eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Interrupted Aortic Arch: Treatment & Medication

Author: Alvin J Chin, MD, Professor of Pediatrics, Division of Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine
Contributor Information and Disclosures

Updated: Oct 6, 2009

Treatment

Medical Care

  • Evaluation of interrupted aortic arch (IAA) as an inpatient in an intensive care setting is advised.
  • Intravenous prostaglandin E1 is indicated promptly to maintain patency of the ductus arteriosus.
  • The need for an arterial line and assisted ventilation can be judged best from the initial ABG measurement.

Surgical Care

  • The arch interruption itself is usually treated with side-to-side anastomosis, rather than with conduit interposition. If the subaortic region is of good size, the ventricular septal defect is usually closed with a patch at the same occasion.
  • When a malalignment-type ventricular septal defect is present, the infundibular septum is not only misplaced but is also frequently hypoplastic. Hence, significant subaortic narrowing is frequently difficult to ameliorate with mere resection of infundibular septal muscle.
  • Two alternative approaches have been adopted: the Ross-Konno procedure and the Norwood-Rastelli procedure.
    • In the Ross-Konno procedure, the aortic outflow region is directly enlarged (Konno) and the aortic valve is replaced with a pulmonary valve autograft (Ross).29 The coronary arterial ostia must be relocated to the autograft, and some sort of right ventricle–to–main pulmonary artery conduit is interposed (Ross). One relative contraindication to the Ross-Konno procedure is an unfavorable coronary artery pattern because this may well limit the efficacy of the Konno procedure.
    • In the Norwood-Rastelli procedure, an interventricular baffle allows left ventricular blood to reach not only the aortic outflow but also the pulmonary annulus (Rastelli), and the main pulmonary artery is transected.30 The proximal portion is anastomosed to the ascending aorta (Norwood) while the distal portion is connected to the right ventricle via a conduit (Rastelli).
  • A recent study reported the successful use of a regional cerebral perfusion technique to correct interrupted aortic arch.31

Consultations

  • Cardiothoracic surgeon
  • Cardiologist
  • Geneticist

Diet

  • No special diet is required.

Activity

  • No exercise restrictions are necessary in later childhood if coexistent subaortic (and/or aortic) hypoplasia has been sufficiently relieved in earlier childhood.

Medication

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

Prostaglandins

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


Alprostadil (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.

Adult

Pediatric

Initial infusion: 0.05-0.1 mcg/kg/min IV
Maintenance infusion: 0.01-0.4 mcg/kg/min IV, titrate to the lowest effective dose
Usual maintenance dose: 0.1 mcg/kg/min IV, but reducing the dosage by 50-90% is often possible

Coadministration with heparin may increase aPTT

Respiratory distress syndrome; persistent fetal circulation

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

May cause apnea, seizures, fever, hypotension, pulmonary overcirculation, or inhibition of platelet aggregation; use cautiously in neonates with bleeding tendencies

More on Interrupted Aortic Arch

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References
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References

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  28. 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].

  29. 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].

  30. 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].

  31. 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].

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Further Reading

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Keywords

interrupted aortic arch, IAA, IAA type A, IAA type B, IAA type C, interrupted left aortic arch, nonrestrictive ventricular septal defect, ductus arteriosus, large aortopulmonary window, truncus arteriosus, CHARGE syndrome, coloboma, heart disease, atresia choanae, retarded growth and development, CNS anomalies, genital hypoplasia, deafness, ear anomalies, DiGeorge syndrome, aortic valve, subaortic stenosis, treatment, diagnosis

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Contributor Information and Disclosures

Author

Alvin J Chin, MD, Professor of Pediatrics, Division of Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine
Alvin J Chin, MD is a member of the following medical societies: American Association for the Advancement of Science and American Heart Association
Disclosure: Nothing to disclose.

Medical Editor

Charles I Berul, MD, Professor of Pediatrics, 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 Congential Electrophysiology Society, and Society for Pediatric Research
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

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.

CME Editor

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.

 
 
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