Aggarwal, Puneet

Himanshu, Kumar

Krishna, Vinay

Razi, Mahmodula

Samrat, Siddarth

Sinha, Santosh Kumar

English

Via Medica Journals

  ONLINE FIRSTThis is a provisional PDF only. Copyedited and fully formatted version will be made available soon.ISSN: 2353-7752e-ISSN: 2353-7760Juvenile Takayasu arteritis presenting with Aortic dissection asinitial presentationAuthors:  Santosh Kumar Sinha, Puneet Aggarwal, Mahmodula Razi, KumarHimanshu, Siddarth Samrat, Vinay KrishnaDOI: 10.5603/FC.a2019.0080Article type: Images In CardiologySubmitted: 2019-01-08Accepted: 2019-01-14Published online: 2019-07-02This article has been peer reviewed and published immediately upon acceptance.It is an open access article, which means that it can be downloaded, printed, and distributed freely,provided the work is properly cited. Powered by TCPDF (www.tcpdf.org)Juvenile Takayasu arteritis presenting with Aortic dissection as initial presentation  Santosh Kumar Sinha, Puneet Aggarwal, Mahmodula Razi, Kumar Himanshu, Siddarth Samrat, Vinay Krishna Department of Cardiology, LPS Institute of Cardiology, G.S.V.M. Medical College, Kanpur, India   A 6-year-old girl presented for evaluation of low grade fever, loss of appetite and weight, and breathlessness for 6weeks. On examination, blood pressure in left arm was 124/88 mmHg, in left arm was 80/58 mmHg while pulses in both lower limbs were weak. According to these observations, the diagnosis of Takayasu arteritis was established. Haemogram was within normal range except for mild anaemia (Hb-9.8 mg/dL), elevated erythrocyte sediment rate (49mm/hr), and raised C — reactive protein (CRP-99mg/L). Chest radiography was normal. Electrocardiogram revealed left ventricular hypertrophy with sinus tachycardia. Echocardiography showed dilated left atria and ventricle, global hypokinesia with severe impairment of systolic function (EF-20%). Multidetector computed tomography scan (MDCT) of aorta showed mild diffuse narrowing of thoraco-abdominal segment with severe narrowing of distal abdominal segment in infra renal segment (Fig. 1A, B). Total occlusion of right brachio-cephalic truck and critical ostial stenosis of left common carotid artery were also seen. A spiral flap suggesting aortic dissection (DeBakey type II; Stanford type III) from the descending aorta (T7–T8 vertebral level) to the distal abdominal aorta was also seen which was further confirmed on contrast aortogram (Fig. 1, 2). Therefore, she was clinically diagnosed as Takayasu Arteritis with aortic dissection (DeBakey type II) based on the EULAR (European League against Rheumatism)/PRES (Paediatric Rheumatology European Society) criteria [1]. Patient succumbed to death because of progressive failure and ventricular tachycardia.  Takayasu arteritis, as reported for first time by Mikito Takayasu in 1908, causes segmental stenosis, dilatation, or aneurysm formation which generally affects the large vessels [2]. Although it can be diagnosed and classified on the basis of EULAR criteria, assessment of the inflammatory status and pattern of vascular involvement is still a major challenge [3]. Clinically TA activity can be assessed by acute-phase reactants (ESR or CRP), new bruits, and new angiographic features. TA causes persistent inflammation of the aorta, but dissection is exceedingly rare because of the dense adventitial fibrosis and intimal scarring, though decreased wall elasticity and aortic mobility due to fibrous adhesion between fibrous adventitia and surrounding tissue may be the reason for dissection in TA [4, 5]. Arteriosclerosis, long-term hypertension, dyslipidemia, and associated autoimmune connective tissue disorders may be the other antecedent factors. In our case, patient was a young girl who got admitted to hospital with aortic dissection as initial presentation and diagnosed with TA after further evaluation. As it was type III DeBakey dissection, patient was managed with medical treatment including decongestive therapy. Also she was in active phase of the disease and before glucocorticoid therapy and immunosuppressive therapy could be started, she succumbed to death because of ventricular arrhythmia. This was also reason; thoracic endovascular aortic repair was not ventured as it had great risks during the active (progressive) stage of TA. In general, TA patients with severe ischemic symptoms (limb claudication, heart failure, etc.) require endovascular intervention or vascular surgery in addition to medical therapy although results are conflicting regarding rate of restenosis and occlusion [6, 7]. Key words: Juvenile Takayasu arteritis; Aortic dissection, Sudden death  Learning points-  References 1. Ozen S, Ruperto N, Dillon MJ, et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis. 2006; 65(7): 936–941, doi: 10.1136/ard.2005.046300, indexed in Pubmed: 16322081. 2. Direskeneli H. Clinical assessment in Takayasu's arteritis: major challenges and controversies. Clin Exp Rheumatol. 2017; 35 Suppl 103(1): 189–193, indexed in Pubmed: 28375833. 3. Keser G, Direskeneli H, Aksu K. Management of Takayasu arteritis: a systematic review. Rheumatology (Oxford). 2014; 53(5): 793–801, doi: 10.1093/rheumatology/ket320, indexed in Pubmed: 24097290. 4. Tyagi S, Nigam A. Aortic dissection: a rare presenting manifestation of Takayasu's aortitis. Indian Heart J. 2008; 60(1): 58–60, indexed in Pubmed: 19212025. 5. Kameyama K, Kuramochi S, Ueda T, et al. Takayasu's aortitis with dissection in systemic lupus erythematosus. Scand J Rheumatol. 1999; 28(3): 187–188, indexed in Pubmed: 10380843. 6. Seyahi E. Takayasu arteritis: an update. Curr Opin Rheumatol. 2017; 29(1): 51–56, doi: 10.1097/BOR.0000000000000343, indexed in Pubmed: 27748689. 7. Fields CE, Bower TC, Cooper LT, et al. Takayasu's arteritis: operative results and influence of disease activity. J Vasc Surg. 2006; 43(1): 64–71, doi: 10.1016/j.jvs.2005.10.010, indexed in Pubmed: 16414389.  Figure legends Figure 1. MDCT of aorta showing total occlusion of right brachio-cephalic truck and critical ostial stenosis of left common carotid artery (red arrow), diffuse narrowing of thoraco-abdominal segment with severe narrowing of distal abdominal aorta involving infra renal segment. Also seen is spiral flap (white arrow) suggesting aortic dissection (DeBakey type II) Figure 2. Contrast aortogram showing spiral flap suggesting aortic dissection of descending aorta (DeBakey type II; Stanford type III)  

Młodzieńcze zapalenie tętnic Takayasu u dziecka z rozwarstwieniem aorty jako pierwszym objawem choroby

https://journals.viamedica.pl/folia_cardiologica/article/download/FC.a2019.0080/48733

Młodzieńcze zapalenie tętnic Takayasu u dziecka z rozwarstwieniem aorty jako pierwszym objawem choroby

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