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

Aortopulmonary Septal Defect: Differential Diagnoses & Workup

Author: Barry A Love, MD, Assistant Professor, Department of Medicine, Division of Cardiology, Assistant Professor, Division Pediatric Cardiology, Pediatrics and Medicine, Division of Pediatric Cardiology, Mount Sinai School of Medicine
Contributor Information and Disclosures

Updated: Mar 16, 2009

Differential Diagnoses

Coronary Artery Anomalies
Patent Ductus Arteriosus
Truncus Arteriosus
Ventricular Septal Defect, General Concepts

Workup

Laboratory Studies

  • Preoperatively in patients with an aortopulmonary septal defect (APSD), obtain a CBC count, cross match, and urinalysis.
  • If the infant has been on diuretics and digoxin preoperatively, checking electrolyte and digoxin levels is prudent.
  • Infants with congestive heart failure (CHF) are at risk for potassium depletion from diuretics and may be at risk for digoxin toxicity from routine dosing during decreased renal perfusion. The combination of a volume-loaded circulation with potassium depletion and an elevated serum digoxin level may be a risk factor for ventricular fibrillation at the time of sternotomy.

Imaging Studies

  • Chest radiography: Radiography typically reveals cardiomegaly with increased pulmonary blood flow. Pulmonary congestion is proportional to the degree of CHF.
  • ECG: ECG typically reveals biventricular hypertrophy or right ventricular hypertrophy. Electrical evidence for left atrial enlargement may present.
  • Echocardiography
    • In infants and children, this test (if performed by experienced personnel and reviewed by a skilled pediatric echocardiographer) should reveal the defect and associated abnormalities in virtually all cases.12,13

      Echocardiographic image of a 1-month-old infant w...

      Echocardiographic image of a 1-month-old infant with a large isolated aortopulmonary septal defect (APSD). The image is a parasternal short-axis view just below the pulmonary artery bifurcation. Aorta at this level is to the right and in the same anterior-posterior plane as the main pulmonary artery (MPA). Right pulmonary artery is seen posterior to the aorta at this level, but the origin of the pulmonary arteries is not visible; it is more superior than this axial image. Normally, a complete wall should be visible for both aorta and pulmonary artery. This image shows the absence of that wall, resulting in the large defect between aorta and pulmonary artery.

      [ CLOSE WINDOW ]
      Echocardiographic image of a 1-month-old infant w...

      Echocardiographic image of a 1-month-old infant with a large isolated aortopulmonary septal defect (APSD). The image is a parasternal short-axis view just below the pulmonary artery bifurcation. Aorta at this level is to the right and in the same anterior-posterior plane as the main pulmonary artery (MPA). Right pulmonary artery is seen posterior to the aorta at this level, but the origin of the pulmonary arteries is not visible; it is more superior than this axial image. Normally, a complete wall should be visible for both aorta and pulmonary artery. This image shows the absence of that wall, resulting in the large defect between aorta and pulmonary artery.

    • Doppler color flow mapping shows retrograde flow in the transverse arch during diastole. By contrast, in an infant with a large left-sided patent ductus arteriosus (PDA) and a left aortic arch, diastolic flow in the arch is antegrade. For patients with poor echocardiographic windows, transesophageal echocardiography or cardiac MRI may be an option.14
    • When an aortopulmonary septal defect is found, the echocardiographer should be especially careful to look for other abnormalities, which are present in 50% of cases of aortopulmonary septal defect. In addition to identifying the aortopulmonary septal defect, the ventricular septum, origins of the pulmonary arteries, aortic arch, presence of a PDA, and origins of the coronary arteries should be carefully scrutinized.
    • In addition, 3-dimensional echocardiography has been used to help characterize an aortopulmonary septal defect.15
  • Cardiac catheterization (CC)
    • Cardiac catheterization should not be required if anatomy has been well defined by noninvasive means and if the clinical and noninvasive data are both consistent with low pulmonary vascular resistance (PVR). If questions or additional incompletely characterized defects are noted, careful hemodynamic and angiographic assessment may be helpful.
    • Beyond infancy, CC may be required to exclude irreversible pulmonary vascular obstructive disease (PVOD) before performing surgical repair. In older children with a diagnosis of a nonrestrictive aortopulmonary window, CC is advisable to establish pulmonary vascular reactivity before performing surgical repair. In the rare instance of a small restrictive defect, catheter therapy with device occlusion of the defect may be an option.

