterior view showed the right and left common carotid arteries with bilateral carotid ducts.
They coursed posteriorly, with the right-sided collateral artery giving rise to the right subclavian artery and right vertebral artery and the left collateral artery supplying the descending aorta and the left subclavian artery. There was also some mild narrowing of the descending aorta. In addition, there were bilateral superior caval veins, with the left one draining to the coronary sinus in presence of a small bridging vein. The pulmonary trunk and arteries were normal as were the left ventricular volumes, masses, and systolic function. Surgical correction was undertaken using cardiopulmonary bypass established through a distal aortic and a single venous cannula. The patient was cooled to 24 C and, after 20 minutes of cooling, the bypass flow was reduced to 50% and isolated to the right carotid artery while the heart was arrested with cold dilute blood cardioplegia. The descending aorta was opened at the level of the arterial ligament, and the opening extended distally and proximally for a distance of about 2 cm in total. A matching incision was made along the left posterolateral aspect of the distal part of the ascending aorta, and a direct side-to-side anastomosis was completed using a combination of interrupted and continuous suture. No attempt was made to perform surgery on the collateral vessels. During the surgical procedure, we found no evidence of the thymus gland, while genetic testing revealed 22q11 deletion, confirming the diagnosis of DiGeorge syndrome. Interruption of the aortic arch is a rare condition that has an estimated incidence of three per million live births.1 The discontinuity of the transverse aortic arch occurs in three patterns. In the so-called type A defect, the interruption is at the isthmus, in other words between the proximal descending aorta and the left subclavian artery. In type B, the commonest variant, it is between the left subclavian and left common carotid arteries. In type C, by far the rarest, the interruption is between the left and the right common carotid arteries. Typical presentation is within the first few weeks of life after ductal closure, resulting in poor perfusion, hypotension, metabolic acidosis,
Figure 2. Cardiac magnetic resonance imaging (MRI) with threedimensional (3D) reconstruction posterior view showed the remnant carotid duct bilaterally.
and shock, requiring prostaglandin infusion to maintain ductal patency until surgical repair. Occasionally, however, patients with interrupted arches can present in childhood or adulthood,2-4 with systemic hypertension, claudication, pulmonary arterial hypertension, differential cyanosis, or sudden collapse. Patients can survive depending on persistent ductal patency, or in rare cases, as in our patient, due to the presence of collateral channels. In most instances, IAA type B is associated with a malalignment-type ventricular septal defect and subaortic obstruction. Here, we found only a bicuspid aortic valve, with no other intracardiac abnormalities. We interpreted the pattern on the basis of bilateral interruption of the fourth pharyngeal arch (aortic arch) arteries (PAAs), coupled with persistence bilaterally of the carotid ducts, with the latter structures providing the collateral channels as revealed in the cardiac MRI (Figure 3). During normal development of the pharyngeal arch arteries, the right-sided artery extending through the fourth pharyngeal arch will form the proximal component of the right subclavian artery, with the distal artery itself derived from the right seventh cervical intersegmental artery. The artery running through the left fourth pharyngeal arch will normally form the transverse aortic arch. The common carotid arteries are formed from the arteries running through the third pharyngeal arches. In normal circumstances, there is regression of the part of the dorsal aorta that joins together the arteries of the third and fourth pharyngeal arches. It is this part that is known as the carotid duct (ductus caroticus). With regression of these dorsal components, it is the more cranial aspect of the remaining dorsal aortas that form the internal carotid arteries (Figure 3A-C). In our case, we propose that the first branch of the ascending aorta is the derivative of the right third PAA, which extends cranially before giving rise to the internal and external carotid arteries. The vessel then turns through almost 180 and extends caudally, with the dorsal component derived from the persisting segment of the right dorsal aorta representing the carotid duct. This channel, in turn, connects with
Downloaded from pch.sagepub.com at Bobst Library, New York University on May 30, 2015
Suntratonpipat et al
337
Figure 3. Schematic representation of normal and abnormal development of the pharyngeal arch arteries (PAAs). A-C, Normal PAA development. The initially bilaterally symmetrical PAAs (A) undergo a remodeling process (B) resulting in the asymmetrical aortic arch arteries seen in the adult (C). The left fourth PAA develops into the transverse aortic arch, and the right fourth PAA develops into the proximal right subclavian artery. The section of dorsal aorta between the third and fourth PAA, the carotid duct, involutes. D-F, Abnormal PAA development that results in the phenotype seen in the patient. Bilateral absence of the fourth PAA (D) and persistence of the carotid duct (E) leads to an interruption of the aortic arch type B (IAA-B), but systemic circulation is maintained via the carotid arteries and persistence of the carotid ducts. PAA indicates pharyngeal arch artery; cd, carotid duct; Ao, aorta; PT, pulmonary trunk; rcc, right common carotid; rsa, right subclavian artery; lcc, left common carotid; aoa, aortic arch; lsa, left subclavian artery; iaa, interrupted aortic arch.
