Pseudoaneurysm of the Descending Aorta Complicating an Untreated Aortic Coarctation Edvin Prifti, MD, PhD, Saimir Kuci, MD, Klodian Krakulli, MD, and Edmond Nuellari, MD Division of Cardiac Surgery, University Hospital Center, Tirana, Albania
A 22-year-old man was referred for severe aortic coarctation. Contrast-enhanced computed tomography confirmed the aortic coarctation diagnosis and showed an aortic pseudoaneurysm arising from the anterior and left surface of the descending aorta, communicating with the aortic lumen with a small neck. Under cardiopulmonary bypass through the femoral vessels, the patient underwent closure of the pseudoaneurysm neck using a synthetic patch and interposition of a prosthetic graft between the left subclavian artery and the descending aorta below the pseudoaneurysm. The patient’s postoperative course was uneventful. (Ann Thorac Surg 2015;99:e3–5) Ó 2015 by The Society of Thoracic Surgeons
T
he untreated aortic coarctation (CoAo) tends to be complicated by hypertension, ascending aortic aneurysm, cardiac failure, rarely endarteritis, and descending aortic aneurysms [1]. The aortic pseudoaneurysm (APD) associated with an untreated CoAo is rarely encountered and usually only in patients with endarteritis. We present an adult man who underwent the resection of an untreated CoAo complicated with an APD of the descending aorta, representing the second reported case of an APD in a patient with CoAo without aortic endarteritis [2].
A 22-year-old man was referred to our division for CoAo. At admission, the femoral pulses were weak, and blood pressure was higher in the upper limb than in the lower limb (180/80 mm Hg vs 120/70 mm Hg). A systolic ejection murmur was audible over the base of the heart and in the left interscapular region. Transthoracic echocardiography showed a tricuspid aortic valve without leaflets dysfunction and a slight dilatation of the ascending aorta associated with a marked isthmus stricture. No evidence of vegetation or valve lesions was present. There was maximal gradient o f 64 mm Hg on Doppler examination. Contrast-enhanced computed tomography angiography confirmed the CoAo diagnosis, demonstrating the APD neck arising from the anterior and left surface of the aorta distal to the CoAo and expected ductus arteriosus location (Fig 1A). The APD measured 62 40 35 mm and communicated with the aortic lumen with a small Accepted for publication Oct 7, 2014. Address correspondence to Dr Prifti, Division of Cardiac Surgery, University Hospital Center of Tirana, RR Dibra 370, Tirana, Albania; e-mail: [email protected].
Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
Fig 1. (A) Preoperative contrast-enhanced computed tomography angiography demonstrates a saccular mass distal to a severe aortic coarctation (CoAo). (B) Postoperative contrast-enhanced computed tomography angiography demonstrates a normal functional graft between the left subclavian artery (LSA) and the descending aorta. (APD ¼ aortic pseudoaneurysm.)
neck. Laboratory data showed the erythrocyte sedimentation rate and C-reactive protein levels were within normal reference ranges. Results of blood cultures were negative. There was no clinical evidence of aortic mycotic endarteritis. A left posterolateral thoracotomy was performed through the fourth rib bed. The distal aortic arch and the descending aorta were carefully mobilized. Strong thrill was felt on the descending aorta just distal to the coarctation, at which a saccular mass was confirmed (Fig 2A). 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.10.027
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CASE REPORT PRIFTI ET AL COAO COMPLICATED BY AORTIC PSEUDOANEURYSM
Fig 2. (A) Intraoperative view of the aortic pseudoaneurysm (APD) distal to the aortic coarctation (CoAo). (B) Opened APD with an excellent view of the entry port and extraanatomic bypass construction. (LSA ¼ left subclavian artery.)
The left femoral vein and artery were cannulated, and partial cardiopulmonary bypass was established. After the lateral clamping of the distal end of the aortic arch and left subclavian artery, the proximal anastomosis was performed with a prosthetic 18-mm Dacron (DuPont, Wilmington, DE) tube. The descending aorta was clamped above and below the APD. The saccular mass was transected, and the entry port was identified (Fig 2B). The APD neck was closed using a synthetic patch, and a distal anastomosis was performed between the Dacron tube and descending aorta. No intercostal arteries were sacrificed. The histopathologic examination of the excised specimen showed that no intimal structure was observed in the saccular pseudoaneurysm. No acute or chronic inflammation was present. Results of bacteriologic cultures of the resected aortic tissue were negative. The patient’s postoperative course was uneventful. A contrast-enhanced computed tomography angiography 6 months later demonstrated the extraanatomic bypass was functioning normally (Fig 1B).
