Aortic Coarctation: Practice Essentials, Background, Pathophysiology (original) (raw)

Practice Essentials

Aortic coarctation is a narrowing of the aorta most commonly found just distal to the origin of the left subclavian artery. The vascular malformation responsible for coarctation is a defect in the vessel media, giving rise to a prominent posterior infolding (the “posterior shelf”), which may extend around the entire circumference of the aorta.

Signs and symptoms

Symptoms of aortic coarctation may include the following:

The diagnosis of coarctation generally can be made on the basis of physical examination. Blood pressure differential and pulse delay are pathognomonic. The following physical findings may be noted:

See Presentation for more detail.

Diagnosis

No specific laboratory tests are necessary for coarctation of the aorta. Imaging studies that may be helpful include the following:

Other studies that may be useful are as follows:

See Workup for more detail.

Management

Medical treatment of neonates with severe aortic coarctation may include the following:

Medical treatment of less severe aortic coarctation beyond the neonatal period may include the following:

At present, the following 3 specific indications exist for intervention:

The following surgical procedures have been performed to treat aortic coarctation:

Catheter-based intervention is now the preferred therapy for recurrent coarctation when the anatomy permits and necessary skills are available. Its use in native or unoperated coarctation is less well established.

See Treatment and Medication for more detail.

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Background

Coarctation of the aorta (CoA), a narrowing of the aorta most commonly found just distal to the origin of the left subclavian artery, is a common condition found in children. Most patients with coarctation have juxtaductal coarctation. Older terms, such as preductal (infantile-type) or postductal (adult-type), are often misleading.

This condition occurs in 40 to 50 of every 100,000 live births and has a male-to-female predominance of 2:1. [1] Aortic coarctation is commonly treated after birth or during childhood.

Coarctation of the aorta is rarely seen in adults [1, 2, 3] However, when affected adults present, they may have a history of a previous coarctation procedure, rupture of an old repair, heart failure, aortic aneurysm, aortic dissection, undersized grafts of previous repairs, intracranial hemorrhage, hypertension with exercise, and infections. [1]

The prognosis for untreated aortic coarctation is poor. About 80% of untreated patients die of aortic dissection or rupture, heart failure, or intracranial hemorrhage. [1] The traditional treatment for coarctation of the aorta is open surgery. A less-invasive treatment option is endovascular balloon dilatation and stent placement. [1]

See the Guidelines section for a summary of guidelines for the management of aortic coarctation in adults.

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Pathophysiology

The vascular malformation responsible for aortic coarctation is a defect in the vessel media, giving rise to a prominent posterior infolding (the "posterior shelf"), which may extend around the entire circumference of the aorta. The gross pathology of coarctation varies considerably. The lesion is often discrete but may be long, segmental, or tortuous in nature.

Histology

The coarctated aortic segment reveals an intimal and medial lesion consisting of thickened ridges that protrude posteriorly and laterally into the aortic lumen. The ductus (ie, patent embryonic remnant) or ligamentum arteriosus (closed and fibrosed) inserts at the same level anteromedially. Intimal proliferation and disruption of elastic tissue may occur distal to the coarctation. At this site, infective endarteritis, intimal dissections, or aneurysms may occur. Cystic medial necrosis occurs commonly in the aorta adjacent to the coarctation site and acts as a substrate for late aneurysm formation or aortic dissection in some patients.

Embryology

Coarctation is due to an abnormality in development of the embryologic left fourth and sixth aortic arches that can be explained by two theories, the ductus tissue theory and the hemodynamic theory.

In the ductus tissue theory, coarctation develops as the result of migration of ductus smooth muscle cells into the periductal aorta, with subsequent constriction and narrowing of the aortic lumen. Commonly, coarctation becomes clinically evident with closure of the ductus arteriosus. This theory does not explain all cases of coarctation. Clinically, coarctation may occur in the presence of a widely patent ductus arteriosus, and it may occur quite distant from the insertion of the ductus arteriosus, such as in the transverse arch or abdominal aorta.

