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Type A interrupted aortic arch accompanied by intracranial aneurysms causing subarachnoid hemorrhage in an adult man
Clinical Imaging 38 (2014) 60–62
Contents lists available at ScienceDirect
Clinical Imaging journal homepage: http://www.clinicalimaging.org
Case Reports
Type A interrupted aortic arch accompanied by intracranial aneurysms causing subarachnoid hemorrhage in an adult man☆ Suat Eren a,⁎, Mecit Kantarci a, Berhan Pirimoglu a, Murteza Cakir b, Hayri Ogul a a b
Ataturk University, School of Medicine, Department of Radiology, Erzurum, Turkey Ataturk University, School of Medicine, Department of Neurosurgery, Erzurum, Turkey
a r t i c l e
i n f o
Article history: Received 3 January 2013 Received in revised form 7 April 2013 Accepted 9 May 2013 Keywords: Interrupted aortic arch Subarachnoid hemorrhage Multidetector computed tomography angiography Digital subtraction angiography
a b s t r a c t Interrupted aortic arch anomaly (IAA) characterized by complete luminal dissociation between the ascending and descending aorta and accounting for less than 1% of all cases of congenital heart disease. IAA is a rare condition in infants that occurs approximately three times per million births. It is usually diagnosed and repaired during the neonatal period and is extremely rare in adults. We present the case of an adult man who was diagnosed with IAA accompanied by intracranial aneurysms causing subarachnoid hemorrhage and demonstrate the imaging findings with 256-slice computed tomography angiography and digital subtraction angiography.
1. Introductıon Interrupted aortic arch, characterized by luminal and anatomic discontinuity between the ascending and descending aorta, is a very rare congenital malformation. The condition is typically diagnosed in neonates and is highly fatal if left untreated [1]. The anomaly was first described by the Viennese surgeon Steidele in 1778 [2], with a prevalence as low as 3 in 1,000,000 live births [3]. Defined as a loss of luminal continuity between the ascending and descending portions of the aorta, this anomaly entails a very poor prognosis without surgical treatment [4]. We reported an unusual diagnosis of a Type A interrupted aortic arch anomaly (IAA) and intracranial aneursyms causing subarachnoid hemorrhage in a 20-year-old hypertensive man. 2. Case report A 20-year-old man was referred to our emergency service with suddenly onset of severe headache and hypertension. The patient had no other significant medical history. In our emergency service, noncontrast enhanced brain computed tomography was performed, and subarachnoid hemorrhage was detected. Digital substraction angiography (DSA) examination was performed immediately afterwards to reveal if there was an intracranial vascular pathology. During ☆ Conflict of Interest: Authors declared that there was no conflict of interest. ⁎ Corresponding author. Ataturk University, School of Medicine, Department of Radiology, Erzurum, Turkey. Tel.: +90-4422316908. E-mail address: [email protected] (S. Eren). 0899-7071/$ – see front matter © 2014 Published by Elsevier Inc. http://dx.doi.org/10.1016/j.clinimag.2013.05.012
© 2014 Published by Elsevier Inc.
the DSA examination, femoral artery pulses were found extremely weak, and catheter did not pass beyond the descending aorta. The descending aorta had a blunt ending (Fig. 1). The arterial blood pressure was 165/95 mmHg in both arms and 80/60 mmHg in the lower extremities. The pulses were equal over both upper extremities. To elucidate the aortic and intracranial arterial anatomy, 256-slice multidetector computed tomography (MDCT) angiography (Somatom High Definition, Siemens) was performed (Fig. 2). Axial, sagittal, and volume-rendered three-dimensional reconstruction images clearly revealed a complete interruption in the aortic arch, approximately 20 mm beyond the origin of the left subclavian artery. Extensive collateral vessels were coursing to interrupted aortic region from the intercostal, paravertebral, and internal mammary arteries. Furthermore, we detected three intracranial aneurysms localized at anterior cerebral artery, at the origin of the left posterior cerebellar artery, and at the M1 division of the left middle cerebral artery ranging diameters of 2 to 5 mm (Fig. 3). Bleeding aneurysm at the M1 division was in irregular shape due to partial thrombosis. Two renal arteries were also seen on the left side. The patient was treated in another center with resection and end-to-end anastomosis of aorta and clipping of intracranial aneurysms by open surgery. The patient is on follow-up. 3. Discussion IAA is congenital absence of anatomic and luminal continuity between the ascending and descending portions of the aorta [4,5]. The first classification system of interrupted aortic arch, introduced by Celoria and Patton in 1959, is still used almost universally. It describes and classifies the site of aortic arch discontinuity, which may be distal
S. Eren et al. / Clinical Imaging 38 (2014) 60–62
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Fig. 3. Three-dimensional volume-rendered image shows three intracranial aneurysms localized at anterior cerebral artery, at the origin of the left posterior cerebellar artery, and at the M1 division of the left middle cerebral artery (white arrows).
