Aortopulmonary Septal Defect Follow-up

  • Author: Barry A Love, MD; Chief Editor: Stuart Berger, MD   more...
 
Updated: Mar 5, 2012
 

Further Inpatient Care

  • If required, preoperative inpatient care of patients with aortopulmonary septal defect (APSD) is directed at managing congestive heart failure (CHF) and completing the diagnostic evaluation in anticipation of surgery.
  • Postoperative care should focus on managing pulmonary hypertension, evaluating for residual defects, and aiding convalescence in anticipation of discharge.
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Further Outpatient Care

  • Provide follow-up care within 1-2 weeks following discharge.
  • Patients are commonly discharged on diuretics, but if a good repair is achieved, most patients can be weaned from cardiac medications soon after discharge.
  • Even in the absence of clinically evident problems, at least one postoperative echocardiogram should be performed during follow-up to evaluate for potentially silent problems.
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Inpatient & Outpatient Medications

  • Endocarditis prophylaxis is no longer recommended for most patients with congenital hart disease with the exception of those who have had prior endocarditis and those with turbulence near patch material.
  • Most patients with repaired and unrepaired aortopulmonary septal defect therefore do not require endocarditis prophylaxis. Refer to the American Heart Association guidelines.[29, 30]
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Transfer

  • Transport patients, if needed, to a facility with the appropriate pediatric and/or pediatric cardiac surgical services.
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Complications

  • The most concerning complication in the repair of aortopulmonary septal defect is perioperative death from pulmonary hypertensive crisis in the child with pulmonary vascular obstructive disease (PVOD).
  • Other generic surgical complications include, but are not limited to, infection, brain injury, and permanent heart block. Although these are considerations, they should be of no higher risk in this procedure than they are in other cardiac procedures using cardiopulmonary bypass.
  • Specific anatomic risks of repair include incomplete closure of the defect and aortic or pulmonary artery distortion. Other complications may relate to repair of associated defects.
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Prognosis

  • Currently, infants with an isolated aortopulmonary septal defect have an excellent prognosis for normal cardiac function and a normal lifestyle.
  • Patients with a more guarded prognosis include older patients with pulmonary resistance of greater than 8 Wood U/m2 at preoperative assessment and those with more complex associated malformations where prognosis depends more on those lesions than on aortopulmonary septal defect.
  • A small incidence of reintervention for stenosis of the great arteries has been reported.
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Patient Education

  • An experienced health care team comprised of nurses, social workers, and spiritual counselors can provide important support to parents of infants with newly diagnosed congenital heart disease.
  • For excellent patient education resources, visit eMedicine's Heart Center. Also, see eMedicine's patient education article Tetralogy of Fallot.
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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.

Specialty Editor Board

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.

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.

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.

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.

References

  1. Bohuta L, Hussein A, Fricke TA, d'Udekem Y, Bennett M, Brizard C, et al. Surgical repair of truncus arteriosus associated with interrupted aortic arch: long-term outcomes. Ann Thorac Surg. May 2011;91(5):1473-7. [Medline].

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

  3. 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].

  4. 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].

  5. 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].

  6. Murin P, Sinzobahamvya N, Blaschczok HC, Photiadis J, Haun C, Asfour B, et al. Aortopulmonary Window Associated with Interrupted Aortic Arch: Report of Surgical Repair of Eight Cases and Review of Literature. Thorac Cardiovasc Surg. Jan 17 2012;[Medline].

  7. 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].

  8. Antonetti I, Lorch D, Coe B, Maxey TS, Nallamshetty L, Dadlani GH, et al. Unrepaired Tetralogy of Fallot with Major Aortopulmonary Collateral Arteries in an Adult Patient. Congenit Heart Dis. Dec 18 2011;[Medline].

  9. 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].

  10. Bajpai P, Shah S, Misri A, Rao S, Suresh P, Maheshwari S. Assessment of operability in d-transposition of great arteries with ventricular septal defect: A practical method. Ann Pediatr Cardiol. Jan 2011;4(1):41-4. [Medline]. [Full Text].

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

  12. 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].

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

  14. Alvarez R, García-Díaz L, Coserria F, Hosseinpour R, Antiñolo G. Aortopulmonary window with atrial septal defect: prenatal diagnosis, management and outcome. Fetal Diagn Ther. 2011;30(4):306-8. [Medline].

  15. 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].

  16. 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].

  17. 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].

  18. 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].

  19. 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].

  20. 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].

  21. 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].

  22. 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].

  23. 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].

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

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

  26. 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].

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

  28. 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].

  29. 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].

  30. 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].

  31. 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].

  32. 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].

  33. 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].

  34. 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].

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

  36. 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].

 
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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.
Angiogram of a small-to-moderate aortopulmonary septal defect in a 4 year-old child. Complete occlusion of the aortopulmonary septal defect with an Amplatzer Duct Occluder. Ao = Ascending aorta; PA = Pulmonary artery.
 
 
 
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