In the hemodynamic theory, coarctation results from the reduced volume of blood flow through the fetal aortic arch and isthmus. In a normal fetus, the aortic isthmus receives a relatively low volume of blood flow. Most of the flow to the descending aorta is derived from the right ventricle through the ductus arteriosus. The left ventricle supplies blood to the ascending aorta and brachiocephalic arteries, and a small portion goes to the aortic isthmus. The aortic isthmus diameter is 70%-80% of the diameter of the neonatal ascending aorta.

Based on this theory, lesions that diminish the volume of left ventricular outflow in the fetus also decrease flow across the aortic isthmus and promote development of coarctation. This helps to explain the common lesions associated with coarctation, such as ventricular septal defect, bicuspid aortic valve, left ventricular outflow obstruction, and tubular hypoplasia of the transverse aortic arch. However, this theory does not explain isolated coarctation without associated intracardiac lesions.

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Etiology

The exact etiology of coarctation of the aorta is not known. Note the following:

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Epidemiology

Coarctation of aorta represents 5%-8% of all congenital heart diseases, [6, 7] with the isolated form comprising 4%-6% of all congenital heart diseases. [8] The prevalence of isolated forms is about 3-4 per 10,000 live births, [6, 8] and males are affected more frequently than females. [9]

Aortic coarctation is seven times more common in white persons than Asian persons. It has a lower incidence among Native Americans than other population groups in Minnesota.

The male-to-female predominance is 1.3-2:1 in most series.

The age at detection of coarctation of the aorta is dependent on the severity of the obstruction and the coexistence of other lesions.

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Prognosis

Patients who are not treated for coarctation of the aorta may reach the age of 35 years [10] ; about 25% survive to age 46 years, [10] and fewer than 20% survive to age 50 years. If coarctation is repaired before the age of 14 years, the 20-year survival rate is 91%. If coarctation is repaired after the age of 14 years, the 20-year survival rate is 79%. The 30-year survival rate is almost doubled with surgical repair, with 72%-98% of these patients reaching adulthood. [11]

After repair of coarctation of the aorta, 97%-98% of patients are NYHA class I. Impaired diastolic left ventricular function and persistent hypertrophy due to increased pressure gradient at the coarctation site during exercise may result in myocardial hypertrophy despite successful hemodynamic results. Overall, left ventricular systolic function is normal or hyperdynamic in these patients

Most women reach childbearing age. If maternal coarctation is not repaired, risks to fetus and mother are increased. The maternal mortality rate is approximately 3%-8%. Note the following:

Complications

Late complications of aortic coarctation include recurrent coarctation, malignant hypertension, left ventricular dysfunction, aortic valve dysfunction, and aneurysm formation with risk of rupture. [11]

Postoperative complications include the following:

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Patient Education

Most adults with aortic coarctation have previously undergone repair; however, continued education regarding exercise, endocarditis and endarteritis prevention, and pregnancy issues is necessary.

For the rare adult with uncorrected coarctation, extensive patient education is necessary on issues ranging from pathology and repair to lifestyle modification and follow-up care.

The medical practitioner must understand that coarctation is a complex lifelong condition that may be repaired but is never truly corrected.

