eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiothoracic Surgery

Coarctation of the Aorta and Interrupted Aortic Arch, Surgical Treatment: Workup

Author: Theodore C Koutlas, MD, Assistant Professor, Department of Surgery, Division of Cardiothoracic Surgery, Pitt County Memorial Hospital
Coauthor(s): David M Maziarz, MD, Fellow, Department of Surgery, Wake Forest University Baptist Medical Center; Clifton C Reade, MD, Fellow, Department of Cardiothoracic Surgery, University of Pennsylvania; Katie Love, MD, Resident Physician, Department of Surgery, Pitt County Memorial Hospital, Brody School of Medicine at East Carolina University
Contributor Information and Disclosures

Updated: Jul 21, 2009

Workup

Laboratory Studies

  • ABG analysis may be ordered to look for acidosis, which is common in severe coarctation in a newborn.
  • The results of ABG analysis are nonspecific.

Imaging Studies

  • Chest radiography
    • Chest radiography may reveal little more than left ventricular hypertrophy in a mild case of coarctation. However, several other findings are considered pathognomonic of coarctation.
    • The classic sign of rib notching, which Meckel first described in 1827. This sign may be clearly evident on posteroanterior radiographs. The notching, a result of dilated intercostal arteries eroding the lower edge of the rib, may be absent in older patients who have not developed collaterals. It is usually absent in young patients (usually <5-6 y) who have not had time to develop clinically significant collaterals.
    • The reverse-3 sign is another classic radiologic finding in coarctation. Observed en face, the upper part of the 3 is formed by the dilated proximal segment coming down into the coarcted segment, whereas the bottom portion of the 3 is formed by the coarcted segment exiting into the normal distal segment of the aorta.
  • Chest CT scanning: CT scan of the chest may be useful in evaluating complex abnormalities and in making the diagnosis in adults.
  • Angiography: Angiography was considered the criterion standard. However, CT and MRI have replaced angiography as their resolution has improved.

    Aortic coarctation visualized by aortic angiograp...

    Aortic coarctation visualized by aortic angiography.

    [ CLOSE WINDOW ]
    Aortic coarctation visualized by aortic angiograp...

    Aortic coarctation visualized by aortic angiography.

  • MRI
    • MRI is similar to CT scanning in that it is most helpful in assessing complex abnormalities.
    • The resolution of MRI is better than that of CT scanning; however, the long exposure times necessary for MRI make it a more difficult to perform in infants than a 30-second rapid spiral chest CT scan.

      Aortic coarctation visualized by MRI.

      Aortic coarctation visualized by MRI.

      [ CLOSE WINDOW ]
      Aortic coarctation visualized by MRI.

      Aortic coarctation visualized by MRI.

    • The risks associated with the sedation necessary for adequate imaging likely outweigh any additional benefit.
  • Echocardiography
    • Echocardiography has gained favor in recent years because its resolution has dramatically increased and its processing power has been improved.
    • Two-dimensional echocardiography can demonstrate the site of coarctation and helps in evaluating for other cardiac anomalies.
    • Color Doppler flow can suggest the magnitude of pressure gradients.
    • Because of its portability, accuracy, and noninvasive nature, echocardiography is the diagnostic test of choice in neonates. Neonates seldom require angiographic study, except in rare cases when the area cannot be visualized well and when the abnormality cannot be effectively ruled out.
    • Advances in transesophageal echocardiography have made it the diagnostic test of choice during surgery, and it provides an excellent noninvasive means for postoperative follow-up care.
    • The most important disadvantage of echocardiography is a consequence of its noninvasive nature. Because the pressure gradients are not measured directly, they may not be as accurate as angiographically determined gradients.
  • Intravascular ultrasonography (IVUS): IVUS is an imaging technique recently used in the diagnosis and treatment of coarctation. Capabilities include measurement of the diameter of the aorta, coarctation, and length of the lesion. This information guides the selection and deployment of stents. It also provides a means by which the endovascular repair can be followed.13

Other Tests

  • Electrocardiography
    • Another nonspecific but noninvasive examination, ECG may reflect signs of right, left, or biventricular hypertrophy, or they findings may be entirely normal in older children and adults.
    • Left ventricular hypertrophy with strain is a common late finding in severe coarctation.
  • Cardiac catheterization
    • This invasive examination is not typically considered necessary because coarctation rarely involves the coronary arteries.
    • Patients with coarctation are at risk for coronary disease later in life because of the latent effects of hypertension; therefore, cardiac catheterization may be useful in a patient examined for recoarctation at an older age.

