Highlights & Basics
- Aortic coarctation is characterized by a blood pressure differential between upper and lower extremities (upper >lower).
- In neonates, may present with shock if there is inadequate systemic perfusion once the ductus arteriosus closes.
- Physical exam may show diminished, absent, or delayed lower extremity pulses compared with upper extremity pulses.
- Diagnosis is made by demonstration of aortic arch narrowing, typically by echocardiography.
- Treatment may be surgical repair or percutaneous balloon dilation or stent placement.
Quick Reference
History & Exam
Key Factors
Other Factors
Diagnostics Tests
Treatment Options
Definition
Epidemiology
Etiology
Pathophysiology
Images
Abdominal coarctation
Two-dimensional suprasternal notch view of the aortic arch. Focal narrowing of the aortic arch (arrow) in the typical juxtaductal region
Addition of color Doppler shows turbulent higher velocity flow after the obstructed area
Three-dimensional computed tomographic reconstruction visualized from posterior to anterior. The area of coarctation is well seen (arrow)
Angiography in the ascending aorta shows a focal area of narrowing after the left subclavian artery
Two-dimensional suprasternal notch view of the aortic arch. Focal narrowing of the aortic arch (arrow) in the typical juxtaductal region
Addition of color Doppler shows turbulent higher velocity flow after the obstructed area
Three-dimensional computed tomographic reconstruction visualized from posterior to anterior. The area of coarctation is well seen (arrow)
Angiography in the ascending aorta shows a focal area of narrowing after the left subclavian artery
After stent placement, the narrowed area is markedly improved
Aortic angiography in an infant after surgical reconstruction of the aortic arch shows marked narrowing at the distal aspect of the surgical repair
Balloon angioplasty of the narrowed region leads to complete resolution of the obstruction
Citations
Erbel R, Aboyans V, Boileau C, et al; Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC). 2014 ESC guidelines on the diagnosis and treatment of aortic diseases: document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. Eur Heart J. 2014 Nov 1;35(41):2873-926.[Abstract][Full Text]
Writing Committee Members, Isselbacher EM, Preventza O, et al. 2022 ACC/AHA Guideline for the diagnosis and management of aortic disease: A report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol. 2022 Dec 13;80(24):e223-e393.[Abstract][Full Text]
Baumgartner H, De Backer J, Babu-Narayan SV, et al. 2020 ESC guidelines for the management of adult congenital heart disease. Eur Heart J. 2020 Aug 29;ehaa554.[Full Text]
Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on clinical practice guidelines. J Am Coll Cardiol. 2019 Apr 2;73(12):e81-e192.[Abstract][Full Text]
Writing Committee; Riambau V, Böckler D, Brunkwall J, et al. Editor's choice - management of descending thoracic aorta diseases: clinical practice guidelines of the European Society for Vascular Surgery (ESVS). Eur J Vasc Endovasc Surg. 2017 Jan;53(1):4-52.[Abstract][Full Text]
1. Amato JJ, Galdieri RJ, Cortoneo JV. Role of extended aortoplasty related to the definition of coarctation of the aorta. Ann Thorac Surg. 1991 Sep;52(3):615-20.[Abstract]
2. Connolly HM, Huston J 3rd, Brown RD Jr, et al. Intracranial aneurysms in patients with coarctation of the aorta: a prospective magnetic resonance angiographic study of 100 patients. Mayo Clin Proc. 2003 Dec;78(12):1491-9.[Abstract]
3. McBride KL, Pignatelli R, Lewin M, et al. Inheritance analysis of congenital left ventricular outflow tract obstruction malformations: segregation, multiplex relative risk, and heritability. Am J Med Genet A. 2005 Apr 15;134A(2):180-6.[Abstract][Full Text]
4. Wessels, MW, Berger, RM, Frohn-Mulder IM, et al. Autosomal dominant inheritance of left ventricular outflow tract obstruction. Am J Med Genet A. 2005 Apr 15;134A(2):171-9.[Abstract]
5. Erbel R, Aboyans V, Boileau C, et al; Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC). 2014 ESC guidelines on the diagnosis and treatment of aortic diseases: document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. Eur Heart J. 2014 Nov 1;35(41):2873-926.[Abstract][Full Text]
6. Rudolph AM, Heymann MA, Spitznas U. Hemodynamic considerations in the development of narrowing of the aorta. Am J Cardiol. 1972 Oct;30(5):514-25.[Abstract]