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  1. [Guideline] Hiratzka LF, Bakris GL, Beckman, et al. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the diagnosis and management of patients with thoracic aortic disease. A report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology,American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, S... J Am Coll Cardiol. 2010 Apr 6. 55(14):e27-e129. [QxMD MEDLINE Link]. [Full Text].
  2. Lala S, Scali ST, Feezor RJ, et al. Outcomes of thoracic endovascular aortic repair in adult coarctation patients. J Vasc Surg. 2018 Feb. 67 (2):369-81.e2. [QxMD MEDLINE Link].
  3. Nakamura E, Nakamura K, Furukawa K, Ishii H, Kawagoe K. Selection of a surgical treatment approach for aortic coarctation in adolescents and adults. Ann Thorac Cardiovasc Surg. 2018 Feb 16. [QxMD MEDLINE Link].
  4. Miettinen OS, Reiner ML, Nadas AS. Seasonal incidence of coarctation of the aorta. Br Heart J. 1970 Jan. 32 (1):103-7. [QxMD MEDLINE Link]. [Full Text].
  5. Weinstein BM, Stemple DL, Driever W, Fishman MC. Gridlock, a localized heritable vascular patterning defect in the zebrafish. Nat Med. 1995 Nov. 1 (11):1143-7. [QxMD MEDLINE Link].
  6. [Guideline] Erbel R, Aboyans V, Boileau C, et al, for the ESC Committee for Practice Guidelines. 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. The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC). Eur Heart J. 2014 Nov 1. 35 (41):2873-926. [QxMD MEDLINE Link]. [Full Text].
  7. Bugeja J, Cutajar D, Zahra C, Parascandalo R, Grech V, DeGiovanni JV. Aortic stenting for neonatal coarctation of the aorta - when should this be considered?. Images Paediatr Cardiol. 2016 Jul-Sep. 18 (3):1-4. [QxMD MEDLINE Link]. [Full Text].
  8. Kailin JA, Santos AB, Yilmaz Furtun B, Sexson Tejtel SK, Lantin-Hermoso R. Isolated coarctation of the aorta in the fetus: a diagnostic challenge. Echocardiography. 2017 Dec. 34 (12):1768-75. [QxMD MEDLINE Link].
  9. Report of the New England Regional Infant Cardiac Program. Pediatrics. 1980 Feb. 65 (2 pt 2):375-461. [QxMD MEDLINE Link].
  10. Suradi H, Hijazi ZM. Current management of coarctation of the aorta. Glob Cardiol Sci Pract. 2015. 2015 (4):44. [QxMD MEDLINE Link]. [Full Text].
  11. Galinanes EL, Krajcer Z. Endovascular treatment of coarctation and related aneurysms. J Cardiovasc Surg (Torino). 2018 Feb. 59 (1):101-10. [QxMD MEDLINE Link].
  12. Swartz MF, Atallah-Yunes N, Meagher C, et al. Surgical strategy for aortic coarctation repair resulting in physiologic arm and leg blood pressures. Congenit Heart Dis. 2011 Nov-Dec. 6 (6):583-91. [QxMD MEDLINE Link].
  13. Blalock A, Park EA. The surgical treatment of experimental coarctation (atresia) of the aorta. Ann Surg. 1944 Mar. 119 (3):445-56. [QxMD MEDLINE Link].
  14. Crafoord C, Nylin G. Congenital coarctation of the aorta and its surgical treatment. J Thorac Cardiovasc Surg. 1945. 14:347-61.
  15. Vossschulte K. Surgical correction of coarctation of the aorta by an "isthmusplastic" operation. Thorax. 1961 Dec. 16:338-45. [QxMD MEDLINE Link].
  16. Waldhausen JA, Nahrwold DL. Repair of coarctation of the aorta with a subclavian flap. J Thorac Cardiovasc Surg. 1966 Apr. 51 (4):532-3. [QxMD MEDLINE Link].
  17. Kenny D, Cao QL, Kavinsky C, Hijazi ZM. Innovative resource utilization to fashion individualized covered stents in the setting of aortic coarctation. Catheter Cardiovasc Interv. 2011 Sep 1. 78 (3):413-8. [QxMD MEDLINE Link].
  18. Meadows J, Minahan M, McElhinney DB, McEnaney K, Ringel R, for the COAST Investigators*. Intermediate outcomes in the prospective, multicenter Coarctation of the Aorta Stent Trial (COAST). Circulation. 2015 May 12. 131 (19):1656-64. [QxMD MEDLINE Link].
  19. Suarez de Lezo J, Romero M, Pan M, et al. Stent repair for complex coarctation of aorta. JACC Cardiovasc Interv. 2015 Aug 24. 8 (10):1368-79. [QxMD MEDLINE Link].
  20. Szkutnik M, Sulik S, Fiszer R, Chodor B, Głowacki J, Bialkowski J. Native aortic coarctation stenting in patients ≥ 46 years old. Postepy Kardiol Interwencyjnej. 2017. 13 (4):302-6. [QxMD MEDLINE Link].
  21. 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. [QxMD MEDLINE Link].
  22. [Guideline] Van Hare GF, Ackerman MJ, Evangelista JA, et al. Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: Task force 4: Congenital heart disease: a scientific statement from the American Heart Association and American College of Cardiology. Circulation. 2015 Dec 1. 132 (22):e281-91. [QxMD MEDLINE Link]. [Full Text].
