Case Study
Recurrent giant sinus of Valsalva aneurysm and ankylosing spondylitis
Asian Cardiovascular & Thoracic Annals 21(4) 450–452 ß The Author(s) 2012 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492312454746 aan.sagepub.com
Suleyman Ercan1, Musa Cakici1, Vedat Davutoglu1, Mehmet Hayri Alici1 and Ahmet Mesut Onat2
Abstract A 48-year-old woman underwent aneurysmectomy and primary suture repair with a pericardial patch for sinus of Valsalva aneurysm secondary to ankylosing spondylitis. The sinus of Valsalva aneurysm recurred one year after surgery, and reached a diameter of 53 mm. Special attention must be paid to the potential relapse of aortic aneurysms that develop secondary to autoimmune disorders, when using primary suture or patch repair.
Keywords Aortic aneurysm, sinus of Valsalva, spondylitis, ankylosing
Introduction Ankylosing spondylitis (AS) is a chronic inflammatory disorder of unknown origin. It possesses distinctive features including a propensity for axial and peripheral arthritis, inflammation at tendinous, ligamentous, or fascial insertions, and a familial pattern of inheritance based on the presence of the class I major histocompatibility complex antigen HLA-B27.1 The most familiar complication of AS is an inflammatory process involving the aortic wall behind and immediately above the sinuses of Valsalva, which may result in sinus of Valsalva aneurysm.
Case report A 48-year-old woman was admitted to the rheumatology outpatient clinic with the recent complaint of worsening hip pain that lasted for approximately 10 years. On physical examination, a flexion, abduction, and external rotation test was positive, and cardiac examination revealed a 2/4 diastolic murmur at the aortic focus. Laboratory test results revealed the presence of the immunogenetic marker, HLA-B27, with negative serology results for rheumatoid factor and antinuclear antibodies. Inflammatory markers were not elevated. Lumbosacral and pelvic radiographs revealed spinal straightening, facet joint sclerosis, and bilateral sacroiliac joint fusion consistent with AS. On
echocardiographic evaluation, an evident isolated sinus of Valsalva aneurysm was observed in the noncoronary cusp of the sinus Valsalva in the aortic root (Figure 1). In parasternal short-axis view, the diameter of the aorta was 57 mm, with grade 1 aortic insufficiency. The patient was recommended to undergo surgery. On coronary angiography, distal occlusion of the right coronary artery and noncritical plaques in the other coronary arteries were observed. The rheumatology outpatient clinic initiated sulfasalazine, chloroquine, and prednisolone treatment. The patient underwent cardiovascular surgery after one month of medical treatment. The sinus of Valsalva aneurysm was resected, and the defect was repaired using a bovine pericardial patch. Within 24 h postoperatively, sulfasalazine, methotrexate, and metoprolol were started. On echocardiography on the 3rd postoperative day, the sinus of Valsalva was measured as 4 cm. During follow-up, the general condition of the patient improved and she was discharged with the above
1 2
Department of Cardiology, Gaziantep University, Gaziantep, Turkey Department of Rheumatology, Gaziantep University, Gaziantep, Turkey
Corresponding author: Vedat Davutoglu, MD, Department of Cardiology, Gaziantep University, Gaziantep, Turkey. Email: [email protected]
Downloaded from aan.sagepub.com at Univ. of Tasmania Library on May 21, 2015
Ercan et al.
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Figure 1. Preoperative echocardiography revealing large sinus Valsalva noncoronary cusp aneurysm (star) in transesophageal echocardiography. AO: aorta; LV: left ventricle.
medication. The sinus of Valsalva aneurysm relapsed within one year after the surgery. On the latest echocardiographic examination, a significant isolated sinus of Valsalva aneurysm with a diameter of 53 mm in parasternal short-axis view was observed in the noncoronary cusp of the sinus Valsalva, and severe aortic insufficiency was noted (Figure 2). The patient was recommended to undergo reoperation; however, she did not accept the surgery.
