Underestimated abdominal vascular pathology in a patient with Takayasu arteritis
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《交互式心脏血管和胸部手术》
a Department of Cardiothoracic Surgery, Thoraxcentre, BD 575, Erasmus Medical Centre Rotterdam, P.O. Box 2040, 3000 CA, Rotterdam, The Netherlands
b Department of Cardiology, Erasmus Medical Centre, Rotterdam, The Netherlands
Abstract
Objective: To describe a 31-year-old female with symptomatic Takayasu disease who was operated for aortic valve replacement. Although she had no preoperative abdominal vascular symptoms, she died on the first postoperative day due to extensive ischemia bowel syndrome. Methods: Echocardiography and computed tomography revealed progressive dilatation and thickening of the ascending aorta, severe aortic regurgitation and diminished left ventricular function from 1998 onwards. In 2000 she was operated and a prosthesis was placed end-to-end distally of the sinutubular junction with combined reduction plasty of this junction. After the operation, in time echocardiography and computed tomography showed progressive irregularities and dilatation of the thoracoabdominal aorta and progressive aortic regurgitation. A staged approach of aortic valve replacement and surgery for the thoracoabdominal aorta was planned. At the time of the reoperation in September 2004 the Takayasu inflammation was, after treatment with a maintenance dosage of prednisone and imuran, in a relative quiescent phase. The aortic valve was uneventfully replaced by a mechanical valve. Results: Medical treatment for Takayasu disease never resulted in the patient in completely normal blood values of white blood cell count, C-reactive protein or erythrocyte sedimentation rate. In August 2004, she was admitted because of severe non-specific thoracic pain and hypertension. During hypertension management, she had a short period of diplégia that was assumed to be due to periods of relapsed relative hypotension. Although the computed tomography revealed severe stenosis of the superior mesenteric artery and the celiac trunk, she was free of abdominal complaints and without further abnormal laboratory findings. One day after the aortic valve replacement a dramatic increase of transaminase and lactate-dehydrogenase with extreme metabolic acidosis appeared. Urgent abdominal surgery was performed and extensive ischemia of the liver, gallbladder, small intestine and the proximal part of the colon were found. Because of the extensive regions of ischemia, no surgical interventions were optional. The patient died one day after abdominal exploration. Conclusions: We conclude that in Takayasu disease scheduled for on pump cardiac surgery, vascular workup should be done, and interventional treatment of asymptomatic but potentially critical lesions should be considered.
Key Words: Abdominal organs; Aortic valve replacement; Computed tomography; cpb-inflammatory response; Inflammatory cells
1. Introduction
Takayasu or pulseless disease is a rare, chronic inflammatory systemic vasculopathy with stenosis, occlusions or aneurysmatic changes of the aorta and its main branches of unknown origin but probably with an autoimmune and genetic etiological predisposition [1,2]. Takayasu arteritis commonly presents in the second or third decade of life, affects mainly women and manifestations depend on the location and severity, and range from asymptomatic to severe neurological and vascular pathology.
Diagnostic criteria were defined by the American College of Rheumatology in 1990 [3] and angiographic classification of Takayasu disease was introduced in 1994 [4]. Nowadays, the diagnosis is also based on clinical findings and histopathologic analysis [5].
The principal therapeutic treatment of Takayasu disease is immunotherapy. Approximately 50% of the patients respond well to corticosteroid therapy [6]. Invasive treatment of vascular problems or surgery should be performed at a time of quiescent disease to prevent complications.
2. Case report
From 1998 a 25-year-old female has been treated in our hospital with corticosteroids and antihypertensive medication because of weariness, high erythrocyte sedimentation rate (ESR), hypertension, loss of weight and periods of non-specific thoracic pain. For years, no diagnosis could be obtained and in time examinations revealed progressive dilatation of the ascending aorta (sinutubular junction 52 mm, ascending aorta 56 mm, arcus aortae 44 mm) without dilatation of the aorta annulus, thickening of the ascending aorta wall, left ventricular end diastolic diameter (LVEDD) of 63 mm, severe aortic regurgitation (AR) of 4/4 and diminished left ventricular function. The descending aorta was found to be normal.
Aorta dilatation with aortitis secondary to syphilis, Reiters syndrome or Beh?et syndrome was excluded. Tests for rheumatoid factor and antinuclear antibody were negative.