Fig. 1. DSA Aortogram acquired by right femoral arterial puncture shows the blunt ending descending aorta. Note the feeding artery on the right side of the descending aorta (arrow).
to the left subclavian artery (Type A), between the left carotid and left subclavian arteries (type B) or between the innominate and left carotid arteries (Type C). The most common type is B (53%), followed by A (43%) and C (4%) [6]. Interrupted aortic arch is associated with additional cardiovascular anomalies, including ventricular septal defect, patent ductus arteriosus, bicuspid aortic valve, left ventricular
outflow tract obstruction, aortopulmonary window, and truncus arteriosus [7]. IAA can be diagnosed by echocardiography, MDCT, magnetic resonance imaging and DSA. The combination of MDCT angiography and DSA enable definitive diagnosis of interrupted aortic arch and associated abnormalities [7,8], as in our patient. In adults, the presentation of IAA ranges from lack of symptoms to limb swelling with differential blood pressures in all extremities. Blood stream of the descending aorta usually is supplied by the intercostal, internal mammary, or paravertebral collaterals, as shown in our patient. Substantial collateral circulation must be present to maintain flow and enable survival [7]. In our case, high blood pressure was presented in both arms. Seventy percent of patients have hypertension refractory to medical management as their presenting symptom. Other common presenting symptoms are claudication, congestive heart failure, and aortic insufficiency [9]. Life-threatening complications are present at initial presentation in 13% of patients; these included coronary artery disease, intracranial hemorrhage, and biventricular heart failure [10,11]. Our patient had hypertension refractory to medical management and life-threatening intracranial aneurysm and hemorrhage. Surgical intervention is necessary to both improve the patient's symptomatology and to prevent potentially fatal sequel [11]. McCrindle et al. reported that the overall survival at 16 years after study entry was found to be 59% in patients with interrupted aortic arch; the survival rate increased to approximately 70% in patients who had undergone preoperative therapy and appropriate surgical techniques [8]. In conclusion, IAA is rarely encountered in adults. MDCT angiography appears to be a useful diagnostic imaging modality in patients with this congenital anomaly. The accurate and early diagnosis of interrupted aortic arch and its associated anomalies is profoundly important for the patient's survival. References
Fig. 2. Multislice computed tomography angiography image in oblique sagittal shows Type A interrupted aortic arch (black arrow), paravertebral collateral vessels (arrowhead), and intracranial aneurysm at the left middle cerebral artery (white arrow). Note the proximal side of the interrupted aortic arch shows more contrast enhancement than distal side.
[1] Bugan B, Iyisoy A, Celik M, et al. Isolated type A interrupted aortic arch in an asymptomatic 19-year-old man. Tex Heart Inst J 2011;38(5):559–61. [2] Steidele RJ. Sammlung verschiedener in der chirurgischpraktischen lehrschule gemachten beobachtungen 2. Vienna:Trattern, 1778; p. 114. [3] Dillman JR, Yarram SG, D’Amico AR, Hernandez RJ. Interrupted aortic arch: spectrum of MRI findings. AJR Am J Roentgenol 2008;190(6):1467–74. [4] Mishra PK. Management strategies for interrupted aortic arch with associated anomalies. Eur J Cardiothorac Surg 2009;35(4):569–76. [5] Gordon AE, Person T, Kavarana M, et al. Interrupted aortic arch in the adult. J Card Surg 2011;26:405–9.
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[6] Celoria GC, Patton RB. Congenital absence of the aortic arch. Am Heart J 1959;58: 407–13. [7] Messner G, Reul GJ, Flamm SD, et al. Interrupted aortic arch in an adult singlestage extra-anatomic repair. Tex Heart Inst J 2002;29:118–21. [8] McCrindle BW, Tchervenkov CI, Konstantinov IE, Williams WG, Neirotti RA, Jacobs ML, et al. Risk factors associated with mortality and interventions in 472 neonates with interrupted aortic arch: a Congenital Heart Surgeons Society study. J Thorac Cardiovasc Surg 2005;129(2):343–50.
[9] Krishna CS, Bhan A, Sharma S, et al. Interruption of aortic arch in adults: surgical experience with extra-anatomic bypass. Tex Heart Inst J 2005;32: 147–50. [10] Gokce M, Kiris A, Karakoc G, et al. Isolated interrupted aortic arch: In a 40-year-old adult. Int J Cardiol 2010;138:e39–41. [11] Mirat J, Galic E, Coric V, et al. Secondary hypertension due to isolated interrupted aortic arch in a 60-year-old person—one year follow up. Collegium Anthropologicum 2010;34(1):307–9.
