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Echocardiography of coarctation of the aorta, aortic arch hypoplasia, and arch interruption: strategies for evaluation of the aortic arch

Published online by Cambridge University Press:  02 February 2017

Suma P. Goudar*
Ward Family Heart Center, Children’s Mercy Hospital, Kansas City, Missouri, United States of America
Sanket S. Shah
Ward Family Heart Center, Children’s Mercy Hospital, Kansas City, Missouri, United States of America
Girish S. Shirali
Ward Family Heart Center, Children’s Mercy Hospital, Kansas City, Missouri, United States of America
Correspondence to: S. P. Goudar, MD, 2401 Gillham Rd, Kansas City, MO 64108, United States of America. Tel: +1 816 234 3255; Fax: +1 816 302 9987; E-mail:



Echocardiography is the modality of choice for the diagnosis and serial follow-up of aortic arch pathology. In this article, we review the types of obstruction of the aortic arch, various classification schemes of coarctation of the aorta and interrupted aortic arch, methodology for optimal echocardiographic imaging of the aortic arch, and key echocardiographic measurements for accurate diagnosis of obstruction and hypoplasia of the aortic arch. Finally, we will discuss the limitations of echocardiography in optimal imaging of the aortic arch and the use of other non-invasive imaging modalities such as CT or MRI to provide additional information in these cases.


Coarctation of the aorta is the more common lesion of the two, with an estimated incidence of four in every 10,000 live births in the United States of America. Interrupted aortic arch is rarer, with an incidence of 19 per one million live births.1 There is a spectrum of pathology of obstruction of the aortic arch, ranging from coarctation of the aorta with and without hypoplasia of the arch to interrupted aortic arch. Both these lesions are frequently encountered in congenital cardiology practice, and will be discussed in the remainder of this article. Obstruction of the aortic arch in the setting of hypoplastic left heart structures or atresia of the aortic valve is beyond the scope of this review and will not be discussed further.

Review Articles
© Cambridge University Press 2017 

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1. Bjornard, K, Riehle-Colarusso, T, Gilboa, SM, Correa, A. Patterns in the prevalence of congenital heart defects, metropolitan Atlanta, 1978 to 2005. Birth Defects Res A Clin Mol Teratol 2013; 97: 8794.CrossRefGoogle ScholarPubMed
2. Bonnet, LM. Congenital stenosis of the aorta. Rev Med Paris 1903; 23: 108.Google Scholar
3. Van Praagh, R, O’Connor, B, Chacko, KA. Aortic coarctation. Pathology of the malformation. First World Congress of Pediatric Cardiac Surgery, Bergamo, June 1988, P5 (abstract).Google Scholar
4. Amanto, JJ, Galdieri, RJ, Cotroneo, JV. Role of extended aortoplasty related to the definition of coarctation of the aorta. Ann Thorac Surg 1991; 52: 615620.CrossRefGoogle Scholar
5. Lopez, L, Colan, SD, Frommelt, PC, Ensing, GJ, Kendall, K, Younoszai, AK, Lai, WW, Geva, T. Recommendations for quantification methods during the performance of a pediatric echocardiogram: a report from the pediatric measurements writing group of the American Society of Echocardiography Pediatric and Congential Heart Disease Council. J Am Soc Echocardiogr 2010; 23: 465495.Google Scholar
6. Parthiban, A, Shirali, GS. Assessment of the structure and function of the aorta by echocardiography. Cardiol Young (Invited review article) 2016.CrossRefGoogle Scholar
7. Cantinotti, M, Scalese, M, Murzi, B, et al. Echocardiographic nomograms for ventricular valvular and arterial dimensions in Caucasian children with a special focus on neonates, infants and toddlers’. J Am Soc Echocardiogr 2014; 27: 179191.CrossRefGoogle ScholarPubMed
8. Pettersen, MD, Du, W, Skerens, ME, Humes, RA. Regression equations for calculation of z-scores of cardiac structures in a large cohort of healthy infants, children and adolescents: an echocardiographic study. J Am Soc Echocardiogr 2008; 21: 922934.CrossRefGoogle Scholar
9. Quezada, E, Lapidus, J, Shauqunessy, R, Chen, Z, Silberbach, M. Aortic dimensions in Turner syndrome. Am J Med Genet A 2015; 167A: 25272532.CrossRefGoogle ScholarPubMed
10. Celoria, GC, Patton, RB. Congenital absence of the aortic arch. Am Heart J 1959; 58: 407.CrossRefGoogle ScholarPubMed
11. Mcrindle, BW, Tchervenkov, CI, Kostantinov, IE, et al. Risk factors associated with mortality and interventions in 472 neonates with interrupted aortic arch: a Congenital Heart Surgeons Society study. J Thorac Cardiovasc Surg 2005; 129: 343350.CrossRefGoogle Scholar

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