Diagnostic Procedures

  • Considered the most objective method of analysis, angiography and aortography have many benefits; however, less invasive diagnostic techniques have largely replaced these studies.
  • Angiography and aortography reflect the location and extent of the coarctation, it delineates any great-vessel involvement, it facilitates the evaluation of any associated cardiac defects, and it allows for the direct measurement of pressure gradients.
  • Angiography and aortography are particularly useful in evaluating recurrent coarctation because balloon angioplasty can be performed at the time of the procedure if necessary.

Histologic Findings

  • Ductal tissue stains lighter than aortic tissue because of its low elastin levels. In a normal aorta, the inner one third of the elastic lamellae of the aorta merges into the internal elastic lamina of the ductus, whereas the outer two thirds should merge into the adventitia.
  • In coarctation, ductal tissue often encircles the lumen of the aorta. As the ductus attempts to close soon after birth, ductal tissue encroaching on the aorta constricts as well, narrowing the aortic lumen.
  • This ectopic tissue growth is not present in all patients. This observation led to the proposal that coarctation is not a result of abnormal tissue growth, but rather, a result of abnormal fetal blood-flow patterns.14

More on Coarctation of the Aorta and Interrupted Aortic Arch, Surgical Treatment

Overview: Coarctation of the Aorta and Interrupted Aortic Arch, Surgical Treatment
Workup: Coarctation of the Aorta and Interrupted Aortic Arch, Surgical Treatment
Treatment: Coarctation of the Aorta and Interrupted Aortic Arch, Surgical Treatment
Follow-up: Coarctation of the Aorta and Interrupted Aortic Arch, Surgical Treatment
Multimedia: Coarctation of the Aorta and Interrupted Aortic Arch, Surgical Treatment
References
[ CLOSE WINDOW ]

References

  1. Schreiber C, Mazzitelli D, Haehnel JC, et al. The interrupted aortic arch: an overview after 20 years of surgical treatment. Eur J Cardiothorac Surg. Sep 1997;12(3):466-9; discussion 469-70. [Medline].

  2. Celoria GC, Patton RB. Congenital absence of the aortic arch. Am Heart J. 1959;58:408.

  3. Brown JW, Ruzmetov M, Okada Y, Vijay P, Rodefeld MD, Turrentine MW. Outcomes in patients with interrupted aortic arch and associated anomalies: a 20-year experience. Eur J Cardiothorac Surg. May 2006;29(5):666-73; discussion 673-4. [Medline].

  4. Shen I, Ungerleider RM; Ohye RG, Suzuki T, Devaney EJ, Bove EL. Coarctation of the Aorta, Interrupted Aortic Arch Complex. In: Kaiser LR, Kron IL, Spray TL. Mastery of Cardiothoracic Surgery. Second. Philadelphia, PA: Lippincott, Williams and Wilkins; 2007:chap 78-79.

  5. Grech V. Diagnostic and surgical trends, and epidemiology of coarctation of the aorta in a population-based study. Int J Cardiol. Feb 28 1999;68(2):197-202. [Medline].

  6. Van Son JA, Mohr FW, Hess H, et al. Early repair of coarctation of the aorta. Ann Thorac Cardiovasc Surg. Aug 1999;5(4):237-44. [Medline].

  7. Moene RJ, Gittenberger-de Groot AC, Oppenheimer-Dekker A, Bartelings MM. Anatomic characteristics of ventricular septal defect associated with coarctation of the aorta. Am J Cardiol. Apr 15 1987;59(9):952-5. [Medline].

  8. Park JK, Dell RB, Ellis K, Gersony WM. Surgical management of the infant with coarctation of the aorta and ventricular septal defect. J Am Coll Cardiol. Jul 1992;20(1):176-80. [Medline].