the right subclavian artery, formed from the right seventh cervical intersegmental artery. The right vertebral artery is also seen arising from this vessel. The second branch from the ascending aorta is the presumed derivative of the left third PAA, which also extends cranially before giving rise to the internal and external carotid arteries. This vessel also then turns through almost 180 and extends caudally, with the abnormal channel created by the persisting carotid duct connecting with the left dorsal aorta. This then continues caudally as the left-sided descending aorta. This vessel also gives rise to the left vertebral artery, and the left subclavian artery (Figure 3D-F). Consideration might be given to interpreting the findings on the basis of formation of a cervical aortic arch. The cervical aortic arch, however, is formed when the fourth PAA become atretic, with the third PAAs then becoming the definitive arch, with separate internal and external carotid arteries arising from the arch. Such cervical arches are found above the level of the clavicle and can be as high as the level of the second cervical vertebra body. Clinical presentation in this setting would typically include a pulsatile mass in the neck, a feature not observed in our patient.
Deletion within chromosome 22q11 is now well recognized as being associated with outflow tract malformations, including tetralogy of Fallot with or without pulmonary atresia, common arterial trunk, and interruption of the aortic arch. Significant associations also exist with isolated anomalies of aortic arch, including cervical aortic arch, double aortic arch, right aortic arch with aberrant left subclavian artery, right aortic arch with mirror-image branching, and vascular rings.5 Our case is unusual not only in the pattern of the collateral channels, which we consider to represent carotid ducts, but also in the relative normality of the intracardiac anatomy. When IAA presents beyond the neonatal period, it is usually accompanied by adequate collateral circulation. In our patient, the arteries normally found in the fourth arches have failed to form, the arteries of the third arch have persisted as the common carotid arteries, and the subclavian arteries are fed through bilaterally persisting carotid ducts, the leftsided carotid duct continuing as the descending aorta. Cardiac MRI angiography using three-dimensional reconstruction permitted us to define not only the site of interruption in the aortic arch but also the entire collateral circulation.
Downloaded from pch.sagepub.com at Bobst Library, New York University on May 30, 2015
338
World Journal for Pediatric and Congenital Heart Surgery 6(2)
Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. John AS, Schaff HV, Drew T. Adult Presentation of Interrupted Aortic Arch: Case Presentation and a Review of the Medical Literature. Congenital Heart Dis. 2011;6(3): 269-275.
2. Celebi A, Yalcin Y, Polat TB, et al, Late presentation of interrupted aortic arch in childhood. Pediatr Int. 2009;51(1): 152-154. 3. Gordon EA, Person T, Kavarana M. Interrupted aortic arch in the adult. J Card Surg. 2011;26(4): 405-409. 4. Dillman JR, Yarram SG, D’Amico AR, Hernandez RJ. Interrupted aortic arch: spectrum of MRI findings. AJR Am J Roentgenol. 2008;190(6): 1467-1474. 5. McElhinney DB, Clark BJ, Weinberg PM. Association of chromosome 22q11 deletion with isolated anomalies of aortic arch laterality and branching. J Am Coll Cardiol. 2001;37(8): 2114-2119.
