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Coarctation and Interrupted Aortic Arch
Michael D. Puchalski, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Coarctation of aorta (CoA)
              • Interrupted aortic arch (IAA)
            • Definitions

              • Coarctation
                • Narrowing of aortic arch
                • Can be discrete or long segment
                  • Discrete typically has narrowing at aortic isthmus
                    • Part of aorta distal to left subclavian take-off and proximal to insertion of ductus arteriosus
              • IAA
                • Ascending aorta supplies one or all head vessels
                • Ductus arteriosus is patent, supplies lower body

            IMAGING

            • General Features

              • Echocardiographic Findings

                • Imaging Recommendations

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      • Staging, Grading, & Classification

                        CLINICAL ISSUES

                        • Presentation

                          • Demographics

                            • Natural History & Prognosis

                              • Treatment

                                DIAGNOSTIC CHECKLIST

                                • Consider

                                  • Image Interpretation Pearls

                                    Selected References

                                    1. Gómez-Montes E et al: Gestational age-specific scoring systems for the prediction of coarctation of the aorta. Prenat Diagn. 34(12):1198-206, 2014
                                    2. Gray RG et al: Long-term follow-up of neonatal coarctation and left-sided cardiac hypoplasia. Am J Cardiol. 111(9):1351-4, 2013
                                    3. Ungerleider RM et al: Contemporary patterns of surgery and outcomes for aortic coarctation: an analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database. J Thorac Cardiovasc Surg. 145(1):150-7; discussion 157-8, 2013
                                    4. Jowett V et al: Sonographic predictors of surgery in fetal coarctation of the aorta. Ultrasound Obstet Gynecol. 40(1):47-54, 2012
                                    5. Quartermain MD et al: Left ventricle to right ventricle size discrepancy in the fetus: the presence of critical congenital heart disease can be reliably predicted. J Am Soc Echocardiogr. 22(11):1296-301, 2009
                                    6. Matsui H et al: Morphological and physiological predictors of fetal aortic coarctation. Circulation. 118(18):1793-801, 2008
                                    7. McBride KL et al: NOTCH1 mutations in individuals with left ventricular outflow tract malformations reduce ligand-induced signaling. Hum Mol Genet. 17(18):2886-93, 2008
                                    8. Puchalski MD et al: Follow-up of aortic coarctation repair in neonates. J Am Coll Cardiol. 44(1):188-91, 2004
                                    9. Surerus E et al: Turner's syndrome in fetal life. Ultrasound Obstet Gynecol. 22(3):264-7, 2003
                                    10. Towbin JA et al: Molecular determinants of left and right outflow tract obstruction. Am J Med Genet. 97(4):297-303, 2000
                                    Related Anatomy
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                                    Related Differential Diagnoses
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                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Imaging

                                        • Pathology

                                          • Clinical Issues

                                            • Diagnostic Checklist

                                              TERMINOLOGY

                                              • Abbreviations

                                                • Coarctation of aorta (CoA)
                                                • Interrupted aortic arch (IAA)
                                              • Definitions

                                                • Coarctation
                                                  • Narrowing of aortic arch
                                                  • Can be discrete or long segment
                                                    • Discrete typically has narrowing at aortic isthmus
                                                      • Part of aorta distal to left subclavian take-off and proximal to insertion of ductus arteriosus
                                                • IAA
                                                  • Ascending aorta supplies one or all head vessels
                                                  • Ductus arteriosus is patent, supplies lower body

                                              IMAGING

                                              • General Features

                                                • Echocardiographic Findings

                                                  • Imaging Recommendations

                                                    DIFFERENTIAL DIAGNOSIS

                                                      PATHOLOGY

                                                      • General Features

                                                        • Staging, Grading, & Classification

                                                          CLINICAL ISSUES

                                                          • Presentation

                                                            • Demographics

                                                              • Natural History & Prognosis

                                                                • Treatment

                                                                  DIAGNOSTIC CHECKLIST

                                                                  • Consider

                                                                    • Image Interpretation Pearls

                                                                      Selected References

                                                                      1. Gómez-Montes E et al: Gestational age-specific scoring systems for the prediction of coarctation of the aorta. Prenat Diagn. 34(12):1198-206, 2014
                                                                      2. Gray RG et al: Long-term follow-up of neonatal coarctation and left-sided cardiac hypoplasia. Am J Cardiol. 111(9):1351-4, 2013
                                                                      3. Ungerleider RM et al: Contemporary patterns of surgery and outcomes for aortic coarctation: an analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database. J Thorac Cardiovasc Surg. 145(1):150-7; discussion 157-8, 2013
                                                                      4. Jowett V et al: Sonographic predictors of surgery in fetal coarctation of the aorta. Ultrasound Obstet Gynecol. 40(1):47-54, 2012
                                                                      5. Quartermain MD et al: Left ventricle to right ventricle size discrepancy in the fetus: the presence of critical congenital heart disease can be reliably predicted. J Am Soc Echocardiogr. 22(11):1296-301, 2009
                                                                      6. Matsui H et al: Morphological and physiological predictors of fetal aortic coarctation. Circulation. 118(18):1793-801, 2008
                                                                      7. McBride KL et al: NOTCH1 mutations in individuals with left ventricular outflow tract malformations reduce ligand-induced signaling. Hum Mol Genet. 17(18):2886-93, 2008
                                                                      8. Puchalski MD et al: Follow-up of aortic coarctation repair in neonates. J Am Coll Cardiol. 44(1):188-91, 2004
                                                                      9. Surerus E et al: Turner's syndrome in fetal life. Ultrasound Obstet Gynecol. 22(3):264-7, 2003
                                                                      10. Towbin JA et al: Molecular determinants of left and right outflow tract obstruction. Am J Med Genet. 97(4):297-303, 2000