Contents lists available at ScienceDirect
Clinical Imaging journal homepage: http://www.clinicalimaging.org
Case Reports
Type A interrupted aortic arch accompanied by intracranial aneurysms causing subarachnoid hemorrhage in an adult man☆ Suat Eren a,⁎, Mecit Kantarci a, Berhan Pirimoglu a, Murteza Cakir b, Hayri Ogul a a b
Ataturk University, School of Medicine, Department of Radiology, Erzurum, Turkey Ataturk University, School of Medicine, Department of Neurosurgery, Erzurum, Turkey
a r t i c l e
i n f o
Article history: Received 3 January 2013 Received in revised form 7 April 2013 Accepted 9 May 2013 Keywords: Interrupted aortic arch Subarachnoid hemorrhage Multidetector computed tomography angiography Digital subtraction angiography
a b s t r a c t Interrupted aortic arch anomaly (IAA) characterized by complete luminal dissociation between the ascending and descending aorta and accounting for less than 1% of all cases of congenital heart disease. IAA is a rare condition in infants that occurs approximately three times per million births. It is usually diagnosed and repaired during the neonatal period and is extremely rare in adults. We present the case of an adult man who was diagnosed with IAA accompanied by intracranial aneurysms causing subarachnoid hemorrhage and demonstrate the imaging findings with 256-slice computed tomography angiography and digital subtraction angiography.
1. Introductıon Interrupted aortic arch, characterized by luminal and anatomic discontinuity between the ascending and descending aorta, is a very rare congenital malformation. The condition is typically diagnosed in neonates and is highly fatal if left untreated [1]. The anomaly was first described by the Viennese surgeon Steidele in 1778 [2], with a prevalence as low as 3 in 1,000,000 live births [3]. Defined as a loss of luminal continuity between the ascending and descending portions of the aorta, this anomaly entails a very poor prognosis without surgical treatment [4]. We reported an unusual diagnosis of a Type A interrupted aortic arch anomaly (IAA) and intracranial aneursyms causing subarachnoid hemorrhage in a 20-year-old hypertensive man. 2. Case report A 20-year-old man was referred to our emergency service with suddenly onset of severe headache and hypertension. The patient had no other significant medical history. In our emergency service, noncontrast enhanced brain computed tomography was performed, and subarachnoid hemorrhage was detected. Digital substraction angiography (DSA) examination was performed immediately afterwards to reveal if there was an intracranial vascular pathology. During ☆ Conflict of Interest: Authors declared that there was no conflict of interest. ⁎ Corresponding author. Ataturk University, School of Medicine, Department of Radiology, Erzurum, Turkey. Tel.: +90-4422316908. E-mail address: [email protected] (S. Eren). 0899-7071/$ – see front matter © 2014 Published by Elsevier Inc. http://dx.doi.org/10.1016/j.clinimag.2013.05.012
© 2014 Published by Elsevier Inc.
the DSA examination, femoral artery pulses were found extremely weak, and catheter did not pass beyond the descending aorta. The descending aorta had a blunt ending (Fig. 1). The arterial blood pressure was 165/95 mmHg in both arms and 80/60 mmHg in the lower extremities. The pulses were equal over both upper extremities. To elucidate the aortic and intracranial arterial anatomy, 256-slice multidetector computed tomography (MDCT) angiography (Somatom High Definition, Siemens) was performed (Fig. 2). Axial, sagittal, and volume-rendered three-dimensional reconstruction images clearly revealed a complete interruption in the aortic arch, approximately 20 mm beyond the origin of the left subclavian artery. Extensive collateral vessels were coursing to interrupted aortic region from the intercostal, paravertebral, and internal mammary arteries. Furthermore, we detected three intracranial aneurysms localized at anterior cerebral artery, at the origin of the left posterior cerebellar artery, and at the M1 division of the left middle cerebral artery ranging diameters of 2 to 5 mm (Fig. 3). Bleeding aneurysm at the M1 division was in irregular shape due to partial thrombosis. Two renal arteries were also seen on the left side. The patient was treated in another center with resection and end-to-end anastomosis of aorta and clipping of intracranial aneurysms by open surgery. The patient is on follow-up. 3. Discussion IAA is congenital absence of anatomic and luminal continuity between the ascending and descending portions of the aorta [4,5]. The first classification system of interrupted aortic arch, introduced by Celoria and Patton in 1959, is still used almost universally. It describes and classifies the site of aortic arch discontinuity, which may be distal
S. Eren et al. / Clinical Imaging 38 (2014) 60–62
61
Fig. 3. Three-dimensional volume-rendered image shows three intracranial aneurysms localized at anterior cerebral artery, at the origin of the left posterior cerebellar artery, and at the M1 division of the left middle cerebral artery (white arrows).
