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Aortopulmonary Septal Defect Treatment & Management

  • Author: Barry A Love, MD; Chief Editor: Stuart Berger, MD  more...
Updated: Feb 05, 2015

Medical Care

Medical palliation of aortopulmonary septal defect (APSD) may be performed for several days to weeks to allow elective surgical scheduling. Because an aortopulmonary window does not spontaneously close, surgical repair is necessary to prevent the development of pulmonary vascular obstructive disease (PVOD). Meanwhile, digoxin and diuretics may provide some symptomatic benefit before surgical repair.

In rare instances (eg, active sepsis), postponing surgery may be desired. In this situation, provide medical therapy (ie, digoxin, inotropic drugs, diuretics) for a brief time in anticipation of surgery.

The effect of vasodilator agents (eg, angiotensin-converting enzyme [ACE] inhibitors, phosphodiesterase inhibitors, nitrates) is uncertain because these drugs all affect pulmonary resistance in addition to systemic resistance.

Intubation and positive pressure ventilation with permissive hypercarbia and limiting inspired oxygen concentration to 21% may help limit pulmonary blood flow in infants with torrential pulmonary flow requiring medical palliation. Consider using lower inspired oxygen concentrations (15-19%) to elevate pulmonary vascular resistance (PVR) in more extreme cases. Sedation and muscle relaxants may prove necessary to limit spontaneous ventilation.

Previous common practice consisted of medically treating a small infant with congestive heart failure (CHF) with the expectation that he or she would grow and become a "better" surgical candidate. This approach is seldom successful because caloric demands of an infant with CHF typically exceed the amount of nutrition delivered by even the most aggressive means.

Case reports and small case series report closure of small aortopulmonary septal defects in the cardiac catheterization (CC) lab. The Rashkind double umbrella device,[21] the Amplatzer duct occluder,[22] the Amplatzer septal occluder, muscular ventricular septal defect (VSD) occluder, and perimembranous VSD occluder have all been used to close small (type I) defects.[23] The limiting factor to catheter closure of these defects is the anatomy. Only relatively small defects with circumferential tissue rims are amenable to transcatheter device closure, limiting this therapeutic option to a relatively small number of patients. See the image below.

Angiogram of a small-to-moderate aortopulmonary se 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.

Important considerations

The following are essential issues to keep in mind:

  • Early recognition of serious heart disease with associated pulmonary hypertension and high pulmonary vascular resistance (PVR) is important, as irreversible pulmonary vascular obstructive disease (PVOD) may develop (one of the most feared scenarios in pediatrics and pediatric cardiology)
  • Realization that heart diseases, including large ventricular septal defects (VSDs), patent ductus arteriosus (PDA), and aortopulmonary septal defect (APSD), may present in this fashion (When PVR does not fall after birth, children may not have symptoms of congestive heart failure [CHF] and may feed and grow normally with a paucity of cardiac findings.)
  • Recognition of loud and single second heart sounds that indicate the existence of pulmonary hypertension, thereby warranting further workup
  • Ensuring clearly discernment of a splitting of the second heart sound with respiration on each cardiac examination on every child, and referral of the patient to a pediatric cardiologist for evaluation if the heart sounds are suspicious
  • Identification of aortopulmonary septal defect as the cause of a large left-to-right shunt causing CHF


Consult a pediatric cardiologist for diagnosis. Then, refer the patient to a competent cardiovascular surgical team experienced in the repair of congenital heart disease.


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

Diet and activity

A high-calorie formula may be needed for infants with CHF perioperatively. Generally, activity is not restricted in patients with this defect, except in those with Eisenmenger syndrome.


Surgical Care

Various surgical techniques allow correction of this lesion.[5, 24, 25, 26, 27, 28]

Most centers use techniques that involve cardiopulmonary bypass.

The aorta and pulmonary artery may be divided, and the defects in the walls may be closed primarily or with patch material. Alternatively, the aorta or pulmonary artery may be opened and the defect patched using autologous, homologous, xenograft, or synthetic material. Two larger case series have reported that transaortic repair is associated with a more favorable outcome and has less risk of causing late pulmonary artery stenosis.

Other defects may be addressed at the same operation; however, in rare instances, a staged approach may be undertaken.

Specific techniques for unique anatomy must be individualized.

A retrospective review (2002-2011) reported good outcomes associated with the use of a single pericardial patch technique for primary repair of an aortopulmonary window with an interrupted aortic arch.[29] In 6 of 11 patients, the interrupted aortic arch was type A; the remaining 5 patients had type B. Median age at the time of surgery was 11 days, with a m ean weight of 2.6 kg; mean follow-up was 6 ± 3 years. There were no early and late deaths nor reoperations. There was one case of postoperative stroke without late sequelae. All patients at last follow-up visit had no recurrent aortic arch obstruction or pulmonary artery branch stenosis.[29]

Postoperatively, evidence of a good surgical repair should be confirmed.