  9. Parr GV, Waldhausen JA, Bharati S, et al. Coarctation in Taussig-Bing malformation of the heart. Surgical significance. J Thorac Cardiovasc Surg. Aug 1983;86(2):280-7. [Medline].

  10. Sadow SH, Synhorst DP, Pappas G. Taussig-Bing anomaly and coarctation of the aorta in infancy: surgical options. Pediatr Cardiol. 1985;6(2):83-9. [Medline].

  11. Campbell M. Natural history of coarctation of the aorta. Br Heart J. Sep 1970;32(5):633-40. [Medline].

  12. Shearer WT, Rutman JY, Weinberg WA, Goldring D. Coarctation of the aorta and cerebrovascular accident: a proposal for early corrective surgery. J Pediatr. Dec 1970;77(6):1004-9. [Medline].

  13. Kpodonu J, Ramaiah VG, Diethrich EB. Intravascular ultrasound imaging as applied to the aorta: a new tool for the cardiovascular surgeon. Ann Thorac Surg. Oct 2008;86(4):1391-8. [Medline].

  14. Rudolph AM, Heymann MA, Spitznas U. Hemodynamic considerations in the development of narrowing of the aorta. Am J Cardiol. Oct 1972;30(5):514-25. [Medline].

  15. Ungerleider RM, Ebert PA. Indications and techniques for midline approach to aortic coarctation in infants and children. Ann Thorac Surg. Nov 1987;44(5):517-22. [Medline].

  16. Alsoufi B, Cai S, Coles JG, Williams WG, Van Arsdell GS, Caldarone CA. Outcomes of different surgical strategies in the treatment of neonates with aortic coarctation and associated ventricular septal defects. Ann Thorac Surg. Oct 2007;84(4):1331-6; discussion 1336-7. [Medline].

  17. Kanter KR, Mahle WT, Kogon BE, Kirshbom PM. What is the optimal management of infants with coarctation and ventricular septal defect?. Ann Thorac Surg. Aug 2007;84(2):612-8; discussion 618. [Medline].

  18. Aebert H, Laas J, Bednarski P, et al. High incidence of aneurysm formation following patch plasty repair of coarctation. Eur J Cardiothorac Surg. 1993;7(4):200-4; discussion 205. [Medline].

  19. Kawauchi M, Tada Y, Asano K, Sudo K. Angiographic demonstration of mesenteric arterial changes in postcoarctectomy syndrome. Surgery. Sep 1985;98(3):602-4. [Medline].

  20. Cunningham JN Jr, Laschinger JC, Spencer FC. Monitoring of somatosensory evoked potentials during surgical procedures on the thoracoabdominal aorta. IV. Clinical observations and results. J Thorac Cardiovasc Surg. Aug 1987;94(2):275-85. [Medline].

  21. Seirafi PA, Warner KG, Geggel RL, et al. Repair of coarctation of the aorta during infancy minimizes the risk of late hypertension. Ann Thorac Surg. Oct 1998;66(4):1378-82. [Medline].

  22. Backer CL, Mavroudis C, Zias EA, et al. Repair of coarctation with resection and extended end-to-end anastomosis. Ann Thorac Surg. Oct 1998;66(4):1365-70; discussion 1370-1. [Medline].

  23. Allen BS, Halldorsson AO, Barth MJ, Ilbawi MN. Modification of the subclavian patch aortoplasty for repair of aortic coarctation in neonates and infants. Ann Thorac Surg. Mar 2000;69(3):877-80; discussion 881. [Medline].

  24. Sciolaro C, Copeland J, Cork R, et al. Long-term follow-up comparing subclavian flap angioplasty to resection with modified oblique end-to-end anastomosis. J Thorac Cardiovasc Surg. Jan 1991;101(1):1-13. [Medline].

  25. Fiore AC, Fischer LK, Schwartz T, et al. Comparison of angioplasty and surgery for neonatal aortic coarctation. Ann Thorac Surg. Nov 2005;80(5):1659-64; discussion 1664-5. [Medline].

  26. Cowley CG, Orsmond GS, Feola P, et al. Long-term, randomized comparison of balloon angioplasty and surgery for native coarctation of the aorta in childhood. Circulation. Jun 28 2005;111(25):3453-6. [Medline].

