Interrupted Aortic Arch Medication

  • Author: Alvin J Chin, MD; Chief Editor: Steven R Neish, MD, SM   more...
 
Updated: Nov 7, 2011
 

Medication Summary

Preoperatively, administer alprostadil (intravenous [IV] prostaglandin E1) in patients with interrupted aortic arch (IAA). No special medications are required postoperatively.

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Prostaglandins

Class Summary

Alprostadil (PGE1) is used for treatment of ductal dependent cyanotic congenital heart disease, which is due to decreased pulmonary blood flow.

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Alprostadil IV (Prostin VR)

 

Used to maintain patency of the ductus arteriosus in neonates with ductal-dependent congenital heart disease until surgery can be performed. Has direct vasodilatation action on the ductus arteriosus and vascular smooth muscle.

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Contributor Information and Disclosures
Author

Alvin J Chin, MD  Professor of Pediatrics, University of Pennsylvania School of Medicine; Attending Physician, Cardiology Division, Children's Hospital of Philadelphia

Alvin J Chin, MD, is a member of the following medical societies: American Association for the Advancement of Science, American Heart Association, and Society for Developmental Biology

Disclosure: Nothing to disclose.

Specialty Editor Board

Charles I Berul, MD  Professor of Pediatrics and Integrative Systems Biology, George Washington University School of Medicine; Chief, Division of Cardiology, Children's National Medical Center

Charles I Berul, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Cardiac Electrophysiology Society, Heart Rhythm Society, Pediatric and Congenital Electrophysiology Society, and Society for Pediatric Research

Disclosure: Johnson & Johnson Consulting fee Consulting

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.

Ameeta Martin, MD  Clinical Associate Professor, Department of Pediatric Cardiology, University of Nebraska College of Medicine

Ameeta Martin, MD is a member of the following medical societies: American College of Cardiology

Disclosure: Nothing to disclose.

Gilbert Z Herzberg, MD  Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center

Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Chief Editor

Steven R Neish, MD, SM  Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine

Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association

Disclosure: Nothing to disclose.

References

  1. Gruber PJ, Epstein JA. Development gone awry: congenital heart disease. Circ Res. Feb 20 2004;94(3):273-83. [Medline].

  2. Dibardino DJ, Heinle JS, Andropoulos DA, Kerr CD, Morales DL, Fraser CD Jr. Aortic atresia and type B interrupted aortic arch: diagnosis by physiologic cerebral monitoring. Ann Thorac Surg. May 2005;79(5):1758-60. [Medline].

  3. Tannous HJ, Moulick AN, Jonas RA. Interrupted aortic arch and aortic atresia with circle of Willis-dependent coronary perfusion. Ann Thorac Surg. Aug 2006;82(2):e11-3. [Medline].

  4. Decaluwe W, Delhaas T, Gewillig M. Aortic atresia, interrupted aortic arch type C perfused by bilateral arterial duct. Eur Heart J. Nov 2005;26(21):2333. [Medline].

  5. Norwood WI, Stellin GJ. Aortic atresia with interrupted aortic arch: reparative operation. J Thorac Cardiovasc Surg. Feb 1981;81(2):239-44. [Medline].

  6. Jongmans MC, Admiraal RJ, van der Donk KP, Vissers LE, Baas AF, Kapusta L. CHARGE syndrome: the phenotypic spectrum of mutations in the CHD7 gene. J Med Genet. Apr 2006;43(4):306-14. [Medline].

  7. Goldmuntz E, Clark BJ, Mitchell LE. Frequency of 22q11 deletions in patients with conotruncal defects. J Am Coll Cardiol. Aug 1998;32(2):492-8. [Medline].

  8. Marino B, Digilio MC, Persiani M. Deletion 22q11 in patients with interrupted aortic arch. Am J Cardiol. Aug 1 1999;84(3):360-1, A9. [Medline].

  9. Chin AJ, Jacobs ML. Morphology of the ventricular septal defect in two types of interrupted aortic arch. J Am Soc Echocardiogr. Mar-Apr 1996;9(2):199-201. [Medline].

  10. Mulay AV, Watterson KG. Isolated right subclavian artery, interrupted aortic arch, and ventricular septal defect. Ann Thorac Surg. Apr 1997;63(4):1163-5. [Medline].

  11. Arnold JS, Werling U, Braunstein EM, Liao J, Nowotschin S, Edelmann W. Inactivation of Tbx1 in the pharyngeal endoderm results in 22q11DS malformations. Development. Mar 2006;133(5):977-87. [Medline].