Discussion Ankylosing spondylitis has several extraskeletal manifestations including acute anterior uveitis and iridocyclitis, which occur in 25% to 30% of patients during the course of the disease.2 Pulmonary involvement, due to fibrosis of the lung, is a late and rare manifestation of AS. Cardiac manifestations are common and include aortic valve incompetence in 3.5%, conduction abnormalities in 8%, cardiomegaly, pericarditis, and aortitis.3 Bulkley and colleagues4 reported the characteristic pathologic features of aortitis associated with AS in their necropsy study. The aortic inflammatory process was limited to the aortic wall behind and immediately above the sinuses of Valsalva, particularly behind and adjacent to the commissures. Nevertheless, the inflammatory process uncommonly extends into the distal ascending and descending thoracic aorta or distal abdominal aorta.5,6 The prevalence of subclinical aortic root and valvular disease may be as high as 82% in patients with AS when studied with transesophageal echocardiography.7 Although the prevalence of cardiac abnormalities appears to be unrelated to the treatment or skeletal disease severity, cardiac manifestations have been correlated with age >45 years and duration of disease >15 years.7 Particular attention should be paid to aortic valve replacement in inflammatory cardiovascular diseases such as AS, because valvular detachment, paravalvular leakage, and pseudoaneurysm formation are frequent
Figure 2. Postoperative echocardiography showing a large relapse of the sinus of Valsalva noncoronary cusp aneurysm (star) in transesophageal echocardiography. AO: aorta.
postoperative complications.8 In our patient, the sinus of Valsalva aneurysm relapsed within one year after the surgery. To the best of our knowledge, although there are numerous reports of sinus of Valsalva aneurysms, relapse after aneurysm surgery has not been reported previously.9 Thus it is crucial to reduce inflammation preoperatively and postoperatively, to reinforce the suture line, and carefully select the operative procedure for treatment of cardiovascular disorders caused by systemic inflammatory diseases.10 In a study by Stamp and colleagues7 of a patient with HLA-B27-associated spondyloarthropathy and severe ascending aortitis, sulfasalazine, prednisolone, cyclophosphamide, or azathioprine treatment was administered preoperatively because of extensive systemic inflammation and rapid expansion of the ascending aortic aneurysm. They focused on reducing potential postoperative complications. Our patient was given sulfasalazine, prednisolone, methotrexate, and metoprolol postoperatively. Despite the long-term use of these medications, relapse of her sinus of Valsalva aneurysm could not be prevented. Yamauchi and colleagues11 employed the modified Bentall technique, and emphasized that the key point in repairing valvular detachment in Takayasu’s arteritis was to reduce the tension on the suture line and aortic annulus. They suggested that aortic root replacement, specifically with an allograft, might have provided favorable results. Special attention must be paid to the potential relapse of aortic aneurysms that develop secondary to autoimmune disorders such as AS, Behc¸et’s disease, psoriatic arthritis, Takayasu’s arteritis, and Reiter’s syndrome, when using primary suture repair and patch repair. Therefore, more radical surgical approaches should be preferred, such as the Bentall technique. Furthermore, patients should be regularly followed up by echocardiography for the potential mechanical complications that may develop postoperatively.
Downloaded from aan.sagepub.com at Univ. of Tasmania Library on May 21, 2015
452
Asian Cardiovascular & Thoracic Annals 21(4)
Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Conflicts of interest statement None declared.
References 1. Gallagher MR and Haid Jr RW. Spondyloarthropathies, including ankylosing spondylitis. In: Winn HR, Youmans JR (eds) Youmans Neurological Surgery, 5th edn. Philadelphia: WB Saunders, 2004, pp. 4459–4475. 2. Van der Linden S. Ankylosing spondylitis. In: Kelley WN, Harris S, Ruddy S (eds) Textbook of Rheumatology, 5th edn. Philadelphia: WB Saunders, 1997, pp. 969–982. 3. Savolaine ER, Ebraheim NA, Stitgen S and Jackson WT. Aortic rupture complicating a fracture of an ankylosed thoracic spine. A case report. Clin Orthop 1991; 272: 136–140. 4. Bulkley BH and Roberts WC. Ankylosing spondylitis and aortic regurgitation: description of the characteristic cardiovascular lesion from study of eight necropsy patients. Circulation 1973; 48: 1014–1027.