More on Aortopulmonary Septal Defect

Overview: Aortopulmonary Septal Defect
Differential Diagnoses & Workup: Aortopulmonary Septal Defect
Treatment & Medication: Aortopulmonary Septal Defect
Follow-up: Aortopulmonary Septal Defect
Multimedia: Aortopulmonary Septal Defect
References
[ CLOSE WINDOW ]

References

  1. Kutsche LM, Van Mierop LH. Anatomy and pathogenesis of aorticopulmonary septal defect. Am J Cardiol. Feb 15 1987;59(5):443-7. [Medline].

  2. Mert M, Paker T, Akcevin A, et al. Diagnosis, management, and results of treatment for aortopulmonary window. Cardiol Young. Oct 2004;14(5):506-11. [Medline].

  3. Dipchand AI, Giuffre M, Freedom RM. Tetralogy of Fallot with non-confluent pulmonary arteries and aortopulmonary septal defect. Cardiol Young. Jan 1999;9(1):75-7. [Medline].

  4. Erez E, Dagan O, Georghiou GP, et al. Surgical management of aortopulmonary window and associated lesions. Ann Thorac Surg. Feb 2004;77(2):484-7. [Medline].

  5. Carminati M, Borghi A, Valsecchi O, et al. Aortopulmonary window coexisting with tetralogy of Fallot: echocardiographic diagnosis. Pediatr Cardiol. Jan 1990;11(1):41-3. [Medline].

  6. Marangi D, Peterson RJ, Ceithaml EL, Marvin WJ Jr. Surgical repair of d-transposition with aortopulmonary window: a case report. J Thorac Cardiovasc Surg. Mar 1996;111(3):671-2. [Medline].

  7. Nelson AW. Aorticopulmonary window in a dog. J Am Vet Med Assoc. May 1 1986;188(9):1055-8. [Medline].

  8. Kiran VS, Singh MK, Shah S, John C, Maheshwari S. Lessons learned from a series of patients with missed aortopulmonary windows. Cardiol Young. Oct 2008;18(5):480-4. [Medline].

  9. Valsangiacomo ER, Smallhorn JF. Images in cardiovascular medicine. Prenatal diagnosis of aortopulmonary window by fetal echocardiography. Circulation. Jun 18 2002;105(24):E192. [Medline].

  10. Waters BL, Allen EF, Gibson PC, Johnston T. Autopsy findings in a severely affected infant with a 2q terminal deletion. Am J Med Genet. Nov 15 1993;47(7):1099-103. [Medline].

  11. Bergstrom CS, Saunders RA, Hutchinson AK, Lambert SR. Iris hypoplasia and aorticopulmonary septal defect: a neurocristopathy. J AAPOS. Jun 2005;9(3):264-7. [Medline].

  12. Balaji S, Burch M, Sullivan ID. Accuracy of cross-sectional echocardiography in diagnosis of aortopulmonary window. Am J Cardiol. Mar 15 1991;67(7):650-3. [Medline].

  13. Horimi H, Hasegawa T, Shiraishi H, et al. Detection of aortopulmonary window with ventricular septal defect by Doppler color flow imaging. Chest. Jan 1992;101(1):280-1. [Medline].

  14. Garver KA, Hernandez RJ, Vermilion RP, Goble M. Images in cardiovascular medicine. Correlative imaging of aortopulmonary window: demonstration with echocardiography, angiography, and MRI. Circulation. Aug 5 1997;96(3):1036-7. [Medline].

  15. Singh A, Mehmood F, Romp RL, Nanda NC, Mallavarapu RK. Live/Real time three-dimensional transthoracic echocardiographic assessment of aortopulmonary window. Echocardiography. Jan 2008;25(1):96-9. [Medline].

  16. Stamato T, Benson LN, Smallhorn JF, Freedom RM. Transcatheter closure of an aortopulmonary window with a modified double umbrella occluder system. Cathet Cardiovasc Diagn. Jun 1995;35(2):165-7. [Medline].

  17. Naik GD, Chandra VS, Shenoy A, et al. Transcatheter closure of aortopulmonary window using Amplatzer device. Catheter Cardiovasc Interv. Jul 2003;59(3):402-5. [Medline].