  27. Karl TR. Surgery is the best treatment for primary coarctation in the majority of cases. J Cardiovasc Med (Hagerstown). Jan 2007;8(1):50-6. [Medline].

  28. Al-Ata J, Arfi AM, Hussain A, Kouatly A, Galal MO. Stent angioplasty: an effective alternative in selected infants with critical native aortic coarctation. Pediatr Cardiol. May-Jun 2007;28(3):183-92. [Medline].

  29. Marshall AC, Perry SB, Keane JF, Lock JE. Early results and medium-term follow-up of stent implantation for mild residual or recurrent aortic coarctation. Am Heart J. Jun 2000;139(6):1054-60. [Medline].

  30. Thanopoulos BD, Hadjinikolaou L, Konstadopoulou GN, et al. Stent treatment for coarctation of the aorta: intermediate term follow up and technical considerations. Heart. Jul 2000;84(1):65-70. [Medline].

  31. Kutty S, Greenberg RK, Fletcher S, Svensson LG, Latson LA. Endovascular stent grafts for large thoracic aneurysms after coarctation repair. Ann Thorac Surg. Apr 2008;85(4):1332-8. [Medline].

  32. Abbott ME. Coarctation of the aorta of the adult type. Am. Heart J. 1928;3:574.

  33. Aeba R, Katogi T, Ueda T, et al. Complications following reparative surgery for aortic coarctation or interrupted aortic arch. Surg Today. 1998;28(9):889-94. [Medline].

  34. Bogers AJ, Contant CM, Hokken RB, Cromme-Dijkhuis AH. Repair of aortic arch interruption by direct anastomosis. Eur J Cardiothorac Surg. Jan 1997;11(1):100-4. [Medline].

  35. DeLeon MM, DeLeon SY, Quinones JA, et al. Management of arch hypoplasia after successful coarctation repair. Ann Thorac Surg. Apr 1997;63(4):975-80. [Medline].

  36. Hirooka K, Fraser CD Jr. One-stage neonatal repair of complex aortic arch obstruction or interruption. Recent experience at Texas Children's Hospital. Tex Heart Inst J. 1997;24(4):317-21. [Medline].

  37. Jahangiri M, Shinebourne EA, Zurakowski D, et al. Subclavian flap angioplasty: does the arch look after itself?. J Thorac Cardiovasc Surg. Aug 2000;120(2):224-9. [Medline].

  38. Jenkins NP, Ward C. Coarctation of the aorta: natural history and outcome after surgical treatment. QJM. Jul 1999;92(7):365-71. [Medline].

  39. Jimenez M, Daret D, Choussat A, Bonnet J. Immunohistological and ultrastructural analysis of the intimal thickening in coarctation of human aorta. Cardiovasc Res. Mar 1999;41(3):737-45. [Medline].

  40. Mainwaring RD, Lamberti JJ. Mid- to long-term results of the two-stage approach for type B interrupted aortic arch and ventricular septal defect. Ann Thorac Surg. Dec 1997;64(6):1782-5; discussion 1785-6. [Medline].

  41. O'Connor AR, Moody AR, Ludman CN. Images in cardiology. Aortic coarctation diagnosed by magnetic resonance angiography. Heart. Jun 1999;81(6):671. [Medline].

  42. Ovaert C, McCrindle BW, Nykanen D, et al. Balloon angioplasty of native coarctation: clinical outcomes and predictors of success. J Am Coll Cardiol. Mar 15 2000;35(4):988-96. [Medline].

  43. Reifenstein GH, Levine SA, Gross RE. Coarctation of the aorta: a review of 104 autopsied cases of the "adult type" 2 years of age or older. Am Heart J. 1947;33:146.

  44. Rothman A. Coarctation of the aorta: an update. Curr Probl Pediatr. Feb 1998;28(2):33-60. [Medline].

  45. Saba SE, Nimri M, Shamaileh Q, et al. Balloon coarctation angioplasty: follow-up of 103 patients. J Invasive Cardiol. Aug 2000;12(8):402-6. [Medline].