  12. Zhang Z, Huynh T, Baldini A. Mesodermal expression of Tbx1 is necessary and sufficient for pharyngeal arch and cardiac outflow tract development. Development. Sep 2006;133(18):3587-95. [Medline].

  13. Iida K, Koseki H, Kakinuma H. Essential roles of the winged helix transcription factor MFH-1 in aortic arch patterning and skeletogenesis. Development. Nov 1997;124(22):4627-38. [Medline].

  14. Winnier GE, Kume T, Deng K. Roles for the winged helix transcription factors MF1 and MFH1 in cardiovascular development revealed by nonallelic noncomplementation of null alleles. Dev Biol. Sep 15 1999;213(2):418-31. [Medline].

  15. Jerome LA, Papaioannou VE. DiGeorge syndrome phenotype in mice mutant for the T-box gene, Tbx1. Nat Genet. Mar 2001;27(3):286-91. [Medline].

  16. Lindsay EA, Vitelli F, Su H. Tbx1 haploinsufficieny in the DiGeorge syndrome region causes aortic arch defects in mice. Nature. Mar 1 2001;410(6824):97-101. [Medline].

  17. Merscher S, Funke B, Epstein JA. TBX1 is responsible for cardiovascular defects in velo-cardio-facial/DiGeorge syndrome. Cell. Feb 23 2001;104(4):619-29. [Medline].

  18. Feiner L, Webber AL, Brown CB, Lu MM, Jia L, Feinstein P. Targeted disruption of semaphorin 3C leads to persistent truncus arteriosus and aortic arch interruption. Development. Aug 2001;128(16):3061-70. [Medline].

  19. Gitler AD, Lu MM, Epstein JA. PlexinD1 and semaphorin signaling are required in endothelial cells for cardiovasculardevelopment. Dev Cell. Jul 2004;7(1):107-16. [Medline].

  20. Yanagisawa H, Hammer RE, Richardson JA. Role of Endothelin-1/Endothelin-A receptor-mediated signaling pathway in the aortic arch patterning in mice. J Clin Invest. Jul 1 1998;102(1):22-33. [Medline].

  21. Makki N, Capecchi MR. Cardiovascular defects in a mouse model of HOXA1 syndrome. Hum Mol Genet. Oct 4 2011;[Medline].

  22. Todorovic V, Frendewey D, Gutstein DE, Chen Y, Freyer L, Finnegan E. Long form of latent TGF-beta binding protein 1 (Ltbp1L) is essential for cardiac outflow tract septation and remodeling. Development. Oct 2007;134(20):3723-32. [Medline].

  23. Li J, Zhu X, Chen M, Cheng L, Zhou D, Lu MM. Myocardin-related transcription factor B is required in cardiac neural crest for smooth muscle differentiation and cardiovascular development. Proc Natl Acad Sci U S A. Jun 21 2005;102(25):8916-21. [Medline].

  24. Cleary JO, McCue K, Price AN, et al. Micro-MRI phenotyping of a novel double-knockout mouse model of congenital heart disease. J Cardio Mag Res. 2010;12(Suppl 1):P1.

  25. Guo C, Sun Y, Zhou B, et al. A Tbx1-Six1/Eya1-Fgf8 genetic pathway controls mammalian cardiovascular and craniofacial morphogenesis. J Clin Invest. Apr 1 2011;121(4):1585-95. [Medline]. [Full Text].

  26. High FA, Jain R, Stoller JZ, et al. Murine Jagged1/Notch signaling in the second heart field orchestrates Fgf8 expression and tissue-tissue interactions during outflow tract development. J Clin Invest. Jul 2009;119(7):1986-96. [Medline]. [Full Text].

  27. Huang ZP, Chen JF, Regan JN, et al. Loss of microRNAs in neural crest leads to cardiovascular syndromes resembling human congenital heart defects. Arterioscler Thromb Vasc Biol. Dec 2010;30(12):2575-86. [Medline]. [Full Text].

  28. Nie X, Wang Q, Jiao K. Dicer activity in neural crest cells is essential for craniofacial organogenesis and pharyngeal arch artery morphogenesis. Mech Dev. Mar-Apr 2011;128(3-4):200-7. [Medline].

  29. Lepore JJ, Mericko PA, Cheng L, Lu MM, Morrisey EE, Parmacek MS. GATA-6 regulates semaphorin 3C and is required in cardiac neural crest for cardiovascular morphogenesis. J Clin Invest. Apr 2006;116(4):929-39. [Medline].