7. Pagni S, Denatale RW, Boltax RS. Takayasu's arteritis: the middle aortic syndrome. Am Surg. 1996 May;62(5):409-12.[Abstract]
8. Abbruzzese PA, Aidala E. Aortic coarctation: an overview. J Cardiovasc Med (Hagerstown). 2007 Feb;8(2):123-8.[Abstract]
9. Attenhofer Jost CH, Schaff HV, Connolly HM, et al. Spectrum of reoperations after repair of aortic coarctation: importance of an individualized approach because of coexistent cardiovascular disease. Mayo Clin Proc. 2002 Jul;77(7):646-53.[Abstract]
10. McBride KL, Marengo L, Canfield M, et al. Epidemiology of noncomplex left ventricular outflow tract obstruction malformations (aortic valve stenosis, coarctation of the aorta, hypoplastic left heart syndrome) in Texas, 1999-2001. Birth Defects Res A Clin Mol Teratol. 2005 Aug;73(8):555-61.[Abstract][Full Text]
11. Sybert VP. Cardiovascular malformations and complications in Turner syndrome. Pediatrics. 1998 Jan;101(1):E11.[Abstract][Full Text]
12. Frieden IJ, Reese V, Cohen D. PHACE syndrome: the association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects and eye abnormalities. Arch Dermatol. 1996 Mar;132(3):307-11.[Abstract]
13. Metry DW, Garzon MC, Drolet BA, et al. PHACE syndrome: current knowledge, future directions. Pediatr Dermatol. 2009 Jul-Aug;26(4):381-98.[Abstract]
14. Writing Committee Members, Isselbacher EM, Preventza O, et al. 2022 ACC/AHA Guideline for the diagnosis and management of aortic disease: A report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol. 2022 Dec 13;80(24):e223-e393.[Abstract][Full Text]
15. Baumgartner H, De Backer J, Babu-Narayan SV, et al. 2020 ESC guidelines for the management of adult congenital heart disease. Eur Heart J. 2020 Aug 29;ehaa554.[Full Text]
16. Wasserman MA, Shea E, Cassidy C, et al. Recommendations for the adult cardiac sonographer performing echocardiography to screen for critical congenital heart disease in the newborn: from the American Society of Echocardiography. J Am Soc Echocardiogr. 2021 Mar;34(3):207-22.[Abstract][Full Text]
17. American College of Radiology. ACR Appropriateness Criteria®: congenital or acquired heart disease. 2023 [internet publication].[Full Text]
18. Snyder AR, Sewer GA, Ritter SB. Echocardiography in pediatric heart disease. St Louis, MO: Mosby; 1997:478-482.
19. Hager A, Kaemmerer H, Leppert A, et al. Follow-up of adults with coarctation of the aorta: comparison of helical CT and MRI, and impact on assessing diameter changes. Chest. 2004 Oct;126(4):1169-76.[Abstract]
20. Shih MC, Tholpady A, Kramer CM, et al. Surgical and endovascular repair of aortic coarctation: normal findings and appearance of complications on CT angiography and MR angiography. AJR Am J Roentgenol. 2006 Sep;187(3):W302-12.[Abstract][Full Text]
21. Wong KK, Fournier A, Fruitman DS, et al. Canadian Cardiovascular Society/Canadian Pediatric Cardiology Association position statement on pulse oximetry screening in newborns to enhance detection of critical congenital heart disease. Can J Cardiol. 2017 Feb;33(2):199-208.[Abstract][Full Text]
22. Mahle WT, Newburger JW, Matherne GP, et al. Role of pulse oximetry in examining newborns for congenital heart disease: a scientific statement from the American Heart Association and American Academy of Pediatrics. Circulation. 2009 Aug 4;120(5):447-58.[Abstract][Full Text]
23. Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on clinical practice guidelines. J Am Coll Cardiol. 2019 Apr 2;73(12):e81-e192.[Abstract][Full Text]
24. Writing Committee; Riambau V, Böckler D, Brunkwall J, et al. Editor's choice - management of descending thoracic aorta diseases: clinical practice guidelines of the European Society for Vascular Surgery (ESVS). Eur J Vasc Endovasc Surg. 2017 Jan;53(1):4-52.[Abstract][Full Text]
25. Pádua LM, Garcia LC, Rubira CJ, et al. Stent placement versus surgery for coarctation of the thoracic aorta. Cochrane Database Syst Rev. 2012 May 16;(5):CD008204.[Abstract][Full Text]
26. Forbes TJ, Kim DW, Du W, et al; CCISC Investigators. Comparison of surgical, stent, and balloon angioplasty treatment of native coarctation of the aorta: an observational study by the CCISC (Congenital Cardiovascular Interventional Study Consortium). J Am Coll Cardiol. 2011 Dec 13;58(25):2664-74.[Abstract]
27. Fiore AC, Fischer LK, Schwartz T, et al. Comparison of angioplasty and surgery for neonatal aortic coarctation. Ann Thorac Surg. 2005 Nov;80(5):1659-64.[Abstract]