Downloaded from pch.sagepub.com at Bobst Library, New York University on May 30, 2015
Childhood Presentation of Interrupted Aortic Arch With Persistent Carotid Ducts
World Journal for Pediatric and Congenital Heart Surgery 2015, Vol. 6(2) 335-338 ª The Author(s) 2014 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135114560830 pch.sagepub.com
Somjate Suntratonpipat, MD1, Simon D. Bamforth, PhD2, Amy-Leigh Johnson, PhD2, Michelle Noga, MD, FRCP3, Robert H. Anderson, MD, PhD, FRCPath2, Jeffrey Smallhorn, MBBS, FRACP, FRCP(C)1, and Edythe Tham, MBBS, FRACP1
Abstract Interrupted aortic arch is a rare condition with typical presentation within the first few weeks of life, as the circulation is dependent upon patency of the arterial duct. Most cases are associated with intracardiac anomalies, the most common being a ventricular septal defect with some degree of hypoplasia and/or obstruction of the left ventricular outflow tract. Presentation beyond infancy is uncommon, and suggests the presence of well-developed collateral circulation. This case of childhood presentation of interrupted aortic arch and intact ventricular septum highlights the very unusual finding of bilateral collateral arteries consistent with persistent carotid ducts. Cardiac MRI angiography with three-dimensional reconstruction defined not only the site of interruption in the aortic arch but also the entire collateral circulation. Keywords interrupted aortic arch, carotid duct, Digeorge syndrome Submitted July 24, 2014; Accepted October 22, 2014.
A six-year-old Mexican boy presented with a systolic click, which was heard by his family physician. He was generally healthy but did tire earlier than his peers, with some exercise limitations. There was no family history of congenital heart disease or sudden unexpected death. On examination, he was a well-grown boy in no distress with dysmorphic features of narrow palpebral fissures, short philtrum, and a downturned mouth. His weight was 25.3 kg (75th percentile), height was 114 cm (10th-25th percentile), and heart rate was 88 beats/ min. Blood pressure in his right arm was 115/53 mm Hg, left arm was 66/37 mm Hg, right leg was 63/16 mm Hg, left leg was 62/22 mm Hg, and peripheral capillary oxygen saturation (SpO2) was 96% in room air. There were no heaves or thrills, normal heart sounds with an ejection click, and a soft II/VI murmur at the apex and lower left sternal border. Radial pulses were normal, but his femoral pulses were weak with no radiofemoral delay. An electrocardiogram showed normal sinus rhythm with a rate of 88 beats/min and right ventricular hypertrophy. An echocardiogram demonstrated a bicuspid aortic valve with no stenosis. The ventricular septum was intact, and there was no evidence of obstruction in the left ventricular outflow tract. An obstructive pattern was observed, nonetheless, in the abdominal aorta and there was interruption of the aortic arch (IAA), although the route of supply to the descending aorta
could not be defined by echocardiography. The left ventricular function was normal. A cardiac magnetic resonance imaging (MRI) study was performed with a 1.5T Siemens Aera scanner (Siemens Healthcare Sector). This revealed that the ascending aorta gave rise only to right and left common carotid arteries, with the right subclavian artery having an aberrant cervical origin. The transverse aortic arch was interrupted between the origins of the left common carotid and the left subclavian artery, the overall pattern being consistent with interrupted aortic arch type B (Figures 1 and 2). Bilateral collateral vessels were seen arising from the bifurcation of the common carotid arteries.
1
Stollery Children’s Hospital, University of Alberta, Edmonton, Canada Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, United Kingdom 3 Department of Radiology and Diagnostic Imaging, University of Alberta, Edmonton, Canada 2
Corresponding Author: Edythe Tham, MBBS, Stollery Children’s Hospital, University of Alberta Division of Pediatric Cardiology and Diagnostic Imaging, 4C2.28 WCM Health Sciences Centre, 8440 112th Street, Edmonton, Alberta T6G 2B7 Canada. Email: [email protected]
Downloaded from pch.sagepub.com at Bobst Library, New York University on May 30, 2015
336
World Journal for Pediatric and Congenital Heart Surgery 6(2)
Left Carotid Duct
Right Carotid Duct Right Common Carotid
Left Carotid Duct Left Common Carotid
Right Subclavian
Left Subclavian
Ascending Aorta
Mild Arch Narrowing
Interrupted Aortic Arch
Left Common Carotid Left Vertebral Left Subclavian
Mild Arch Narrowing Descending Aorta
Right Common Carotid Right Vertebral Right Subclavian
Ascending Aorta Interrupted Aortic Arch
Descending Aorta
Figure 1. Cardiac magnetic resonance imaging (MRI) with threedimensional (3D) reconstruction of the thoracic aorta, and the major branching anterior view showed the right and left common carotid arteries with bilateral carotid ducts.