5. Kawasuji M, Hetzer R, Oelert H, Stauch G and Borst HG. Aortic valve replacement and ascending aorta replacement in ankylosing spondylitis: report of three surgical cases and review of the literature. Thorac Cardiovasc Surg 1982; 30: 310–314. 6. Stamp L, Lambie N and O’Donnell J. HLA-B-27 associated spondyloarthropathy and severe ascending aortitis. J Rheumatol 2000; 27: 2038–2040. 7. Roldan CA, Chavez J, Wiest PW, Qualls CR and Crawford MH. Aortic root disease and valve disease associated with ankylosing spondylitis. J Am Coll Cardiol 1998; 32: 1397–1404. 8. Isomura T, Hisatomi K, Yanagi I, Shimada S, Uraguchi K, Aoyagi S, et al. The surgical treatment of aortic regurgitation secondary to aortitis. Ann Thorac Surg 1988; 45: 181–185. 9. Hirose T, Kameda Y, Yoshikawa Y, Abe T, Hayata Y and Taniguchi S. Aortic root replacement for Valsalva sinus aneurysm with lupus erythematosus. Asian Cardiovasc Thorac Ann 2012; 20: 193–195. 10. Moro H, Hayashi J, Ohzeki H, Sogawa M, Nakayama T and Namura O. Surgical management of cardiovascular lesions caused by systemic inflammatory diseases. Thorac Cardiovasc Surg 1999; 47: 106–110. 11. Yamauchi M, Eishi K, Sasako K, Nakano K, Isobe F and Kawashima Y. A case of aortitis operated twice Bentall’s operation due to valve (graft) detachment. Jpn J Thorac Surg 1994; 47: 1083–1085.
Downloaded from aan.sagepub.com at Univ. of Tasmania Library on May 21, 2015
Recurrent giant sinus of Valsalva aneurysm and ankylosing spondylitis
Asian Cardiovascular & Thoracic Annals 21(4) 450–452 ß The Author(s) 2012 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492312454746 aan.sagepub.com
Suleyman Ercan1, Musa Cakici1, Vedat Davutoglu1, Mehmet Hayri Alici1 and Ahmet Mesut Onat2
Abstract A 48-year-old woman underwent aneurysmectomy and primary suture repair with a pericardial patch for sinus of Valsalva aneurysm secondary to ankylosing spondylitis. The sinus of Valsalva aneurysm recurred one year after surgery, and reached a diameter of 53 mm. Special attention must be paid to the potential relapse of aortic aneurysms that develop secondary to autoimmune disorders, when using primary suture or patch repair.
Keywords Aortic aneurysm, sinus of Valsalva, spondylitis, ankylosing
Introduction Ankylosing spondylitis (AS) is a chronic inflammatory disorder of unknown origin. It possesses distinctive features including a propensity for axial and peripheral arthritis, inflammation at tendinous, ligamentous, or fascial insertions, and a familial pattern of inheritance based on the presence of the class I major histocompatibility complex antigen HLA-B27.1 The most familiar complication of AS is an inflammatory process involving the aortic wall behind and immediately above the sinuses of Valsalva, which may result in sinus of Valsalva aneurysm.