Surgery in the year 2000 showed wall abnormalities of the arcus and a normal diameter. Adhesions from the aorta to the pericardium were noticed. The aorta annular region was not dilated (24 mm) and the aortic cusps and Valsalva's sinuses were normal. A Vascutec prothesis (24 mm) was placed end-to-end distally of the sinutubular junction and implanted with combined reduction plasty of the sinutubular junction to perform a valve sparing reconstruction. The prothesis extended into the inner arcus aorta. At the end of the operation transesophageal echocardiography showed cusps with good coaptation, a small centrically aortic valve regurgitation of maximum grade 2/4 which we accepted.
Pathological examination of the aorta revealed intimal fibrocellular thickening, elastic degeneration of the tunica media and infiltration of inflammatory cells extending from the small media to the adventitial layer.
The patient was discharged with moderate AR (2–3/4).
The pathological findings combined with the persistent non-specific thoracic pain, despite control of the inflammatory reaction with steroid treatment, the progressive irregularities of the descending aorta vessel wall in 2000 and a positive somatostatin scan sign at the time of active inflammation [7], suggested Takayasu arteritis.
Dilatation and thoracoabdominal aorta vessel wall abnormalities and AR were progressive with abnormal left ventricular dimensions and persisting hypertension. In March 2004 she uneventfully delivered a healthy son. In August 2004 she was admitted because of severe non-specific thoracic pain and hypertension (170/117 mmHg). During hypertension management, she had a short period of diplégia that was assumed to be due to periods of relapsed relative hypotension.
Examinations revealed a poor left ventricular function, wall motion abnormalities, LVEDD of 65 mm, left ventricular end systolic diameter of 51 mm, AR (3–4/4) and a dilated descending aorta of 50 mm with abnormal and irregular origin of the coeliac trunc (CT) and superior mesenteric artery (SMA). A staged approach of aortic valve replacement and surgery for the thoracoabdominal aorta was planned.
At the time of operation the inflammation was, after treatment with a maintenance dosage of prednisone and imuran, in a relative quiescent phase: white blood cell count was 14090/mm3, C-reactive protein was 38 mg/dl and ESR was 14 mm. Medication did not result in completely normal values.
The aortic valve was replaced by a mechanical valve (St. Jude Medical 23 mm) in September 2004. The operation was uneventful and she could be weaned from mechanical ventilation 1 day after reoperation. She had elevated transaminase and lactate-dehydrogenesis (LDH) levels (GOT 2623 U/l, GPT 2303 U/l, LDH 4594 U/l), normal arterial blood gas values and no renal dysfunction. Later that day a dramatic increase of transaminase and LDH values appeared (GOT 7130 U/l, GPT 5041 U/l, LDH 8349 U/l). The arterial blood gas sample showed extreme metabolic acidosis (pH 7.13, pCO2 3.7 kPa, pO2 19.1 kPa, act.bicarb 8.8 mmol/l, std.bicarb 10.5 kPa, base excess – 19 mmol/l, O2sat 98%, lactate 18.0 mmol/l, ammonia 32 μmol/l). Because of a high probability of ischemia of abdominal organs, urgent abdominal surgery was performed. Extensive ischemia of the liver, gallbladder, small intestine and the proximal part of the colon were found. Because of the extensive regions of ischemia no surgical interventions were optional. The patient died one day after abdominal exploration.
Autopsy revealed an active inflammatory phase of Takayasu disease (Figs. 1 and 2). The thoracoabdominal aorta was dilated with extensive giant cell arteritis, tunica media destruction, intimal fibrocellular thickening and thrombosis. The CT and SMA were occluded due to inflammatory reaction and thrombosis.
3. Discussion
An aortic valve sparing operation in 2000 was performed because of the young age of the patient and her wish for pregnancy. In that setting, a grade 2/4 native AR was accepted, although implantation of an allograft conduit was contemplated [8]. During this operation the aortic arch already showed abnormalities.
Nevertheless, at that time the computed tomography scan did not reveal pathology of the descending aorta.
Consecutive computed tomography-scans from our patient revealed progressive thoracoabdominal aortic dilatation (5 cm) and irregular origin of the TC and SMA in the years after the aorta ascending replacement in 2000. The computed tomography scan before aortic valve replacement in 2004 revealed severe stenosis of the CT and SMA origin but the patient did not have abdominal complaints in the past or in the preoperative period. Even when the antihypertensive therapy was incremented the patient was free of abdominal complaints and without abnormal laboratory findings. On the other hand, she did develop transient diplégia without objectivation of a vascular cause.