They coursed posteriorly, with the right-sided collateral artery giving rise to the right subclavian artery and right vertebral artery and the left collateral artery supplying the descending aorta and the left subclavian artery. There was also some mild narrowing of the descending aorta. In addition, there were bilateral superior caval veins, with the left one draining to the coronary sinus in presence of a small bridging vein. The pulmonary trunk and arteries were normal as were the left ventricular volumes, masses, and systolic function. Surgical correction was undertaken using cardiopulmonary bypass established through a distal aortic and a single venous cannula. The patient was cooled to 24 C and, after 20 minutes of cooling, the bypass flow was reduced to 50% and isolated to the right carotid artery while the heart was arrested with cold dilute blood cardioplegia. The descending aorta was opened at the level of the arterial ligament, and the opening extended distally and proximally for a distance of about 2 cm in total. A matching incision was made along the left posterolateral aspect of the distal part of the ascending aorta, and a direct side-to-side anastomosis was completed using a combination of interrupted and continuous suture. No attempt was made to perform surgery on the collateral vessels. During the surgical procedure, we found no evidence of the thymus gland, while genetic testing revealed 22q11 deletion, confirming the diagnosis of DiGeorge syndrome. Interruption of the aortic arch is a rare condition that has an estimated incidence of three per million live births.1 The discontinuity of the transverse aortic arch occurs in three patterns. In the so-called type A defect, the interruption is at the isthmus, in other words between the proximal descending aorta and the left subclavian artery. In type B, the commonest variant, it is between the left subclavian and left common carotid arteries. In type C, by far the rarest, the interruption is between the left and the right common carotid arteries. Typical presentation is within the first few weeks of life after ductal closure, resulting in poor perfusion, hypotension, metabolic acidosis,
Figure 2. Cardiac magnetic resonance imaging (MRI) with threedimensional (3D) reconstruction posterior view showed the remnant carotid duct bilaterally.
and shock, requiring prostaglandin infusion to maintain ductal patency until surgical repair. Occasionally, however, patients with interrupted arches can present in childhood or adulthood,2-4 with systemic hypertension, claudication, pulmonary arterial hypertension, differential cyanosis, or sudden collapse. Patients can survive depending on persistent ductal patency, or in rare cases, as in our patient, due to the presence of collateral channels. In most instances, IAA type B is associated with a malalignment-type ventricular septal defect and subaortic obstruction. Here, we found only a bicuspid aortic valve, with no other intracardiac abnormalities. We interpreted the pattern on the basis of bilateral interruption of the fourth pharyngeal arch (aortic arch) arteries (PAAs), coupled with persistence bilaterally of the carotid ducts, with the latter structures providing the collateral channels as revealed in the cardiac MRI (Figure 3). During normal development of the pharyngeal arch arteries, the right-sided artery extending through the fourth pharyngeal arch will form the proximal component of the right subclavian artery, with the distal artery itself derived from the right seventh cervical intersegmental artery. The artery running through the left fourth pharyngeal arch will normally form the transverse aortic arch. The common carotid arteries are formed from the arteries running through the third pharyngeal arches. In normal circumstances, there is regression of the part of the dorsal aorta that joins together the arteries of the third and fourth pharyngeal arches. It is this part that is known as the carotid duct (ductus caroticus). With regression of these dorsal components, it is the more cranial aspect of the remaining dorsal aortas that form the internal carotid arteries (Figure 3A-C). In our case, we propose that the first branch of the ascending aorta is the derivative of the right third PAA, which extends cranially before giving rise to the internal and external carotid arteries. The vessel then turns through almost 180 and extends caudally, with the dorsal component derived from the persisting segment of the right dorsal aorta representing the carotid duct. This channel, in turn, connects with
Downloaded from pch.sagepub.com at Bobst Library, New York University on May 30, 2015
Suntratonpipat et al
337
Figure 3. Schematic representation of normal and abnormal development of the pharyngeal arch arteries (PAAs). A-C, Normal PAA development. The initially bilaterally symmetrical PAAs (A) undergo a remodeling process (B) resulting in the asymmetrical aortic arch arteries seen in the adult (C). The left fourth PAA develops into the transverse aortic arch, and the right fourth PAA develops into the proximal right subclavian artery. The section of dorsal aorta between the third and fourth PAA, the carotid duct, involutes. D-F, Abnormal PAA development that results in the phenotype seen in the patient. Bilateral absence of the fourth PAA (D) and persistence of the carotid duct (E) leads to an interruption of the aortic arch type B (IAA-B), but systemic circulation is maintained via the carotid arteries and persistence of the carotid ducts. PAA indicates pharyngeal arch artery; cd, carotid duct; Ao, aorta; PT, pulmonary trunk; rcc, right common carotid; rsa, right subclavian artery; lcc, left common carotid; aoa, aortic arch; lsa, left subclavian artery; iaa, interrupted aortic arch.
