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Midaortic syndrome: 30 years of experience with medical, endovascular and surgical management

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Abstract

Background

Midaortic syndrome is often associated with refractory hypertension. The aim of our study was to better understand the short- and medium-term outcomes in this patient population utilizing a multidisciplinary management approach.

Methods

We conducted a review of patients with midaortic syndrome treated at our institution over the past 30 years.

Results

Fifty-three patients presented at a median age of 6.7 years (birth to 28.7 years). Thirty-five patients (66 %) underwent invasive management (percutaneous techniques: 21; surgical techniques: 5; both: 9). Percutaneous interventions were acutely successful in decreasing the gradient across the obstruction and degree of luminal stenosis. However, freedom from reintervention was 58 % at 1 year and 33 % at 5 years. Freedom from reintervention after a surgical procedure was longer: 83 % at 1 year and 72 % at 10 years. At the most recent follow-up, the majority of patients (69 %) were normotensive. The median duration between time of presentation and achievement of blood pressure control was 5.7 (0.4–21.1) years. The median number of anti-hypertensive medications was 1 (0–5).

Conclusions

A multidisciplinary management strategy which couples comprehensive medical management with catheter-based and surgical interventions can lead to adequate blood pressure control and preservation of end-organ function in patients with midaortic syndrome.

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References

  1. Quain R (1847) Partial coarctation of the abdominal aorta. Trans Path Soc London 1:244–246

    Google Scholar 

  2. Sen PK, Kinare SG, Engineer SD, Parulkar GB (1963) The Middle Aortic Syndrome. Br Heart J 25:610–618

    Article  PubMed  CAS  Google Scholar 

  3. Hallett JW Jr, Brewster DC, Darling RC, O’Hara PJ (1980) Coarctation of the abdominal aorta: current options in surgical management. Ann Surg 191:430–437

    Article  PubMed  Google Scholar 

  4. Stanley JC, Criado E, Eliason JL, Upchurch GR Jr, Berguer R, Rectenwald JE (2008) Abdominal aortic coarctation: surgical treatment of 53 patients with a thoracoabdominal bypass, patch aortoplasty, or interposition aortoaortic graft. J Vasc Surg 48:1073–1082

    Article  PubMed  Google Scholar 

  5. D’Souza SJ, Tsai WS, Silver MM, Chait P, Benson LN, Silverman E, Hebert D, Balfe JW (1998) Diagnosis and management of stenotic aorto-arteriopathy in childhood. J Pediatr 132:1016–1022

    Article  PubMed  Google Scholar 

  6. Graham LM, Zelenock GB, Erlandson EE, Coran AG, Lindenauer SM, Stanley JC (1979) Abdominal aortic coarctation and segmental hypoplasia. Surgery 86:519–529

    PubMed  CAS  Google Scholar 

  7. Onat T, Zeren E (1969) Coarctation of the abdominal aorta. Review of 91 cases. Cardiologia 54:140–157

    Article  PubMed  CAS  Google Scholar 

  8. Senning A, Johansson L (1960) Coarctation of the abdominal aorta. J Thorac Cardiovasc Surg 40:517–523

    PubMed  CAS  Google Scholar 

  9. Ellis D, Shapiro R, Scantlebury VP, Simmons R, Towbin R (1995) Evaluation and management of bilateral renal artery stenosis in children: a case series and review. Pediatr Nephrol 9:259–267

    Article  PubMed  CAS  Google Scholar 

  10. Bergamini TM, Bernard JD, Mavroudis C, Backer CL, Muster AJ, Richardson JD (1995) Coarctation of the abdominal aorta. Ann Vasc Surg 9:352–356

    Article  PubMed  CAS  Google Scholar 

  11. De Bakey ME, Garrett HE, Howell JF, Morris GC (1969) Coarctation of the abdominal aorta with renal arterial stenosis: Surgical considerations. Ann Surg 165:830–843

    Article  Google Scholar 

  12. Delis KT, Gloviczki P (2005) Middle aortic syndrome: from presentation to contemporary open surgical and endovascular treatment. Perspect Vasc Surg Endovasc Ther 17:187–203

    Article  PubMed  Google Scholar 

  13. Giordano JM, Leavitt RY, Hoffman G, Fauci AS (1991) Experience with surgical treatment of Takayasu’s disease. Surgery 109:252–258

    PubMed  CAS  Google Scholar 

  14. Mickley V, Fleiter T (1998) Coarctations of descending and abdominal aorta: long-term results of surgical therapy. J Vasc Surg 28:206–214

    Article  PubMed  CAS  Google Scholar 

  15. Taketani T, Miyata T, Morota T, Takamoto S (2005) Surgical treatment of atypical aortic coarctation complicating Takayasu’s arteritis–experience with 33 cases over 44 years. J Vasc Surg 41:597–601

