Surgical Approach to Coarctation of the Aorta and Interrupted Aortic Arch Workup

  • Author: Theodore C Koutlas, MD; Chief Editor: John Kupferschmid, MD   more...
 
Updated: Apr 16, 2012
 

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.
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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. See the image below. Aortic coarctation visualized by aortic angiographAortic 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. See the image below. 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.[14]
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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.
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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.
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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.[15]
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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, Thoracic Surgeon, St. Francis Cardiovascular & Thoracic Associates

David M Maziarz 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  Fellow, Trauma, Surgical Critical Care, and Acute Care Surgery, University of Louisville School of Medicine, Assistant Clinical Instructor, Department of Surgery, University of Louisville Hospital

Katie Love, MD is a member of the following medical societies: American College of Surgeons, Eastern Association for the Surgery of Trauma, and Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Mary C Mancini, MD, PhD  Professor and Chief of Cardiothoracic Surgery, Department of Surgery, Louisiana State University School of Medicine in Shreveport

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.

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.

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Aortic coarctation visualized by aortic angiography.
Aortic coarctation visualized by MRI.
 
 
 
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