  18. Trehan V, Nigam A, Tyagi S. Percutaneous closure of nonrestrictive aortopulmonary window in three infants. Catheter Cardiovasc Interv. Feb 15 2008;71(3):405-11. [Medline].

  19. Backer CL, Mavroudis C. Surgical management of aortopulmonary window: a 40-year experience. Eur J Cardiothorac Surg. May 2002;21(5):773-9. [Medline].

  20. Di Bella I, Gladstone DJ. Surgical management of aortopulmonary window. Ann Thorac Surg. Mar 1998;65(3):768-70. [Medline].

  21. Hew CC, Bacha EA, Zurakowski D, et al. Optimal surgical approach for repair of aortopulmonary window. Cardiol Young. Jul 2001;11(4):385-90. [Medline].

  22. Prasad TR, Valiathan MS, Shyamakrishnan KG, Venkitachalam CG. Surgical management of aortopulmonary septal defect. Ann Thorac Surg. Jun 1989;47(6):877-9. [Medline].

  23. Tkebuchava T, von Segesser LK, Vogt PR, et al. Congenital aortopulmonary window: diagnosis, surgical technique and long-term results. Eur J Cardiothorac Surg. Feb 1997;11(2):293-7. [Medline].

  24. Nishimura RA, Carabello BA, Faxon DP, et al. ACC/AHA 2008 guideline update on valvular heart disease: focused update on infective endocarditis: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines: endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation. Aug 19 2008;118(8):887-96. [Medline]. [Full Text].

  25. Wilson W, Taubert KA, Gewitz M, et al. Prevention of infective endocarditis: guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation. Oct 9 2007;116(15):1736-54. [Medline]. [Full Text].

  26. Chopra PS, Reed WH, Wilson AD, Rao PS. Delayed presentation of anomalous circumflex coronary artery arising from pulmonary artery following repair of aortopulmonary window in infancy. Chest. Dec 1994;106(6):1920-2. [Medline].

  27. Grunenfelder J, Zund G, Vogt PR, Turina MI. Aortopulmonary window with anomalous origin of the right coronary artery. Ann Thorac Surg. Jan 1999;67(1):233-5. [Medline].

  28. Izumoto H, Ishihara K, Fujii Y, et al. AP Window and Anomalous Origin of the Right Coronary Artery From the Window. Annals of Thoracic Surgery. 1999;68:557-9. [Medline].

  29. McElhinney DB, Reddy VM, Tworetzky W, et al. Early and late results after repair of aortopulmonary septal defect and associated anomalies in infants <6 months of age. Am J Cardiol. Jan 15 1998;81(2):195-201. [Medline].

  30. Tulloh RM, Rigby ML. Transcatheter umbrella closure of aorto-pulmonary window. Heart. May 1997;77(5):479-80. [Medline].

  31. van Son JA, Puga FJ, Danielson GK, et al. Aortopulmonary window: factors associated with early and late success after surgical treatment. Mayo Clin Proc. Feb 1993;68(2):128-33. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

aortopulmonary septal defect, APSD, aorticopulmonary septal defect, aortopulmonary window, AP window, aortopulmonary fenestration, heart disease, treatment, diagnosis, patent ductus arteriosus, PDA, interrupted aortic arch, ventricular septal defect, VSD, tetralogy of Fallot, aortic atresia, transposition of the great arteries, double aortic arch, congestive heart failure, pulmonary vascular obstructive disease, coarctation of the aorta, cyanosis, hepatomegaly, failure to thrive, VACTERL association

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Barry A Love, MD, Assistant Professor, Department of Medicine, Division of Cardiology, Assistant Professor, Division Pediatric Cardiology, Pediatrics and Medicine, Division of Pediatric Cardiology, Mount Sinai School of Medicine
Disclosure: Nothing to disclose.

Medical Editor

Paul M Seib, MD, Associate Professor of Pediatrics, University of Arkansas for Medical Sciences; Medical Director, Cardiac Catheterization Laboratory, Co-Medical Director, Cardiovascular Intensive Care Unit, Arkansas Children's Hospital
Paul M Seib, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Arkansas Medical Society, International Society for Heart and Lung Transplantation, and Society for Cardiac Angiography and Interventions
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

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.

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

Stuart Berger, MD, Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin
Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.