    Article  PubMed  Google Scholar 

  16. O’Neill JA Jr, Berkowitz H, Fellows KJ, Harmon CM (1995) Midaortic syndrome and hypertension in childhood. J Pediatr Surg 30:164–171, discussion 171–162

    Article  PubMed  Google Scholar 

  17. Sharma S, Bahl VK, Saxena A, Kothari SS, Talwar KK, Rajani M (1999) Stenosis in the aorta caused by non-specific aortitis: results of treatment by percutaneous stent placement. Clin Radiol 54:46–50

    Article  PubMed  CAS  Google Scholar 

  18. Sharma S, Shrivastava S, Kothari SS, Kaul U, Rajani M (1994) Influence of angiographic morphology on the acute and longer-term outcome of percutaneous transluminal angioplasty in patients with aortic stenosis due to nonspecific aortitis. Cardiovasc Intervent Radiol 17:147–151

    Article  PubMed  CAS  Google Scholar 

  19. Fava MP, Foradori GB, Garcia CB, Cruz FO, Aguilar JG, Kramer AS, Valdes FE (1993) Percutaneous transluminal angioplasty in patients with Takayasu arteritis: five-year experience. J Vasc Interv Radiol 4:649–652

    Article  PubMed  CAS  Google Scholar 

  20. Rao SA, Mandalam KR, Rao VR, Gupta AK, Joseph S, Unni MN, Subramanyan R, Neelakandhan KS (1993) Takayasu arteritis: initial and long-term follow-up in 16 patients after percutaneous transluminal angioplasty of the descending thoracic and abdominal aorta. Radiology 189:173–179

    PubMed  CAS  Google Scholar 

  21. Tyagi S, Kaul UA, Nair M, Sethi KK, Arora R, Khalilullah M (1992) Balloon angioplasty of the aorta in Takayasu’s arteritis: initial and long-term results. Am Heart J 124:876–882

    Article  PubMed  CAS  Google Scholar 

  22. Siwik ES, Perry SB, Lock JE (2003) Endovascular stent implantation in patients with stenotic aortoarteriopathies: early and medium-term results. Catheter Cardiovasc Interv 59:380–386

    Article  PubMed  Google Scholar 

  23. National High Blood Pressure Education Program Working Group on High Blood Pressure in Children and Adolescents (2004) The fourth report on the diagnosis, evaluation, and treatment of high blood pressure in children and adolescents. Pediatrics 114:555–576

    Google Scholar 

  24. Dionne JM, Abitbol CL, Flynn JT (2012) Hypertension in infancy: diagnosis, management and outcome. Pediatr Nephrol 27:17–32

    Article  PubMed  Google Scholar 

  25. Whitworth JA (2003) 2003 World Health Organization (WHO)/International Society of Hypertension (ISH) statement on management of hypertension. J Hypertens 21:1983–1992

    Article  PubMed  Google Scholar 

  26. Schwartz GJ, Haycock GB, Edelmann CM Jr, Spitzer A (1976) A simple estimate of glomerular filtration rate in children derived from body length and plasma creatinine. Pediatrics 58:259–263

    PubMed  CAS  Google Scholar 

  27. Stevens LA, Coresh J, Greene T, Levey AS (2006) Assessing kidney function—measured and estimated glomerular filtration rate. N Engl J Med 354:2473–2483

    Article  PubMed  CAS  Google Scholar 

  28. Khan MS, Moore JW (2000) Treatment of abdominal aortic pseudoaneurysm with covered stents in a pediatric patient. Catheter Cardiovasc Interv 50:445–448

    Article  PubMed  CAS  Google Scholar 

  29. Kim HB, Vakili K, Modi BP, Ferguson MA, Guillot AP, Potanos KM, Prabhu SP, Fishman SJ (2012) A novel treatment for the midaortic syndrome. N Engl J Med 367:2361–2362

    Article  PubMed  CAS  Google Scholar 

  30. Lillehei CW, Shamberger RC (2001) Staged reconstruction for middle aortic syndrome. J Pediatr Surg 36:1252–1254

    Article  PubMed  CAS  Google Scholar 

  31. Friedman JM, Arbiser J, Epstein JA, Gutmann DH, Huot SJ, Lin AE, McManus B, Korf BR (2002) Cardiovascular disease in neurofibromatosis 1: report of the NF1 Cardiovascular Task Force. Genet Med 4:105–111

    Article  PubMed  CAS  Google Scholar 

  32. Hamilton SJ, Friedman JM (2000) Insights into the pathogenesis of neurofibromatosis 1 vasculopathy. Clin Genet 58:341–344