Before the operation the patient had no abdominal complaints and the laboratory findings were normal in spite of severe abdominal main vessel stenosis.
We speculate that the specific alterations of normal extracorporeal circulation with non-pulsatile flow and reduced mean arterial pressure may have predisposed to intestinal ischemia, exaggerated by temporary further disturbance of the immune system.
We conclude that in Takayasu disease, scheduled for on pump cardiac surgery, vascular workup should be done and interventional treatment of asymptomatic but potentially critical lesions should be considered.
Acknowledgements
We acknowledge the illustrations by Dr. NNT Goemaere and clinical data by Dr. PLA van Daele.
References
Sheikhzadeh A, Tettenborn I, Noohi F, Eftekharzadeh M, Schnabel A. Occlusive thromboaortopathy (Takayasu disease): clinical and angiographic features and a brief review of literature. Angiology 2002; 53:29–40.
Fietta P. Systemic vasculitides: immunogenetics and familial clustering. Clin Exp Rheumatol 2004; 22:238–251.
Arend WP, Michel BA, Bloch DA, Hunder GG, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY, Lie JT, Lightfoot RW Jr. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum 1990; 33:1129–1134.
Moriwaki R, Noda M, Yajima M, Sharma BK, Numano F. Clinical manifestations of Takayasu arteritis in India and Japan – new classification of angiographic findings. Angiology 1997; 48:369–379.
Kissin E, Merkel P. Diagnostic imaging in Takayasu arteritis. Curr Opin Rheumatol 2004; 16:31–37.
Johnston SL, Lock RJ, Gompels MM. Takayasu arteritis: a review. J Clin Pathol 2002; 55:481–486.
Belhocine T, Blockmans D, Hustinx R, Vandevivere J, Mortelmans L. Imaging of large vessel vasculitis with FDG PET: illusion or reality? A critical review of the literature data. Eur J Nucl Med Mol Imaging 2003; 30:1305–1313.
Sarsam M, Yacoub M. Remodelling of the aortic valve annulus. J Thorac Cardiovasc Surg 1993; 105:435–438.
Related Article
ICVTS on-line discussion A
Mohamed Fahmy Ibrahim and Amal Refaat
Interactive CardioVascular and Thoracic Surgery 2006 5: 453.(Joost M. Hartmana, Jos A.)
b Department of Cardiology, Erasmus Medical Centre, Rotterdam, The Netherlands
Abstract
Objective: To describe a 31-year-old female with symptomatic Takayasu disease who was operated for aortic valve replacement. Although she had no preoperative abdominal vascular symptoms, she died on the first postoperative day due to extensive ischemia bowel syndrome. Methods: Echocardiography and computed tomography revealed progressive dilatation and thickening of the ascending aorta, severe aortic regurgitation and diminished left ventricular function from 1998 onwards. In 2000 she was operated and a prosthesis was placed end-to-end distally of the sinutubular junction with combined reduction plasty of this junction. After the operation, in time echocardiography and computed tomography showed progressive irregularities and dilatation of the thoracoabdominal aorta and progressive aortic regurgitation. A staged approach of aortic valve replacement and surgery for the thoracoabdominal aorta was planned. At the time of the reoperation in September 2004 the Takayasu inflammation was, after treatment with a maintenance dosage of prednisone and imuran, in a relative quiescent phase. The aortic valve was uneventfully replaced by a mechanical valve. Results: Medical treatment for Takayasu disease never resulted in the patient in completely normal blood values of white blood cell count, C-reactive protein or erythrocyte sedimentation rate. In August 2004, she was admitted because of severe non-specific thoracic pain and hypertension. During hypertension management, she had a short period of diplégia that was assumed to be due to periods of relapsed relative hypotension. Although the computed tomography revealed severe stenosis of the superior mesenteric artery and the celiac trunk, she was free of abdominal complaints and without further abnormal laboratory findings. One day after the aortic valve replacement a dramatic increase of transaminase and lactate-dehydrogenase with extreme metabolic acidosis appeared. Urgent abdominal surgery was performed and extensive ischemia of the liver, gallbladder, small intestine and the proximal part of the colon were found. Because of the extensive regions of ischemia, no surgical interventions were optional. The patient died one day after abdominal exploration. Conclusions: We conclude that in Takayasu disease scheduled for on pump cardiac surgery, vascular workup should be done, and interventional treatment of asymptomatic but potentially critical lesions should be considered.