  23. [Guideline] Mitchell JH, Haskell W, Snell P, Van Camp SP. Task Force 8: classification of sports. J Am Coll Cardiol. 2005 Apr 19. 45 (8):1364-7. [QxMD MEDLINE Link]. [Full Text].
  24. [Guideline] Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Adults with Congenital Heart Disease). Circulation. 2008 Dec 2. 118(23):2395-451. [QxMD MEDLINE Link]. [Full Text].
  25. Brown ML, Burkhart HM, Connolly HM, et al. Coarctation of the aorta: lifelong surveillance is mandatory following surgical repair. J Am Coll Cardiol. 2013 Sep 10. 62 (11):1020-5. [QxMD MEDLINE Link].
  26. Abbruzzese PA, Aidala E. Aortic coarctation: an overview. J Cardiovasc Med (Hagerstown). 2007 Feb. 8 (2):123-8. [QxMD MEDLINE Link].
  27. 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. [QxMD MEDLINE Link].
  28. Butera G, Piazza L, Chessa M, et al. Covered stents in patients with complex aortic coarctations. Am Heart J. 2007 Oct. 154 (4):795-800. [QxMD MEDLINE Link].
  29. Celermajer DS, Greaves K. Survivors of coarctation repair: fixed but not cured. Heart. 2002 Aug. 88 (2):113-4. [QxMD MEDLINE Link].
  30. Connolly HM. Pregnancy in women with coarctation of the thoracic aorta. ACC Curr J Rev. 1997. 55:6-7.
  31. Fawzy ME, Awad M, Hassan W, Al Kadhi Y, Shoukri M, Fadley F. Long-term outcome (up to 15 years) of balloon angioplasty of discrete native coarctation of the aorta in adolescents and adults. J Am Coll Cardiol. 2004 Mar 17. 43 (6):1062-7. [QxMD MEDLINE Link].
  32. Golden AB, Hellenbrand WE. Coarctation of the aorta: stenting in children and adults. Catheter Cardiovasc Interv. 2007 Feb 1. 69 (2):289-99. [QxMD MEDLINE Link].
  33. Harlan JL, Doty DB, Brandt B 3rd, Ehrenhaft JL. Coarctation of the aorta in infants. J Thorac Cardiovasc Surg. 1984 Dec. 88 (6):1012-9. [QxMD MEDLINE Link].
  34. Hornung TS, Benson LN, McLaughlin PR. Interventions for aortic coarctation. Cardiol Rev. 2002 May-Jun. 10 (3):139-48. [QxMD MEDLINE Link].
  35. Karl TR. Surgery is the best treatment for primary coarctation in the majority of cases. J Cardiovasc Med (Hagerstown). 2007 Jan. 8 (1):50-6. [QxMD MEDLINE Link].
  36. Konen E, Merchant N, Provost Y, McLaughlin PR, Crossin J, Paul NS. Coarctation of the aorta before and after correction: the role of cardiovascular MRI. AJR Am J Roentgenol. 2004 May. 182 (5):1333-9. [QxMD MEDLINE Link].
  37. Perloff JK, ed. The Clinical Recognition of Congenital Heart Disease. 3rd ed. Philadelphia, Pa: WB Saunders and Co; 1987. 125-60.
  38. Ramnarine I. Role of surgery in the management of the adult patient with coarctation of the aorta. Postgrad Med J. 2005 Apr. 81 (954):243-7. [QxMD MEDLINE Link].
  39. Rothman A. Coarctation of the aorta: an update. Curr Probl Pediatr. 1998 Feb. 28 (2):33-60. [QxMD MEDLINE Link].
  40. Toro-Salazar OH, Steinberger J, Thomas W, Rocchini AP, Carpenter B, Moller JH. Long-term follow-up of patients after coarctation of the aorta repair. Am J Cardiol. 2002 Mar 1. 89 (5):541-7. [QxMD MEDLINE Link].
  41. Varma C, McLaughlin PR, Hermiller JB, Tavel ME. Coarctation of the aorta in an adult: problems of diagnosis and management. Chest. 2003 May. 123 (5):1749-52. [QxMD MEDLINE Link].
  42. von Schulthess GK, Higashino SM, Higgins SS, Didier D, Fisher MR, Higgins CB. Coarctation of the aorta: MR imaging. Radiology. 1986 Feb. 158 (2):469-74. [QxMD MEDLINE Link].
  43. Webb G. Treatment of coarctation and late complications in the adult. Semin Thorac Cardiovasc Surg. 2005 Summer. 17 (2):139-42. [QxMD MEDLINE Link].
  44. Reller MD, Strickland MJ, Riehle-Colarusso T, Mahle WT, Correa A. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr. 2008 Dec. 153 (6):807-13. [QxMD MEDLINE Link]. [Full Text].
  45. Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002 Jun 19. 39 (12):1890-900. [QxMD MEDLINE Link]. [Full Text].
  46. Cinteza EE, Filip C, Bogdan A, Nicolescu AM, Mahmoud H. Atretic aortic coarctation - transradial approach. Case series and review of the literature. Rom J Morphol Embryol. 2017. 58 (3):1029-33. [QxMD MEDLINE Link].
  47. Gounley J, Chaudhury R, Vardhan M, et al. Does the degree of coarctation of the aorta influence wall shear stress focal heterogeneity?. Conf Proc IEEE Eng Med Biol Soc. 2016 Aug. 2016:3429-32. [QxMD MEDLINE Link].