Fig. 1. DSA Aortogram acquired by right femoral arterial puncture shows the blunt ending descending aorta. Note the feeding artery on the right side of the descending aorta (arrow).
to the left subclavian artery (Type A), between the left carotid and left subclavian arteries (type B) or between the innominate and left carotid arteries (Type C). The most common type is B (53%), followed by A (43%) and C (4%) [6]. Interrupted aortic arch is associated with additional cardiovascular anomalies, including ventricular septal defect, patent ductus arteriosus, bicuspid aortic valve, left ventricular
outflow tract obstruction, aortopulmonary window, and truncus arteriosus [7]. IAA can be diagnosed by echocardiography, MDCT, magnetic resonance imaging and DSA. The combination of MDCT angiography and DSA enable definitive diagnosis of interrupted aortic arch and associated abnormalities [7,8], as in our patient. In adults, the presentation of IAA ranges from lack of symptoms to limb swelling with differential blood pressures in all extremities. Blood stream of the descending aorta usually is supplied by the intercostal, internal mammary, or paravertebral collaterals, as shown in our patient. Substantial collateral circulation must be present to maintain flow and enable survival [7]. In our case, high blood pressure was presented in both arms. Seventy percent of patients have hypertension refractory to medical management as their presenting symptom. Other common presenting symptoms are claudication, congestive heart failure, and aortic insufficiency [9]. Life-threatening complications are present at initial presentation in 13% of patients; these included coronary artery disease, intracranial hemorrhage, and biventricular heart failure [10,11]. Our patient had hypertension refractory to medical management and life-threatening intracranial aneurysm and hemorrhage. Surgical intervention is necessary to both improve the patient's symptomatology and to prevent potentially fatal sequel [11]. McCrindle et al. reported that the overall survival at 16 years after study entry was found to be 59% in patients with interrupted aortic arch; the survival rate increased to approximately 70% in patients who had undergone preoperative therapy and appropriate surgical techniques [8]. In conclusion, IAA is rarely encountered in adults. MDCT angiography appears to be a useful diagnostic imaging modality in patients with this congenital anomaly. The accurate and early diagnosis of interrupted aortic arch and its associated anomalies is profoundly important for the patient's survival. References
Fig. 2. Multislice computed tomography angiography image in oblique sagittal shows Type A interrupted aortic arch (black arrow), paravertebral collateral vessels (arrowhead), and intracranial aneurysm at the left middle cerebral artery (white arrow). Note the proximal side of the interrupted aortic arch shows more contrast enhancement than distal side.
[1] Bugan B, Iyisoy A, Celik M, et al. Isolated type A interrupted aortic arch in an asymptomatic 19-year-old man. Tex Heart Inst J 2011;38(5):559–61. [2] Steidele RJ. Sammlung verschiedener in der chirurgischpraktischen lehrschule gemachten beobachtungen 2. Vienna:Trattern, 1778; p. 114. [3] Dillman JR, Yarram SG, D’Amico AR, Hernandez RJ. Interrupted aortic arch: spectrum of MRI findings. AJR Am J Roentgenol 2008;190(6):1467–74. [4] Mishra PK. Management strategies for interrupted aortic arch with associated anomalies. Eur J Cardiothorac Surg 2009;35(4):569–76. [5] Gordon AE, Person T, Kavarana M, et al. Interrupted aortic arch in the adult. J Card Surg 2011;26:405–9.
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[6] Celoria GC, Patton RB. Congenital absence of the aortic arch. Am Heart J 1959;58: 407–13. [7] Messner G, Reul GJ, Flamm SD, et al. Interrupted aortic arch in an adult singlestage extra-anatomic repair. Tex Heart Inst J 2002;29:118–21. [8] McCrindle BW, Tchervenkov CI, Konstantinov IE, Williams WG, Neirotti RA, Jacobs ML, et al. Risk factors associated with mortality and interventions in 472 neonates with interrupted aortic arch: a Congenital Heart Surgeons Society study. J Thorac Cardiovasc Surg 2005;129(2):343–50.
[9] Krishna CS, Bhan A, Sharma S, et al. Interruption of aortic arch in adults: surgical experience with extra-anatomic bypass. Tex Heart Inst J 2005;32: 147–50. [10] Gokce M, Kiris A, Karakoc G, et al. Isolated interrupted aortic arch: In a 40-year-old adult. Int J Cardiol 2010;138:e39–41. [11] Mirat J, Galic E, Coric V, et al. Secondary hypertension due to isolated interrupted aortic arch in a 60-year-old person—one year follow up. Collegium Anthropologicum 2010;34(1):307–9.