Case report A 48-year-old woman was admitted to the rheumatology outpatient clinic with the recent complaint of worsening hip pain that lasted for approximately 10 years. On physical examination, a flexion, abduction, and external rotation test was positive, and cardiac examination revealed a 2/4 diastolic murmur at the aortic focus. Laboratory test results revealed the presence of the immunogenetic marker, HLA-B27, with negative serology results for rheumatoid factor and antinuclear antibodies. Inflammatory markers were not elevated. Lumbosacral and pelvic radiographs revealed spinal straightening, facet joint sclerosis, and bilateral sacroiliac joint fusion consistent with AS. On
echocardiographic evaluation, an evident isolated sinus of Valsalva aneurysm was observed in the noncoronary cusp of the sinus Valsalva in the aortic root (Figure 1). In parasternal short-axis view, the diameter of the aorta was 57 mm, with grade 1 aortic insufficiency. The patient was recommended to undergo surgery. On coronary angiography, distal occlusion of the right coronary artery and noncritical plaques in the other coronary arteries were observed. The rheumatology outpatient clinic initiated sulfasalazine, chloroquine, and prednisolone treatment. The patient underwent cardiovascular surgery after one month of medical treatment. The sinus of Valsalva aneurysm was resected, and the defect was repaired using a bovine pericardial patch. Within 24 h postoperatively, sulfasalazine, methotrexate, and metoprolol were started. On echocardiography on the 3rd postoperative day, the sinus of Valsalva was measured as 4 cm. During follow-up, the general condition of the patient improved and she was discharged with the above
1 2
Department of Cardiology, Gaziantep University, Gaziantep, Turkey Department of Rheumatology, Gaziantep University, Gaziantep, Turkey
Corresponding author: Vedat Davutoglu, MD, Department of Cardiology, Gaziantep University, Gaziantep, Turkey. Email: [email protected]
Downloaded from aan.sagepub.com at Univ. of Tasmania Library on May 21, 2015
Ercan et al.
451
Figure 1. Preoperative echocardiography revealing large sinus Valsalva noncoronary cusp aneurysm (star) in transesophageal echocardiography. AO: aorta; LV: left ventricle.
medication. The sinus of Valsalva aneurysm relapsed within one year after the surgery. On the latest echocardiographic examination, a significant isolated sinus of Valsalva aneurysm with a diameter of 53 mm in parasternal short-axis view was observed in the noncoronary cusp of the sinus Valsalva, and severe aortic insufficiency was noted (Figure 2). The patient was recommended to undergo reoperation; however, she did not accept the surgery.
Discussion Ankylosing spondylitis has several extraskeletal manifestations including acute anterior uveitis and iridocyclitis, which occur in 25% to 30% of patients during the course of the disease.2 Pulmonary involvement, due to fibrosis of the lung, is a late and rare manifestation of AS. Cardiac manifestations are common and include aortic valve incompetence in 3.5%, conduction abnormalities in 8%, cardiomegaly, pericarditis, and aortitis.3 Bulkley and colleagues4 reported the characteristic pathologic features of aortitis associated with AS in their necropsy study. The aortic inflammatory process was limited to the aortic wall behind and immediately above the sinuses of Valsalva, particularly behind and adjacent to the commissures. Nevertheless, the inflammatory process uncommonly extends into the distal ascending and descending thoracic aorta or distal abdominal aorta.5,6 The prevalence of subclinical aortic root and valvular disease may be as high as 82% in patients with AS when studied with transesophageal echocardiography.7 Although the prevalence of cardiac abnormalities appears to be unrelated to the treatment or skeletal disease severity, cardiac manifestations have been correlated with age >45 years and duration of disease >15 years.7 Particular attention should be paid to aortic valve replacement in inflammatory cardiovascular diseases such as AS, because valvular detachment, paravalvular leakage, and pseudoaneurysm formation are frequent
Figure 2. Postoperative echocardiography showing a large relapse of the sinus of Valsalva noncoronary cusp aneurysm (star) in transesophageal echocardiography. AO: aorta.