Key Words: Abdominal organs; Aortic valve replacement; Computed tomography; cpb-inflammatory response; Inflammatory cells
1. Introduction
Takayasu or pulseless disease is a rare, chronic inflammatory systemic vasculopathy with stenosis, occlusions or aneurysmatic changes of the aorta and its main branches of unknown origin but probably with an autoimmune and genetic etiological predisposition [1,2]. Takayasu arteritis commonly presents in the second or third decade of life, affects mainly women and manifestations depend on the location and severity, and range from asymptomatic to severe neurological and vascular pathology.
Diagnostic criteria were defined by the American College of Rheumatology in 1990 [3] and angiographic classification of Takayasu disease was introduced in 1994 [4]. Nowadays, the diagnosis is also based on clinical findings and histopathologic analysis [5].
The principal therapeutic treatment of Takayasu disease is immunotherapy. Approximately 50% of the patients respond well to corticosteroid therapy [6]. Invasive treatment of vascular problems or surgery should be performed at a time of quiescent disease to prevent complications.
2. Case report
From 1998 a 25-year-old female has been treated in our hospital with corticosteroids and antihypertensive medication because of weariness, high erythrocyte sedimentation rate (ESR), hypertension, loss of weight and periods of non-specific thoracic pain. For years, no diagnosis could be obtained and in time examinations revealed progressive dilatation of the ascending aorta (sinutubular junction 52 mm, ascending aorta 56 mm, arcus aortae 44 mm) without dilatation of the aorta annulus, thickening of the ascending aorta wall, left ventricular end diastolic diameter (LVEDD) of 63 mm, severe aortic regurgitation (AR) of 4/4 and diminished left ventricular function. The descending aorta was found to be normal.
Aorta dilatation with aortitis secondary to syphilis, Reiters syndrome or Beh?et syndrome was excluded. Tests for rheumatoid factor and antinuclear antibody were negative.
Surgery in the year 2000 showed wall abnormalities of the arcus and a normal diameter. Adhesions from the aorta to the pericardium were noticed. The aorta annular region was not dilated (24 mm) and the aortic cusps and Valsalva's sinuses were normal. A Vascutec prothesis (24 mm) was placed end-to-end distally of the sinutubular junction and implanted with combined reduction plasty of the sinutubular junction to perform a valve sparing reconstruction. The prothesis extended into the inner arcus aorta. At the end of the operation transesophageal echocardiography showed cusps with good coaptation, a small centrically aortic valve regurgitation of maximum grade 2/4 which we accepted.
Pathological examination of the aorta revealed intimal fibrocellular thickening, elastic degeneration of the tunica media and infiltration of inflammatory cells extending from the small media to the adventitial layer.
The patient was discharged with moderate AR (2–3/4).
The pathological findings combined with the persistent non-specific thoracic pain, despite control of the inflammatory reaction with steroid treatment, the progressive irregularities of the descending aorta vessel wall in 2000 and a positive somatostatin scan sign at the time of active inflammation [7], suggested Takayasu arteritis.
Dilatation and thoracoabdominal aorta vessel wall abnormalities and AR were progressive with abnormal left ventricular dimensions and persisting hypertension. In March 2004 she uneventfully delivered a healthy son. In August 2004 she was admitted because of severe non-specific thoracic pain and hypertension (170/117 mmHg). During hypertension management, she had a short period of diplégia that was assumed to be due to periods of relapsed relative hypotension.
Examinations revealed a poor left ventricular function, wall motion abnormalities, LVEDD of 65 mm, left ventricular end systolic diameter of 51 mm, AR (3–4/4) and a dilated descending aorta of 50 mm with abnormal and irregular origin of the coeliac trunc (CT) and superior mesenteric artery (SMA). A staged approach of aortic valve replacement and surgery for the thoracoabdominal aorta was planned.
At the time of operation the inflammation was, after treatment with a maintenance dosage of prednisone and imuran, in a relative quiescent phase: white blood cell count was 14090/mm3, C-reactive protein was 38 mg/dl and ESR was 14 mm. Medication did not result in completely normal values.