    Article  PubMed  CAS  Google Scholar 

  33. Lin AE, Birch PH, Korf BR, Tenconi R, Niimura M, Poyhonen M, Armfield Uhas K, Sigorini M, Virdis R, Romano C, Bonioli E, Wolkenstein P, Pivnick EK, Lawrence M, Friedman JM (2000) Cardiovascular malformations and other cardiovascular abnormalities in neurofibromatosis 1. Am J Med Genet 95:108–117

    Article  PubMed  CAS  Google Scholar 

  34. Oderich GS, Sullivan TM, Bower TC, Gloviczki P, Miller DV, Babovic-Vuksanovic D, Macedo TA, Stanson A (2007) Vascular abnormalities in patients with neurofibromatosis syndrome type I: clinical spectrum, management, and results. J Vasc Surg 46:475–484

    Article  PubMed  Google Scholar 

  35. Sorof JM, Cardwell G, Franco K, Portman RJ (2002) Ambulatory blood pressure and left ventricular mass index in hypertensive children. Hypertension 39:903–908

    Article  PubMed  CAS  Google Scholar 

  36. Levy D, Garrison RJ, Savage DD, Kannel WB, Castelli WP (1990) Prognostic implications of echocardiographically determined left ventricular mass in the Framingham Heart Study. N Engl J Med 322:1561–1566

    Article  PubMed  CAS  Google Scholar 

  37. Koren MJ, Devereux RB, Casale PN, Savage DD, Laragh JH (1991) Relation of left ventricular mass and geometry to morbidity and mortality in uncomplicated essential hypertension. Ann Intern Med 114:345–352

    Article  PubMed  CAS  Google Scholar 

  38. Brown DW, Giles WH, Croft JB (2000) Left ventricular hypertrophy as a predictor of coronary heart disease mortality and the effect of hypertension. Am Heart J 140:848–856

    Article  PubMed  CAS  Google Scholar 

  39. Tummolo A, Marks SD, Stadermann M, Roebuck DJ, McLaren CA, Hamilton G, Dillon MJ, Tullus K (2009) Mid-aortic syndrome: long-term outcome of 36 children. Pediatr Nephrol 24:2225–2232

    Article  PubMed  Google Scholar 

  40. Stanley JC, Graham LM, Whitehouse WM Jr, Zelenock GB, Erlandson EE, Cronenwett JL, Lindenauer SM (1981) Developmental occlusive disease of the abdominal aorta and the splanchnic and renal arteries. Am J Surg 142:190–196

    Article  PubMed  CAS  Google Scholar 

  41. Ishii K, Isono M, Kasai N, Nakano T, Kubo T, Inoue R, Nomura Y (2001) Midaortic syndrome in childhood associated with a ruptured cerebral aneurysm: a case report. Surg Neurol 55:209–212

    Article  PubMed  CAS  Google Scholar 

  42. Sethna CB, Kaplan BS, Cahill AM, Velazquez OC, Meyers KE (2008) Idiopathic mid-aortic syndrome in children. Pediatr Nephrol 23:1135–1142

    Article  PubMed  Google Scholar 

  43. Sharma S, Thatai D, Saxena A, Kothari SS, Guleria S, Rajani M (1996) Renovascular hypertension resulting from nonspecific aortoarteritis in children: midterm results of percutaneous transluminal renal angioplasty and predictors of restenosis. Am J Roentgenol 166:157–162

    Article  CAS  Google Scholar 

  44. Messina LM, Reilly LM, Goldstone J, Ehrenfeld WK, Ferrell LD, Stoney RJ (1986) Middle aortic syndrome. Effectiveness and durability of complex arterial revascularization techniques. Ann Surg 204:331–339

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Department of Cardiology, Boston Children’s Hospital and Harvard Medical School, Boston, MA, 02115, USA

    Diego Porras & James E. Lock

  2. Division of Nephrology, Boston Children’s Hospital and Harvard Medical School, Boston, MA, USA

    Deborah R. Stein & Michael A. Ferguson

  3. Division of Interventional Radiology, Boston Children’s Hospital and Harvard Medical School, Boston, MA, USA

    Gulraiz Chaudry & Ahmad Alomari

  4. Department of Surgery, Boston Children’s Hospital and Harvard Medical School, Boston, MA, USA

    Khashayar Vakili, Steven J. Fishman & Heung B. Kim

  5. Department of Cardiology, Boston Children’s Hospital, 300 Longwood Avenue, Boston, MA, 02115, USA

    Diego Porras

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  1. Diego Porras
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  2. Deborah R. Stein
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  3. Michael A. Ferguson
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Correspondence to Diego Porras.

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Porras, D., Stein, D.R., Ferguson, M.A. et al. Midaortic syndrome: 30 years of experience with medical, endovascular and surgical management. Pediatr Nephrol 28, 2023–2033 (2013). https://doi.org/10.1007/s00467-013-2514-8

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  • DOI: https://doi.org/10.1007/s00467-013-2514-8

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