  30. Singh N, Trivedi CM, Lu M, et al. Histone Deacetylase 3 Regulates Smooth Muscle Differentiation in Neural Crest Cells and Development of the Cardiac Outflow Tract. Circ Res. Sep 29 2011;[Medline].

  31. van der Flier A, Badu-Nkansah K, Whittaker CA, et al. Endothelial alpha5 and alphav integrins cooperate in remodeling of the vasculature during development. Development. Jul 2010;137(14):2439-49. [Medline]. [Full Text].

  32. Watanabe Y, Miyagawa-Tomita S, Vincent SD, et al. Role of mesodermal FGF8 and FGF10 overlaps in the development of the arterial pole of the heart and pharyngeal arch arteries. Circ Res. Feb 19 2010;106(3):495-503. [Medline]. [Full Text].

  33. Yagi H, Furutani Y, Hamada H. Role of TBX1 in human del22q11.2 syndrome. Lancet. Oct 25 2003;362(9393):1366-73. [Medline].

  34. Storti S, Vittorini S, Lascone MR, et al. Association between 5,10-methylenetetrahydrofolate reductase C677T and A1298C polymorphisms and conotruncal heart defects. Clin Chem Lab Med. Mar 2003;41(3):276-80. [Medline].

  35. Christiansen J, Dyck JD, Elyas BG, Lilley M, Bamforth JS, Hicks M, et al. Chromosome 1q21.1 contiguous gene deletion is associated with congenital heart disease. Circ Res. Jun 11 2004;94(11):1429-35. [Medline].

  36. Belangero SI, Bellucco FT, Cernach MC, et al. Interrupted aortic arch type B in A patient with cat eye syndrome. Arq Bras Cardiol. May 2009;92(5):e29-31, e56-8. [Medline].

  37. Apfel HD, Levenbraun J, Quaegebeur JM. Usefulness of preoperative echocardiography in predicting left ventricular outflow obstruction after primary repair of interrupted aortic arch with ventricular septal defect. Am J Cardiol. Aug 15 1998;82(4):470-3. [Medline].

  38. Hirooka K, Fraser CD Jr. Ross-Konno procedure with interrupted aortic arch repair in a premature neonate. Ann Thorac Surg. Jul 1997;64(1):249-51. [Medline].

  39. Steger V, Heinemann MK, Irtel von Brenndorff C. Combined Norwood and Rastelli procedure for repair of interrupted aortic arch with subaortic stenosis. Thorac Cardiovasc Surg. Jun 1998;46(3):156-8. [Medline].

  40. Zhang H, Cheng P, Hou J, Li L, Liu H, Liu R, et al. Regional cerebral perfusion for surgical correction of neonatal aortic arch obstruction. Perfusion. Sep 16 2009;[Medline].

  41. Jacobs ML, Chin AJ, Rychik J. Interrupted aortic arch. Impact of subaortic stenosis on management and outcome. Circulation. Nov 1 1995;92(9 Suppl):II128-31. [Medline].

 
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Suprasternal echocardiographic identification of interrupted aortic arch Type B. Upper left: Frontal (coronal) view showing the takeoffs of the innominate artery (InnA) and left common carotid artery (LCCA). No connection is seen with the distal aorta. Upper right: Slightly more dorsal (posterior) frontal view showing the main pulmonary artery (MPA), large left-sided patent ductus arteriosus (PDA), and left-sided upper descending aorta (DescAo). The ascending aorta (AscAo) is not in discernible continuity with the descending aorta. Lower left: Left oblique view showing the LCCA takeoff and no discernible aortic arch. Lower right: sagittal view showing the origin of the left subclavian artery (LSCA) from the DescAo. Other Abbreviations: InnV= innominate vein; l=left; LPA=left pulmonary artery; p,l = posterior and leftward; s=superior; SVC=superior vena cava.
Section A depicts a subcostal frontal echocardiogram of interrupted aortic arch (IAA) type B with transposition of the great arteries. Section B shows a high parasternal echocardiogram showing that the innominate artery (Inn A) and left common carotid artery (LCCA) arise from the ascending aorta (a ao). In section C, the left subclavian artery (LSCA) arises from the descending aorta (desc ao), which is perfused by the ductus arteriosus.
This is the suprasternal sagittal ultrasonographic view of the patient shown in Media file 2. Arch continuity has now been restored by a side-to-side anastomosis. Abbreviations are as follows: a ao = ascending aorta and desc ao = descending aorta.
 
 
 
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