postoperative complications.8 In our patient, the sinus of Valsalva aneurysm relapsed within one year after the surgery. To the best of our knowledge, although there are numerous reports of sinus of Valsalva aneurysms, relapse after aneurysm surgery has not been reported previously.9 Thus it is crucial to reduce inflammation preoperatively and postoperatively, to reinforce the suture line, and carefully select the operative procedure for treatment of cardiovascular disorders caused by systemic inflammatory diseases.10 In a study by Stamp and colleagues7 of a patient with HLA-B27-associated spondyloarthropathy and severe ascending aortitis, sulfasalazine, prednisolone, cyclophosphamide, or azathioprine treatment was administered preoperatively because of extensive systemic inflammation and rapid expansion of the ascending aortic aneurysm. They focused on reducing potential postoperative complications. Our patient was given sulfasalazine, prednisolone, methotrexate, and metoprolol postoperatively. Despite the long-term use of these medications, relapse of her sinus of Valsalva aneurysm could not be prevented. Yamauchi and colleagues11 employed the modified Bentall technique, and emphasized that the key point in repairing valvular detachment in Takayasu’s arteritis was to reduce the tension on the suture line and aortic annulus. They suggested that aortic root replacement, specifically with an allograft, might have provided favorable results. Special attention must be paid to the potential relapse of aortic aneurysms that develop secondary to autoimmune disorders such as AS, Behc¸et’s disease, psoriatic arthritis, Takayasu’s arteritis, and Reiter’s syndrome, when using primary suture repair and patch repair. Therefore, more radical surgical approaches should be preferred, such as the Bentall technique. Furthermore, patients should be regularly followed up by echocardiography for the potential mechanical complications that may develop postoperatively.
Downloaded from aan.sagepub.com at Univ. of Tasmania Library on May 21, 2015
452
Asian Cardiovascular & Thoracic Annals 21(4)
Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Conflicts of interest statement None declared.
References 1. Gallagher MR and Haid Jr RW. Spondyloarthropathies, including ankylosing spondylitis. In: Winn HR, Youmans JR (eds) Youmans Neurological Surgery, 5th edn. Philadelphia: WB Saunders, 2004, pp. 4459–4475. 2. Van der Linden S. Ankylosing spondylitis. In: Kelley WN, Harris S, Ruddy S (eds) Textbook of Rheumatology, 5th edn. Philadelphia: WB Saunders, 1997, pp. 969–982. 3. Savolaine ER, Ebraheim NA, Stitgen S and Jackson WT. Aortic rupture complicating a fracture of an ankylosed thoracic spine. A case report. Clin Orthop 1991; 272: 136–140. 4. Bulkley BH and Roberts WC. Ankylosing spondylitis and aortic regurgitation: description of the characteristic cardiovascular lesion from study of eight necropsy patients. Circulation 1973; 48: 1014–1027.
5. Kawasuji M, Hetzer R, Oelert H, Stauch G and Borst HG. Aortic valve replacement and ascending aorta replacement in ankylosing spondylitis: report of three surgical cases and review of the literature. Thorac Cardiovasc Surg 1982; 30: 310–314. 6. Stamp L, Lambie N and O’Donnell J. HLA-B-27 associated spondyloarthropathy and severe ascending aortitis. J Rheumatol 2000; 27: 2038–2040. 7. Roldan CA, Chavez J, Wiest PW, Qualls CR and Crawford MH. Aortic root disease and valve disease associated with ankylosing spondylitis. J Am Coll Cardiol 1998; 32: 1397–1404. 8. Isomura T, Hisatomi K, Yanagi I, Shimada S, Uraguchi K, Aoyagi S, et al. The surgical treatment of aortic regurgitation secondary to aortitis. Ann Thorac Surg 1988; 45: 181–185. 9. Hirose T, Kameda Y, Yoshikawa Y, Abe T, Hayata Y and Taniguchi S. Aortic root replacement for Valsalva sinus aneurysm with lupus erythematosus. Asian Cardiovasc Thorac Ann 2012; 20: 193–195. 10. Moro H, Hayashi J, Ohzeki H, Sogawa M, Nakayama T and Namura O. Surgical management of cardiovascular lesions caused by systemic inflammatory diseases. Thorac Cardiovasc Surg 1999; 47: 106–110. 11. Yamauchi M, Eishi K, Sasako K, Nakano K, Isobe F and Kawashima Y. A case of aortitis operated twice Bentall’s operation due to valve (graft) detachment. Jpn J Thorac Surg 1994; 47: 1083–1085.
Downloaded from aan.sagepub.com at Univ. of Tasmania Library on May 21, 2015