Residual anatomic problems may be anticipated from preoperative anatomy and include, but not be limited to, pulmonary artery stenosis or distortion, residual left-to-right shunt at the aortopulmonary septal defect site, and ascending aortic obstruction or distortion. Postoperative data should be consistent with a complete repair.

If a pulmonary artery catheter was left in place, it should indicate low pulmonary artery pressure and pulmonary artery oxygen saturation less than 80%.

An elevated pulmonary artery pressure may indicate pulmonary artery vasoreactivity or a persistent left-to-right shunt. Pulmonary artery saturation and left atrial pressure should differentiate the two conditions. If concerns persist, transthoracic or transesophageal echocardiography may be informative. Rarely, CC may be needed to detect residual abnormalities. A case report indicated successful transcatheter closure of a late-onset residual shunt following repair of an aortopulmonary septal defect with the use of a muscular ventricular septal occlude.[30]

Apart from anatomic concerns, an older infant or child with elevated preoperative PVR is at risk for postoperative pulmonary hypertension that may require aggressive management.

Inhaled nitric oxide may be useful in the management of postoperative pulmonary hypertension by acting as a selective pulmonary arteriolar vasodilator.

Other drugs such as sildenafil or calcium channel blockers may provide ongoing pulmonary vasodilatation.

Contributor Information and Disclosures

Barry A Love, MD Assistant Professor , Department of Medicine, Division of Cardiology, Assistant Professor, Division Pediatric Cardiology, Director, Pediatric Electrophysiology Service, Department of Pediatrics, Division of Pediatric Cardiology, Mount Sinai School of Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

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 Emeritus Professor of Pediatrics, 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, Society for Developmental Biology, American Heart Association

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD Medical Director of The Heart Center, Children's Hospital of Wisconsin; Associate Professor, Department of Pediatrics, Section of Pediatric Cardiology, Medical College 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, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Additional Contributors

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, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

  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. 2011 May. 91(5):1473-7. [Medline].

  2. Kutsche LM, Van Mierop LH. Anatomy and pathogenesis of aorticopulmonary septal defect. Am J Cardiol. 1987 Feb 15. 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. 2004 Oct. 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. 1999 Jan. 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. 2004 Feb. 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. 2012 Jan 17. [Medline].

  7. Carminati M, Borghi A, Valsecchi O, et al. Aortopulmonary window coexisting with tetralogy of Fallot: echocardiographic diagnosis. Pediatr Cardiol. 1990 Jan. 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. 2011 Dec 18. [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. 1996 Mar. 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. 2011 Jan. 4(1):41-4. [Medline]. [Full Text].

  11. Nelson AW. Aorticopulmonary window in a dog. J Am Vet Med Assoc. 1986 May 1. 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. 2008 Oct. 18(5):480-4. [Medline].

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

  14. Alvarez R, Garcia-Diaz L, Coserria F, Hosseinpour R, Antinolo 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. 1993 Nov 15. 47(7):1099-103. [Medline].

  16. Bergstrom CS, Saunders RA, Hutchinson AK, Lambert SR. Iris hypoplasia and aorticopulmonary septal defect: a neurocristopathy. J AAPOS. 2005 Jun. 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. 1991 Mar 15. 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. 1992 Jan. 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. 1997 Aug 5. 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. 2008 Jan. 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. 1995 Jun. 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. 2003 Jul. 59(3):402-5. [Medline].

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

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

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

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

  27. Prasad TR, Valiathan MS, Shyamakrishnan KG, Venkitachalam CG. Surgical management of aortopulmonary septal defect. Ann Thorac Surg. 1989 Jun. 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. 1997 Feb. 11(2):293-7. [Medline].

  29. Roubertie F, Kalfa D, Vergnat M, Ly M, Lambert V, Belli E. Aortopulmonary window and the interrupted aortic arch: midterm results with use of the single-patch technique. Ann Thorac Surg. 2015 Jan. 99(1):186-91. [Medline].

  30. Li X, Zhu D, Feng Y. Transcatheter closure of late-onset residual aortopulmonary septal defect using a muscular ventricular septal occluder. Int Heart J. 2014. 55(1):89-91. [Medline].

  31. Nishimura RA, Otto CM, Bonow RO, et al, for the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. 2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol. 2014 Jun 10. 63(22):e57-185. [Medline].

  32. 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. 2008 Aug 19. 118(8):887-96. [Medline]. [Full Text].

  33. 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. 2007 Oct 9. 116(15):1736-54. [Medline]. [Full Text].

  34. 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. 1994 Dec. 106(6):1920-2. [Medline].

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

  36. 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].

  37. McElhinney DB, Reddy VM, Tworetzky W, et al. Early and late results after repair of aortopulmonary septal defect and associated anomalies in infants 1111Am J Cardiol</i>. 1998 Jan 15. 81(2):195-201. [Medline].

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

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

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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