the right subclavian artery, formed from the right seventh cervical intersegmental artery. The right vertebral artery is also seen arising from this vessel. The second branch from the ascending aorta is the presumed derivative of the left third PAA, which also extends cranially before giving rise to the internal and external carotid arteries. This vessel also then turns through almost 180 and extends caudally, with the abnormal channel created by the persisting carotid duct connecting with the left dorsal aorta. This then continues caudally as the left-sided descending aorta. This vessel also gives rise to the left vertebral artery, and the left subclavian artery (Figure 3D-F). Consideration might be given to interpreting the findings on the basis of formation of a cervical aortic arch. The cervical aortic arch, however, is formed when the fourth PAA become atretic, with the third PAAs then becoming the definitive arch, with separate internal and external carotid arteries arising from the arch. Such cervical arches are found above the level of the clavicle and can be as high as the level of the second cervical vertebra body. Clinical presentation in this setting would typically include a pulsatile mass in the neck, a feature not observed in our patient.
Deletion within chromosome 22q11 is now well recognized as being associated with outflow tract malformations, including tetralogy of Fallot with or without pulmonary atresia, common arterial trunk, and interruption of the aortic arch. Significant associations also exist with isolated anomalies of aortic arch, including cervical aortic arch, double aortic arch, right aortic arch with aberrant left subclavian artery, right aortic arch with mirror-image branching, and vascular rings.5 Our case is unusual not only in the pattern of the collateral channels, which we consider to represent carotid ducts, but also in the relative normality of the intracardiac anatomy. When IAA presents beyond the neonatal period, it is usually accompanied by adequate collateral circulation. In our patient, the arteries normally found in the fourth arches have failed to form, the arteries of the third arch have persisted as the common carotid arteries, and the subclavian arteries are fed through bilaterally persisting carotid ducts, the leftsided carotid duct continuing as the descending aorta. Cardiac MRI angiography using three-dimensional reconstruction permitted us to define not only the site of interruption in the aortic arch but also the entire collateral circulation.
Downloaded from pch.sagepub.com at Bobst Library, New York University on May 30, 2015
338
World Journal for Pediatric and Congenital Heart Surgery 6(2)
Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. John AS, Schaff HV, Drew T. Adult Presentation of Interrupted Aortic Arch: Case Presentation and a Review of the Medical Literature. Congenital Heart Dis. 2011;6(3): 269-275.
2. Celebi A, Yalcin Y, Polat TB, et al, Late presentation of interrupted aortic arch in childhood. Pediatr Int. 2009;51(1): 152-154. 3. Gordon EA, Person T, Kavarana M. Interrupted aortic arch in the adult. J Card Surg. 2011;26(4): 405-409. 4. Dillman JR, Yarram SG, D’Amico AR, Hernandez RJ. Interrupted aortic arch: spectrum of MRI findings. AJR Am J Roentgenol. 2008;190(6): 1467-1474. 5. McElhinney DB, Clark BJ, Weinberg PM. Association of chromosome 22q11 deletion with isolated anomalies of aortic arch laterality and branching. J Am Coll Cardiol. 2001;37(8): 2114-2119.
Downloaded from pch.sagepub.com at Bobst Library, New York University on May 30, 2015