Comment Aortic rupture is the major cause of death of the untreated CoAo. Hence, correction of the APD is required
Ann Thorac Surg 2015;99:e3–5
before rupture. In our patient, preoperative contrastenhanced computed tomography angiography revealed a large APD on the descending aorta with a well-defined entry neck. The etiology of APD formation is unclear, but several possibilities have been suggested, including hypertension in the upper part of the body, infection, congenital weakness in the aortic wall, and jet stream through the coarctation [3]. CoAo produces conditions for the development of endothelial injury and subsequent infection by the same pathogenic mechanism as endocarditis of the heart valves [4]. The infective lesion usually occurs in the aorta downstream from the high-gradient stenosis at the site of impact of the high velocity jet. After the first reported case of an APD in an untreated CoAo in 1948 [5], only 15 similar cases have been reported, and most of these patients had a mycotic pseudoaneurysm. Our patient is the second reported case of an descending APD in an untreated CoAo not caused by aortic endarteritis. In coarctation, three hemodynamic situations lead to the stripping of the endothelium: a high-velocity abnormal jet stream, the flow from a high pressure to a low pressure chamber, and the presence of a narrow orifice between 2 chambers capable of creating a pressure gradient [4]. The APD in the present patient was located on the low blood pressure side of the coarctation, and therefore, hypertension was not the cause. Such a poststenotic APD may be directly attributable to jet streams of blood coming through the coarctation and impinging on the distal aortic wall [2, 3]. The jet stream was probably the cause of the APD in our patient. The diagnosis is often made on echocardiography, but many APDs are discovered when rupture or external compression occurs [6]. The APD in our patient was clinically silent, and no mediastinal mass was seen on the chest roentgenogram. The APD was identified by computed tomography. Surgical treatment, timing of the operative intervention, and the type of material used for the aortic reconstruction remain controversial. In situ reconstruction of the aorta with a homograft seems to be the preferable surgical treatment for mycotic APD [7]. Excision of the infected aortic tissue and end-to-end anastomosis is another surgical option in mycotic APD [2, 5, 8]. The interposition of a synthetic graft between the left subclavian artery and the descending aorta below the APD seems to be the technique that is usually used, with excellent outcome. The APD in an untreated CoAo is a rare complication requiring prompt surgical correction. The extraanatomic bypass and resection of APD seems to offer excellent early and late outcome.
References 1. Oliver JM, Gallego P, Gonzalez A, Aroca A, Bret M, Mesa JM. Risk factors for aortic complications in adults with coarctation of the aorta. J Am Coll Cardiol 2004;44:1641–7. 2. Oi K, Yoshida T, Takeshita M, Tsuruta G. False aneurysm on distal part of coarctation of the aorta in a parous Turner syndrome patient. Gen Thorac Cardiovasc Surg 2013;61:531–3.
Ann Thorac Surg 2015;99:e3–5
3. Mitchell IM, Pollock JC. Coarctation of the aorta and poststenotic aneurysm formation. Br Heart J 1990;64: 332–3. 4. Owens CG, Johnston N, Campalani GF, et al. Aortic coarctation endarteritis resulting in mycotic pseudoaneurysm and acute mediastinitis. Acta Cardiol 2010;65:463–5. 5. Shumacker HB. Coarctation and aneurysm of the aorta. Report of a case treated by excision and end-to-end suture of aorta. Ann Surg 1948;127:655–65.
CASE REPORT PRIFTI ET AL COAO COMPLICATED BY AORTIC PSEUDOANEURYSM
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6. Jaleleddine Z, Sana C, Faker G, Adel K. Infective endarteritis and false mycotic aneurysm complicating aortic coarctation. Ann Pediatr Cardiol 2012;5:197–9. 7. Barth H, Moosdorf R, Bauer J, Schranz D, Akinturk H. Mycotic pseudoaneurysm of the aorta in children. Pediatr Cardiol 2000;21:263–6. 8. Avanzas P, Garcia-Fernandez MA, Quiles J, Datino T, Moreno M. Pseudoaneurysm complicating aortic coarctation in a pregnant woman. Int J Cardiol 2004;97:157–8.