Author

Sandy N Shah, DO, MBA, FACC, FACP, FACOI Cardiologist

Sandy N Shah, DO, MBA, FACC, FACP, FACOI is a member of the following medical societies: American College of Cardiology, American College of Osteopathic Internists, American College of Physicians, American Medical Association, American Osteopathic Association, American Society of Nuclear Cardiology, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Coauthor(s)

Arti N Shah, MD, MS, FACC, FACP, CEPS-AC, CEDS Assistant Professor of Medicine, Mount Sinai School of Medicine; Director of Electrophysiology, Elmhurst Hospital Center and Queens Hospital Center

Arti N Shah, MD, MS, FACC, FACP, CEPS-AC, CEDS is a member of the following medical societies: American Association of Cardiologists of Indian Origin, American College of Cardiology, American College of Physicians, American Heart Association, Cardiac Electrophysiology Society, European Heart Rhythm Society, European Society of Cardiology, Heart Rhythm Society, New York Academy of Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Yasmine S Ali, MD, MSCI, FACC, FACP Assistant Clinical Professor of Medicine, Vanderbilt University School of Medicine; President, LastSky Writing, LLC

Yasmine S Ali, MD, MSCI, FACC, FACP is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Heart Association, American Medical Association, American Medical Writers Association, National Lipid Association, Tennessee Medical Association

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