28. Wright GE, Nowak CA, Goldberg CS, et al. Extended resection and end-to-end anastomosis for aortic coarctation in infants: results of a tailored surgical approach. Ann Thorac Surg. 2005 Oct;80(4):1453-9.[Abstract]
29. Pandey R, Jackson M, Ajab S, et al. Subclavian flap repair: review of 399 patients at median follow-up of fourteen years. Ann Thorac Surg. 2006 Apr;81(4):1420-8.[Abstract]
30. Vohra HA, Adamson L, Haw MP. Does surgical correction of coarctation of the aorta in adults reduce established hypertension? Interact Cardiovasc Thorac Surg. 2009 Jan;8(1):123-7.[Abstract][Full Text]
31. Backer CL, Stewart RD, Kelle AM, et al. Use of partial cardiopulmonary bypass for coarctation repair through a left thoracotomy in children without collaterals. Ann Thorac Surg. 2006 Sep;82(3):964-72.[Abstract]
32. Shah L, Hijazi Z, Sandhu S, et al. Use of endovascular stents for the treatment of coarctation of the aorta in children and adults: immediate and midterm results. J Invasive Cardiol. 2005 Nov;17(11):614-8.[Abstract]
33. Mahadevan VS, Vondermuhll IF, Mullen MJ. Endovascular aortic coarctation stenting in adolescents and adults: angiographic and hemodynamic outcomes. Catheter Cardiovasc Interv. 2006 Feb;67(2):268-75.[Abstract]
34. National Institute for Health and Care Excellence. Balloon angioplasty with or without stenting for coarctation or recoarctation of the aorta in adults and children. July 2004 [internet publication].[Full Text]
35. Golden AB, Hellenbrand WE. Coarctation of the aorta: stenting in children and adults. Catheter Cardiovasc Interv. 2007 Feb 1;69(2):289-99.[Abstract]
36. Wong D, Benson LN, Van Arsdell GS, et al. Balloon angioplasty is preferred to surgery for aortic coarctation. Cardiol Young. 2008 Feb;18(1):79-88.[Abstract]
37. Hu ZP, Wang ZW, Dai XF, et al. Outcomes of surgical versus balloon angioplasty treatment for native coarctation of the aorta: a meta-analysis. Ann Vasc Surg. 2014 Feb;28(2):394-403.[Abstract]
38. Wu Y, Jin X, Kuang H, et al. Is balloon angioplasty superior to surgery in the treatment of paediatric native coarctation of the aorta: a systematic review and meta-analysis. Interact Cardiovasc Thorac Surg. 2019 Feb 1;28(2):291-300.[Abstract][Full Text]
39. Pedra CA, Fontes VF, Esteves CA, et al. Stenting vs. balloon angioplasty for discrete unoperated coarctation of the aorta in adolescents and adults. Catheter Cardiovasc Interv. 2005 Apr;64(4):495-506.[Abstract]
40. Marelli A, Beauchesne L, Colman J, et al. Canadian Cardiovascular Society 2022 guidelines for cardiovascular interventions in adults with congenital heart disease. Can J Cardiol. 2022 Apr;38(7):862-96.[Full Text]
41. Carr JA. The results of catheter-based therapy compared with surgical repair of adult aortic coarctation. J Am Coll Cardiol. 2006 Mar 21;47(6):1101-7.[Abstract]
42. Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2019 Apr 2;139(14):e698-e800.[Abstract][Full Text]
43. Moltzer E, Mattace Raso FU, Karamermer Y, et al. Comparison of Candesartan versus Metoprolol for treatment of systemic hypertension after repaired aortic coarctation. Am J Cardiol. 2010 Jan 15;105(2):217-22.[Abstract]
44. von KY, Aydin AM, Bernhardt AM, et al. Aortic aneurysms after correction of aortic coarctation: a systematic review. Vasa. 2010;39:3-16.
45. Canniffe C, Ou P, Walsh K, et al. Hypertension after repair of aortic coarctation: a systematic review. Int J Cardiol. 2013 Sep 10;167(6):2456-61.[Abstract]
46. Toro-Salazar OH, Steinberger J, Thomas W, et al. Long-term follow-up of patients after coarctation of the aorta repair. Am J Cardiol. 2002 Mar 1;89(5):541-7.[Abstract]
47. Kovacs AH, Brouillette J, Ibeziako P, et al. Psychological outcomes and interventions for individuals with congenital heart disease: a scientific statement from the American Heart Association. Circ Cardiovasc Qual Outcomes. 2022 Aug;15(8):e000110.[Full Text]
48. de Bono J, Freeman LJ. Aortic coarctation repair - lost and found: the role of local long term specialised care. Int J Cardiol. 2005 Sep 30;104(2):176-83.[Abstract]
49. John AS, Jackson JL, Moons P, et al. Advances in managing transition to adulthood for adolescents with congenital heart disease: a practical approach to transition program design: a scientific statement from the American Heart Association. J AM Heart Assoc. 2022 Apr;11(7)[Full Text]
50. Habib G, Lancellotti P, Antunes MJ, et al. 2015 ESC guidelines for the management of infective endocarditis. Eur Heart J. 2015 Nov 21;36(44):3075-128.[Abstract][Full Text]
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