A 22-year-old man was referred for severe aortic coarctation. Contrast-enhanced computed tomography confirmed the aortic coarctation diagnosis and showed an aortic pseudoaneurysm arising from the anterior and left surface of the descending aorta, communicating with the aortic lumen with a small neck. Under cardiopulmonary bypass through the femoral vessels, the patient underwent closure of the pseudoaneurysm neck using a synthetic patch and interposition of a prosthetic graft between the left subclavian artery and the descending aorta below the pseudoaneurysm. The patient’s postoperative course was uneventful. (Ann Thorac Surg 2015;99:e3–5) Ó 2015 by The Society of Thoracic Surgeons
T
he untreated aortic coarctation (CoAo) tends to be complicated by hypertension, ascending aortic aneurysm, cardiac failure, rarely endarteritis, and descending aortic aneurysms [1]. The aortic pseudoaneurysm (APD) associated with an untreated CoAo is rarely encountered and usually only in patients with endarteritis. We present an adult man who underwent the resection of an untreated CoAo complicated with an APD of the descending aorta, representing the second reported case of an APD in a patient with CoAo without aortic endarteritis [2].
A 22-year-old man was referred to our division for CoAo. At admission, the femoral pulses were weak, and blood pressure was higher in the upper limb than in the lower limb (180/80 mm Hg vs 120/70 mm Hg). A systolic ejection murmur was audible over the base of the heart and in the left interscapular region. Transthoracic echocardiography showed a tricuspid aortic valve without leaflets dysfunction and a slight dilatation of the ascending aorta associated with a marked isthmus stricture. No evidence of vegetation or valve lesions was present. There was maximal gradient o f 64 mm Hg on Doppler examination. Contrast-enhanced computed tomography angiography confirmed the CoAo diagnosis, demonstrating the APD neck arising from the anterior and left surface of the aorta distal to the CoAo and expected ductus arteriosus location (Fig 1A). The APD measured 62 40 35 mm and communicated with the aortic lumen with a small Accepted for publication Oct 7, 2014. Address correspondence to Dr Prifti, Division of Cardiac Surgery, University Hospital Center of Tirana, RR Dibra 370, Tirana, Albania; e-mail: [email protected].
Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
Fig 1. (A) Preoperative contrast-enhanced computed tomography angiography demonstrates a saccular mass distal to a severe aortic coarctation (CoAo). (B) Postoperative contrast-enhanced computed tomography angiography demonstrates a normal functional graft between the left subclavian artery (LSA) and the descending aorta. (APD ¼ aortic pseudoaneurysm.)
neck. Laboratory data showed the erythrocyte sedimentation rate and C-reactive protein levels were within normal reference ranges. Results of blood cultures were negative. There was no clinical evidence of aortic mycotic endarteritis. A left posterolateral thoracotomy was performed through the fourth rib bed. The distal aortic arch and the descending aorta were carefully mobilized. Strong thrill was felt on the descending aorta just distal to the coarctation, at which a saccular mass was confirmed (Fig 2A). 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.10.027
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CASE REPORT PRIFTI ET AL COAO COMPLICATED BY AORTIC PSEUDOANEURYSM
Fig 2. (A) Intraoperative view of the aortic pseudoaneurysm (APD) distal to the aortic coarctation (CoAo). (B) Opened APD with an excellent view of the entry port and extraanatomic bypass construction. (LSA ¼ left subclavian artery.)
The left femoral vein and artery were cannulated, and partial cardiopulmonary bypass was established. After the lateral clamping of the distal end of the aortic arch and left subclavian artery, the proximal anastomosis was performed with a prosthetic 18-mm Dacron (DuPont, Wilmington, DE) tube. The descending aorta was clamped above and below the APD. The saccular mass was transected, and the entry port was identified (Fig 2B). The APD neck was closed using a synthetic patch, and a distal anastomosis was performed between the Dacron tube and descending aorta. No intercostal arteries were sacrificed. The histopathologic examination of the excised specimen showed that no intimal structure was observed in the saccular pseudoaneurysm. No acute or chronic inflammation was present. Results of bacteriologic cultures of the resected aortic tissue were negative. The patient’s postoperative course was uneventful. A contrast-enhanced computed tomography angiography 6 months later demonstrated the extraanatomic bypass was functioning normally (Fig 1B).