The aortic valve was replaced by a mechanical valve (St. Jude Medical 23 mm) in September 2004. The operation was uneventful and she could be weaned from mechanical ventilation 1 day after reoperation. She had elevated transaminase and lactate-dehydrogenesis (LDH) levels (GOT 2623 U/l, GPT 2303 U/l, LDH 4594 U/l), normal arterial blood gas values and no renal dysfunction. Later that day a dramatic increase of transaminase and LDH values appeared (GOT 7130 U/l, GPT 5041 U/l, LDH 8349 U/l). The arterial blood gas sample showed extreme metabolic acidosis (pH 7.13, pCO2 3.7 kPa, pO2 19.1 kPa, act.bicarb 8.8 mmol/l, std.bicarb 10.5 kPa, base excess – 19 mmol/l, O2sat 98%, lactate 18.0 mmol/l, ammonia 32 μmol/l). Because of a high probability of ischemia of abdominal organs, urgent abdominal surgery was performed. Extensive ischemia of the liver, gallbladder, small intestine and the proximal part of the colon were found. Because of the extensive regions of ischemia no surgical interventions were optional. The patient died one day after abdominal exploration.
Autopsy revealed an active inflammatory phase of Takayasu disease (Figs. 1 and 2). The thoracoabdominal aorta was dilated with extensive giant cell arteritis, tunica media destruction, intimal fibrocellular thickening and thrombosis. The CT and SMA were occluded due to inflammatory reaction and thrombosis.
3. Discussion
An aortic valve sparing operation in 2000 was performed because of the young age of the patient and her wish for pregnancy. In that setting, a grade 2/4 native AR was accepted, although implantation of an allograft conduit was contemplated [8]. During this operation the aortic arch already showed abnormalities.
Nevertheless, at that time the computed tomography scan did not reveal pathology of the descending aorta.
Consecutive computed tomography-scans from our patient revealed progressive thoracoabdominal aortic dilatation (5 cm) and irregular origin of the TC and SMA in the years after the aorta ascending replacement in 2000. The computed tomography scan before aortic valve replacement in 2004 revealed severe stenosis of the CT and SMA origin but the patient did not have abdominal complaints in the past or in the preoperative period. Even when the antihypertensive therapy was incremented the patient was free of abdominal complaints and without abnormal laboratory findings. On the other hand, she did develop transient diplégia without objectivation of a vascular cause.
Before the operation the patient had no abdominal complaints and the laboratory findings were normal in spite of severe abdominal main vessel stenosis.
We speculate that the specific alterations of normal extracorporeal circulation with non-pulsatile flow and reduced mean arterial pressure may have predisposed to intestinal ischemia, exaggerated by temporary further disturbance of the immune system.
We conclude that in Takayasu disease, scheduled for on pump cardiac surgery, vascular workup should be done and interventional treatment of asymptomatic but potentially critical lesions should be considered.
Acknowledgements
We acknowledge the illustrations by Dr. NNT Goemaere and clinical data by Dr. PLA van Daele.
References
Sheikhzadeh A, Tettenborn I, Noohi F, Eftekharzadeh M, Schnabel A. Occlusive thromboaortopathy (Takayasu disease): clinical and angiographic features and a brief review of literature. Angiology 2002; 53:29–40.
Fietta P. Systemic vasculitides: immunogenetics and familial clustering. Clin Exp Rheumatol 2004; 22:238–251.
Arend WP, Michel BA, Bloch DA, Hunder GG, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY, Lie JT, Lightfoot RW Jr. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum 1990; 33:1129–1134.
Moriwaki R, Noda M, Yajima M, Sharma BK, Numano F. Clinical manifestations of Takayasu arteritis in India and Japan – new classification of angiographic findings. Angiology 1997; 48:369–379.
Kissin E, Merkel P. Diagnostic imaging in Takayasu arteritis. Curr Opin Rheumatol 2004; 16:31–37.
Johnston SL, Lock RJ, Gompels MM. Takayasu arteritis: a review. J Clin Pathol 2002; 55:481–486.
Belhocine T, Blockmans D, Hustinx R, Vandevivere J, Mortelmans L. Imaging of large vessel vasculitis with FDG PET: illusion or reality? A critical review of the literature data. Eur J Nucl Med Mol Imaging 2003; 30:1305–1313.
Sarsam M, Yacoub M. Remodelling of the aortic valve annulus. J Thorac Cardiovasc Surg 1993; 105:435–438.
Related Article
ICVTS on-line discussion A
Mohamed Fahmy Ibrahim and Amal Refaat
Interactive CardioVascular and Thoracic Surgery 2006 5: 453.(Joost M. Hartmana, Jos A.)