  46. Sakopoulos AG, Hahn TL, Turrentine M, Brown JW. Recurrent aortic coarctation: is surgical repair still the gold standard?. J Thorac Cardiovasc Surg. Oct 1998;116(4):560-5. [Medline].

  47. Thanopoulos BV, Eleftherakis N, Tzanos K, Skoularigis I, Triposkiadis F. Stent implantation for adult aortic coarctation. J Am Coll Cardiol. Nov 25 2008;52(22):1815-6. [Medline].

  48. Tlaskal T, Hucin B, Hruda J, et al. Results of primary and two-stage repair of interrupted aortic arch. Eur J Cardiothorac Surg. Sep 1998;14(3):235-42. [Medline].

  49. Vitullo DA, DeLeon SY, Graham LC, et al. Extended end-to-end repair and enlargement of the entire arch in complex coarctation. Ann Thorac Surg. Feb 1999;67(2):528-31. [Medline].

  50. Wong CH, Watson B, Smith J. The use of left heart bypass in adult and recurrent coarctation repair. Eur J Cardiothorac Surg. Dec 2001;20(6):1199-201. [Medline].

  51. Wood MK. Acyanotic cardiac lesions with normal pulmonary blood flow. Neonatal Netw. Apr 1998;17(3):5-11. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

coarctation of the aorta and interrupted aortic arch, CoA, coarctation tubular hypoplasia, IAA, interrupted aortic arch, infantile coarctation, adult coarctation, preductal coarctation, postductal coarctation, juxtaductal coarctation, recoarctation, tubular hypoplasia, pseudocoarctation, DiGeorge syndrome, DiGeorge's syndrome, ventricular septal defect, VSD, Turner syndrome, Turner's syndrome, aortopulmonary window, left ventricular outflow tract obstruction, double-outlet right ventricle, truncus arteriosus, bicuspid aortic valve, hypertension, treatment, diagnosis

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Theodore C Koutlas, MD, Assistant Professor, Department of Surgery, Division of Cardiothoracic Surgery, Pitt County Memorial Hospital
Theodore C Koutlas, MD is a member of the following medical societies: American College of Surgeons, Society of Thoracic Surgeons, and Southern Thoracic Surgical Association
Disclosure: Nothing to disclose.

Coauthor(s)

David M Maziarz, MD, Fellow, Department of Surgery, Wake Forest University Baptist Medical Center
David M Maziarz, MD is a member of the following medical societies: American College of Surgeons
Disclosure: Nothing to disclose.

Clifton C Reade, MD, Fellow, Department of Cardiothoracic Surgery, University of Pennsylvania
Clifton C Reade, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Surgeons, Society of Thoracic Surgeons, and Southeastern Surgical Congress
Disclosure: Nothing to disclose.

Katie Love, MD, Resident Physician, Department of Surgery, Pitt County Memorial Hospital, Brody School of Medicine at East Carolina University
Katie Love, MD is a member of the following medical societies: American College of Surgeons
Disclosure: Nothing to disclose.

Medical Editor

Daniel S Schwartz, MD, FACS, Assistant Clinical Professor of Cardiothoracic Surgery, Mount Sinai School of Medicine; Chief of Thoracic Surgery, Huntington Hospital
Daniel S Schwartz, MD, FACS is a member of the following medical societies: American College of Chest Physicians, American College of Surgeons, Society of Thoracic Surgeons, and Western Thoracic Surgical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Mary C Mancini, MD, PhD, Professor, Department of Surgery, Louisiana State University Health Sciences Center
Mary C Mancini, MD, PhD is a member of the following medical societies: American Association for Thoracic Surgery, American College of Surgeons, American Surgical Association, Phi Beta Kappa, Society of Thoracic Surgeons, and Southern Surgical Association
Disclosure: Nothing to disclose.

CME Editor

Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine
Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine
Disclosure: Baxter Honoraria Consulting

Chief Editor

John Kupferschmid, MD, Director of Congenital Heart Surgery, Department of Surgery, Methodist Children's Hospital at San Antonio
John Kupferschmid, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, Society of Thoracic Surgeons, and Society of Thoracic Surgeons
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.