Comment Aortic rupture is the major cause of death of the untreated CoAo. Hence, correction of the APD is required
Ann Thorac Surg 2015;99:e3–5
before rupture. In our patient, preoperative contrastenhanced computed tomography angiography revealed a large APD on the descending aorta with a well-defined entry neck. The etiology of APD formation is unclear, but several possibilities have been suggested, including hypertension in the upper part of the body, infection, congenital weakness in the aortic wall, and jet stream through the coarctation [3]. CoAo produces conditions for the development of endothelial injury and subsequent infection by the same pathogenic mechanism as endocarditis of the heart valves [4]. The infective lesion usually occurs in the aorta downstream from the high-gradient stenosis at the site of impact of the high velocity jet. After the first reported case of an APD in an untreated CoAo in 1948 [5], only 15 similar cases have been reported, and most of these patients had a mycotic pseudoaneurysm. Our patient is the second reported case of an descending APD in an untreated CoAo not caused by aortic endarteritis. In coarctation, three hemodynamic situations lead to the stripping of the endothelium: a high-velocity abnormal jet stream, the flow from a high pressure to a low pressure chamber, and the presence of a narrow orifice between 2 chambers capable of creating a pressure gradient [4]. The APD in the present patient was located on the low blood pressure side of the coarctation, and therefore, hypertension was not the cause. Such a poststenotic APD may be directly attributable to jet streams of blood coming through the coarctation and impinging on the distal aortic wall [2, 3]. The jet stream was probably the cause of the APD in our patient. The diagnosis is often made on echocardiography, but many APDs are discovered when rupture or external compression occurs [6]. The APD in our patient was clinically silent, and no mediastinal mass was seen on the chest roentgenogram. The APD was identified by computed tomography. Surgical treatment, timing of the operative intervention, and the type of material used for the aortic reconstruction remain controversial. In situ reconstruction of the aorta with a homograft seems to be the preferable surgical treatment for mycotic APD [7]. Excision of the infected aortic tissue and end-to-end anastomosis is another surgical option in mycotic APD [2, 5, 8]. The interposition of a synthetic graft between the left subclavian artery and the descending aorta below the APD seems to be the technique that is usually used, with excellent outcome. The APD in an untreated CoAo is a rare complication requiring prompt surgical correction. The extraanatomic bypass and resection of APD seems to offer excellent early and late outcome.
References 1. Oliver JM, Gallego P, Gonzalez A, Aroca A, Bret M, Mesa JM. Risk factors for aortic complications in adults with coarctation of the aorta. J Am Coll Cardiol 2004;44:1641–7. 2. Oi K, Yoshida T, Takeshita M, Tsuruta G. False aneurysm on distal part of coarctation of the aorta in a parous Turner syndrome patient. Gen Thorac Cardiovasc Surg 2013;61:531–3.
Ann Thorac Surg 2015;99:e3–5
3. Mitchell IM, Pollock JC. Coarctation of the aorta and poststenotic aneurysm formation. Br Heart J 1990;64: 332–3. 4. Owens CG, Johnston N, Campalani GF, et al. Aortic coarctation endarteritis resulting in mycotic pseudoaneurysm and acute mediastinitis. Acta Cardiol 2010;65:463–5. 5. Shumacker HB. Coarctation and aneurysm of the aorta. Report of a case treated by excision and end-to-end suture of aorta. Ann Surg 1948;127:655–65.
CASE REPORT PRIFTI ET AL COAO COMPLICATED BY AORTIC PSEUDOANEURYSM
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6. Jaleleddine Z, Sana C, Faker G, Adel K. Infective endarteritis and false mycotic aneurysm complicating aortic coarctation. Ann Pediatr Cardiol 2012;5:197–9. 7. Barth H, Moosdorf R, Bauer J, Schranz D, Akinturk H. Mycotic pseudoaneurysm of the aorta in children. Pediatr Cardiol 2000;21:263–6. 8. Avanzas P, Garcia-Fernandez MA, Quiles J, Datino T, Moreno M. Pseudoaneurysm complicating aortic coarctation in a pregnant woman. Int J Cardiol 2004;97:157–8.
